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Cystic fibrosis-related diabetes: an update on pathophysiology, diagnosis, and treatment

  • Crésio Alves EMAIL logo , Thais Della-Manna and Cristiano Tulio Maciel Albuquerque
Published/Copyright: July 10, 2020
Journal of Pediatric Endocrinology and Metabolism
From the journal Journal of Pediatric Endocrinology and Metabolism Volume 33 Issue 7

Abstract

Cystic fibrosis (CF) is a highly prevalent autosomal recessive disorder that is caused by mutations in the CF transmembrane conductance regulator (CFTR) gene (7q31.2), which encodes the CFTR chloride-anion channel that is expressed in several tissues. Life expectancy has increased significantly over the past few decades due to therapeutic advances and early diagnosis through neonatal screening. However, new complications have been identified, including CF-related diabetes (CFRD). The earliest detectable glycemic abnormality is postprandial hyperglycemia that progresses into fasting hyperglycemia. CFRD is associated with a decline in lung function, impairments in weight gain and growth, pubertal development, and increased morbidity and mortality. Annual screening with oral glucose tolerance test is recommended beginning at the age of 10, and screenings are recommended for any age group during the first 48 h of hospital admission. Fasting plasma glucose levels ≥126 mg/dL (7.0 mmol/L) or 2-h postprandial plasma glucose levels ≥200 mg/dL (11.1 mmol/L) that persist for more than 48 h are diagnostic criteria for CFRD. Under stable health condition, the diagnosis is made when laboratory abnormalities in accordance with the American Diabetes Association criteria are detected for the first time; however, levels of HbA1c <6.5% do not rule out the diagnosis. Treatment for CFRD includes insulin replacement and a hypercaloric and hyperproteic diet that does not restrict carbohydrates, fats or salt, and diabetes self-management education. The most important CFRD complications are nutritional and pulmonary disease deterioration, though the microvascular complications of diabetes have already been described.

Keywords: cystic fibrosis; diabetes; pathophysiology; treatment
Corresponding author: Crésio Alves, Associate Professor of Pediatrics and Chief of the Pediatric Endocrinology Unit, Hospital Universitario Prof. Edgard Santos, Faculty of Medicine, Federal University of Bahia, Rua Plínio Moscoso, 222/601, Salvador, Bahia, 40.157-190, Brazil, Phone: +55 71 99178 4055, E-mail:

  1. Research funding: None declared.

  2. Author contribution: All the authors have accepted responsibility for the entire content of this submitted manuscript and approved its submission.

  3. Informed consent: Informed consent was obtained from all individuals included in this study.

  4. Ethical approval: This is a review article and the ethical statement therefore not required.

  5. Conflict of interest: Authors state no conflict of interest.

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Received: 2019-10-15
Accepted: 2020-03-10
Published Online: 2020-07-10
Published in Print: 2020-07-28

© 2020 Walter de Gruyter GmbH, Berlin/Boston

Articles in the same Issue

  1. Frontmatter
  2. Review Articles
  3. Definition and early diagnosis of metabolic syndrome in children
  4. Cystic fibrosis-related diabetes: an update on pathophysiology, diagnosis, and treatment
  5. Original Articles
  6. Association of sleep characteristics with adiposity markers in children
  7. Prevalence of abdominal obesity in non-obese adolescents: a North Indian adolescent study
  8. Utility of estimated glucose disposal rate for predicting metabolic syndrome in children and adolescents with type-1 diabetes
  9. Continuous glucose monitoring reduces pubertal hyperglycemia of type 1 diabetes
  10. Association between eating behavior, anthropometric and biochemical measurements, and peptide YY (PYY) hormone levels in obese adolescents in outpatient care
  11. Autoimmune hyperthyroidism in children & adolescents in Sudan: a 13 years’ experience of a Paediatric Endocrinology Clinic
  12. Timing, prevalence, and dynamics of thyroid disorders in children and adolescents affected with Down syndrome
  13. Assessment of the most common CYP21A2 point mutations in a cohort of congenital adrenal hyperplasia patients from Egypt
  14. Quality of life and associated factors in parents of children with late diagnosed phenylketonuria. A cross sectional study in a developing country (Tunisia)
  15. Genetic analysis and long-term treatment monitoring of 11 children with glycogen storage disease type IIIa
  16. Growth and metabolic effects of long-term recombinant human growth hormone (rhGH) treatment in short children born small for gestational age: GH-RAST study
  17. Menstrual cycle, reproductive function, body mass index, and metabolic profiles of women with former central precocious puberty: 10–20-year longitudinal cohort study in southern Thailand
  18. Letter to the Editors
  19. European Society of Paediatric Radiology (ESPR) Child Abuse Taskforce Committee: a response to Miller et al.
  20. The correct formula to calculate triglyceride-glucose index (TyG)
  21. Case Reports
  22. Octreotide-related exocrine pancreatic insufficiency (EPI) in congenital hyperinsulinism
  23. Improvement in glycaemic parameters using SGLT-2 inhibitor and GLP-1 agonist in combination in an adolescent with diabetes mellitus and Prader-Willi syndrome: a case report
  24. Three patients with glucose-6 phosphatase catalytic subunit 3 deficiency
  25. Efficacy and safety of denosumab treatment in a prepubertal patient with cherubism
https://doi.org/10.1515/jpem-2019-0484
Keywords for this article
cystic fibrosis; diabetes; pathophysiology; treatment

Articles in the same Issue

  1. Frontmatter
  2. Review Articles
  3. Definition and early diagnosis of metabolic syndrome in children
  4. Cystic fibrosis-related diabetes: an update on pathophysiology, diagnosis, and treatment
  5. Original Articles
  6. Association of sleep characteristics with adiposity markers in children
  7. Prevalence of abdominal obesity in non-obese adolescents: a North Indian adolescent study
  8. Utility of estimated glucose disposal rate for predicting metabolic syndrome in children and adolescents with type-1 diabetes
  9. Continuous glucose monitoring reduces pubertal hyperglycemia of type 1 diabetes
  10. Association between eating behavior, anthropometric and biochemical measurements, and peptide YY (PYY) hormone levels in obese adolescents in outpatient care
  11. Autoimmune hyperthyroidism in children & adolescents in Sudan: a 13 years’ experience of a Paediatric Endocrinology Clinic
  12. Timing, prevalence, and dynamics of thyroid disorders in children and adolescents affected with Down syndrome
  13. Assessment of the most common CYP21A2 point mutations in a cohort of congenital adrenal hyperplasia patients from Egypt
  14. Quality of life and associated factors in parents of children with late diagnosed phenylketonuria. A cross sectional study in a developing country (Tunisia)
  15. Genetic analysis and long-term treatment monitoring of 11 children with glycogen storage disease type IIIa
  16. Growth and metabolic effects of long-term recombinant human growth hormone (rhGH) treatment in short children born small for gestational age: GH-RAST study
  17. Menstrual cycle, reproductive function, body mass index, and metabolic profiles of women with former central precocious puberty: 10–20-year longitudinal cohort study in southern Thailand
  18. Letter to the Editors
  19. European Society of Paediatric Radiology (ESPR) Child Abuse Taskforce Committee: a response to Miller et al.
  20. The correct formula to calculate triglyceride-glucose index (TyG)
  21. Case Reports
  22. Octreotide-related exocrine pancreatic insufficiency (EPI) in congenital hyperinsulinism
  23. Improvement in glycaemic parameters using SGLT-2 inhibitor and GLP-1 agonist in combination in an adolescent with diabetes mellitus and Prader-Willi syndrome: a case report
  24. Three patients with glucose-6 phosphatase catalytic subunit 3 deficiency
  25. Efficacy and safety of denosumab treatment in a prepubertal patient with cherubism
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