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广泛脑手术后的下丘脑和垂体功能障碍:对更多知识的渴望

Hypothalamic and Pituitary Dysfunction After Extensive Brain Surgery: There Is Thirst for More Knowledge.

作者信息

Galbiati Francesca, Stamatiades George A, Bi Wenya L, Abreu Ana Paula

机构信息

Division of Endocrinology, Diabetes and Hypertension, Brigham and Women's Hospital, Harvard Medical School, Boston, MA 02115, USA.

Department of Neurosurgery, Brigham and Women's Hospital, Harvard Medical School, Boston, MA 02115, USA.

出版信息

JCEM Case Rep. 2023 Nov 16;1(6):luad137. doi: 10.1210/jcemcr/luad137. eCollection 2023 Nov.

Abstract

Craniopharyngiomas are tumors originating from the infundibular stalk, extending to the parasellar and suprasellar region, thereby conferring multiple risks of this region. In particular, hypothalamic and pituitary damage related to its natural history as well as treatment effects of craniopharyngiomas substantially affect life expectancy and quality of life. Here, we describe an adult patient presenting with polyuria, memory, and visual field impairment secondary to concurrent craniopharyngioma and intraventricular glioma. He was treated with surgical resection with postoperative course notable for hypothalamic-pituitary dysfunction, including central hypothyroidism, central adrenal insufficiency, arginine vasopressin deficiency (AVP-D, formerly diabetes insipidus) with loss of sense of thirst, and hypothalamic hypothermia. The adipsia, combined with memory dysfunction, challenged the management of constant fluctuations in his sodium (129-168 mEq/L), with ultimate treatment through vasopressin repletion, fixed fluid intake, strict urine output monitoring, and close counseling of the patient and his caregiver. This case exemplifies the complexity of the endocrine care of patients with craniopharyngiomas and highlights the need for step-wise algorithms in the treatment of hypothalamic deficiencies such as adipsia.

摘要

颅咽管瘤是起源于漏斗柄的肿瘤,延伸至鞍旁和鞍上区域,从而带来该区域的多种风险。特别是,与颅咽管瘤的自然病史以及治疗效果相关的下丘脑和垂体损伤会严重影响预期寿命和生活质量。在此,我们描述一名成年患者,其因同时患有颅咽管瘤和脑室内胶质瘤而出现多尿、记忆力减退和视野缺损。他接受了手术切除治疗,术后病程以下丘脑 - 垂体功能障碍为显著特征,包括中枢性甲状腺功能减退、中枢性肾上腺皮质功能不全、精氨酸血管加压素缺乏(AVP - D,以前称为尿崩症)伴口渴感丧失以及下丘脑体温过低。渴感缺失与记忆功能障碍相结合,给其钠水平持续波动(129 - 168 mEq/L)的管理带来挑战,最终通过补充血管加压素、固定液体摄入量、严格监测尿量以及对患者及其护理人员进行密切指导来进行治疗。该病例体现了颅咽管瘤患者内分泌护理的复杂性,并强调了在治疗诸如渴感缺失等下丘脑功能缺陷时采用逐步算法的必要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e2a1/10652246/5cd3e6ff2b9a/luad137f1.jpg

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