Acute Pancreatitis due to Hyperchylomicronemia
Chylomicronemia-induced pancreatitis is a potentially life-threatening condition that warrants emergency treatment. Most patients with this problem have an underlying genetic disorder of triglyceride metabolism on which is superimposed one or more forms o
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Acute Pancreatitis due to Hyperchylomicronemia P. Barton Duell
Précis 1. Clinical setting: severe abdominal pain, nausea, and emesis 2. Diagnosis: a. History: Usually recent onset of severe abdominal pain, often after a period of increased intake of dietary fat and/or alcohol, or uncontrolled diabetes. Episodes also can be provoked by the recent addition of triglyceride-raising medications such as oral estrogen therapy, beta-adrenergic blockers, or corticosteroids in patients with baseline hypertriglyceridemia. Some patients may have a history of recurrent bouts of abdominal pain and/or transient outbreaks of small pimple-like lesions (i.e., eruptive xanthomas) on their trunk and extremities. b. Physical Examination: Abdominal tenderness often with peritoneal signs, eruptive xanthomas (sometimes present only on the buttocks), lipemia retinalis. Blood may appear lipemic ex vivo. Fever, diaphoresis, hypotension may be present depending on the severity of illness. c. Laboratory values: Plasma triglyceride concentrations >2,500–3,000 mg/dL are usually required to cause acute pancreatitis, but the triglyceride concentration can drop by more than 1,000 mg/dL per day if the patient is NPO with nausea and emesis for 1–2 days prior to presentation. Whole blood will appear lipemic like tomato soup; plasma or serum will have the appearance of whole milk or cream depending on the severity of hypertriglyceridemia. Pseudohyponataremia, pseudohemogloginemia, pseudohypoxemia, pseudohyperbilirubinemia, falsely normal amylase concentrations, and thyroid hormone abnormalities may be found during chylomicronemia. Clarification of
P.B. Duell, M.D. (*) Division of Endocrinology, Diabetes, and Clinical Nutrition, Oregon Health and Science University, 3181 SW Sam Jackson Park Road, L465, Portland, OR 97086, USA e-mail: [email protected]
L. Loriaux (ed.), Endocrine Emergencies: Recognition and Treatment, Contemporary Endocrinology 74, DOI 10.1007/978-1-62703-697-9_21, © Springer Science+Business Media New York 2014
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plasma or serum by ultracentrifugation will correct most of these abnormalities. Hyperglycemia and hypothyroidism need to be identified and treated. Other laboratory abnormalities related to complications of pancreatitis are often seen (e.g., hypocalcemia, hypoalbuminemia, anemia). d. Imaging: Abdominal CT imaging is useful for diagnosis and staging of pancreatitis regardless of the etiology. 3. Treatment—The primary treatment for chylomicronemia-induced pancreatitis involves making the patient NPO, giving fluid resuscitation and analgesia as needed, and waiting for plasma chylomicrons to be cleared by zero order kinetics. Plasmapheresis is sometimes considered, but the benefit is unproven. Intravenous fat emulsions should be avoided. If hyperglycemia is present, an insulin drip is indicated to reduce free fatty acid release from tissues, decrease the glucose substrate for hepatic triglyceride synthesis, and restore lipoprotein lipase (LPL) activity. Patients with hypothryoidism need to receive thyroid h
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