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Nonketotic hyperglycinemia. Effects of therapy with strychnine.
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- Author(s): Warburton D; Boyle RJ; Keats JP; Vohr B; Peuschel S; Oh W
- Source:
American journal of diseases of children (1960) [Am J Dis Child] 1980 Mar; Vol. 134 (3), pp. 273-5.
- Publication Type:
Case Reports; Journal Article
- Language:
English
- Additional Information
- Source:
Publisher: American Medical Association Country of Publication: United States NLM ID: 0370471 Publication Model: Print Cited Medium: Print ISSN: 0002-922X (Print) Linking ISSN: 0002922X NLM ISO Abbreviation: Am J Dis Child Subsets: MEDLINE
- Publication Information:
Publication: Chicago Il : American Medical Association
Original Publication: Chicago, Ill. : American Medical Association, c1960-1993.
- Subject Terms:
- Abstract:
Nonketotic hyperglycinemia was diagnosed in identical twins with lethargy and respiratory failure in the neonatal period. Therapy with strychnine (0.32 mg/kg/day) resulted in great reductions in CSF and plasma glycine levels and improvement in muscle tone, respiration, and ability to suck. Myoclonic seizures were partially controlled by therapy with clonazepam. Higher dosages of strychnine (up to 2.0 mg/kg/day) were needed to counteract the increased lethargy following administration of clonazepam. At 5 months of age, the twins' developmental performance remained below the 1-month level despite adequate somatic growth. The twins died suddenly of status epilepticus at 6 1/2 months of age.
- Accession Number:
5PE9FDE8GB (Clonazepam)
H9Y79VD43J (Strychnine)
TE7660XO1C (Glycine)
- Publication Date:
Date Created: 19800301 Date Completed: 19800523 Latest Revision: 20190716
- Publication Date:
20240829
- Accession Number:
10.1001/archpedi.1980.02130150031008
- Accession Number:
6767393
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