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615220
Enf. del Ciclo de la Urea
Description
Mind Map on Enf. del Ciclo de la Urea, created by adahemy.tm on 09/03/2014.
Mind Map by
adahemy.tm
, updated more than 1 year ago
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adahemy.tm
over 10 years ago
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Resource summary
Enf. del Ciclo de la Urea
Definición
Defecto de enzimas
Catalizan ciclo de la urea
El ciclo no se completa
Se acumula NH3
Hiperamoniemia
Clínica
HAR
Excepto def. OTC
Vómitos
Estupor
Coma
Def. NAGS, CPS y OTC
Hombres
Inicio a pocos días
AS, AL, ASA
Presentación tardía
Expresión
Introd. alimentos ricos
Proteínas
Aumento
Transaminasas
Hiperamoniemia
Afectación protrombina
Pruebas y Examenes
AA anormales en sangre y orina
Nivel anormal ac. Orótico
> Amoniaco en sangre
Nivel anormal ac. en sangre
Tratamiento
Restricción de la proteína de la dieta
Hemodiálisis
Evitar edema cerebral
Mejorar producción ornitina
Arginina 200-700 mg/kg/dia
Control niveles de carnitina
Bajos, carnitina oral
Suplementar aporte calórico
Transplante hepático
Tipos
Aciduria arginosuccinica
Def. Arginasa
Def CFS
Citrulinemia
Def NAGS
Def OTC
Media attachments
CICLO_UREA (image/png)
coma (image/jpg)
vitaminas.gif (image/gif)
descarga (image/jpg)
medicamentos (image/jpg)
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