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Acute pancreatitis secondary to severe hypertriglyceridaemia in a patient with type 1a glycogen storage disease: Emergent use of plasmapheresis

Lookup NU author(s): Dr Nicola Leech


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© 2017, Society for the Study of Inborn Errors of Metabolism (SSIEM). Acute pancreatitis is a well-recognised complication of hypertriglyceridaemia. High serum triglycerides may develop in the autosomal recessive disorder glycogen storage disease (GSD). Plasmapheresis has been effective in reducing triglyceride levels in pancreatitis secondary to other conditions but not previously described in GSD. We describe a 16-year-old male with type 1a GSD who presented with severe abdominal pain, tachycardia and tachypnoea. Abdominal computed tomography (CT) demonstrated acute pancreatitis. Serum triglycerides were 91.8 mM. Despite intravenous fluids and morphine sulphate, he remained seriously ill, and plasmapheresis was therefore started. After daily plasma exchange for 6 days, triglyceride levels dropped to 5 mM. This was associated with a rapid resolution of pancreatitis. Plasmapheresis is effective in rapidly reducing hypertriglyceridaemia from numerous causes, including glycogen storage disease, and may facilitate recovery from acute pancreatitis.

Publication metadata

Author(s): Rivers E, Reynolds BC, Bunn S, Leech NJ, Straker J, Lambert HJ

Publication type: Book Chapter

Publication status: Published

Book Title: JIMD Reports

Year: 2018

Volume: 42

Pages: 1-4

Online publication date: 14/10/2018

Acceptance date: 29/09/2018

Publisher: Wiley - V C H Verlag GmbH & Co. KGaA


DOI: 10.1007/8904_2017_64

Library holdings: Search Newcastle University Library for this item

ISBN: 9783662583647


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