Cystic fibrosis-related diabetes

Czech version

Authors: Koloušková Stanislava; Skalická Veronika; Bartošová Jana; Lebl Jan
Authors‘ workplace: Praha ; Pediatrická klinika, 2. lékařská, fakulta, Univerzita Karlova, a Fakultní nemocnice v Motole
Published in: Čes-slov Pediat 2023; 78 (5): 258-260.
Category:
doi: https://doi.org/10.55095/CSPediatrie2023/043

Overview

Cystic fibrosis (CF) is an autosomal recessive disorder. It is caused by mutations in CFTR gene that lead to chloride channel dysfunction and consequent changes in composition and properties of the secrets. The clinical presentation is dominated by the involvement of the lungs and pancreas. Thanks to novel therapies, the life expectancy of CF patients has improved significantly within the past years; anyway, the complication rate has also increased. One of them is cystic fibrosis-related diabetes (CFRD). After the age of 30 years old, up to 50 % of patients are currently developing impaired glucose tolerance or overt CFRD. Therefore regular screening is provided using oGTT, and new technologies such as continuous glucose monitoring (sensor) will also be employed in the near future. According to current guidelines, early insulin treatment is recommended. In advanced stages, insulin pump treatment linked with a sensor (hybrid closed loop system) is helpful. The CFRD diet differs from diets in other types of diabetes. Neither energy nor fat intake is reduced. Carbohydrate consumption is only partially restricted, especially the pop drinks.

Keywords:

screening – therapy – cystic fibrosis related diabetes (CFRD)

Sources

1. Rosan RC, Shwachman H, Kulczycki I. Diabetes mellitus and cystic fibrosis of the pancreas. Am J Dis Child 1962; 104: 625–63.

2. Moran A, Hardin D, Rodman, et al. Diagnosis, screening and management of cystic fibrosis related diabetes mellitus. A consensus conference report. Diabet Res Clin Pract 1999; 45: 61–73.

3. Finkelstain SM, Wielinski CL, Elliot GR, et al. Diabetes mellitus associated with cystic fibrosis. J Pediatr 1988; 112: 373–377.

4. Hardin D, Leblanc A, Para L, Seilheimer DK. Hepatic insulin resistance and defects in substrate utilization in cystic fibrosis. Diabetes 1999; 49: 082–1087.

5. Hardin D, Leblanc A, Marschall G, et al. Mechanism of insulin resistance in cystic fibrosis. Am J Physiol Endocrinol Metab 2001; 281: 1022–1028.

6. Lanng S, Thorsteinsson B, Nerup J, et al. Influence of the development of diabetes mellitus on clinical status in patients with cystic fibrosis. Eur J Pediatr 1992; 15: 684–697.

7. Ionescu AA, Nixon LS, Luzio S, et al. Pulmonary function, body composition, and protein catabolism in adults with cystic fibrosis. Am J Respir Crit Care Med 2002; 165(4): 495–500.

8. Ode KL, Ballman M, Batterrati A, et al. ISPAD Clinical Practice Consensus Guidelines 2022: Management of cystic fibrosis-related diabetesin children and adolescents. Pediatr Diabetes 2022; 23: 1212–1228.

9. Wanke T, Formanek D, Auinger M, et al. Inspiratory muscle performance and pulmonary function changes in insulin-dependent diabetes mellitus. Am Rev Respir Dis 1991; 143: 97–100.

10. Lohr M, Goerchen P, Nizze H, et al. CF associated islet changes may provide a basis for diabetes: a immunocytological and morphometrical study. Virchow Ach A Pathol Anat 1989; 414: 179–185.

11. Lanng S, Thorsteinsson B, Nerup J, et al. Diabetes mellitus in cystic fibrosis: effect of insulin therapy on lung function and infections. Acta Paediatr 1994; 83: 849–853.

12. Nousia-Arvanitakis S, Galli-Tsinopoulou A, Karamouzis M. Insulin improves clinical status of patients with cystic fibrosis related diabetes mellitus. Acta Paediatr 2001; 90: 515–519