Liver transplantation for acute intermittent porphyria:a viable treatment?

作     者：Faisal S Dar Koji Asai Ali Raza Haque Thomas Cherian Mohamed Rela Nigel Heaton 

作者机构：Kings College London School of Medicine at King's College HospitalInstitute of Liver StudiesKing's College Hospital 

出 版 物：《Hepatobiliary & Pancreatic Diseases International》 (国际肝胆胰疾病杂志（英文版）)

年 卷 期：2010年第9卷第1期

页      面：93-96页

核心收录：

学科分类：1002[医学-临床医学] 100210[医学-外科学(含：普外、骨外、泌尿外、胸心外、神外、整形、烧伤、野战外)] 10[医学] 

基　　金：Medical Research Council [G0600698B] Funding Source: researchfish 

主　　题：liver transplantation acute intermittent porphyria erythropoietic porphyria metabolic liver diseases acute liver failure porphyrias 

摘      要：BACKGROUND:Acute intermittent porphyria (AIP) is the most common hepatic *** clinical presentation includes severe disabling and life-threatening neurovisceral symptoms and acute psychiatric *** 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 *** transplantation (LT) replaces hepatic enzymes and can restore normal excretion of ALA and PBG and prevent acute ***:Two cases of LT for AIP were identified retrospectively from a prospectively maintained LT ***:LT was successful with resolution of AIP in two patients who suffered from repeated acute ***:LT can correct the underlying metabolic abnormality in AIP and improves quality of life significantly.