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Rivers E, Reynolds BC, Bunn S, et al. Acute Pancreatitis Secondary to Severe Hypertriglyceridaemia in a Patient with Type 1a Glycogen Storage Disease: Emergent Use of Plasmapheresis. JIMD Rep. 2017;42:1-4doi: 10.1007/8904_2017_64.
Rivers, E., Reynolds, B. C., Bunn, S., Leech, N. J., Straker, J., & Lambert, H. J. (2018). Acute Pancreatitis Secondary to Severe Hypertriglyceridaemia in a Patient with Type 1a Glycogen Storage Disease: Emergent Use of Plasmapheresis. JIMD reports, 421-4. https://doi.org/10.1007/8904_2017_64
Rivers, E, et al. "Acute Pancreatitis Secondary to Severe Hypertriglyceridaemia in a Patient with Type 1a Glycogen Storage Disease: Emergent Use of Plasmapheresis." JIMD reports vol. 42 (2018): 1-4. doi: https://doi.org/10.1007/8904_2017_64
Rivers E, Reynolds BC, Bunn S, Leech NJ, Straker J, Lambert HJ. Acute Pancreatitis Secondary to Severe Hypertriglyceridaemia in a Patient with Type 1a Glycogen Storage Disease: Emergent Use of Plasmapheresis. JIMD Rep. 2018;42:1-4. doi: 10.1007/8904_2017_64. Epub 2017 Oct 14. PMID: 29030854; PMCID: PMC6226393.
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JIMD Rep. 2018;42:1-4. doi: 10.1007/8904_2017_64. Epub 2017 Oct 14.

Acute Pancreatitis Secondary to Severe Hypertriglyceridaemia in a Patient with Type 1a Glycogen Storage Disease: Emergent Use of Plasmapheresis.

JIMD reports

E Rivers, B C Reynolds, S Bunn, N J Leech, J Straker, H J Lambert

Affiliations

  1. Great North Children's Hospital, Newcastle upon Tyne, UK.
  2. Royal Hospital for Children, Glasgow, UK. [email protected].
  3. Royal Victoria Infirmary, Newcastle upon Tyne, UK.

PMID: 29030854 PMCID: PMC6226393 DOI: 10.1007/8904_2017_64

Abstract

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.

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