特发性孤立性肾上腺皮质激素缺乏症:一项单中心回顾性研究。

IF 1.6 4区 医学 Q4 ENDOCRINOLOGY & METABOLISM Experimental and Clinical Endocrinology & Diabetes Pub Date : 2023-10-01 Epub Date: 2023-09-08 DOI:10.1055/a-2135-7708
Chengyan Yang, Xinpei Li, Xiaoqing Ma
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引用次数: 0

摘要

特发性孤立性促肾上腺皮质激素缺乏症(IIAD)是一种罕见的疾病,临床漏诊率和误诊率较高。本研究回顾性收集了2014年1月至2022年12月济宁市第一人民医院17例IIAD患者的临床表现、实验室检查结果和治疗反应信息。总结其临床特点,并对相关数据进行分析。因此,大多数IIAD患者为男性(94.12%),发病年龄在13至80岁之间。主要表现为厌食症(88.24%)、恶心(70.59%)、呕吐(47.06%)、疲劳(64.71%)和神经或精神症状(88.24%,中位诊断时间为2个月,最长诊断时间为10年。实验室检查多为低钠血症(88.24%)和低血糖症(70.59%),补充糖皮质激素后症状和实验室指标恢复正常。IIAD发病隐匿,症状不典型;它经常被误诊为胃肠道、神经系统或精神疾病。本研究的目的是提高临床医生对IIAD的理解,对有不明原因胃肠道症状、神经和精神症状、低钠血症或低血糖的患者应进行IIAD评估,并确保早期诊断和治疗。
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Idiopathic Isolated Adrenocorticotropic Hormone Deficiency: A Single-Center Retrospective Study.

Idiopathic isolated adrenocorticotrophic hormone deficiency (IIAD) is rare, with high clinical omission and misdiagnosis rates. This study retrospectively collected information on clinical presentation, laboratory findings, and treatment response of 17 patients with IIAD at Jining No. 1 People's Hospital from January 2014 to December 2022. The clinical characteristics were summarized, and the pertinent data were analyzed. As a result, most of the patients with IIAD were male (94.12%), with age at onset ranging from 13 to 80 years. The primary manifestations were anorexia (88.24%), nausea (70.59%), vomiting (47.06%), fatigue (64.71%), and neurological or psychiatric symptoms (88.24%). The median time to diagnosis was 2 months and the longest was 10 years. Laboratory tests mostly showed hyponatremia (88.24%) and hypoglycemia (70.59%). The symptoms and laboratory indicators returned to normal after supplementing patients with glucocorticoids. IIAD has an insidious onset and atypical symptoms; it was often misdiagnosed as gastrointestinal, neurological, or psychiatric disease. The aim of this study was to improve clinicians' understanding of IIAD, patients with unexplained gastrointestinal symptoms, neurological and psychiatric symptoms, hyponatremia, or hypoglycemia should be evaluated for IIAD and ensure early diagnosis and treatment.

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来源期刊
CiteScore
4.10
自引率
5.60%
发文量
72
审稿时长
3 months
期刊介绍: Publishing outstanding articles from all fields of endocrinology and diabetology, from molecular biology to clinical research, this journal is a brilliant resource. Since being published in English in 1983, the popularity of this journal has grown steadily, reflecting the importance of this publication within its field. Original contributions and short communications appear in each issue along with reviews addressing current topics. In addition, supplementary issues are published each year presenting abstracts or proceedings of national and international scientific meetings. The journal was initially published in German and is still the oldest endocrinological periodical in the German-language market!
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