Diabetes and prediabetes in children with cystic fibrosis

Abstract

Purpose of review Glucose metabolism alterations in cystic fibrosis range from the classic cystic fibrosis-related diabetes (CFRD) to forms of glucose intolerance and prediabetes. The aim of the present work is to review the most up-to-date novelties in terms of CFRD diagnosis and therapy. This review is timely and relevant because it allows an update for the early and correct classification of glucose abnormalities in cystic fibrosis and because it favours an appropriate therapeutic approach. Recent findings Confirm that Oral Glucose Tolerance Test is still the diagnostic gold standard despite the advent of continuous glucose monitoring (CGM) systems; this latter is spreading very rapidly, however, to date, there is still no strong evidence to hypothesize the use of CGM for diagnostic purposes. CGM has indeed proven to be very useful in managing and guiding CFRD therapy. Summary Tailored and personalized insulin therapy is still the recommended therapy for children and adolescents with CFRD, although nutritional intervention and oral hypoglycaemic treatment are equally important and efficacious. Finally CFTR modulators have allowed the increase of the life expectancy of cystic fibrosis patients and have proven effective not only in improving the pulmonary function and the nutritional status but also the glucose control.

An Update in Cystic Fibrosis-Related Diabetes in Children and Adolescents

“…CFRD frequently presents with an absence of overt symptoms, leading to prolonged periods of undiagnosed status. As highlighted by Schiaffini et al (2023), various forms of overt diabetes and prediabetes can exhibit a convergence of early glucose metabolism anomalies [31]. Acute and abrupt onsets, typified by ketoacidosis, are exceedingly rare, although they may be encountered in certain instances, such as during respiratory infections or when corticosteroids therapy is administered, accentuating the tissue resistance to insulin.…”

“…Currently, the established standard for screening and diagnosing CFRD is the OGTT. Both the Cystic Fibrosis Foundation (CFF) and the American Diabetes Association (ADA) recommend conducting this test annually for all CF patients aged 10 years and older [10,31,33]. Although the International Society for Pediatric and Adolescent Diabetes (ISPAD) guidelines recommend initiating CFRD screening at the age of 10 years, some studies propose an earlier start, either from the age of 6 years or after 3 years following the CF diagnosis.…”

“…The primary and recommended treatment modality for children and adolescents diagnosed with CFRD is insulin. It is noteworthy that children with CFRD exhibit a higher sensitivity to insulin compared to those with T1DM [31]. Insulin, an anabolic hormone, plays a critical role in regulating glucose metabolism.…”

“…Presently, several clinically effective insulin regimens are available, and the choice of regimen should be tailored based on the patient's glycemic fluctuations, disease severity, and the specific insulin preparations at hand, in accordance with insights from Ode et al in 2019 [67]. These diverse regimens include long-acting insulin administered once daily, intensified insulin treatment involving multiple daily injections with prandial insulin supplements, typically using rapid-acting insulin analogs, or insulin pump therapy that delivers continuous basal insulin with boluses at mealtimes [31,69]. It is important to note that a consensus regarding the optimal insulin regimen for CFRD management has yet to be established [6].…”

“…CFRD with fasting hyperglycemia: Noted when the fasting glucose levels are >7 mmol/L (>126 mg/dL) and the 2-h plasma glucose levels are ≥11.1 mmol/L (≥200 mg/dL) [4,14,31,34,36,37].…”