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hyperargininemia/albumin
Veza se sprema u međuspremnik
3 rezultatima
Anesthetic management of a patient with arginase deficiency undergoing liver transplantation.
Samo registrirani korisnici mogu prevoditi članke
Prijava Registriraj se
A 20-year-old female underwent orthotopic liver transplantation for arginase deficiency, a urea cycle disorder. A hyperammonemic state was prevented by the administration of lipid and carbohydrate substrate and avoidance of protein loading (including human albumin) and prolonged fasting. Caval
Coagulation Disturbances in Patients with Argininemia.
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Prijava Registriraj se
BACKGROUND
Argininemia is an autosomal recessive urea cycle disorder (UCD). Unlike other UCD, hyperammonemia is rarely seen. Patients usually present in childhood with neurological symptoms. Uncommon presentations like neonatal cholestasis or cirrhosis have been reported. Although transient
Sodium phenylbutyrate improved the clinical state in an adult patient with arginase 1 deficiency.
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Prijava Registriraj se
An adult female patient was diagnosed with arginase 1 deficiency (ARG1-D) at 4 years of age, and had been managed with protein restriction combined with sodium benzoate therapy. Though the treatment was successful in ameliorating hyperammonemia, hyperargininemia persisted. After being under control
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