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Two novel CPS1 mutations in a case of carbamoyl phosphate synthetase 1 deficiency causing hyperammonemia and leukodystrophy

BACKGROUND: Carbamoyl phosphate synthetase 1 deficiency (CPS1D) is a rare autosomal recessive disorder of the urea cycle, mostly characterized by hyperammonemia and the concomitant leukodystrophy. The onset of CPS1D can be at any age, and the clinical manifestations are variable and atypical. Geneti...

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Bibliografski detalji
Izdano u:J Clin Lab Anal
Glavni autori: Chen, Xihui, Yuan, Lijuan, Sun, Mao, Liu, Qingbo, Wu, Yuanming
Format: Artigo
Jezik:Inglês
Izdano: John Wiley and Sons Inc. 2018
Teme:
Online pristup:https://ncbi.nlm.nih.gov/pmc/articles/PMC6816849/
https://ncbi.nlm.nih.gov/pubmed/29314318
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1002/jcla.22375
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