CT findings of glycogen storage disease I complicated with pancreatitis: A case report

The incidence of glycogen storage disease type I (GSD I) in the overall population is 1/100,000.[1] Hyperlipidemia in patients with GSD I can induce pancreatitis. Three cases of GSD I complicated with pancreatitis have been reported.[2] Here, the computed tomography (CT) features of GSD I complicated with pancreatitis are reported for the first time.

A 22-year-old female presents with growth retardation for 20 years and recurrent epigastric pain for 3 years. No abnormality in physical examination. Laboratory examination: GPT 81 U/L, GOT 111 U/L, DBIL 1.7 umol/L, TBIL 0.7 umol/L, Albumen 41.4 g/L, blood ammonia 54 umol/L, fasting blood glucose 3.02 mmol/L, G6PD 1829 U/L, lactic acid 7.9 mmol/L, triglyceride 18.79 mmol/L, TCH 9.46 mmol/L, uric acid 510 umol/L, urinary protein +++ (3.0) g/L.

The CT findings of the upper abdomen show that the liver is obviously enlarged, and the density of the liver is obviously uneven on plain scan. Unclear boundaries and increased blood vessels of the pancreas are found, especially in the head of the pancreas. The patient is diagnosed with GSD I complicated with pancreatitis.

The patient undergoes split liver transplantation and splenectomy under general anesthesia in our hospital.

Upper abdominal CT is reexamined half a month and 2 and a half months after the operation. It is found that the transplanted liver is not enlarged and the density is not abnormal. The pancreas shrinks, its boundary is clear, and its blood vessels decrease, especially in the head of the pancreas.

The density of the liver depends on the relative amount of glycogen and fat, which can be increased, normal, or decreased. Hyperlipidemia in patients with GSD I can induce pancreatitis.