A Case of Kawasaki Disease in NICU

Abstract

[Background] Kawasaki disease (KD) in children younger than 3 months of age is rarely observed. The rarity of disease in early infants may be due to protective effects of transplacental immunity and/or low level of exposure to infectious agent. We report a case of an infant who developed KD in a neonatal intensive care unit (NICU). [Case report] A male infant was born at 31 weeks of gestation; weighed 1772g, and admitted to our NICU. At 84 days of age, he presented with high fever, exanthema and dyspnea. He was diagnosed with pneumonia and treated with intravenous antibiotics and gammaglobulin (150 mg/kg/day, 3 consecutive days). The high fever lasted even after improvement of respiratory lesions, which were followed by bilateral conjectival injection, bright red lips and edematous changes in palms and soles. He was diagnosed with KD at 93 days of age (9 days of illness). Right and left coronary arteries were dilated up to 2 mm. He was treated with high-dose gammaglobulin (1500 mg/kg/dose), however, fever and other symptoms persisted. He developed pericardial effusion and bilateral coronary arterial aneurysms (4.5 mm), despite 4 additional gammaglobulin injections (1000 mg/kg/dose, respectively). Beginning at 24 days of illness, he was treated with intravenous ulinastatin (15000 U/kg/day) for 5 days, and fever and all other symptoms disappeared. He was discharged at 63 days of illness. Follow-up cardiac catheterization and angiography at 2 months after discharge confirmed bilateral coronary arterial aneurysms. [Discussion] The present case developed KD even in extremely isolated environment as NICU. One possible explanation is that premature infants receive insufficent passive immunity from their mothers. In this infant, ulinastatin therapy was effective. Thus this treatment should be considered for young infants who are resistant to intravenous gammaglobulin therapy