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两例垂体柄中断综合征:早期检测与管理的重要性

Two cases of pituitary stalk interruption syndrome: importance of early detection and management.

作者信息

Zakkor Mohammed Deeb, Khana Firas, Abdulrazzak Mohammed, Kreid Sedra, Kayyali Alae, Al Hussein Hachem

机构信息

Department of Endocrinology, Aleppo University Hospital, Aleppo.

Faculty of Medicine, University of Aleppo, Aleppo, Syria.

出版信息

Ann Med Surg (Lond). 2024 May 6;86(6):3776-3780. doi: 10.1097/MS9.0000000000002123. eCollection 2024 Jun.

Abstract

INTRODUCTION AND IMPORTANCE

Pituitary stalk interruption syndrome (PSIS) is a rare congenital condition affecting the pituitary gland and its stalk, leading to hormonal imbalances. PSIS can present with a wide range of symptoms, including delayed puberty and short stature.

CASE PRESENTATION

This paper discusses two cases of PSIS in patients with a history of growth hormone deficiency. The first case is of a 26-year-old male presenting with fatigue and loss of appetite, while the second case is of a 14-year-old male presenting with delayed puberty. Blood tests revealed hormonal imbalances, and a subsequent MRI confirmed the diagnosis of PSIS. Hormonal supplements were prescribed to manage the condition, and follow-up appointments were scheduled to monitor progress.

CLINICAL DISCUSSION

PSIS can present with a wide range of symptoms, and can be diagnosed at different ages. Early diagnosis and management of PSIS are crucial to prevent long-term complications such as short stature, impaired cognitive function, and infertility. The use of hormonal supplements, as seen in both cases, is essential to manage the hormonal imbalances associated with PSIS. Testosterone replacement therapy is used to treat hypogonadism, while thyroxine and hydrocortisone are used to manage hypothyroidism and adrenal insufficiency, respectively.

CONCLUSION

Early diagnosis and management of PSIS through hormonal supplements are crucial to prevent long-term complications. It is essential to monitor patients' progress through follow-up appointments to ensure optimal management of the condition.

摘要

引言与重要性

垂体柄阻断综合征(PSIS)是一种罕见的先天性疾病,影响垂体及其柄部,导致激素失衡。PSIS可表现出广泛的症状,包括青春期延迟和身材矮小。

病例报告

本文讨论了两例有生长激素缺乏病史的PSIS患者。第一例是一名26岁男性,表现为疲劳和食欲不振,第二例是一名14岁男性,表现为青春期延迟。血液检查显示激素失衡,随后的核磁共振成像(MRI)确诊为PSIS。已开处激素补充剂来治疗该病症,并安排了随访预约以监测进展情况。

临床讨论

PSIS可表现出广泛的症状,且可在不同年龄被诊断出来。PSIS的早期诊断和治疗对于预防诸如身材矮小、认知功能受损和不孕等长期并发症至关重要。如两例病例所示,使用激素补充剂对于治疗与PSIS相关的激素失衡至关重要。睾酮替代疗法用于治疗性腺功能减退,而甲状腺素和氢化可的松分别用于治疗甲状腺功能减退和肾上腺功能不全。

结论

通过激素补充剂对PSIS进行早期诊断和治疗对于预防长期并发症至关重要。通过随访预约监测患者的进展情况以确保对该病症进行最佳治疗是必不可少的。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cf31/11152810/c6a9d97fb55a/ms9-86-3776-g001.jpg

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