1. Diversities of Mechanism in Patients with VHL Syndrome and diabetes: A Report of Two Cases and Literature Review
- Author
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Wang,Yanlei, Liu,Zhaoxiang, Zhao,Wenhui, Cao,Chenxiang, Xiao,Luqi, Xiao,Jianzhong, Wang,Yanlei, Liu,Zhaoxiang, Zhao,Wenhui, Cao,Chenxiang, Xiao,Luqi, and Xiao,Jianzhong
- Abstract
Yanlei Wang,* Zhaoxiang Liu,* Wenhui Zhao, Chenxiang Cao, Luqi Xiao, Jianzhong Xiao Department of Endocrinology, Beijing Tsinghua Changgung Hospital, School of Clinical Medicine, Tsinghua University, Beijing, 102218, Peopleâs Republic of China*These authors contributed equally to this workCorrespondence: Jianzhong Xiao, Department of Endocrinology, Beijing Tsinghua Changgung Hospital, School of Clinical Medicine, Tsinghua University, No. 168 Litang Road, Changping District, Beijing, 102218, Peopleâs Republic of China, Tel/Fax +8610-5611-9057, Email xjza01150@btch.edu.cnBackground: Von Hippel-Lindau (VHL) syndrome is characterized by tumorous lesions affecting multiple organs. Pancreatic involvement in VHL syndrome can present as endocrine tumors and pancreatic cysts, which can interfere with both exocrine and endocrine functions of the pancreas. Diabetes is an uncommon complication of VHL syndrome.Purpose: This study aims to summarize the various mechanisms of diabetes in VHL syndrome by reporting two cases and conducting a literature review.Methods: We analyzed the clinical and imaging data of two patients with VHL syndrome and diabetes. Additionally, we reviewed the existing literature to explore the clinical diversities and management strategies for VHL syndrome complicated with diabetes.Results: The first patient presented with liver metastasis of pancreatic neuroendocrine tumor and multiple pheochromocytoma. After surgery, the patientâs diabetic control improved, as evidenced by a significant reduction in insulin dosage. This indicates a potential insulin resistance due to elevated metanephrine levels prior to surgery and partial insulin deficiency caused by distal pancreatectomy. The second patient had multiple hemangioblastomas, as well as multiple pancreatic cysts and positive pancreatic islet autoantibodies. Diabetes in this case may be attributed to pancreatic lesions and the coexistence of autoimmune insulitis. A literature r
- Published
- 2024