Pancreatitis, familial hypertriglyceridemia, and pregnancy

Interrelationships between pregnancy, hypertriglyceridemia, and pancreatitis were assessed in three women with familial hypertriglyceridemia. One subject had known familial hypertriglyceridemia, familial type V hyperlipoproteinemia, prior to conception. In this woman a progressive increase in triglyceride levels to more than 3,000 mg/dl during the first two trimesters required dietary intervention and hospitalization at 28 weeks' gestation. Use of an isocaloric National Institutes of Health type V diet reduced triglyceride levels to less than 900 mg/dl; the pregnancy was uneventful with term delivery of a healthy neonate. The familial hypertriglyceridemia was covert in the other two women until term. In one subject, subsequently shown to have familial type V, acute hemorrhagic pancreatitis with a pancreatic pseudocyst, shock, and hypocalcemia developed at 39 weeks' gestation; the neonate was safely delivered, and the mother survived. In the second, entirely asymptomatic subject, triglyceride levels greater than 5,000 mg/dl were discovered incidentally at term cesarean section during delivery of a healthy neonate. With a fat restricted diet, plasma triglyceride levels abruptly fell post partum to less than 500 mg/dl, and subsequent studies revealed familial type III hyperlipoproteinemia. Routine quantitation of plasma cholesterol and triglyceride levels or simple visual examination of fasting plasma for triglyceride-induced opacity or "milky" appearance should be done during early pregnancy. This would allow the obstetrician to identify women with severe familial hypertriglyceridemia prior to the superimposition of the physiologic hyperlipidemia of pregnancy upon familial hypertriglyceridemia with resultant, and often catastrophic, acute pancreatitis.