冯·希佩尔-林道病的内分泌表现
Endocrine Manifestations of Von Hippel-Landau Disease.
作者信息
Pradhan Roma, George Nelson, Mandal Kaushik, Agarwal Amit, Gupta Sushil K
机构信息
Department of Endocrine Surgery, Dr. RMLIMS, Lucknow, Uttar Pradesh, India.
Department of Endocrine Surgery, SGPGIMS, Lucknow, Uttar Pradesh, India.
出版信息
Indian J Endocrinol Metab. 2019 Jan-Feb;23(1):159-164. doi: 10.4103/ijem.IJEM_252_18.
Von Hippel-Lindau (VHL) disease is an autosomal dominant disorder characterized by various endocrine, nonendocrine, benign, and malignant tumors in various organs. VHL tumor suppressor gene, located on short arm of chromosome 3 is responsible for this. Pheochromocytoma (PCC) is one of the important endocrine manifestations that needs to be ruled out in case of VHL suspicion. In this review, we summarize the endocrine manifestations of VHL disease and their management while giving case history of five such cases.
冯·希佩尔-林道（VHL）病是一种常染色体显性疾病，其特征是在各个器官中出现各种内分泌、非内分泌、良性和恶性肿瘤。这是由位于3号染色体短臂上的VHL肿瘤抑制基因所致。嗜铬细胞瘤（PCC）是VHL病疑似病例中需要排除的重要内分泌表现之一。在本综述中，我们总结了VHL病的内分泌表现及其治疗方法，并给出了5例此类病例的病史。
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