Creating a data dictionary for pediatric autonomic disorders

Our data dictionary is divided into nine sections. The first section is “Signs/Symptoms,” in which the parts of what would be considered the history of the present illness would be obtained. The second section is “Triggers,” which delineates known or suspected events or other factors that initiated the onset of the signs or symptoms. The third section is “Co-morbid Disorders,” denoting various co-occurring diseases, disorders, and syndromes, followed by the fourth section, “Family History,” which lists the diseases and syndromes determined to occur in family members. The fifth and sixth sections are “Medications” and “Medical Devices,” respectively, allowing for recording of pharmacologic and other therapeutic interventions. The seventh section is the specific “Physical Examination Findings” typically associated with these disorders, followed by the extensive eighth section on “Testing,” including laboratory, imaging, and autonomic testing. The last section is on “Diagnoses.” These nine sections are divided in the manner of the documentation of a classic history and physical examination. As the electronic health record advances, these categories may change, further supporting the notion of the data dictionary being a fluid, “living” document that will be prospectively updated every 3–5 years. Terminology concept Definition Data element Permissible values Source of definition Comments Signs/symptoms Objective evidence of disease perceptible to the examining practitioner (sign) and subjective evidence of disease perceived by the patient (symptom) National Cancer Institute (NCI) Thesaurus NCI Thesaurus Code C100104 Cardiovascular Pertaining to the heart or blood vessels NCI Thesaurus NCI Thesaurus Code C25292   Dizziness A nonspecific term indicating a sensation of lightheadedness, unsteadiness, turning, vertigo, or rocking Modified from NCI Thesaurus AND Reilly BM (1990) Dizziness. In: Walker HK, et al. (eds.) Clinical Methods: The History, Physical, and Laboratory Examinations, 3 rd Ed. Butterworth, Boston, Ch. 212 [ 20 ] NCI Thesaurus Code C37943   Lightheadedness A sensation of faintness or wooziness that may be accompanied by visual or auditory changes, diaphoresis, nausea, or tachycardia Modified from NCI Thesaurus NCI Thesaurus Code C118255   Vertigo A sensation of motion as if the external world is moving around the patient, or as if the patient is moving or spinning Modified from NCI Thesaurus NCI Thesaurus Code C38057   Blurred vision The loss of visual acuity (sharpness of vision) resulting in a loss of ability to see small details NCI Thesaurus NCI Thesaurus Code C27123   Tunnel vision Loss of peripheral vision with retention of central field of vision www.medicinenet.com   Blackout A transient loss of vision characterized by perceived complete loss of light and color due to hypoxia and/or hypotension   Grayout A transient loss of vision characterized by a perceived dimming of light and color due to hypoxemia and/or hypotension   Whiteout A transient loss of vision characterized by perceived brightening of light due to hypoxia and/or hypotension   Presyncope An episode of lightheadedness which may precede an episode of syncope Modified from NCI Thesaurus NCI Thesaurus Code C80100   Syncope A transient loss of consciousness and postural tone due to cerebral hypoperfusion, characterized by a rapid onset, short duration, and spontaneous complete recovery Brignole M, et al. Eur Heart J 2018;39:1883–1948 [ 21 ] 2017 AHA/ACC Key Data Elements and Definitions for Ambulatory Electronic Health Records in Pediatric and Congenital Cardiology [ 17 ]   Raynaud phenomenon An episodic vasoconstriction followed by vasodilation resulting in discoloration of the skin and pain in the affected areas, often involving fingers or toes. Classically associated with triphasic color changes (white, blue, red) but may be biphasic. Often occurs in response to cold temperatures or emotional stress. May be primary or secondary to an underlying autoimmune disease Modified from NCI Thesaurus NCI Thesaurus Code C50724   Venous pooling The accumulation of blood in veins of the extremities or splanchnic circulation due to gravitational pull when a person assumes an upright position Also called dependent acrocyanosis   Excessive venous pooling The disproportionate accumulation of blood in the veins when a person changes position from supine to seated position or standing, especially in the lower extremities, that can appear as acrocyanosis with or without edema Stewart JM and Weldon A. J Appl Physiol (1985) 2000;89(4):1505–12 [ 22 ]   Palpitations Sensation of irregular, rapid, and/or forceful beating of the heart Modified from NCI Thesaurus NCI Thesaurus Code C37999   Tachycardia An abnormally rapid heart rate exceeding the 95th percentile for age Modified from NCI Thesaurus NCI Thesaurus Code C38029   Exercise intolerance A condition of inability or decreased ability to perform physical exercise at the normally expected level or duration for people of that age, size, sex, and muscle mass Vissing J. International Neurology 2016 John Wiley &Sons p. 516. Hoboken. [ 23 ]   Neurologic Pertaining to the nerves or nervous system NCI Thesaurus NCI Thesaurus Code C25262   Headache Pain in various parts of the head, not necessarily confined to the area of distribution of any nerve Modified from NCI Thesaurus NCI Thesaurus Code C34661   Migraine Recurrent headache disorder manifesting in attacks lasting 4–72 h. Typical characteristics of the headache are unilateral location, pulsating quality, moderate or severe intensity, aggravation by routine physical activity, and association with nausea and/or photophobia and phonophobia Olesen J, et al. Cephalalgia 2018;38:1–211 [ 24 ]   Tension-type headache Episodes of headache, typically bilateral, pressing or tightening in quality and of mild to moderate intensity, lasting minutes to days. The pain does not worsen with routine physical activity and is not associated with nausea, although photophobia or phonophobia may be present Olesen J, et al. Cephalalgia 2018;38:1–211 [ 24 ]   Cluster headache Attacks of severe, strictly unilateral pain which is orbital, supraorbital, temporal or in any combination of these sites, lasting 15–180 min, and occurring from once every other day to eight times a day. The pain is associated with ipsilateral conjunctival injection, lacrimation, nasal congestion, rhinorrhea, forehead and facial sweating, miosis, ptosis and/or eyelid edema, and/or with restlessness or agitation Olesen J, et al. Cephalalgia 2018;38:1–211 [ 24 ]   New daily persistent headache Persistent headache, daily from its onset, which is clearly remembered. The pain lacks characteristic features, and may be migraine-like or tension-type-like, or have elements of both Olesen J, et al. Cephalalgia 2018;38:1–211 [ 24 ]   Fatigue Overall tiredness and lack of energy that is not improved with rest Modified from NCI Thesaurus NCI Thesaurus Code C3036   Excessive fatigue Overall tiredness and lack of energy disproportionate to the level of exertion   Brain Fog An informal designation used to refer to several different forms of cognitive dysfunction characterized by slowed mentation, memory loss or dysfunction, and/or difficulty concentrating   Insomnia A sleep disorder characterized by difficulty in falling asleep and/or remaining asleep NCI Thesaurus NCI Thesaurus Code C28286   Paresthesia Altered sensation, as in tingling or “pins and needles” NCI Thesaurus NCI Thesaurus Code C50771   Pain An unpleasant sensory and emotional experience associated with, or resembling that associated with, actual or potential tissue damage Raja SN, et al. Pain 2020;161(9):1976–82 [ 25 ]    Myalgia Painful sensation originating from a muscle or group of muscles NCI Thesaurus NCI Thesaurus Code C27009    Arthralgia Pain in a joint NCI Thesaurus NCI Thesaurus Code C50464    Abdominal pain Painful sensation in the abdominal region NCI Thesaurus NCI Thesaurus Code C26682    Pelvic pain Painful sensation in the pelvis NCI Thesaurus NCI Thesaurus Code C40122   Heat intolerance A feeling of being overheated when the surrounding temperature rises https://medlineplus.gov/ency/article/003094.htm   Cold intolerance An abnormal sensitivity to a cold environment or cold temperatures https://medlineplus.gov/ency/article/003095.htm   Diaphoresis A clinical finding in which there is excessive or unpredictable sweating 2017 AHA/ACC Key Data Elements and Definitions for Ambulatory Electronic Health Records in Pediatric and Congenital Cardiology [ 17 ]   Hyperhidrosis Excessive perspiration NCI Thesaurus NCI Thesaurus Code C78345   Hypohidrosis Impaired ability (partial or complete) to produce or deliver sweat to the surface of the skin in the presence of an appropriate stimulus Leung AKC J R Soc Promot Health 1999;119(2):101–7 [ 26 ]   Anhidrosis Lack of sweating or the ability to sweat when provoked by the appropriate stimulus NCI Thesaurus NCI Thesaurus Code C34385   Alacrima Lack of tears due to absence or hyposecretion of tears, or to agenesis or improper development of lacrimal gland ducts and associated structures Adams J et al. Clin Genet 2018;94(1):54–60 [ 27 ]   Hyperpyrexia Extreme elevation of body temperature above normal NCI Thesaurus NCI Thesaurus Code C111817   Xerophthalmia Dryness of the eye(s) due to inadequate production of tears Modified from NCI Thesaurus NCI Thesaurus Code C34503   Numbness Decrease or loss of superficial sensation in an anatomic region of the body Modified from NCI Thesaurus NCI Thesaurus Code C34857   Photophobia Increased sensitivity of the eyes to light, which can result in the avoidance of light exposure NCI Thesaurus NCI Thesaurus Code C28210   Hyperacusis A disorder in which an individual has an abnormally low noise tolerance, and increased sensitivity to sounds NCI Thesaurus NCI Thesaurus Code C116366   Seizure A transient occurrence of signs and/or symptoms due to abnormal excessive or synchronous neuronal activity in the brain Fisher RS, et al. Epilepsia 2005;46(4):470–2 [ 28 ]   Gastrointestinal Pertaining to the upper alimentary tract, stomach, or intestines Modified from NCI Thesaurus NCI Thesaurus Code C13359   Nausea A difficult-to-describe sensation of sick or queasy feeling usually perceived as being in the stomach that can escalate in severity and may precede vomiting Stern RM, Koch KL, Andrews PLR (2001) Nausea: Mechanisms and Management. Oxford University Press, New York [ 29 ]   Vomiting/emesis Forceful ejection of the contents of the stomach through the mouth NCI Thesaurus NCI Thesaurus Code C3442   Early satiety The sensation of feeling full or no longer hungry earlier while consuming a non-oversized meal or after eating less than usual preventing completion of a meal Modified from www.medlineplus.gov   Constipation Difficulties in defecation that include infrequent bowel movements, hard or lumpy stools, excessive straining, sensation of incomplete evacuation or blockage and, in some instances, the use of manual maneuvers to facilitate evacuation Aziz I, et al. Expert Rev Gastroenterol Hepatol 2020;14(1):39–46 [ 30 ]   Diarrhea A disorder characterized by passage of three or more loose or liquid stools per day (or more frequent passage than is normal for the individual) www.who.int/news-room/fact-sheets/detail/diarrhoeal-disease   Abdominal bloating The feeling of increased pressure within the abdomen Iovino P, et al. World J Gastroenterol 2014;20(39):14407–19 [ 31 ]   Abdominal distension Protrusion of the abdomen NCI Thesaurus NCI Thesaurus Code C78161   Visceral hypersensitivity Heightened sensitivity or reduced thresholds for pain and discomfort associated with the gastrointestinal tract Modified from Mertz H. Aliment Pharmacol Ther 2003;17(5):623–33 [ 32 ] AND Faure C, Di Lorenzo C, Thapar N. (2017) Pediatric Neurogastroenterology Gastrointestinal Motility and Functional Disorders in Children, 2 nd Ed. Springer International Publishing, Switzerland [ 33 ] Also called visceral hyperalgesia   Xerostomia Dryness of the oral mucosa secondary to a decrease in saliva production, or a change in saliva composition NCI Thesaurus NCI Thesaurus Code C26917   Dysphagia A symptom referring to difficulty in swallowing NCI Thesaurus NCI Thesaurus Code C2980   Aerophagia An episodic or chronic disorder in which patients swallow large quantities of air which accumulate in the gastrointestinal tract to cause abdominal distention and bloating Bredenoord AJ Clin Gastroenterol Hepatol 2013;11(1):6–12 [ 34 ]   Respiration/control of breathing Pertaining to the organs involved in the exchange of carbon dioxide and oxygen, including the trachea, bronchi, and lung parenchyma Modified from NCI Thesaurus NCI Thesaurus Code C25656   Apnea Transient cessation of respiration lasting longer than 6 s Modified from NCI Thesaurus NCI Thesaurus Code C26698   Obstructive sleep apnea Cessation of air flow during sleep due to upper airway obstruction NCI Thesaurus NCI Thesaurus Code C116337   Dyspnea A subjective experience of shortness of breath   Exertional dyspnea A subjective sense of shortness of breath with physical exertion Pianosi PT Pediatr Exerc Sci 2018;30(4):442–9 [ 35 ]   Cyanosis A bluish or purplish discoloration of the skin and/or mucous membranes resulting from an increased amount of deoxygenated hemoglobin in the blood Modified from NCI Thesaurus NCI Thesaurus Code C26737   Central cyanosis Cyanosis that is noted to occur over the entire body surface area and visible mucosa www.ncbi.nlm.nih.gov   Perioral cyanosis Cyanosis that is noted to occur surrounding the mouth that may or may not include the lips   Peripheral cyanosis Cyanosis that is noted to occur in the distal upper and lower extremities Modified from www.ncbi.nlm.nih.gov Also called acrocyanosis   Breath-holding spell A clinical condition in which there is a brief involuntary cessation of breathing at end expiration in response to emotional or painful stimulus seen in infants, toddlers, and children 2017 AHA/ACC Key Data Elements and Definitions for Ambulatory Electronic Health Records in Pediatric and Congenital Cardiology [ 17 ]   Simple breath-holding spell A clinical condition in which there is a breath-holding spell without syncope 2017 AHA/ACC Key Data Elements and Definitions for Ambulatory Electronic Health Records in Pediatric and Congenital Cardiology [ 17 ]   Complex breath-holding spell A clinical condition in which there is a breath-holding spell associated with syncope 2017 AHA/ACC Key Data Elements and Definitions for Ambulatory Electronic Health Records in Pediatric and Congenital Cardiology [ 17 ]   Pallid breath-holding spell A clinical condition in which there is a breath-holding spell associated with pale appearance 2017 AHA/ACC Key Data Elements and Definitions for Ambulatory Electronic Health Records in Pediatric and Congenital Cardiology [ 17 ]   Cyanotic breath-holding spell A clinical condition in which there is a breath-holding spell associated with cyanotic appearance 2017 AHA/ACC Key Data Elements and Definitions for Ambulatory Electronic Health Records in Pediatric and Congenital Cardiology [ 17 ]   Breath holding Episodic apnea, which is more common in young children and usually resolves by age six NCI Thesaurus NCI Thesaurus Code C121558   Chest-related symptoms   Chest pain Discomfort felt in the upper abdomen, thorax, neck, or shoulders 2017 AHA/ACC Key Data Elements and Definitions for Ambulatory Electronic Health Records in Pediatric and Congenital Cardiology [ 17 ]   Chest pressure Sense of weight or compression felt in the thorax that may radiate to contiguous body parts   Chest wall pain Discomfort felt in the upper abdomen, thorax, neck, or shoulders whose source is musculoskeletal. Pain may be reproducible by palpation   Other systems   Joint hypermobility The capability that a joint (or a group of joints) has to move, passively and/or actively, beyond normal limits along physiological axes Castori M, et al. Am J Med Genet C Semin Med Genet 2017;175(1):148–57 [ 36 ]   Easy bleeding Bleeding that is disproportionate to the offending trauma NCI Thesaurus NCI Thesaurus Code C95552   Easy bruising Appearance of skin bruises following perceived minimal contact or injury NCI Thesaurus NCI Thesaurus Code C119027   Joint subluxation A partial or brief and reversible abnormal separation where two or more bones meet   Joint dislocation An abnormal separation where two or more bones meet NCI Thesaurus NCI Thesaurus Code C26750   Hyperelastic skin Skin that can be stretched beyond what is considered normal, and returns to normal after it is stretched www.medlineplus.gov   Dysmenorrhea Abnormally painful abdominal cramps during menstruation NCI Thesaurus NCI Thesaurus Code C34559   Menorrhagia Heavy bleeding during menstruation Modified from NCI Thesaurus NCI Thesaurus Code C26829   Metrorrhagia Uterine bleeding that occurs during a time not associated with the normal menstrual period NCI Thesaurus NCI Thesaurus Code C34818   Pruritus An intense itching sensation NCI Thesaurus NCI Thesaurus Code C3344   Urticaria A transient, itchy skin eruption characterized by raised pale wheals with a surrounding red flare Modified from NCI Thesaurus NCI Thesaurus Code C3432   Flushing Reddening of the skin from a transient increase in blood flow NCI Thesaurus NCI Thesaurus Code C26775   Dermatographism (Darier sign) Urticaria due to physical allergy in which a pale, raised welt or wheal with a red flare on each side is elicited by stroking or scratching the skin https://medical-dictionary.thefreedictionary.com/dermatographism   Food intolerance An adverse reaction to food(s) in which there is difficulty digesting or metabolizing a particular food not due to an immunologic etiology Bruijnzeel-Koomen C, et al. Allergy 1995;50(8):623–35 [ 37 ]   Food allergies An adverse reaction to food(s) due to an immunologic mechanism Sicherer SH and Sampson HA. J Allergy Clin Immunol 2014;133(2):291–307 [ 38 ] Triggers Something that sets into motion some course of events; a device that activates or releases or causes something to happen NCI Thesaurus NCI Thesaurus Code C54190  Infection The invasion and multiplication of microorganisms such as bacteria,  viruses , and parasites that are not normally present within the body www.medicinenet.com  Epstein–Barr virus A species of Herpetoviridae that is responsible for infectious mononucleosis (glandular fever) NCI Thesaurus NCI Thesaurus Code C14204  Mononucleosis A clinical entity characterized by pharyngitis, cervical lymph node enlargement, fatigue and fever, which results most often from a primary Epstein–Barr virus infection Balfour HH Jr, et al. Clin Transl Immunology 2015;4(2):e33 [ 39 ]  Upper respiratory tract infection An infectious process affecting the upper respiratory tract (nose, paranasal sinuses, pharynx, or larynx) Modified from NCI Thesaurus NCI Thesaurus Code C35650  Influenza An acute viral infection of the respiratory tract, occurring in isolated cases, in epidemics, or in pandemics; it is caused by serologically different strains of viruses (influenza viruses) designated A, B, and C, has a 3-day incubation period, and usually lasts for 3–10 days NCI Thesaurus NCI Thesaurus Code C53482  Gastroenteritis An inflammatory disorder that affects the upper and/or lower gastrointestinal tract Modified from NCI Thesaurus NCI Thesaurus Code C34632  Pneumonia An acute, acute and chronic, or chronic inflammation focally or diffusely affecting the lung parenchyma, due to infections, treatment (e.g., radiation), or exposure (inhalation) to chemicals NCI Thesaurus NCI Thesaurus Code C3333  Sinusitis An acute or chronic inflammatory process affecting the mucous membranes of any sinus cavity NCI Thesaurus NCI Thesaurus Code C35024  Varicella A highly contagious viral infection caused by the varicella zoster virus NCI Thesaurus NCI Thesaurus Code C96407  Severe acute respiratory syndrome coronavirus 2 A positive-sense single-stranded RNA virus in the genus Betacoronavirus NCI Thesaurus NCI Thesaurus Code C169076 Also called SARS-CoV-2, SARS coronavirus 2  COVID-19 infection An acute infection of the respiratory tract that is caused by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) NCI Thesaurus NCI Thesaurus Code C171133  Enterovirus A (+) strand single-strand RNA virus of the picornavirus family NCI Thesaurus NCI Thesaurus Code C14203  Hand, foot, and mouth disease A clinical syndrome that is usually caused by enterovirus infection, and that is characterized by fever, anorexia, and painful sores in the mouth, distal extremities, and/or other sites, including the buttocks NCI Thesaurus NCI Thesaurus Code C128439  Cellulitis Inflammation of the dermis and subcutaneous tissues caused by a bacterial infection NCI Thesaurus NCI Thesaurus Code C26715  Appendicitis Acute inflammation of the vermiform appendix NCI Thesaurus NCI Thesaurus Code C35145  Concussion Concussion is a traumatic brain injury induced by biomechanical forces McCrory P, et al. Br J Sports Med 2017;51(11):877–887 [ 40 ]  Surgery A diagnostic or treatment procedure performed by manual and/or instrumental means, often involving an incision and the removal or replacement of a diseased organ or tissue NCI Thesaurus NCI Thesaurus Code C15329  Menarche Onset of menses NCI Thesaurus NCI Thesaurus Code C3228  Adolescent growth spurt The fast and intense increase in the rate of growth in height and weight that occurs during the pubertal stage of the human life cycle Modified from Soliman A, et al. Indian J Endocrinol Metab 2014;18(suppl 1):S53-S62 [ 41 ] Co-morbid disorders The coexistence of two or more disease processes NCI Thesaurus NCI Thesaurus Code C16457  Syncope A transient loss of consciousness and postural tone due to cerebral hypoperfusion, characterized by a rapid onset, short duration, and spontaneous complete recovery Brignole M, et al. Eur Heart J 2018;39:1883–1948 [ 21 ] 2017 AHA/ACC Key Data Elements and Definitions for Ambulatory Electronic Health Records in Pediatric and Congenital Cardiology [ 17 ]  Reflex syncope A transient loss of consciousness and postural tone due to a reflex response that encompasses vasodilation and/or bradycardia leading to systemic hypotension and cerebral hypoperfusion https://www.uptodate.com/contents/reflex-syncope-in-adults-and-adolescents-treatment 2017 AHA/ACC Key Data Elements and Definitions for Ambulatory Electronic Health Records in Pediatric and Congenital Cardiology [ 17 ] Brignole M, et al. Eur Heart J 2018;39:e43-e80 [ 21 ]  Neurally mediated syncope A type of reflex syncope typically triggered by orthostasis leading to vasodilation and/or bradycardia that includes vasodepressor cardioinhibitory, and mixed syncope Modified from Brignole M, et al. Eur Heart J 2018;39:e43-e80 [ 21 ] Also called vasovagal syncope, neurocardiogenic syncope  Vasodepressor syncope A type of neurally mediated syncope characterized primarily by reflex vasodilation Modified from Brignole M, et al. Eur Heart J 2018;39:e43-e80 [ 21 ]  Cardioinhibitory syncope A type of neurally mediated syncope characterized primarily by reflex bradycardia Modified from Brignole M, et al. Eur Heart J 2018;39:e43-e80 [ 21 ]  Mixed syncope A type of neurally mediated syncope characterized by reflex vasodilation and reflex bradycardia Modified from Brignole M, et al. Eur Heart J 2018;39:e43-e80 [ 21 ]  Situational syncope A type of reflex syncope typically triggered by specific events, such as micturition, gastrointestinal stimulation, cough or sneeze, or exercise Modified from Brignole M, et al. Eur Heart J 2018;39:e43-e80 [ 21 ]  Syncope due to orthostatic hypotension A syncopal event due to an inadequate increase in peripheral resistance and heart rate or due to severe hypovolemia upon standing Modified from Brignole M, et al. Eur Heart J 2018;39:e43-e80 [ 21 ]  Orthostatic hypotension A progressive and sustained drop in blood pressure upon standing from a sitting or supine position in which there is a decrease of systolic blood pressure by ≥ 20 mmHg and/or a decrease of diastolic blood pressure by ≥ 10 mmHg within the first 3 min of upright position Modified from Brignole M, et al. Eur Heart J 2018;39:e43-e80 [ 21 ]  Initial orthostatic hypotension A sudden drop in blood pressure upon standing from a sitting or supine position in which there is a decrease of systolic blood pressure by ≥ 40 mmHg and/or a decrease in diastolic blood pressure by ≥ 20 mmHg within the first 15 s of standing that spontaneously resolves with a relatively rapid return to pre-challenge values Modified from Brignole M, et al. Eur Heart J 2018;39:e43-e80 [ 21 ]  Cardiac syncope A syncopal event due to a primary cardiac etiology, such as an arrhythmia or structural lesion  Postural orthostatic tachycardia syndrome A type of chronic orthostatic intolerance lasting ≥ 3 months associated with excessive upright sinus tachycardia in the absence of orthostatic hypotension, plus a constellation of typically daily symptoms that may include lightheadedness, dizziness, nausea, dyspnea, diaphoresis, headache, fatigue, and other symptoms of autonomic dysfunction. Excessive tachycardia is defined by present consensus as a sustained heart rate increase of at least 30 bpm in adults (40 bpm for adolescents), or a heart rate > 120 bpm (in adults), within 10 min of upright tilt table testing Modified from 2017 AHA/ACC Key Data Elements and Definitions for Ambulatory Electronic Health Records in Pediatric and Congenital Cardiology [ 17 ] Also called POTS  Hyperadrenergic postural orthostatic tachycardia syndrome A phenotype of postural orthostatic tachycardia syndrome in which there is excessive sympathetic vasoconstriction, increased peripheral resistance, and typically excessively high serum catecholamine levels, with greatly elevated upright tachycardia and hypertension  Neuropathic postural orthostatic tachycardia syndrome A phenotype of postural orthostatic tachycardia syndrome in which there is impaired sympathetic activity in the lower extremities or splanchnic vasculature with arteriolar dysregulation  Dysautonomia A condition in which altered function of one or more components of the autonomic nervous system adversely affects health Goldstein DS Principles of Autonomic Medicine v 4.0 [ 42 ] Also called autonomic nervous system dysfunction  Post-COVID-19 syndrome A condition in which there are multiorgan symptoms and complications beyond the initial period of acute infection and illness with SARS-CoV-2 Venkatesan P Lancet Respir Med 2021;9(2):129 [ 43 ] Also called long COVID, long-haul COVID, post-acute sequelae of SARS-CoV-2 infection (PASC)  Myalgic encephalomyelitis-chronic fatigue syndrome A syndrome of unknown etiology characterized by an unexplained persistent or relapsing chronic fatigue that is of at least 6 months’ duration, is not the result of ongoing exertion, is not substantially alleviated by rest, and results in substantial reduction of previous levels of occupational, educational, social, or personal activities NCI Thesaurus NCI Thesaurus Code C3037  Inappropriate sinus tachycardia A sinus heart rate > 100 bpm at rest (with a mean 24-h heart rate > 90 bpm not due to primary causes) and is associated with distressing symptoms of palpitations Sheldon RS Heart Rhythm 2015;12(6):e41-e63 [ 44 ]  Hypertension The average clinic-measured systolic and/or diastolic blood pressure ≥ 95th percentile for age, sex, and height percentiles Flynn JT Pediatrics 2017;140(3):e20171904 [ 45 ]  Hypotension Low systolic blood pressure, as defined by age parameters: 0–1 year: < 5th percentile for gestational and/or postnatal age 1–10 years:  10 years: < 90/50 mmHg Dionne J Hypertension 2021;77(3):781–787 [ 46 ] Kleinman ME Circulation 2010;122(18Suppl3):S876-S908 [ 47 ]  Celiac disease An autoimmune genetic disorder with an unknown pattern of inheritance that damages the small intestine due to intolerance to dietary gluten Modified from NCI Thesaurus NCI Thesaurus Code C26714  Thyroid disease A disorder in which there is abnormal function of the thyroid gland due to causes such as autoimmune disease, pituitary disease, toxins, or malignancy  Hashimoto thyroiditis An autoimmune disorder caused by the production of autoantibodies against thyroid tissue. There is progressive destruction of the thyroid follicles leading to hypothyroidism NCI Thesaurus NCI Thesaurus Code C27191  Graves disease Hyperthyroidism associated with diffuse hyperplasia of the thyroid gland (goiter), resulting from production of antibodies that are directed against the thyrotropin receptor complex of the follicular epithelial cells. As a result, the thyroid gland enlarges and secretes increased amounts of thyroid hormones NCI Thesaurus NCI Thesaurus Code C3071  Eosinophilic esophagitis Allergic inflammation of the esophagus. Morphologically, it is characterized by the presence of eosinophils infiltrating the esophageal epithelium NCI Thesaurus NCI Thesaurus Code C27105  Diabetes mellitus A metabolic disorder characterized by abnormally high blood sugar levels due to diminished production of insulin or insulin resistance/desensitization NCI Thesaurus NCI Thesaurus Code C2985  Type 1 diabetes mellitus A chronic condition characterized by minimal or absent production of insulin by the pancreas NCI Thesaurus NCI Thesaurus Code C2986  Type 2 diabetes mellitus A type of diabetes mellitus that is characterized by insulin resistance or desensitization and increased blood glucose levels NCI Thesaurus NCI Thesaurus Code C26747  Inflammatory bowel disease A spectrum of small and large bowel inflammatory diseases of unknown etiology. It includes Crohn’s disease, ulcerative colitis, microscopic colitis, and colitis of indeterminate type Modified from NCI Thesaurus NCI Thesaurus Code C3138  Crohn’s disease A gastrointestinal disorder characterized by chronic inflammation involving all layers of the intestinal wall, noncaseating granulomas affecting the intestinal wall and regional lymph nodes, and transmural fibrosis NCI Thesaurus NCI Thesaurus Code C2965  Ulcerative colitis An inflammatory bowel disease involving the mucosal surface of the large intestine and rectum NCI Thesaurus NCI Thesaurus Code C2952  Irritable bowel syndrome Gastrointestinal symptoms characterized by chronic abdominal pain and altered bowel habits in the absence of any organic cause. One of the functional gastrointestinal disorders Modified from NCI Thesaurus NCI Thesaurus Code C82343  Visceral hypersensitivity Heightened sensitivity or reduced thresholds for pain and discomfort associated with the gastrointestinal tract Modified from Mertz H. Aliment Pharmacol Ther 2003;17(5):623–33 [ 32 ] AND Faure C, Di Lorenzo C, Thapar N. (2017) Pediatric Neurogastroenterology Gastrointestinal Motility and Functional Disorders in Children, 2 nd Ed. Springer International Publishing, Switzerland [ 33 ] Also called visceral hyperalgesia  Joint hypermobility The capability that a joint (or a group of joints) has to move, passively and/or actively, beyond normal limits along physiological axes Castori M, et al. Am J Med Genet C Semin Med Genet 2017;175(1):148–57 [ 36 ]  Ehlers–Danlos syndrome A group of inherited connective tissue disorders characterized by loose and fragile skin and joint hypermobility NCI Thesaurus NCI Thesaurus Code C34568 Also called EDS   Hypermobile EDS A type of Ehlers–Danlos syndrome characterized by generalized joint hypermobility, plus systemic manifestations of a generalized connective tissue disorder, positive family history, and/or musculoskeletal complications, plus exclusion of other types of Ehlers–Danlos syndrome Malfait F, et al. Am J Med Genet C Semin Med Genet 2017;175(1):8–26 [ 48 ] Also called hEDS   Classical EDS A type of Ehlers–Danlos syndrome characterized by skin hyperextensibility, generalized joint hypermobility, plus other minor criteria associated with a mutation in collagen in the COL5A1 , COL5A2 , COL1A1 , or COL1A2 genes Malfait F, et al. Am J Med Genet C Semin Med Genet 2017;175(1):8–26 [ 48 ] Also called cEDS   Vascular EDS A type of Ehlers–Danlos syndrome characterized by arterial, uterine, or gastrointestinal rupture at a young age, atraumatic formation of carotid-cavernous fistula, plus other minor criteria associated with a mutation in collagen in the COL3A1 gene Malfait F, et al. Am J Med Genet C Semin Med Genet 2017;175(1):8–26 [ 48 ] Also called vEDS  Hypermobility spectrum disorder A group of clinically relevant conditions related to joint hypermobility in which phenotypic domains are limited to the musculoskeletal system Castori M, et al. Am J Med Genet C Semin Med Genet 2017;175(1):148–57 [ 36 ] Also called HSD  Mast cell activation syndrome A mast cell activation disorder in which there are episodic multisystem symptoms consistent with mast cell activation, appropriate response to medications targeting mast cell activation, and documented increase in validated markers of mast cell activation Akin C. J Allergy Clin Immunol 2017;140(2):349–55 [ 49 ] Also called MCAS  Median arcuate ligament syndrome A condition characterized by abdominal pain attributed to compression of the celiac artery and the celiac ganglia by the median arcuate ligament www.wikipedia.org Also called MALS  Amplified musculoskeletal pain syndrome A condition in which the nerves register normal stimuli (lack of tissue injury) as pain, resulting in feelings of intense pain with even minor sensory input across the entire body Modified from NCI Thesaurus NCI Thesaurus Code C119048  Chronic regional pain syndrome A chronic (lasting longer than 6 months) pain condition that most often affects one limb usually after an injury characterized by prolonged or excessive pain and changes in skin color, temperature, and/or swelling in the affected area www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Complex-Regional-Pain-Syndrome-Fact-Sheet  Cyclic vomiting syndrome A condition characterized by recurrent, self-limiting stereotypical episodes of vomiting associated with intense nausea, pallor, and lethargy. One of the functional gastrointestinal disorders, it can be a migraine precursor Modified from NCI Thesaurus NCI Thesaurus Code C117014  Anorexia nervosa A disorder characterized by a refusal to maintain a minimally normal body weight, an intense fear of gaining weight, a disturbance in body image, and, in postmenarchal females, the development of amenorrhea Modified from NCI Thesaurus NCI Thesaurus Code C34387  Bulimia nervosa A disorder characterized by recurrent episodes of binge-eating over which the individual feels a lack of control; these episodes of binge-eating are followed by recurrent compensatory behavior to prevent weight gain, usually self-induced vomiting NCI Thesaurus NCI Thesaurus Code C34440  Binge-eating disorder A disorder characterized by recurrent episodes of binge-eating without the affected individual’s effort to make up for the binge episodes by excessively exercising, purging, and/or inappropriately using medications, like diet pills or laxatives www.medicinenet.com  Avoidant/restrictive food intake disorder An eating or feeding disturbance (e.g., apparent lack of interest in eating or food; avoidance based on the sensory characteristics of food; concern about aversive consequences of eating) as manifested by persistent failure to meet appropriate nutritional and/or energy needs associated with one (or more) of the following: 1. Significant weight loss (or failure to achieve expected weight gain or faltering growth in children) 2. Significant nutritional deficiency 3. Dependence on enteral feeding or oral nutritional supplements 4. Marked interference with psychosocial functioning DSM-V  Overweight Suggested by having a high amount of body fat in which the body mass index is between 25 and 29.9 kg/m 2 (adult criteria), or at/above the 85th up to 95th percentile for age and gender. This is confirmed by other more precise methods Modified from NCI Thesaurus PLUS Centers for Disease Control NCI Thesaurus Code C94250  Obesity Suggested by having a high amount of body fat in which the body mass index (BMI) is 30 kg/m 2 or more (adult criteria), or at/above the 95th percentile for age and gender. This is confirmed by other more precise methods Modified from NCI Thesaurus PLUS Centers for Disease Control NCI Thesaurus Code C3283  Anxiety disorder A category of psychiatric disorders which are characterized by anxious feelings or fear often accompanied by physical symptoms associated with anxiety NCI Thesaurus NCI Thesaurus Code C2878  Panic disorder An anxiety disorder characterized by multiple unexpected panic attacks with persistent concern of recurring attacks NCI Thesaurus NCI Thesaurus Code C34890  Depression A mental condition marked by ongoing feelings of sadness, despair, loss of energy, and difficulty dealing with normal daily life NCI Thesaurus NCI Thesaurus Code C2982  Obsessive–compulsive disorder A disorder characterized by the presence of persistent and recurrent irrational thoughts (obsessions), resulting in marked anxiety and repetitive excessive behaviors (compulsions) as a way to try to decrease that anxiety NCI Thesaurus NCI Thesaurus Code C88411  Attention deficit/hyperactivity disorder A neurodevelopmental disorder defined by impairing levels of inattention, disorganization, and/or hyperactivity–impulsivity DSM-V Also called ADHD  Attention deficit/hyperactivity disorder, combined presentation ADHD in which both inattention and hyperactivity–impulsivity are met for the past 6 months DSM-V  Attention deficit/hyperactivity disorder, predominantly inattentive presentation ADHD in which inattention is met but hyperactivity–impulsivity is not met for the past 6 months DSM-V  Attention deficit/hyperactivity disorder, predominantly hyperactive–impulsive presentation ADHD in which hyperactivity–impulsivity is met but inattention is not met for the past 6 months DSM-V  Oppositional defiant disorder A behavior disorder characterized by a persistent pattern of defiant, disobedient, and hostile behavior towards authority figures, manifested by a frequent loss of temper, arguing, becoming angry or vindictive, or other negativistic behaviors NCI Thesaurus NCI Thesaurus Code C92565  Somatic symptom disorder A. One or more somatic symptoms that are distressing or result in significant disruption of daily life B. Excessive thoughts, feelings, or behaviors related to the somatic symptoms or associated health concerns C. Although any one somatic symptom may not be continuously present, the state of being symptomatic is persistent (typically more than 6 months) DSM-V  Conversion disorder (functional neurological symptom disorder) A. One or more symptoms of altered voluntary motor or sensory function B. Clinical findings provide evidence of incompatibility between the symptom and recognized neurological or medical conditions C. The symptom or deficit is not better explained by another medical or mental disorder D. The symptom or deficit causes clinically significant distress or impairment in social, occupational, or other important areas of functioning or warrants medical evaluation DSM-V  Functional gastrointestinal disorders A group of gastrointestinal disorders that occur in which, after appropriate medical evaluation, the symptoms cannot be attributed to another medical condition. This includes functional nausea and vomiting disorders, functional abdominal pain disorders, and functional defecation disorders Adapted from Hyams JS, et al. Gastroenterology 2016; S0016-5085(16)00181-5 [ 50 ]  Sjögren syndrome An autoimmune disorder affecting the salivary and lacrimal glands. Morphologically, it is characterized by the presence of lymphocytic and plasmacytic infiltrates that cause destruction of these glands NCI Thesaurus NCI Thesaurus Code C26883  Systemic lupus erythematosus An autoimmune multiorgan disease typically associated with vasculopathy and autoantibody production NCI Thesaurus NCI Thesaurus Code C3201  Juvenile idiopathic arthritis A group of chronic, inflammatory childhood disorders of unknown etiology that primarily involve joints NCI Thesaurus NCI Thesaurus Code C114357  Polycystic ovary syndrome A disorder that manifests as multiple cysts on the ovaries. It results in hormonal imbalances and leads to irregular and abnormal menstrual periods, excess growth of hair, acne eruptions, and obesity NCI Thesaurus NCI Thesaurus Code C26862  Endometriosis The growth of functional endometrial tissue in anatomic sites outside the uterine body NCI Thesaurus NCI Thesaurus Code C3014  Rapid-onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation A neurocristopathy characterized by hypothalamic dysfunction with inability to maintain normal salt–water balance, growth hormone deficiency, hypothyroidism, early or late puberty, increased prolactin concentration, hypoventilation, attenuated/absent peripheral and central chemoreceptor responsiveness, and autonomic dysfunction Modified from NCI Thesaurus NCI Thesaurus Code C121944 Also called ROHHAD  Congenital central hypoventilation syndrome A neurocristopathy associated with heterozygous PHOX2B gene mutations characterized by hypoventilation and hypoxemia typically during sleep with attenuated/absent peripheral and central chemoreceptor responsiveness, and autonomic dysregulation; symptoms typically appear in the newborn period and can co-occur with Hirschsprung disease and neural crest tumors Modified from NCI Thesaurus NCI Thesaurus Code C98889 Also called CCHS  Tracheostomy A surgically created external opening into the trachea NCI Thesaurus NCI Thesaurus Code C122643  Hereditary and sensory neuropathy, type I An autosomal dominant condition caused by mutation(s) in the SPTLC1 gene characterized by distal sensory impairment with variable autonomic and motor involvement NCI Thesaurus NCI Thesaurus Code C170433  Hereditary and sensory neuropathy, type IIA (HSAN2A) A rare congenital disorder caused by various mutations in the WNK1 gene characterized by sensory loss, self-mutilation, and mild or absent autonomic involvement Modified from https://www.ncbi.nlm.nih.gov/books/NBK49247/ and https://www.omim.org/entry/201300  Hereditary and sensory neuropathy, type IIB (HSAN2B, Morvan’s disease; congenital sensory neuropathy) A rare autosomal recessive disorder caused by various mutations in the FAM134B gene characterized by decreased distal sensation and variable autonomic involvement Modified from https://www.ncbi.nlm.nih.gov/books/NBK49247/ and https://www.omim.org/entry/613115  Hereditary and sensory neuropathy, type III (HSAN3, familial dysautonomia; Riley–Day syndrome) A rare congenital disorder caused by a founder mutation in the ELP-1 gene characterized by initial hypotonia and feeding difficulties with subsequent blood pressure instability and hypertensive vomiting attacks alternating with hypotension due to loss of sensory inputs from cranial nerves IX and X resulting in afferent baroreflex failure Modified from https://www.omim.org/entry/223900  Hereditary and sensory neuropathy, type IV (HSAN4, congenital insensitivity to pain with anhidrosis; CIPA) A rare autosomal recessive disorder caused by different mutations in the NTRK1 gene affecting peripheral sensory and autonomic fibers, and is characterized by inability to feel pain and temperature that leads to repeated unintentional self-injuries, severe cognitive impairment, and anhidrosis that leads to hyperpyrexia and febrile seizures Modified from NCI Thesaurus and https://www.omim.org/entry/256800 NCI Thesaurus Code C118633  Hereditary and sensory neuropathy, type V (HSAN5) An autosomal recessive condition caused by mutations in the NGF or NTRK1 genes affecting peripheral small fibers characterized by loss of pain sensation, particularly in the extremities, and variable anhidrosis, with normal cognitive function as compared with HSAN4 Modified from NCI Thesaurus and https://www.omim.org/entry/608654 NCI Thesaurus Code C156360  Hereditary and sensory neuropathy, type VI (HSAN6) An extremely rare inherited disorder caused by mutations in the DST gene characterized by neonatal expression of diminished pain and labile blood pressure https://www.ncbi.nlm.nih.gov/medgen/761278 and https://www.omim.org/entry/614653  Hereditary and sensory neuropathy, type VII (HSAN7) A rare disorder caused by mutations in the SCN11A gene, characterized by insensitivity to pain as well as gastrointestinal dysfunction Modified from NCI Thesaurus and https://www.omim.org/entry/615548 NCI Thesaurus Code C125388  Hereditary and sensory neuropathy, type VIII (HSAN8) An autosomal recessive disorder caused by mutations in the PRDM12 gene characterized by congenital insensitivity to pain; anhidrosis and alacrima may occur Modified from https://www.ncbi.nlm.nih.gov/medgen/894363 and https://www.omim.org/entry/616488  Rett syndrome A progressive neurologic disorder caused by mutations in the MECP2 gene on chromosome X. It almost exclusively affects girls. It is characterized by breath-holding spells, flushing, repetitive hand motions, lack of advancement in language, and regression in developmental milestones. Other signs and symptoms include slowed head growth, scoliosis, breathing abnormalities (including sleep-disordered breathing), and sleep disturbances Modified from NCI Thesaurus NCI Thesaurus Code C75488  Hirschsprung disease A congenital disorder characterized by the absence of myenteric ganglion cells in the distal colon resulting in a functional stenosis of the distal colon and a massive distention of the proximal colon NCI Thesaurus NCI Thesaurus Code C34700  Neuroblastoma A neuroblastic tumor characterized by the presence of neuroblastic cells, the absence of ganglion cells, and the absence of a prominent Schwannian stroma formation NCI Thesaurus NCI Thesaurus Code C3270  Ganglioneuroma A benign neuroblastic tumor of the sympathetic nervous system that occurs in childhood. Ganglioneuroma typically arises from the sympathetic chain in the mediastinum or adrenal glands Modified from NCI Thesaurus NCI Thesaurus Code C3049  Ganglioneuroblastoma A neuroblastic tumor characterized by the presence of neuroblastic cells, ganglion cells, and a stroma with Schwannian differentiation constituting more than 50% of the tumor volume NCI Thesaurus NCI Thesaurus Code C3790  Paraganglioma A benign or malignant neoplasm arising from paraganglia located along the sympathetic or parasympathetic nerves, including carotid body tumors Modified from NCI Thesaurus NCI Thesaurus Code C3308  Breath-holding spell A clinical condition in which there is a brief involuntary cessation of breathing at end expiration in response to emotional or painful stimulus seen in infants, toddlers, and children 2017 AHA/ACC Key Data Elements and Definitions for Ambulatory Electronic Health Records in Pediatric and Congenital Cardiology [ 17 ]  Simple breath-holding spell A clinical condition in which there is a breath-holding spell without syncope 2017 AHA/ACC Key Data Elements and Definitions for Ambulatory Electronic Health Records in Pediatric and Congenital Cardiology [ 17 ]  Complex breath-holding spell A clinical condition in which there is a breath-holding spell associated with syncope 2017 AHA/ACC Key Data Elements and Definitions for Ambulatory Electronic Health Records in Pediatric and Congenital Cardiology [ 17 ]  Pallid breath-holding spell A clinical condition in which there is a breath-holding spell associated with pale appearance 2017 AHA/ACC Key Data Elements and Definitions for Ambulatory Electronic Health Records in Pediatric and Congenital Cardiology [ 17 ]  Cyanotic breath-holding spell A clinical condition in which there is a breath-holding spell associated with cyanotic appearance 2017 AHA/ACC Key Data Elements and Definitions for Ambulatory Electronic Health Records in Pediatric and Congenital Cardiology [ 17 ]  Seizure disorder A brain disorder characterized by episodes of abnormally increased neuronal discharge resulting in transient episodes of sensory or motor neurological dysfunction, or psychic dysfunction that may or may not be associated with loss of consciousness or convulsions NCI Thesaurus NCI Thesaurus Code C3020 Also called epilepsy  Spinal cord injury Traumatic damage of the spinal cord NCI Thesaurus NCI Thesaurus Code C50750  Autonomic dysreflexia A form of paroxysmal autonomic adrenergic overactivity due to disinhibited spinal reflexes in spinal cord injury above the T6 level Novak P. Am J Med 2019;132(4):420–436 [ 51 ] Family history A record of a patient’s background regarding health and disease events of blood relatives NCI Thesaurus NCI Thesaurus Code C17726  Syncope A transient loss of consciousness and postural tone due to cerebral hypoperfusion, characterized by a rapid onset, short duration, and spontaneous complete recovery Brignole M, et al. Eur Heart J 2018;39:1883–1948 [ 21 ] 2017 AHA/ACC Key Data Elements and Definitions for Ambulatory Electronic Health Records in Pediatric and Congenital Cardiology [ 17 ]  Reflex syncope A transient loss of consciousness and postural tone due to a reflex response that encompasses vasodilation and/or bradycardia leading to systemic hypotension and cerebral hypoperfusion https://www.uptodate.com/contents/reflex-syncope-in-adults-and-adolescents-treatment 2017 AHA/ACC Key Data Elements and Definitions for Ambulatory Electronic Health Records in Pediatric and Congenital Cardiology [ 17 ] Brignole M, et al. Eur Heart J 2018;39:e43-e80 [ 21 ]  Neurally mediated syncope A type of reflex syncope typically triggered by orthostasis leading to vasodilation and/or bradycardia that includes vasodepressor cardioinhibitory, and mixed syncope Modified from Brignole M, et al. Eur Heart J 2018;39:e43-e80 [ 21 ] Also called vasovagal syncope, neurocardiogenic syncope  Vasodepressor syncope A type of neurally mediated syncope characterized primarily by reflex vasodilation Modified from Brignole M, et al. Eur Heart J 2018;39:e43-e80 [ 21 ]  Cardioinhibitory syncope A type of neurally mediated syncope characterized primarily by reflex bradycardia Modified from Brignole M, et al. Eur Heart J 2018;39:e43-e80 [ 21 ]  Mixed syncope A type of neurally mediated syncope characterized by reflex vasodilation and reflex bradycardia Modified from Brignole M, et al. Eur Heart J 2018;39:e43-e80 [ 21 ]  Situational syncope A type of reflex syncope typically triggered by specific events, such as micturition, gastrointestinal stimulation, cough or sneeze, or exercise Modified from Brignole M, et al. Eur Heart J 2018;39:e43-e80 [ 21 ]  Syncope due to orthostatic hypotension A syncopal event due to an inadequate increase in peripheral resistance and heart rate or due to severe hypovolemia upon standing Modified from Brignole M, et al. Eur Heart J 2018;39:e43-e80 [ 21 ]  Orthostatic hypotension A progressive and sustained drop in blood pressure upon standing from a sitting or supine position in which there is a decrease of systolic blood pressure by ≥ 20 mmHg and/or a decrease of diastolic blood pressure by ≥ 10 mmHg within the first 3 min of upright position Modified from Brignole M, et al. Eur Heart J 2018;39:e43-e80 [ 21 ]  Initial orthostatic hypotension A sudden drop in blood pressure upon standing from a sitting or supine position in which there is a decrease of systolic blood pressure by ≥ 40 mmHg and/or a decrease in diastolic blood pressure by ≥ 20 mmHg within the first 15 s of standing that spontaneously resolves with a relatively rapid return to pre-challenge values Modified from Brignole M, et al. Eur Heart J 2018;39:e43-e80 [ 21 ]  Cardiac syncope A syncopal event due to a primary cardiac etiology, such as an arrhythmia or structural lesion  Postural orthostatic tachycardia syndrome A type of chronic orthostatic intolerance lasting ≥ 3 months associated with excessive upright sinus tachycardia in the absence of orthostatic hypotension, plus a constellation of typically daily symptoms that may include lightheadedness, dizziness, nausea, dyspnea, diaphoresis, headache, fatigue, and other symptoms of autonomic dysfunction. Excessive tachycardia is defined by present consensus as a sustained heart rate increase of at least 30 bpm in adults (40 bpm for adolescents), or a heart rate > 120 bpm (in adults), within 10 min of upright tilt table testing Modified from 2017 AHA/ACC Key Data Elements and Definitions for Ambulatory Electronic Health Records in Pediatric and Congenital Cardiology [ 17 ] Also called POTS  Hyperadrenergic postural orthostatic tachycardia syndrome A phenotype of postural orthostatic tachycardia syndrome in which there is excessive sympathetic vasoconstriction, increased peripheral resistance, and typically excessively high serum catecholamine levels, with greatly elevated upright tachycardia and hypertension  Neuropathic postural orthostatic tachycardia syndrome A phenotype of postural orthostatic tachycardia syndrome in which there is impaired sympathetic activity in the lower extremities or splanchnic vasculature with arteriolar dysregulation  Dysautonomia A condition in which altered function of one or more components of the autonomic nervous system adversely affects health Goldstein DS Principles of Autonomic Medicine v 4.0 [ 42 ] Also called autonomic nervous system dysfunction  Post COVID-19 Syndrome A condition in which there are multiorgan symptoms and complications beyond the initial period of acute infection and illness with SARS-CoV-2 Venkatesan P Lancet Respir Med 2021;9(2):129 [ 43 ] Also called long COVID, long-haul COVID, post-acute sequelae of SARS-CoV-2 infection (PASC)  Inappropriate sinus tachycardia A sinus heart rate > 100 bpm at rest (with a mean 24-h heart rate > 90 bpm not due to primary causes) and is associated with distressing symptoms of palpitations Sheldon RS Heart Rhythm 2015;12(6):e41-e63 [ 44 ]  Hypertension The average clinic-measured systolic and/or diastolic blood pressure ≥ 95th percentile for age, sex, and height percentiles Flynn JT Pediatrics 2017;140(3):e20171904 [ 45 ]  Myalgic encephalomyelitis-chronic fatigue syndrome A syndrome of unknown etiology characterized by an unexplained persistent or relapsing chronic fatigue that is of at least 6 months’ duration, is not the result of ongoing exertion, is not substantially alleviated by rest, and results in substantial reduction of previous levels of occupational, educational, social, or personal activities NCI Thesaurus NCI Thesaurus Code C3037  Joint hypermobility The capability that a joint (or a group of joints) has to move, passively and/or actively, beyond normal limits along physiological axes Castori M, et al. Am J Med Genet C Semin Med Genet 2017;175(1):148–57 [ 36 ]  Hypermobility spectrum disorders A group of clinically relevant conditions related to joint hypermobility in which phenotypic domains are limited to the musculoskeletal system Castori M, et al. Am J Med Genet C Semin Med Genet 2017;175(1):148–57 [ 36 ] Also called HSD  Ehlers–Danlos syndrome A group of inherited connective tissue disorders characterized by loose and fragile skin and joint hypermobility NCI Thesaurus NCI Thesaurus Code C34568 Also called EDS   Hypermobile EDS A type of Ehlers–Danlos syndrome characterized by generalized joint hypermobility, plus systemic manifestations of a generalized connective tissue disorder, positive family history, and/or musculoskeletal complications, plus exclusion of other types of Ehlers–Danlos syndrome Malfait F, et al. Am J Med Genet C Semin Med Genet 2017;175(1):8–26 [ 48 ] Also called hEDS   Classical EDS A type of Ehlers–Danlos syndrome characterized by skin hyperextensibility, generalized joint hypermobility, plus other minor criteria associated with a mutation in collagen in the COL5A1 , COL5A2 , COL1A1 , or COL1A2 genes Malfait F, et al. Am J Med Genet C Semin Med Genet 2017;175(1):8–26 [ 48 ] Also called cEDS   Vascular EDS A type of Ehlers–Danlos syndrome characterized by arterial, uterine, or gastrointestinal rupture at a young age, atraumatic formation of carotid-cavernous fistula, plus other minor criteria associated with a mutation in collagen in the COL3A1 gene Malfait F, et al. Am J Med Genet C Semin Med Genet 2017;175(1):8–26 [ 48 ] Also called vEDS  Dizziness A nonspecific term indicating a sensation of lightheadedness, unsteadiness, turning, vertigo, or rocking Modified from NCI Thesaurus AND Reilly BM (1990) Dizziness. In: Walker HK, et al. (eds.) Clinical Methods: The History, Physical, and Laboratory Examinations, 3 rd Ed. Butterworth, Boston, Ch. 212 [ 20 ] NCI Thesaurus Code C37943  Lightheadedness A feeling of faintness or wooziness, or a sensation of motion within the head that may be accompanied by visual or auditory changes, diaphoresis, nausea, or tachycardia Modified from NCI Thesaurus NCI Thesaurus Code C118255  Vertigo A feeling of movement, a sensation as if the external world was revolving around the patient Modified from NCI Thesaurus NCI Thesaurus Code C38057  Syncope A transient loss of consciousness and postural tone due to cerebral hypoperfusion, characterized by a rapid onset, short duration, and spontaneous complete recovery Brignole M, et al. Eur Heart J 2018;39:1883–1948 [ 14 ] 2017 AHA/ACC Key Data Elements and Definitions for Ambulatory Electronic Health Records in Pediatric and Congenital Cardiology [ 17 ]  Migraine A common, severe type of vascular headache often associated with increased sympathetic activity, resulting in nausea, vomiting, and light sensitivity NCI Thesaurus NCI Thesaurus Code C89715  Mast cell activation syndrome A mast cell activation disorder in which there are episodic multisystem symptoms consistent with mast cell activation, appropriate response to medications targeting mast cell activation, and documented increase in validated markers of mast cell activation Akin C. J Allergy Clin Immunol 2017;140(2):349–55 [ 49 ] Also called MCAS  Sjögren syndrome An autoimmune disorder affecting the salivary and lacrimal glands. Morphologically, it is characterized by the presence of lymphocytic and plasmacytic infiltrates which cause destruction of these glands NCI Thesaurus NCI Thesaurus Code C26883  Systemic lupus erythematosus An autoimmune multiorgan disease typically associated with vasculopathy and autoantibody production NCI Thesaurus NCI Thesaurus Code C3201  Rheumatoid arthritis A chronic, systemic autoimmune disorder characterized by inflammation in the synovial membranes and articular surfaces NCI Thesaurus NCI Thesaurus Code C2884  Juvenile idiopathic arthritis A group of chronic, inflammatory childhood disorders of unknown etiology that primarily involve joints NCI Thesaurus NCI Thesaurus Code C114357  Thyroid disease The classification of autoimmune thyroid disease (AITD) includes Hashimoto thyroiditis (HT) or chronic autoimmune thyroiditis and its variants, Graves disease (GD) and autoimmune atrophic thyroiditis or primary myxedema NCI Thesaurus NCI Thesaurus Code C91510  Hashimoto thyroiditis An autoimmune disorder caused by the production of autoantibodies against thyroid tissue. There is progressive destruction of the thyroid follicles leading to hypothyroidism NCI Thesaurus NCI Thesaurus Code C27191  Graves disease Hyperthyroidism associated with diffuse hyperplasia of the thyroid gland (goiter), resulting from production of antibodies that are directed against the thyrotropin receptor complex of the follicular epithelial cells. As a result, the thyroid gland enlarges and secretes increased amounts of thyroid hormones NCI Thesaurus NCI Thesaurus Code C3071  Pheochromocytoma A benign or malignant neuroendocrine neoplasm of the sympathetic nervous system that secretes catecholamines. It arises from the chromaffin cells of the adrenal medulla NCI Thesaurus NCI Thesaurus Code C3326  Carcinoid syndrome A syndrome characterized by signs and symptoms caused by advanced carcinoid tumors. They include skin flushing, diarrhea, wheezing, and tachycardia NCI Thesaurus NCI Thesaurus Code C3215  Paraganglioma A benign or malignant neoplasm arising from paraganglia located along the sympathetic or parasympathetic nerves NCI Thesaurus NCI Thesaurus Code C3308  Hirschsprung disease A congenital disorder characterized by the absence of myenteric ganglion cells in the distal colon NCI Thesaurus NCI Thesaurus Code C34700  Rapid-onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation A neurocristopathy characterized by hypothalamic dysfunction with inability to maintain normal salt–water balance, growth hormone deficiency, hypothyroidism, early or late puberty, increased prolactin concentration, hypoventilation, attenuated/absent peripheral and central chemoreceptor responsiveness, and autonomic dysfunction Modified from NCI Thesaurus NCI Thesaurus Code C121944 Also called ROHHAD  Congenital central hypoventilation syndrome A neurocristopathy associated with heterozygous PHOX2B gene mutations characterized by hypoventilation and hypoxemia typically during sleep with attenuated/absent peripheral and central chemoreceptor responsiveness, and autonomic dysregulation; symptoms typically appear in the newborn period and can co-occur with Hirschsprung disease and neural crest tumors Modified from NCI Thesaurus NCI Thesaurus Code C98889 Also called CCHS  Tracheostomy A surgically created external opening into the trachea NCI Thesaurus NCI Thesaurus Code C122643  Neuroblastoma A neuroblastic tumor characterized by the presence of neuroblastic cells, the absence of ganglion cells, and the absence of a prominent Schwannian stroma formation NCI Thesaurus NCI Thesaurus Code C3270  Ganglioneuroma A benign neuroblastic tumor of the sympathetic nervous system that occurs in childhood. Ganglioneuroma typically arises from the sympathetic chain in the mediastinum or adrenal glands Modified from NCI Thesaurus NCI Thesaurus Code C3049  Ganglioneuroblastoma A neuroblastic tumor characterized by the presence of neuroblastic cells, ganglion cells, and a stroma with Schwannian differentiation constituting more than 50% of the tumor volume NCI Thesaurus NCI Thesaurus Code C3790  Neurofibromatosis type 1 An autosomal dominant form of neurofibromatosis caused by mutation(s) in the NF1 gene, encoding neurofibromin. The clinical features of this condition may include smooth bordered areas of hyperpigmentation, known as cafe-au-lait spots, axillary freckling, Lisch nodules, short stature, cutaneous and subcutaneous tumors (neurofibromas), optic nerve gliomas, precocious puberty, and growth hormone excess NCI Thesaurus NCI Thesaurus Code C3273  Neurofibromatosis type 2 An autosomal dominant disorder caused by mutations in the NF2 tumor suppressor gene. It is characterized by the development of peripheral and central nervous system tumors including acoustic schwannomas, neurofibromas, gliomas, and meningiomas NCI Thesaurus NCI Thesaurus Code C3274  Rett syndrome A progressive neurologic disorder caused by mutations in the MECP2 gene on chromosome X. It almost exclusively affects girls. It is characterized by breath-holding spells, flushing, repetitive hand motions, lack of advancement in language, and regression in developmental milestones. Other signs and symptoms include slowed head growth, scoliosis, breathing abnormalities (including sleep-disordered breathing), and sleep disturbances Modified from NCI Thesaurus NCI Thesaurus Code C75488  Waardenburg syndrome A rare, autosomal dominant inherited syndrome caused by mutations in the PAX3 , MITF , and SNAI2 genes. Signs and symptoms include hearing loss, dystopia canthorum (widely spaced inner corners of the eyes), and changes in the color of the skin, hair (white forelock), and eyes Modified from NCI Thesaurus NCI Thesaurus Code C85222  Visceral hypersensitivity Heightened sensitivity or reduced thresholds for pain and discomfort associated with the gastrointestinal tract Modified from Mertz H. Aliment Pharmacol Ther 2003;17(5):623–33 [ 32 ] AND Faure C, Di Lorenzo C, Thapar N. (2017) Pediatric Neurogastroenterology Gastrointestinal Motility and Functional Disorders in Children, 2 nd Ed. Springer International Publishing, Switzerland [ 33 ] Also called visceral hyperalgesia  Multiple endocrine neoplasia An autosomal dominant inherited neoplastic syndrome characterized by the development of various endocrine neoplasms and abnormalities in various anatomic sites NCI Thesaurus NCI Thesaurus Code C6432  Simple breath-holding spell A clinical condition in which there is a breath-holding spell without syncope 2017 AHA/ACC Key Data Elements and Definitions for Ambulatory Electronic Health Records in Pediatric and Congenital Cardiology [ 17 ]  Complex breath-holding spell A clinical condition in which there is a breath-holding spell associated with syncope 2017 AHA/ACC Key Data Elements and Definitions for Ambulatory Electronic Health Records in Pediatric and Congenital Cardiology [ 17 ]  Pallid breath-holding spell A clinical condition in which there is a breath-holding spell associated with pale appearance 2017 AHA/ACC Key Data Elements and Definitions for Ambulatory Electronic Health Records in Pediatric and Congenital Cardiology [ 17 ]  Cyanotic breath-holding spell A clinical condition in which there is a breath-holding spell associated with cyanotic appearance 2017 AHA/ACC Key Data Elements and Definitions for Ambulatory Electronic Health Records in Pediatric and Congenital Cardiology [ 17 ]  Seizure disorder A brain disorder characterized by episodes of abnormally increased neuronal discharge resulting in transient episodes of sensory or motor neurological dysfunction, or psychic dysfunction that may or may not be associated with loss of consciousness or convulsions NCI Thesaurus NCI Thesaurus Code C3020 Also called epilepsy  Sudden death An unexpected death without warning NCI Thesaurus NCI Thesaurus Code C107103  Sudden cardiac death An unexpected death from a cardiac cause within a short time period from the onset of symptoms NCI Thesaurus NCI Thesaurus Code C50911 Medications A drug product that contains one or more active and/or inactive ingredients; it is intended to treat, prevent or alleviate the symptoms of disease NCI Thesaurus NCI Thesaurus Code C459  Fludrocortisone A synthetic adrenocortical steroid possessing very potent mineralocorticoid properties and high glucocorticoid activity; it is used only for its mineralocorticoid effects www.rxlist.com  Midodrine A direct-acting prodrug and sympathomimetic agent with antihypotensive properties NCI Thesaurus NCI Thesaurus Code C61846  Droxidopa A synthetic amino acid analog that is directly metabolized to norepinephrine by dopa-decarboxylase https://www.accessdata.fda.gov/drugsatfda_docs/label/2017/203202s007lbl.pdf  Beta blocker Natural or synthetic beta adrenergic antagonists selectively or non-selectively blocking or diminishing physiologic beta-adrenergic agonist actions on the sympathetic system NCI Thesaurus NCI Thesaurus Code C29576  Propranolol A synthetic, nonselective beta-adrenergic receptor blocker with antianginal, antiarrhythmic, antihypertensive properties NCI Thesaurus NCI Thesaurus Code C62073  Atenolol A synthetic isopropylamino-propanol derivative used as an antihypertensive, hypotensive and antiarrhythmic, atenolol acts as a peripheral, cardioselective beta blocker specific for beta-1 adrenergic receptors, without intrinsic sympathomimetic effects NCI Thesaurus NCI Thesaurus Code C28836  Metoprolol A cardioselective competitive beta-1 adrenergic receptor antagonist with antihypertensive properties and devoid of intrinsic sympathomimetic activity NCI Thesaurus NCI Thesaurus Code C61845  Nadolol A nonselective beta-adrenergic antagonist with antihypertensive and antiarrhythmic activities NCI Thesaurus NCI Thesaurus Code C29280  Nebivolol A beta-1 adrenergic receptor antagonist with antihypertensive and vasodilatory activity NCI Thesaurus NCI Thesaurus Code C66221  Ivabradine An orally bioavailable, hyperpolarization-activated, cyclic nucleotide-gated (HCN) channel blocker, with negative chronotropic activity that selectively binds to the intracellular portion of the HCN channel pore and blocks HCN channels in the pacemaker cells within the sinoatrial (SA) node NCI Thesaurus NCI Thesaurus Code C65995  Pyridostigmine The bromide salt form of pyridostigmine, a quaternary ammonium carbamate derivative and an acetylcholinesterase inhibitor NCI Thesaurus NCI Thesaurus Code C47697  Desmopressin A synthetic octapeptide, and an analog of human hormone arginine vasopressin with antidiuretic and coagulant activities NCI Thesaurus NCI Thesaurus Code C61701  Stimulant Any agent capable of having a stimulating effect on the central nervous system (CNS) NCI Thesaurus NCI Thesaurus Code C47795  Methylphenidate A synthetic central nervous system stimulant NCI Thesaurus NCI Thesaurus Code C62045  Dexmethylphenidate The d -threo-enantiomer of methylphenidate, a central nervous system stimulant, appearing to block the reuptake of norepinephrine and dopamine into the presynaptic neuron, increasing their availability in the extracellular space NCI Thesaurus NCI Thesaurus Code C26642  Mixed amphetamine salts A combination of two synthetic agents with central nervous system stimulant activity NCI Thesaurus NCI Thesaurus Code C26643  Lisdexamfetamine The dimesylate form and prodrug of the d -isomer of amphetamine, a non-catecholamine sympathomimetic amine with central nervous system (CNS)-stimulating activity NCI Thesaurus NCI Thesaurus Code C76628  Atomoxetine The hydrochloride salt of atomoxetine, a phenoxy-3-propylamine derivative and selective non-stimulant, norepinephrine reuptake inhibitor with cognitive-enhancing activity NCI Thesaurus NCI Thesaurus Code C47405  Modafinil A synthetic central nervous system stimulant with wakefulness-promoting activity NCI Thesaurus NCI Thesaurus Code C26661  Armodafinil The R-enantiomer of the racemic synthetic agent modafinil with central nervous system (CNS) stimulant and wakefulness-promoting activities NCI Thesaurus NCI Thesaurus Code C65241  Psychotropic medication A classification of medication that acts on the central nervous system, affecting brain function, level of consciousness, mood, emotions, and behavior NCI Thesaurus NCI Thesaurus Code C82395  Sertraline The hydrochloride salt of sertraline, a synthetic derivative of naphthalenamine with anti-serotoninergic and anti-depressant properties NCI Thesaurus NCI Thesaurus Code C29451  Citalopram A bicyclic phthalene derivative and selective serotonin reuptake inhibitor (SSRI), with antidepressant and anxiolytic activities NCI Thesaurus NCI Thesaurus Code C61680  Escitalopram The active S-stereoisomer of the selective serotonin reuptake inhibitor (SSRI) citalopram with antidepressant, anti-obsessive–compulsive and antibulimic properties NCI Thesaurus NCI Thesaurus Code C61754  Fluoxetine The hydrochloride salt form of fluoxetine, a diphenhydramine derivative and selective serotonin reuptake inhibitor with antidepressant, antiobsessional, and antibulimic effects NCI Thesaurus NCI Thesaurus Code C2829  Bupropion An aminoketone with antidepressant activity NCI Thesaurus NCI Thesaurus Code C62012  Venlafaxine A synthetic phenethylamine bicyclic derivative with antidepressant activity NCI Thesaurus NCI Thesaurus Code C1278  Paroxetine The hydrochloride salt form of paroxetine, a phenylpiperidine derivative and a selective serotonin reuptake inhibitor (SSRI) with antidepressant and anxiolytic properties NCI Thesaurus NCI Thesaurus Code C1837  Duloxetine The hydrochloride salt of duloxetine, a fluoxetine derivative belonging to the class of selective serotonin (5-HT) and norepinephrine (NE) reuptake inhibitors (SSNRIs) and exhibiting antidepressant activity NCI Thesaurus NCI Thesaurus Code C65496  Aripiprazole A quinoline derivate and atypical anti-psychotic agent NCI Thesaurus NCI Thesaurus Code C47403  Olanzapine A synthetic derivative of thienobenzodiazepine with antipsychotic, antinausea, and antiemetic activities NCI Thesaurus NCI Thesaurus Code C47639  Quetiapine A dibenzothiazepine derivative with antipsychotic property NCI Thesaurus NCI Thesaurus Code C61917  Risperidone A benzisoxazole derivative with antipsychotic property NCI Thesaurus NCI Thesaurus Code C29416  Ziprasidone A benzothiazolylpiperazine derivative and an atypical antipsychotic agent with an antischizophrenic property NCI Thesaurus NCI Thesaurus Code C47788  Mirtazapine A synthetic tetracyclic derivative of the piperazino-azepines with antidepressant activity NCI Thesaurus NCI Thesaurus Code C29265  Buspirone An anxiolytic agent chemically and pharmacologically unrelated to benzodiazepines, barbiturates, or other sedative/hypnotic drugs NCI Thesaurus NCI Thesaurus Code C62013  Trazodone A synthetic triazolopyridine derivative, antidepressant and sedative, trazodone acts as a serotonin uptake inhibitor, chemically unrelated to tricyclic, tetracyclic, or other antidepressants NCI Thesaurus NCI Thesaurus Code C29510  Antihistamine Any agent that binds to the histamine H1 receptor, thereby antagonizing histamine mediated allergic reactions, such as bronchoconstriction, vasodilation, and upregulated capillary permeability NCI Thesaurus NCI Thesaurus Code C29578  Cetirizine Cetirizine is a metabolite of hydroxyzine and a selective peripheral histamine H1-receptor antagonist NCI Thesaurus NCI Thesaurus Code C1042  Loratadine A piperidine histamine H1-receptor antagonist with anti-allergic properties and without sedative effects NCI Thesaurus NCI Thesaurus Code C29162  Fexofenadine A second generation, long-lasting selective histamine H1 receptor antagonist with antiinflammatory property NCI Thesaurus NCI Thesaurus Code C61764  Diphenhydramine A first generation antihistamine and ethanolamine with sedative and anti-allergic properties NCI Thesaurus NCI Thesaurus Code C61728  Cyproheptadine The hydrochloride salt of a synthetic methyl-piperidine derivative with antihistaminic and anti-serotoninergic properties NCI Thesaurus NCI Thesaurus Code C28949  Acid suppression   Ranitidine A member of the class of histamine H2-receptor antagonists with antacid activity NCI Thesaurus NCI Thesaurus Code C29412   Cimetidine A histamine H(2)-receptor antagonist NCI Thesaurus NCI Thesaurus Code C374   Famotidine A propanimidamide and histamine H2-receptor antagonist with antacid activity NCI Thesaurus NCI Thesaurus Code C29045   Omeprazole A benzimidazole with selective and irreversible proton pump inhibition activity NCI Thesaurus NCI Thesaurus Code C716   Lansoprazole A substituted benzimidazole prodrug with selective and irreversible proton pump inhibitor activity NCI Thesaurus NCI Thesaurus Code C29150   Esomeprazole The S-isomer of omeprazole, with gastric proton pump inhibitor activity NCI Thesaurus NCI Thesaurus Code C65538   Pantoprazole A substituted benzimidazole and proton pump inhibitor with antacid activity NCI Thesaurus NCI Thesaurus Code C29346   Rabeprazole An alpha-pyridylmethylsulfinyl benzimidazole and a selective and irreversible proton pump inhibitor with antisecretory property NCI Thesaurus NCI Thesaurus Code C29410  Clonidine A centrally-acting alpha-adrenergic agonist that also acts on imidazoline receptors that inhibits central sympathetic activity with antihypertensive activity Modified from NCI Thesaurus NCI Thesaurus Code C380  Carbidopa A hydrazine derivative of dopa and a peripheral dopa decarboxylase inhibitor that is used as an adjunct to levodopa administration to prevent peripheral biosynthesis of levodopa to dopamine NCI Thesaurus NCI Thesaurus Code C47431  Dexmedetomidine A short-acting imidazole derivate and active d -isomer of medetomidine with antihypertensive, analgesic, anxiolytic, and sedative properties Modified from NCI Thesaurus NCI Thesaurus Code C47479  Erythromycin A broad-spectrum, macrolide antibiotic with antibacterial activity NCI Thesaurus NCI Thesaurus Code C476  Pregabalin A 3-isobutyl derivative of gamma-amino butyric acid (GABA) with anti-convulsant, anti-epileptic, anxiolytic, and analgesic activities NCI Thesaurus NCI Thesaurus Code C64625  Naltrexone A noroxymorphone derivative with competitive opioid antagonistic property NCI Thesaurus NCI Thesaurus Code C62055  Nonsteroidal anti-inflammatory drug A pharmacological agent that is not a steroid and has potential anti-inflammatory, analgesic, antipyretic, and antiplatelet activities NCI Thesaurus NCI Thesaurus Code C257   Ibuprofen (note similar to naproxen) A propionic acid derivate and nonsteroidal anti-inflammatory drug (NSAID) with anti-inflammatory, analgesic, and antipyretic effects NCI Thesaurus NCI Thesaurus Code C561   Naproxen A propionic acid derivative and a nonsteroidal anti-inflammatory drug (NSAID) with anti-inflammatory, antipyretic, and analgesic activities NCI Thesaurus NCI Thesaurus Code C680   Ketorolac A synthetic pyrrolizine carboxylic acid derivative with anti-inflammatory, analgesic, and antipyretic activities NCI Thesaurus NCI Thesaurus Code C1219   Celecoxib A nonsteroidal anti-inflammatory drug (NSAID) with a diaryl-substituted pyrazole structure NCI Thesaurus NCI Thesaurus Code C1728  Ondansetron A carbazole derivative with antiemetic activity NCI Thesaurus NCI Thesaurus Code C1119  Granisetron An indazole derivative with antiemetic properties NCI Thesaurus NCI Thesaurus Code C62031  Aprepitant A small molecule, high-affinity substance P antagonist with antiemetic activity NCI Thesaurus NCI Thesaurus Code C49173  Scopolamine A tropane alkaloid derived from plants of the nightshade family (Solanaceae), specifically Hyoscyamus niger and Atropa belladonna , with anticholinergic, antiemetic, and antivertigo properties NCI Thesaurus NCI Thesaurus Code C47712  Meclizine A synthetic piperazine with anti-emetic, sedative and histamine H1 antagonistic properties NCI Thesaurus NCI Thesaurus Code C61610  Melatonin A hormone involved in sleep regulatory activity, and a tryptophan-derived neurotransmitter, which inhibits the synthesis and secretion of other neurotransmitters such as dopamine and GABA NCI Thesaurus NCI Thesaurus Code C2267  Zolpidem The tartrate salt of an imidazopyridine with non-benzodiazepine benzodiazepine-receptor agonist and sedative-hypnotic activities NCI Thesaurus NCI Thesaurus Code C29553  Eszopiclone A nonbenzodiazepine, cyclopyrrolone, and active stereoisomer of zopiclone with hypnotic and sedative activity and without significant anxiolytic activity NCI Thesaurus NCI Thesaurus Code C65545  Laxatives Synthetic compounds or mixtures administered orally or rectally with laxative activity. A laxative preparation is used to promote bowel movement to treat constipation or to clean the colon for rectal and bowel examinations NCI Thesaurus NCI Thesaurus Code C29697   Lubiprostone A bicyclic fatty acid derived from prostaglandin E1 and a chloride channel activator with laxative activity NCI Thesaurus NCI Thesaurus Code C66040   Psyllium The husk of the dried ripe seeds of Plantago psyllium , P. indica , P. ovata , and P. arenaria plants with laxative and cholesterol-lowering activities NCI Thesaurus NCI Thesaurus Code C29250   Docusate sodium The sodium salt of docusate, a dioctyl salt and an emollient laxative with stool-softening activity NCI Thesaurus NCI Thesaurus Code C29000   Senna An extract made from the dried leaflets on the pods of Cassia angustifolia or Cassia acutifolia with cathartic activity NCI Thesaurus NCI Thesaurus Code C61324   Mineral oil A mixture of liquid paraffinic, naphthenic, and polycyclic aromatic hydrocarbons obtained from petroleum in which the amount and types of aromatic hydrocarbons are dependent on the treatment process NCI Thesaurus NCI Thesaurus Code C29264   Linaclotide A synthetic, fourteen amino acid peptide and agonist of intestinal guanylate cyclase type C (GC-C), which is structurally related to the guanylin peptide family, with secretagogue, analgesic, and laxative activities NCI Thesaurus NCI Thesaurus Code C81001   Prucalopride A highly selective serotonin 5-HT4 receptor agonist that has been shown to stimulate gut motility Quigley EMM Therap Adv Gastroenterol 2012;5(1):23–30 [ 52 ]   Lactulose A disaccharide (sugar) synthesized from fructose and galactose; lactulose is used to treat constipation and some liver diseases NCI Thesaurus NCI Thesaurus Code C29148   Polyethylene glycol 3350 A polymer made by joining molecules of ethylene oxide and water together in a repeating pattern used as an osmotic laxative Modified from NCI Thesaurus NCI Thesaurus Code C762   Bisacodyl A synthetic pyridinylmethylene-diacetate ester derivative stimulant laxative, Bisacodyl acts with a parasympathetic effect directly on mucosal sensory nerves, increasing peristaltic contractions NCI Thesaurus NCI Thesaurus Code C28870   Tegaserod A 5-HT4 agonist that functions as a motility agent, by stimulating the peristaltic reflex and intestinal secretion, as an analgesic by inhibition of visceral sensitivity www.drugs.com   Cisapride A substituted piperidinyl benzamide prokinetic agent. Cisapride facilitates release of acetylcholine from the myenteric plexus, resulting in increased gastrointestinal motility. In addition, cisapride has been found to act as a serotonin agonist, stimulating type 4 receptors, and a serotonin 5-HT3 receptor antagonist NCI Thesaurus NCI Thesaurus Code C1210   Metoclopramide A substituted benzamide and a derivative of para-aminobenzoic acid (PABA) that is structurally related to procainamide, with gastroprokinetic and antiemetic effects NCI Thesaurus NCI Thesaurus Code C62046  Divalproex A stable coordination compound composed of sodium valproate and valproic acid with anticonvulsant and antiepileptic activities NCI Thesaurus NCI Thesaurus Code C28996  Amitriptyline A derivative of dibenzocycloheptadiene and a tricyclic antidepressant. Amitriptyline inhibits the re-uptake of norepinephrine and serotonin by the presynaptic neuronal membrane in the central nervous system (CNS), thereby increasing the synaptic concentration of norepinephrine and serotonin. Due to constant stimulation to these receptors, amitriptyline may produce a downregulation of adrenergic and serotonin receptors, which may contribute to the antidepressant activity NCI Thesaurus NCI Thesaurus Code C62005  Nortriptyline A tricyclic antidepressant agent used for short-term treatment of various forms of depression. Nortriptyline blocks the norepinephrine presynaptic receptors, thereby blocking the reuptake of this neurotransmitter and raising the concentration in the synaptic cleft in the CNS. Nortriptyline also binds to alpha-adrenergic, histaminergic and cholinergic receptors. Long-term treatment with nortriptyline produces a downregulation of adrenergic receptors due to the increased stimulation of these receptors NCI Thesaurus NCI Thesaurus Code C62060  Gabapentin A synthetic analog of the neurotransmitter gamma-aminobutyric acid with anticonvulsant activity NCI Thesaurus NCI Thesaurus Code C1108  Triptans A family of tryptamine-based drugs used as abortive medication in the treatment of migraine and cluster headaches www.wikipedia.org   Sumatriptan A sulfonamide triptan that selectively binds to and activates serotonin 5-HT1D receptors in the central nervous system, thereby constricting cerebral blood vessels NCI Thesaurus NCI Thesaurus Code C1240   Rizatriptan A triptan that selectively binds to and activates serotonin 5-HT1B receptors expressed in intracranial arteries, and to 5-HT1D receptors located on peripheral trigeminal sensory nerve terminals in the meninges and central terminals in brain stem sensory nuclei NCI Thesaurus NCI Thesaurus Code C61930   Eletriptan A triptan that binds to and acts at serotonin 5-HT1B receptors located on intracranial blood vessels which leads to vasoconstriction. This drug may also exerts its effects by binding to and activating 5-HT 1D receptors on sensory nerve endings in the trigeminal system, which results in the inhibition of pro-inflammatory neuropeptide release NCI Thesaurus NCI Thesaurus Code C47508   Zolmitriptan A triptan that selectively binds to and activates serotonin (5-HT) 1B receptors expressed in intracranial arteries and 5-HT 1D receptors located on peripheral trigeminal sensory nerve terminals in the meninges and central terminals in brainstem sensory nuclei, resulting in constriction of cranial vessels, reduction of vessel pulsation and inhibition of nociceptive transmission NCI Thesaurus NCI Thesaurus Code C47789  Dihydroergotamine mesylate An ergot derivative with agonistic activity for alpha-adrenergic, serotonergic, and dopaminergic receptors NCI Thesaurus NCI Thesaurus Code C47491  Topiramate A sulfamate-substituted monosaccharide with anticonvulsant property NCI Thesaurus NCI Thesaurus Code C47764  Onabotulinumtoxin A An injectable formulation of a neurotoxin derived through the fermentation of the Hall strain of Clostridium botulinum type A with neuromuscular transmission inhibitory and analgesic activities NCI Thesaurus NCI Thesaurus Code C82623  Cromolyn sodium A synthetic mast cell stabilizer with anti-inflammatory activity NCI Thesaurus NCI Thesaurus Code C61691  Montelukast It is a type of leukotriene receptor antagonist and a type of antiasthmatic agent NCI Thesaurus NCI Thesaurus Code C66189  Quercetin A polyphenolic flavonoid with potential chemopreventive activity NCI Thesaurus NCI Thesaurus Code C792  Aspirin An orally administered non-steroidal antiinflammatory agent; acetylsalicylic acid NCI Thesaurus NCI Thesaurus Code C287  Epinephrine A hormone and neurotransmitter NCI Thesaurus NCI Thesaurus Code C2292 Medical device Any physical object that is useful for prevention, diagnosis, monitoring, or treatment of disease or other conditions NCI Thesaurus NCI Thesaurus Code C16830  Compression stockings Specialized hosiery worn on the lower extremities available in varying degrees of pressure that creates increased peripheral venous return  Abdominal binder A wide band fastened snugly about the abdomen for support https://medical-dictionary.thefreedictionary.com/abdominal+binder  Gastric pacemaker A device that is implanted under the skin and has electrodes that attach to the stomach that is programmed to stimulate the stomach to empty NCI Thesaurus NCI Thesaurus Code C157863  Cefaly An external trigeminal nerve stimulation device used for the treatment of migraine www.cefaly.us  Transcutaneous electrical nerve stimulation A non-invasive form of electroanalgesia that uses conductive gel pads to transmit electrical energy from an external stimulator to peripheral nerves NCI Thesaurus NCI Thesaurus Code C21032  Vagus nerve stimulator A medical device that delivers electrical impulses to the vagus nerve, which can be implanted or external  IB-Stim A medical device that delivers electrical impulses to cranial nerve branches as a peripheral electrical nerve field stimulator to reduce abdominal pain Physical examination findings A systemic evaluation of the body and its functions using visual inspection, palpation, percussion, and auscultation NCI Thesaurus NCI Thesaurus Code C20989  Vital signs Vital signs (such as height, weight, and blood pressure) directly measure an individual's current state of attributes NCI Thesaurus NCI Thesaurus Code C154628   Heart rate A measurement that describes the frequency of rate of contractions of the systemic (often left) ventricle measured within a unit time Numeric, in bpm 0–300 2017 AHA/ACC Key Data Elements and Definitions for Ambulatory Electronic Health Records in Pediatric and Congenital Cardiology [ 17 ]   Supine heart rate Heart rate measured in a posterior recumbent body position whereby the person lies on its back and faces upward Numeric, in bpm 0–300 NCI Thesaurus NCI Thesaurus Code C62167   Sitting heart rate Heart rate measured in the posture of one who occupies a seat Numeric, in bpm 0–300 NCI Thesaurus NCI Thesaurus Code C62122   Standing heart rate Heart rate measured during the act of assuming or maintaining an erect upright position Numeric, in bpm 0–300 NCI Thesaurus NCI Thesaurus Code C62126   Blood pressure A measurement that describes the pressure of the circulating blood against the walls of the blood vessels. Measurement is taken from the right arm Numeric, in mmHg; displayed as systolic pressure over diastolic pressure 0–200 2017 AHA/ACC Key Data Elements and Definitions for Ambulatory Electronic Health Records in Pediatric and Congenital Cardiology [ 17 ]   Supine blood pressure Blood pressure measured in a posterior recumbent body position whereby the person lies on its back and faces upward Numeric, in mmHg; displayed as systolic pressure over diastolic pressure 0–200 NCI Thesaurus NCI Thesaurus Code C62167   Seated blood pressure Blood pressure measured in the posture of one who occupies a seat Numeric, in mmHg; displayed as systolic pressure over diastolic pressure 0–200 NCI Thesaurus NCI Thesaurus Code C62122   Standing blood pressure Blood pressure measured during the act of assuming or maintaining an erect upright position Numeric, in mmHg; displayed as systolic pressure over diastolic pressure 0–200 NCI Thesaurus NCI Thesaurus Code C62126  Blood pressure, other extremity A measurement that describes the pressure of the circulating blood against the walls of the blood vessels. Measurement is taken from an extremity other than the right arm Numeric, in mmHg; displayed as systolic pressure over diastolic pressure 0–200 Note: denote which extremity (LUE, RLE, LLE)  Pulse pressure The force of a heart contraction measured by the difference between the diastolic and systolic blood pressure measurements Numeric, in mmHg; displayed as systolic pressure over diastolic pressure 0–200 NCI Thesaurus NCI Thesaurus Code C100945   Height A measurement that describes the vertical measurement or distance from the base, or bottom, of the patient, to the top of the patient; this can be taken as the dimension of extension of a patient who cannot stand Numeric, in cm 10–230 2017 AHA/ACC Key Data Elements and Definitions for Ambulatory Electronic Health Records in Pediatric and Congenital Cardiology [ 17 ]   Weight A measurement that describes the vertical force exerted by a mass of the patient as a result of gravity Numeric, in kg 0.2–500 2017 AHA/ACC Key Data Elements and Definitions for Ambulatory Electronic Health Records in Pediatric and Congenital Cardiology [ 17 ]   Oxygen saturation A measurement that describes the determination of oxygen-hemoglobin saturation of blood Numeric, in % 20–100 Modified from 2017 AHA/ACC Key Data Elements and Definitions for Ambulatory Electronic Health Records in Pediatric and Congenital Cardiology [ 17 ]   Respiratory rate A measurement that describes the rate of breathing (inhalation and exhalation) measured within a unit time Numeric, in breaths per minute 0–100 2017 AHA/ACC Key Data Elements and Definitions for Ambulatory Electronic Health Records in Pediatric and Congenital Cardiology [ 17 ]   Tidal volume The volume of air moved into and out of the lungs per breath during breathing at rest Modified from NCI Thesaurus NCI Thesaurus Code C111324  Nystagmus A condition where the eyes move rapidly and uncontrollably www.aao.org/eye-health/diseases/what-is-nystagmus  Joint hypermobility The capability that a joint (or a group of joints) has to move, passively and/or actively, beyond normal limits along physiological axes Castori M, et al. Am J Med Genet C Semin Med Genet 2017;175(1):148–57 [ 36 ]   Finger Any of the digits of the hand NCI Thesaurus NCI Thesaurus Code C32608   Wrist A joint between the distal end of the radius and the proximal row of carpal bones NCI Thesaurus NCI Thesaurus Code C33894   Elbow A type of hinge joint located between the forearm and upper arm NCI Thesaurus NCI Thesaurus Code C32497   Shoulder A ball-and-socket joint at the upper end of the humerus, located at the junction of humerus and scapula NCI Thesaurus NCI Thesaurus Code C33548   Knee A joint connecting the lower part of the femur with the upper part of the tibia NCI Thesaurus NCI Thesaurus Code C32898   Waist The part of the body in humans between the ribs and the hips, usually the narrowest part of the torso www.dictionary.com   Ankle A gliding joint between the distal ends of the tibia and fibula and the proximal end of the talus NCI Thesaurus NCI Thesaurus Code C32078   Beighton score A clinical assessment method for classifying generalized joint hypermobility consisting of nine separate tests. A score of 5/9, or greater, in adults, and 6/9, or greater, in children indicates a positive test Numeric, in integers 0–9 Juul-Kristensen B Am J Med Genet C Semin Med Genet 1751):116–147 [ 53 ]  Venous pooling The accumulation of blood in veins of the extremities or splanchnic circulation due to gravitational pull when a person assumes an upright position Also called dependent acrocyanosis  Pallor A clinical finding in which there appears to be a loss of color and/or perfusion, or a white appearance 2017 AHA/ACC Key Data Elements and Definitions for Ambulatory Electronic Health Records in Pediatric and Congenital Cardiology [ 17 ]  Diaphoresis A clinical finding in which there is excessive or unpredictable sweating 2017 AHA/ACC Key Data Elements and Definitions for Ambulatory Electronic Health Records in Pediatric and Congenital Cardiology [ 17 ]  Mydriasis Dilation of the pupil www.medicinenet.com  Miosis Constriction of the pupil www.medicinenet.com  Alacrima Lack of tears due to absence or hyposecretion of tears, or to agenesis or improper development of lacrimal gland ducts and associated structures Adams J Clin Genet 2018;94(1):54–60 [ 26 ]  Cyanosis A bluish or purplish discoloration of the skin and mucous membranes resulting from an increased amount of deoxygenated hemoglobin in the blood Modified from NCI Thesaurus NCI Thesaurus Code C26737   Central cyanosis Cyanosis that is noted to occur over the entire body surface area and visible mucosa www.ncbi.nlm.nih.gov   Perioral cyanosis Cyanosis that is noted to occur surrounding the mouth that may or may not include the lips   Peripheral cyanosis Cyanosis that is noted to occur in the upper and lower extremities www.ncbi.nlm.nih.gov Also called acrocyanosis  Dermatographism (Darier sign) Urticaria due to physical allergy in which a pale, raised welt or wheal with a red flare on each side is elicited by stroking or scratching the skin https://medical-dictionary.thefreedictionary.com/dermatographism  Allodynia Pain due to a stimulus that does not normally provoke pain www.ncbi.nlm.nih.gov  Urticaria A transient, itchy skin eruption characterized by hives Modified from NCI Thesaurus NCI Thesaurus Code C3432  Areflexia A finding indicating the complete absence of neurological reflexes NCI Thesaurus NCI Thesaurus Code C115420  Hyperreflexia Autonomic nervous system overreaction to stimuli, most commonly after spinal cord injury at a T-5 level and above NCI Thesaurus NCI Thesaurus Code C43248  Anhidrosis Lack of sweating or the ability to sweat when provoked by the appropriate stimulus NCI Thesaurus NCI Thesaurus Code C34385  Scoliosis A congenital or acquired spinal deformity characterized by lateral curvature of the spine NCI Thesaurus NCI Thesaurus Code C78603  Hypotonia A condition of decreased tone of the skeletal muscles and diminished resistance to passive stretching NCI Thesaurus NCI Thesaurus Code C87070 Testing A procedure for critical evaluation NCI Thesaurus NCI Thesaurus Code C15336  Laboratory Any procedure that involves testing or manipulating a sample of blood, urine, or other body substance in a laboratory setting NCI Thesaurus NCI Thesaurus Code C25294   Morning (AM) cortisol A hormone made by the adrenal cortex. Measurement is made between 6 and 8 AM Numeric, in mcg/dl 0–100 NCI Thesaurus, values based on ABIM Laboratory Test Reference Ranges Jan 2019 NCI Thesaurus Code C2290   Thyroid-stimulating hormone A peptide hormone secreted by the anterior pituitary that promotes the growth of the thyroid gland and stimulates the synthesis of thyroid hormones and the release of thyroxine by the thyroid gland Numeric, in μU/ml 0–50 NCI Thesaurus, values based on ABIM Laboratory Test Reference Ranges Jan 2019 NCI Thesaurus Code C2280   T4/thyroxine A hormone synthesized and secreted by the thyroid gland containing four iodine atoms and is converted to triiodothyronine (T3) in the body, influencing metabolism and organ function Numeric, in µg/dl 0–50 NCI Thesaurus, values based on ABIM Laboratory Test Reference Ranges Jan 2019 NCI Thesaurus Code C2302   Ferritin An iron protein complex, containing up to 23% iron, formed by the union of ferric iron with apoferritin; it is found in the intestinal mucosa, spleen, bone marrow, reticulocytes, and liver, and regulates iron storage and transport from the intestinal lumen to plasma Numeric, in ng/ml 0–500 NCI Thesaurus, values based on ABIM Laboratory Test Reference Ranges Jan 2019 NCI Thesaurus Code C16577   Hemoglobin The red respiratory protein of erythrocytes, consisting of approximately 3.8% heme and 96.2% globin (64.5 KD), which as oxyhemoglobin (HbO 2 ) transports oxygen from the lungs to the tissues where the oxygen is readily released and HbO 2 becomes Hb Numeric, in g/dl 0–30 NCI Thesaurus, values based on ABIM Laboratory Test Reference Ranges Jan 2019 NCI Thesaurus Code C16676   Hematocrit A measure of the volume of red blood cells expressed as a percentage of the total blood volume Numeric, in % 0–90 NCI Thesaurus, values based on ABIM Laboratory Test Reference Ranges Jan 2019 NCI Thesaurus Code C64796   Blood urea nitrogen A quantitative measurement of the amount of urea nitrogen present in a serum sample Numeric, in mg/dl 0–40 NCI Thesaurus, values based on ABIM Laboratory Test Reference Ranges Jan 2019 NCI Thesaurus Code C61019   Creatinine The breakdown product of creatine, a constituent of muscle tissue, that is excreted by the kidney and whose serum level is used to evaluate kidney function Numeric, in mg/dl 0–5 NCI Thesaurus, values based on ABIM Laboratory Test Reference Ranges Jan 2019 NCI Thesaurus Code C399   Tryptase A family of serine endoproteinases usually expressed in mast cell secretory granules Numeric, in ng/ml 0–50 NCI Thesaurus, values based on ABIM Laboratory Test Reference Ranges Jan 2019 NCI Thesaurus Code C120030    Baseline A starting point to which things may be compared NCI Thesaurus NCI Thesaurus Code C25213    Post-flush A point in time after systemic flushing has occurred   Urinary n -methylhistamine The major metabolite of histamine Afrin LB and Molderings GJ. World J Hematol 2014;3(1):1–17 [ 54 ]   Urinary prostaglandin-D2 A prostaglandin that binds to the receptor PTGDR as well as CRTH2, and is a major prostaglandin produced by mast cells www.wikipedia.org   Urinary 11β-prostaglandin F2α The most abundant metabolic product of prostaglandins released by activated mast cells www.mayocliniclabs.com   IgA anti-tissue transglutaminase An autoantibody against the transglutaminase protein found in celiac disease Numeric, in U/ml 0–50 www.wikipedia.org , values based on ABIM Laboratory Test Reference Ranges Jan 2019   IgA anti-endomysial reactivity An autoantibody against tissue transglutaminase found in endomysium, seen in celiac disease www.wikipedia.org   Calprotectin A heterodimeric or heterotetrameric protein complex comprised of protein S100A8 and protein S100A9 expressed in the cytosol of neutrophils and macrophages and plays a role in multiple host defense related processes including immunomodulation, neutrophil chemotaxis and both antibacterial and antifungal responses NCI Thesaurus NCI Thesaurus Code C105971   Antinuclear antibody An autoimmune antibody that is directed against structures within the nucleus of the cell Numeric, as ratio 1:1 to 1:16,000 NCI Thesaurus, values based on ABIM Laboratory Test Reference Ranges Jan 2019 NCI Thesaurus Code C70547   C-reactive protein An annular pentameric protein of hepatic origin found in blood plasma cleaved during biological activation and is associated with host defense mechanisms and inflammatory responses Numeric, in ng/ml 0–20 www.wikipedia.org , NCI Thesaurus, values based on ABIM Laboratory Test Reference Ranges Jan 2019 NCI Thesaurus Code C60651   Erythrocyte sedimentation rate A quantitative measurement of the distance that red blood cells travel in one hour in a sample of unclotted blood Numeric, in mm/h 0–120 NCI Thesaurus, values based on ABIM Laboratory Test Reference Ranges Jan 2019 NCI Thesaurus Code C74611  Tilt-table testing A medical procedure, also called head upright tilt testing, which assesses orthostatic tolerance utilizing passive upright posture on a mechanical tilt table Also called head up tilt test  Standing test A medical procedure which assesses orthostatic tolerance utilizing active upright posture while standing motionless    Heart rate A measurement that describes the frequency of rate of contractions of the systemic (often left) ventricle measured within a unit time Numeric, in bpm 0–300 2017 AHA/ACC Key Data Elements and Definitions for Ambulatory Electronic Health Records in Pediatric and Congenital Cardiology [ 17 ]     Supine heart rate Heart rate measured in a posterior recumbent body position whereby the person lies on its back and faces upward Numeric, in bpm 0–300 NCI Thesaurus NCI Thesaurus Code C62167     Upright heart rate Heart rate measured while in the upright posture during tilt table testing or standing test Numeric, in bpm 0–300    Blood pressure A measurement that describes the pressure of the circulating blood against the walls of the blood vessels. Measurement is taken from the right arm Numeric, in mmHg; displayed as systolic pressure over diastolic pressure 0–200 2017 AHA/ACC Key Data Elements and Definitions for Ambulatory Electronic Health Records in Pediatric and Congenital Cardiology [ 17 ]     Supine blood pressure Blood pressure measured while lying down during tilt table testing or standing test Numeric, in mmHg; displayed as systolic pressure over diastolic pressure 0–200 NCI Thesaurus NCI Thesaurus Code C62167     Seated blood pressure Blood pressure measured in the posture of one who occupies a seat Numeric, in mmHg; displayed as systolic pressure over diastolic pressure 0–200 NCI Thesaurus NCI Thesaurus Code C62122     Upright blood pressure Blood pressure that is measured while in the upright posture during tilt table testing or standing test Numeric, in mmHg; displayed as systolic pressure over diastolic pressure 0–200    End-tidal CO 2 A noninvasive measurement of exhaled carbon dioxide at the end of exhalation Numeric, in mmHg 20–70 Modified from www.aastweb.org Technical Guideline End-Tidal CO2 May 2018    Near infrared regional spectroscopy/NIRS A spectroscopic method to measure regional blood flow/oxygenation to the forehead, often used in comparison to skeletal and splanchnic light-based technology, used to noninvasively monitor tissue oxygen status Pelicer A and del Carmen Bravo M. Semin Fetal Neonatal Med 2011;16(1):42–9 [ 55 ]    Oxygen saturation A measurement that describes the determination of oxygen-hemoglobin saturation of blood Numeric, in % 20–100 2017 AHA/ACC Key Data Elements and Definitions for Ambulatory Electronic Health Records in Pediatric and Congenital Cardiology [ 17 ]    Duration of supine position Amount of time spent while lying down in the face-up position prior to tilt table testing Numeric, in min 0–120    Duration of upright tilt Amount of time spent while on the tilt table in the upright position during testing Numeric, in min 0–120    Angle of incline The angle at which a tilt table is elevated during testing Numeric, in degrees 0–85  Thermoregulatory sweat testing A test that evaluates the integrity of central and peripheral sympathetic sudomotor pathways from the central nervous system to cutaneous sweat glands by raising core body temperature and visualizing degree and extend of sweat production with an indicator dye Illigens BMW and Gibbons CH Clin Auton Res 2009;19(2):79–87 [ 56 ]  Quantitative sudomotor axon reflex testing (QSART) A test that evaluates postganglionic sympathetic cholinergic sudomotor function by measuring the axon-reflex mediated sweat response over time and has achieved widespread clinical use. Sweat glands are stimulated via iontophoresis of a cholinergic agent and the sweat production is measured as an increase of humidity through a hygrometer Illigens BMW and Gibbons CH Clin Auton Res 2009;19(2):79–87 [ 56 ]  Sweat output The amount of perspiration generated during QSART testing Numeric, in μl/cm 2 0.5–10 Illigens BMW and Gibbons CH Clin Auton Res 2009;19(2):79–87 [ 56 ]  Valsalva A test that evaluates (1) sympathetic adrenergic functions using the blood pressure responses; and (2) cardiovagal (parasympathetic) functions using the heart rate responses. Valsalva maneuver consists of forced expiration against the resistance of a semi closed glottis with the expiratory pressure during strain at 40 mmHg for 15–20 s Modified from Novak P J Vis Exp 2011;53:2502 [ 57 ]  Valsalva phase 1 heart rate Measurement of the minimum heart rate in the first phase of Valsalva testing Numeric, in bpm 0–300  Valsalva phase 1 blood pressure Measurement of the maximum blood pressure in the first phase of Valsalva testing Numeric, in mmHg; displayed as systolic pressure over diastolic pressure 0–200  Valsalva phase 2 heart rate Measurement of the maximum heart rate in the second phase of Valsalva testing Numeric, in bpm 0–300  Valsalva phase 2 blood pressure Measurement of the minimum blood pressure in the second phase of Valsalva testing Numeric, in mmHg; displayed as systolic pressure over diastolic pressure 0–200  Valsalva phase 3 heart rate Measurement of the maximum heart rate in the third phase of Valsalva testing Numeric, in bpm 0–300  Valsalva phase 3 blood pressure Measurement of the minimum blood pressure in the third phase of Valsalva testing Numeric, in mmHg; displayed as systolic pressure over diastolic pressure 0–200  Valsalva phase 4 heart rate Measurement of the minimum heart rate in the fourth phase of Valsalva testing Numeric, in bpm 0–300  Valsalva phase 4 blood pressure Measurement of the maximum blood pressure in the fourth phase of Valsalva testing Numeric, in mmHg; displayed as systolic pressure over diastolic pressure 0–200  Valsalva ratio The ratio of maximal tachycardia to the maximal bradycardia induced by the Valsalva maneuver Numeric, no units 1.00–2.00 Levin AB Am J Cardiol 1966;18(1):90–9 [ 58 ] Novak P J Vis Exp 2011;53:2502 [ 57 ]  Maximal decrease in mean blood pressure in phase 2 The difference between the highest and lowest average blood pressure measurements during phase 2 Numeric, in mmHg; displayed as systolic pressure over diastolic pressure 0–200  Maximal pulse pressure decrease in phase 2 The difference in pulse pressure between the maximal and the lowest blood pressure measurements in phase 2 Numeric, in mmHg; displayed as systolic pressure over diastolic pressure 0–200  Overshoot in phase 4 The difference between the maximal and the baseline blood pressure measurement in phase 4 Numeric, in mmHg; displayed as systolic pressure over diastolic pressure 0–200  Pressure recovery time The amount of time for blood pressure to return to baseline after phase 4 Numeric, in seconds 0–10  Pupillometry A technique used to measure the diameter of the pupil in response to stimuli NCI Thesaurus NCI Thesaurus Code C120540  Pupil diameter A measurement of the widest part of the opening of the iris through which light passes Numeric, in mm 2–8  Initial pupil diameter A measurement of the pupil diameter at baseline prior to light stimulus Numeric, in mm 2–8 Winston M, et al. Clin Auton Res 2020;30(1):43–51 [ 59 ]  End pupil diameter A measurement of the pupil diameter after light stimulus Numeric, in mm 2–8 Winston M, et al. Clin Auton Res 2020;30(1):43–51 [ 59 ]  Percent pupillary constriction (delta) The percent decrease in pupil diameter after light stimulus Numeric, in % -50–0 Winston M, et al. Clin Auton Res 2020;30(1):43–51 [ 59 ]  Pupillary latency The time delay from light stimulus to onset of pupillary constriction Numeric, in seconds 0–5 Winston M, et al. Clin Auton Res 2020;30(1):43–51 [ 59 ]  Average constriction velocity The mean speed of pupillary constriction after light stimulus Numeric, in mm/s -5–0 Winston M, et al. Clin Auton Res 2020;30(1):43–51 [ 59 ]  Maximum constriction velocity The peak speed of pupillary constriction after light stimulus Numeric, in mm/s -8–0 Winston M, et al. Clin Auton Res 2020;30(1):43–51 [ 59 ]  Average re-dilation velocity The mean speed of pupillary relaxation after removal of light stimulus Numeric, in mm/s 0–5 Winston M, et al. Clin Auton Res 2020;30(1):43–51 [ 59 ]  T75 Time from light stimulus until pupil diameter returns to 75% of initial pupil diameter after re-dilation Numeric, in seconds 0.5–5.0 Winston M, et al. Clin Auton Res 2020;30(1):43–51 [ 59 ]  Gastric emptying A test that evaluates the process of emptying food from the stomach, including wireless motility capsule, scintigraphy, and breath test Modified from www.medicinenet.com  Sitz marker A test that evaluates the transit of food and/or stool through the intestinal tract using radiopaque markers Kim ER and Rhee PL J Neurogastroenterol Motil 2012;18(1):94–99 [ 60 ]  SmartPill (wireless motility capsule) A proprietary ingestible capsule for gastrointestinal tract functional assessment that measures pressure, pH, temperature, and transit time as it travels through the entire gastrointestinal tract NCI Thesaurus NCI Thesaurus Code C132255  Endoscopy A diagnostic or therapeutic procedure in which an endoscope is inserted in a tubular organ to examine the structural architecture and/or remove abnormal tissues NCI Thesaurus NCI Thesaurus Code C16546  Esophagogastroduodenoscopy An endoscopic procedure that visualizes the upper part of the gastrointestinal tract up to the duodenum NCI Thesaurus NCI Thesaurus Code C78144  Colonoscopy Endoscopic examination of the luminal surface of the colon NCI Thesaurus NCI Thesaurus Code C16540  Abdominal ultrasonography A method of ultrasound imaging in which the ultrasound probe is pressed against the skin of the abdomen to create an image of the abdominal organs NCI Thesaurus NCI Thesaurus Code C71375  Celiac artery Doppler ultrasonography Diagnostic imaging that uses sound waves (ultrasound) applying the Doppler effect, with frequency-shifted ultrasound reflections produced by moving targets (usually red blood cells) in the bloodstream along the ultrasound axis in direct proportion to the velocity of movement of the targets, to determine both direction and velocity of blood flow in the celiac artery Modified from NCI Thesaurus NCI Thesaurus Code C62781  CT angiography of celiac axis Angiography that uses multi-detector computed tomography to assess the anatomy of the celiac artery NCI Thesaurus NCI Thesaurus Code C157338  Percutaneous celiac ganglion block The injection of a local anesthetic with the aim of inhibiting neurotransmission without the ablation of neurons Chak A Gastrointest Endosc 2009;69(2 Suppl):S172-3 [ 61 ]  Electrogastrogram A test in which the electrical current generated by the muscle of the stomach is sensed and recorded www.medicinenet.com  Electrocardiogram The record produced by the variations in electrical potential caused by electrical activity of the heart muscle and detected at the body surface, as a method for studying the action of the heart muscle 2017 AHA/ACC Key Data Elements and Definitions for Ambulatory Electronic Health Records in Pediatric and Congenital Cardiology [ 17 ]  Echocardiogram The recording of the position and motion of the heart walls or internal structures of the heart by the echo obtained from beams of the ultrasonic waves directed through the chest wall NCI Thesaurus NCI Thesaurus Code C37945  Holter monitor An ambulatory electrocardiography technique that records a continuous electrocardiographic rhythm pattern for 24 h or more to detect heart arrhythmias and assess heart rate variability Modified from NCI Thesaurus NCI Thesaurus Code C38064  Cardiac event monitor A medical device used to record the electrical activity of the heart. Often, the wearer will input a signal that is used as an event marker when they feel symptoms such as tachycardia, as the device continuously records cardiac activity NCI Thesaurus NCI Thesaurus Code C80401  Implantable loop recorder A subcutaneous device used for diagnosing heart rhythm disorders Bisignani A, et al. J Arrhythm 2019;35(1):25–32 [ 62 ] Diagnoses The investigation, analysis and recognition of the presence and nature of disease, condition, or injury from expressed signs and symptoms; also, the scientific determination of any kind; the concise results of such an investigation NCI Thesaurus NCI Thesaurus Code C15220  Dizziness A nonspecific term indicating a sensation of lightheadedness, unsteadiness, turning, vertigo, or rocking Modified from NCI Thesaurus AND Reilly BM (1990) Dizziness. In: Walker HK, et al. (eds.) Clinical Methods: The History, Physical, and Laboratory Examinations, 3 rd Ed. Butterworth, Boston, Ch. 212 [ 20 ] NCI Thesaurus Code C37943  Lightheadedness A feeling of faintness or wooziness, or a sensation of motion within the head that may be accompanied by visual or auditory changes, diaphoresis, nausea, or tachycardia Modified from NCI Thesaurus NCI Thesaurus Code C118255  Vertigo A feeling of movement, a sensation as if the external world was revolving around the patient Modified from NCI Thesaurus NCI Thesaurus Code C38057  Orthostatic intolerance A condition in which there is the presence of ≥ 1 symptoms (e.g., dizziness, lightheadedness, nausea, dyspnea, vision change) occurring specifically when assuming or maintaining upright position and resolving in the seated or supine position 2017 AHA/ACC Key Data Elements and Definitions for Ambulatory Electronic Health Records in Pediatric and Congenital Cardiology [ 17 ]  Chronic orthostatic intolerance A condition in which there is orthostatic intolerance lasting for at least 3 months with associated functional impairment 2017 AHA/ACC Key Data Elements and Definitions for Ambulatory Electronic Health Records in Pediatric and Congenital Cardiology [ 17 ]  Syncope A transient loss of consciousness and postural tone due to cerebral hypoperfusion, characterized by a rapid onset, short duration, and spontaneous complete recovery Brignole M, et al. Eur Heart J 2018;39:1883–1948 [ 21 ] 2017 AHA/ACC Key Data Elements and Definitions for Ambulatory Electronic Health Records in Pediatric and Congenital Cardiology [ 17 ]  Reflex syncope A transient loss of consciousness and postural tone due to a reflex response that encompasses vasodilation and/or bradycardia leading to systemic hypotension and cerebral hypoperfusion https://www.uptodate.com/contents/reflex-syncope-in-adults-and-adolescents-treatment 2017 AHA/ACC Key Data Elements and Definitions for Ambulatory Electronic Health Records in Pediatric and Congenital Cardiology [ 17 ] Brignole M, et al. Eur Heart J 2018;39:e43-e80 [ 21 ]  Neurally mediated syncope A type of reflex syncope typically triggered by orthostasis leading to vasodilation and/or bradycardia that includes vasodepressor cardioinhibitory, and mixed syncope Modified from Brignole M, et al. Eur Heart J 2018;39:e43-e80 [ 21 ] Also called vasovagal syncope, neurocardiogenic syncope  Vasodepressor syncope A type of neurally mediated syncope characterized primarily by reflex vasodilation Modified from Brignole M, et al. Eur Heart J 2018;39:e43-e80 [ 21 ]  Cardioinhibitory syncope A type of neurally mediated syncope characterized primarily by reflex bradycardia Modified from Brignole M, et al. Eur Heart J 2018;39:e43-e80 [ 21 ]  Mixed syncope A type of neurally mediated syncope characterized by reflex vasodilation and reflex bradycardia Modified from Brignole M, et al. Eur Heart J 2018;39:e43-e80 [ 21 ]  Situational syncope A type of reflex syncope typically triggered by specific events, such as micturition, gastrointestinal stimulation, cough or sneeze, or exercise Modified from Brignole M, et al. Eur Heart J 2018;39:e43-e80 [ 21 ]  Syncope due to orthostatic hypotension A syncopal event due to an inadequate increase in peripheral resistance and heart rate or due to severe hypovolemia upon standing Modified from Brignole M, et al. Eur Heart J 2018;39:e43-e80 [ 21 ]  Orthostatic hypotension A progressive and sustained drop in blood pressure upon standing from a sitting or supine position in which there is a decrease of systolic blood pressure by ≥ 20 mmHg and/or a decrease of diastolic blood pressure by ≥ 10 mmHg within the first 3 min of upright position Modified from Brignole M, et al. Eur Heart J 2018;39:e43-e80 [ 21 ]  Initial orthostatic hypotension A sudden drop in blood pressure upon standing from a sitting or supine position in which there is a decrease of systolic blood pressure by ≥ 40 mmHg and/or a decrease in diastolic blood pressure by ≥ 20 mmHg within the first 15 s of standing that spontaneously resolves with a relatively rapid return to pre-challenge values Modified from Brignole M, et al. Eur Heart J 2018;39:e43-e80 [ 21 ]  Cardiac syncope A syncopal event due to a primary cardiac etiology, such as an arrhythmia or structural lesion  Postural orthostatic tachycardia syndrome A type of chronic orthostatic intolerance lasting ≥ 3 months associated with excessive upright sinus tachycardia in the absence of orthostatic hypotension, plus a constellation of typically daily symptoms that may include lightheadedness, dizziness, nausea, dyspnea, diaphoresis, headache, fatigue, and other symptoms of autonomic dysfunction. Excessive tachycardia is defined by present consensus as a sustained heart rate increase of at least 30 bpm in adults (40 bpm for adolescents), or a heart rate > 120 bpm (in adults), within 10 min of upright tilt-table testing Modified from 2017 AHA/ACC Key Data Elements and Definitions for Ambulatory Electronic Health Records in Pediatric and Congenital Cardiology [ 17 ] Also called POTS  Hyperadrenergic postural orthostatic tachycardia syndrome A phenotype of postural orthostatic tachycardia syndrome in which there is excessive sympathetic vasoconstriction, increased peripheral resistance, and typically excessively high serum catecholamine levels, with greatly elevated upright tachycardia and hypertension  Neuropathic postural orthostatic tachycardia syndrome A phenotype of postural orthostatic tachycardia syndrome in which there is impaired sympathetic activity in the lower extremities or splanchnic vasculature with arteriolar dysregulation  Dysautonomia A condition in which altered function of one or more components of the autonomic nervous system adversely affects health Goldstein DS Principles of Autonomic Medicine v 4.0 [ 42 ] Also called autonomic nervous system dysfunction  Inappropriate sinus tachycardia A sinus heart rate > 100 bpm at rest (with a mean 24-h heart rate > 90 bpm not due to primary causes) and is associated with distressing symptoms of palpitations Sheldon RS Heart Rhythm 2015;12(6):e41-e63 [ 44 ]  Hypertension The average clinic-measured systolic and/or diastolic blood pressure ≥ 95th percentile for age, sex, and height percentiles Flynn JT Pediatrics 2017;140(3):e20171904 [ 45 ]  Hypotension Low systolic blood pressure, as defined by age parameters: 0–1 year: < 5th percentile for gestational and/or postnatal age 1–10 years:  10 years: < 90/50 mmHg Dionne J Hypertension 2021;77(3):781–787 [ 46 ] Kleinman ME Circulation 2010;122(18Suppl3):S876-S908 [ 47 ]  Post COVID-19 syndrome A condition in which there are multiorgan symptoms and complications beyond the initial period of acute infection and illness with SARS-CoV-2 Venkatesan P Lancet Respir Med 2021;9(2):129 [ 43 ] Also called long COVID, long-haul COVID, post-acute sequelae of SARS-CoV-2 infection (PASC)  Myalgic encephalomyelitis-chronic fatigue syndrome A syndrome of unknown etiology characterized by an unexplained persistent or relapsing chronic fatigue that is of at least 6 months’ duration, is not the result of ongoing exertion, is not substantially alleviated by rest, and results in substantial reduction of previous levels of occupational, educational, social, or personal activities NCI Thesaurus NCI Thesaurus Code C3037  Psychogenic pseudosyncope The appearance of transient loss of consciousness in the absence of true loss of consciousness Raj V Auton Neurosci 2014;184:66–72 [ 63 ]  Sinus tachycardia A disorder characterized by an electrocardiographic finding of abnormally rapid heart rate with its origin in the sinus node NCI Thesaurus NCI Thesaurus Code C26889  Tachycardia secondary to arrhythmia A disorder characterized by an electrocardiographic finding of an abnormally rapid heart rate due to an abnormal heart rhythm  Mast cell activation syndrome A mast cell activation disorder in which there are episodic multisystem symptoms consistent with mast cell activation, appropriate response to medications targeting mast cell activation, and documented increase in validated markers of mast cell activation Akin C. J Allergy Clin Immunol 2017;140(2):349–55 [ 49 ] Also called MCAS  Ehlers–Danlos syndrome A group of inherited connective tissue disorders characterized by loose and fragile skin and joint hypermobility NCI Thesaurus NCI Thesaurus Code C34568 Also called EDS   Hypermobile EDS A type of Ehlers–Danlos syndrome characterized by generalized joint hypermobility, plus systemic manifestations of a generalized connective tissue disorder, positive family history, and/or musculoskeletal complications, plus exclusion of other types of Ehlers–Danlos syndrome Malfait F, et al. Am J Med Genet C Semin Med Genet 2017;175(1):8–26 [ 48 ] Also called hEDS   Classical EDS A type of Ehlers–Danlos syndrome characterized by skin hyperextensibility, generalized joint hypermobility, plus other minor criteria associated with a mutation in collagen in the COL5A1 , COL5A2 , COL1A1 , or COL1A2 genes Malfait F, et al. Am J Med Genet C Semin Med Genet 2017;175(1):8–26 [ 48 ] Also called cEDS   Vascular EDS A type of Ehlers–Danlos syndrome characterized by arterial, uterine, or gastrointestinal rupture at a young age, atraumatic formation of carotid-cavernous fistula, plus other minor criteria associated with a mutation in collagen in the COL3A1 gene Malfait F, et al. Am J Med Genet C Semin Med Genet 2017;175(1):8–26 [ 48 ] Also called vEDS  Hypermobility spectrum disorder A group of clinically relevant conditions related to joint hypermobility in which phenotypic domains are limited to the musculoskeletal system Castori M, et al. Am J Med Genet C Semin Med Genet 2017;175(1):148–57 [ 36 ] Also called HSD  Median arcuate ligament syndrome A condition characterized by abdominal pain attributed to compression of the celiac artery and the celiac ganglia by the median arcuate ligament www.wikipedia.org Also called MALS  Small fiber neuropathy Neuropathy caused by damage to the small myelinated (A-delta) fibers or unmyelinated C fibers in the peripheral nerves NCI Thesaurus NCI Thesaurus Code C125389  Sjögren syndrome An autoimmune disorder affecting the salivary and lacrimal glands. Morphologically, it is characterized by the presence of lymphocytic and plasmacytic infiltrates which cause destruction of these glands NCI Thesaurus NCI Thesaurus Code C26883  Irritable bowel syndrome Gastrointestinal symptoms characterized by chronic abdominal pain and altered bowel habits in the absence of any organic cause. One of the functional gastrointestinal disorders Modified from NCI Thesaurus NCI Thesaurus Code C82343  Rumination An eating disorder most commonly observed in infants and adolescents characterized by the repeated regurgitation and rechewing of food for a period of at least 1 month; this behavior is not associated with a gastrointestinal or other medical reason. One of the functional gastrointestinal disorders  Nausea A difficult-to-describe sensation of sick or queasy feeling usually perceived as being in the stomach that can escalate in severity and may precede vomiting Stern RM, Koch KL, Andrews PLR (2001) Nausea: Mechanisms and Management. Oxford University Press, New York [ 29 ]  Constipation Difficulties in defecation that include infrequent bowel movements, hard or lumpy stools, excessive straining, sensation of incomplete evacuation or blockage and, in some instances, the use of manual maneuvers to facilitate evacuation Aziz I, et al. Expert Rev Gastroenterol Hepatol 2020;14:39–46 [ 30 ]  Gastroparesis Paralysis of the muscles of the stomach wall resulting in delayed emptying of the gastric contents into the small intestine NCI Thesaurus NCI Thesaurus Code C80512  Dysmotility A descriptive term used to describe diseases of the gastrointestinal tract in which the muscles and/or nerves do not work normally Modified from www.medicinenet.com  Migraine A common, severe type of vascular headache often associated with increased sympathetic activity, resulting in nausea, vomiting, and light sensitivity NCI Thesaurus NCI Thesaurus Code C89715  Tension headache A headache associated with muscle tightness that may radiate to other parts of the body NCI Thesaurus NCI Thesaurus Code C117070  Cluster headache A headache disorder that is characterized by periodic severe, unilateral orbital, supraorbital, and/or temporal pain, and is associated with ipsilateral cranial autonomic symptoms NCI Thesaurus NCI Thesaurus Code C117077  New persistent daily headache A bilateral, unremitting headache of mild to moderate intensity that occurs daily and may be associated with photophobia, phonophobia, or mild nausea NCI Thesaurus NCI Thesaurus Code C117082  Craniocervical instability A pathological condition in which ligamentous connections from the skull to the spine are incompetent Henderson FC Am J Med Genet C Semin Med Genet 2017;175(1):195–211 [ 64 ]  Cerebrospinal fluid leak The loss of cerebrospinal fluid into the surrounding tissues NCI Thesaurus NCI Thesaurus Code C50487  Thyroid disease The classification of autoimmune thyroid disease (AITD) includes Hashimoto thyroiditis (HT) or chronic autoimmune thyroiditis and its variants, Graves disease (GD) and autoimmune atrophic thyroiditis or primary myxedema NCI Thesaurus NCI Thesaurus Code C91510   Graves disease Hyperthyroidism associated with diffuse hyperplasia of the thyroid gland (goiter), resulting from production of antibodies that are directed against the thyrotropin receptor complex of the follicular epithelial cells. As a result, the thyroid gland enlarges and secretes increased amounts of thyroid hormones NCI Thesaurus NCI Thesaurus Code C3071   Hashimoto thyroiditis An autoimmune disorder caused by the production of autoantibodies against thyroid tissue. There is progressive destruction of the thyroid follicles leading to hypothyroidism NCI Thesaurus NCI Thesaurus Code C27191  Adrenal failure, hypoadrenalism, Addison disease A long-standing and persistent diminished production of adrenocortical hormones due to a disorder originating within the adrenal glands NCI Thesaurus NCI Thesaurus Code C26689  Pheochromocytoma A benign or malignant neuroendocrine neoplasm of the sympathetic nervous system that secretes catecholamines. It arises from the chromaffin cells of the adrenal medulla NCI Thesaurus NCI Thesaurus Code C3326  Carcinoid syndrome A syndrome characterized by signs and symptoms caused by advanced carcinoid tumors. They include skin flushing, diarrhea, wheezing, and tachycardia NCI Thesaurus NCI Thesaurus Code C3215  Paraganglioma A benign or malignant neoplasm arising from paraganglia located along the sympathetic or parasympathetic nerves NCI Thesaurus NCI Thesaurus Code C3308  Neuroblastoma A neuroblastic tumor characterized by the presence of neuroblastic cells, the absence of ganglion cells, and the absence of a prominent Schwannian stroma formation NCI Thesaurus NCI Thesaurus Code C3270  Ganglioneuroma A benign neuroblastic tumor of the sympathetic nervous system that occurs in childhood. Ganglioneuroma typically arises from the sympathetic chain in the mediastinum or adrenal glands Modified from NCI Thesaurus NCI Thesaurus Code C3049  Ganglioneuroblastoma A neuroblastic tumor characterized by the presence of neuroblastic cells, ganglion cells, and a stroma with Schwannian differentiation constituting more than 50% of the tumor volume NCI Thesaurus NCI Thesaurus Code C3790  Eosinophilic esophagitis Allergic inflammation of the esophagus. Morphologically, it is characterized by the presence of eosinophils infiltrating the esophageal epithelium NCI Thesaurus NCI Thesaurus Code C27105  Visceral hypersensitivity Heightened sensitivity or reduced thresholds for pain and discomfort associated with the gastrointestinal tract Modified from Mertz H. Aliment Pharmacol Ther 2003;17(5):623–33 [ 31 ] AND Faure C, Di Lorenzo C, Thapar N. (2017) Pediatric Neurogastroenterology Gastrointestinal Motility and Functional Disorders in Children, 2nd Ed. Springer International Publishing, Switzerland [ 33 ] Also called visceral hyperalgesia  Gastroesophageal reflux The backward flow of stomach acid contents into the esophagus (the tube that connects the mouth to the stomach) NCI Thesaurus NCI Thesaurus Code C92560  Breath-holding spell A clinical condition in which there is a brief involuntary cessation of breathing at end expiration in response to emotional or painful stimulus seen in infants, toddlers, and children 2017 AHA/ACC Key Data Elements and Definitions for Ambulatory Electronic Health Records in Pediatric and Congenital Cardiology [ 17 ]  Simple breath-holding spell A clinical condition in which there is a breath-holding spell without syncope 2017 AHA/ACC Key Data Elements and Definitions for Ambulatory Electronic Health Records in Pediatric and Congenital Cardiology [ 17 ]  Complex breath-holding spell A clinical condition in which there is a breath-holding spell associated with syncope 2017 AHA/ACC Key Data Elements and Definitions for Ambulatory Electronic Health Records in Pediatric and Congenital Cardiology [ 17 ]  Pallid breath-holding spell A clinical condition in which there is a breath-holding spell associated with pale appearance 2017 AHA/ACC Key Data Elements and Definitions for Ambulatory Electronic Health Records in Pediatric and Congenital Cardiology [ 17 ]  Cyanotic breath-holding spell A clinical condition in which there is a breath-holding spell associated with cyanotic appearance 2017 AHA/ACC Key Data Elements and Definitions for Ambulatory Electronic Health Records in Pediatric and Congenital Cardiology [ 17 ]  Seizure disorder A brain disorder characterized by episodes of abnormally increased neuronal discharge resulting in transient episodes of sensory or motor neurological dysfunction, or psychic dysfunction that may or may not be associated with loss of consciousness or convulsions NCI Thesaurus NCI Thesaurus Code C3020 Also called epilepsy  Spinal cord injury Traumatic damage of the spinal cord NCI Thesaurus NCI Thesaurus Code C50750  Autonomic dysreflexia A form of paroxysmal autonomic adrenergic overactivity due to disinhibited spinal reflexes in spinal cord injury above the T6 level Novak P. Am J Med 2019;132(4):420–436 [ 51 ]  Anxiety disorder A category of psychiatric disorders which are characterized by anxious feelings or fear often accompanied by physical symptoms associated with anxiety NCI Thesaurus NCI Thesaurus Code C2878  Panic disorder An anxiety disorder characterized by multiple unexpected panic attacks with persistent concern of recurring attacks NCI Thesaurus NCI Thesaurus Code C34890  Depression A mental condition marked by ongoing feelings of sadness, despair, loss of energy, and difficulty dealing with normal daily life NCI Thesaurus NCI Thesaurus Code C2982  Obsessive–compulsive disorder A disorder characterized by the presence of persistent and recurrent irrational thoughts (obsessions), resulting in marked anxiety and repetitive excessive behaviors (compulsions) as a way to try to decrease that anxiety NCI Thesaurus NCI Thesaurus Code C88411  Attention deficit/hyperactivity disorder A neurodevelopmental disorder defined by impairing levels of inattention, disorganization, and/or hyperactivity–impulsivity DSM-V Also called ADHD  Somatic symptom disorder A. One or more somatic symptoms that are distressing or result in significant disruption of daily life B. Excessive thoughts, feelings, or behaviors related to the somatic symptoms or associated health concerns C. Although any one somatic symptom may not be continuously present, the state of being symptomatic is persistent (typically more than 6 months) DSM-V  Conversion disorder (functional neurological symptom disorder) A. One or more symptoms of altered voluntary motor or sensory function B. Clinical findings provide evidence of incompatibility between the symptom and recognized neurological or medical conditions C. The symptom or deficit is not better explained by another medical or mental disorder D. The symptom or deficit causes clinically significant distress or impairment in social, occupational, or other important areas of functioning or warrants medical evaluation DSM-V  Hereditary and sensory neuropathy, type I An autosomal dominant condition caused by mutation(s) in the SPTLC1 gene characterized by distal sensory impairment with variable autonomic and motor involvement NCI Thesaurus NCI Thesaurus Code C170433  Hereditary and sensory neuropathy, type IIA (HSAN2A) A rare congenital disorder caused by various mutations in the WNK1 gene characterized by sensory loss, self-mutilation, and mild or absent autonomic involvement Modified from https://www.ncbi.nlm.nih.gov/books/NBK49247/ and https://www.omim.org/entry/201300  Hereditary and sensory neuropathy, type IIB (HSAN2B, Morvan’s disease; congenital sensory neuropathy) A rare autosomal recessive disorder caused by various mutations in the FAM134B gene characterized by decreased distal sensation and variable autonomic involvement Modified from https://www.ncbi.nlm.nih.gov/books/NBK49247/ and https://www.omim.org/entry/613115  Hereditary and sensory neuropathy, type III (HSAN3, familial dysautonomia; Riley–Day syndrome) A rare congenital disorder caused by a founder mutation in the ELP-1 gene characterized by initial hypotonia and feeding difficulties with subsequent blood pressure instability and hypertensive vomiting attacks alternating with hypotension due to loss of sensory inputs from cranial nerves IX and X resulting in afferent baroreflex failure Modified from https://www.omim.org/entry/223900  Hereditary and sensory neuropathy, type IV (HSAN4, congenital insensitivity to pain with anhidrosis; CIPA) A rare autosomal recessive disorder caused by different mutations in the NTRK1 gene affecting peripheral sensory and autonomic fibers, and is characterized by inability to feel pain and temperature that leads to repeated unintentional self-injuries, severe cognitive impairment, and anhidrosis that leads to hyperpyrexia and febrile seizures Modified from NCI Thesaurus and https://www.omim.org/entry/256800 NCI Thesaurus Code C118633  Hereditary and sensory neuropathy, type V (HSAN5) An autosomal recessive condition caused by mutations in the NGF or NTRK1 genes affecting peripheral small fibers characterized by loss of pain sensation, particularly in the extremities, and variable anhidrosis, with normal cognitive function as compared with HSAN4 Modified from NCI Thesaurus and https://www.omim.org/entry/608654 NCI Thesaurus Code C156360  Hereditary and sensory neuropathy, type VI (HSAN6) An extremely rare inherited disorder caused by mutations in the DST gene characterized by neonatal expression of diminished pain and labile blood pressure https://www.ncbi.nlm.nih.gov/medgen/761278 and https://www.omim.org/entry/614653  Hereditary and sensory neuropathy, type VII (HSAN7) A rare disorder caused by mutations in the SCN11A gene, characterized by insensitivity to pain as well as gastrointestinal dysfunction Modified from NCI Thesaurus and https://www.omim.org/entry/615548 NCI Thesaurus Code C125388  Hereditary and sensory neuropathy, type VIII (HSAN8) An autosomal recessive disorder caused by mutations in the PRDM12 gene characterized by congenital insensitivity to pain; anhidrosis and alacrima may occur Modified from https://www.ncbi.nlm.nih.gov/medgen/894363 and https://www.omim.org/entry/616488  Rett syndrome A progressive neurologic disorder caused by mutations in the MECP2 gene on chromosome X. It almost exclusively affects girls. It is characterized by breath-holding spells, flushing, repetitive hand motions, lack of advancement in language, and regression in developmental milestones. Other signs and symptoms include slowed head growth, scoliosis, breathing abnormalities (including sleep-disordered breathing), and sleep disturbances Modified from NCI Thesaurus NCI Thesaurus Code C75488  Hirschsprung disease A congenital disorder characterized by the absence of myenteric ganglion cells in the colon that occurs distally but can involve the proximal colon resulting in a functional stenosis of the distal colon and a massive distention of the proximal colon Modified from NCI Thesaurus NCI Thesaurus Code C34700  Rapid-onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation A neurocristopathy characterized by hypothalamic dysfunction with inability to maintain normal salt-water balance, growth hormone deficiency, hypothyroidism, early or late puberty, increased prolactin concentration, hypoventilation, attenuated/absent peripheral and central chemoreceptor responsiveness, and autonomic dysfunction Modified from NCI Thesaurus NCI Thesaurus Code C121944 Also called ROHHAD  Congenital central hypoventilation syndrome A neurocristopathy associated with heterozygous PHOX2B gene mutations characterized by hypoventilation and hypoxemia typically during sleep with attenuated/absent peripheral and central chemoreceptor responsiveness, and autonomic dysregulation; symptoms typically appear in the newborn period and can co-occur with Hirschsprung disease and neural crest tumors Modified from NCI Thesaurus NCI Thesaurus Code C98889 Also called CCHS  Tracheostomy A surgically created external opening into the trachea NCI Thesaurus NCI Thesaurus Code C122643 HR heart rate, BP blood pressure, bpm beats per minute Brignole M, et al. Eur Heart J 2018;39:1883–1948 [ 21 ] 2017 AHA/ACC Key Data Elements and Definitions for Ambulatory Electronic Health Records in Pediatric and Congenital Cardiology [ 17 ] Modified from Mertz H. Aliment Pharmacol Ther 2003;17(5):623–33 [ 32 ] AND Faure C, Di Lorenzo C, Thapar N. (2017) Pediatric Neurogastroenterology Gastrointestinal Motility and Functional Disorders in Children, 2 nd Ed. Springer International Publishing, Switzerland [ 33 ] NCI Thesaurus Code C169076 Also called SARS-CoV-2, SARS coronavirus 2 Brignole M, et al. Eur Heart J 2018;39:1883–1948 [ 21 ] 2017 AHA/ACC Key Data Elements and Definitions for Ambulatory Electronic Health Records in Pediatric and Congenital Cardiology [ 17 ] https://www.uptodate.com/contents/reflex-syncope-in-adults-and-adolescents-treatment 2017 AHA/ACC Key Data Elements and Definitions for Ambulatory Electronic Health Records in Pediatric and Congenital Cardiology [ 17 ] Brignole M, et al. Eur Heart J 2018;39:e43-e80 [ 21 ] Low systolic blood pressure, as defined by age parameters: 0–1 year: < 5th percentile for gestational and/or postnatal age 1–10 years:  10 years: < 90/50 mmHg Dionne J Hypertension 2021;77(3):781–787 [ 46 ] Kleinman ME Circulation 2010;122(18Suppl3):S876-S908 [ 47 ] Modified from Mertz H. Aliment Pharmacol Ther 2003;17(5):623–33 [ 32 ] AND Faure C, Di Lorenzo C, Thapar N. (2017) Pediatric Neurogastroenterology Gastrointestinal Motility and Functional Disorders in Children, 2 nd Ed. Springer International Publishing, Switzerland [ 33 ] NCI Thesaurus Code C34568 Also called EDS An eating or feeding disturbance (e.g., apparent lack of interest in eating or food; avoidance based on the sensory characteristics of food; concern about aversive consequences of eating) as manifested by persistent failure to meet appropriate nutritional and/or energy needs associated with one (or more) of the following: 1. Significant weight loss (or failure to achieve expected weight gain or faltering growth in children) 2. Significant nutritional deficiency 3. Dependence on enteral feeding or oral nutritional supplements 4. Marked interference with psychosocial functioning A. One or more somatic symptoms that are distressing or result in significant disruption of daily life B. Excessive thoughts, feelings, or behaviors related to the somatic symptoms or associated health concerns C. Although any one somatic symptom may not be continuously present, the state of being symptomatic is persistent (typically more than 6 months) A. One or more symptoms of altered voluntary motor or sensory function B. Clinical findings provide evidence of incompatibility between the symptom and recognized neurological or medical conditions C. The symptom or deficit is not better explained by another medical or mental disorder D. The symptom or deficit causes clinically significant distress or impairment in social, occupational, or other important areas of functioning or warrants medical evaluation NCI Thesaurus Code C121944 Also called ROHHAD NCI Thesaurus Code C98889 Also called CCHS NCI Thesaurus Code C3020 Also called epilepsy Brignole M, et al. Eur Heart J 2018;39:1883–1948 [ 21 ] 2017 AHA/ACC Key Data Elements and Definitions for Ambulatory Electronic Health Records in Pediatric and Congenital Cardiology [ 17 ] https://www.uptodate.com/contents/reflex-syncope-in-adults-and-adolescents-treatment 2017 AHA/ACC Key Data Elements and Definitions for Ambulatory Electronic Health Records in Pediatric and Congenital Cardiology [ 17 ] Brignole M, et al. Eur Heart J 2018;39:e43-e80 [ 21 ] NCI Thesaurus Code C34568 Also called EDS Brignole M, et al. Eur Heart J 2018;39:1883–1948 [ 14 ] 2017 AHA/ACC Key Data Elements and Definitions for Ambulatory Electronic Health Records in Pediatric and Congenital Cardiology [ 17 ] NCI Thesaurus Code C121944 Also called ROHHAD NCI Thesaurus Code C98889 Also called CCHS Modified from Mertz H. Aliment Pharmacol Ther 2003;17(5):623–33 [ 32 ] AND Faure C, Di Lorenzo C, Thapar N. (2017) Pediatric Neurogastroenterology Gastrointestinal Motility and Functional Disorders in Children, 2 nd Ed. Springer International Publishing, Switzerland [ 33 ] NCI Thesaurus Code C3020 Also called epilepsy Modified from www.aastweb.org Technical Guideline End-Tidal CO2 May 2018 Levin AB Am J Cardiol 1966;18(1):90–9 [ 58 ] Novak P J Vis Exp 2011;53:2502 [ 57 ] Brignole M, et al. Eur Heart J 2018;39:1883–1948 [ 21 ] 2017 AHA/ACC Key Data Elements and Definitions for Ambulatory Electronic Health Records in Pediatric and Congenital Cardiology [ 17 ] https://www.uptodate.com/contents/reflex-syncope-in-adults-and-adolescents-treatment 2017 AHA/ACC Key Data Elements and Definitions for Ambulatory Electronic Health Records in Pediatric and Congenital Cardiology [ 17 ] Brignole M, et al. Eur Heart J 2018;39:e43-e80 [ 21 ] Dionne J Hypertension 2021;77(3):781–787 [ 46 ] Kleinman ME Circulation 2010;122(18Suppl3):S876-S908 [ 47 ] NCI Thesaurus Code C34568 Also called EDS Modified from Mertz H. Aliment Pharmacol Ther 2003;17(5):623–33 [ 31 ] AND Faure C, Di Lorenzo C, Thapar N. (2017) Pediatric Neurogastroenterology Gastrointestinal Motility and Functional Disorders in Children, 2nd Ed. Springer International Publishing, Switzerland [ 33 ] NCI Thesaurus Code C3020 Also called epilepsy A. One or more somatic symptoms that are distressing or result in significant disruption of daily life B. Excessive thoughts, feelings, or behaviors related to the somatic symptoms or associated health concerns C. Although any one somatic symptom may not be continuously present, the state of being symptomatic is persistent (typically more than 6 months) A. One or more symptoms of altered voluntary motor or sensory function B. Clinical findings provide evidence of incompatibility between the symptom and recognized neurological or medical conditions C. The symptom or deficit is not better explained by another medical or mental disorder D. The symptom or deficit causes clinically significant distress or impairment in social, occupational, or other important areas of functioning or warrants medical evaluation Hereditary and sensory neuropathy, type III (HSAN3, familial dysautonomia; Riley–Day syndrome) NCI Thesaurus Code C121944 Also called ROHHAD NCI Thesaurus Code C98889 Also called CCHS HR heart rate, BP blood pressure, bpm beats per minute

The concept for creating the data dictionary came out of an initial research meeting of the Pediatric Section of the American Autonomic Society sponsored by the Children’s Hospital of Philadelphia in 2017. At the time, it was felt that the data dictionary would be used as a foundational dictionary for internal research projects within our group. However, over time, it became apparent that the dictionary could be applicable in multiple other disciplines, and should be made available for other providers and researchers working in the emerging discipline of pediatric autonomic medicine. The dictionary needed to be harmonized with existing catalogs of common data elements, essentially integrating and unifying components from the various known resources into our dictionary. To that end, as best as possible, we utilized the National Cancer Institute Thesaurus (NCIt) [ 19 ] as the source for dictionary terms and definitions, as it is one of the largest compilations of biomedical lexicons. At times, some of the NCIt definitions were out of date, inadequate, or inconsistent across similar terms, so in our capacity as subject matter experts, we elected to modify them. In other instances, the specific required term did not exist in the NCIt, prompting acquisition of needed definitions for terms from other consensus-driven documents or single subject matter expert publications. If none of those options could be used, we generated our own definitions with support from existing literature. The work for this project encompassed numerous in-person meetings, virtual meetings, and email correspondences. Because there is so much overlap of ANS dysregulation with other, non-ANS disorders, our dictionary not surprisingly appears to include widely disparate terms across medical conditions and their care. But it is for this very reason, such as the overlap of postural orthostatic tachycardia syndrome with entities such as joint hypermobility syndromes, functional gastrointestinal disorders, and mast cell activation syndrome, that this inclusive approach was taken. This strategy allows for meticulous documentation of the signs and symptoms across these disorders, patient and family histories, the spectrum of clinical testing necessary to correctly diagnose affected patients, and the monitoring of longitudinal treatments. As in much of medicine, the understanding of clinical syndromes and their pathophysiology, genetic basis, and other aspects of their manifestations evolve over time. Research augments the ability to comprehend these various relationships as well as to better care for these patients. Because of this, it is understood that our data dictionary will need to be a dynamic instrument, updated over time as science and medicine advance and evolve. However, it will also be a signpost to be used by adult ANS providers, who can take advantage of the work done here to create an adult-patient-based ANS data dictionary as well as to be able to care for children with autonomic conditions who advance into adulthood. The data dictionary can be a tool useful for integration with other data vocabularies, such as in offering reciprocity of new terms and updated definitions introduced by our group to the NCIt or inclusion in larger multidisciplinary studies. Lastly, it can be a step forward in helping clinicians, researchers, and informaticists to find gaps in our definitions and data content with the aim to optimize the instrument we have prepared.

Though long considered to have been “discovered” in 1921, the initial descriptions of the ANS were in 1903 and 1908 by Langley [ 9 ] and Kappers [ 10 ], respectively. However, as occurs when new findings are described in medicine and science, there is often discussion and dispute around the appropriate terminology for common use. This includes disagreement on the nomenclature utilized to describe the ANS [ 11 ]. Similar discourse continues today. For example, naming conventions around syncope have evolved in the past 20 years [ 12 – 14 ], although much of the change in today’s literature attempts to be more evidence-based as opposed to opinion-based. To that end, the use of language and, specifically, nomenclature, with its appropriate and agreed-upon terms and definitions, is key to the ability of practitioners to care for their patients and for researchers to advance the discipline in coordinated fashion. Other groups within medicine have recognized this, such as neonatology, emergency medicine, ambulatory pediatric cardiology, and neurologists who manage stroke, and have taken steps to provide data dictionaries for general use [ 15 – 18 ], the benefits of which are numerous. At a minimum, a commonly agreed upon vocabulary allows clinicians both to ensure that they are caring for patients with appropriately defined conditions and to allow communication among providers in the joint care of these patients. Increasingly, though, common agreed-upon terminology is facilitating research. It allows multi-center studies to attempt to ensure that the data they gather are consistent, rigorous, and reporting on homogeneous cohorts. Furthermore, with today’s increased use of complex computing and compilation, the ability to parse “big data” is more easily applied with a standardized set of terms and definitions. These data dictionaries can be incorporated by governmental agencies for billing and surveillance, as they are often assembled by committees composed of subject matter experts within the specific field, and therefore represent an ideal source for ensuring that common language is used within this oversight. Typically with adoption by the federal government, insurance payors will also implement the use of these terms, facilitating the ability to document, bill, and reimburse for these diagnoses and related procedures. The harmonization of these data dictionaries with existing clinical use dictionaries, either by direct juxtaposition and incorporation or by acknowledging overlapping terms and updating them to be “more correct,” allows for even larger data analyses across disciplines not previously considered. Lastly, having the availability of these lexicons for general use means that they can also incorporated by the companies who manufacture electronic health records in a vendor-agnostic way. This then feeds back both into the above benefits as well as allowing clinicians to capture discrete data while generating documentation, and to build datasets of patient information that help to improve internal clinic function and to advance clinical and translational research. Recognizing these numerous benefits, the Pediatric Section of the American Autonomic Society set out to generate a data dictionary specific to pediatric disorders of the ANS. The end product data dictionary is designed to be used both for clinical documentation and for research purposes. Although disorders of orthostatic intolerance are the most frequently diagnosed ANS disorder in children and adolescents, inclusion of less commonly identified autonomic disorders, such as the hereditary sensory and autonomic neuropathies, Rett syndrome, ROHHAD, and CCHS, allow for practitioners and researchers to leverage the same advantages afforded by this dictionary. Within informatics, incorporation of existing terms and definitions from other established terminologies is the more desired approach. This allows for better harmonization with other dictionaries in the future, and it saves the informaticists from having to recreate an entire set of terms and definitions for each application.

The majority of ANS-related conditions, as with the large majority of medical disorders, are predominantly described in adults, involving diabetes mellitus, Parkinson’s disease, multiple system atrophy, and pure autonomic failure, to name a few [ 2 ]. This likely follows from a larger proportion of clinical and research personnel in the field of adult medicine. However, there are also manifestations of ANS disorders in pediatric and adolescent patients [ 4 , 5 ]. The earliest description of the ANS in the context of pediatric patients was in 1927 [ 6 ], in which leukocyte levels were assessed in the setting of induced vasoconstriction and vasodilation. Since that time, the medical literature has evolved, growing with increasing documentation of awareness, recognition, description, and interventions for ANS disorders in childhood. However, the breadth of understanding of the ANS in the context of children remains limited as compared with adults, despite significant advances in the field as well as attempts to review and summarize them to encourage greater awareness among providers [ 7 ]. Most notable is the difference in the various ANS disorders observed and reported in the pediatric population as compared to adults. The aforementioned disorders listed above that are seen in adults are not described in children and adolescents. However, a subset of ANS disorders overlap between these populations, including orthostatic intolerance, various subtypes of syncope, initial orthostatic hypotension, orthostatic hypotension, and postural orthostatic tachycardia syndrome. There are also ANS-affecting conditions that tend to be uniquely identified in childhood. These are often the rare syndromic and genetic conditions that involve ANS disorders, including the various hereditary and sensory neuropathies (such as familial dysautonomia), Rett syndrome, rapid-onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation (ROHHAD), and congenital central hypoventilation syndrome (CCHS), as well as certain tumors of neural crest origin, such as neuroblastoma, ganglioneuroblastoma, and ganglioneuroma [ 7 ]. Interestingly, breath-holding spells, seen in infants up through young children, may be manifestations of neurocardiogenic syncope, which also can be found in adults. However, the way that they present, with episodes specifically triggered with mild pain or anger, and typically occurring after prolonged crying at end-expiration, is unique to children. Breath-holding spells are actually relatively common, occurring in up to almost 5% of children [ 8 ].

The autonomic nervous system (ANS) controls involuntary bodily functions, both at rest and during activity, affecting virtually all organ systems. Consequently, there is a wide range of conditions associated with dysregulation of the ANS. One of the earliest documented disorders of the ANS was reported in 1928, when carcinoid tumors of the appendix were described [ 1 ]. Over the next century, a panoply of diverse disorders, derangements, and dysfunctions of the ANS have been delineated, including diseases primary to the ANS as well as ANS disturbances secondary to other conditions [ 2 ]. A medical literature search for “autonomic nervous system” in 2022 identified nearly 170,000 entries, and a similar search for “autonomic nervous system disease” listed over 41,000 entries [ 3 ]. Although these represent substantially fewer literature entries compared with myocardial infarction (> 270,000 entries) or cerebrovascular accident (> 410,000 entries), it is noteworthy that research in myocardial infarction and cerebrovascular accident seem to have more relatively “recent” research, with inflection points around the 1960s and 1970s, respectively, as compared with the slow steady growth of ANS publications since the 1930s [ 3 ]. In other words, while disorders that affect single organs have not only had an explosion of relatively recent research, they have also compiled a significant body of study; the same cannot be said about the ANS. Despite the much wider range of ANS involvement and disorders across the human body as compared with numerous conditions that act on isolated organs, there is a relatively limited knowledge about the ANS, its function, and its pathophysiologic processes, especially as it pertains to pediatric patients, the progression into adulthood, prognosis, and effect on other disorders.