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囊性纤维化的内分泌并发症。

Endocrine Complications of Cystic Fibrosis.

机构信息

Division of Endocrinology & Diabetes, University of Pennsylvania Perelman School of Medicine, Children's Hospital of Philadelphia, Room 14363, Roberts Building for Pediatric Research, 2716 South Street, Philadelphia, PA 19146, USA.

Division of Endocrinology & Diabetes, Department of Pediatrics, University of Pennsylvania Perelman School of Medicine, Children's Hospital of Philadelphia, Room 7547, The Hub for Clinical Collaboration, 3501 Civic Center Blvd, Philadelphia, PA 19104, USA.

出版信息

Clin Chest Med. 2022 Dec;43(4):773-789. doi: 10.1016/j.ccm.2022.06.013.

Abstract

Endocrine comorbidities have become increasingly important medical considerations as improving cystic fibrosis (CF) care increases life expectancy. Although the underlying pathophysiology of CF-related diabetes remains elusive, the use of novel technologies and therapeutics seeks to improve both CF-related outcomes and quality of life. Improvements in the overall health of those with CF have tempered concerns about pubertal delay and short stature; however, other comorbidities such as hypogonadism and bone disease are increasingly recognized. Following the introduction of highly effective modulator therapies there are many lessons to be learned about their long-term impact on endocrine comorbidities.

摘要

随着囊性纤维化(CF)治疗水平的提高,患者的预期寿命逐渐延长,内分泌合并症也成为了愈发重要的医学关注点。虽然 CF 相关糖尿病的潜在病理生理学仍难以捉摸,但新型技术和疗法的应用旨在改善 CF 相关结局和生活质量。CF 患者整体健康状况的改善减轻了人们对青春期延迟和身材矮小的担忧;然而,诸如性腺功能减退和骨病等其他合并症的问题日益凸显。在引入高效调节剂治疗后,我们从这些治疗对内分泌合并症的长期影响中学到了很多经验。

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