Clinical Characteristics of Children with Combined Pituitary Hormone Deficiency and the Effects of Growth Hormone Treatment.

IF 1.2 4区 医学 Q3 PEDIATRICS Klinische Padiatrie Pub Date : 2023-12-04 DOI:10.1055/a-2186-9304
Özge Besci, Reyhan Deveci Sevim, Kübra Yüksek Acinikli, Korcan Demir, Gönül Çatlı, Bayram Özhan, Tolga Ünüvar, Ahmet Anık, Ayhan Abacı, Ayça Altıncık
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Abstract

Aim: We aimed to describe the clinical characteristics of patients with congenital combined pituitary hormone deficiency (CPHD) and evaluate the first-year growth responses of individuals with CPHD and isolated growth hormone deficiency (IGHD) in order to establish the influence of other hormone deficiencies on growth response.

Patients and methods: This retrospective study was conducted in four tertiary care centers in Turkey. The records of patients diagnosed with CPHD (n=39) and severe IGHD (n=50) were collected. Cases with acquired lesions or chronic diseases were not included in the study. Data are presented as median (interquartile range).

Results: Among 39 patients (13 females; 33%) with a diagnosis of CPHD, the majority of patients (64%) presented initially with combined deficits at baseline examination, whereas isolated deficiencies (36%) were less prevalent. Among all patients with GH deficiency, TSH, ACTH, FSH/LH, and ADH deficiencies were present in 94%, 74%, 44%, and 9% of patients, respectively. Patients with CPHD were diagnosed at a younger age (4.9 (8.4) vs. 11.6 (4.1), p<0.001, respectively) and had lower peak GH concentrations (0.4 (1.8) vs. 3.7 (2.9), p<0.001, respectively) than patients with IGHD. Patients with IGHD and CPHD had similar first-year growth responses (Δheight SD score of 0.55 (0.63) vs. 0.76 (0.71), respectively, p=0.45).

Conclusions: We established the nature and timing of numerous hormonal deficits emerging over time. We also identified that the existence of CPHD did not hinder growth response.

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儿童合并垂体激素缺乏的临床特点及生长激素治疗的效果。
目的:我们旨在描述先天性联合垂体激素缺乏症(CPHD)患者的临床特征,评估CPHD合并孤立性生长激素缺乏症(IGHD)患者第一年的生长反应,以确定其他激素缺乏症对生长反应的影响。患者和方法:本回顾性研究在土耳其的四个三级保健中心进行。收集诊断为CPHD (n=39)和重度IGHD (n=50)的患者记录。获得性病变或慢性疾病的病例不包括在研究中。数据以中位数(四分位数范围)表示。结果:39例患者中,女性13例;33%)诊断为CPHD,大多数患者(64%)在基线检查时最初表现为合并缺陷,而孤立缺陷(36%)不太普遍。在所有生长激素缺乏症患者中,TSH、ACTH、FSH/LH和ADH缺乏症分别出现在94%、74%、44%和9%的患者中。CPHD患者的诊断年龄较低(4.9(8.4)比11.6(4.1))。结论:我们确定了随着时间的推移出现的许多激素缺陷的性质和时间。我们还发现CPHD的存在并不会阻碍生长反应。
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来源期刊
Klinische Padiatrie
Klinische Padiatrie 医学-小儿科
CiteScore
1.10
自引率
0.00%
发文量
135
审稿时长
6-12 weeks
期刊介绍: Das Forum für wissenschaftliche Information in der Kinderheilkunde ausgewählte Originalarbeiten aus allen Bereichen der Pädiatrie Visite: Ihr Forum für interessante Krankengeschichten und außergewöhnliche Kasuistiken aktuelle Fortschritte in Diagnostik und Therapie jährliche Schwerpunkthefte: Ergebnisse der pädiatrischen Onkologie plus Medizin und Markt topaktuelle Informationen aus der Industrie
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