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Epidemiology

Cystic fibrosis is the most common life-limiting autosomal recessive disease among people of European heritage.

Recent European surveys by CF Europeⁱ and studies based on the European CF Patient Registry revealed nearly 50.000 people living with CF in Europe, but this is probably an underestimate due to lower access to diagnosis and care in newer EU countries and eastern Europe.

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.

Although technically a rare disease, cystic fibrosis is ranked as one of the most widespread life-shortening genetic diseases. It is most common among nations in the Western world. Approximately 1 in 25 people of European descent, and one in 30 of Caucasian Americans,^[89] 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.^[90][91]An exception in Europe is Finland, where only one in 80 people carry a CF mutation.^[92]

In the United States, 1 in 4,000 children are born with CF.^[93] In 1997, about 1 in 3,300 caucasian children in the United States was born with cystic fibrosis. In contrast, only 1 in 15,000 African American children suffered from cystic fibrosis, and in Asian Americans the rate was even lower at 1 in 32,000.^[94]

The distribution of CF alleles varies among populations. 

ΔF508 is the most common CF mutation worldwide, but the frequency depends on the country. The frequency of ΔF508 carriers has been estimated to be 1:200 in northern Sweden, 1:143 in Lithuania, and 1:38 in Denmark. ^[100] ΔF508 does occur in Finland, but it is a minority allele there. Cystic fibrosis is diagnosed in males and females equally.

Gender gap?

For reasons that remain unclear, data has shown that males tend to have a longer life expectancy than females,^[95][96] however recent studies suggest this gender gap may no longer exist perhaps due to improvements in health care facilities,^[97][98] while a recent study from Ireland identified a link between the female hormone oestrogen and worse outcomes in CF.^[99]

CF patient registries

Many countries have national CF patient registries which can be consulted via the local association's websites. 

The purpose of the European ECFS Patient Registry is to measure, survey and compare aspects of cystic fibrosis and its treatment in Europe, thereby encouraging new standards of dealing with the disease, to provide data for epidemiological research and to identify special patient groups suitable for multi-centre trials. For more info about the European Patient registry look at the article about this registry under Research.

More than 40 years ago, the CF Foundation created the American Cystic Fibrosis Patient Registry to track the health of people with CF in the United States. The information in this registry allows caregivers and researchers to identify new health trends, recognize the most effective treatments and design clinical trials for potential therapies. The registry anonymously reports patient data from more than 26,000 people with CF who receive care at CFF accredited centers. The information collected includes:

• height
• weight
• gender
• genotype
• lung function test results
• pancreatic enzyme use
• length of hospitalizations
• home IV use
• complications related to CF

By providing data through the patient registry, the Foundation educates and hopes to foster stronger partnerships among people with CF, their families and care center staff.

References :

89. 2008 ^ Cystic Fibrosis Foundation - Genetic Carrier Testing Updated 07/09/07
90. ^ Rosenstein BJ, Cutting GR (April 1998). "The diagnosis of cystic fibrosis: a consensus statement. Cystic Fibrosis Foundation Consensus Panel". J. Pediatr. 132 (4): 589–95. doi:10.1016/S0022-3476(98)70344-0. PMID 9580754.
91. ^ Hamosh A, FitzSimmons SC, Macek M, Knowles MR, Rosenstein BJ, Cutting GR (February 1998). "Comparison of the clinical manifestations of cystic fibrosis in black and white patients". J. Pediatr. 132 (2): 255–9. doi:10.1016/S0022-3476(98)70441-X. PMID 9506637.
92. ^ Hytönen M, Patjas M, Vento SI, et al. (December 2001). "Cystic fibrosis gene mutations deltaF508 and 394delTT in patients with chronic sinusitis in Finland". Acta Otolaryngol. 121 (8): 945–7. PMID 11813900.
93. ^^{[unreliable medical source?]}About Cystic Fibrosis
94. ^ Genetic testing for cystic fibrosis Genetic Testing for Cystic Fibrosis. National Institutes of Health, Consensus Development Conference Statement. April 14–16, 1997. Retrieved on November 20, 2009.
95. ^ Rosenfeld M, Davis R, FitzSimmons S, Pepe M, Ramsey B (May 1997). "Gender gap in cystic fibrosis mortality". Am. J. Epidemiol. 145 (9): 794–803. PMID 9143209.
96. ^ Coakley RD, Sun H, Clunes LA, et al. (December 2008). "17beta-Estradiol inhibits Ca2+-dependent homeostasis of airway surface liquid volume in human cystic fibrosis airway epithelia". J. Clin. Invest. 118 (12): 4025–35. doi:10.1172/JCI33893. PMC 2582929. PMID 19033671.
97. ^ Verma N, Bush A, Buchdahl R (October 2005). "Is there still a gender gap in cystic fibrosis?". Chest 128 (4): 2824–34. doi:10.1378/chest.128.4.2824. PMID 16236961.
98. ^ Moran A, Dunitz J, Nathan B, Saeed A, Holme B, Thomas W (September 2009). "Cystic fibrosis-related diabetes: current trends in prevalence, incidence, and mortality". Diabetes Care 32 (9): 1626–31. doi:10.2337/dc09-0586. PMC 2732133. PMID 19542209.
99. ^ CF worse for women 'due to effect of oestregen' - The Irish Times - Tue, Aug 10, 2010
100. ^ Wennberg C, Kucinskas V (1994). "Low frequency of the delta F508 mutation in Finno-Ugrian and Baltic populations". Hum. Hered. 44 (3): 169–71. doi:10.1159/000154210. PMID 8039801.

Source indication: Wikipedia - http://en.wikipedia.org/wiki/Cystic_fibrosis

License notice: The work is released under CC-BY-SA
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