Kawasaki disease masquerading as jaundice.

While the myriad manifestations of Kawasaki disease are increasingly being recognized, there are still many instances where this diagnosis can come as a surprise to the clinician. The authors report one such child who appeared to have acute infective hepatitis at presentation but went on to develop the full clinical picture of Kawasaki disease. This is a rarely reported presentation of Kawasaki disease.

Symptomatic myocarditis in Kawasaki disease.

Kawasaki disease (KD) is an acute febrile illness of infants and young children that is characterized by a medium vessel vasculitis, most commonly involving the coronary arteries. Though subclinical myocarditis is rather common in KD, symptomatic congestive heart failure is extremely uncommon. The authors report a 9-y-old boy who developed heart failure (ejection fraction 28%) in the acute phase of KD. He was initially treated with intravenous immunoglobulin (2 g/kg) without much clinical improvement. He was then given 5 daily pulses of intravenous methylprednisolone followed by tapering doses of oral prednisolone. The child showed prompt clinical recovery and remains well on follow-up. The present case serves to highlight the fact that methylprednisolone can be considered upfront as rescue therapy in children with KD who have symptomatic congestive cardiac failure during the acute stage of the disease.

Is Kawasaki disease incidence rising in Chandigarh, North India?

OBJECTIVE: To estimate incidence of Kawasaki disease (KD) over time among children in the city of Chandigarh, North India. PATIENTS AND METHODS: We analysed records of all children with KD below 15 years of age at the Pediatric Allergy Immunology Unit, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, from January 1994 to December 2008. Diagnosis and treatment of KD were based on American Heart Association criteria. Among these cases, children residing in Chandigarh were identified. Yearly incidence was calculated and compared from 1994 to 2008. RESULTS: During this period, 196 children were diagnosed as KD. Of these, 80 (40.8%; 48 boys, 32 girls) resided in Chandigarh. Four among these had coronary artery abnormalities (CAA) on echocardiography, while two had mitral regurgitation. Comparison of yearly data revealed increasing incidence of disease from 0.51 cases in 1994 to 4.54 cases per 100,000 children below 15 years of age in 2007. Majority (93.7%) of cases occurred in children ≤10 years of age with the highest incidence reported in the seventh year of life. Monthly distribution of disease showed two peaks with a maximum number of cases presenting in October followed by a second peak in May with a nadir in the month of February. CONCLUSION: Increasing incidence of KD in Chandigarh could be due to increasing clinical recognition as a result of greater awareness among paediatricians in the city, or may represent an actual increase in numbers. Striking differences from KD series reported from other countries include the older median age of our patients, low rate of CAA and a different bimodal seasonality, which may be epidemiologic clues to the nature of this vasculitis.

QT interval dispersion in North Indian children with Kawasaki disease without overt coronary artery abnormalities.

Increased QT interval dispersion has been associated with an increased risk for ventricular arrhythmias and sudden cardiac events. We examined the QT interval dispersion in 20 North Indian children with Kawasaki disease (KD) with no coronary artery abnormalities on echocardiography compared the same with matched controls. The study population consisted of 20 children in convalescent phase of KD and 20 age and sex-matched healthy controls. Intervals were measured with the use of a digital caliper with least count of 0.01 mm by a single blinded observer. The QTc dispersion was calculated as the difference between the maximum and minimum corrected QT intervals in 12 and 8 leads (i.e. the 6 precordial leads, the shortest extremity lead, and the median of the 5 other extremity leads). Of the 480 leads obtained (12 per subject), 36 were excluded from analysis (15 because of poor T wave formation and 11 because of presence of U waves). Children with KD had significantly higher QTc dispersion in 12 lead (67.08 ± 17.72 ms compared to 47.63 ± 16.48 ms in controls P ≤ 0.001) as well as 8 lead (60.51 ± 18.54 ms compared to 42.92 ± 18.03 ms in controls P ≤ 0.001) analysis. There was no correlation between delay in IVIG therapy and QT interval dispersion. In conclusion, QT interval dispersion is significantly increased in North Indian children with KD. The dispersion is indicative of inhomogenous ventricular repolarization and may represent increased risk for developing ventricular arrhythmia in this population.

Exercise myocardial perfusion imaging to evaluate inducible ischaemia in children with Kawasaki disease.

OBJECTIVE: Kawasaki disease (KD) is an acute multi-system vasculitis of unknown aetiology, which occurs predominantly in infants and young children. Coronary artery abnormalities may occur in 15-25% of patients who are not treated in the acute phase of the disease with a high dose of intravenous immunoglobulin. Myocardial perfusion imaging is used as a modality to monitor the cardiovascular effects of the disease. The objective of our study was to assess the feasibility and results of exercise myocardial perfusion scintigraphy in children with Kawasaki disease. METHODS: We performed stress myocardial perfusion imaging in 84 patients suffering from KD. The diagnosis of KD was based on the criteria laid down by the American Heart Association. Myocardial perfusion imaging was performed using either thallium or technetium-99m tetrofosmin. Physical exercise using the Bruce protocol was the most frequent cardiac stressor (74 patients) whereas in few patients (seven patients) dobutamine was used to increase the heart rate. RESULTS: Stress-induced reversible perfusion defects were found only in 12 of the total number of patients. Among these two had coronary artery abnormalities on echocardiography. Four of these patients had achieved adequate heart rate with stress. Two among these patients had a repeat imaging done after 1 year and the perfusion defects showed complete resolution in them. CONCLUSION: This study thus suggests that reversible perfusion defects are seen in asymptomatic patients with KD and that the presence of perfusion defects may not be associated with echocardiographic demonstration of coronary abnormalities. The treadmill test is a reasonable stress protocol for these patients.

Endothelial dysfunction in a cohort of North Indian children with Kawasaki disease without overt coronary artery involvement.

OBJECTIVE: Kawasaki disease (KD) is a diffuse necrotizing vasculitis with predominant involvement of coronary arteries. Endothelial dysfunction has been implicated as an important event in the pathogenesis of arteriosclerosis, coronary vasoconstriction, hypertension, and myocardial ischemia. We examined the presence of endothelial dysfunction in North Indian children (of Caucasoid ethnicity) with KD without overt coronary artery involvement. METHODS: Twenty children (mean age 8.4+/-2.3 years; range 4.5-12.1 years) in the convalescent phase of KD were studied. All had received intravenous immunoglobulin during the acute phase of the disease. The interval between acute episode and enrolment ranged from 3 to 78 months (mean 25.3+/-20.1 months). High-resolution ultrasonography was used to analyze brachial artery responses to reactive hyperemia (with increased flow causing endothelium-dependent dilatation) and sublingual nitroglycerine (causing endothelium-independent dilatation). Flow-mediated dilatation was also studied in an equal number of healthy age- and sex-matched controls. Carotid artery stiffness index (SI) was calculated and compared in all subjects using previously published equations. RESULTS: Significant differences were observed between the percent flow mediated dilatation in children with KD (5.7+/-9.2%) compared with controls (12.2+/-8.9%, p=0.017). Sublingual nitroglycerine-mediated dilatation in children with KD was 28.5+/-12.3%. Carotid artery SI was higher in children with KD (2.81+/-0.77 U) when compared to controls (2.32+/-0.80 U), but it failed to meet statistical significance (p=0.058). CONCLUSION: Brachial arteries of children with KD show evidence of endothelial dysfunction. This may point towards possibility of preclinical arteriosclerosis in children with KD even in the absence of coronary artery abnormalities.

Left main coronary artery compression by dilated main pulmonary artery in endocardial cushion defect.

A 56-year-old man was evaluated for exertional dyspnoea. Chest X-ray showed mild cardiomegaly and a dilated main pulmonary artery. On echocardiogram he was found to have ostium primum atrial septal defect with moderate tricuspid insufficiency. Cardiac catheterization revealed an oximetry step-up of 14% at low right atrium with angiogram demonstrating a cleft in the mitral valve, an elongated left ventricular outflow tract and ventricular septal defect closed by a septal aneurysm. Coronary angiogram revealed ostial compression of the left main coronary artery with the rest of the coronary artery anatomy being normal.