Abstract
BackgroundPregnancy in patients with lipoprotein lipase deficiency is associated with high risk of maternal pancreatitis and fetal death. A very low fat diet (< 10% of calories) is the primary treatment modality for the prevention of acute pancreatitis, a rare but potentially serious complication of severe hypertriglyceridemia. Since pregnancy can exacerbate hypertriglyceridemia in the genetic absence of lipoprotein lipase, a further reduction of dietary fat intake to < 1–2% of total caloric intake may be required during the pregnancy, along with the administration of a fibrate. It is uncertain if essential fatty acid deficiency will develop in the mother and fetus with this extremely low fat diet, or whether fibrates will cross the placenta and concentrate in the fetus.Case presentationA 23 year-old gravida 1 woman with primary lipoprotein lipase deficiency was seen at 7 weeks of gestation in the Lipid Clinic for management of severe hypertriglyceridemia that had worsened with pregnancy. While on her habitual fat intake of 10% of total calories, her pregnancy resulted in an exacerbation of the hypertriglyceridemia, which prompted further restriction of fat intake to < 2% of total calories, as well as administration of gemfibrozil at a lower than average dose. The level of gemfibrozil, as the active metabolite, in the venous and arterial fetal cord blood was within the expected therapeutic range for adults. The clinical signs and a biomarker of essential fatty acid deficiency, namely the ratio of 20:3 [n-9] to 20:4 [n-6] fatty acids, were closely monitored throughout her pregnancy. Despite her extremely low fat diet, the levels of essential fatty acids measured in the mother and in the fetal blood immediately postpartum were normal. Normal essential fatty acid levels may have been achieved by the topical application of sunflower oil.ConclusionsAn extremely low fat diet in combination with topical sunflower oil and gemfibrozil administration was safely implemented in pregnancy associated with the severe hypertriglyceridemia of lipoprotein lipase deficiency.
Highlights
Pregnancy in patients with lipoprotein lipase deficiency is associated with high risk of maternal pancreatitis and fetal death
Primary lipoprotein lipase (LPL) deficiency is a rare autosomal recessive disorder characterized by severe hypertriglyceridemia, due to the accumulation in plasma of chylomicrons and very low density lipoproteins (VLDL) that result from the absence of LPL activity [1]
Would essential fatty acid (EFA) deficiency develop in the mother and fetus as a result of severe maternal dietary fat restriction? Second, would gemfibrozil cross the placenta and concentrate in the fetus? The strategies utilized to prevent EFA deficiency and the fetal nutritional information obtained from studies at birth will address these questions and concerns
Summary
Children with primary LPL deficiency can be effectively managed on fat-restricted diets and grow normally into adulthood They can present with extreme elevation of TG levels with serious acute pancreatitis. Analysis of the parent compound and metabolites did not detect excessive accumulation in the fetal cord circulation in contrast to the reports in animal models While this observation needs to be independently confirmed, adverse drug effects in the infants born to mothers on gemfibrozil or other fibrates have not been reported. The patient developed pancreatitis during her pregnancy, the use of an extremely low fat diet together with a fibrate helped limit the increase in the triglycerides, and her pancreatitis was neither life threatening nor adversely affected fetal survival. Http://www.biomedcentral.com/1471-2393/4/27 eridemia, where EFA deficiency and recurrent pancreatitis are major concerns
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