Corticosteroids Add-on Therapy in the Acute Phase of Kawasaki Disease

PURPOSE: Recently, clinical trials of steroid add-on therapy were reported with variable results in Kawasaki disease. We analyzed the clinical outcomes of patients at high risk of with Kawasaki disease(> or = 4 points of Harada score) treated by three commonly used different treatment regimens, with or without corticosteroids. METHODS: Medical records of 96 children with Kawasaki disease treated with one of the threee regimens were reviewed retrospectively. Regimen 1 was aspirin(100 mg/kg/day) plus intravenous gamma globulin 2 g/kg single dose; regimen 2, aspirin(100 mg/kg/day) plus intravenous gamma globulin 1 g/kg single dose; regimen 3, regimen 2 plus prednisolone(2 mg/kg/day), followed by tapering two weeks and pulse therapy of methyl prednisolone performed in cases of retreatment. Also low dose aspirin was given in all three regimens for eight weeks after the acute phase. The cardiovascular and laboratory evaluations were performed on acute phase, immediate after acute phase, and subacute phase, eight weeks after treatment. RESULTS: The frequency of coronary artery lesions and laboratory findings in the three different regimens were similar. The more rapid control of fever after treatment was noted in regimen 3. Furthermore the frequency of retreatment was decreased in regimen 3 compared to the other two regimens. CONCLUSIONS: Steroid add-on therapy showed some beneficial outcome compared to conventional treatment regimens. The role of steroid in the treatment of Kawasaki disease should be reassessed in systemic manner.

A Case of Poland Syndrome with Diabetic Ketoacidosis / 대한소아내분비학회지

Poland syndrome is characterized by an absent of the pectoralis major muscle, with ipsilateral defect of the upper extremity, usually syndactyly. The incidence of this syndrome has been estimated at 1 per 32,000 persons and more than 400 patients have been described worldwide after Alfred Poland's report. but there was no report of Poland syndrome associated with Diabetic Mellitus in childhood. Recently, we experienced a 14 year-old female patient who showed typical Poland syndrome, a left pectoralis muscle hypoplasia and left synbrachydactyly, associated with Diabetes mellitus complicated by ketoacidosis.