Hypogonadism as a consequence of craniopharyngioma in female patients: comparison of childhood and adult onset and effects of estrogen replacement therapy.

IF 3 3区 医学 Q2 ENDOCRINOLOGY & METABOLISM Endocrine Pub Date : 2024-09-01 Epub Date: 2024-05-18 DOI:10.1007/s12020-024-03872-7
Irena Ilovayskaya, Vita Zektser
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Abstract

Purpose: (1) to compare clinical, biochemical features in female patients with hypoestrogenism due to childhood- and adult-onset CP; (2) to reveal effects of estrogen replacement therapy in female patients with childhood-onset CP.

Methods: Thirty-seven women that received specific treatment for CP in the period from 1980 to 2019 were recruited: 21 with childhood-onset and 16 with adult-onset CP. Clinical and hormonal characteristics were evaluated. Seventeen-beta-estradiol 2 mg and dydrogesterone 10 mg in sequential regiment was used in 18 childhood-onset cases. Mean follow-up was 31 months.

Results: Amenorrheic women with childhood- and adult-onset CP presented with the same complaints except for lack of genital hair and breast hypoplasia, which were common in patients with childhood-onset CP. BMI was lower in childhood-onset CP group, as was the proportion of overweight patients. They had more favorable lipid profile. The levels of estradiol, testosterone and DHEA-S were low and did not differ. Uterine and ovary volumes were reduced in all patients, but the decline was noticeable in the childhood-onset group. Mineral bone density of lumbar vertebrae was diminished in childhood-onset group. Estrogen therapy in these patients led to clinical improvement: increase in BMD in lumbar spine without negative changes in BMI and/or lipid profile.

Conclusions: Study showed that women with childhood-onset CP had less negative metabolic changes. However, they have more pronounced breast and uterus hypoplasia and lower BMD in lumbar spine. The estrogen replacement therapy led to clinical improvement and BMD increase in lumbar spine without increase of BMI and/or lipid profile changes.

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女性颅咽管瘤导致的性腺功能减退症:儿童期和成年期发病情况的比较以及雌激素替代疗法的影响。
目的:(1)比较儿童期和成年期CP引起的雌激素过少女性患者的临床和生化特征;(2)揭示雌激素替代疗法对儿童期CP女性患者的影响:方法:招募了37名在1980年至2019年期间接受过CP特殊治疗的女性患者,其中21名为儿童型CP患者,16名为成人型CP患者。对临床和激素特征进行了评估。18例儿童发病者使用了2毫克17-倍他雌二醇和10毫克地屈孕酮的序贯疗法,平均随访时间为31个月。平均随访时间为 31 个月:结果:患有儿童期和成年期CP的闭经妇女的主诉相同,但缺乏生殖器毛发和乳房发育不良在儿童期CP患者中很常见。儿童期发病的 CP 组患者的体重指数较低,超重患者的比例也较低。他们的血脂状况更佳。雌二醇、睾酮和 DHEA-S 水平较低,且无差异。所有患者的子宫和卵巢体积都有所下降,但儿童发病组的下降幅度更大。儿童发病组的腰椎骨矿物质骨密度降低。对这些患者进行雌激素治疗后,临床症状有所改善:腰椎骨密度增加,而体重指数和/或血脂状况没有发生负面变化:研究表明,儿童期发病的 CP 妇女的代谢负面变化较小。结论:研究表明,儿童期发病的 CP 妇女的负面代谢变化较小,但她们的乳房和子宫发育不良更明显,腰椎的 BMD 更低。雌激素替代疗法可改善临床症状,增加腰椎的 BMD,但不会增加 BMI 和/或血脂变化。
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来源期刊
Endocrine
Endocrine ENDOCRINOLOGY & METABOLISM-
CiteScore
6.50
自引率
5.40%
发文量
295
审稿时长
1.5 months
期刊介绍: Well-established as a major journal in today’s rapidly advancing experimental and clinical research areas, Endocrine publishes original articles devoted to basic (including molecular, cellular and physiological studies), translational and clinical research in all the different fields of endocrinology and metabolism. Articles will be accepted based on peer-reviews, priority, and editorial decision. Invited reviews, mini-reviews and viewpoints on relevant pathophysiological and clinical topics, as well as Editorials on articles appearing in the Journal, are published. Unsolicited Editorials will be evaluated by the editorial team. Outcomes of scientific meetings, as well as guidelines and position statements, may be submitted. The Journal also considers special feature articles in the field of endocrine genetics and epigenetics, as well as articles devoted to novel methods and techniques in endocrinology. Endocrine covers controversial, clinical endocrine issues. Meta-analyses on endocrine and metabolic topics are also accepted. Descriptions of single clinical cases and/or small patients studies are not published unless of exceptional interest. However, reports of novel imaging studies and endocrine side effects in single patients may be considered. Research letters and letters to the editor related or unrelated to recently published articles can be submitted. Endocrine covers leading topics in endocrinology such as neuroendocrinology, pituitary and hypothalamic peptides, thyroid physiological and clinical aspects, bone and mineral metabolism and osteoporosis, obesity, lipid and energy metabolism and food intake control, insulin, Type 1 and Type 2 diabetes, hormones of male and female reproduction, adrenal diseases pediatric and geriatric endocrinology, endocrine hypertension and endocrine oncology.
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