Transient nonketotic hyperglycinemia: Two case reports and literature review

Aliefendioglu D., Aslan A., Coskun T., DURSUN A., Cakmak F., Kesimer M.

PEDIATRIC NEUROLOGY, vol.28, no.2, pp.151-155, 2003 (SCI-Expanded) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 28 Issue: 2
  • Publication Date: 2003
  • Doi Number: 10.1016/s0887-8994(02)00501-5
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Page Numbers: pp.151-155
  • Hacettepe University Affiliated: Yes


Transient nonketotic hyperglycinemia is characterized by clinical and biochemical findings similar to those seen in classic nonketotic hyperglycinemia. Abnormalities in amino acids are partially or completely resolved in a period ranging from days to months. Almost all patients with the classic form of nonketotic hyperglycinemia survive with severe neurologic sequela, whereas most of the patients with the transient form exhibit normal development. Therefore, distinguishing the transient form of nonketotic hyperglycinemia from classic nonketotic hyperglycinemia is important to predict outcome of the patients.