Liver transplantation for acute intermittent porphyria:a viable treatment?

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-a...

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Published inHepatobiliary & pancreatic diseases international Vol. 9; no. 1; pp. 93 - 96
Main Authors Dar, Faisal S, Asai, Koji, Haque, Ali Raza, Cherian, Thomas, Rela, Mohamed, Heaton, Nigel
Format Journal Article
LanguageEnglish
Published Singapore 01.02.2010
Subjects
acute
Adult
diseases
erythropoietic
failure
Female
Humans
intermittent
liver
Liver - enzymology
Liver Transplantation
metabolic
porphyria
porphyrias
Porphyrias, Hepatic - surgery
Quality of Life
Retrospective Studies
transplantation
Treatment Outcome
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Summary: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.
Bibliography:Faisal S Dar,Koji Asai,Ali Raza Haque,Thomas Cherian,Mohamed Rela and Nigel Heaton Kings College London School of Medicine at King’s College Hospital,Institute of Liver Studies,King’s College Hospital,Denmark Hill,Camberwell,London SE5 9RS,United Kingdom
33-1391/R
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ISSN:1499-3872