Potential of essential fatty acid deficiency with extremely low fat diet in lipoprotein lipase deficiency during pregnancy: A case report

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Potential of essential fatty acid deficiency with extremely low fat diet in lipoprotein lipase deficiency during pregnancy: A case report

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dc.contributor.author Tsai, Elaine C. en_US
dc.contributor.author Brown, Judy A. en_US
dc.contributor.author Veldee, Megan Y. en_US
dc.contributor.author Anderson, Gregory J. en_US
dc.contributor.author Chait, Alan en_US
dc.contributor.author Brunzell, John D. en_US
dc.date.accessioned 2010-05-06T20:08:15Z
dc.date.available 2010-05-06T20:08:15Z
dc.date.issued 2004 en_US
dc.identifier.citation Tsai E, Brown J, Veldee M, et al. Potential of essential fatty acid deficiency with extremely low fat diet in lipoprotein lipase deficiency during pregnancy: A case report. BMC Pregnancy and Childbirth. 2004;4(1):27. en_US
dc.identifier.other 10.1186/1471-2393-4-27 en_US
dc.identifier.uri http://www.biomedcentral.com/1471-2393/4/27 en_US
dc.identifier.uri http://hdl.handle.net/1773/15864
dc.description.abstract Background: Pregnancy in patients with lipoprotein lipase deficiency is associated with high risk of maternal pancreatitis and fetal death. A very low fat diet (less than 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 less than 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 presentation: A 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 less than 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. Conclusions: An 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. en_US
dc.description.sponsorship ECT is supported by grants from the American Diabetes Association and Seattle Epidemiologic Research and Information Center. en_US
dc.language.iso en_US en_US
dc.title Potential of essential fatty acid deficiency with extremely low fat diet in lipoprotein lipase deficiency during pregnancy: A case report en_US
dc.type Article en_US


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