Bridges N, Rowe R, Holt R I G
Chelsea and Westminster Hospital, London and the Royal Brompton Hospital, London, UK.
University Hospital of South Manchester, Manchester, UK.
Diabet Med. 2018 Apr 23. doi: 10.1111/dme.13652.
Individuals with cystic fibrosis and pancreatic insufficiency have a gradual decline in insulin secretion over time, which results in an increase in the prevalence of diabetes with age; up to 50% of adults with cystic fibrosis aged over 35 years have diabetes. Cystic fibrosis-related diabetes differs from Type 1 and Type 2 diabetes in several ways; there is a pattern of insulin deficiency with reduced and delayed insulin response to carbohydrates but a sparing of basal insulin that results in glucose abnormalities, which are frequently characterized by normal fasting glucose and postprandial hyperglycaemia. Insulin deficiency and hyperglycaemia, even at levels which do not reach the threshold for a diagnosis of diabetes, have an adverse impact on lung function and clinical status in people with cystic fibrosis. Although the risk of microvascular complications occurs as in other forms of diabetes, the main reason for treatment is to prevent deterioration in lung function and weight loss; treatment may therefore be required at an earlier stage than for other types of diabetes. Treatment is usually with insulin, but management needs to take into account all the other medical issues that arise in cystic fibrosis.
患有囊性纤维化和胰腺功能不全的个体，其胰岛素分泌会随着时间逐渐下降，这导致糖尿病患病率随年龄增长而增加；35岁以上的成年囊性纤维化患者中，高达50%患有糖尿病。囊性纤维化相关糖尿病在几个方面不同于1型和2型糖尿病；存在胰岛素缺乏的模式，对碳水化合物的胰岛素反应减少且延迟，但基础胰岛素相对保留，从而导致血糖异常，其特征通常是空腹血糖正常和餐后高血糖。即使胰岛素缺乏和高血糖水平未达到糖尿病诊断阈值，也会对囊性纤维化患者的肺功能和临床状况产生不利影响。虽然微血管并发症的风险与其他形式的糖尿病一样存在，但治疗的主要原因是防止肺功能恶化和体重减轻；因此，可能需要比其他类型糖尿病更早开始治疗。治疗通常使用胰岛素，但管理需要考虑到囊性纤维化中出现的所有其他医疗问题。
