Argininosuccinic Aciduria-A Rare Indication for Liver Transplant: Report of Two Cases.
Exp Clin Transplant
; 15(5): 581-584, 2017 Oct.
Article
em En
| MEDLINE
| ID: mdl-26768012
ABSTRACT
Argininosuccinic aciduria is a urea cycle disorder caused by an argininosuccinate lyase enzyme deficiency that ends with nitrogen accumulation as ammonia. Argininosuccinic aciduria patients are at risk for long-term complications including poor neurocognitive outcome, hepatic disease, and systemic hypertension despite strict pharmacologic and dietary therapy. As the liver is the principle site of activity of the urea cycle, it is logical that a liver transplant should be an option, with careful patient selection, even in the absence of cirrhosis. We present 2 pediatric argininosuccinic aciduria patients who underwent a living-donor liver transplant from their mothers. After the liver transplant, the general well-being of the patients and their quality of life improved significantly. Liver transplant should be an option for argininosuccinic aciduria patients to prevent further neurologic deterioration and improve the patient's quality of life.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Transplante de Fígado
/
Acidúria Argininossuccínica
/
Doença Hepática Terminal
Tipo de estudo:
Diagnostic_studies
/
Etiology_studies
Aspecto:
Patient_preference
Limite:
Child, preschool
/
Humans
/
Male
Idioma:
En
Revista:
Exp Clin Transplant
Assunto da revista:
TRANSPLANTE
Ano de publicação:
2017
Tipo de documento:
Article
País de afiliação:
Turquia