AFLP is a rare but life-threatening disease that occurs primarily in the third trimester,characterized by rapid progress,so far , there is no report about recovery before partus. AFLP has been more frequently reported in association with fatty acide oxidation disorders.Recent research indicate that fetal deficiency of the long chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) seems to predispose women to AFLP.Patients of AFLP often present with nonspecific symptoms,the main differential diagnoses for AFLP are HELLP syndrome,Fulminant Hepatitis of Pregnancy and Intrahepatic Cholestasis of Pregnancy.Historically,liver biopsy is regarded as gold standard of diagnosis.Nowadays,with the improvement of acquaintance about AFLP,liver biopsy is not mandatory for diagnosis and differential diagnosis in most cases if clinical and biochemical findings for AFLP are available.In severe cases,the patient can died of multisystem involvement and disseminated intravascular coagulation.Early diagnosis,expeditious delivery and intensive supportive care are essential to optimal maternal-fatal outcomes and remain as the mainstay treatment for AFLP.The mortality of AFLP was lower with promot diagnosis and treatment,The data Morgan etal statisticsed indicates that the mortality of AFLP is one in 16000 to 15000 pregnancies.Present research reveals that 25% fatal LCHAD deficiency are associated with neonatal hereditary metabolic disease and the recurrence in subsequent pregnancies is unknown,high-risk pregnancies should be warned of gene detection and long-term following, along with the newborn infants of AFLP. |