Intractable metabolic acidosis in a child with propionic acidemia undergoing liver transplantation: a case report / 대한마취과학회지
Korean Journal of Anesthesiology
;
: 257-261, 2013.
Artigo
em Inglês
| WPRIM
| ID: wpr-78998
ABSTRACT
Propionic acidemia (PA) is a rare autosomal recessive disorder of metabolism caused by deficient activity of the mitochondrial enzyme propionyl-CoA carboxylase. The clinical manifestations are metabolic acidosis, poor feeding, lethargy, vomiting, osteoporosis, neurological dysfunction, pancytopenia, developmental retardation and cardiomyopathy. Liver transplantation has recently been considered as one of the treatment options for patients with PA. This case report describes several anesthetic considerations for patients with PA undergoing liver transplantation. Understanding the patient's status and avoiding events that may precipitate metabolic acidosis are important for anesthetic management of patients with PA. In conclusion, anesthesia should be focused on minimizing the severity of metabolic acidosis with following considerations (1) maintaining optimal tissue perfusion by avoiding hypotension, (2) preventing hypoglycemia, and (3) providing bicarbonate to compensate for the acidosis.
Texto completo:
DisponíveL
Índice:
WPRIM (Pacífico Ocidental)
Assunto principal:
Osteoporose
/
Pancitopenia
/
Perfusão
/
Acidose
/
Vômito
/
Acil Coenzima A
/
Transplante de Fígado
/
Metilmalonil-CoA Descarboxilase
/
Dietilpropiona
/
Letargia
Limite:
Criança
/
Humanos
Idioma:
Inglês
Revista:
Korean Journal of Anesthesiology
Ano de publicação:
2013
Tipo de documento:
Artigo
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