Department of Pediatrics, University of Iowa Stead Family Children's Hospital, Iowa City, IA, USA; Fraternal Order of Eagles Diabetes Research Center, University of Iowa, Iowa City, IA, USA.
Children's Hospital Colorado, University of Colorado, Aurora, CO, USA; Barbara Davis Center for Childhood Diabetes, Aurora, CO, USA.
J Cyst Fibros. 2019 Oct;18 Suppl 2:S10-S18. doi: 10.1016/j.jcf.2019.08.003.
Cystic Fibrosis Related Diabetes Mellitus (CFRD) drives excess pulmonary morbidity and mortality in patients with cystic fibrosis (CF). The recommended treatment is insulin therapy. Insulin therapy in CF should be customized to the specific patient. CF patients typically require intensive insulin regimens such as multiple daily injections or insulin pump therapy, but frequently require lower doses than in type 1 diabetes mellitus. Patients with CF may also need insulin to cover intravenous or enteral feedings. Pre-diabetic glycaemic abnormalities are also associated with clinical decline in cystic fibrosis prior to the diagnosis of CFRD, however, whether and how this should be treated is not fully determined. There is also interest, but inadequate data regarding other treatments besides insulin (i.e., oral medications) for treatment of pre-diabetes or CFRD. CFTR potentiator and corrector therapy has yet to demonstrate an effect on the rate of CFRD, but may improve insulin secretion. There is great opportunity for further research to better understand when and how best to treat glycaemic abnormalities in cystic fibrosis.
囊性纤维化相关性糖尿病（CFRD）可导致囊性纤维化（CF）患者肺部发病率和死亡率升高。推荐的治疗方法是胰岛素治疗。CF 患者的胰岛素治疗应根据具体患者进行定制。CF 患者通常需要强化胰岛素治疗方案，如多次皮下注射或胰岛素泵治疗，但通常需要的剂量低于 1 型糖尿病。CF 患者还可能需要胰岛素来覆盖静脉或肠内喂养。在诊断 CFRD 之前，糖尿病前期的血糖异常也与囊性纤维化的临床恶化有关，然而，是否以及如何治疗尚未完全确定。此外，关于除胰岛素（即口服药物）以外的其他治疗方法治疗糖尿病前期或 CFRD 的兴趣也很大，但数据不足。CFTR 增效剂和校正剂治疗尚未显示对 CFRD 发生率有影响，但可能改善胰岛素分泌。有很大的机会进行进一步的研究，以更好地了解何时以及如何最好地治疗囊性纤维化中的血糖异常。
