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hyperargininemia/carbohydrate

Sábháiltear an nasc chuig an gearrthaisce
AiltTrialacha cliniciúlaPaitinní
3 torthaí

Treatment of hyperargininaemia due to arginase deficiency with a chemically defined diet.

Ní féidir ach le húsáideoirí cláraithe ailt a aistriú
Logáil Isteach / Cláraigh
A brother and sister aged 11 and 17 years have been reported previously to have hyperargininaemia and arginase deficiency: they were treated with a semi-synthetic diet consisting of fat, carbohydrate, minerals, vitamins and essential amino acids in amounts equivalent to 0.55-0.65 g protein kg-1

Arginase Deficiency

Ní féidir ach le húsáideoirí cláraithe ailt a aistriú
Logáil Isteach / Cláraigh
Clinical characteristics: Arginase deficiency in untreated individuals is characterized by episodic hyperammonemia of variable degree that is infrequently severe enough to be life threatening or to cause death. Most commonly, birth and early childhood are normal.

Anesthetic management of a patient with arginase deficiency undergoing liver transplantation.

Ní féidir ach le húsáideoirí cláraithe ailt a aistriú
Logáil Isteach / Cláraigh
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
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