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__NOTOC__
{{DiseaseDisorder infobox
| Name          = Haemophilia B
| ICD10          = {{ICD10|D|67||d|65}}
| ICD9          = {{ICD9|286.1}}
| ICDO          =
| Image          =
| Caption        =
| OMIM          = 306900
| MedlinePlus    = 000539
| DiseasesDB    = 5561
| MeshID        = D002836
}}
{{Search infobox}}


'''For  patient  information click [[{{PAGENAME}}  (patient information)|here]]'''
{{CMG}}
{{SK}} Haemophilia B; hæmophilia B; haemophilia type B; hereditary factor IX deficiency; christmas disease
==Overview==
'''Hemophilia B''' is a [[coagulation|blood clotting]] disorder caused by a [[mutation]] of the [[Factor IX]] [[gene]]. It is the second most common form of [[haemophilia]], rarer than [[haemophilia A]]. It is sometimes called '''Christmas disease''' after Stephen Christmas, the first patient described with this disease.<ref>{{WhoNamedIt|synd|2321|Christmas' disease}}</ref>  In addition, the first report of its identification was published in the Christmas edition of the [[British Medical Journal]].<ref>Biggs RA, Douglas AS, MacFarlane RG, Dacie JV, Pittney WR, Merskey C, O'Brien JR. ''Christmas disease: a condition previously mistaken for haemophilia.'' [[British Medical Journal|Br Med J]] 1952;2:1378-1382. PMID 12997790.</ref>
==Pathophysiology==
Factor IX deficiency leads to an increased propensity for [[haemorrhage]]. This is in response to mild trauma or even spontaneously, such as in joints ([[hemarthrosis|haemarthrosis]]) or muscles.
===Genetics===
The factor IX gene is located on the [[X chromosome]] (Xq27.1-q27.2). It is inherited [[X-linked]] recessive, which explains why - as in haemophilia A - mostly males are generally affected.
==Treatment==
Treatment (bleeding prophylaxis) is by intravenous infusion of factor IX.
==References==
{{reflist|2}}
==Related Chapters==
*[[Haemophilia]]
*[[Haemophilia A]]
*[[Haemophilia C]]
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{{Hematology}}
[[es:Hemofilia B]]
[[pt:Hemofilia B]]
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Latest revision as of 22:21, 6 June 2016