A treatment of non-ketotic hyperglycinaemia.
The treatment comprising a special diet (without glycine, serine, and with a reduced amount of threonine), strychnine nitrate and ursodesoxycholic acid (UDCA) led to normoglycinaemia in this form of severe non-ketotic glycine encephalopathy. Diet and treatment were well tolerated but without significant effect upon psychomotor development. This treatment should be more effective if administered before irreversible brain damage occurs, particularly in moderate and chronic forms of NKH.
D000138:Acidosis; D000592:Amino Acid Metabolism, Inborn Errors / Q000235:genetics / Q000628:therapy*; D002675:Child, Preschool; D003131:Combined Modality Therapy; D003840:Deoxycholic Acid / Q000031:analogs & derivatives*; D005260:Female; D005526:Food, Formulated; D005998:Glycine / Q000097:blood*; D006801:Humans; D007662:Ketosis; D013331:Strychnine / Q000627:therapeutic use*; D014580:Ursodeoxycholic Acid / Q000627:therapeutic use*
D003840:Deoxycholic Acid; D014580:Ursodeoxycholic Acid; D013331:Strychnine; D005998:Glycine