Clinical Features and Analysis in Pituitary Stalk Interruption Syndrome

IF 2.3 4区 医学 Q3 ENDOCRINOLOGY & METABOLISM
Qiuxuan Guo, Jing Zhao, Shuang Yu
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引用次数: 0

Abstract

Objective. Pituitary stalk interruption syndrome (PSIS) is characterized by the absence of pituitary stalk, pituitary hypoplasia, and ectopic posterior pituitary. Because the etiology and clinical cognition of PSIS remain elusive, we analyzed the clinical features of PSIS in Chinese patients. Methods. A retrospective analysis was conducted on the clinical presentation, laboratory data, imaging examination, and management of 24 PSIS inpatients from our center over 10 years. Results. Among the 24 PSIS patients, there were 22 males (91.7%) and 2 females (8.3%). Growth hormone deficiency was present in all 24 cases (100%), hypogonadism in 24 cases (100%), secondary adrenal insufficiency in 22 cases (91.2%), and hypothyroidism in 21 cases (87.5%). 20 cases (83.3%) of PSIS patients exhibited deficiencies in four anterior pituitary hormones, 3 cases (12.5%) exhibited deficiencies in three anterior pituitary hormones, and 1 case (4.2%) exhibited deficiencies in two anterior pituitary hormones, with none exhibiting deficiencies in posterior pituitary hormones. Among the 24 PSIS patients, 12 had a history of growth hormone therapy before admission, and 12 had no such history. Additionally, 19 cases (79.2%) with PSIS were complicated by dyslipidemia, 15 cases (62.5%) were complicated by nonalcoholic fatty liver disease, and 9 cases (37.5%) were complicated by hyperuricemia. Conclusions. PSIS often presents with growth retardation and hypogonadotropic hypogonadism, but in some cases, short stature is not exhibited. PSIS is prone to complications such as dyslipidemia, nonalcoholic fatty liver disease, and hyperuricemia, increasing the risk of cardiovascular and cerebrovascular diseases. In clinical practice, the diagnostic ability of PSIS should be improved, and pituitary function and complications should be evaluated in a timely manner to avoid delayed treatment.
垂体柄中断综合征的临床特征与分析
目的:垂体柄中断综合征(PSIS垂体柄中断综合征(PSIS)以垂体柄缺失、垂体发育不良和垂体后叶异位为特征。由于 PSIS 的病因和临床认知仍不明确,我们分析了中国患者 PSIS 的临床特征。研究方法我们对本中心 24 例 PSIS 住院患者 10 年来的临床表现、实验室数据、影像学检查和处理进行了回顾性分析。结果。在 24 名 PSIS 患者中,男性 22 名(占 91.7%),女性 2 名(占 8.3%)。所有 24 例(100%)均存在生长激素缺乏症,24 例(100%)存在性腺功能减退症,22 例(91.2%)存在继发性肾上腺功能不全,21 例(87.5%)存在甲状腺功能减退症。20 例 PSIS 患者(83.3%)表现出四种垂体前叶激素缺乏,3 例(12.5%)表现出三种垂体前叶激素缺乏,1 例(4.2%)表现出两种垂体前叶激素缺乏,没有人表现出垂体后叶激素缺乏。在 24 例 PSIS 患者中,12 例在入院前曾接受过生长激素治疗,12 例没有此类病史。此外,19 例 PSIS 患者(79.2%)并发血脂异常,15 例(62.5%)并发非酒精性脂肪肝,9 例(37.5%)并发高尿酸血症。结论。PSIS 常表现为生长迟缓和性腺功能减退,但有些病例并不表现为身材矮小。PSIS 易引起血脂异常、非酒精性脂肪肝和高尿酸血症等并发症,增加心脑血管疾病的风险。在临床实践中,应提高 PSIS 的诊断能力,及时评估垂体功能和并发症,避免延误治疗。
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来源期刊
International Journal of Endocrinology
International Journal of Endocrinology ENDOCRINOLOGY & METABOLISM-
CiteScore
5.20
自引率
0.00%
发文量
147
审稿时长
1 months
期刊介绍: International Journal of Endocrinology is a peer-reviewed, Open Access journal that provides a forum for scientists and clinicians working in basic and translational research. The journal publishes original research articles, review articles, and clinical studies that provide insights into the endocrine system and its associated diseases at a genomic, molecular, biochemical and cellular level.
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