Abstract
Kawasaki disease is an acute febrile condition seen in children. However, it is also well recognized that some patients do not fulfill the classic diagnostic criteria for the diagnosis of kawasaki disease. The incomplete form of kawasaki disease is termed as ‘Incomplete KD’ or ‘Atypical KD’. We present a case of a 6 year old child with a history of prolonged fever, periorbital, oral and lip changes, changes in the extremities and an erythamatous, maculopapular rash. Based on the physical exam and her echocardiogram that showed right coronary artery dilatation, Intravenous immune globulin was administered in this patient. This patient was refractory to two doses of intravenous immune globulin and therefore was started on methylprednisolone, to which she responded dramatically. The diagnostic dilemma primarily arose when this child presented with joint pain a day after her discharge from the hospital and a positive laboratory workup. So, was this a case of incomplete Kawasaki refractory to intravenous immuno globulin therapy or systemic juvenile idiopathic arthritis? We suggest that physicians should be cognizant of the fact that they must individualize every patient’s management to the best of their knowledge and judgment, rather than merely going by the guidelines.
Highlights
On examination, she had an irritable and toxic look with bilateral peri-orbital swelling and cracked lips
Based on the physical exam and her echocardiogram that showed right coronary artery dilatation, Intravenous immune globulin was administered in this patient
This patient was refractory to two doses of intravenous immune globulin and was started on methylprednisolone, to which she responded dramatically
Summary
Kawasaki disease (KD) is an acute febrile condition seen in children. Even though it was first reported in Japan about 30 years ago, the original diagnostic criteria defined by Dr Tomisaku Kawasaki in 1967 are still authentic and widely used today. The tests showed that the patient had a C-reactive protein level of ≥3.0 mg/dL and fulfilled ≥3 supplemental laboratory criteria (albumin ≤3.0 g/dL, anaemia for age, platelets after 7 days ≥45 × 104/μL, WBC counts ≥15 000/ μL and urine ≥10 WBCs/ high-power field). We considered this patient to be suffering from incomplete KD. As long as the disadvantages of undertreating appear to outweigh the disadvantages of overtreating, IVIG will be used injudiciously to treat these children This approach of treating children at risk even though the diagnosis is uncertain should result in fewer children with incomplete KD going untreated and subsequently developing coronary artery aneurysms. With increasing knowledge, diagnostic accuracy will improve over time, thereby ensuring that proper therapy is instituted correctly in patients with KD and SJIA
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