Abstract
Background: Many studies have shown that CFRD has a negative impact on CF prognosis. Current guidelines advise to screen for CFRD with an OGTT yearly from the age of 10 - 12. In our center we do not routinely screen for CFRD because the OGTT is cumbersome and not an ideal screening test. We therefore want to exclude unfavourable clinical evolution due to late diagnosis. Methods: 23 CF patients with diagnosis of CFRD < 18 years old were matched to a control patient. Clinical evolution (BMI, lung function, and chronic infection treatment burden) was analyzed starting 2 years before until 2 years after diagnosis of CFRD. Results: In the 2 years before diagnosis of CFRD, BMI and LF were similar for both groups while need for IV AB treatment was higher in the CFRD group. In the 2 years following diagnosis and treatment, LF decline was worse in the CFRD despite more IV AB treatments. BMI was still comparable. We conclude that clinical status was comparable between cases and controls in the 2 years preceding the diagnosis of CFRD. However, the need for IV antibiotic treatment seems to precede the faster lung function decline after CFRD diagnosis.
Highlights
Cystic fibrosis (CF) is a life-shortening, multi-organ disease, caused by mutations in the CF transmembrane conductance regulator (CFTR) gene
Baseline disease characteristics were similar in both groups apart from median HbA1C at time of diagnosis which was higher in the CF related diabetes (CFRD) group (p < 0.001)
In a large data set from the European Epidemiologic Registry of Cystic Fibrosis (ERCF) (7566 CF patients), mean FEV1 % was significantly lower in pediatric patients diagnosed with CFRD [5]
Summary
Cystic fibrosis (CF) is a life-shortening, multi-organ disease, caused by mutations in the CF transmembrane conductance regulator (CFTR) gene. CF related diabetes (CFRD) is one of the most common comorbidities of this disease [2] with about 50% of CF patients developing diabetes by the age of 30 years old. Many studies have shown that CFRD has a negative impact on CF prognosis, with worse nutritional status, more severe lung disease and higher mortality [4]-[12]. Clinical evolution (BMI, lung function, and chronic infection treatment burden) was analyzed starting 2 years before until 2 years after diagnosis of CFRD. We conclude that clinical status was comparable between cases and controls in the 2 years preceding the diagnosis of CFRD. The need for IV antibiotic treatment seems to precede the faster lung function decline after CFRD diagnosis
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