Sandouk Zahrae, Khan Farah, Khare Swapnil, Moran Antoinette
Internal Medicine, Division of Metabolism, Endocrinology & Diabetes, University of Michigan, Ann Arbor, MI, USA.
Internal Medicine, Division of Metabolism, Endocrinology and Nutrition, University of Washington, Seattle, WA, USA.
J Clin Transl Endocrinol. 2021 Nov 19;26:100278. doi: 10.1016/j.jcte.2021.100278. eCollection 2021 Dec.
Cystic fibrosis related diabetes (CFRD) occurs in at least 40-50% of adults with CF. With other forms of diabetes, microvascular and macrovascular disease are the major causes of morbidity and mortality. Macrovascular disease is rare in CF. While microvascular disease does occur in this population, there are CF-specific diabetes complications that have a more important impact on prognosis. The additional diagnosis of diabetes in CF is associated with decreased lung function, poor nutritional status, and an overall increase in mortality from lung disease. These negative findings start even before the clinical diagnosis of CFRD, during the period when patients experience abnormal glucose tolerance related to insulin insufficiency. The main mechanisms by which CFRD negatively affects prognosis are thought to be a combination of 1) protein catabolism, decreased lean body mass and undernutrition resulting from insulin insufficiency, and 2) an increased pro-inflammatory and pro-infectious state related to intermittent hyperglycemia. With the introduction of CFTR modulators, the care of CF patients has been revolutionized and many aspects of CF health such as BMI and lung function are improving. The impact of these drugs on the adverse prognosis related to the diagnosis of diabetes in CF, as well as the potential to delay or prevent onset of CFRD remain to be determined.
囊性纤维化相关糖尿病（CFRD）发生在至少40%-50%的成年囊性纤维化患者中。在其他类型的糖尿病中，微血管和大血管疾病是发病和死亡的主要原因。大血管疾病在囊性纤维化患者中很少见。虽然微血管疾病确实在该人群中发生，但存在一些特定于囊性纤维化的糖尿病并发症，它们对预后有更重要的影响。囊性纤维化患者中糖尿病的额外诊断与肺功能下降、营养状况差以及肺部疾病导致的总体死亡率增加有关。这些负面结果甚至在CFRD临床诊断之前就已出现，即在患者出现与胰岛素不足相关的葡萄糖耐量异常期间。CFRD对预后产生负面影响的主要机制被认为是以下两者的结合：1）胰岛素不足导致的蛋白质分解代谢、瘦体重减少和营养不良，以及2）与间歇性高血糖相关的促炎和促感染状态增加。随着囊性纤维化跨膜传导调节因子（CFTR）调节剂的引入，囊性纤维化患者的护理发生了革命性变化，囊性纤维化健康的许多方面，如体重指数（BMI）和肺功能都在改善。这些药物对囊性纤维化患者糖尿病诊断相关不良预后的影响，以及延迟或预防CFRD发病的潜力仍有待确定。
