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). CASE REPORT: 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. STUDY DESIGN: 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.

Triglyceride-rich lipoproteins are associated with hypertension in preeclampsia.

Disorders of the lipoprotein metabolism are a major cause of endothelial dysfunction that may result in hypertension and proteinuria, clinical hallmarks of preeclampsia (PE). Lipoproteins and low-density lipoprotein (LDL) subfractions were investigated in 15 women with severe PE and compared with 23 women with a normal course of pregnancy. Compared with normal pregnancy, in PE apolipoprotein (apo)B in very low-density lipoprotein was increased by 76% (P = 0.008), and the triglyceride content of intermediate dense lipoproteins (IDL) was increased by 51% (P < 0.001); cholesterol and apoB in LDL were decreased by 26% (P = 0.005) and 23% (P = 0.016), respectively. Although not significant, the LDL profile was dominated by the most buoyant LDL-1. ApoB in the most dense LDL (dLDL), namely LDL-5 and LDL-6, was significantly decreased by 49% (P < 0.001) and 55% (P < 0.001), respectively. Diastolic blood pressure was positively correlated with the triglyceride content of IDL (r = 6.31; P < 0.001 and r = 0.352; P = 0.033 by partial correlation controlling for the presence or absence of PE) and negatively correlated with the concentration of apoB in dLDL (r = -0.500; P = 0.002). In addition, IDL triglycerides correlated negatively with infant birth weight percentile (r = -0.373; P = 0.027) and positively with proteinuria (r = 0.430; P = 0.014). Low birth weight was associated with high IDL triglycerides and low rather than high concentrations of dLDL. Triglyceride-rich remnants are known to cause endothelial dysfunction. Because the triglyceride content of IDL was positively correlated with elevated blood pressure and proteinuria, triglyceride-rich remnant lipoproteins might contribute to the pathophysiology of PE.