妊娠期急性胰腺炎和家族性乳糜泻综合征:病例报告和文献综述

Batoul Jaafar, Jessica Abou Chaaya, Shahed Ammar, Ibrahim Salti
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摘要

急性胰腺炎很少发生在妊娠期,而高甘油三酯血症是导致妊娠期急性胰腺炎的第四大原因。高甘油三酯血症(家族性乳糜微粒血症综合征是其中最严重的一种)是导致妊娠期胰腺炎的四大主要原因之一。全血浆置换术(TPE)作为一种治疗和预防手段,被认为是一种有效而安全的干预措施。一名患有 FCS 的 22 岁女性在妊娠第 21 周时出现急性高甘油三酯血症性胰腺炎。尽管接受了药物治疗,但由于血清甘油三酯水平失控,她在两周的随访中开始服用 TPE。在这些疗程中,甘油三酯从 55.0% 降至 77.5%。尽管采取了这些干预措施,胰腺炎还是在第 34 周复发。在胎儿心率下降后,进行了紧急剖腹产。产后甘油三酯下降了 57%,但仍高于 1,000 mg/dl。妊娠期的 FCS 难以控制,而且经常对各种药物治疗无效。TPE 可以帮助延长妊娠期,但并不是一种明确的治疗方法。治疗妊娠期高甘油三酯血症的新疗法有待更多的安全性测试。
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Acute pancreatitis in pregnancy and familial chylomicronemia syndrome: case report and literature review
Acute pancreatitis rarely occurs in pregnancy, with hypertriglyceridemia being the fourth leading cause during pregnancy. Hypertriglyceridemia, of which Familial Chylomicronemia Syndrome is the most severe form, ranks among the four principal causes of pancreatitis in pregnancy. Total Plasma exchange (TPE) has been found to be an effective and safe intervention both as a therapeutic and a prophylactic act. A 22-year-old female with FCS presented at the 21st week of gestation with acute hypertriglyceridemia pancreatitis. Despite medical management, she was then started on TPE at the two-week follow-up after serum triglyceride level was out of control. The triglyceride dropped from 55.0 % to 77.5 % during these sessions. Despite these interventions, pancreatitis recurred in week 34. An emergency C-section was carried out after a drop in the fetal heart rate. Postpartum triglycerides dropped by 57 % but remained above 1,000 mg/dl. FCS is difficult to manage during pregnancy, and it frequently fails to respond to various pharmacologic lines. TPE can help prolong a pregnancy, but it is not a definite treatment. Novel therapies for hypertriglyceridemia in pregnancy await additional safety testing.
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