Cystic-fibrosis related-diabetes (CFRD) is preceded by and associated with growth failure and deteriorating lung function

Abstract

Background: Impaired glucose metabolism and cystic fibrosis (CF)-related diabetes (CFRD) are associated with insufficient weight gain and impaired lung function in children and adolescents with CF. We have asked whether imminent CFRD may be a cause of poor growth in children and adolescents. Methods: A retrospective case control study including 32 patients with CF with or without diabetes was conducted. Sixteen pairs, matched according to age, gender and exocrine pancreatic insufficiency, were analysed. Standard deviation scores (SDS) of height, growth, weight, body mass index (BMI), forced vital capacity (FVC), forced expiratory volume in the first second (FEV1) and forced expiratory flow at 75% of expired FVC (FEF75) were recorded during a mean observation period of 13 years per patient. Results: SDS of height and weight were reduced in CF patients with diabetes compared to those without, not only at the point of diagnosis (both p < 0.05) but years before the evidence of diabetes. Afterwards there was a significant decline in height (p < 0.001) and weight (p < 0.01) SDS in CFRD patients and an increasing difference between the height and weight of CF patients with or without diabetes. In contrast, no significant reduction of BMI-SDS was observed in CFRD patients. All analysed lung function parameters showed a marked decline in CFRD patients starting 1 year prior to the diagnosis of diabetes. Conclusions: Deteriorating growth, reduced weight and impaired lung function are related to the development of CFRD and are obvious several years before the actual diagnosis of diabetes.

Impaired glucose tolerance, body mass index and respiratory function in patients with cystic fibrosis: A systematic review

“…Although patients with CF may eventually be diagnosed with CFRD, impaired glucose tolerance (IGT) may be present before CFRD is diagnosed . Decline in glucose metabolism can precipitate growth of bacteria in the lungs, which decreases lung function and survival . While, it is well established that CFRD leads to a worsening in lung function and nutritional status, the impact of impaired glucose tolerance on lung function and nutritional status continues to be investigated.…”

Ventilation inhomogeneity is associated with OGTT‐derived insulin secretory defects in cystic fibrosis

“…Unlike type 1 and type 2 diabetes, macrovascular complications are not observed, while microvascular complications have been occasionally reported . However, CFRD has a deleterious effect on nutritional status and pulmonary function, starting years before its onset, and it has been considered one of the main risk factor for mortality …”

Diabetes mellitus in pediatric solid organ recipients without and with cystic fibrosis: An analysis from the German-Austrian diabetes database (Diabetes Patienten Verlaufsdokumentation)

“…PTDM and CFRD share some common aspects like reduced first‐phase insulin secretion, elements of insulin resistance, and the absence of antibodies . Underweight is a significant risk factor for the development of diabetes in CF . The incidence of thinness is greatly increased in CF after SOT and might further aggravate the risk of diabetes development …”

“…9 Underweight is a significant risk factor for the development of diabetes in CF. 11,12 The incidence of thinness is greatly increased in CF after SOT and might further aggravate the risk of diabetes development. 13 The diagnosis of diabetes in SOT patients may be delayed or the onset of diabetes may remain unrecognized.…”