Cystic fibrosis epidemiology and demographics: Difference between revisions

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{{CMG}} {{AE}}
{{CMG}} {{AE}}
{{Cystic fibrosis}}
{{Cystic fibrosis}}
==Overview==
==Overview==


==Epidemiology and Demographics==
==Epidemiology and Demographics==
[[Image:autorecessive.jpg|thumb|right|Cystic Fibrosis has an autosomal recessive pattern of inheritance.]]
===Incidence===
*The incidence/prevalence of [disease name] is approximately [number range] per 100,000 individuals worldwide.
*In [year], the incidence/prevalence of [disease name] was estimated to be [number range] cases per 100,000 individuals worldwide.
 
===Prevalence===
*The incidence/prevalence of [disease name] is approximately [number range] per 100,000 individuals worldwide.
*In [year], the incidence/prevalence of [disease name] was estimated to be [number range] cases per 100,000 individuals worldwide.
*The prevalence of [disease/malignancy] is estimated to be [number] cases annually.
 
===Case-fatality rate/Mortality rate===
*In [year], the incidence of [disease name] is approximately [number range] per 100,000 individuals with a case-fatality rate/mortality rate of [number range]%.
*The case-fatality rate/mortality rate of [disease name] is approximately [number range].
 
===Age===
*Patients of all age groups may develop [disease name].
*The incidence of [disease name] increases with age; the median age at diagnosis is [#] years.
*[Disease name] commonly affects individuals younger than/older than [number of years] years of age.
*[Chronic disease name] is usually first diagnosed among [age group].
*[Acute disease name] commonly affects [age group].


Cystic fibrosis is the most common life-limiting autosomal recessive disease among people of European heritage. In the United States, approximately 30,000 individuals have CF; most are diagnosed by six months of age. Canada has approximately 3,000 citizens with CF. Approximately 1 in 25 people of European descent and 1 in 22 people of Ashkenazi Jewish descent is a carrier of a cystic fibrosis mutation. Although CF is less common in these groups, approximately 1 in 46 Hispanics, 1 in 65 Africans and 1 in 90 Asians carry at least one abnormal CFTR gene.<ref>Rosenstein BJ and Cutting GR. ''The diagnosis of cystic fibrosis: a consensus statement. Cystic Fibrosis Foundation Consensus Panel.'' J Pediatr. 1998 Apr;132(4):589-95. Review. PMID 9580754</ref><ref>Hamosh A, Fitz-Simmons SC, Macek M Jr, Knowles MR, Rosenstein BJ, Cutting GR. ''Comparison of the clinical manifestations of cystic fibrosis in black and white patients.'' J Pediatr. 1998 Feb;132(2):255-9. PMID 9506637</ref><ref>Kerem B, Chiba-Falek O, Kerem E. ''Cystic fibrosis in Jews: frequency and mutation distribution.'' Genet Test. 1997;1(1):35-9. Review. PMID 10464623</ref>
===Race===
*There is no racial predilection to [disease name].
*[Disease name] usually affects individuals of the [race 1] race. [Race 2] individuals are less likely to develop [disease name].
===Gender===
*[Disease name] affects men and women equally.
*[Gender 1] are more commonly affected by [disease name] than [gender 2]. The [gender 1] to [gender 2] ratio is approximately [number > 1] to 1.
===Region===
*The majority of [disease name] cases are reported in [geographical region].


Cystic fibrosis is diagnosed in males and females equally. For unclear reasons, males tend to have a longer [[life expectancy]] than females.<ref>Rosenfeld, M, Davis, R, FitzSimmons, S, et al ''Gender gap in cystic fibrosis mortality.'' Am J Epidemiol 1997 145,794–803</ref> Life expectancy for people with CF depends largely upon access to health care. In 1959, the median age of survival of children with cystic fibrosis was six months. In the United States, the life expectancy for infants born in 2006 with CF is 36.8 years, based upon data compiled by the [[Cystic Fibrosis Foundation]].<ref name="median">{{cite web| date = [[April 26]], [[2006]]| url=http://www.cff.org/aboutCFFoundation/NewsEvents/2006NewsArchive/index.cfm?ID=2711&TYPE=1132| title =New Statistics Show CF Patients Living Longer| publisher =[[Cystic Fibrosis Foundation]]| accessdate =2007-12-09}}</ref>
*[Disease name] is a common/rare disease that tends to affect [patient population 1] and [patient population 2].


The Cystic Fibrosis Foundation also compiles lifestyle information about American adults with CF. In 2004, the foundation reported that 91% had graduated high school and 54% had at least some college education. Employment data revealed 12.6% of adults were disabled and 9.9% were unemployed. Marital information showed that 59% of adults were single and 36% were married or living with a partner. In 2004, 191 American women with CF were pregnant.
===Developed Countries===


===Theories about the prevalence of CF===
===Developing Countries===
The [[ΔF508]] mutation is estimated to be up to 52,000 years old.<ref>Wiuf C. ''Do delta F508 heterozygotes have a selective advantage?'' Genet Res. 2001 Aug;78(1):41-7. PMID 11556136</ref> Numerous hypotheses have been advanced as to why such a lethal mutation has persisted and spread in the human population. Other common autosomal recessive diseases such as [[sickle-cell anemia]] have been found to protect carriers from other diseases, a concept known as [[heterozygote advantage]]. Resistance to the following have all been proposed as possible sources of [[heterozygote advantage]]:


* Cholera: With the discovery that [[cholera]] [[toxin]] requires normal host CFTR proteins to function properly, it was hypothesized that carriers of mutant CFTR genes benefited from resistance to cholera and other causes of diarrhea.<ref>Gabriel SE, Brigman KN, Koller BH, Boucher RC, Stutts MJ. ''Cystic fibrosis heterozygote resistance to cholera toxin in the cystic fibrosis mouse model.'' Science. 1994 Oct 7;266(5182):107-9. PMID 7524148</ref> Further studies have not confirmed this hypothesis.<ref>Cuthbert AW, Halstead J, Ratcliff R, Colledge WH, Evans MJ. ''The genetic advantage hypothesis in cystic fibrosis heterozygotes: a murine study.'' J Physiol. 1995 Jan 15;482 (Pt 2):449-54. PMID 7714835</ref><ref>Hogenauer C, Santa Ana CA, Porter JL, Millard M, Gelfand A, Rosenblatt RL, Prestidge CB, Fordtran JS. ''Active intestinal chloride secretion in human carriers of cystic fibrosis mutations: an evaluation of the hypothesis that heterozygotes have subnormal active intestinal chloride secretion.'' Am J Hum Genet. 2000 Dec;67(6):1422–7. Epub 2000 Oct 30. PMID 11055897.</ref>
* Typhoid: Normal CFTR proteins are also essential for the entry of [[Salmonella typhi]] into cells,<ref>Pier GB, Grout M, Zaidi T, Meluleni G, Mueschenborn SS, Banting G, Ratcliff R, Evans MJ, Colledge WH. ''Salmonella typhi uses CFTR to enter intestinal epithelial cells.'' Nature. 1998 May 7;393(6680):79–82. PMID 9590693</ref> suggesting that carriers of mutant CFTR genes might be resistant to [[typhoid fever]]. No ''in vivo'' study has yet confirmed this. In both cases, the low level of cystic fibrosis outside of Europe, in places where both cholera and typhoid fever are [[endemic (epidemiology)|endemic]], is not immediately explicable.
* Diarrhoea: It has also been hypothesized that the prevalence of CF in Europe might be connected with the development of cattle domestication. In this hypothesis, carriers of a single mutant CFTR chromosome had some protection from diarrhoea caused by [[lactose intolerance]], prior to the appearance of the mutations that created lactose tolerance.<ref>Modiano G, Ciminelli BM, Pignatti PF. ''Cystic Fibrosis: Cystic fibrosis and lactase persistence: a possible correlation.'' Eur J Hum Genet. 2007 Mar;15(3):255-9. PMID: 17180122.</ref>
* Tuberculosis: Poolman and Galvani from Yale University have added another possible explanation - that carriers of the gene have some resistance to TB.<ref>[http://www.newscientist.com/article/dn10013-cystic-fibrosis-gene-protect Cystic fibrosis gene protects against tuberculosis]</ref><ref> [http://dx.doi.org/10.1016/j.cub.2006.09.009 Footprint fears for new TB threat]</ref>


==References==
==References==

Revision as of 17:41, 4 February 2018

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Overview

Epidemiology and Demographics

Incidence

  • The incidence/prevalence of [disease name] is approximately [number range] per 100,000 individuals worldwide.
  • In [year], the incidence/prevalence of [disease name] was estimated to be [number range] cases per 100,000 individuals worldwide.

Prevalence

  • The incidence/prevalence of [disease name] is approximately [number range] per 100,000 individuals worldwide.
  • In [year], the incidence/prevalence of [disease name] was estimated to be [number range] cases per 100,000 individuals worldwide.
  • The prevalence of [disease/malignancy] is estimated to be [number] cases annually.

Case-fatality rate/Mortality rate

  • In [year], the incidence of [disease name] is approximately [number range] per 100,000 individuals with a case-fatality rate/mortality rate of [number range]%.
  • The case-fatality rate/mortality rate of [disease name] is approximately [number range].

Age

  • Patients of all age groups may develop [disease name].
  • The incidence of [disease name] increases with age; the median age at diagnosis is [#] years.
  • [Disease name] commonly affects individuals younger than/older than [number of years] years of age.
  • [Chronic disease name] is usually first diagnosed among [age group].
  • [Acute disease name] commonly affects [age group].

Race

  • There is no racial predilection to [disease name].
  • [Disease name] usually affects individuals of the [race 1] race. [Race 2] individuals are less likely to develop [disease name].

Gender

  • [Disease name] affects men and women equally.
  • [Gender 1] are more commonly affected by [disease name] than [gender 2]. The [gender 1] to [gender 2] ratio is approximately [number > 1] to 1.

Region

  • The majority of [disease name] cases are reported in [geographical region].
  • [Disease name] is a common/rare disease that tends to affect [patient population 1] and [patient population 2].

Developed Countries

Developing Countries

References


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