A case report of acute pancreatitis with glycogen storage disease type IA in an adult patient and review of the literature

Jiaoyu Ai, Wenhua He, Xin Huang, Yao Wu, Yupeng Lei, Chen Yu, Kivanc Görgülü, Kalliope N Diakopoulos, Nonghua Lu, Yin Zhu
Medicine (Baltimore) 2020 October 16, 99 (42): e22644

RATIONALE: Glycogen storage disease type IA (GSD IA) is an inherited disorder of glycogen metabolism characterized by fasting hypoglycemia, hyperuricemia, and hyperlipidemia including hypertriglyceridemia (HTG). Patients have a higher risk of developing acute pancreatitis (AP) because of HTG. AP is a potentially life-threatening disease with a wide spectrum severity. Nevertheless, almost no reports exist on GSD IA-induced AP in adult patients.

PATIENT CONCERNS: A 23-year-old male patient with GSD 1A is presented, who developed moderate severe AP due to HTG.

DIAGNOSES: The GSD 1A genetic background of this patient was confirmed by Sanger sequencing. Laboratory tests, along with abdominal enhanced-computed tomography, were used for the diagnosis of HTG and AP.

INTERVENTIONS: This patient was transferred to the intensive care unit and treated by reducing HTG, suppressing gastric acid, inhibiting trypsin activity, and relieving hyperuricemia and gout.

OUTCOMES: Fifteen days after hospital admission, the patient had no complaints about abdominal pain and distention. Follow-up of laboratory tests displayed almost normal values. Reexamination by computed tomography exhibited a reduction in peripancreatic necrotic fluid collection compared with the initial stage.

LESSONS: Fast and long-term reduction of triglycerides along with management of AP proved effective in relieving suffering of an adult GSD IA-patient and improving prognosis. Thus, therapeutic approaches have to be renewed and standardized to cope with all complications, especially AP, and enable a better outcome so that patients can master the disease.

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