国际肝胆胰疾病杂志（英文版）
國際肝膽胰疾病雜誌（英文版）
국제간담이질병잡지（영문판）
Hepatobiliary & Pancreatic Diseases International
2010年
1期
93-96
,共4页
liver transplantation%acute intermittent porphyria%erythropoietic porphyria%metabolic liver diseases%acute liver failure%porphyrias
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. <br> METHOD:Two cases of LT for AIP were identiifed retro-spectively from a prospectively maintained LT database. <br> RESULT:LT was successful with resolution of AIP in two patients who suffered from repeated acute attacks. <br> CONCLUSION:LT can correct the underlying metabolic abnormality in AIP and improves quality of life signiifcantly.
