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==Overview== | ==Overview== | ||
[[Hemophilia A]] is sometimes referred to as “The Royal Disease”, as it affected many members of the English, German, Russian and Spanish monarchies in the 19th and 20th centuries. <ref name="pmid22551339">{{cite journal| author=Franchini M, Mannucci PM| title=Past, present and future of hemophilia: a narrative review. | journal=Orphanet J Rare Dis | year= 2012 | volume= 7 | issue= | pages= 24 | pmid=22551339 | doi=10.1186/1750-1172-7-24 | pmc=3502605 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22551339 }} </ref> | |||
Clinical distinct features of the Hemophilia disease can be noted in ancient pieces of evidence as old as 2nd century A.D. However documented scientific study of the disease was first performed in the early 19th century. [[Hemophilia A|Hemophilia]] is sometimes referred to as “The Royal Disease”, as it affected many members of the English, German, Russian and Spanish monarchies in the 19th and 20th centuries.<ref name="pmid22551339">{{cite journal| author=Franchini M, Mannucci PM| title=Past, present and future of hemophilia: a narrative review. | journal=Orphanet J Rare Dis | year= 2012 | volume= 7 | issue= | pages= 24 | pmid=22551339 | doi=10.1186/1750-1172-7-24 | pmc=3502605 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22551339 }} </ref>Progress towards more precise and efficient recognition of the disease, its rare forms and treatments is currently on its way. | |||
==Historical Perspective== | ==Historical Perspective== | ||
The disease was first described by John Conrad Otto, an American physician, in 1803 based on his observation of affected men in certain families. <ref> History of Bleeding Disorders | National Hemophilia Foundation. Available at https://www.hemophilia.org/Bleeding-Disorders/History-of-Bleeding-Disorders . Accessed on July 30, 2016 </ref> In 1947, the first distinction between Hemophilia A and [[Hemophilia B]] was made by Dr. Alfredo Pavlovsky from Argentina. In 1965, through her work in the [[coagulation cascade]], Dr. Judith Graham Pool discovered that [[cryoprecipitate]] left from thawing plasma was rich in [[Factor VIII]]. Soon after, the first use of cryoprecipitate to treat hemophilia A was pioneered. | * The disease was first described by John Conrad Otto, an American physician, in 1803 based on his observation of affected men in certain families.<ref>History of Bleeding Disorders | National Hemophilia Foundation. Available at https://www.hemophilia.org/Bleeding-Disorders/History-of-Bleeding-Disorders . Accessed on July 30, 2016 </ref> | ||
* In 1947, the first distinction between Hemophilia A and [[Hemophilia B]] was made by Dr. Alfredo Pavlovsky from Argentina. He demonstrated that a blood donation from one Hemophilia patient could solve the clotting disorder of another Hemophilia patient which leads to recognition of two distinct types of factor deficiencies<ref>PAVLOVSKY, A. (1947). CONTRIBUTION TO THE PATHOGENESIS OF HEMOPHILIA. ''Blood,'' 2(2), 185-191. Accessed July 19, 2018. Retrieved from <nowiki>http://www.bloodjournal.org/content/2/2/185</nowiki>.</ref>. | |||
* In 1965, through her work in the [[coagulation cascade]], Dr. Judith Graham Pool discovered that [[cryoprecipitate]] left from thawing plasma was rich in [[Factor VIII]]. | |||
* Soon after, the first use of cryoprecipitate to treat hemophilia A was pioneered<ref name="pmid5852902">{{cite journal| author=Pool JG, Shannon AE| title=Production of high-potency concentrates of antihemophilic globulin in a closed-bag system. | journal=N Engl J Med | year= 1965 | volume= 273 | issue= 27 | pages= 1443-7 | pmid=5852902 | doi=10.1056/NEJM196512302732701 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=5852902 }}</ref>. | |||
* Later on, with the development of factor concentrates, the treatment approach was changed to regular intermittent factor administration rather than treating the bleeding episodes per se. | |||
* With advances in orthopedic surgeries, the skeletal complications of the disease were better controlled. | |||
* The discovery of [[desmopressin]] in 1977 is also known as an important step towards more efficient, less harmful prevention and treatment strategy for many Hemophilia patients. | |||
* Recently, more advances like the introduction of recombinant coagulation factor and immune-modifications have led to more effective treatment of a wider range of Hemophilia patients<ref name="pmid24911674">{{cite journal| author=Franchini M, Mannucci PM| title=The history of hemophilia. | journal=Semin Thromb Hemost | year= 2014 | volume= 40 | issue= 5 | pages= 571-6 | pmid=24911674 | doi=10.1055/s-0034-1381232 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24911674 }}</ref>. | |||
==References== | ==References== | ||
{{reflist|2}} | {{reflist|2}} | ||
Latest revision as of 18:35, 28 October 2018
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Fahd Yunus, M.D. [2]
Overview
Clinical distinct features of the Hemophilia disease can be noted in ancient pieces of evidence as old as 2nd century A.D. However documented scientific study of the disease was first performed in the early 19th century. Hemophilia is sometimes referred to as “The Royal Disease”, as it affected many members of the English, German, Russian and Spanish monarchies in the 19th and 20th centuries.[1]Progress towards more precise and efficient recognition of the disease, its rare forms and treatments is currently on its way.
Historical Perspective
- The disease was first described by John Conrad Otto, an American physician, in 1803 based on his observation of affected men in certain families.[2]
- In 1947, the first distinction between Hemophilia A and Hemophilia B was made by Dr. Alfredo Pavlovsky from Argentina. He demonstrated that a blood donation from one Hemophilia patient could solve the clotting disorder of another Hemophilia patient which leads to recognition of two distinct types of factor deficiencies[3].
- In 1965, through her work in the coagulation cascade, Dr. Judith Graham Pool discovered that cryoprecipitate left from thawing plasma was rich in Factor VIII.
- Soon after, the first use of cryoprecipitate to treat hemophilia A was pioneered[4].
- Later on, with the development of factor concentrates, the treatment approach was changed to regular intermittent factor administration rather than treating the bleeding episodes per se.
- With advances in orthopedic surgeries, the skeletal complications of the disease were better controlled.
- The discovery of desmopressin in 1977 is also known as an important step towards more efficient, less harmful prevention and treatment strategy for many Hemophilia patients.
- Recently, more advances like the introduction of recombinant coagulation factor and immune-modifications have led to more effective treatment of a wider range of Hemophilia patients[5].
References
- ↑ Franchini M, Mannucci PM (2012). "Past, present and future of hemophilia: a narrative review". Orphanet J Rare Dis. 7: 24. doi:10.1186/1750-1172-7-24. PMC 3502605. PMID 22551339.
- ↑ History of Bleeding Disorders | National Hemophilia Foundation. Available at https://www.hemophilia.org/Bleeding-Disorders/History-of-Bleeding-Disorders . Accessed on July 30, 2016
- ↑ PAVLOVSKY, A. (1947). CONTRIBUTION TO THE PATHOGENESIS OF HEMOPHILIA. Blood, 2(2), 185-191. Accessed July 19, 2018. Retrieved from http://www.bloodjournal.org/content/2/2/185.
- ↑ Pool JG, Shannon AE (1965). "Production of high-potency concentrates of antihemophilic globulin in a closed-bag system". N Engl J Med. 273 (27): 1443–7. doi:10.1056/NEJM196512302732701. PMID 5852902.
- ↑ Franchini M, Mannucci PM (2014). "The history of hemophilia". Semin Thromb Hemost. 40 (5): 571–6. doi:10.1055/s-0034-1381232. PMID 24911674.