Cystic fibrosis natural history, complications and prognosis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: , Shaghayegh Habibi, M.D.[2]
Overview
If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
OR
Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
OR
Prognosis is generally excellent/good/poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [#]%.
Natural History
Malnutrition and poor growth (due to loss of pancreatic exocrine function) leads to death in the first decade of life for most untreated patients.[1]
Complications
Lung involvement:
In patients with cystic fibrosis the most significant changes and complications are seen in airways. The primary genetic defect causes chronic pulmonary infections. P. aeruginosa is the most common one, followed by S aureus and H influenzae.[2][3]
- Infancy: the most common bacteria cultured is S. aureus with H. influenzae increasing during childhood
- Adolescence and young adulthood: the commonest pathogen cultured is P. aeruginosa
Reproductive system involvement:
In cystic fibrosis 98% of men are infertile. Aspermia results from atresia or absent vasa deferentia and abnormal or absent seminal vesicles.[4]
Gastrointestinal involvement:
Prognosis
- Life expectancy of patients with cystic fibrosis has been increased over past decades because of better symptomatic treatment strategies.
- In patients with cystic fibrosis, obstructive lung disease and other lung complications are currently the primary causes of morbidity and are responsible for 80% of mortality.[1][5]
References
- ↑ 1.0 1.1 Cutting GR (2015). "Cystic fibrosis genetics: from molecular understanding to clinical application". Nat. Rev. Genet. 16 (1): 45–56. doi:10.1038/nrg3849. PMC 4364438. PMID 25404111.
- ↑ Edmondson C, Davies JC (2016). "Current and future treatment options for cystic fibrosis lung disease: latest evidence and clinical implications". Ther Adv Chronic Dis. 7 (3): 170–83. doi:10.1177/2040622316641352. PMC 4907071. PMID 27347364.
- ↑ Ratjen FA (2009). "Cystic fibrosis: pathogenesis and future treatment strategies". Respir Care. 54 (5): 595–605. PMID 19393104.
- ↑ Ratjen F, Döring G (2003). "Cystic fibrosis". Lancet. 361 (9358): 681–9. doi:10.1016/S0140-6736(03)12567-6. PMID 12606185.
- ↑ Pettit RS, Fellner C (July 2014). "CFTR Modulators for the Treatment of Cystic Fibrosis". P T. 39 (7): 500–11. PMC 4103577. PMID 25083129.