Editorial

Cystic Fibrosis: Not Just an Exocrinopathy Anymore?

Authors: Denis Hadjiliadis, MD, MHS, FRCP(C), FCCP

Abstract

Cystic fibrosis (CF) is the most common life-shortening genetic disease in whites. Recent progress has resulted in improvement in median survival for patients in the Cystic Fibrosis Foundation Registry from 16 years in 1970 to 36.5 in 2005.1 In the early years, it was thought that the disease was mainly confined to the exocrine portion of the endocrine system. The major manifestations were malnutrition, diarrhea, and steatorrhea because of exocrine pancreatic insufficiency, sinus disease, bronchiectasis, respiratory infections, and respiratory failure due to abnormally thick and tenacious mucous in the respiratory system. Diabetes mellitus was initially a rare occurrence and it was thought that it was not a manifestation of CF.2 However, as survival increased, and patients grew older, it became obvious that a significant percentage developed diabetes. Currently, approximately 30% of adult CF patients over 30 years have CF-related diabetes.1 The current prevalence of diabetes in adult CF patients provides an example of the evolution of the disease over the last few decades.

This content is limited to qualifying members.

Existing members, please login first

If you have an existing account please login now to access this article or view purchase options.

Purchase only this article ($25)

Create a free account, then purchase this article to download or access it online for 24 hours.

Purchase an SMJ online subscription ($75)

Create a free account, then purchase a subscription to get complete access to all articles for a full year.

Purchase a membership plan (fees vary)

Premium members can access all articles plus recieve many more benefits. View all membership plans and benefit packages.

References

1. Cystic Fibrosis Foundation. Patient Registry 2005 Annual Data Report. Bethesda, MD, Cystic Fibrosis Foundation, 2006.
 
2. Moran A, Hardin D, Rodman D, et al. Diagnosis, screening and management of cystic fibrosis related diabetes mellitus: a consensus conference report. Diabetes Res Clin Pract 1999;45:61–73.
 
3. Soriano EPB, Fischman D, Cheriyath P. Membranoproliferative glomerulonephritis in patients with cystic fibrosis: coincidence or comorbidity? a case series. South Med J 2008;101:641–645.
 
4. Bertenshaw C, Watson AR, Lewis S, et al. Survey of acute renal failure in patients with cystic fibrosis in the UK. Thorax 2007;62:541–545.
 
5. Al-Aloul M, Miller H, Stockton P, et al. Acute renal failure in CF patients infected by the Liverpool epidemic Pseudomonas aeruginosa strain (LES). J Cyst Fibros 2005;4:197–201.
 
6. Allen JL. Progressive nephropathy in a patient with cystic fibrosis and diabetes. N Engl J Med 1986;315:764.
 
7. Smith KD, Alpers CE. Pathogenic mechanisms in membranoproliferative glomerulonephritis. Curr Opin Nephrol Hypertens 2005;14:396–403.