Paediatrics and International Child Health

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Kawasaki disease shock syndrome: case report Hui-Fang Yang, Wei-Liang Chen, Chia-Ning Chang, Shyi-Jou Chen & HuengChuen Fan To cite this article: Hui-Fang Yang, Wei-Liang Chen, Chia-Ning Chang, Shyi-Jou Chen & HuengChuen Fan (2016) Kawasaki disease shock syndrome: case report, Paediatrics and International Child Health, 36:1, 76-78, DOI: 10.1179/2046905515Y.0000000002 To link to this article: http://dx.doi.org/10.1179/2046905515Y.0000000002

Published online: 27 Apr 2016.

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Date: 10 July 2016, At: 20:12

Case Report

Kawasaki disease shock syndrome: case report Hui-Fang Yang1, Wei-Liang Chen1, Chia-Ning Chang2, Shyi-Jou Chen2, Hueng-Chuen Fan2 1

Departments of Family Medicine & Community Health and 2Pediatrics, Tri-Service General Hospital, National Defence Medical Center, Taipei, Taiwan

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Kawasaki disease (KD) is a systemic vasculitis which presents with stable vital signs. Shock rarely occurs in such cases, but it may occur in the acute phase of KD. This report describes a 7-year-old boy with KD shock syndrome (KDSS) who presented with persistent fever, injected conjunctiva, a polymorphic skin rash, echocardiography indicating coronary artery dilatation, and shock. The patient’s haemodynamic status markedly improved with immunoglobulin therapy. Early recognition of KDSS can be challenging; however, delay in diagnosis can increase the risk of coronary artery abnormalities and death. Keywords: Immunoglobulin, Kawasaki disease, Shock

Introduction Kawasaki disease (KD), an idiopathic acute systemic inflammatory childhood illness, is most common in Asian populations, and the highest annual incidence (approximately 215/100,000) is in children in Japan aged ,5 years.1 In Taiwan, the incidence rate is 69/ 100,000 children ,5 years, which is 10 times greater than in Western countries.2 Moreover, the incidence of development of coronary artery aneurysm in KD patients is as high as 7.2% in Taiwan.2 Immune-mediated mechanisms are hypothesised as the underlying pathophysiology of KD, and intravenous immunoglobulin (IVIG) therapy is found to rapidly resolve KD-associated inflammation. If untreated, however, the incidence of coronary artery abnormalities, including vasculitis, ectasia and aneurysm, can be high, ranging from 20% to 25%.3 Some patients may develop coronary artery thrombosis, stenosis or myocardial infarction; others may even die. Diagnosis of KD depends on excluding other diseases and recognizing the typical constellation of clinical features: fever for at least 5 days and at least four of five principal signs [polymorphous skin rash, changes in the hands and feet (erythema, oedema or peeling), bilateral non-exudative bulbar conjunctival injection, changes in the lips and oral cavity, and

Correspondence to: H-C Fan, Department of Pediatrics, Tri-Service General Hospital, National Defense Medical Center, No. 325, Sec. 2, Chenggong Road, Neihu District, Taipei City 114, Taiwan (ROC). Fax: z886 2 8792 7293; email: [email protected]

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cervical lymphadenopathy of .1.5 cm].4 At present, there are no confirmatory laboratory tests for KD. Shock is defined as sustained hypotension (a decrease in blood pressure of .20% from the baseline) or clinical signs of inadequate organ perfusion.5 Shock rarely occurs in patients with KD; however, in patients who do present with shock, it is a clue to the diagnosis of KD shock syndrome (KDSS). KDSS is potentially fatal, and a case-control study reported that a number of children with a presumptive diagnosis of toxic shock syndrome (TSS) or septic shock were subsequently diagnosed with KDSS.6 Therefore, there is considerable overlap between the clinical features of KDSS, septic shock and TSS. This report describes a 7-year-old boy who presented with KDSS.

Case Report A previously healthy 7-year-old boy presented with a 3-day history of fever, anorexia and vomiting. On examination, he was alert and appeared well; there was no palpable cervical lymphadenopathy. The cardiorespiratory examination was normal with no audible heart murmurs. Positive clinical findings were an injected throat, red and oedematous conjunctiva, and an erythematous maculopapular rash on the truck, arms and legs. Investigations. Haemoglobin was 10.6 g/dl; white blood cell (WBC) count 10.16109/L; platelets 476109/L and C-reactive protein 219 mg/L (,8 mg/ L). Urinalysis demonstrated protein 2z and pyuria (10–12 WBC/high power field). There was no history

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Figure 1 1A Echocardiography demonstrating a dilated right coronary artery of 0.38 cm (arrow). 1B Echocardiography demonstrating a dilated left anterior descending coronary artery of 0.35 cm (arrow)

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of allergy or recent travel. He was admitted with a provisional diagnosis of urinary tract infection. On day 1, ampicillin and gentamicin were administered. However, the patient became drowsy and could not tolerate the pain. He was tachycardic (121 beats/ min), tachypnoeic (38 breaths/min) and hypotensive (66/31 mmHg). His extremities were warm, but the capillary refill time was .2 s. Investigations. Haemoglobin was 12.2 g/dl; WBCs 8.56109/L; platelets 656109/L; D-dimer 5324 mg/L (,500 mg/L); fibrinogen 4.54 g/L (2–4); fibrin degradation products .40,000 mg/L (,10,000); prothrombin time 12.6 s; partial thromboplastin time 59 s; albumin 24 g/L; sodium 139 mmol/L; potassium 3.5 mmol/L; troponin-I 0.07 mg/L (,0.01); aspartate aminotransferase 30 U/L; and alanine aminotransferase 27 U/L. Septic shock or TSS was suspected and 0.9% normal saline (20 ml/kg) was initially administered and then inotropic agents such as dopamine (15 mg/h) and dobutamine (3 mg/h) were rapidly infused. Vancomycin (60 mg/kg) and ceftriaxone (83 mg/kg) were also administered. However, the patient’s condition deteriorated with the development of severe hypotension (55/28 mmHg), tachycardia (142 beats/min) and tachypnoea (40 breaths/min). He was transferred to the paediatric intensive care unit as an emergency. On day 2, respiratory distress with sudden oxygen desaturation to 80% as well as worsening tachypnoea (60 breaths/min) occurred, which prompted intubation and mechanical ventilation. Two-dimensional echocardiography indicated mild tricuspid regurgitation, moderate mitral regurgitation, a dilated right coronary artery (0.38 cm, Z-score 4.45) (Fig. 1A), a dilated left anterior descending coronary artery (0.35 cm, Z-score 3.85) (Fig. 1B) and an ejection fraction of 62%. Cultures of the pharynx and throat, blood and urine were negative. Virus profiles (Epstein–Barr virus, herpes simplex virus, cytomegalovirus, enterovirus, H1N1 influenza virus and

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adenovirus) and the anti-streptolysin O titre were negative. Incomplete KD was therefore diagnosed. IVIG (2 g/kg) and aspirin (100 mg/kg21 day21) were administered on day 4. Thereafter, the fever, drowsiness, labile blood pressure and oxygen desaturation improved markedly. On day 6, the patient was extubated and transferred to the paediatric ward. On day 9, peri-ungual desquamation of the extremities was noted. On day 11, the patient recovered completely without sequelae. At 1-year follow-up, the dilated left anterior descending coronary artery and right coronary artery had returned to normal.

Discussion Because shock rarely occurs in KD,5–7 clinicians may overlook this condition in patients exhibiting shock at the initial presentation. If clinicians fail to recognise the full spectrum of the disease severity and if it is untreated, KDSS can be fatal. A report estimated that the incidence of KDSS in KD patients aged .5 years was significantly higher than in younger patients (2.20% vs 1.37%).7 Age may be an associated factor in the progression of KD to KDSS, a theory supported by this case report. The risk factors for the progression of KD to KDSS include female gender, higher CRP levels and band counts, and lower platelet counts, albumin levels and haemoglobin concentrations.5,6 Additionally, more than half of KDSS patients do not respond to the initial IVIG therapy and may require a second course or multidrug therapy, such as corticosteroids or monoclonal antibodies.5,6 High CRP levels, low haemoglobin and albumin levels, consumptive coagulopathy with thrombocytopaenia, a positive D-dimer result and prolonged partial thromboplastin time occurred in this patient, all of which might have contributed to the progression to KDSS. However, male gender, age, no band form in the WBC count, normal left ventricle systolic function,

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early diagnosis of KDSS and appropriate treatment may explain our patient’s good response to IVIG therapy and his excellent prognosis without sequelae. The mechanisms of severe hypotension in KD remain unclear and are probably multiple (cytokine dysregulation, myocardial dysfunction and excessive inflammation).6 The vascular endothelial growth factor (VEGF), a potent vascular permeability factor, induces the development of fenestrae in venous and capillary endothelia.8 Cytokine dysregulation may upregulate VEGF overexpression, increase vascular permeability, and cause hypoalbuminaemia during the acute phase of KD,9 leading to altered vasomotor tone and decreased vascular resistance, thereby causing haemodynamic instability. Inflammatory cytokines are reported to cause myocardial dysfunction and damage to the vascular endothelial cells,6 leading to hypotension. In this case, consumptive coagulopathy, which is rarely noted in cases of KD, may have developed owing to increased inflammatory cascades. These findings also suggest that the vasculitis underlying KDSS may be more intensive than that underlying KD. Additionally, there was no evidence of sepsis: all his cultures were negative. TSS was excluded because of the lack of specific echocardiographic findings. Therefore, the possibility of septic shock and TSS were ruled out. This case highlights the rarity and diagnostic difficulty associated with KDSS and how the unusual

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haemodynamic profile and coronary artery abnormalities related to this condition can be life-threatening. However, the early identification of KDSS and IVIG therapy resulted in a favourable outcome without sequelae.

References 1 Nakamura Y, Yashiro M, Uehara R, Sadakane A, Chihara I, Aoyama Y, et al. Epidemiologic features of Kawasaki disease in Japan: results of the 2007–2008 nationwide survey. J Eepidemiol. 2010;20:302–7. 2 Huang WC, Huang LM, Chang IS, Chang LY, Chiang BL, Chen PJ, et al. Epidemiologic features of Kawasaki disease in Taiwan, 2003–2006. Pediatrics. 2009;123:e401–5. 3 Kato H, Sugimura T, Akagi T, Sato N, Hashino K, Maeno Y, et al. Long-term consequences of Kawasaki disease: a 10- to 21-year follow-up study of 594 patients. Circulation. 1996;94: 1379–85. 4 Rowley AH, Shulman ST. Kawasaki disease. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics, 18th edn. Philadephia: Saunders Elsevier. 2007; 1036–42. 5 Kanegaye JT, Wilder MS, Molkara D, Frazer JR, Pancheri J, Tremoulet AH, et al. Recognition of a Kawasaki disease shock syndrome. Pediatrics. 2009;123:e783–9. 6 Dominguez SR, Friedman K, Seewald R, Anderson MS, Willis L, Glode MP. Kawasaki disease in a pediatric intensive care unit: a case-control study. Pediatrics. 2008;122:e786–90. 7 Lin MT, Fu CM, Huang SK, Huang SC, Wu MH. Populationbased study of Kawasaki disease shock syndrome in Taiwan. Pediatr Infect Dis J. 2013;32:1384–6. 8 Roberts WG, Palade GE. Increased microvascular permeability and endothelial fenestration induced by vascular endothelial growth factor. J Cell Sci. 1995;108:2369–79. 9 Natterer J, Perez MH, Di Bernardo S. Capillary leak leading to shock in Kawasaki disease without myocardial dysfunction. Cardiol Young. 2012;22:349–52.

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Kawasaki disease shock syndrome: case report.

Kawasaki disease (KD) is a systemic vasculitis which presents with stable vital signs. Shock rarely occurs in such cases, but it may occur in the acut...
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