CASE REPORTS Nonketotic Hyperglycinemia in a

Hyperglycinemia represents a group of disorders characterized by elevated con-centrations of glycine in body fluids. Two types exist, the ketotic and non ketotic. In the ketotic type, the most striking feature is ketoacidosis, which begins early in life and in which hyperglycinemia is secondarily associated with organic acidemias. Nonketotic hyperglycinemia (NKH) is a disorder of glycine metabolism due to a molecular defect in the glycine cleavage system (GCS)(1,2). Though NKH is a well documented entity, to the best of our knowledge there are no reports of this dis-order from India, the result of inadequate diagnostic facilities. We report a case of NKH in a neonate, with a review of its re-cent therapeutics. Case Report A male baby weighing 2490 g was born at term, after an uneventful antenatal peri-od, to a 25-year-old fifth gravida mother. He was the product of a non-consanguine-ous marriage. There was history of early neonatal deaths of previous 3 male term in-fants. They had no history of birth asphyxi