Cystic fibrosis epidemiology and demographics

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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]

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Overview

The incidence of cystic fibrosis is approximately 1 in 2500 livebirths. It is a life-limiting disease (100% mortality rate), and a cure for the disease remains elusive. Most patients with cystic fibrosis are diagnosed in first 2 years of life. The onset of symptoms is before the first month of life in 12%, between 1-6 months of age in 75%, and between 6-12 months of age in 7% of patients. Although cystic fibrosis has been reported in all racial and ethnic groups, it mostly affects Caucasians of Northern European descent. It affects men and women equally.

Epidemiology and Demographics

Incidence

  • The incidence of cystic fibrosis is approximately 40 in 100,000 live births worldwide.
  • The incidence of cystic fibrosis is approximately 25 in 100,000 newborns in the US.[1][2]

Prevalence

  • Cystic fibrosis affects more than 30,000 people in the United States and 80,000 people worldwide.[3]

Mortality rate

  • Cystic fibrosis is a life-limiting disease (100% mortality rate), and a cure for the disease remains elusive.[4]

Age

  • Most patients with cystic fibrosis are diagnosed in first 2 years of life. The onset of symptoms is before the first month of life in 12%, between 1-6 months of age in 75%, and between 6-12 months of age in 7% of patients.[5]

Race

  • Although cystic fibrosis has been reported in all racial and ethnic groups, it mostly affects Caucasians of Northern European descent.
  • Cystic fibrosis is the most lethal genetic disease among Caucasians
  • Cystic fibrosis is approximately reported in different ethnic groups as follow:[6][7]
Prevalence of cystic fibrosis according to race
Race Prevalence (per 100,000 person years)
Caucasian 40
Hispanic 7.4
African-American 6.6

Gender

  • Cystic fibrosis affects men and women equally. Women with cystic fibrosis have a shortened life expectancy compared to men.[8]

References

  1. Farrell PM, White TB, Ren CL, Hempstead SE, Accurso F, Derichs N, Howenstine M, McColley SA, Rock M, Rosenfeld M, Sermet-Gaudelus I, Southern KW, Marshall BC, Sosnay PR (February 2017). "Diagnosis of Cystic Fibrosis: Consensus Guidelines from the Cystic Fibrosis Foundation". J. Pediatr. 181S: S4–S15.e1. doi:10.1016/j.jpeds.2016.09.064. PMID 28129811.
  2. Ratjen F, Döring G (2003). "Cystic fibrosis". Lancet. 361 (9358): 681–9. doi:10.1016/S0140-6736(03)12567-6. PMID 12606185.
  3. Brown SD, White R, Tobin P (May 2017). "Keep them breathing: Cystic fibrosis pathophysiology, diagnosis, and treatment". JAAPA. 30 (5): 23–27. doi:10.1097/01.JAA.0000515540.36581.92. PMID 28441669.
  4. Pittman JE, Ferkol TW (August 2015). "The Evolution of Cystic Fibrosis Care". Chest. 148 (2): 533–542. doi:10.1378/chest.14-1997. PMC 4524331. PMID 25764168.
  5. Ernst MM, Johnson MC, Stark LJ (April 2010). "Developmental and psychosocial issues in cystic fibrosis". Child Adolesc Psychiatr Clin N Am. 19 (2): 263–83, viii. doi:10.1016/j.chc.2010.01.004. PMC 2874200. PMID 20478499.
  6. Pettit RS, Fellner C (July 2014). "CFTR Modulators for the Treatment of Cystic Fibrosis". P T. 39 (7): 500–11. PMC 4103577. PMID 25083129.
  7. 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.
  8. Harness-Brumley CL, Elliott AC, Rosenbluth DB, Raghavan D, Jain R (December 2014). "Gender differences in outcomes of patients with cystic fibrosis". J Womens Health (Larchmt). 23 (12): 1012–20. doi:10.1089/jwh.2014.4985. PMC 4442553. PMID 25495366.



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