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颅咽管瘤患者的内分泌和代谢合并症的患病率:一项全国性队列研究。

Prevalence of Endocrine and Metabolic Comorbidities in a National Cohort of Patients with Craniopharyngioma.

机构信息

Department of Endocrinology, Diabetes and Metabolism, University Children's Hospital, University Medical Center Ljubljana, Ljubljana, Slovenia.

Medical Faculty, University of Ljubljana, Ljubljana, Slovenia.

出版信息

Horm Res Paediatr. 2020;93(1):46-57. doi: 10.1159/000507702. Epub 2020 May 27.

Abstract

OBJECTIVE

The major part of craniopharyngioma (CP) morbidity is the tumor and/or treatment-related damage, which results in impaired function of the hypothalamic-pituitary axes and metabolic derangements. The aim of the study was to analyze the prevalence of long-term endocrine and metabolic comorbidities in a national cohort of CP patients based on the age at diagnosis and histology criteria.

DESIGN

A retrospective-prospective longitudinal cohort analysis.

METHODS

Forty-six patients with CP treated from 1979 onwards (19 with childhood-onset disease) in a single university institution were included in our study. Median follow-up from presentation was 12.8 years (interquartile range: 8.3-22.2 years) and comparable between age-at-diagnosis and histological subtype groups. Data on tumor histology were extracted from patients' records and re-evaluated if tissue samples were available (n = 32).

RESULTS

Childhood-onset patients presented more frequently with headache, and adult-onset with visual impairment. Prevalence of at least one pituitary axis affected increased from 54% at presentation to 100% at follow-up in childhood-onset and from 41 to 93% in adult-onset CP. Growth hormone deficiency, central diabetes insipidus, and panhypopituitarism were more prevalent in childhood-onset adamantinomatous CP (aCP) and least prevalent in adult-onset papillary CP (pCP). At follow-up, metabolic syndrome (MetS) was diagnosed in 80% of childhood-onset and 68% of adult-onset patients (p = 0.411). In the latter group, it tended to be more frequent in the aCP than pCP subtype (80 vs. 50%, p = 0.110).

CONCLUSIONS

Long-term endocrine and metabolic complications are very frequent in childhood- and adult-onset CP patients of both histological subtypes. The prevalence of MetS was higher compared to the largest cohort previously reported.

摘要

目的

颅咽管瘤(CP)的大部分发病率是由肿瘤和/或治疗相关的损伤引起的,这会导致下丘脑-垂体轴功能受损和代谢紊乱。本研究的目的是根据诊断时的年龄和组织学标准,分析基于全国 CP 患者队列的长期内分泌和代谢合并症的患病率。

设计

回顾性前瞻性纵向队列分析。

方法

纳入了一家大学附属医院 1979 年以来治疗的 46 例 CP 患者(19 例为儿童发病)。从发病到随访的中位时间为 12.8 年(四分位距:8.3-22.2 年),在年龄组和组织学亚型组之间无差异。从患者记录中提取肿瘤组织学数据,如果有组织样本可用(n=32),则对其进行重新评估。

结果

儿童发病患者更常表现为头痛,而成人发病患者更常表现为视力障碍。至少一个垂体轴受累的患病率从发病时的 54%增加到儿童发病患者的随访时的 100%,以及成人发病 CP 患者的 41%至 93%。在儿童发病的牙釉质型 CP(aCP)中,生长激素缺乏症、中枢性尿崩症和全垂体功能减退症更为常见,而在成人发病的乳头型 CP(pCP)中则最为少见。在随访时,80%的儿童发病和 68%的成人发病患者被诊断为代谢综合征(MetS)(p=0.411)。在后一组中,aCP 比 pCP 亚型更为常见(80%比 50%,p=0.110)。

结论

儿童和成人发病的 CP 患者无论组织学亚型如何,长期的内分泌和代谢并发症都非常常见。与之前报道的最大队列相比,MetS 的患病率更高。

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