58:
507:, which allows for neatly packaged bottles of factor IX concentrates. With the rise of factor IX concentrates it became easier for people to get treatment at home. Although these advances in medicine had a significant positive impact on the treatment of haemophilia, there were many complications that came with it. By the early 1980s, scientists discovered that the medicines they had created were transferring blood-borne
271:
1542:
200:
exfoliation of primary dentition, or prolonged bleeding after an invasive procedure/tooth extraction; In severe haemophilia, there may be spontaneous bleeding from the oral tissues (e.g. soft palate, tongue, buccal mucosa), lips and gingiva, with ecchymoses. In rare cases, haemarthrosis (bleeding into joint space) of the temporomandibular joint (TMJ) may be observed.
799:
503:, requiring large amounts of FFP to treat an actual bleeding episode, which resulted in the person requiring hospitalization. By the mid-1960s scientists found a way to get a larger amount of factor IX from FFP. By the late 1960s, pharmaceutical scientists found methods to separate the factor IX from
203:
Patients with haemophilia will experience many episodes of oral bleeding over their lifetime. Average 29.1 bleeding events per year are serious enough to require factor replacement in F VIII-deficient patients which 9% involved oral structures. Children with severe haemophilia have significant lower
166:
Most individuals who have
Hemophilia B and experience symptoms are men. The prevalence of Hemophilia B in the population is about one in 40,000; Hemophilia B represents about 15% of patients with hemophilia. Many women carriers of the disease have no symptoms. However, an estimated 10-25% of women
199:
Patients with bleeding disorders show a higher incidence of periodontal disease as well as dental caries, concerning the fear of bleeding which leads to a lack of oral hygiene and oral health care. The most prominent oral manifestation of a mild haemophilia B would be gingival bleeding during
1348:
Nathwani, Amit C.; Reiss, Ulreke M.; Tuddenham, Edward G.D.; Rosales, Cecilia; Chowdary, Pratima; McIntosh, Jenny; Della Peruta, Marco; Lheriteau, Elsa; Patel, Nishal; Raj, Deepak; Riddell, Anne; Pie, Jun; Rangarajan, Savita; Bevan, David; Recht, Michael; Shen, Yu-Min; Halka, Kathleen G.;
398:
Surgical treatment, including a simple dental extraction, must be planned to minimize the risk of bleeding, excessive bruising, or haematoma formation. Soft vacuum-formed splints can be used to provide local protection following a dental extraction or prolonged post-extraction bleed.
1349:
Basner-Tschakarjan, Etiena; Mingozzi, Federico; High, Katherine A.; Allay, James; Kay, Mark A.; Ng, Catherine Y.C.; Zhou, Junfang; Cancio, Maria; Morton, Christopher L.; Gray, John T.; Srivastava, Deokumar; Nienhuis, Arthur W.; Davidoff, Andrew M. (20 November 2014).
519:, the virus that causes AIDS. With the rise of these deadly viruses, scientists had to find improved methods for screening the blood products they received from donors. In 1982, scientists made a breakthrough in medicine and were able to clone factor IX
1437:
1422:
428:
487:
in the period during which blood was not routinely screened for this virus. He became an active worker for the
Canadian Hemophilia Society and campaigned for transfusion safety ever since getting infected, but developed
537:
In 2009, an analysis of genetic markers revealed that haemophilia B was the blood disease affecting many
European royal families of the United Kingdom, Germany, Russia and Spain: so-called "Royal Disease".
435:
Stephen
Christmas (12 February 1947 – 20 December 1993) was the first patient described to have Christmas disease (or Haemophilia B) in 1952 by a group of British doctors. Christmas was born to a
1588:
455:. The family returned to London in 1952 to visit their relatives, and during the trip Stephen was admitted to hospital. A sample of his blood was sent to the Oxford Haemophilia Centre in
999:
Chowdary, Pratima; Shapiro, Susan; Makris, Mike; Evans, Gillian; Boyce, Sara; Talks, Kate; Dolan, Gerard; Reiss, Ulrike; Phillips, Mark; Riddell, Anne; Peralta, Maria R. (2022-07-21).
419:(FIX) protein, normal levels of the protein were observed with low doses of the therapy but immunosuppression was necessitated to decrease the risk of vector-related immune responses.
213:
2448:
285:
Factor IX deficiency can cause interference of the coagulation cascade, thereby causing spontaneous haemorrhage when there is trauma. Factor IX when activated activates
523:. With this new development it decreased the risk of the many viruses. Although the new factor was created, it was not available for haemophilia B patients until 1997.
984:
Andrew Brewer, Maria Elvira Correa (May 2006). "Guildelines for Dental
Treatment of Patients with Inherited Bleeding Disorders" (PDF). Treatment of Hemophilia.
2504:
2368:
1581:
2404:
2215:
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1574:
495:
In the 1950s and 1960s, with newfound technology and gradual advances in medicine, pharmaceutical scientists found a way to take the factor IX from
157:, the first patient described with haemophilia B. In addition, the first report of its identification was published in the Christmas edition of the
2711:
2559:
452:
376:
should be avoided once the diagnosis is made since they can exacerbate a bleeding episode. Any surgical procedure should be done with concomitant
951:
776:
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832:
662:
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499:(FFP) and give it to those with haemophilia B. Though they found a way to treat the disease, the FFP contained only a small amount of
2625:
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2409:
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2489:
2208:
1942:
1894:
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386:(Hemgenix) was approved for medical use in the United States in November 2022. It is the first gene therapy approved by the US
2658:
2238:
1661:
1948:
1110:
Giangrande PL (June 2003). "Six characters in search of an author: the history of the nomenclature of coagulation factors".
2740:
2254:
1934:
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1922:
1885:
358:
30:
This article is about the bleeding disorder with factor IX deficiency. For the disorder with factor VIII deficiency, see
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provide a binding site for both cofactors. This complex (in the coagulation pathway) will eventually activate factor X.
2796:
2721:
2615:
2269:
2201:
2075:
1651:
255:– where patients experience episodes of excessive bleeding in childhood but have few bleeding problems after puberty.
562:
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1875:
1698:
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2146:
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383:
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2116:
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827:
Glick, Michael (2015). Burket's Oral
Medicine. USA: People's Medical Publishing House. pp. 473, 475, 481, 482.
1899:
2831:
2756:
2680:
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2399:
1557:
1295:
Franchini, Massimo; Frattini, Francesco; Crestani, Silvia; Sissa, Cinzia; Bonfanti, Carlo (1 January 2013).
713:
159:
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Treatment is given intermittently, when there is significant bleeding. It includes intravenous infusion of
2771:
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2422:
2020:
1995:
1953:
464:
412:
350:
2603:
2435:
2015:
2005:
1823:
149:
Haemophilia B was first recognized as a distinct disease entity in 1952. It is also known by the eponym
471:, which is normally decreased in classic hemophilia, but a different protein, which received the name
451:, Canada, with his family, and was there at the age of two years that hemophilia was diagnosed at the
2821:
2391:
2317:
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1771:
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2136:
2010:
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496:
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329:
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66:
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1127:
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957:
828:
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658:
627:
480:
240:
154:
142:, and resulting in a deficiency of factor IX. It is less common than factor VIII deficiency (
104:
74:
204:
prevalence of dental caries and lower plaque scores compared with matched, healthy controls.
2766:
2640:
2131:
1851:
1791:
1786:
1646:
1378:
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1326:
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472:
448:
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1965:
1671:
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This lack of protein attachment to the DNA was thereby turning off the gene that produces
236:
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1250:
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carriers have mild symptoms; in rare cases, women may have moderate or severe symptoms.
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57:
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1988:
1978:
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1123:
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552:
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342:
188:
143:
31:
1514:
1139:
2386:
1618:
1166:
618:
Konkle, Barbara A.; Josephson, Neil C.; Nakaya
Fletcher, Shelley (1 January 1993).
504:
408:
221:
1153:
Schramm, Wolfgang (November 2014). "The history of haemophilia – a short review".
591:
1468:
816:"Hemophilia A" (PDF). College of Dental Hygienists of Ontario. September 2, 2015.
2224:
2161:
2126:
2101:
1973:
1781:
1613:
1602:
1498:
468:
320:
301:
279:
131:
79:
270:
2151:
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1727:
1520:
1479:
877:
619:
354:
290:
180:
1374:
1322:
1174:
1026:
411:
candidate for haemophilia B called FLT180 were announced, it works using an
316:
The diagnosis for haemophilia B can be done via the following tests/methods:
2096:
2060:
1683:
1509:
1431:
1259:
1234:
733:
512:
500:
476:
416:
369:
346:
305:
259:
184:
139:
17:
1392:
1340:
1268:
1182:
1131:
1080:
1034:
751:
631:
228:
recessive trait, which explains why males are affected in greater numbers.
1366:
1017:
1000:
427:
341:
The differential diagnosis for this inherited condition is the following:
2111:
2065:
2050:
1909:
1740:
1598:
1351:"Long-Term Safety and Efficacy of Factor IX Gene Therapy in Hemophilia B"
286:
244:
225:
176:
135:
1414:
1195:(Subscription may be required or content may be available in libraries.)
1057:"Novel gene therapy could reduce bleeding risk for haemophilia patients"
2070:
1313:
2193:
2055:
1426:
1001:"Phase 1–2 Trial of AAVS3 Gene Therapy in Patients with Hemophilia B"
456:
440:
373:
294:
718:"Christmas disease: a condition previously mistaken for haemophilia"
712:
Biggs R, Douglas AS, MacFarlane RG, Dacie JV, Pitney WR, Merskey C,
282:, which can be either spontaneously or in response to mild trauma.
777:"FDA Approves First Gene Therapy to Treat Adults with Hemophilia B"
2672:
508:
269:
211:
921:"Factor IX Deficiency: Background, Pathophysiology, Epidemiology"
802:
This article incorporates text from this source, which is in the
2668:
2509:
1457:
895:"Hemophilia B: Practice Essentials, Background, Pathophysiology"
520:
489:
134:
disorder causing easy bruising and bleeding due to an inherited
2738:
2236:
2197:
1570:
1235:"Genotype Analysis Identifies the Cause of the "Royal Disease""
846:
300:
Factor IX becomes active eventually in coagulation by cofactor
516:
484:
248:
684:"Haemophilia B (Factor IX Deficiency) information | Patient"
1297:"Treatment of hemophilia B: focus on recombinant factor IX"
657:(19th ed.). Philadelphia: Saunders. pp. 1700–1.
278:
Factor IX deficiency leads to an increased propensity for
251:
of people with a rare and unusual form of haemophilia B –
1208:"Case Closed: Famous Royals Suffered From Hemophilia"
1404:
2689:
2667:
2639:
2602:
2568:
2479:
2358:
2326:
2298:
2245:
2086:
2043:
1964:
1908:
1850:
1843:
1836:
1764:
1726:
1639:
1632:
1489:
1408:
988:: 9 – via World Federation of Hemophilia (WFH).
113:
103:
95:
85:
73:
47:
42:
771:
769:
767:
765:
763:
761:
1233:Evgeny I. Rogaev; et al. (8 October 2009).
592:"Hemophilia B: MedlinePlus Medical Encyclopedia"
2415:Danon disease/glycogen storage disease Type IIb
1081:"Transformational therapy cures haemophilia B"
443:. He was the son of film and television actor
2520:Color blindness (red and green, but not blue)
2505:Alpha-thalassemia mental retardation syndrome
2209:
1582:
8:
2405:Glucose-6-phosphate dehydrogenase deficiency
847:"OMIM Entry - # 306900 - HEMOPHILIA B; HEMB"
27:Genetic X-linked recessive bleeding disorder
2735:
2233:
2216:
2202:
2194:
1847:
1840:
1636:
1589:
1575:
1567:
1405:
927:. Medscape. 24 August 2016. Archived from
109:Bleeding scores, Coagulation factor assays
56:
39:
2265:X-linked severe combined immunodeficiency
1382:
1330:
1312:
1258:
1016:
741:
467:discovered that he was not deficient in
426:
2659:X-linked nephrogenic diabetes insipidus
2584:Hypohidrotic ectodermal dysplasia (EDA)
574:
2594:X-linked endothelial corneal dystrophy
872:
870:
868:
866:
613:
611:
2550:Charcot–Marie–Tooth disease (CMTX2-3)
2374:Ornithine transcarbamylase deficiency
2350:X-linked adrenal hypoplasia congenita
1105:
1103:
1101:
889:
887:
678:
676:
674:
586:
584:
582:
580:
578:
262:, which prevents excessive bleeding.
220:The factor IX gene is located on the
7:
2285:X-linked lymphoproliferative disease
2255:Chronic granulomatous disease (CYBB)
2631:Emery–Dreifuss muscular dystrophy 1
2340:Spinal and bulbar muscular atrophy
65:This condition is inherited in an
25:
2410:Pyruvate dehydrogenase deficiency
956:. London: Springer. p. 416.
2490:X-linked intellectual disability
1943:platelet storage pool deficiency
1895:Heparin-induced thrombocytopenia
1540:
1206:Michael Price (8 October 2009).
1124:10.1046/j.1365-2141.2003.04333.x
797:
2336:Androgen insensitivity syndrome
1355:New England Journal of Medicine
1005:New England Journal of Medicine
558:Haemophilia in European royalty
533:Haemophilia in European royalty
51:Hemophilia B, Christmas disease
2712:Simpson–Golabi–Behmel syndrome
1662:Activated protein C resistance
1301:Biologics: Targets and Therapy
1167:10.1016/j.thromres.2013.10.020
415:(AAV) to restore the clotting
1:
2681:AMELX Amelogenesis imperfecta
2621:Centronuclear myopathy (MTM1)
2318:X-linked sideroblastic anemia
690:. 3 July 2014. Archived from
655:Nelson textbook of pediatrics
481:blood and plasma transfusions
390:(FDA) to treat Hemophilia B.
187:), and bleeding into joints (
2827:X-linked recessive disorders
2707:Smith–Fineman–Myers syndrome
2555:Pelizaeus–Merzbacher disease
2449:Purine–pyrimidine metabolism
1756:Trousseau sign of malignancy
783:Food and Drug Administration
479:). Stephen was dependent on
388:Food and Drug Administration
372:and/or blood transfusions.
274:Coagulation (FIX is on left)
2797:Craniofrontonasal dysplasia
2722:Nasodigitoacoustic syndrome
2270:X-linked agammaglobulinemia
2076:Nonthrombocytopenic purpura
1652:Antithrombin III deficiency
2848:
2792:Orofaciodigital syndrome 1
2626:Conradi–Hünermann syndrome
2379:Oculocerebrorenal syndrome
2031:Congenital afibrinogenemia
1935:Glanzmann's thrombasthenia
1699:Essential thrombocythaemia
901:. Medscape. 24 August 2016
530:
492:and died from it in 1993.
453:Hospital for Sick Children
407:In July 2022 results of a
179:, urinary tract bleeding (
29:
2752:X-linked hypophosphatemia
2747:
2734:
2612:Becker muscular dystrophy
2275:Hyper-IgM syndrome type 1
2232:
2147:Gastrointestinal bleeding
1949:Hermansky–Pudlak syndrome
1718:Antiphospholipid syndrome
1609:
653:Kliegman, Robert (2011).
475:in his honour (and later
384:Etranacogene dezaparvovec
224:(Xq27.1-q27.2). It is an
64:
55:
2717:Mohr–Tranebjærg syndrome
2260:Wiskott–Aldrich syndrome
2117:Subconjunctival bleeding
2107:Intracranial haemorrhage
1923:Bernard–Soulier syndrome
1886:Upshaw–Schulman syndrome
1860:Thrombocytopenic purpura
1689:Sticky platelet syndrome
563:von Willebrand's disease
483:, and was infected with
359:Bernard–Soulier syndrome
243:were preventing two key
239:showed that two sets of
2757:Focal dermal hypoplasia
2471:Occipital horn syndrome
2400:Carbohydrate metabolism
1260:10.1126/science.1180660
734:10.1136/bmj.2.4799.1378
160:British Medical Journal
2772:Incontinentia pigmenti
2579:Dyskeratosis congenita
2423:Lipid storage disorder
2345:KAL1 Kallmann syndrome
2021:Factor XIII deficiency
2001:Hypoprothrombinemia/II
1996:von Willebrand disease
1954:Gray platelet syndrome
465:Robert Gwyn Macfarlane
432:
413:adeno-associated virus
351:von Willebrand disease
337:Differential diagnosis
275:
247:from attaching to the
217:
175:Symptoms include easy
2495:Coffin–Lowry syndrome
2436:Mucopolysaccharidosis
2016:Factor XII deficiency
2006:Factor VII deficiency
1824:Renal vein thrombosis
1367:10.1056/NEJMoa1407309
1281:subscription required
1212:ScienceNOW Daily News
1018:10.1056/NEJMoa2119913
953:Diagnostic hematology
950:Beck, Norman (2009).
430:
394:Dental considerations
273:
215:
117:Factor IX concentrate
2787:Lujan–Fryns syndrome
2454:Lesch–Nyhan syndrome
2392:Adrenoleukodystrophy
2290:Properdin deficiency
1772:Deep vein thrombosis
1667:Protein S deficiency
1657:Protein C deficiency
304:(specifically IXa).
253:haemophilia B Leyden
99:Factor IX deficiency
2589:X-linked ichthyosis
2137:Pulmonary haematoma
2011:Factor X deficiency
1900:May–Hegglin anomaly
1677:Prothrombin G20210A
1251:2009Sci...326..817R
1155:Thrombosis Research
527:Society and culture
497:fresh frozen plasma
2762:Fragile X syndrome
2572:and related tissue
2239:X-linked recessive
2044:Signs and symptoms
1819:Pulmonary embolism
1624:Bleeding diathesis
1490:External resources
1314:10.2147/BTT.S31582
787:. 22 November 2022
447:. He emigrated to
433:
330:Coagulation factor
276:
260:clotting factor IX
218:
171:Signs and symptoms
67:X-linked recessive
2809:
2808:
2805:
2804:
2741:X-linked dominant
2730:
2729:
2690:No primary system
2191:
2190:
2187:
2186:
2039:
2038:
2026:Dysfibrinogenemia
1910:Platelet function
1832:
1831:
1711:Purpura fulminans
1535:
1534:
1361:(21): 1994–2004.
1187: – via
931:on 6 October 2016
833:978-1-60795-188-9
728:(4799): 1378–82.
664:978-1-4377-0755-7
241:genetic mutations
155:Stephen Christmas
151:Christmas disease
138:of the gene for
121:
120:
105:Diagnostic method
37:Medical condition
16:(Redirected from
2839:
2767:Aicardi syndrome
2736:
2234:
2218:
2211:
2204:
2195:
2132:Haemopericardium
1852:Thrombocytopenia
1848:
1841:
1797:Lowenberg's sign
1647:Clotting factors
1637:
1591:
1584:
1577:
1568:
1544:
1543:
1406:
1396:
1386:
1344:
1334:
1316:
1282:
1280:
1262:
1230:
1224:
1223:
1221:
1219:
1203:
1197:
1196:
1193:
1186:
1150:
1144:
1143:
1107:
1096:
1095:
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1092:
1077:
1071:
1070:
1068:
1067:
1053:
1047:
1046:
1020:
996:
990:
981:
975:
974:
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947:
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874:
861:
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843:
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680:
669:
668:
650:
644:
642:
640:
638:
615:
606:
605:
603:
602:
588:
473:Christmas factor
449:Toronto, Ontario
60:
40:
21:
2847:
2846:
2842:
2841:
2840:
2838:
2837:
2836:
2812:
2811:
2810:
2801:
2743:
2726:
2702:McLeod syndrome
2685:
2663:
2649:Alport syndrome
2635:
2598:
2564:
2525:Ocular albinism
2475:
2441:Hunter syndrome
2354:
2322:
2294:
2241:
2228:
2222:
2192:
2183:
2157:Haemoperitoneum
2082:
2035:
1966:Clotting factor
1960:
1904:
1828:
1777:Bancroft's sign
1760:
1751:Virchow's triad
1722:
1672:Factor V Leiden
1628:
1605:
1595:
1565:
1564:
1563:
1545:
1541:
1536:
1531:
1530:
1485:
1484:
1417:
1403:
1347:
1294:
1291:
1289:Further reading
1286:
1285:
1232:
1231:
1227:
1217:
1215:
1205:
1204:
1200:
1194:
1191:
1152:
1151:
1147:
1112:Br. J. Haematol
1109:
1108:
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1074:
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1055:
1054:
1050:
998:
997:
993:
982:
978:
968:
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932:
919:
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893:
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885:
876:
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864:
855:
853:
845:
844:
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821:
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798:
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788:
775:
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759:
711:
710:
706:
697:
695:
682:
681:
672:
665:
652:
651:
647:
636:
634:
617:
616:
609:
600:
598:
596:medlineplus.gov
590:
589:
576:
571:
544:
535:
529:
425:
405:
396:
378:tranexamic acid
366:
339:
326:Bleeding scores
314:
268:
266:Pathophysiology
237:Merlin Crossley
233:George Brownlee
210:
197:
183:), nosebleeds (
173:
126:, also spelled
38:
35:
28:
23:
22:
15:
12:
11:
5:
2845:
2843:
2835:
2834:
2832:Coagulopathies
2829:
2824:
2814:
2813:
2807:
2806:
2803:
2802:
2800:
2799:
2794:
2789:
2784:
2782:CHILD syndrome
2779:
2774:
2769:
2764:
2759:
2754:
2748:
2745:
2744:
2739:
2732:
2731:
2728:
2727:
2725:
2724:
2719:
2714:
2709:
2704:
2699:
2697:Barth syndrome
2693:
2691:
2687:
2686:
2684:
2683:
2677:
2675:
2665:
2664:
2662:
2661:
2656:
2654:Dent's disease
2651:
2645:
2643:
2637:
2636:
2634:
2633:
2628:
2623:
2618:
2608:
2606:
2600:
2599:
2597:
2596:
2591:
2586:
2581:
2575:
2573:
2566:
2565:
2563:
2562:
2557:
2552:
2543:
2542:
2537:
2535:Norrie disease
2532:
2522:
2517:Eye disorders:
2513:
2512:
2507:
2502:
2497:
2485:
2483:
2481:Nervous system
2477:
2476:
2474:
2473:
2467:Menkes disease
2457:
2456:
2444:
2443:
2431:
2430:
2418:
2417:
2412:
2407:
2395:
2394:
2382:
2381:
2376:
2364:
2362:
2356:
2355:
2353:
2352:
2347:
2342:
2332:
2330:
2324:
2323:
2321:
2320:
2315:
2310:
2304:
2302:
2296:
2295:
2293:
2292:
2287:
2282:
2277:
2272:
2267:
2262:
2257:
2251:
2249:
2243:
2242:
2237:
2230:
2229:
2223:
2221:
2220:
2213:
2206:
2198:
2189:
2188:
2185:
2184:
2182:
2181:
2180:
2179:
2171:
2170:
2169:
2167:Haematosalpinx
2164:
2159:
2154:
2149:
2141:
2140:
2139:
2134:
2129:
2121:
2120:
2119:
2114:
2109:
2104:
2099:
2090:
2088:
2084:
2083:
2081:
2080:
2079:
2078:
2068:
2063:
2058:
2053:
2047:
2045:
2041:
2040:
2037:
2036:
2034:
2033:
2028:
2023:
2018:
2013:
2008:
2003:
1998:
1993:
1992:
1991:
1986:
1981:
1970:
1968:
1962:
1961:
1959:
1958:
1957:
1956:
1951:
1939:
1938:
1937:
1927:
1926:
1925:
1914:
1912:
1906:
1905:
1903:
1902:
1897:
1891:
1890:
1889:
1888:
1883:
1873:
1872:
1871:
1869:Evans syndrome
1856:
1854:
1845:
1838:
1834:
1833:
1830:
1829:
1827:
1826:
1821:
1816:
1815:
1814:
1809:
1804:
1802:Peabody's sign
1799:
1794:
1789:
1784:
1779:
1768:
1766:
1762:
1761:
1759:
1758:
1753:
1748:
1743:
1738:
1732:
1730:
1724:
1723:
1721:
1720:
1715:
1714:
1713:
1703:
1702:
1701:
1696:
1694:Thrombocytosis
1691:
1681:
1680:
1679:
1674:
1669:
1664:
1659:
1654:
1643:
1641:
1634:
1630:
1629:
1627:
1626:
1621:
1616:
1610:
1607:
1606:
1596:
1594:
1593:
1586:
1579:
1571:
1546:
1539:
1538:
1537:
1533:
1532:
1529:
1528:
1517:
1506:
1494:
1493:
1491:
1487:
1486:
1483:
1482:
1471:
1460:
1449:
1434:
1418:
1413:
1412:
1410:
1409:Classification
1402:
1401:External links
1399:
1398:
1397:
1345:
1290:
1287:
1284:
1283:
1225:
1198:
1145:
1097:
1072:
1048:
1011:(3): 237–247.
991:
976:
962:
942:
912:
883:
862:
838:
819:
808:
757:
704:
670:
663:
645:
620:"Hemophilia B"
607:
573:
572:
570:
567:
566:
565:
560:
555:
550:
543:
540:
531:Main article:
528:
525:
461:Rosemary Biggs
445:Eric Christmas
424:
421:
404:
401:
395:
392:
365:
362:
357:disorders and
338:
335:
334:
333:
327:
324:
323:screening test
313:
310:
267:
264:
209:
206:
196:
193:
172:
169:
153:, named after
132:blood clotting
119:
118:
115:
111:
110:
107:
101:
100:
97:
93:
92:
89:
83:
82:
77:
71:
70:
62:
61:
53:
52:
49:
45:
44:
36:
26:
24:
14:
13:
10:
9:
6:
4:
3:
2:
2844:
2833:
2830:
2828:
2825:
2823:
2820:
2819:
2817:
2798:
2795:
2793:
2790:
2788:
2785:
2783:
2780:
2778:
2777:Rett syndrome
2775:
2773:
2770:
2768:
2765:
2763:
2760:
2758:
2755:
2753:
2750:
2749:
2746:
2742:
2737:
2733:
2723:
2720:
2718:
2715:
2713:
2710:
2708:
2705:
2703:
2700:
2698:
2695:
2694:
2692:
2688:
2682:
2679:
2678:
2676:
2674:
2670:
2666:
2660:
2657:
2655:
2652:
2650:
2647:
2646:
2644:
2642:
2638:
2632:
2629:
2627:
2624:
2622:
2619:
2617:
2613:
2610:
2609:
2607:
2605:
2604:Neuromuscular
2601:
2595:
2592:
2590:
2587:
2585:
2582:
2580:
2577:
2576:
2574:
2571:
2567:
2561:
2558:
2556:
2553:
2551:
2548:
2545:
2544:
2541:
2540:Choroideremia
2538:
2536:
2533:
2530:
2526:
2523:
2521:
2518:
2515:
2514:
2511:
2508:
2506:
2503:
2501:
2500:MASA syndrome
2498:
2496:
2493:
2491:
2487:
2486:
2484:
2482:
2478:
2472:
2468:
2465:
2463:
2459:
2458:
2455:
2452:
2450:
2446:
2445:
2442:
2439:
2437:
2433:
2432:
2429:
2428:Fabry disease
2425:
2424:
2420:
2419:
2416:
2413:
2411:
2408:
2406:
2403:
2401:
2397:
2396:
2393:
2390:
2388:
2384:
2383:
2380:
2377:
2375:
2372:
2370:
2366:
2365:
2363:
2361:
2357:
2351:
2348:
2346:
2343:
2341:
2337:
2334:
2333:
2331:
2329:
2325:
2319:
2316:
2314:
2313:Haemophilia B
2311:
2309:
2308:Haemophilia A
2306:
2305:
2303:
2301:
2297:
2291:
2288:
2286:
2283:
2281:
2278:
2276:
2273:
2271:
2268:
2266:
2263:
2261:
2258:
2256:
2253:
2252:
2250:
2248:
2244:
2240:
2235:
2231:
2226:
2219:
2214:
2212:
2207:
2205:
2200:
2199:
2196:
2178:
2177:Haemarthrosis
2175:
2174:
2172:
2168:
2165:
2163:
2160:
2158:
2155:
2153:
2150:
2148:
2145:
2144:
2142:
2138:
2135:
2133:
2130:
2128:
2125:
2124:
2122:
2118:
2115:
2113:
2110:
2108:
2105:
2103:
2100:
2098:
2095:
2094:
2092:
2091:
2089:
2085:
2077:
2074:
2073:
2072:
2069:
2067:
2064:
2062:
2059:
2057:
2054:
2052:
2049:
2048:
2046:
2042:
2032:
2029:
2027:
2024:
2022:
2019:
2017:
2014:
2012:
2009:
2007:
2004:
2002:
1999:
1997:
1994:
1990:
1987:
1985:
1982:
1980:
1977:
1976:
1975:
1972:
1971:
1969:
1967:
1963:
1955:
1952:
1950:
1947:
1946:
1945:
1944:
1940:
1936:
1933:
1932:
1931:
1928:
1924:
1921:
1920:
1919:
1916:
1915:
1913:
1911:
1907:
1901:
1898:
1896:
1893:
1892:
1887:
1884:
1882:
1879:
1878:
1877:
1874:
1870:
1867:
1866:
1865:
1861:
1858:
1857:
1855:
1853:
1849:
1846:
1842:
1839:
1835:
1825:
1822:
1820:
1817:
1813:
1810:
1808:
1805:
1803:
1800:
1798:
1795:
1793:
1792:Louvel's sign
1790:
1788:
1787:Lisker's sign
1785:
1783:
1780:
1778:
1775:
1774:
1773:
1770:
1769:
1767:
1763:
1757:
1754:
1752:
1749:
1747:
1744:
1742:
1739:
1737:
1736:Thrombophilia
1734:
1733:
1731:
1729:
1725:
1719:
1716:
1712:
1709:
1708:
1707:
1704:
1700:
1697:
1695:
1692:
1690:
1687:
1686:
1685:
1682:
1678:
1675:
1673:
1670:
1668:
1665:
1663:
1660:
1658:
1655:
1653:
1650:
1649:
1648:
1645:
1644:
1642:
1638:
1635:
1631:
1625:
1622:
1620:
1617:
1615:
1612:
1611:
1608:
1604:
1600:
1597:Disorders of
1592:
1587:
1585:
1580:
1578:
1573:
1572:
1569:
1561:
1560:
1559:
1558:Haemophilia B
1553:
1549:
1527:
1526:Haemophilia B
1523:
1522:
1518:
1516:
1512:
1511:
1507:
1505:
1501:
1500:
1496:
1495:
1492:
1488:
1481:
1477:
1476:
1472:
1470:
1466:
1465:
1461:
1459:
1455:
1454:
1450:
1448:
1444:
1443:
1439:
1435:
1433:
1429:
1428:
1424:
1420:
1419:
1416:
1411:
1407:
1400:
1394:
1390:
1385:
1380:
1376:
1372:
1368:
1364:
1360:
1356:
1352:
1346:
1342:
1338:
1333:
1328:
1324:
1320:
1315:
1310:
1306:
1302:
1298:
1293:
1292:
1288:
1278:
1274:
1270:
1266:
1261:
1256:
1252:
1248:
1245:(5954): 817.
1244:
1240:
1236:
1229:
1226:
1213:
1209:
1202:
1199:
1190:
1189:ScienceDirect
1184:
1180:
1176:
1172:
1168:
1164:
1160:
1156:
1149:
1146:
1141:
1137:
1133:
1129:
1125:
1121:
1118:(5): 703–12.
1117:
1113:
1106:
1104:
1102:
1098:
1086:
1082:
1076:
1073:
1062:
1058:
1052:
1049:
1044:
1040:
1036:
1032:
1028:
1024:
1019:
1014:
1010:
1006:
1002:
995:
992:
989:
987:
980:
977:
965:
963:9781848002951
959:
955:
954:
946:
943:
930:
926:
922:
916:
913:
900:
896:
890:
888:
884:
879:
873:
871:
869:
867:
863:
852:
848:
842:
839:
836:
834:
830:
823:
820:
817:
812:
809:
805:
804:public domain
786:
784:
778:
772:
770:
768:
766:
764:
762:
758:
753:
749:
744:
739:
735:
731:
727:
723:
719:
715:
708:
705:
694:on 2024-02-26
693:
689:
685:
679:
677:
675:
671:
666:
660:
656:
649:
646:
633:
629:
625:
621:
614:
612:
608:
597:
593:
587:
585:
583:
581:
579:
575:
568:
564:
561:
559:
556:
554:
553:Haemophilia C
551:
549:
548:Haemophilia A
546:
545:
541:
539:
534:
526:
524:
522:
518:
514:
510:
506:
502:
498:
493:
491:
486:
482:
478:
474:
470:
466:
462:
458:
454:
450:
446:
442:
438:
429:
422:
420:
418:
414:
410:
402:
400:
393:
391:
389:
385:
381:
379:
375:
371:
363:
361:
360:
356:
352:
348:
344:
343:haemophilia A
336:
331:
328:
325:
322:
319:
318:
317:
311:
309:
307:
303:
298:
296:
292:
288:
283:
281:
272:
265:
263:
261:
256:
254:
250:
246:
242:
238:
234:
229:
227:
223:
214:
207:
205:
201:
195:Complications
194:
192:
190:
189:haemarthrosis
186:
182:
178:
170:
168:
164:
162:
161:
156:
152:
147:
145:
144:haemophilia A
141:
137:
133:
129:
125:
124:Haemophilia B
116:
112:
108:
106:
102:
98:
94:
91:Easy bruising
90:
88:
84:
81:
78:
76:
72:
68:
63:
59:
54:
50:
46:
43:Haemophilia B
41:
33:
32:haemophilia A
19:
2546:
2516:
2488:
2460:
2447:
2434:
2421:
2398:
2387:Dyslipidemia
2385:
2367:
2312:
1983:
1941:
1929:
1917:
1807:Pratt's sign
1619:coagulopathy
1556:
1555:
1554:profile for
1551:
1519:
1508:
1497:
1473:
1462:
1451:
1436:
1421:
1358:
1354:
1304:
1300:
1242:
1238:
1228:
1216:. Retrieved
1211:
1201:
1158:
1154:
1148:
1115:
1111:
1089:. Retrieved
1087:. 2022-07-21
1084:
1075:
1064:. Retrieved
1061:ScienceDaily
1060:
1051:
1008:
1004:
994:
985:
983:
979:
967:. Retrieved
952:
945:
933:. Retrieved
929:the original
924:
915:
903:. Retrieved
898:
878:"Hemophilia"
854:. Retrieved
850:
841:
826:
822:
815:
811:
789:. Retrieved
780:
725:
721:
707:
696:. Retrieved
692:the original
687:
654:
648:
635:. Retrieved
623:
599:. Retrieved
595:
536:
494:
434:
409:gene therapy
406:
397:
382:
367:
349:deficiency,
340:
315:
299:
297:conversion.
289:which helps
284:
277:
257:
252:
230:
222:X chromosome
219:
216:X chromosome
202:
198:
174:
165:
158:
150:
148:
128:hemophilia B
127:
123:
122:
18:Hemophilia B
2822:Haemophilia
2300:Hematologic
2162:Haematocele
2127:Haemothorax
2102:Haemoptysis
1974:Haemophilia
1930:aggregation
1812:Rose's sign
1782:Homans sign
1614:Coagulation
1499:MedlinePlus
791:22 November
643:update 2014
624:GeneReviews
469:Factor VIII
321:Coagulation
302:factor VIII
280:haemorrhage
80:Haematology
48:Other names
2816:Categories
2369:Amino acid
2152:Haemobilia
1746:Thrombosis
1521:Patient UK
1475:DiseasesDB
1091:2022-08-03
1066:2022-08-03
856:2016-10-07
714:O'Brien JR
698:2016-04-21
601:2016-09-21
569:References
511:, such as
439:family in
355:fibrinogen
291:fibrinogen
181:haematuria
2360:Metabolic
2328:Endocrine
2227:disorders
2097:Epistaxis
2061:Haematoma
1684:Platelets
1515:emerg/240
1510:eMedicine
1375:0028-4793
1323:1177-5475
1307:: 33–38.
1277:206522975
1218:9 October
1175:1879-2472
1161:: S4–S9.
1043:250697905
1027:0028-4793
969:7 October
935:7 October
925:eMedicine
905:7 October
899:eMedicine
637:7 October
513:hepatitis
501:factor IX
477:Factor IX
431:Factor IX
417:factor IX
370:factor IX
364:Treatment
347:factor XI
312:Diagnosis
306:Platelets
231:In 1990,
185:epistaxis
140:factor IX
114:Treatment
75:Specialty
2641:Urologic
2616:Duchenne
2225:X-linked
2143:abdomen
2112:Hyphaema
2066:Petechia
2051:Bleeding
1918:adhesion
1844:By cause
1837:Bleeding
1741:Thrombus
1640:By cause
1633:Clotting
1603:clotting
1599:bleeding
1393:25409372
1341:23430394
1269:19815722
1183:24513149
1140:22694905
1132:12780784
1085:BBC News
1035:35857660
851:omim.org
752:12997790
722:Br Med J
716:(1952).
632:20301668
542:See also
459:, where
403:Research
287:factor X
245:proteins
226:X-linked
208:Genetics
177:bruising
136:mutation
87:Symptoms
2462:Mineral
2087:By site
2071:Purpura
1765:By site
1548:Scholia
1469:D002836
1384:4278802
1332:3575125
1247:Bibcode
1239:Science
743:2022306
688:Patient
509:viruses
437:British
423:History
130:, is a
69:manner.
2547:Other:
2247:Immune
2173:joint
2123:torso
2056:Bruise
1979:A/VIII
1550:has a
1504:000539
1458:306900
1391:
1381:
1373:
1339:
1329:
1321:
1275:
1267:
1214:. AAAS
1192:
1181:
1173:
1138:
1130:
1041:
1033:
1025:
960:
831:
750:
740:
661:
630:
515:, and
505:plasma
457:Oxford
441:London
374:NSAIDS
332:assays
295:fibrin
96:Causes
2673:tooth
2560:SMAX2
2093:head
1728:Clots
1552:topic
1447:286.1
1273:S2CID
1136:S2CID
1039:S2CID
785:(FDA)
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2669:Bone
2570:Skin
2510:PHF8
2280:IPEX
1989:C/XI
1984:B/IX
1601:and
1480:5561
1464:MeSH
1453:OMIM
1442:9-CM
1389:PMID
1371:ISSN
1337:PMID
1319:ISSN
1265:PMID
1220:2009
1179:PMID
1171:ISSN
1128:PMID
1031:PMID
1023:ISSN
971:2016
958:ISBN
937:2016
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793:2022
748:PMID
659:ISBN
639:2016
628:PMID
521:gene
490:AIDS
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1881:TTP
1864:ITP
1706:DIC
1438:ICD
1432:D67
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1363:doi
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1255:doi
1243:326
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1159:134
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