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
Internal Medicine, Glangwili General Hospital, Carmarthen, GBR.
Medical Education and Simulation, Postgraduate Medical Institute, Peshawar, PAK.
Cureus. 2025 May 14;17(5):e84110. doi: 10.7759/cureus.84110. eCollection 2025 May.
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.
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。