title: Transient remnant removal disease in acute fatty liver of pregnancy.
abstract: OBJECTIVE
  To elucidate the potential role of an altered lipid metabolism in the pathophysiology of acute fatty liver of pregnancy (AFLP).
  
  
  METHODS
  We report on two otherwise healthy women in the 34th gestational week who presented with symptoms of AFLP. Besides characteristic symptoms like nausea, abdominal pain, highly elevated serum amino transferase levels, and increased creatinine concentrations, the patients' clotting system showed consumption and/or decreased synthesis of coagulation factors. Pregnancies were terminated by elective cesarean section because of worsening symptoms. Blood tests normalized quickly and both the mothers and their baby boys could be dismissed in healthy condition.
  
  
  METHODS
  Blood samples were collected shortly before delivery and 5, 15, 70, and 110 days afterwards. Lipids and apolipoproteins (apo) were analyzed in whole plasma as well as in very low density, intermediate density, low density (LDL), and high density lipoprotein. Total LDL was further separated into 6 LDL subfractions by equilibrium density ultracentrifugation.
  
  
  RESULTS
  Before delivery, the LDL subfraction pattern was characterized by the virtual absence of intermediate and most dense LDL. Lipoprotein electrophoresis showed the presence of beta-migrating VLDL. Within days after delivery, the distribution of apoB-containing lipoproteins returned to normal. Genetic variations of apoE, lipoprotein lipases, and the long-chain 3-hydroxyacyl-coenzyme A dehydrogenase were not detected in any of the patients.
  
  
  CONCLUSIONS
  The lipoprotein metabolism in the acute phase of AFLP was reminiscent of hepatic lipase deficiency, a disorder characterized by impaired removal of lipoprotein remnants. As these triglyceride-rich particles cause endothelial dysfunction, they may contribute to the pathophysiology of AFLP.
authors.affiliation: Department of Obstetrics and Gynecology, University of Freiburg, Freiburg, Germany.
authors.forename: Birgit
authors.initials: B
authors.lastname: Wetzka
authors.forename: Michael M
authors.initials: MM
authors.lastname: Hoffmann
authors.forename: Isolde
authors.initials: I
authors.lastname: Friedrich
authors.forename: Manfred W
authors.initials: MW
authors.lastname: Baumstark
authors.forename: Hans Peter
authors.initials: HP
authors.lastname: Zahradnik
authors.forename: Winfried
authors.initials: W
authors.lastname: März
authors.forename: Karl
authors.initials: K
authors.lastname: Winkler
mesh_terms: D000208:Acute Disease; D000328:Adult; D016632:Apolipoprotein A-I / Q000378:metabolism; D001055:Apolipoproteins B / Q000378:metabolism; D001057:Apolipoproteins E / Q000235:genetics / Q000378:metabolism; D015415:Biomarkers / Q000097:blood; D005234:Fatty Liver / Q000378:metabolism* / Q000503:physiopathology*; D005260:Female; D005838:Genotype; D006801:Humans; D008071:Lipoprotein Lipase / Q000378:metabolism; D008074:Lipoproteins / Q000378:metabolism; D008075:Lipoproteins, HDL / Q000378:metabolism; D053419:Lipoproteins, IDL; D008077:Lipoproteins, LDL / Q000378:metabolism; D008079:Lipoproteins, VLDL / Q000378:metabolism; D010641:Phenotype; D011247:Pregnancy; D011248:Pregnancy Complications / Q000378:metabolism* / Q000503:physiopathology*; D011263:Pregnancy Trimester, Third / Q000378:metabolism; D014280:Triglycerides / Q000378:metabolism
chemical_list: D016632:Apolipoprotein A-I; D001055:Apolipoproteins B; D001057:Apolipoproteins E; D015415:Biomarkers; D008074:Lipoproteins; D008075:Lipoproteins, HDL; D053419:Lipoproteins, IDL; D008077:Lipoproteins, LDL; D008079:Lipoproteins, VLDL; D014280:Triglycerides; D008071:Lipoprotein Lipase