Original articleGlucose Tolerance and Insulin Secretion, Morbidity, and Death in Patients with Cystic Fibrosis
Section snippets
Study Population and Design
The study included 237 patients with CF (109 boys, 128 girls), followed up at Necker-Enfants Malades Hospital between 1988 and 2005. The diagnosis of CF was based on typical clinical manifestations and a positive sweat testing result. The common CFTR mutation ΔF508 was present in 90% of the patients, and all had exocrine pancreatic deficiency.
Glucose tolerance was evaluated by use of serial OGTT. 517 OGTTs were performed in 206 patients (2.5 ± 1.3 per patient, range 1 to 7). 31 patients had
Age and Abnormalities of Glucose Tolerance
The longitudinal follow-up of glucose tolerance, performed with all OGTTs, showed that abnormalities of glucose tolerance appeared early in life, as 20% of the patients had IGT at 10 years of age (Figure 1). The cumulative risk increased with age to 82% at 30 years. The figures for diabetes were >20% at 15 years, 45% at 20 years, and 70% at 30 years. Thirty percent of the patients were on insulin at 20 years and 40% at 30 years. Abnormalities tended to be earlier in girls (Figure 2; available
Discussion
Our study of 237 young patients with CF, by use of serial OGTTs, shows that abnormalities of glucose tolerance appeared early in life and increased with age to high rates in the third decade. They were associated with a higher rate of pulmonary deterioration requiring transplantation and with earlier death. Abnormalities of glucose metabolism were mainly correlated with deficient insulin secretion, and insulin resistance might be an additional but not predominant factor. These results are of
References (38)
- et al.
Abnormal glucose metabolism in cystic fibrosis
J Pediatr
(1998) - et al.
Insulin resistance is associated with decreased clinical status in cystic fibrosis
J Pediatr
(1997) - et al.
Insulin sensitivity and metabolic clearance rate of insulin in cystic fibrosis
Metabolism
(1994) - et al.
Diabetes mellitus associated with cystic fibrosis
J Pediatr
(1988) Glucose intolerance in cystic fibrosis patients
Paediatr Respir Rev
(2001)- et al.
Genetic determination of diabetes mellitus in patients with cystic fibrosisMulticenter Cystic Fibrosis Study Group
J Pediatr
(1995) - et al.
Epidemiology of cystic fibrosis-related diabetes
J Pediatr
(2005) Cystic Fibrosis Foundation Patient Registry Annual Data Report, 1998
(1999)- et al.
Diabetes mellitus in Danish cystic fibrosis patients: prevalence and late diabetic complications
Acta Paediatr
(1994) - et al.
Longitudinal evaluation of glucose tolerance and insulin secretion in non-diabetic children and adolescents with cystic fibrosis: results of a two-year follow-up
Acta Paediatr
(1993)
Insulin sensitivity in cystic fibrosis
Diabetes
Roles of insulin resistance and beta-cell dysfunction in the pathogenesis of glucose intolerance in cystic fibrosis
J Clin Endocrinol Metab
First-phase insulin response to intravenous glucose in cystic fibrosis patients with different degrees of glucose tolerance
J Pediatr Endocrinol
Insulin sensitivity and insulin clearance in cystic fibrosis patients with normal and diabetic glucose tolerance
Clin Endocrinol (Oxf)
Cystic Fibrosis Foundation Patient Registry Annual Data Report, 1997
Influence of the development of diabetes mellitus on clinical status in patients with cystic fibrosis
Eur J Pediatr
Body mass index variations: centiles from birth to 87 years
Eur J Clin Nutr
Use of the oral glucose tolerance test to assess insulin release and insulin sensitivity
Diabetes Care
Use and abuse of HOMA modeling
Diabetes Care
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