Refeeding syndrome in a young woman with argininosuccinate lyase deficiency

Autor: M. Stuy, G.-F. Chen, J.M. Masonek, B.F. Scharschmidt
Jazyk: angličtina
Rok vydání: 2015
Předmět:
Zdroj: Molecular Genetics and Metabolism Reports, Vol 4, Iss C, Pp 6-8 (2015)
Druh dokumentu: article
ISSN: 2214-4269
DOI: 10.1016/j.ymgmr.2015.03.008
Popis: A severely chronically protein and calorie restricted young woman with argininosuccinate lyase deficiency developed transient refeeding syndrome (RFS) and hyperammonemia after modest diet liberalization following initiation of glycerol phenylbutyrate (GPB). The patient required IV supportive care and supplementation with potassium, magnesium and calcium. She is now doing well on GPB and an appropriate maintenance diet. Susceptibility to RFS should be considered in chronically nutritionally restricted patients with metabolic disorders after liberalization of diet.
Databáze: Directory of Open Access Journals