Cystic Fibrosis-Related Diabetes

Cystic fibrosis-related diabetes (CFRD) occurs in about

20% of adolescents with cystic fibrosis and is the most

common comorbidity in cystic fibrosis. The primary defect

is insulin insufficiency, exacerbated by insulin resistance

especially in times of illness or with glucocorticoid therapy.

The presence of CFRD is associated with worse nutritional

status, more severe lung disease, and greater mortality.

Patients with cystic fibrosis should be routinely screened

starting by age 10, and treatment has been shown to

improve outcomes.

in children. In borderline or asymptomatic cases, a fasting

plasma glucose level >125 mg/dL (7 mmol/L) or a plasma

glucose level above 200 mg/dL (11.1 mmol/L) 2 hours after

an oral glucose load (1.75 g glucose/kg up to a maximum of

75 g) on 2 separate days confirms the diagnosis. Impaired

(not yet diabetic) fasting glucose values are 100–125 mg/dL

(5.5–6.9 mmol/L) and impaired 2-hour values are 140–200 mg/

dL (7.8–11.1 mmol/L). Children with impaired fasting

glucose or impaired glucose tolerance and no islet autoantibodies

are at high risk of T2D and require careful follow-up

and lifestyle modification with weight loss, if obese. An

HbA1c ≥ 6.5 (48 mmol/L) is also diagnostic of diabetes. However,

most clinical laboratories do not perform HbA1c assays

that meet diagnostic criteria (ie, NGSP certified and standardized

to the Diabetes Control and Complications Trial reference

assay). For this reason HbA1c measurements are prone to

error and must be interpreted with caution, especially in the

absence of other signs or symptoms of diabetes. Additionally,

the HbA1c is less sensitive than blood glucose-based criteria

and may underestimate dysglycemia in young children whose

progression to T1D can be especially rapid.

Source : CURRENT Diagnosis and Treatment Pediatrics 24th ed 2018