Open AccessA case report of acute pancreatitis with glycogen storage disease type IA in an adult patient and review of the literature
Author(s) -
Jiaoyu Ai,
Wenhua He,
Xin Huang,
Yao Wu,
Yifeng Lei,
Yu Chen,
Kıvanç Görgülü,
Kalliope N. Diakopoulos,
ghua Lü,
Yin Zhu
Publication year - 2020
Publication title -
medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.59
H-Index - 148
eISSN - 1536-5964
pISSN - 0025-7974
DOI - 10.1097/md.0000000000022644
Subject(s) - medicine , acute pancreatitis , hyperuricemia , hypertriglyceridemia , pancreatitis , glycogen storage disease , hypoglycemia , abdominal pain , gastroenterology , gout , intensive care unit , hyperlipidemia , uric acid , disease , surgery , diabetes mellitus , insulin , endocrinology , triglyceride , cholesterol
Abstract 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.