Liver transplantation for acute intermittent porphyria: a viable treatment?
Hepatobiliary Pancreat Dis Int
; 9(1): 93-6, 2010 Feb.
Article
en En
| MEDLINE
| ID: mdl-20133237
BACKGROUND: Acute intermittent porphyria (AIP) is the most common hepatic porphyria. Its clinical presentation includes severe disabling and life-threatening neurovisceral symptoms and acute psychiatric symptoms. These symptoms result from the overproduction and accumulation of porphyrin precursors, 5-aminoleuvulinic acid (ALA) and porphobilinogen (PBG). The effect of medical treatment is transient and is not effective once irreversible neurological damage has occurred. Liver transplantation (LT) replaces hepatic enzymes and can restore normal excretion of ALA and PBG and prevent acute attacks. METHOD: Two cases of LT for AIP were identified retrospectively from a prospectively maintained LT database. RESULT: LT was successful with resolution of AIP in two patients who suffered from repeated acute attacks. CONCLUSION: LT can correct the underlying metabolic abnormality in AIP and improves quality of life significantly.
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Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Trasplante de Hígado
/
Porfirias Hepáticas
Tipo de estudio:
Observational_studies
/
Prognostic_studies
Aspecto:
Patient_preference
Límite:
Adult
/
Female
/
Humans
Idioma:
En
Revista:
Hepatobiliary Pancreat Dis Int
Asunto de la revista:
GASTROENTEROLOGIA
Año:
2010
Tipo del documento:
Article
País de afiliación:
Reino Unido
Pais de publicación:
Singapur