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Kawasaki Disease in India

Kawasaki Disease in India    

Kawasaki disease (KD) is An acute self-limiting  SystemicVasculitis of unknown etiology.
Kawasaki Disease was first described by Dr Tomisaku Kawasaki in Japan in 1967, since then prevalence of KD is increasing in all countries as per the reports in journals. This is more prevalent in Asian pacific regions.  Kawasaki Syndrome (KS) is the most common cause of acquired heart disease in the U.S. and Japan. High endemic rate of 150 per 100,000 in children less than 5 years of age in Japan first in the world. There was general consensus among the physicians that the number of diagnosed cases of KD is increasing in India. Incidence of KD in India is so far not available. But recent publications and presentations on KD showed that number of cases are increasing. At first, many physicians responded that increased awareness of the syndrome accounted for the growing incidence and that KD most likely had been misclassified as drug reactions or viral or bacterial toxin-mediated illness. Most often overlapping picture of Measels may misguide the physicians and pediatricians in accurate diagnosis of KD. Males are more affected (1.6:1) and 80% will seen less than 5 years of age. Morbidity and Mortality from KD is due to Cardiac Sequelae.

Untreated children with KS are at risk for development of potentially fatal coronary artery aneurysms. If you treat with intravenous immunoglobulin (IVIG), we can prevent coronary artery dilatation to 4-6% from 25% without IVIG. More than 50% of aneurisms of coronary artery will subside by 2-5 years of age and evidence suggests that even after regression, vascular endothelial dysfunction may persist, hence children with KD need follow up.  

We had found that possible differences in the clinical presentation of KD in India as compared to other countries. First, while 85% of KS patients in the U.S. and Japan are less than 5 years of age, in India there was a higher proportion of older patients. Second, peeling of the extremities coincided with the acute phase of fever and often occurred within the first 10 days after fever onset. In the U.S and Japan, this desquamation generally occurs after 10 days, during convalescence from KD. Third, thrombocytosis was frequently noted during the early, acute phase of the illness whereas in the U.S., only 50% of children presenting within the first 10 days after fever onset had a platelet count greater than 450,000/ µL. Early peeling of the extremities and elevated platelet count were frequently cited as helpful in establishing the diagnosis of KD. Despite these apparent differences in clinical presentation and course of the illness which should be explored more fully, these cases seems to fit into the recognized spectrum of KS seen throughout the world.  (Indian pediatrics; Dr Jane C.Burns)

Despite more than 40 years of research, its cause remains a mystery.  Possible etiology behind the KD was published like viral, bacterial, fungal, superantigen theories, but no one could able to prove the exact cause. As per our first animal model created for KD ( Dr.Saji Philip; Pediatric Research 2004; 55(2) : 211-216) we had found that immuno pathogenesis in KD could be like type III hypersensitive reaction leading to immune complex vasculitis. Efforts should continue to increase physician awareness and to encourage research on the characterization of KD in India with emphasis on possible differences in clinical presentation, disease course, and outcome in Indian children. One impediment to the evaluation and treatment of cardiovascular sequelae in these children is the scarcity of pediatric cardiologists to perform echocardiographic imaging of the coronary arteries. Work to develop criteria for normal coronary artery internal diameters in southern Indian children should be validated and adapted for different populations throughout India. Whether KD is new or old in India, the KD disease burden is likely to pose a significant challenge to the health care system in India in the coming years due to the high cost of treatment and the potential for lifelong cardiovascular sequelae. In our animal model studies, simple antioxidant therapy will be helpful in reduction of vasculitis. Along with gold standard therapy with Aspirin and IVIG, you can give high doses of vitamin A, C and E for 10days will reduce the coronary artery incidence further and clinical studies are going on for this combination (Table 1). Hence any rash with fever, patients should ask to the doctor to reconfirm the diagnosis and differential diagnosis to be considered for the diagnosis of KD.