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{{Hemophilia}}
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==Overview==
==Overview==
The prevalence of hemophilia is estimated to be 20,000 cases annually. The incidence of hemophilia is estimated to be 1 in every 10,000 births( or 1 in 5,000 male births) for hemophilia A and 1 in 50,000 births for hemophilia B.
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==
==Epidemiology and Demographics==
===Incidence===
*The mean [[incidence]] of hemophilia from 1982-91 was 1 in 5,032 live male births.<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>
*The [[incidence]] of [[Congenital disorder|congenital]] hemophilia A is 1 in 5000 boys/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>https://www.nhlbi.nih.gov/health-topics/hemophilia</ref>
*The [[incidence]] of [[Congenital disorder|congenital]] hemophilia B is about 1 in 30,000 boys/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>
*400 [[Hemophilia|hemophilic]] babies are born every year in the United States.<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>https://www.nhlbi.nih.gov/health-topics/hemophilia</ref><ref>{{Cite web | title =CDC Hemophilia Epidemiology and demographics| url =http://www.cdc.gov/ncbddd/hemophilia/data.html }}</ref>
===Prevalence===
===Prevalence===
Currently, about 20,000 males in the United States are living with the disorder. Currently, the number of people with hemophilia in the United States is estimated to be about 20,000, based on expected births and deaths since 1994. Haemophilia A accounts for around 80% of cases. Haemophilia B (also known as Christmas disease) accounts for the remaining 20% of cases and results from a deficiency of coagulation Factor IX.<ref>{{Cite web | title = Hemophilia Epidemiology and demographics| url =http://www.cdc.gov/ncbddd/hemophilia/data.html }}</ref>
*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.<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>
===Incidence===
*The [[prevalence]] of hemophilia in Swedish population was 13.7 per 100, 000 males in 1980.<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="pmid7148514">{{cite journal |vauthors=Larsson SA, Nilsson IM, Blombäck M |title=Current status of Swedish hemophiliacs. I. A demographic survey |journal=Acta Med Scand |volume=212 |issue=4 |pages=195–200 |date=1982 |pmid=7148514 |doi= |url=}}</ref>
Each year, about 400 babies are born with the disorder in United States. Haemophilia usually occurs in males and less often in females. Haemophilia is rare, with only about 1 instance in every 10,000 births (or 1 in 5,000 male births) for haemophilia A and 1 in 50,000 births for haemophilia B. The incidence of Haemophilia B is around 1 in 25,000 male births. Haemophilia C (also known as plasma thromboplastin antecedent (PTA) deficiency or Rosenthal syndrome) is the fourth most common coagulation disorder after von Willebrand's disease and haemophilia A and B. In the USA it is thought to affect 1 in 100,000 of the adult population, making it 10% as common as haemophilia A.
*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.<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="pmid2917132">{{cite journal |vauthors=Rosendaal FR, Varekamp I, Smit C, Bröcker-Vriends AH, van Dijck H, Vandenbroucke JP, Hermans J, Suurmeijer TP, Briët E |title=Mortality and causes of death in Dutch haemophiliacs, 1973-86 |journal=Br. J. Haematol. |volume=71 |issue=1 |pages=71–6 |date=January 1989 |pmid=2917132 |doi= |url=}}</ref><ref name="pmid2049704">{{cite journal |vauthors=Walker I |title=Survey of the Canadian hemophilia population |journal=Can J Public Health |volume=82 |issue=2 |pages=127–9 |date=1991 |pmid=2049704 |doi= |url=}}</ref><ref name="pmid6403138">{{cite journal |vauthors=Rizza CR, Spooner RJ |title=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 |journal=Br Med J (Clin Res Ed) |volume=286 |issue=6369 |pages=929–33 |date=March 1983 |pmid=6403138 |doi= |url=}}</ref><ref name="pmid7740446">{{cite journal |vauthors=Koumbarelis E, Rosendaal FR, Gialeraki A, Karafoulidou A, Noteboom WM, Loizou C, Panayotopoulou C, Markakis C, Mandalaki T |title=Epidemiology of haemophilia in Greece: an overview |journal=Thromb. Haemost. |volume=72 |issue=6 |pages=808–13 |date=December 1994 |pmid=7740446 |doi= |url=}}</ref><ref name="pmid7722566">{{cite journal |vauthors=Ghirardini A, Schinaia N, Chiarotti F, De Biasi R, Rodeghiero F, Binkin N |title=Epidemiology of hemophilia and of HIV infection in Italy. GICC. Gruppo Italiano Coagulopatie Congenite |journal=J Clin Epidemiol |volume=47 |issue=11 |pages=1297–306 |date=November 1994 |pmid=7722566 |doi= |url=}}</ref>
===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 [[Patient|patients]] with moderate/mild hemophilia it was increased by a factor of 1.19.<ref name="pmid17446349">{{cite journal |vauthors=Darby SC, Kan SW, Spooner RJ, Giangrande PL, Hill FG, Hay CR, Lee CA, Ludlam CA, Williams M |title=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 |journal=Blood |volume=110 |issue=3 |pages=815–25 |date=August 2007 |pmid=17446349 |doi=10.1182/blood-2006-10-050435 |url=}}</ref>
*The overall [[life expectancy]] of the [[Patient|patients]] with hemophilia did not notably change between 1972 and 2001.<ref name="pmid16460432">{{cite journal |vauthors=Plug I, Van Der Bom JG, Peters M, Mauser-Bunschoten EP, De Goede-Bolder A, Heijnen L, Smit C, Willemse J, Rosendaal FR |title=Mortality and causes of death in patients with hemophilia, 1992-2001: a prospective cohort study |journal=J. Thromb. Haemost. |volume=4 |issue=3 |pages=510–6 |date=March 2006 |pmid=16460432 |doi=10.1111/j.1538-7836.2006.01808.x |url=}}</ref>
*[[HIV AIDS|AIDS]] is the main cause of death (26%) and 22% of the deaths are because of [[hepatitis C]].<ref name="pmid16460432">{{cite journal |vauthors=Plug I, Van Der Bom JG, Peters M, Mauser-Bunschoten EP, De Goede-Bolder A, Heijnen L, Smit C, Willemse J, Rosendaal FR |title=Mortality and causes of death in patients with hemophilia, 1992-2001: a prospective cohort study |journal=J. Thromb. Haemost. |volume=4 |issue=3 |pages=510–6 |date=March 2006 |pmid=16460432 |doi=10.1111/j.1538-7836.2006.01808.x |url=}}</ref>
 
===Age===
*[[Prevalence]] rates are higher among 5-14 year olds.<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>
===Race===
===Race===
Hemophilia affects people from all racial and ethnic groups. However Hemophilia C predominantly occurs in Jews of Ashkenazi descent.
*Hemophilia affects people from all racial and ethnic groups. However [[Zygosity|heterozygous]] [[factor XI]] [[deficiency]] occurs very frequently among Ashkenazi Jews.<ref>http://www.wfh.org</ref><ref name="pmid8180339">{{cite journal |vauthors=Berg LP, Varon D, Martinowitz U, Wieland K, Kakkar VV, Cooper DN |title=Combined factor VIII/factor XI deficiency may cause intra-familial clinical variability in haemophilia A among Ashkenazi Jews |journal=Blood Coagul. Fibrinolysis |volume=5 |issue=1 |pages=59–62 |date=February 1994 |pmid=8180339 |doi= |url=}}</ref>
 
===Gender===
===Gender===
Hemophilia A and B occurs more often in males and less often in females. Hemophilia C is mild form of Hemophilia affecting both sexes equally.
*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>


==References==
==References==

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

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

Race

Gender

References

  1. ↑ 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.
  2. ↑ https://www.nhlbi.nih.gov/health-topics/hemophilia
  3. ↑ https://www.nhlbi.nih.gov/health-topics/hemophilia
  4. ↑ "CDC Hemophilia Epidemiology and demographics".
  5. ↑ 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.
  6. ↑ 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.
  7. ↑ Walker I (1991). "Survey of the Canadian hemophilia population". Can J Public Health. 82 (2): 127–9. PMID 2049704.
  8. ↑ 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.
  9. ↑ 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.
  10. ↑ 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.
  11. ↑ 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. ↑ 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.
  13. ↑ http://www.wfh.org
  14. ↑ 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.
  15. ↑ 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.
  16. ↑ 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.
  17. ↑ Kadir RA, Economides DL, Lee CA (January 1999). "Factor XI deficiency in women". Am. J. Hematol. 60 (1): 48–54. PMID 9883805.

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