Management of maple syrup urine disease in the peri-operative period.

McCarron, E; McCormack, O; Cronin, T; McGowan, A; Healy, M L; O'Rourke, D; Crushell, E; Ravi, N; Reynolds, J V

McCarron, E; McCormack, O; Cronin, T; McGowan, A; Healy, M L; O'Rourke, D; Crushell, E; Ravi, N; Reynolds, J V.

Afiliación

McCarron E; Trinity Centre, St James's Hospital, Dublin.
McCormack O; Trinity Centre, St James's Hospital, Dublin.
Cronin T; Trinity Centre, St James's Hospital, Dublin.
McGowan A; Trinity Centre, St James's Hospital, Dublin.
Healy ML; Trinity Centre, St James's Hospital, Dublin.
O'Rourke D; Trinity Centre, St James's Hospital, Dublin.
Crushell E; Trinity Centre, St James's Hospital, Dublin.
Ravi N; Trinity Centre, St James's Hospital, Dublin.
Reynolds JV; Trinity Centre, St James's Hospital, Dublin.

Ir Med J ; 106(9): 277-8, 2013 Oct.

Article en En | MEDLINE | ID: mdl-24416851

ABSTRACT

ABSTRACT

Maple syrup urine disease (MSUD) has an incidence of 1125,000 newborns in Ireland. Patients, when fasting, or in a catabolic state build up toxic metabolites leading to progressive neurological dysfunction. We describe the necessary peri-operative management of a patient with MSUD who developed symptomatic gallstones requiring a laparoscopic cholecystectomy.

Asunto(s)

Colecistectomía Laparoscópica; Cálculos Biliares/diagnóstico; Cálculos Biliares/etiología; Cálculos Biliares/cirugía; Enfermedad de la Orina de Jarabe de Arce/complicaciones; Pancreatocolangiografía por Resonancia Magnética; Femenino; Humanos; Pruebas de Función Hepática; Adulto Joven

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Colección: 01-internacional Banco de datos: MEDLINE Asunto principal: Cálculos Biliares / Colecistectomía Laparoscópica / Enfermedad de la Orina de Jarabe de Arce Límite: Adult / Female / Humans Idioma: En Revista: Ir Med J Año: 2013 Tipo del documento: Article

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