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*With an [[Sex linkage|X-linked]] [[Heredity|inheritance]] pattern, [[Congenital disorder|congenital]] hemophilia exclusively affects boys and/or men.<ref name="pmid9840909">{{cite journal |vauthors=Soucie JM, Evatt B, Jackson D |title=Occurrence of hemophilia in the United States. The Hemophilia Surveillance System Project Investigators |journal=Am. J. Hematol. |volume=59 |issue=4 |pages=288–94 |date=December 1998 |pmid=9840909 |doi= |url=}}</ref><ref name="pmid16295028">{{cite journal |vauthors=Pruthi RK |title=Hemophilia: a practical approach to genetic testing |journal=Mayo Clin. Proc. |volume=80 |issue=11 |pages=1485–99 |date=November 2005 |pmid=16295028 |doi=10.4065/80.11.1485 |url=}}</ref> | *With an [[Sex linkage|X-linked]] [[Heredity|inheritance]] pattern, [[Congenital disorder|congenital]] hemophilia exclusively affects boys and/or men.<ref name="pmid9840909">{{cite journal |vauthors=Soucie JM, Evatt B, Jackson D |title=Occurrence of hemophilia in the United States. The Hemophilia Surveillance System Project Investigators |journal=Am. J. Hematol. |volume=59 |issue=4 |pages=288–94 |date=December 1998 |pmid=9840909 |doi= |url=}}</ref><ref name="pmid16295028">{{cite journal |vauthors=Pruthi RK |title=Hemophilia: a practical approach to genetic testing |journal=Mayo Clin. Proc. |volume=80 |issue=11 |pages=1485–99 |date=November 2005 |pmid=16295028 |doi=10.4065/80.11.1485 |url=}}</ref> | ||
*Hemophilia C with an [[Autosomal|autosomal transmission]] can affect the male and as well as female population.<ref name="pmid27216469">{{cite journal |vauthors=Wheeler AP, Gailani D |title=Why factor XI deficiency is a clinical concern |journal=Expert Rev Hematol |volume=9 |issue=7 |pages=629–37 |date=July 2016 |pmid=27216469 |pmc=4950943 |doi=10.1080/17474086.2016.1191944 |url=}}</ref><ref name="pmid9883805">{{cite journal |vauthors=Kadir RA, Economides DL, Lee CA |title=Factor XI deficiency in women |journal=Am. J. Hematol. |volume=60 |issue=1 |pages=48–54 |date=January 1999 |pmid=9883805 |doi= |url=}}</ref> | *Hemophilia C with an [[Autosomal|autosomal transmission]] can affect the male and as well as female population.<ref name="pmid27216469">{{cite journal |vauthors=Wheeler AP, Gailani D |title=Why factor XI deficiency is a clinical concern |journal=Expert Rev Hematol |volume=9 |issue=7 |pages=629–37 |date=July 2016 |pmid=27216469 |pmc=4950943 |doi=10.1080/17474086.2016.1191944 |url=}}</ref><ref name="pmid9883805">{{cite journal |vauthors=Kadir RA, Economides DL, Lee CA |title=Factor XI deficiency in women |journal=Am. J. Hematol. |volume=60 |issue=1 |pages=48–54 |date=January 1999 |pmid=9883805 |doi= |url=}}</ref> | ||
==References== | ==References== | ||
{{reflist|2}} | {{reflist|2}} | ||
Latest revision as of 14:47, 2 August 2019
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sabawoon Mirwais, M.B.B.S, M.D.[2]
Overview
The prevalence of hemophilia is estimated to be 20,000 cases in the United States annually. The age-adjusted prevalence of hemophilia in six US states (Oklahoma, Massachusetts, Colorado, Georgia, Louisiana, and New York) in 1994 was 13.4 cases per 100, 000 males. The incidence of hemophilia is estimated to be 1 in 5,000 male births for hemophilia A and 1 in 30,000 births for hemophilia B.
Epidemiology and Demographics
Incidence
- The mean incidence of hemophilia from 1982-91 was 1 in 5,032 live male births.[1]
- The incidence of congenital hemophilia A is 1 in 5000 boys/men.[1][2]
- The incidence of congenital hemophilia B is about 1 in 30,000 boys/men.[1]
- 400 hemophilic babies are born every year in the United States.[1][3][4]
Prevalence
- The age-adjusted prevalence of hemophilia in six US states (Oklahoma, Massachusetts, Colorado, Georgia, Louisiana, and New York) in 1994 was 13.4 cases per 100, 000 males.[1]
- The prevalence of hemophilia in Swedish population was 13.7 per 100, 000 males in 1980.[1][5]
- The prevalence of hemophilia in other countries identified through contact with specialized hemophilia treatment centers range from 9.7 to 20.5 per 100, 000 males.[1][6][7][8][9][10]
Mortality rate
- In the United Kingdom, during 1977-1999, the all-cause mortality rate in severe hemophilia was higher than the corresponding age- and calendar year–specific all-cause mortality rate in the general male population by a factor of 2.69, while for patients with moderate/mild hemophilia it was increased by a factor of 1.19.[11]
- The overall life expectancy of the patients with hemophilia did not notably change between 1972 and 2001.[12]
- AIDS is the main cause of death (26%) and 22% of the deaths are because of hepatitis C.[12]
Age
- Prevalence rates are higher among 5-14 year olds.[1]
Race
- Hemophilia affects people from all racial and ethnic groups. However heterozygous factor XI deficiency occurs very frequently among Ashkenazi Jews.[13][14]
Gender
- With an X-linked inheritance pattern, congenital hemophilia exclusively affects boys and/or men.[1][15]
- Hemophilia C with an autosomal transmission can affect the male and as well as female population.[16][17]
References
- ↑ 1.0 1.1 1.2 1.3 1.4 1.5 1.6 1.7 1.8 Soucie JM, Evatt B, Jackson D (December 1998). "Occurrence of hemophilia in the United States. The Hemophilia Surveillance System Project Investigators". Am. J. Hematol. 59 (4): 288–94. PMID 9840909.
- ↑ https://www.nhlbi.nih.gov/health-topics/hemophilia
- ↑ https://www.nhlbi.nih.gov/health-topics/hemophilia
- ↑ "CDC Hemophilia Epidemiology and demographics".
- ↑ Larsson SA, Nilsson IM, Blombäck M (1982). "Current status of Swedish hemophiliacs. I. A demographic survey". Acta Med Scand. 212 (4): 195–200. PMID 7148514.
- ↑ Rosendaal FR, Varekamp I, Smit C, Bröcker-Vriends AH, van Dijck H, Vandenbroucke JP, Hermans J, Suurmeijer TP, Briët E (January 1989). "Mortality and causes of death in Dutch haemophiliacs, 1973-86". Br. J. Haematol. 71 (1): 71–6. PMID 2917132.
- ↑ Walker I (1991). "Survey of the Canadian hemophilia population". Can J Public Health. 82 (2): 127–9. PMID 2049704.
- ↑ Rizza CR, Spooner RJ (March 1983). "Treatment of haemophilia and related disorders in Britain and Northern Ireland during 1976-80: report on behalf of the directors of haemophilia centres in the United Kingdom". Br Med J (Clin Res Ed). 286 (6369): 929–33. PMID 6403138.
- ↑ Koumbarelis E, Rosendaal FR, Gialeraki A, Karafoulidou A, Noteboom WM, Loizou C, Panayotopoulou C, Markakis C, Mandalaki T (December 1994). "Epidemiology of haemophilia in Greece: an overview". Thromb. Haemost. 72 (6): 808–13. PMID 7740446.
- ↑ Ghirardini A, Schinaia N, Chiarotti F, De Biasi R, Rodeghiero F, Binkin N (November 1994). "Epidemiology of hemophilia and of HIV infection in Italy. GICC. Gruppo Italiano Coagulopatie Congenite". J Clin Epidemiol. 47 (11): 1297–306. PMID 7722566.
- ↑ Darby SC, Kan SW, Spooner RJ, Giangrande PL, Hill FG, Hay CR, Lee CA, Ludlam CA, Williams M (August 2007). "Mortality rates, life expectancy, and causes of death in people with hemophilia A or B in the United Kingdom who were not infected with HIV". Blood. 110 (3): 815–25. doi:10.1182/blood-2006-10-050435. PMID 17446349.
- ↑ 12.0 12.1 Plug I, Van Der Bom JG, Peters M, Mauser-Bunschoten EP, De Goede-Bolder A, Heijnen L, Smit C, Willemse J, Rosendaal FR (March 2006). "Mortality and causes of death in patients with hemophilia, 1992-2001: a prospective cohort study". J. Thromb. Haemost. 4 (3): 510–6. doi:10.1111/j.1538-7836.2006.01808.x. PMID 16460432.
- ↑ http://www.wfh.org
- ↑ Berg LP, Varon D, Martinowitz U, Wieland K, Kakkar VV, Cooper DN (February 1994). "Combined factor VIII/factor XI deficiency may cause intra-familial clinical variability in haemophilia A among Ashkenazi Jews". Blood Coagul. Fibrinolysis. 5 (1): 59–62. PMID 8180339.
- ↑ Pruthi RK (November 2005). "Hemophilia: a practical approach to genetic testing". Mayo Clin. Proc. 80 (11): 1485–99. doi:10.4065/80.11.1485. PMID 16295028.
- ↑ Wheeler AP, Gailani D (July 2016). "Why factor XI deficiency is a clinical concern". Expert Rev Hematol. 9 (7): 629–37. doi:10.1080/17474086.2016.1191944. PMC 4950943. PMID 27216469.
- ↑ Kadir RA, Economides DL, Lee CA (January 1999). "Factor XI deficiency in women". Am. J. Hematol. 60 (1): 48–54. PMID 9883805.