A Case of Lipoprotein Lipase Deficiency which Became Manifest in the Third Trimester of Pregnancy

A patient with lipoprotein lipase deficiency which became manifest in the third trimester of pregnancy was reported. A 28-year-old woman, gravida 2, para 2, with a pregnancy of 37 weeks gestational age was admitted to the hospital because of high fever, abdominal pain, nausea and vomiting. On admission, marked tenderness in the upper abdomen and ascites were noted. Serum amylase, elastase 1 and triglyceride were markedly elevated to 2, 240u, 3, 200ng/dl and 11, 360mg/dl, respectively. A diagnosis of acute pancreatitis secondary to hyperchylomicronemia was made. A cesarean section delivery resulted in the birth of a healthy female infant of 2, 990g on the second hospital day, when treatments with total parenteral nutrition, antibiotics and Gabexate (FOY) were begun. The patient followed a favorable course after the delivery. Pancreatitis and hyperchylomicronemia were subsided in one month. The serum level of triglyceride declined to a normal value in 45 days concomitant with a lowering of apolipoproteins B, C-II, C-III and E. On 75th hospital day the patient was discharged. Lipoprotein lipase activities in post heparin plasma measured one month and seven months after the onset were decreased to 25% of the normal value. A fat load test done one year later disclosed a slow clearance of triglyceride in vivo. However, even the patient had been taking 50g of fat per day, the fasting serum level of triglyceride remained normal for 12 months.The transient hyperchylomicronemia may be attributable to a partial LPL deficiency which became manifest during a course of pregnancy.