Jichu yixue yu linchuang (Mar 2021)
Analysis of 2 cases of glycogen storage disease typeⅠb with Crohn's disease in children
Abstract
Objective To investigate the clinical characteristics of glycogen storage disease typeⅠb(GSDⅠb) with Crohn's Disease(CD) and to improve the recognition of the disease. Methods Retrospectively analyze the clinical characteristics of 2 cases diagnosed with the GSDⅠb and CD in the pediatric department of Peking Union Medical College Hospital. Results A boy and a girl were included in the study. They were genetically diagnosed as GSDⅠb at 2 years age. Gastrointestinal symptoms including abdominal pain and diarrhea were observed at 4, 5 years old respectively. They were diagnosed as CD with endoscopic characteristics and pathology results. Besides corn starch, granulocyte colony-stimulating factor(G-CSF) was used to elevate the neutrophil to above 1.0×109/L. The gastrointestinal symptoms got alleviated. 5-aminosalicylic acid agents were used as supportive treatment. Conclusions Evaluation of the gastrointestinal system is necessary as the abdominal pain or diarrhea were observed in GSDⅠb patients. G-CSF with a relatively low dose is the mainstay to elevate neutrophil with few side effects. Splenomegaly is the most common side effect. Monitor renal function is necessary in the use of 5-ASA.
