Arterial switch operation for transposition of the great arteries: experience from 2000-2002 in Taiwan.

Arterial switch operation (ASO) is considered the procedure of choice for transposition of great arteries (TGA). The results and long-term prognosis improved with recent advances in perioperative management. We herein analyze the clinical outcome of patients undergoing ASO at our institution during the past 3 years.

Poor outcome for neonatal-type nonketotic hyperglycinemia treated with high-dose sodium benzoate and dextromethorphan.

Neonatal-type nonketotic hyperglycinemia treatment remains unsatisfactory, even if started early. A review of six patients who underwent treatment for neonatal-type nonketotic hyperglycinemia in our hospital is presented. All patients were treated with a standardized protocol.

Fatal coxsackievirus A16 infection.

A 15-month-old boy presenting with hand, foot and mouth disease died of myocarditis and intractable shock caused by coxsackievirus A16 infection. It is apparent that coxsackievirus A16 infection is not always a benign infection.

Phenotype and genotype analyses of ornithine transcarbamylase deficiency in Taiwanese.

Background And Purpose: Ornithine transcarbamylase (OTC) deficiency is the most common inherited urea cycle disorder. It is an X-linked semidominant disease with variable severity affecting both males and females. The characteristics and course have not been assessed in Taiwanese.