[多发性垂体激素缺乏症患者的长期随访数据]。

IF 0.5 Q4 PEDIATRICS
Aysegul Elvan-Tuz, Elvan Bayramoglu, Semra Cetinkaya
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引用次数: 0

摘要

缺乏两种或两种以上垂体激素被称为多发性垂体激素缺乏症(MPHD)。据估计,全球发病率约为1/8,000:介绍MPHD患者的诊断过程、临床发现和长期随访情况:对 1999 年至 2015 年期间确诊的 MPHD 患者进行评估。对临床表现、人体测量、影像学检查和临床演变进行了分析。对激素状况进行了评估,包括生长激素(GH)、促甲状腺激素(TSH)、促肾上腺皮质激素(ACTH)、促卵泡激素/促黄体生成素(FSH/LH)和催乳素(PRL)。数据采用学生 t 检验和 Mann-Whitney U 检验进行评估。相关性采用斯皮尔曼相关性检验。P值小于0.05为具有统计学意义:45名患者中,55.6%为男性,平均发病年龄为(5.6 ± 3.9)岁(0-14.4)岁,中位骨龄为(3.5 ± 2.3)岁(0.5-11)岁。入院时,88.9%的病例发现 GH 缺乏,77.8%的病例发现 TSH 缺乏,33.3%的病例发现 ACTH 缺乏,22.2%的病例发现 FSH/LH 缺乏,17.8%的病例发现 PRL 缺乏。在随访期间,62%的病例增加了其他激素缺乏症。平均随访时间为 9.18 ± 3.6 (3.02-17.2) 年:结论:MPHD患者的临床表现千差万别,其中以GH和TSH缺乏症最为常见。我们的研究结果表明,遗传性身高潜力可通过 GH 治疗来实现。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
[Long-term follow-up data of patients with Multiple Pituitary Hormone Deficiency].

The deficiency of two or more pituitary hormones is called multiple pituitary hormone deficiencies (MPHD). Its prevalence is estimated to be about 1/8,000 worldwide.

Objective: To present the diagnosis processes, clinical findings, and long-term follow-up of patients with MPHD.

Patients and method: Between 1999 and 2015, patients diagnosed with MPHD were evaluated. Clinical presentation, anthropometry, imaging studies, and clinical evolution were analyzed. Hormone status was evaluated, including growth hormone (GH), thyroid-stimulating hormone (TSH), adrenocorticotropic hormone (ACTH), follicle-stimulating hormone/luteinizing hormone (FSH/LH), and prolactin (PRL). Data were assessed using the student's t-test and the Mann-Whitney U test. Spearman's correlation was used for correlations. A p-value < 0.05 was considered statistically significant.

Results: Forty-five patients were included; 55.6% were male, the mean age at presentation was 5.6 ± 3.9 (0-14.4) years, and the median bone age was 3.5 ± 2.3 (0.5-11) years. At admission, GH deficiency was found in 88.9% of the cases, TSH deficiency in 77.8%, ACTH deficiency in 33.3%, FSH/LH deficiency in 22.2%, and PRL deficiency in 17.8%. During the follow-up, 62% of the cases added other hormone deficiencies. The mean follow-up period was 9.18 ± 3.6 (3.02-17.2) years.

Conclusion: Patients with MPHD have very different clinical presentations, with GH and TSH deficiency being the most common in this study. Additional hormonal deficiencies can occur even years after the initial diagnosis and our results demonstrate that genetic height potential is achieved with GH treatment.

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