The average annual rate of incidence, 105.0/100,000 in children <5-year-old is the second highest reported rate in the world.

The postero-superior walls of the atrial chambers in hearts with isomeric atrial appendages can be analysed on the basis of a compound structure made of bilateral systemic venous components, a central pulmonary venous component, and the body of the atrium. Hearts with isomeric right appendages have absence or hypoplasia of the pulmonary venous component, while hearts with isomeric left appendages have hypoplastic systemic venous components.

Intracardiac thrombosis in patients with a dilated cardiomyopathy can be life threatening. This study investigated the incidence, risk factors, and outcome of intracardiac thrombosis in children with dilated cardiomyopathy. A retrospective review of the clinical records was performed in 83 children with dilated cardiomyopathy diagnosed from January 1995 to December 2008. Intracardiac thrombi were detected in 5 patients (6.0%). The intracardiac thrombi were found mainly in the left ventricle (n = 3). One patient had a thrombus in the left atrium at the time of diagnosis, and a right ventricular thrombus was found in 1 patient with unrepaired ventricular septal defect complicated by pulmonary hypertension. Intracardiac thrombosis developed during rapid deterioration of ventricular function, and all patients had a poor ejection fraction of the left ventricle. All patients were treated with heparinization, and thrombectomy was performed in 1 patient. Three patients achieved complete resolution of the thrombus without further embolic complications. Careful evaluation and aggressive anticoagulation are necessary for the prevention of intracardiac thrombosis in children with poor ventricular function, especially during rapid deterioration of ventricular function.

We report two cases of microvillus inclusion disease and these are the first cases in Korea. The two babies (one baby had a sibling who died of diarrhea in the neonatal period) had excreted their stools up to 200 ml/kg per day since several days after birth. Workup's included extensive infectious, immunologic, hormonal and rheumatologic studies, all of which were negative or normal. Diagnosis rested on the ultrastructural finding of intracytoplasmic inclusions that contained intact microvilli on electron microscopy. We tried somatostatin analogue (octreotide, 4 micrograms/kg/day), cholestyramine (up to 4g t.i.d.), steroid (prednisone, 2 mg/kg/day) and intravenous epidermal growth factor (100 ng/kg/hr for 2 weeks), but there was mild improvement with cholestyramine (decrease stool volume) and epidermal growth factor (increase the number of microvilli per cell) but no improvement was noted with the other treatments. Although it is a rare disorder and the prognosis of microvillus inclusion disease is poor, it must be considered if an infant has chronic secretory diarrhea.