Transient remnant removal disease in acute fatty liver of pregnancy.
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.
Wetzka
Birgit
B
Department of Obstetrics and Gynecology, University of Freiburg, Freiburg, Germany.
Hoffmann
Michael M
MM
Friedrich
Isolde
I
Baumstark
Manfred W
MW
Zahradnik
Hans Peter
HP
März
Winfried
W
Winkler
Karl
K
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
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