Kawasaki disease with shock as the primary manifestation: How to distinguish from toxic shock syndrome？ A Case Report and Literature Review

Kawasaki disease with shock as the primary manifestation: How to distinguish from toxic shock syndrome？ A Case Report and Literature Review | Research Square window.SnipcartSettings = { analytics: { enabled: false } }; (function() { var accessVector = localStorage.getItem('access_vector') || ''; window.dataLayer = window.dataLayer || []; if (accessVector) { window.dataLayer.push({ user: { profile: { profileInfo: { snid: accessVector } } } }); } })(); (function(w,d,s,l,i){w[l]=w[l]||[];w[l].push({'gtm.start':new Date().getTime(),event:'gtm.js'});var f=d.getElementsByTagName(s)[0],j=d.createElement(s),dl=l!='dataLayer'?'&l='+l:'';j.async=true;j.src='https://www.googletagmanager.com/gtm.js?id='+i+dl;f.parentNode.insertBefore(j,f);})(window,document,'script','dataLayer','GTM-K279D39R'); Browse Preprints In Review Journals COVID-19 Preprints AJE Video Bytes Research Tools Research Promotion AJE Professional Editing AJE Rubriq About Preprint Platform In Review Editorial Policies Our Team Advisory Board Help Center Sign In Submit a Preprint Cite Share Download PDF Case Report Kawasaki disease with shock as the primary manifestation: How to distinguish from toxic shock syndrome？ A Case Report and Literature Review Weijuan Wang, Huixia Wang, Huijiao Wang, Jun Cheng This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-3967125/v1 This work is licensed under a CC BY 4.0 License Status: Posted Version 1 posted You are reading this latest preprint version Abstract Background Kawasaki disease (KD) is a vasculitis syndrome of small to medium-sized arteries that has typical clinical characteristics such as fever, rash, cervical lymphadenopathy, conjunctivitis, and mucosal changes. Cardiac manifestations, including coronary artery aneurysms, myocarditis, myocardial infarction, and sudden cardiac death, are the most serious complications observed in KD. On rare occasions, it may accompanied with reduced organ perfusion due to systolic hypotension, a condition known as Kawasaki disease shock syndrome (KDSS). KDSS is a serious complication that can be presented to the emergency department(ED) as an initial feature when typical clinical symptoms of KD have not be detected. Case Presentation We report the case of a twelve-year-old boy admitted with prolonged fever, bilateral non-purulent conjunctivitis, and shock symptoms such as hypotension and tachycardia. Laboratory findings showed elevated inflammatory markers, hypoalbuminemia, and sterile pyuria. He was initially treated with intravenous cefotaxime and vancomycin considering the possible diagnosis of toxic shock syndrome(TSS), while the treatment was not effective. Subsequent Chest computerized tomography(CT) and ultrasound identified pulmonary consolidation and polyserous effusion. Echocardiography revealed mild biatrial dilatation and mild valvular regurgitation with preserved left ventricular function. After a multidisciplinary consultation, a diagnosis of KDSS was made. To prevent coronary artery lesions and other severe complications, the patient immediately received immunoglobulin, corticoid, and acetylsalicylic acid. Soon afterwards, he showed significant improvement, with resolution of fever, polyserous effusions, and hypotension. Follow-up echocardiography revealed normal results. Conclusions This case indicated that elder children with KD are even more likely to develop KDSS. Clinicians should maintain a high index of suspicion for KD and consider pulmonary involvement and polyserous effusions as potential complications. For children with KD, any symptoms pointing to infection should be carefully considered. When there is no etiologic evidence, antibiotics should be used with caution. Our case also highlights the importance of considering KDSS as a differential diagnosis in children presenting with prolonged fever and shock. Early recognition, timely treatment, and close monitoring are key to preventing severe complications and ensuring favorable outcomes in patients with KDSS. kawasaki disease Kawasaki disease shock syndrome polyserous effusion pulmonary consolidation toxic shock syndrome Figures Figure 1 Figure 2 Figure 3 Background Kawasaki disease (KD), also known as mucocutaneous lymph node syndrome, is an acute rheumatological illness mostly affecting children between six months and five years of age ( 1 ). But less commonly, it can affect children of ages beyond this range( 2 ). The diagnosis of KD involves identifying the presence of a persistent febrile state persisting for a minimum duration of five days, concomitant with the presence of no less than four characteristic manifestations from the following diagnostic criteria: (I) non-purulent conjunctivitis bilaterally exhibiting sparing of the limbal region, (II) oropharyngeal mucosal changes encompassing erythematous fissured lips, a tongue displaying a strawberry-like appearance, or diffuse erythema within the oropharynx, (III) a generalized rash of indeterminate nature, (IV) edema and erythema affecting the extremities, and (V) unilateral cervical lymphadenopathy( 3 , 4 ). However, certain atypical features are not included in the classical criteria, which can be present in a small subset of children with KD, making early detection of KD difficult. Kawasaki disease shock syndrome(KDSS) was first described by Kanegaye et al. in 2009, which is characterized by the simultaneous presence of KD symptoms and features of shock such as hypotension, increased capillary refill time(CRT), and increased pulse rate( 3 ). The specific etiology of profound hypotension in KDSS is still not fully understood, but it is hypothesized to be related to ongoing vasculitis, leakage of capillaries, impaired myocardial function, and dysregulation of cytokines at a systemic level. For children exhibit signs of shock at an early stage of KD before typical clinical symptoms presented, the diagnosis and treatment will be challenging. While KDSS can affect multiple organs, polyserous effusions are typically rare, with no reported case published in the literature as far as we know. There are only a few reported cases describing KDSS complicated by the presence of bilateral pleural effusion ( 5 , 6 ). Case Presentation Presenting Concerns A twelve-year-old boy was brought to our hospital with complaint of fever accompanied by headaches that had persistent for four days. The boy had been in good health until four days ago when he developed a fever which was, according to his parents, high-grade and almost continuous with only mildly relieved by paracetamol. On second day of illness, he developed generalized maculopapular rashes on the body. He was taken to a local hospital for treatment with these complaints. Laboratory testing indicated leukocytosis with increased neutrophils. Thus he was treated with antibiotics (erythromycin for one day and azithromycin for two days). The antibiotics and antipyretics given in the local hospital could not adequately control his fever, and his condition progressively deteriorated. Therefore, he was referred to our center on day 4 of the course. Clinical Findings The child was presented to our hospital in the emergency department (ED), where an examination revealed that the child exhibited signs of shock, including a prolonged CRT of more than four seconds, weak peripheral pulses, hypotension, and tachycardia. The blood pressure, temperature, oxygen saturation, and pulse rate during the initial assessment of the patient at the ED were 87/56 mmHg, 38.5 degree centigrade, 97%, and 128 beats per minute, respectively. Abdomen examination revealed slight tenderness in the periumbilical area, and was otherwise unremarkable with no hepatosplenomegaly. To address the condition, a single bolus of fluid (20 ml/kg) were administered rapidly, then blood investigations including blood cultures were conducted. Subsequently, the child was transferred to the pediatric intensive care unit (PICU) for meticulous surveillance and monitoring. Table 1 presents the noteworthy results of the comprehensive blood tests conducted, providing valuable insights into the patient's condition. Table 1 Some laboratory parameters during hospitalization Blood investigation Day 1 Day2 Day3 Day4 Day5 Day7 Day10 CRP[mg/dl] 83.80 87.89 81.17 34.34 18.00 8.55 <6 hsTnT[ng/ml] 4.4 BNP[pg/ml] 288.40 927.50 879.00 784.50 160.50 - 19.40 Hemoglobin (g/dl) 10.7 10.5 10.6 10.9 11.4 12.6 13.0 White blood cells (×10 9 ) 15.33 15.62 10.41 8.84 9.28 12.16 5.82 Neutrophil (%) 81.8 75.6 74.3 57.7 39.9 40.1 36.9 Platelet (×10 9 ) 218 190 242 301 316 361 326 Procalcitonin (ng/ml) 0.49 0.43 0.22 0.10 0.07 - - Albumin (g/L) 27.28 27.56 30.61 31.63 34.19 35.76 39.87 ESR (MM/HR) 13 15 10 Diagnostic assessment and therapeutic intervention On further examination, he was found to have bilateral non-purulent conjunctivitis, along with edematous limbs. The initial echocardiography did not show any diastolic dysfunction, and the systolic function was normal too, with an ejection fraction of 60%. The laboratory results indicated leukocytosis with increased neutrophils and positive C-reactive peptide(CRP), while erythrocyte sedimentation rate (ESR) was normal. Since the inflammatory markers were very high, meanwhile, the child presented with shock and maculopapular rashes, the preliminary diagnosis of toxic shock syndrome (TSS) was considered. Thus, we initiated anti-infection treatment with intravenous cefotaxime and vancomycin. Continuous infusion of norepinephrine was also adopted because of persistent hemodynamic instability and hypotension. Corticoid therapy was initiated because of capillary leak and shock. Figure 1 shows clinical course in the acute phase of KDSS. Initial work-up showed hypoalbuminemia with a decreased albumin level of 27.28 g/l. To improve this, a 20% albumin transfusion was administered at a dosage of one gram per kilogram of the child's weight in two separate days. Additionally, the initial chest X-ray revealed bronchopneumonia. Increased leukocytes(36.46/HFP) in urine sediment was also found. There was a significant elevation of brain natriuretic peptide (BNP). No sign of other organ dysfunction was found. On the second day of admission, the child continued to experience a persistent high-spiking fever. And the CRP level elevated even higher in spite of receiving powerful intravenous antibiotic treatment. Chest computerized tomography (CT) showed lung exudation and consolidation (Fig. 2 A,B). An echocardiography was repeated that revealed mild biatrial dilatation and mild valvular regurgitation with preserved left ventricular function. Furthermore, an ultrasound examination of the abdomen and chest was conducted, which identified moderate amount of bilateral pleural effusion and mild ascites, as well as mild free fluid in the pelvic region (Fig. 3 A,B,C,D). About ten ml of hydrothorax was collected and analyzed, indicating transudate. The results of EB virus nucleic acid test, SARS-Cov-2 nucleic acid test, sputum culture, hydrothorax culture, and blood culture were all negative. A multidisciplinary consultation was held. According to the clinical manifestation, features, and laboratory examination results (fever lasting for 5 days, bilateral non-purulent conjunctivitis, edematous limbs, elevated blood leukocyte count and CRP, sterile pyuria, and evidence of hypoperfusion), the patient was diagnosed with KDSS on the basis of incomplete Kawasaki disease. And the lung consolidation was determined to be a manifestation of KDSS in the lung, rather than TSS complicated by a pulmonary bacterial infection. To address this condition, intravenous immunoglobulin (IVIG) was administered at a dosage of 30 grams a day for two days. Additionally, acetylsalicylic acid (3 mg/kg/day) was started to prevent thrombosis and for anti-inflammation. Careful monitoring of the fever was implemented as part of the management plan. The patient experienced a significant improvement in the condition, with the fever resolved rapidly, and hemodynamics stabilized which enabled norepinephrine withdrawal after a 2-days usage. On the fifth day of admission, the diagnosis was reconfirmed by the observation of desquamation (skin peeling) on the hands and feet. 8 days after admission, ultrasound examination of the abdomen showed a gradual resolution of the peritoneal effusion (Fig. 3 E,F). Soon afterwards, chest CT also showed a significant absorption of inflammation in the lung (Fig. 2 C,D), indicating a positive response to treatment. Antibiotic therapy was stopped after the blood culture result returned negative. 11 days after admission, he was discharged from the hospital in good condition. Follow-up and outcomes To monitor the possibility of cardiac complications associated with KD, the patient was scheduled for follow-up visits at two weeks and six weeks. Fortunately, echocardiography performed during these visits revealed normal results. As a result, the use of acetylsalicylic acid was discontinued after six weeks since the echocardiography findings remained normal. In summary, the patient's condition improved dramatically, with the resolution of fever and shock. Follow-up echocardiography revealed normal results, leading to the cessation of acetylsalicylic acid medication. Discussion KD, which was first described in 1967 by Dr. Tomisaku Kawasaki, is diagnosed frequently in the Asian pediatric populations. Cardiac manifestations, including coronary artery aneurysms, myocarditis, myocardial infarction, and sudden cardiac death, are the most serious complications observed in KD. However, KD can also present in the form of KDSS. Since KD is rarely seen in anyone over 9 years of age( 7 ), after the peak age of onset, it is not often considered and is probably underdiagnosed to some degree. Interestingly, age > 10 years has been reported as one of the risk factors for progression to KDSS( 8 ). It is crucial to be cognizant of the potential occurrence of KDSS, since prompt recognition and management are necessary to ensure optimal patient outcomes ( 9 ). Although the specific etiology of profound hypotension in KDSS is still not fully understood, there are hypotheses supporting vasculitis with capillary leakage, myocardial function defect, and dysregulation of cytokines at a systemic level, resulting in cardiogenic and/or distributive shock ( 3 , 10 ). In our case, the boy had severe vascular leakage and hypoalbuminemia, with pleural effusions and ascites. In our opinion, the shock was probably due to a significant inflammatory state and vascular leakage, as no sign of remarkable cardiac dysfunction on echocardiography showed. Natterer et al also described three cases of KDSS present with normal myocardial function( 5 ). However, the significant elevation of BNP still suggested some myocardial inflammation. Undeniably, myocardial dysfunction in our case may have been underestimated own to timely application of vasoactive agonists, and subsequent appropriate management of KDSS. Our case illustrates that, in the pediatric population, when children with shock or hypotension require admission to the PICU and fail to respond to antibiotic therapy, it is crucial to consider the possibility of KDSS, even if the diagnostic criteria for KD was not initially fulfilled. In addition, it is important to adopt a comprehensive diagnostic approach that beyond traditional criteria. Timely recognition and appropriate management are crucial to optimize patient outcomes in these intractable clinical scenarios ( 10 – 13 ). In our case study, a twelve-year-old boy exhibited persistent fever, bilateral non-purulent conjunctivitis, edematous limbs, unresponsive shock, low levels of albumin, significantly elevated levels of CRP. Despite the presence of these clinical indicators, the diagnosis of KDSS was not definitively confirmed until a subsequent phase of the illness. The delayed diagnosis is due to the considerable overlap in clinical features between TSS and KDSS. For children with KD, multiple systems including the respiratory system can be affected( 14 ). Common pulmonary manifestations mainly include bronchopneumonia, hydropneumothorax, and pleural effusion. A few studies have reported that KD may appear secondary to lung consolidation, which often occurs due to Streptococcus, Staphylococcus, Mycoplasma, EB virus, coronavirus, or parvovirus infection( 15 ). Thus, in the current case, it was essential to identify whether lung consolidation appeared secondary to KDSS or TSS. Considering that, the blood cultures were consistently negative, the transudate did not contain bacterial infection, moreover, lung consolidation appeared during KDSS progression. Thus, pathological changes observed in the lung were considered to be related to KD. In addition, cardiac valve regurgitation, which was seen in our case might be another discriminating factor between KDSS and TSS, since previous studies have found that echocardiographic abnormalities including cardiac valve regurgitation were considerably more common among patients with KDSS( 16 – 17 ). In terms of treatment, despite fluid resuscitation and maintenance of hemodynamic stability are similar, the course of disease evolution between KDSS or TSS are different. Generally speaking, multiple organ dysfunction in KDSS was less severe and mostly transient. Meanwhile, shock associated with KD can be controlled relatively easily by vasoactive agent. On the contrary, once the blood pressure drops in septic shock or TSS, the condition is often very critical, the incidence of multiple organ failure is high, and the mortality is significantly increased. Specific antibiotics and glucocorticoid are used to treat TSS, which still leads to high mortality rates (up to 44%) after treatment( 18 ). For KDSS, IVIG should be administered as soon as possible to achieve a good prognosis, while the use of antibiotics is not required. When unusual pulmonary changes similar to bacterial infection appear, KDSS and TSS should be identified based on monism. For our case, according to the principles of KD therapy, consolidation was absorbed rapidly, and the course of treatment was much shorter than the cure course of consolidation in the bilateral lungs caused by a bacterial infection, which further confirmed the diagnosis of KD-related pulmonary changes. Our case highlights that children with KD may present with a toxic shock-like illness. And it is essential to promptly recognize and remain vigilant for the manifestation of KDSS. Early detection enables timely initiation of treatment, which can have a profound impact on patient outcomes. To the best of our knowledge, this represents the first documented case of KDSS with polyserous effusion reported globally. This underscores the rarity of such occurrences and emphasizes the importance of further research and continued awareness of this complex clinical entity. Conclusions Clinicians should maintain a high index of suspicion for KD and consider pulmonary involvement and polyserous effusions as potential complications. Elder children suffering from KD are even more likely to develop KDSS. This case also highlights the importance of considering KDSS as a differential diagnosis in children presenting with prolonged fever and shock. Early recognition and timely treatment are key to preventing severe complications and ensuring favorable outcomes in patients with KDSS. Further research and awareness are needed to better understand the underlying mechanisms of KD, its varied presentations, and the optimal management strategies. By expanding our knowledge and improving diagnostic capabilities, we can enhance the care provided to children affected by this challenging disease. Abbreviations KD Kawasaki disease KDSS Kawasaki disease shock syndrome ED emergency department TSS toxic shock syndrome CT computerized tomography CRT capillary refill time PICU pediatric intensive care unit CRP C-reactive peptide ESR erythrocyte sedimentation rate BNP brain natriuretic peptide IVIG intravenous immunoglobulin Declarations Acknowledgements Not applicable. Author contributions W.W. wrote the manuscript and conducted data collection. H.X.W. provided supervision, critical review, and manuscript revision. J.C. played a crucial role in case selection. H.J.W. was involved in patient management, clinical assessment, and interpretation of data. All authors contributed to the article and approved the final manuscript. Funding This study was conducted without any funding or involvement from external sources. Data availability All data generated or analyzed during this study are included in this published article. Ethics approval and consent to participate This case report, obtained informed consent from the legal guardians of the participant. Ethical approval of the study was obtained from the Ethics Committee of Northwestern Women's and Children's Hospital. Consent for publication Legal guardians of the patient provided written informed consent for the publication of this case report and the material it includes. A copy of the consent form is available for review by the Editor of this journal. Competing interests The authors declare no competing interests. References Nugud AA, Nugud A, Wafadari D, Abuhammour W. Kawasaki shock syndrome in an Arab female: case report of a rare manifestation and review of literature. BMC Pediatr. 2019;19:295. 10.1186/s12887-019-1662-9 . Rowley AH, Shulman ST. Pathogenesis and management of Kawasaki disease. Expert Rev Anti Infect Ther. 2010;8:197–203. 10.1586/eri.09.109 . Kanegaye JT, Wilder MS, Molkara D, Frazer JR, Pancheri J, Tremoulet AH, et al. Recognition of a Kawasaki disease shock syndrome. Pediatrics. 2009;123:783–9. 10.1542/peds.2008-1871 . Mandal S, Pande A, Mandal D, Sarkar A, Kahali D, Panja M. Various coronary artery complications of Kawasaki disease: series of 5 cases and review of literature. J Cardiovasc Dis Res. 2012;3:231–5. 10.4103/0975-3583.98900 . Natterer J, Perez MH, Di Bernardo S. Capillary leak leading to shock in Kawasaki disease without myocardial dysfunction. Cardiol Young. 2012;22:349–52. 10.1017/S1047951111001314 . Alghamdi LSA, Alzahrani AYB, Alghamdi FA, ALghamdi SJ. A Challenging Diagnosis of Kawasaki Disease Shock Syndrome Complicated by Bilateral Pleural Effusion: A Case Report and Literature Review. Cureus. 2023;15(11):e49671. 10.7759/cureus.49671 . Fukazawa R, Kobayashi T, Mikami M, Saji T, Hamaoka K, Kato H, et al. Nationwide survey of patients with giant coronary aneurysm secondary to Kawasaki disease 1999–2010 in Japan. Circ J. 2017;82:239–46. 10.1253/circj.CJ-17-0433 . Taddio A, Rossi ED, Monasta L, Pastore S, Tommasini A, Lepore L, et al. Describing Kawasaki shock syndrome: results from a retrospective study and literature review. Clin Rheumatol. 2016;36:223–8. 10.1007/s10067-016-3316-8 . McCrindle BW, Rowley AH, Newburger JW, Burns JC, Bolger AF, Gewitz M, et al. Diagnosis, treatment, and long-term management of Kawasaki disease: a scientific statement for health professionals from the American Heart Association. Circulation. 2017;135:927–99. 10.1161/CIR.0000000000000484 . Rife E, Gedalia A. Kawasaki disease: an update. Curr Rheumatol Rep. 2020;22:75. 10.1007/s11926-020-00941-4 . Ayusawa M, Sonobe T, Uemura S, Ogawa S, Nakamura Y, Kiyosawa N, et al. Revision of diagnostic guidelines for Kawasaki disease (the 5th revised edition). Pediatr Int. 2005;47:232–4. 10.1111/j.1442-200x.2005.02033.x . Son MBF, Newburger JW. Kawasaki disease. Pediatr Rev. 2018;39:78–90. 10.1542/pir.2016-0182 . Burgner D, Harnden A. Kawasaki disease: what is the epidemiology telling us about the etiology? Int J Infect Dis. 2005;9:185–94. 10.1016/j.ijid.2005.03.002 . Lee MN, Cha JH, Ahn HM, Yoo JH, Kim HS, Sohn S, et al. Mycoplasma pneumoniae infection in patients with Kawasaki disease. Korean J Pediatr. 2011;54:123–7. 10.3345/kjp.2011.54.3.123 . Singh S, Gupta A, Jindal AK, Gupta A, Suri D, Rawat A, et al. Pulmonary presentation of Kawasaki disease-A diagnostic challenge. Pediatr Pulmonol. 2018;53:103–7. 10.1002/ppul.23885 . Power A, Runeckles K, Manlhiot C, Dragulescu A, Guerguerian AM, McCrindle BW. Kawasaki Disease Shock Syndrome Versus Septic Shock: Early Differentiating Features Despite Overlapping Clinical Profiles. J Pediatr-US. 2020;231:162–7. 10.1016/j.jpeds.2020.12.002 . Lin Y, Cheng MC, Lo MH, Chien SJ. Early differentiation of Kawasaki disease shock syndrome and toxic shock syndrome in a pediatric intensive care unit. Pediatr Infect Dis J. 2015;34(11):1163–7. 10.1097/INF.0000000000000852 . Lamagni TL, Darenberg J, Luca-Harari B, Siljander T, Efstratiou A, Henriques-Normark B, et al. Epidemiology of severe Streptococcus pyogenes disease in Europe. J Clin Microbiol. 2008;46:2359–67. 10.1128/JCM.00422-08 . Additional Declarations No competing interests reported. Cite Share Download PDF Status: Posted Version 1 posted You are reading this latest preprint version Research Square lets you share your work early, gain feedback from the community, and start making changes to your manuscript prior to peer review in a journal. As a division of Research Square Company, we’re committed to making research communication faster, fairer, and more useful. We do this by developing innovative software and high quality services for the global research community. Our growing team is made up of researchers and industry professionals working together to solve the most critical problems facing scientific publishing. Also discoverable on Platform About Our Team In Review Editorial Policies Advisory Board Help Center Resources Author Services Accessibility API Access RSS feed Manage Cookie Preferences © Research Square 2026 | ISSN 2693-5015 (online) Privacy Policy Terms of Service Do Not Sell My Personal Information {"props":{"pageProps":{"initialData":{"identity":"rs-3967125","acceptedTermsAndConditions":true,"allowDirectSubmit":true,"archivedVersions":[],"articleType":"Case Report","associatedPublications":[],"authors":[{"id":275668344,"identity":"a0661b3e-a2ce-4d4e-89e5-febf85a17805","order_by":0,"name":"Weijuan Wang","email":"","orcid":"","institution":"Northwest Women's and Children's Hospital","correspondingAuthor":false,"submittingAuthor":false,"prefix":"","firstName":"Weijuan","middleName":"","lastName":"Wang","suffix":""},{"id":275668345,"identity":"9f61e1fb-b683-422e-b6f0-65ec42349378","order_by":1,"name":"Huixia Wang","email":"","orcid":"","institution":"Northwest Women's and Children's Hospital","correspondingAuthor":false,"submittingAuthor":false,"prefix":"","firstName":"Huixia","middleName":"","lastName":"Wang","suffix":""},{"id":275668346,"identity":"293e7acf-ce7e-4562-bc63-85d8b2db7f6f","order_by":2,"name":"Huijiao Wang","email":"","orcid":"","institution":"Northwest Women's and Children's Hospital","correspondingAuthor":false,"submittingAuthor":false,"prefix":"","firstName":"Huijiao","middleName":"","lastName":"Wang","suffix":""},{"id":275668347,"identity":"dea909b8-6619-436b-90ea-c5d0f1bde4ca","order_by":3,"name":"Jun Cheng","email":"data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAAZAAAAAyAQMAAABI0h/eAAAABlBMVEX///8AAABVwtN+AAAACXBIWXMAAA7EAAAOxAGVKw4bAAAAyUlEQVRIiWNgGAWjYBACxvmPDxz4UGEjx8/eQKQW5oa0xIMzzqQZS/YcIFILe0OO8WHelsOJG24kEKmFt+FYwmHehsOJM2c+3niDocYmmqAWycbmAwfn7kg37pdOK7ZgOJaW20BIi2EzW8KBt2esZWfOzjGTYGw4TFiL/TEegwO8bcyMG26eIVILYw+PwUHeNmfFDTd4iNUygy0BGshAvyQQ4xfGGcyHP0Ci8vDGGx9qbAhrQQYGEgmkKIdoIVXHKBgFo2AUjAwAAJGGSXbADdTEAAAAAElFTkSuQmCC","orcid":"","institution":"Northwest Women's and Children's Hospital","correspondingAuthor":true,"submittingAuthor":false,"prefix":"","firstName":"Jun","middleName":"","lastName":"Cheng","suffix":""}],"badges":[],"createdAt":"2024-02-18 14:04:36","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-3967125/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-3967125/v1","draftVersion":[],"editorialEvents":[],"editorialNote":"","failedWorkflow":false,"files":[{"id":52032104,"identity":"6b135d6a-f8e8-4c9d-b3d0-aa5bd3ea1f2a","added_by":"auto","created_at":"2024-03-05 16:35:58","extension":"png","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":226361,"visible":true,"origin":"","legend":"\u003cp\u003eClinical course in the acute phase of KDSS. IABP, intra-aortic balloon pumping; IVIG, intravenous immunoglobulin; SBP, systolic blood pressure; DBP, diastolic blood pressure; HR, heart rate.\u003c/p\u003e","description":"","filename":"Figure1.png","url":"https://assets-eu.researchsquare.com/files/rs-3967125/v1/b096cec07b20572c73466a95.png"},{"id":52032103,"identity":"ef328efd-f254-48bb-8d9d-e7e088f44d74","added_by":"auto","created_at":"2024-03-05 16:35:58","extension":"jpg","order_by":2,"title":"Figure 2","display":"","copyAsset":false,"role":"figure","size":128710,"visible":true,"origin":"","legend":"\u003cp\u003eLung consolidation and pleural effusion on CT. \u003cstrong\u003e(A,B)\u003c/strong\u003e Chest CT during the acute phase of disease revealed lung consolidation and pleural effusion. \u003cstrong\u003e(C,D)\u003c/strong\u003e Lung consolidation and pleural effusion gradually absorbed after treatment\u003c/p\u003e","description":"","filename":"Figure2.jpg","url":"https://assets-eu.researchsquare.com/files/rs-3967125/v1/e59198fec8b19089493acf87.jpg"},{"id":52034037,"identity":"126f133b-2aa9-4cc1-af50-dc4e81ae8801","added_by":"auto","created_at":"2024-03-05 16:43:58","extension":"jpg","order_by":3,"title":"Figure 3","display":"","copyAsset":false,"role":"figure","size":271000,"visible":true,"origin":"","legend":"\u003cp\u003e\u003cstrong\u003e(A,B,C,D)\u003c/strong\u003eUltrasound during the acute phase of disease revealed bilateral pleural effusion, ascites, and pelvic effusion. \u003cstrong\u003e(E,F) \u003c/strong\u003eUltrasound showed a gradual resolution of the peritoneal effusion in response to therapy.\u003c/p\u003e","description":"","filename":"Figure3.jpg","url":"https://assets-eu.researchsquare.com/files/rs-3967125/v1/52ab681ed096e084fd1ab574.jpg"},{"id":55344912,"identity":"5dbb6f5b-86eb-4054-af2c-da3cf59dde4b","added_by":"auto","created_at":"2024-04-26 03:38:29","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":718753,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-3967125/v1/f557a132-5638-48ea-8690-5c9ee996bdf2.pdf"}],"financialInterests":"No competing interests reported.","formattedTitle":"Kawasaki disease with shock as the primary manifestation: How to distinguish from toxic shock syndrome？ A Case Report and Literature Review","fulltext":[{"header":"Background","content":"\u003cp\u003eKawasaki disease (KD), also known as mucocutaneous lymph node syndrome, is an acute rheumatological illness mostly affecting children between six months and five years of age (\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e). But less commonly, it can affect children of ages beyond this range(\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e). The diagnosis of KD involves identifying the presence of a persistent febrile state persisting for a minimum duration of five days, concomitant with the presence of no less than four characteristic manifestations from the following diagnostic criteria: (I) non-purulent conjunctivitis bilaterally exhibiting sparing of the limbal region, (II) oropharyngeal mucosal changes encompassing erythematous fissured lips, a tongue displaying a strawberry-like appearance, or diffuse erythema within the oropharynx, (III) a generalized rash of indeterminate nature, (IV) edema and erythema affecting the extremities, and (V) unilateral cervical lymphadenopathy(\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e, \u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e). However, certain atypical features are not included in the classical criteria, which can be present in a small subset of children with KD, making early detection of KD difficult. Kawasaki disease shock syndrome(KDSS) was first described by Kanegaye et al. in 2009, which is characterized by the simultaneous presence of KD symptoms and features of shock such as hypotension, increased capillary refill time(CRT), and increased pulse rate(\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e). The specific etiology of profound hypotension in KDSS is still not fully understood, but it is hypothesized to be related to ongoing vasculitis, leakage of capillaries, impaired myocardial function, and dysregulation of cytokines at a systemic level. For children exhibit signs of shock at an early stage of KD before typical clinical symptoms presented, the diagnosis and treatment will be challenging. While KDSS can affect multiple organs, polyserous effusions are typically rare, with no reported case published in the literature as far as we know. There are only a few reported cases describing KDSS complicated by the presence of bilateral pleural effusion (\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e, \u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e).\u003c/p\u003e"},{"header":"Case Presentation","content":"\u003cdiv id=\"Sec3\" class=\"Section2\"\u003e \u003ch2\u003ePresenting Concerns\u003c/h2\u003e \u003cp\u003eA twelve-year-old boy was brought to our hospital with complaint of fever accompanied by headaches that had persistent for four days. The boy had been in good health until four days ago when he developed a fever which was, according to his parents, high-grade and almost continuous with only mildly relieved by paracetamol. On second day of illness, he developed generalized maculopapular rashes on the body. He was taken to a local hospital for treatment with these complaints. Laboratory testing indicated leukocytosis with increased neutrophils. Thus he was treated with antibiotics (erythromycin for one day and azithromycin for two days). The antibiotics and antipyretics given in the local hospital could not adequately control his fever, and his condition progressively deteriorated. Therefore, he was referred to our center on day 4 of the course.\u003c/p\u003e \u003c/div\u003e \u003cdiv id=\"Sec4\" class=\"Section2\"\u003e \u003ch2\u003eClinical Findings\u003c/h2\u003e \u003cp\u003eThe child was presented to our hospital in the emergency department (ED), where an examination revealed that the child exhibited signs of shock, including a prolonged CRT of more than four seconds, weak peripheral pulses, hypotension, and tachycardia. The blood pressure, temperature, oxygen saturation, and pulse rate during the initial assessment of the patient at the ED were 87/56 mmHg, 38.5 degree centigrade, 97%, and 128 beats per minute, respectively. Abdomen examination revealed slight tenderness in the periumbilical area, and was otherwise unremarkable with no hepatosplenomegaly. To address the condition, a single bolus of fluid (20 ml/kg) were administered rapidly, then blood investigations including blood cultures were conducted. Subsequently, the child was transferred to the pediatric intensive care unit (PICU) for meticulous surveillance and monitoring. Table\u0026nbsp;\u003cspan refid=\"Tab1\" class=\"InternalRef\"\u003e1\u003c/span\u003e presents the noteworthy results of the comprehensive blood tests conducted, providing valuable insights into the patient's condition.\u003c/p\u003e \u003cp\u003e \u003cdiv class=\"gridtable\"\u003e\u003ctable float=\"Yes\" id=\"Tab1\" border=\"1\"\u003e \u003ccaption language=\"En\"\u003e \u003cdiv class=\"CaptionNumber\"\u003eTable 1\u003c/div\u003e \u003cdiv class=\"CaptionContent\"\u003e \u003cp\u003eSome laboratory parameters during hospitalization\u003c/p\u003e \u003c/div\u003e \u003c/caption\u003e \u003ccolgroup cols=\"8\"\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c1\" colnum=\"1\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c2\" colnum=\"2\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c3\" colnum=\"3\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c4\" colnum=\"4\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c5\" colnum=\"5\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c6\" colnum=\"6\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c7\" colnum=\"7\"\u003e\u003c/div\u003e \u003cdiv align=\"left\" class=\"colspec\" colname=\"c8\" colnum=\"8\"\u003e\u003c/div\u003e \u003cthead\u003e \u003ctr\u003e \u003cth align=\"left\" colname=\"c1\"\u003e \u003cp\u003eBlood investigation\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c2\"\u003e \u003cp\u003eDay 1\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c3\"\u003e \u003cp\u003eDay2\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c4\"\u003e \u003cp\u003eDay3\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c5\"\u003e \u003cp\u003eDay4\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c6\"\u003e \u003cp\u003eDay5\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c7\"\u003e \u003cp\u003eDay7\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c8\"\u003e \u003cp\u003eDay10\u003c/p\u003e \u003c/th\u003e \u003c/tr\u003e \u003c/thead\u003e \u003ctbody\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eCRP[mg/dl]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e83.80\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e87.89\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e81.17\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e34.34\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e18.00\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e8.55\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c8\"\u003e \u003cp\u003e\u0026lt;6\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003ehsTnT[ng/ml]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e4.4\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c8\"\u003e\u0026nbsp;\u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eBNP[pg/ml]\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e288.40\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e927.50\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e879.00\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e784.50\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e160.50\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e-\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c8\"\u003e \u003cp\u003e19.40\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eHemoglobin\u003c/p\u003e \u003cp\u003e(g/dl)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e10.7\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e10.5\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e10.6\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e10.9\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e11.4\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e12.6\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c8\"\u003e \u003cp\u003e13.0\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eWhite blood cells (\u0026times;10\u003csup\u003e9\u003c/sup\u003e)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e15.33\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e15.62\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e10.41\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e8.84\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e9.28\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e12.16\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c8\"\u003e \u003cp\u003e5.82\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eNeutrophil (%)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e81.8\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e75.6\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e74.3\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e57.7\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e39.9\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e40.1\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c8\"\u003e \u003cp\u003e36.9\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003ePlatelet (\u0026times;10\u003csup\u003e9\u003c/sup\u003e)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e218\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e190\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e242\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e301\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e316\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e361\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c8\"\u003e \u003cp\u003e326\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eProcalcitonin\u003c/p\u003e \u003cp\u003e(ng/ml)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e0.49\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e0.43\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e0.22\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e0.10\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e0.07\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e-\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c8\"\u003e \u003cp\u003e-\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eAlbumin (g/L)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e27.28\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e27.56\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003e30.61\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003e31.63\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e34.19\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e \u003cp\u003e35.76\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c8\"\u003e \u003cp\u003e39.87\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003eESR (MM/HR)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003e13\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003e15\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c7\"\u003e\u0026nbsp;\u003c/td\u003e \u003ctd align=\"left\" colname=\"c8\"\u003e \u003cp\u003e10\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003c/tbody\u003e \u003c/colgroup\u003e \u003c/table\u003e\u003c/div\u003e \u003c/p\u003e \u003c/div\u003e \u003cdiv id=\"Sec5\" class=\"Section2\"\u003e \u003ch2\u003eDiagnostic assessment and therapeutic intervention\u003c/h2\u003e \u003cp\u003eOn further examination, he was found to have bilateral non-purulent conjunctivitis, along with edematous limbs. The initial echocardiography did not show any diastolic dysfunction, and the systolic function was normal too, with an ejection fraction of 60%. The laboratory results indicated leukocytosis with increased neutrophils and positive C-reactive peptide(CRP), while erythrocyte sedimentation rate (ESR) was normal. Since the inflammatory markers were very high, meanwhile, the child presented with shock and maculopapular rashes, the preliminary diagnosis of toxic shock syndrome (TSS) was considered. Thus, we initiated anti-infection treatment with intravenous cefotaxime and vancomycin. Continuous infusion of norepinephrine was also adopted because of persistent hemodynamic instability and hypotension. Corticoid therapy was initiated because of capillary leak and shock. Figure\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003e shows clinical course in the acute phase of KDSS. Initial work-up showed hypoalbuminemia with a decreased albumin level of 27.28 g/l. To improve this, a 20% albumin transfusion was administered at a dosage of one gram per kilogram of the child's weight in two separate days. Additionally, the initial chest X-ray revealed bronchopneumonia. Increased leukocytes(36.46/HFP) in urine sediment was also found. There was a significant elevation of brain natriuretic peptide (BNP). No sign of other organ dysfunction was found.\u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003eOn the second day of admission, the child continued to experience a persistent high-spiking fever. And the CRP level elevated even higher in spite of receiving powerful intravenous antibiotic treatment. Chest computerized tomography (CT) showed lung exudation and consolidation (Fig.\u0026nbsp;\u003cspan refid=\"Fig2\" class=\"InternalRef\"\u003e2\u003c/span\u003eA,B). An echocardiography was repeated that revealed mild biatrial dilatation and mild valvular regurgitation with preserved left ventricular function. Furthermore, an ultrasound examination of the abdomen and chest was conducted, which identified moderate amount of bilateral pleural effusion and mild ascites, as well as mild free fluid in the pelvic region (Fig.\u0026nbsp;\u003cspan refid=\"Fig3\" class=\"InternalRef\"\u003e3\u003c/span\u003eA,B,C,D). About ten ml of hydrothorax was collected and analyzed, indicating transudate. The results of EB virus nucleic acid test, SARS-Cov-2 nucleic acid test, sputum culture, hydrothorax culture, and blood culture were all negative. A multidisciplinary consultation was held. According to the clinical manifestation, features, and laboratory examination results (fever lasting for 5 days, bilateral non-purulent conjunctivitis, edematous limbs, elevated blood leukocyte count and CRP, sterile pyuria, and evidence of hypoperfusion), the patient was diagnosed with KDSS on the basis of incomplete Kawasaki disease. And the lung consolidation was determined to be a manifestation of KDSS in the lung, rather than TSS complicated by a pulmonary bacterial infection. To address this condition, intravenous immunoglobulin (IVIG) was administered at a dosage of 30 grams a day for two days. Additionally, acetylsalicylic acid (3 mg/kg/day) was started to prevent thrombosis and for anti-inflammation. Careful monitoring of the fever was implemented as part of the management plan. The patient experienced a significant improvement in the condition, with the fever resolved rapidly, and hemodynamics stabilized which enabled norepinephrine withdrawal after a 2-days usage. On the fifth day of admission, the diagnosis was reconfirmed by the observation of desquamation (skin peeling) on the hands and feet. 8 days after admission, ultrasound examination of the abdomen showed a gradual resolution of the peritoneal effusion (Fig.\u0026nbsp;\u003cspan refid=\"Fig3\" class=\"InternalRef\"\u003e3\u003c/span\u003eE,F). Soon afterwards, chest CT also showed a significant absorption of inflammation in the lung (Fig.\u0026nbsp;\u003cspan refid=\"Fig2\" class=\"InternalRef\"\u003e2\u003c/span\u003eC,D), indicating a positive response to treatment. Antibiotic therapy was stopped after the blood culture result returned negative. 11 days after admission, he was discharged from the hospital in good condition.\u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003c/div\u003e \u003cdiv id=\"Sec6\" class=\"Section2\"\u003e \u003ch2\u003eFollow-up and outcomes\u003c/h2\u003e \u003cp\u003eTo monitor the possibility of cardiac complications associated with KD, the patient was scheduled for follow-up visits at two weeks and six weeks. Fortunately, echocardiography performed during these visits revealed normal results. As a result, the use of acetylsalicylic acid was discontinued after six weeks since the echocardiography findings remained normal.\u003c/p\u003e \u003cp\u003eIn summary, the patient's condition improved dramatically, with the resolution of fever and shock. Follow-up echocardiography revealed normal results, leading to the cessation of acetylsalicylic acid medication.\u003c/p\u003e \u003c/div\u003e"},{"header":"Discussion","content":"\u003cp\u003eKD, which was first described in 1967 by Dr. Tomisaku Kawasaki, is diagnosed frequently in the Asian pediatric populations. Cardiac manifestations, including coronary artery aneurysms, myocarditis, myocardial infarction, and sudden cardiac death, are the most serious complications observed in KD. However, KD can also present in the form of KDSS. Since KD is rarely seen in anyone over 9 years of age(\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e), after the peak age of onset, it is not often considered and is probably underdiagnosed to some degree. Interestingly, age\u0026thinsp;\u0026gt;\u0026thinsp;10 years has been reported as one of the risk factors for progression to KDSS(\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e). It is crucial to be cognizant of the potential occurrence of KDSS, since prompt recognition and management are necessary to ensure optimal patient outcomes (\u003cspan citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e).\u003c/p\u003e \u003cp\u003eAlthough the specific etiology of profound hypotension in KDSS is still not fully understood, there are hypotheses supporting vasculitis with capillary leakage, myocardial function defect, and dysregulation of cytokines at a systemic level, resulting in cardiogenic and/or distributive shock (\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e, \u003cspan citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e). In our case, the boy had severe vascular leakage and hypoalbuminemia, with pleural effusions and ascites. In our opinion, the shock was probably due to a significant inflammatory state and vascular leakage, as no sign of remarkable cardiac dysfunction on echocardiography showed. Natterer et al also described three cases of KDSS present with normal myocardial function(\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e). However, the significant elevation of BNP still suggested some myocardial inflammation. Undeniably, myocardial dysfunction in our case may have been underestimated own to timely application of vasoactive agonists, and subsequent appropriate management of KDSS.\u003c/p\u003e \u003cp\u003eOur case illustrates that, in the pediatric population, when children with shock or hypotension require admission to the PICU and fail to respond to antibiotic therapy, it is crucial to consider the possibility of KDSS, even if the diagnostic criteria for KD was not initially fulfilled. In addition, it is important to adopt a comprehensive diagnostic approach that beyond traditional criteria. Timely recognition and appropriate management are crucial to optimize patient outcomes in these intractable clinical scenarios (\u003cspan additionalcitationids=\"CR11 CR12\" citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR13\" class=\"CitationRef\"\u003e13\u003c/span\u003e).\u003c/p\u003e \u003cp\u003eIn our case study, a twelve-year-old boy exhibited persistent fever, bilateral non-purulent conjunctivitis, edematous limbs, unresponsive shock, low levels of albumin, significantly elevated levels of CRP. Despite the presence of these clinical indicators, the diagnosis of KDSS was not definitively confirmed until a subsequent phase of the illness. The delayed diagnosis is due to the considerable overlap in clinical features between TSS and KDSS.\u003c/p\u003e \u003cp\u003eFor children with KD, multiple systems including the respiratory system can be affected(\u003cspan citationid=\"CR14\" class=\"CitationRef\"\u003e14\u003c/span\u003e). Common pulmonary manifestations mainly include bronchopneumonia, hydropneumothorax, and pleural effusion. A few studies have reported that KD may appear secondary to lung consolidation, which often occurs due to Streptococcus, Staphylococcus, Mycoplasma, EB virus, coronavirus, or parvovirus infection(\u003cspan citationid=\"CR15\" class=\"CitationRef\"\u003e15\u003c/span\u003e). Thus, in the current case, it was essential to identify whether lung consolidation appeared secondary to KDSS or TSS.\u003c/p\u003e \u003cp\u003eConsidering that, the blood cultures were consistently negative, the transudate did not contain bacterial infection, moreover, lung consolidation appeared during KDSS progression. Thus, pathological changes observed in the lung were considered to be related to KD. In addition, cardiac valve regurgitation, which was seen in our case might be another discriminating factor between KDSS and TSS, since previous studies have found that echocardiographic abnormalities including cardiac valve regurgitation were considerably more common among patients with KDSS(\u003cspan citationid=\"CR16\" class=\"CitationRef\"\u003e16\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR17\" class=\"CitationRef\"\u003e17\u003c/span\u003e).\u003c/p\u003e \u003cp\u003eIn terms of treatment, despite fluid resuscitation and maintenance of hemodynamic stability are similar, the course of disease evolution between KDSS or TSS are different. Generally speaking, multiple organ dysfunction in KDSS was less severe and mostly transient. Meanwhile, shock associated with KD can be controlled relatively easily by vasoactive agent. On the contrary, once the blood pressure drops in septic shock or TSS, the condition is often very critical, the incidence of multiple organ failure is high, and the mortality is significantly increased. Specific antibiotics and glucocorticoid are used to treat TSS, which still leads to high mortality rates (up to 44%) after treatment(\u003cspan citationid=\"CR18\" class=\"CitationRef\"\u003e18\u003c/span\u003e). For KDSS, IVIG should be administered as soon as possible to achieve a good prognosis, while the use of antibiotics is not required. When unusual pulmonary changes similar to bacterial infection appear, KDSS and TSS should be identified based on monism. For our case, according to the principles of KD therapy, consolidation was absorbed rapidly, and the course of treatment was much shorter than the cure course of consolidation in the bilateral lungs caused by a bacterial infection, which further confirmed the diagnosis of KD-related pulmonary changes.\u003c/p\u003e \u003cp\u003eOur case highlights that children with KD may present with a toxic shock-like illness. And it is essential to promptly recognize and remain vigilant for the manifestation of KDSS. Early detection enables timely initiation of treatment, which can have a profound impact on patient outcomes. To the best of our knowledge, this represents the first documented case of KDSS with polyserous effusion reported globally. This underscores the rarity of such occurrences and emphasizes the importance of further research and continued awareness of this complex clinical entity.\u003c/p\u003e"},{"header":"Conclusions","content":"\u003cp\u003eClinicians should maintain a high index of suspicion for KD and consider pulmonary involvement and polyserous effusions as potential complications. Elder children suffering from KD are even more likely to develop KDSS. This case also highlights the importance of considering KDSS as a differential diagnosis in children presenting with prolonged fever and shock. Early recognition and timely treatment are key to preventing severe complications and ensuring favorable outcomes in patients with KDSS. Further research and awareness are needed to better understand the underlying mechanisms of KD, its varied presentations, and the optimal management strategies. By expanding our knowledge and improving diagnostic capabilities, we can enhance the care provided to children affected by this challenging disease.\u003c/p\u003e"},{"header":"Abbreviations","content":"\u003cp\u003eKD Kawasaki disease\u003c/p\u003e\n\u003cp\u003eKDSS Kawasaki disease shock syndrome\u003c/p\u003e\n\u003cp\u003eED emergency department\u003c/p\u003e\n\u003cp\u003eTSS toxic shock syndrome\u003c/p\u003e\n\u003cp\u003eCT computerized tomography\u003c/p\u003e\n\u003cp\u003eCRT capillary refill time\u003c/p\u003e\n\u003cp\u003ePICU pediatric intensive care unit\u003c/p\u003e\n\u003cp\u003eCRP C-reactive peptide\u003c/p\u003e\n\u003cp\u003eESR erythrocyte sedimentation rate\u003c/p\u003e\n\u003cp\u003eBNP brain natriuretic peptide\u003c/p\u003e\n\u003cp\u003eIVIG intravenous immunoglobulin\u003c/p\u003e"},{"header":"Declarations","content":"\u003cp\u003e\u003cstrong\u003eAcknowledgements\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eNot applicable.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAuthor contributions\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eW.W. wrote the manuscript and conducted data collection. H.X.W. provided supervision, critical review, and manuscript revision. J.C. played a crucial role in case selection. H.J.W. was involved in patient management, clinical assessment, and interpretation of data. All authors contributed to the article and approved the final manuscript.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eFunding\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThis study was conducted without any funding or involvement from external sources.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eData availability\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eAll data generated or analyzed during this study are included in this published article.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eEthics approval and consent to participate\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThis case report, obtained informed consent from the legal guardians of the participant. Ethical approval of the study was obtained from the Ethics Committee of Northwestern Women\u0026apos;s and Children\u0026apos;s Hospital.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConsent for publication\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eLegal guardians of the patient provided written informed consent for the publication of this case report and the material it includes. A copy of the consent form is available for review by the Editor of this journal.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eCompeting interests\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe authors declare no competing interests.\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\u003cli\u003e\u003cspan\u003eNugud AA, Nugud A, Wafadari D, Abuhammour W. Kawasaki shock syndrome in an Arab female: case report of a rare manifestation and review of literature. BMC Pediatr. 2019;19:295. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.1186/s12887-019-1662-9\u003c/span\u003e\u003cspan address=\"10.1186/s12887-019-1662-9\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eRowley AH, Shulman ST. Pathogenesis and management of Kawasaki disease. Expert Rev Anti Infect Ther. 2010;8:197\u0026ndash;203. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.1586/eri.09.109\u003c/span\u003e\u003cspan address=\"10.1586/eri.09.109\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eKanegaye JT, Wilder MS, Molkara D, Frazer JR, Pancheri J, Tremoulet AH, et al. Recognition of a Kawasaki disease shock syndrome. Pediatrics. 2009;123:783\u0026ndash;9. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.1542/peds.2008-1871\u003c/span\u003e\u003cspan address=\"10.1542/peds.2008-1871\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eMandal S, Pande A, Mandal D, Sarkar A, Kahali D, Panja M. Various coronary artery complications of Kawasaki disease: series of 5 cases and review of literature. J Cardiovasc Dis Res. 2012;3:231\u0026ndash;5. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.4103/0975-3583.98900\u003c/span\u003e\u003cspan address=\"10.4103/0975-3583.98900\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eNatterer J, Perez MH, Di Bernardo S. Capillary leak leading to shock in Kawasaki disease without myocardial dysfunction. Cardiol Young. 2012;22:349\u0026ndash;52. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.1017/S1047951111001314\u003c/span\u003e\u003cspan address=\"10.1017/S1047951111001314\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eAlghamdi LSA, Alzahrani AYB, Alghamdi FA, ALghamdi SJ. A Challenging Diagnosis of Kawasaki Disease Shock Syndrome Complicated by Bilateral Pleural Effusion: A Case Report and Literature Review. Cureus. 2023;15(11):e49671. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.7759/cureus.49671\u003c/span\u003e\u003cspan address=\"10.7759/cureus.49671\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eFukazawa R, Kobayashi T, Mikami M, Saji T, Hamaoka K, Kato H, et al. Nationwide survey of patients with giant coronary aneurysm secondary to Kawasaki disease 1999\u0026ndash;2010 in Japan. Circ J. 2017;82:239\u0026ndash;46. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.1253/circj.CJ-17-0433\u003c/span\u003e\u003cspan address=\"10.1253/circj.CJ-17-0433\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eTaddio A, Rossi ED, Monasta L, Pastore S, Tommasini A, Lepore L, et al. Describing Kawasaki shock syndrome: results from a retrospective study and literature review. Clin Rheumatol. 2016;36:223\u0026ndash;8. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.1007/s10067-016-3316-8\u003c/span\u003e\u003cspan address=\"10.1007/s10067-016-3316-8\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eMcCrindle BW, Rowley AH, Newburger JW, Burns JC, Bolger AF, Gewitz M, et al. Diagnosis, treatment, and long-term management of Kawasaki disease: a scientific statement for health professionals from the American Heart Association. Circulation. 2017;135:927\u0026ndash;99. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.1161/CIR.0000000000000484\u003c/span\u003e\u003cspan address=\"10.1161/CIR.0000000000000484\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eRife E, Gedalia A. Kawasaki disease: an update. Curr Rheumatol Rep. 2020;22:75. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.1007/s11926-020-00941-4\u003c/span\u003e\u003cspan address=\"10.1007/s11926-020-00941-4\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eAyusawa M, Sonobe T, Uemura S, Ogawa S, Nakamura Y, Kiyosawa N, et al. Revision of diagnostic guidelines for Kawasaki disease (the 5th revised edition). Pediatr Int. 2005;47:232\u0026ndash;4. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.1111/j.1442-200x.2005.02033.x\u003c/span\u003e\u003cspan address=\"10.1111/j.1442-200x.2005.02033.x\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eSon MBF, Newburger JW. Kawasaki disease. Pediatr Rev. 2018;39:78\u0026ndash;90. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.1542/pir.2016-0182\u003c/span\u003e\u003cspan address=\"10.1542/pir.2016-0182\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eBurgner D, Harnden A. Kawasaki disease: what is the epidemiology telling us about the etiology? Int J Infect Dis. 2005;9:185\u0026ndash;94. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.1016/j.ijid.2005.03.002\u003c/span\u003e\u003cspan address=\"10.1016/j.ijid.2005.03.002\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eLee MN, Cha JH, Ahn HM, Yoo JH, Kim HS, Sohn S, et al. Mycoplasma pneumoniae infection in patients with Kawasaki disease. Korean J Pediatr. 2011;54:123\u0026ndash;7. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.3345/kjp.2011.54.3.123\u003c/span\u003e\u003cspan address=\"10.3345/kjp.2011.54.3.123\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eSingh S, Gupta A, Jindal AK, Gupta A, Suri D, Rawat A, et al. Pulmonary presentation of Kawasaki disease-A diagnostic challenge. Pediatr Pulmonol. 2018;53:103\u0026ndash;7. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.1002/ppul.23885\u003c/span\u003e\u003cspan address=\"10.1002/ppul.23885\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003ePower A, Runeckles K, Manlhiot C, Dragulescu A, Guerguerian AM, McCrindle BW. Kawasaki Disease Shock Syndrome Versus Septic Shock: Early Differentiating Features Despite Overlapping Clinical Profiles. J Pediatr-US. 2020;231:162\u0026ndash;7. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.1016/j.jpeds.2020.12.002\u003c/span\u003e\u003cspan address=\"10.1016/j.jpeds.2020.12.002\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eLin Y, Cheng MC, Lo MH, Chien SJ. Early differentiation of Kawasaki disease shock syndrome and toxic shock syndrome in a pediatric intensive care unit. Pediatr Infect Dis J. 2015;34(11):1163\u0026ndash;7. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.1097/INF.0000000000000852\u003c/span\u003e\u003cspan address=\"10.1097/INF.0000000000000852\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eLamagni TL, Darenberg J, Luca-Harari B, Siljander T, Efstratiou A, Henriques-Normark B, et al. Epidemiology of severe Streptococcus pyogenes disease in Europe. J Clin Microbiol. 2008;46:2359\u0026ndash;67. \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e10.1128/JCM.00422-08\u003c/span\u003e\u003cspan address=\"10.1128/JCM.00422-08\" targettype=\"DOI\" class=\"RefTarget\"\u003e\u003c/span\u003e\u003c/span\u003e.\u003c/span\u003e\u003c/li\u003e\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":true,"highlight":"","institution":"","isAcceptedByJournal":false,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true},"keywords":"kawasaki disease, Kawasaki disease shock syndrome, polyserous effusion, pulmonary consolidation, toxic shock syndrome","lastPublishedDoi":"10.21203/rs.3.rs-3967125/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-3967125/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003ch2\u003eBackground\u003c/h2\u003e \u003cp\u003eKawasaki disease (KD) is a vasculitis syndrome of small to medium-sized arteries that has typical clinical characteristics such as fever, rash, cervical lymphadenopathy, conjunctivitis, and mucosal changes. Cardiac manifestations, including coronary artery aneurysms, myocarditis, myocardial infarction, and sudden cardiac death, are the most serious complications observed in KD. On rare occasions, it may accompanied with reduced organ perfusion due to systolic hypotension, a condition known as Kawasaki disease shock syndrome (KDSS). KDSS is a serious complication that can be presented to the emergency department(ED) as an initial feature when typical clinical symptoms of KD have not be detected.\u003c/p\u003e\u003ch2\u003eCase Presentation\u003c/h2\u003e \u003cp\u003eWe report the case of a twelve-year-old boy admitted with prolonged fever, bilateral non-purulent conjunctivitis, and shock symptoms such as hypotension and tachycardia. Laboratory findings showed elevated inflammatory markers, hypoalbuminemia, and sterile pyuria. He was initially treated with intravenous cefotaxime and vancomycin considering the possible diagnosis of toxic shock syndrome(TSS), while the treatment was not effective. Subsequent Chest computerized tomography(CT) and ultrasound identified pulmonary consolidation and polyserous effusion. Echocardiography revealed mild biatrial dilatation and mild valvular regurgitation with preserved left ventricular function. After a multidisciplinary consultation, a diagnosis of KDSS was made. To prevent coronary artery lesions and other severe complications, the patient immediately received immunoglobulin, corticoid, and acetylsalicylic acid. Soon afterwards, he showed significant improvement, with resolution of fever, polyserous effusions, and hypotension. Follow-up echocardiography revealed normal results.\u003c/p\u003e\u003ch2\u003eConclusions\u003c/h2\u003e \u003cp\u003eThis case indicated that elder children with KD are even more likely to develop KDSS. Clinicians should maintain a high index of suspicion for KD and consider pulmonary involvement and polyserous effusions as potential complications. For children with KD, any symptoms pointing to infection should be carefully considered. When there is no etiologic evidence, antibiotics should be used with caution. Our case also highlights the importance of considering KDSS as a differential diagnosis in children presenting with prolonged fever and shock. Early recognition, timely treatment, and close monitoring are key to preventing severe complications and ensuring favorable outcomes in patients with KDSS.\u003c/p\u003e","manuscriptTitle":"Kawasaki disease with shock as the primary manifestation: How to distinguish from toxic shock syndrome？ A Case Report and Literature Review","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2024-03-05 16:35:53","doi":"10.21203/rs.3.rs-3967125/v1","editorialEvents":[{"type":"communityComments","content":0}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true}}],"origin":"","ownerIdentity":"a165f7a4-e03d-4860-8bc0-b52135539c0a","owner":[],"postedDate":"March 5th, 2024","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"posted","subjectAreas":[],"tags":[],"updatedAt":"2024-04-26T03:37:54+00:00","versionOfRecord":[],"versionCreatedAt":"2024-03-05 16:35:53","video":"","vorDoi":"","vorDoiUrl":"","workflowStages":[]},"version":"v1","identity":"rs-3967125","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-3967125","identity":"rs-3967125","version":["v1"]},"buildId":"WrCJVZZCHTDjtuVLN7oU0","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}