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多伊格-波特综合征

Doege-Potter Syndrome.

作者信息

Ahluwalia N, Attia R, Green A, Cane P, Routledge T

机构信息

Guy's and St Thomas' NHS Foundation Trust , UK.

出版信息

Ann R Coll Surg Engl. 2015 Oct;97(7):e105-7. doi: 10.1308/rcsann.2015.0023.

Abstract

Doege-Potter syndrome is a rare paraneoplastic syndrome presenting as a hypoinsulinaemic hypoglycaemia from the ectopic secretion of a prohormone of insulin-like growth factor II (IGF-II) from a solitary fibrous tumour. Surgical resection is curative in the majority of cases. If, however, the diagnosis is not suspected and treatment is delayed, it can lead to hypoxic cerebral injury or death. The underlying tumour can be a benign or malignant pleural tumour but may be present in extrapleural sites. For a diagnosis of Doege-Potter syndrome, symptoms attributable to hypoglycaemia and low blood glucose levels should be present along with the secretion of prohormone IGF-II. We report a case of severe hypoglycaemia in a 76-year-old inpatient admitted for resection of a recurrent left-sided pleural tumour. Investigation revealed true hypoglycaemia and Doege-Potter syndrome was diagnosed. The tumour was completely resected and the patient made a full recovery with no further hypoglycaemic episodes.

摘要

多伊格-波特综合征是一种罕见的副肿瘤综合征,表现为因孤立性纤维瘤异位分泌胰岛素样生长因子II(IGF-II)的前体激素而导致的低胰岛素血症性低血糖。在大多数情况下,手术切除可治愈。然而,如果未怀疑到该诊断且治疗延迟,可能会导致缺氧性脑损伤或死亡。潜在的肿瘤可以是良性或恶性胸膜肿瘤,但也可能出现在胸膜外部位。对于多伊格-波特综合征的诊断,除了前体激素IGF-II的分泌外,还应出现低血糖相关症状和低血糖水平。我们报告一例76岁住院患者,因复发性左侧胸膜肿瘤切除术入院,出现严重低血糖。检查发现为真性低血糖,诊断为多伊格-波特综合征。肿瘤被完全切除,患者完全康复,未再发生低血糖发作。

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Doege-Potter Syndrome.多伊格-波特综合征
Ann R Coll Surg Engl. 2015 Oct;97(7):e105-7. doi: 10.1308/rcsann.2015.0023.

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