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一名患有乳头型颅咽管瘤的患者出现类似厌食-恶病质综合征的下丘脑神经内分泌功能障碍。

Anorexia-cachexia syndrome-like hypothalamic neuroendocrine dysfunction in a patient with a papillary craniopharyngioma.

作者信息

Balcázar-Hernández Lourdes, Vargas-Ortega Guadalupe, Valverde-García Yelitza, Mendoza-Zubieta Victoria, González-Virla Baldomero

机构信息

Endocrinology Department.

Anatomic Pathology Department, Hospital de Especialidades, Centro Médico Nacional Siglo XXI, Colonia Doctores, Mexico CityMexico.

出版信息

Endocrinol Diabetes Metab Case Rep. 2017 Apr 21;2017. doi: 10.1530/EDM-17-0018. eCollection 2017.

DOI:10.1530/EDM-17-0018
PMID:28469924
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5409936/
Abstract

UNLABELLED

The craniopharyngiomas are solid cystic suprasellar tumors that can present extension to adjacent structures, conditioning pituitary and hypothalamic dysfunction. Within hypothalamic neuroendocrine dysfunction, we can find obesity, behavioral changes, disturbed circadian rhythm and sleep irregularities, imbalances in the regulation of body temperature, thirst, heart rate and/or blood pressure and alterations in dietary intake (like anorexia). We present a rare case of anorexia-cachexia syndrome like a manifestation of neuroendocrine dysfunction in a patient with a papillary craniopharyngioma. Anorexia-cachexia syndrome is a complex metabolic process associated with underlying illness and characterized by loss of muscle with or without loss of fat mass and can occur in a number of diseases like cancer neoplasm, non-cancer neoplasm, chronic disease or immunodeficiency states like HIV/AIDS. The role of cytokines and anorexigenic and orexigenic peptides are important in the etiology. The anorexia-cachexia syndrome is a clinical entity rarely described in the literature and it leads to important function limitation, comorbidities and worsening prognosis.

LEARNING POINTS

Suprasellar lesions can result in pituitary and hypothalamic dysfunction.The hypothalamic neuroendocrine dysfunction is commonly related with obesity, behavioral changes, disturbed circadian rhythm and sleep irregularities, but rarely with anorexia-cachexia.Anorexia-cachexia syndrome is a metabolic process associated with loss of muscle, with or without loss of fat mass, in a patient with neoplasm, chronic disease or immunodeficiency states.Anorexia-cachexia syndrome results in important function limitation, comorbidities that influence negatively on treatment, progressive clinical deterioration and bad prognosis that can lead the patient to death.Anorexia-cachexia syndrome should be suspected in patients with emaciation and hypothalamic lesions.

摘要

未标注

颅咽管瘤是鞍上实性囊性肿瘤,可侵犯相邻结构,导致垂体和下丘脑功能障碍。在下丘脑神经内分泌功能障碍中,可出现肥胖、行为改变、昼夜节律紊乱和睡眠不规律、体温调节失衡、口渴、心率和/或血压异常以及饮食摄入改变(如厌食)。我们报告了一例罕见的厌食-恶病质综合征,它是一名乳头状颅咽管瘤患者神经内分泌功能障碍的表现。厌食-恶病质综合征是一种与潜在疾病相关的复杂代谢过程,其特征是伴有或不伴有脂肪量减少的肌肉量减少,可发生于多种疾病,如癌症肿瘤、非癌症肿瘤、慢性疾病或免疫缺陷状态(如HIV/AIDS)。细胞因子以及厌食性和促食欲肽在病因学中起重要作用。厌食-恶病质综合征是一种在文献中很少描述的临床实体,它会导致重要的功能受限、合并症和预后恶化。

学习要点

鞍上病变可导致垂体和下丘脑功能障碍。下丘脑神经内分泌功能障碍通常与肥胖、行为改变、昼夜节律紊乱和睡眠不规律有关,但很少与厌食-恶病质有关。厌食-恶病质综合征是一种在肿瘤、慢性疾病或免疫缺陷状态患者中与肌肉量减少(伴有或不伴有脂肪量减少)相关的代谢过程。厌食-恶病质综合征会导致重要的功能受限、对治疗产生负面影响的合并症、临床进行性恶化和不良预后,可导致患者死亡。对于消瘦且有下丘脑病变的患者,应怀疑患有厌食-恶病质综合征。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8b7d/5409936/d668897003e3/edmcr-2017-170018-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8b7d/5409936/a41f55beb684/edmcr-2017-170018-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8b7d/5409936/cf356a945eec/edmcr-2017-170018-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8b7d/5409936/d0b87360651d/edmcr-2017-170018-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8b7d/5409936/d668897003e3/edmcr-2017-170018-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8b7d/5409936/a41f55beb684/edmcr-2017-170018-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8b7d/5409936/cf356a945eec/edmcr-2017-170018-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8b7d/5409936/d0b87360651d/edmcr-2017-170018-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8b7d/5409936/d668897003e3/edmcr-2017-170018-g004.jpg

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本文引用的文献

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Neuroendocrine Disorders in Pediatric Craniopharyngioma Patients.小儿颅咽管瘤患者的神经内分泌紊乱
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