Abstract
A case of chronic pancreatitis in an 8-year-old boy with glycogen storage disease type 1a (GSD 1a) is presented. This patient had a history of hyperlipidaemia unresponsive to dietary therapy, e.g., a carbohydraterich diet, uncooked cornstarch, and nocturnal intragastric tube feedings. He had recently suffered bouts of abdominal pain and diarrhoea. Serum amylase and trypsin were elevated, abdominal CT revealed the presence of a pseudocyst of the pancreas. The presence of chronic pancreatitis was confirmed by endoscopic retrograde cholangiopancreatography and an infected pseudocyst was removed at laparotomy.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
Abbreviations
- ERCP:
-
endoscopic retrograde cholangiopancreatography
- GSD-1a:
-
type 1a glycogen storage disease
References
Brandborg LL (1978) Acute pancreatitis. Sleisenger MH, Fordtran JS (eds) Gastrointestinal disease, 2nd edn; Saunders, Philadelphia, pp 1416–1454
Fernandes J, Jansen H, Jansen TC (1979) Nocturnal gastric drip feeding in glucose-6-phosphatase deficient children. Pediatr Res 13:25–31
Fernandes J, Alaupovic P, Wit JM (1989) Gastric drip feeding in patients with glycogen storage disease type 1: Its effects on growth and plasma lipids and apolipoproteins. Pediatr Res 25:327–331
Frey C, Redo SF (1963) Inflammatory lesions of the pancreas in infancy and childhood. Pediatrics 32:93–102
Greene HL, Slonim AE, Burr IM (1979) Type 1 glycogen storage disease: a metabolic basis for advances in treatment. Adv Pediatr 26:63–92
Greene HL, Slonim AE, Burr IM, Moran JR (1980) Type 1 glycogen storage disease: Five years of management with nocturnal intragastric feeding. J Pediatr 96:590–595
Hendren WH, Greep JM, Patton AS (1965) Pancreatitis in childhood: experience in 15 cases. Arch Dis Child 40:132–145
McGarry JD, Mannaerts GP, Foster DW (1977) A possible role for malonyl CoA in the regulation of hepatic fatty acid oxidation and ketogenesis. J Clin Invest 60:265–270
Michels VV, Beaudet A (1980) Hemorrhagic pancreatitis in a patient with glycogen storage disease type I. Clin Genet 17:220–222
Ohtani T, Soyano M, Gu E, Kito H, Sugiyama K, Iwamura H (1985) Acute hemorrhagic pancreatitis in a patient with glycogen storage disease type I. J Jpn Pediatr Soc 89:1838–1844 (in Japanese)
Stanley CA, Mills JL, Baker L (1981) Intragastric feeding in type 1 glycogen storage disease: Factors affecting the control of lactic acidemia. Pediatr Res 15:1504–1508
Yoshioka M, Imamura K, Ohira S (1982) Pancreatitis in a case with glycogen storage disease type I. Internal Medicine 50: 765–773 (in Japanese)
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Kikuchi, M., Hasegawa, K., Handa, I. et al. Chronic pancreatitis in a child with glycogen storage disease type 1. Eur J Pediatr 150, 852–853 (1991). https://doi.org/10.1007/BF01955007
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF01955007