cgjim 1-1 hypertriglyceridemia and Pregnancy

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Hypertriglyceridemia due to a complete lipoprotein lipase deficiency is a rare disease. The condition is exacerbated by the increased estrogen during pregnancy. This is the case of a woman who required several plasmaphereses during her pregnancy in order to prevent pancreatitis.

Hypertriglyceridemia due to a complete lipoprotein lipase deficiency is a rare disease (the prevalence in Quebec is 200 in 1,000,000). The condition is exacerbated by the increased estrogen during pregnancy, thus increasing the risks of pancreatitis and associated mortality, which may reach 20%.1 Although plasmapheresis has been described as a treatment for acute pancreatitis, its use in preventing this complication of hypertriglyceridemia is rare.2 This is the case of a woman who required several plasmaphereses during her pregnancy in order to prevent pancreatitis.

Case

The patient was a 24-year-old woman of French-Canadian origin, gravida 2 abortus 1, who was diagnosed with an LPL deficiency (double heterozygous mutation for LPL gene G188E and P207) shortly after she was born. Prior to this pregnancy, the patient’s hypertriglyceridemia was well controlled through a low-fat diet. From the start of the pregnancy, the patient was managed by a multidisciplinary team including a nutritionist. A diet containing 12.5 g/day of long-chain fats (normal diet 75 g/day) kept her triglyceride (TG) level below 20 mmol/L until the seventeenth week of pregnancy. Subsequently, her TG level rose to 39 mmol/L, despite a decrease in long-chain fat consumption to 0.5g/day, the use of fenofibrate up to 200 mg, and two 36-hour periods of fasting.

The first plasmapheresis took place at 25 weeks of pregnancy. Since the patient refused any blood products, the plasma exchanges were made with physiological saline and artificial colloids. Her triglyceride level fell to below 20 mmol/L. The effect did not last long, and her TG level reached 71 mmol/L. At the twenty-eighth week of pregnancy, her TG level dropped to 21 mmol/L after two plasmaphereses in three days, and was maintained at that level by four additional plasmaphereses until she gave birth, in the thirty-fourth week of pregnancy. The newborn, a girl weighing 2.5 kg, was healthy. The day after she gave birth, her TG was 16 mmol/L.

At no time did the patient show signs or symptoms of pancreatitis or coagulation or infection disorders. Throughout the pregnancy, the patient’s physical examination was normal. She experienced episodes of hypotension and hypocalcemia during the plasmaphereses, which were quickly corrected. Several times, catheter thromboses required local thrombolysis.

Discussion

This case illustrates how difficult it can be to control TG levels in pregnant patients, despite a very-low-fat diet. The use of fenofibrate is theoretically ineffective in people with LPL deficiency, but a few successful cases during pregnancy have been described.3 We opted for plasmaphereses because, despite the conventional treatment, her triglyceride levels remained dangerously high. Indeed, several cases of acute pancreatitis in pregnancy have been described with TG ≤ 35 mmol/L.4 In our opinion, the morbidity and mortality associated with pancreatitis, both for the baby and the mother, justified the plasmaphereses despite the small amount of current literature supporting our decision.

References

1. Montgomery WH, Miller FC. Pancreatitis and pregnancy. Obstet Gynecol 1970;35:658–664.

2. Dittrich E, Schmaldiesnt S, et coll. Immunoadsorption and plasma exchange in pregnancy. Kidney Blood Press Res 2002;25:232–239.

3. Tsai C E, Brown JA, et coll. Potential of essential fatty acid deficiency with extremely low fat diet in lipoprotein lipase deficiency during pregnancy: a case report. BMC Pregnancy Childbirth 2004;4(1):27.

4. Archard JM, Westell PF. Pancreatitis related to severe acute hypertriglyceridemia during pregnancy treatment with lipoprotein apheresis. Intens Care Med 1991;17:236–237.