Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0030305 (pancreatitis)
 16,014 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Chylomicronemia syndrome (CMS) is a rare disorder characterized by the presence of chylomicrons in the fasting state causing a milky appearance of plasma, eruptive xanthomas, and hepatosplenomegaly; an acute and potentially life threatening complication is severe acute pancreatitis. The underlying defects are inborn errors of metabolism such as deficiencies of lipoprotein lipase (LPL) or apoprotein C-II (apo C-II) as well as familial hypertriglyceridemia. Moreover, CMS can be precipitated when mild hypertriglyceridemia is exacerbated by additional factors such diabetes mellitus, ethanol abuse, or pregnancy. The purpose of the present study was to retrospectively analyze the results of therapeutic plasma exchange (TPE) in 5 patients transferred to our hospital for severe acute pancreatitis due to chylomicronemia syndrome. In a total of 7 TPE sessions, on average 3,286 +/- 247 ml of plasma (i.e., about 1 patient plasma volume) were treated per session. Triglyceride (TG) levels were decreased from 4,972 +/- 2,469 mg/dl on admission to 1,614 +/- 1,276 mg/dl (-70%) after the TPE sessions, and a further decrease was achieved by conservative treatment. Part of the TG reducing effect of the treatment was probably due to heparin induced lipolysis. Acute pancreatitis was resolved in all cases, and 1 pregnant patient delivered without problems at term. In summary, 1 or 2 TPE sessions sufficed to substantially decrease the bulk of triglycerides in acutely exacerbated chylomicronemia syndrome causing a rapid resolution of acute severe pancreatitis.
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PMID:Therapeutic plasma exchange in patients with chylomicronemia syndrome complicated by acute pancreatitis. 1042 20

Chylomicronemia syndrome is a rare disorder characterized by the presence of chylomicrons in the fasting state. An acute and potentially life-threatening complication of chyiomicronemia syndrome is severe acute pancreatitis. We report a case of a 24-year-old primigravida with severe hypertriglyceridemia-induced pancreatitis. We reviewed the clinical course and treatment of hypertriglyceridemia-induced pancreatitis. She was admitted in the 37th week of gestation with severe abdominal pain, which was radiating to the back, and having uterine contractions. Cesarean delivery was performed under spinal anesthesia, and a healthy male infant was born. Intraoperative findings included milky peritoneal fluid collection. Elevated pancreatic enzymes with significant hypertriglyceridemia (10,092 mg/dL) suggesting acute pancreatitis were also found on chemical analysis. The diagnosis of acute pancreatitis was confirmed by computed tomography scan. Treatment with continuous intravenous insulin--glucose, cessation of oral intake, and nasogastric decompression--dramatically decreased the triglyceride levels to 608 mg/dL within five days. She was discharged as symptom free with strict dietary intervention after 10 days. Intravenous insulin is a low-cost and effective alternative treatment in hypertriglyceridemia-induced pancreatitis during pregnancy. To our knowledge, such a high triglyceride level has not previously been reported in pregnancy.
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PMID:Severe hypertriglyceridemia-induced pancreatitis during pregnancy. 1662 82