Department of Paediatrics and Adolescent Medicine, United Christian Hospital, 130 Hip Wo Street, Kwun Tong, Kowloon, Hong Kong, China
HNT Hui ( 許寧達 ) MBChB, MRCP, MRCPCH
YC Ho ( 何鈺照 ) MBChB, FHKCPaed, FHKAM(Paed)
HB Chan ( 陳衍標 ) MBBS, FRCPCH, FHKCPaed
PLS Ip ( 葉麗嫦 ) MBBS(Syd), FRCPCH, FHKCPaed
Division of Clinical Biochemisty, Queen Mary Hospital, 102 Pokfulam Road, Hong Kong, China
SCF Tam ( 譚志輝 ) MBBS, FRCPA, FHKCPath
Department of Chemical Pathology, Prince of Wales Hospital, 30-32 Ngan Shing Street, Shatin, N.T., Hong Kong, China
NLS Tang ( 鄧亮生 ) MBChB, MD
Correspondence to: Dr HNT Hui
Received August 16, 2003
Nonketotic hyperglycinaemia or nonketotic hyperglycinaemic encephalopathy (NKH) is an autosomal recessive inborn error of glycine metabolism. We report a baby with the disease treated with sodium benzoate, dextromethorphan and anticonvulsants who succumbed at 4 months of age.
