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鞍区黄色肉芽肿伴生长激素缺乏症:病例报告及文献复习

Xanthogranuloma of the sellar region accompanied by growth hormone deficiency: case report and literature review.

作者信息

Kobayashi Mami, Yagasaki Hideaki, Kobayashi Koji, Ogiwara Masakazu, Kinouchi Hiroyuki, Sugita Kanji

机构信息

Department of Neonatology, Yamanashi Prefectural Central Hospital, 1-1-1 Fujimi, Kofu, Yamanashi 400-8506, Japan.

Department of Pediatrics, Faculty of Medicine, University of Yamanashi, Shimokato, Chuo, Yamanashi, Japan.

出版信息

J Pediatr Endocrinol Metab. 2018 Oct 25;31(10):1161-1164. doi: 10.1515/jpem-2018-0050.

Abstract

Background Xanthogranuloma of the sellar region is a rare entity. Its pathology is controversial and it is difficult to strictly differentiate it from craniopharyngioma or Rathke's cyst. Case presentation We report a case of xanthogranuloma accompanied by growth hormone deficiency in an 11-year-old girl. She did not show any other pituitary hormone deficiency or neurological symptoms before operation. The preoperative diagnosis was craniopharyngioma, but histological findings showed small areas of epithelium. Thus, the final diagnosis was xanthogranuloma. Xanthogranuloma is an important cause of growth delay. We reviewed 16 cases reported after 2000, and included our case, of xanthogranuloma in children. Conclusions Endocrinological symptoms are often regarded as one of the few apparent symptoms in xanthogranuloma compared with craniopharyngioma. Therefore, we should follow up carefully and accumulate cases.

摘要

背景 鞍区黄色肉芽肿是一种罕见的疾病。其病理存在争议,很难将其与颅咽管瘤或拉克氏囊肿严格区分开来。病例报告 我们报告一例11岁女孩患有黄色肉芽肿并伴有生长激素缺乏症。术前她未表现出任何其他垂体激素缺乏或神经症状。术前诊断为颅咽管瘤,但组织学检查结果显示有小面积上皮。因此,最终诊断为黄色肉芽肿。黄色肉芽肿是生长发育迟缓的一个重要原因。我们回顾了2000年后报道的16例儿童黄色肉芽肿病例,包括我们的病例。结论 与颅咽管瘤相比,内分泌症状在黄色肉芽肿中常被视为少数明显症状之一。因此,我们应仔细随访并积累病例。

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