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本文引用的文献

1
Fatty acid abnormalities in cystic fibrosis-the missing link for a cure?囊性纤维化中的脂肪酸异常——治愈的关键环节缺失?
iScience. 2024 Oct 11;27(11):111153. doi: 10.1016/j.isci.2024.111153. eCollection 2024 Nov 15.
2
The ageing of people living with cystic fibrosis: what to expect now?囊性纤维化患者的老龄化:现在有哪些预期?
Eur Respir Rev. 2024 Oct 30;33(174). doi: 10.1183/16000617.0071-2024. Print 2024 Oct.
3
Cystic fibrosis liver disease in the new era of cystic fibrosis transmembrane conductance regulator (CFTR) modulators.囊性纤维化跨膜电导调节因子(CFTR)调节剂时代的囊性纤维化肝病。
Paediatr Respir Rev. 2024 Jun;50:54-61. doi: 10.1016/j.prrv.2023.12.005. Epub 2023 Dec 28.
4
An Update in Cystic Fibrosis-Related Diabetes in Children and Adolescents.儿童和青少年囊性纤维化相关糖尿病的最新进展
Children (Basel). 2023 Nov 30;10(12):1879. doi: 10.3390/children10121879.
5
Advances in cystic fibrosis-related diabetes: Current status and future directions.囊性纤维化相关糖尿病的进展:现状与未来方向。
Diabetes Metab Syndr. 2023 Nov;17(11):102899. doi: 10.1016/j.dsx.2023.102899. Epub 2023 Nov 2.
6
The Bidirectional Link Between Diabetes and Kidney Disease: Mechanisms and Management.糖尿病与肾脏疾病之间的双向联系:机制与管理
Cureus. 2023 Sep 20;15(9):e45615. doi: 10.7759/cureus.45615. eCollection 2023 Sep.
7
Effects of insulin therapy optimization with sensor augmented pumps on glycemic control and body composition in people with cystic fibrosis-related diabetes.传感器增强型胰岛素泵优化胰岛素治疗对囊性纤维化相关性糖尿病患者血糖控制和身体成分的影响。
Front Endocrinol (Lausanne). 2023 Aug 31;14:1228153. doi: 10.3389/fendo.2023.1228153. eCollection 2023.
8
The Effects of Elexacaftor, Tezacaftor, and Ivacaftor (ETI) on Blood Glucose in Patients With Cystic Fibrosis: A Systematic Review.依列卡福妥、替扎卡福妥和依伐卡托(ETI)对囊性纤维化患者血糖的影响:一项系统评价。
Cureus. 2023 Jul 11;15(7):e41697. doi: 10.7759/cureus.41697. eCollection 2023 Jul.
9
Cystic Fibrosis-Related Diabetes Workshop: Research Priorities Spanning Disease Pathophysiology, Diagnosis, and Outcomes.囊性纤维化相关性糖尿病研讨会:涵盖疾病病理生理学、诊断和结局的研究重点。
Diabetes. 2023 Jun 1;72(6):677-689. doi: 10.2337/db22-0949.
10
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Diabetes Metab. 2023 May;49(3):101444. doi: 10.1016/j.diabet.2023.101444. Epub 2023 Apr 6.

当两个世界碰撞:应对囊性纤维化合并糖尿病

When Two Worlds Collide: Navigating Diabetes in Cystic Fibrosis.

作者信息

Ahmad Wisal, Khan Jansher, Hashmat Ahmad J, Khan Anusha, Ahmad Aftab, Dildar Jalal, Sikander Meenal, Ahmad Sabeena, Zakir Shayan, Raza Syed S, Awan Saira K, Varrassi Giustino

机构信息

Internal Medicine, Glangwili General Hospital, Carmarthen, GBR.

Medical Education and Simulation, Postgraduate Medical Institute, Peshawar, PAK.

出版信息

Cureus. 2025 May 14;17(5):e84110. doi: 10.7759/cureus.84110. eCollection 2025 May.

DOI:10.7759/cureus.84110
PMID:40519490
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12166097/
Abstract

Cystic fibrosis (CF) is an autosomal recessive genetic disorder characterized by the dysfunction of the cystic fibrosis transmembrane conductance regulator (CFTR) gene on the long (q) arm of chromosome number 7. It is characterized by the buildup of thick, sticky mucus that can damage various body organs. The most commonly affected organs are the pancreas, liver, intestines, and lungs. Diabetes mellitus is a complex multi-system metabolic disorder. It is characterized by the relative or absolute insufficiency of insulin secretion with or without concomitant insulin resistance, leading to high blood sugar levels. One of the feared complications of CF is cystic fibrosis-related diabetes (CFRD). In this narrative review, we examine various treatment options, their mechanisms of action, their side effects, and their impact on the lives of patients with CFRD. In conclusion, insulin remains the cornerstone of treatment in the management of CFRD. However, oral medications for diabetes can also be considered safe and effective, in selected patients, with stable liver function and preserved lung capacity.

摘要

囊性纤维化(CF)是一种常染色体隐性遗传疾病,其特征是7号染色体长臂上的囊性纤维化跨膜传导调节因子(CFTR)基因功能异常。其特点是会积聚浓稠、黏性的黏液,可损害身体各个器官。最常受影响的器官是胰腺、肝脏、肠道和肺部。糖尿病是一种复杂的多系统代谢紊乱疾病。其特征是胰岛素分泌相对或绝对不足,伴或不伴有胰岛素抵抗,导致血糖水平升高。CF令人担忧的并发症之一是囊性纤维化相关糖尿病(CFRD)。在这篇叙述性综述中,我们研究了各种治疗方案、它们的作用机制、副作用以及它们对CFRD患者生活的影响。总之,胰岛素仍然是CFRD治疗的基石。然而,对于肝功能稳定且肺功能良好的特定患者,糖尿病口服药物也可被认为是安全有效的。