Familial Chylomicronemia Syndrome-Induced Acute Necrotizing Pancreatitis during Pregnancy

• Published in: Revista Brasileira de ginecologia e obstetrícia Vol. 43; no. 3; pp. 220 - 224
• Main Authors: Coronado Arroyo, Julia Cristina, Concepción Zavaleta, Marcio José, García Villasante, Eilhart Jorge, Kcomt Lam, Mikaela, Concepción Urteaga, Luis Alberto, Zavaleta Gutiérrez, Francisca Elena
• Format: Journal Article
• Language: English; Portuguese
• Published: Rua do Matoso 170, Rio de Janeiro, RJ, CEP 20270-135, Brazil Thieme Revinter Publicações Ltda 01.03.2021; Federação Brasileira das Sociedades de Ginecologia e Obstetrícia
• Subjects: Adult; APACHE; Case Report; Diagnosis, Differential; familial chylomicronemia; Female; Humans; Hyperlipoproteinemia Type I - complications; Hyperlipoproteinemia Type I - diagnosis; Hyperlipoproteinemia Type I - diagnostic imaging; necrotizing pancreatitis; OBSTETRICS & GYNECOLOGY; Pancreatitis, Acute Necrotizing - complications; Pancreatitis, Acute Necrotizing - diagnosis; Pancreatitis, Acute Necrotizing - diagnostic imaging; Pregnancy; Pregnancy Complications - diagnosis; Pregnancy Complications - diagnostic imaging; Prenatal Diagnosis; necrotizing pancreatitis; familial chylomicronemia; pregnancy
• ISSN: 0100-7203; 1806-9339; 1806-9339
• DOI: 10.1055/s-0040-1722173

Abstract Acute pancreatitis is a rare condition in pregnancy, associated with a high mortality rate. Hypertriglyceridemia represents its second most common cause. We present the case of a 38-year-old woman in the 24 th week of gestation with a history of hypertriglyceridemia and recurrent episodes of pancreatitis. She was admitted to our hospital with acute pancreatitis due to severe hypertriglyceridemia. She was stabilized and treated with fibrates. Despite her favorable clinical course, she developed a second episode of acute pancreatitis complicated by multi-organ dysfunction and pancreatic necrosis, requiring a necrosectomy. The pregnancy was ended by cesarean section, after which three plasmapheresis sessions were performed. She is currently asymptomatic with stable triglyceride levels. Acute pancreatitis due to hypertriglyceridemia represents a diagnostic and therapeutic challenge in pregnant women, associated with serious maternal and fetal complications. When primary hypertriglyceridemia is suspected, such as familial chylomicronemia syndrome, the most important objective is preventing the onset of pancreatitis.