下垂体干中断综合征的临床特征和分析
Qiuxuan Guo1, Jing Zhao2, Shuang Yu3
1Department of Endocrinology, Fuqing City Hospital Affiliated to Fujian Medical University, Fuqing 350300, Fujian, China.
International journal of endocrinology
|June 3, 2024
概括
在中国患者中,垂体茎中断综合征 (PSIS) 通常会导致多种垂体激素缺乏症,特别是在男性中. 早期诊断和管理对于解决相关的代谢并发症,如失脂症和脂肪肝疾病至关重要.
科学领域:
- 内分泌学 在内分泌学.
- 儿科内分泌学 儿科内分泌学
- 代谢障碍 代谢障碍 代谢障碍
背景情况:
- 垂体茎中断综合征 (PSIS) 是一种罕见的先天性疾病,其特征是缺少垂体茎,垂体缺血和异位垂体后垂体.
- 目前尚不完全了解PSIS的病因和临床表现,因此需要对其特征和相关疾病进行进一步的研究.
研究的目的:
- 在一组中国患者中分析下垂体茎中断综合征 (PSIS) 的临床特征,内分泌缺陷和常见并发症.
- 提高对PSIS的理解,以改善临床诊断和管理.
主要方法:
- 在10年的时间里,对24名被诊断患有PSIS的住院患者进行了回顾性分析.
- 收集的数据包括临床表现,实验室结果,成像发现和治疗结果.
主要成果:
- 该研究在24名PSIS患者中发现了22名男性 (91.7%) 和2名女性 (8.3%).
- 所有患者都表现出生长激素缺陷 (100%) 和阴性腺体缺陷 (100%). 其他常见的缺陷包括二次上腺功能不全 (91.2%) 和甲状腺功能低下 (87.5%).
- 常见的并发症包括脂质不良症 (79.2%),非酒精性脂肪性肝病 (62.5%),高尿血 (37.5%).
结论:
- 皮质神经综合症主要影响男性,并与多种前垂体激素缺陷有关.
- 相关的代谢并发症,如失脂症和非酒精性脂肪性肝病,增加心血管和脑血管风险.
- 及时评估垂体功能和并发症对于有效管理和预防延迟治疗至关重要.
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