A Case of Moyamoya Disease in a Child with Alagille Syndrome

Alagille syndrome is a autosomal dominant disorder characterized by intrahepatic bile duct paucity and resultant chronic cholestasis in combination with cardiac(mainly peripheral pulmonary stenosis), skeletal, ocular, and facial abnormalities. In addition to the pulmonary stenosis, in large series, anecdotal reports of vascular lesions have concerned the renal artery, aorta, hepatic artery, carotid artery, celiac artery or subclavian artery. Theses diffuse vascular abnormalities, which appear to be a feature of Alagille syndrome, suggest Notch signaling pathway defects affect angiogenesis. The associations of Alagille syndrome with moyamoya disease, the chronic cerebrovascular occlusive disease, were reported and suggested as additional evidence of vasculopathy of Alagille syndrome. We report another 25 month-old Alagille syndrome girl who presented with acute left hemiparesis and was diagnosed with moyamoya disease through the cerebral angiographic study.

A Case of Mucoepidermoid Carcinoma of Bronchus / 소아알레르기및호흡기학회지

Primary bronchial neoplasms in children, especially malignant lesions, are extremely rare. We report a case of a 10-year old boy with intermediate-grade mucoepidermoid carcinoma of the right intermediate bronchus, presenting as collapse and bronchiectasis after pneumonia. He was diagnosed by bronchoscopic biopsy. The lobectomy was performed. The patient is in good condition 1 year after operation. All reported cases of bronchial mucoepidermoid tumors in children had histologically low or intermediate grade(well differentiated) variety with a benign clinical course. The optimal surgical therapy for bronchial mucoepidermoid tumors is identical. There must be total removal of either mass with the sacrifice of as little normal lung as possible.

Frequencies and Risk Factors for Microvascular Complications in Patients with Type 1 Diabetes Mellitus / 대한소아내분비학회지

PURPOSE: This study was undertaken to identify the frequencies and the risk factors of microvascular complications in subjects with type 1 diabetes mellitus METHODS: The frequencies and their relation to risk factors of microvascular complications were analyzed in 29 type 1 diabetes mellitus subjects with duration of disease more than 5 years. Microvascular disease was defined as the presence of either retinopathy, microalbuminuria or neuropathy. RESULTS: The overall prevalence rate of microvascular disease was 8/29(27.6%). Retinopathy has developed in 3 patients(10.3%), microalbuminuria in 7 patients(24.0 %) and neuropathy in 5 patients(17.2%). The mean HbA1C was significantly higher in the patients with microvascular complications(11.6+/-.2% in microvascular complication group vs 9.3+/-.6% in control group). CONCLUSION: In childhood onset type 1 diabetes mellitus, poor glycemic control is an important risk factor for microvascular complications.