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Lack of detailed natural history and outcomes data for neonates and toddlers with haemophilia hampers the provision of optimal management of the disorder. We report an analysis of prospective data collected from 580 neonates and toddlers aged 0–2 years with haemophilia enrolled in the Universal Data Collection (UDC) surveillance project of the Centers for Disease Control and Prevention (CDC). This study focuses on a cohort of babies with haemophilia whose diagnosis was established before the age of two. The mode of delivery, type and severity of haemophilia, onset and timing of haemorrhages, site(s) of bleeding, provision of prophylaxis with coagulation factor replacement therapy, and the role played by the federally funded Haemophilia Treatment Centers (HTC) in the management of these infants with haemophilia were evaluated. Seventy‐five per cent of haemophilic infants were diagnosed early, in the first month of life, especially those with a family history or whose mothers were known carriers; infants of maternal carriers were more likely to be delivered by C‐section. Involvement of an HTC prior to delivery resulted in avoidance of the use of assisted deliveries with vacuum and forceps. Bleeding from the circumcision site was the most common haemorrhagic complication, followed by intra‐ and extra‐cranial haemorrhages and bleeding from heel stick blood sampling. Eight per cent of the infants were administered factor concentrate within 24 h of birth; more than half were treated to prevent bleeding. 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The gynaecological and obstetric management of women with inherited coagulation disorders requires close collaboration between obstetrician\u002Fgynaecologists and haematologists. Ideally these women should be managed in a joint disciplinary clinic where expertise and facilities are available to provide comprehensive assessment of the bleeding disorder and a combined plan of management. The haematologist should arrange and interpret laboratory tests and make provision for appropriate replacement therapy. 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A, 2000, Endometrial resection and ablation versus hysterectomy for heavy menstrual bleeding, Cochrane Database Syst Rev, CD000329",{},{"id":23,"text":2041,"url":23,"identifiers":2042},"10.1093\u002Fhumrep\u002F17.11.2795",{"doi":2041},{"id":23,"text":2044,"url":23,"identifiers":2045},"10.1016\u002FS1074-3804(05)60205-8",{"doi":2044},{"id":23,"text":2047,"url":23,"identifiers":2048},"10.1111\u002Fj.1365-2516.2004.00915.x",{"doi":2047},{"id":23,"text":895,"url":23,"identifiers":2050},{},{"id":23,"text":907,"url":23,"identifiers":2052},{"doi":909},{"id":23,"text":914,"url":23,"identifiers":2054},{"doi":914},{"id":23,"text":892,"url":23,"identifiers":2056},{"doi":892},{"id":23,"text":919,"url":23,"identifiers":2058},{"doi":919},{"id":23,"text":2060,"url":23,"identifiers":2061},"Bottini E, 1991, Prevention of hemoperitoneum during ovulation by oral contraceptives in women with type III von Willebrand disease and afibrinogenemia. Case reports, Haematologica, 76, 431",{},{"id":23,"text":924,"url":23,"identifiers":2063},{"doi":924},{"id":23,"text":936,"url":23,"identifiers":2065},{"doi":936},{"id":23,"text":2067,"url":23,"identifiers":2068},"10.1093\u002Foxfordjournals.humrep.a019537",{"doi":2067},{"id":23,"text":2070,"url":23,"identifiers":2071},"10.1542\u002Fpeds.68.5.661",{"doi":2070},{"id":23,"text":2073,"url":23,"identifiers":2074},"10.1001\u002Farchpedi.1997.02170460043007",{"doi":2073},{"id":23,"text":2076,"url":23,"identifiers":2077},"10.1016\u002FS1083-3188(00)00076-0",{"doi":2076},{"id":23,"text":2079,"url":23,"identifiers":2080},"10.1001\u002Farchpedi.154.12.1226",{"doi":2079},{"id":23,"text":996,"url":23,"identifiers":2082},{"doi":996},{"id":23,"text":990,"url":23,"identifiers":2084},{"doi":990},{"id":23,"text":2086,"url":23,"identifiers":2087},"10.1055\u002Fs-2003-38832",{"doi":2086},{"id":23,"text":1017,"url":23,"identifiers":2089},{"doi":1017},{"id":23,"text":999,"url":23,"identifiers":2091},{"doi":999},{"id":23,"text":1014,"url":23,"identifiers":2093},{"doi":1014},{"id":23,"text":1033,"url":23,"identifiers":2095},{"doi":1033},{"id":23,"text":1036,"url":23,"identifiers":2097},{"doi":1036},{"id":23,"text":2099,"url":23,"identifiers":2100},"10.1056\u002FNEJM198807283190401",{"doi":2099},{"id":23,"text":1005,"url":23,"identifiers":2102},{"doi":1005},{"id":23,"text":1011,"url":23,"identifiers":2104},{"doi":1011},{"id":23,"text":904,"url":23,"identifiers":2106},{"doi":904},{"id":2108,"createTime":2109,"updateTime":2109,"relativeEntities":2110,"slug":2111,"properties":2112,"entityType":117,"verifyStatus":118,"verifyTime":2124,"verifyNote":120,"languages":2125,"translateLanguages":23,"viewCount":24,"primaryUrl":2126,"fullTextUrl":23,"authors":2127,"publicationType":180,"publisherRelationship":2296,"citationCount":2350,"citationInfo":2351,"publishDate":2354,"publishYear":2352,"citationAnalyzeStatus":22,"lastCitationAnalyze":23,"indexDatabases":2355,"openAccess":23,"references":2356,"isForceReanalyzing":274},"abec01ae-361d-46cc-a218-5de440d0f620","2025-02-02T15:31:29.318+00:00",[],"Obstetric-management-in-von-Willebrand-s-disease-a-report-of-24-pregnancies-and-a-reivesw-of-the-literature",{"openalex":2113,"mag":2115,"abstract":2117,"title":2119,"pm":2121,"doi":2123},{"VOID":2114},"W2104125149",{"VOID":2116},"2104125149",{"EN":2118},"\u003Cjats:title>Summary\u003C\u002Fjats:title>\u003Cjats:p>The case records of 13 patients (24 pregnancies) with von Willebrand's disease (vWD) were studies rettospectively. The overall incidence of primary and secondary post‐partum haemorrhage (PPH) was 15.8% and 25% respectively, all primary PPH occurring in tyre 2 discase (3\u002F14 deliveries, 21.4%). The risk of primary PPH in type 2 patients who did not receive prophylactic factor VIII was 37.5% (3\u002F8 deliveries).\u003C\u002Fjats:p>\u003Cjats:p>Factor VIII coagulant activity (VIII:C) and von Willebrand factor antigen (vWF:Ag) rose above bascline values by a factor of at least 1.5 during the pregnancy in most case. More severely affected patients were less likely to benefit significatntly. A baseline VIII:C of <15 iu\u002Fdl (4\u002F14 cases) was predictive of a third trimester level of <15 iu\u002Fdl. Improvements in the von Willebrand factor activity were less marked. The baseline von Willebrand factor activity was <15 iu\u002Fdl in all patients with serial data, none of whom achieved a third‐trimester von Willebrand factor activity of >50 iu\u002Fdl. The bleeding times were unaltered significantly in all but one of the cases, reflecting a general failure of the primary haemostatic defect to improve with pregnancy.\u003C\u002Fjats:p>\u003Cjats:p>The findings demonstrate that coagulation parameters do not universally improve in pregnancy in vWD, especially when preconception levels are low. The risk of primary PPH is generally higher in type 2 diseases. The level of factor VIII:C is not a good predictor of the risk of primary PPH in type 2 patients. Secondary PPH is a significatnt risk in both type 1 and type 2 patients.\u003C\u002Fjats:p>",{"EN":2120},"Obstetric management in von Willebrand's disease: a report of 24 pregnancies and a reivesw of the literature",{"VOID":2122},"27214325",{"VOID":975},"2025-02-02T15:31:29.317+00:00",[122],"https:\u002F\u002Fonlinelibrary.wiley.com\u002Fdoi\u002F10.1111\u002Fj.1365-2516.1995.tb00056.x",[2128,2177,2218,2257],{"id":2129,"sortIndex":24,"researcher":23,"roles":2130,"affiliations":2131,"properties":2172,"displayName":2174,"givenName":23,"familyName":23},"45c1d058-82f4-46bc-b51b-38173f0eb389",[],[2132,2140,2148,2156,2164],{"id":2133,"sortIndex":24,"affiliation":2134,"properties":23},"1d7bb289-db12-45de-a82b-dc0d6ffbf8b5",{"id":2133,"createTime":23,"updateTime":23,"relativeEntities":2135,"slug":23,"properties":2136,"entityType":23,"verifyStatus":23,"verifyTime":23,"verifyNote":23,"languages":23,"translateLanguages":23,"viewCount":23,"url":23,"parentIds":2139,"statistic":23},[],{"title":2137},{"EN":2138},"B. H. Ramsahoye, M.R.C.P., Registrar, Department of Haematology, University Hospital of Wales, Cardiff.",[],{"id":2141,"sortIndex":146,"affiliation":2142,"properties":23},"fc376f1e-9791-4f50-911f-e2b072475bd8",{"id":2141,"createTime":23,"updateTime":23,"relativeEntities":2143,"slug":23,"properties":2144,"entityType":23,"verifyStatus":23,"verifyTime":23,"verifyNote":23,"languages":23,"translateLanguages":23,"viewCount":23,"url":23,"parentIds":2147,"statistic":23},[],{"title":2145},{"EN":2146},"Departments of Haematology and Obstetrics and Gynaecology, University of Wales, Cardiff",[],{"id":2149,"sortIndex":164,"affiliation":2150,"properties":23},"65d1dcdc-0f70-42bc-af66-e0db593970ea",{"id":2149,"createTime":23,"updateTime":23,"relativeEntities":2151,"slug":23,"properties":2152,"entityType":23,"verifyStatus":23,"verifyTime":23,"verifyNote":23,"languages":23,"translateLanguages":23,"viewCount":23,"url":23,"parentIds":2155,"statistic":23},[],{"title":2153},{"EN":2154},"H. 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Y., 1984, Haemostatasis in normal pregnancy, Thrombos Haemostas, 52, 17",{},{"id":23,"text":1555,"url":23,"identifiers":2363},{"doi":1555},{"id":23,"text":2365,"url":23,"identifiers":2366},"Conti M., 1986, Pregnancy in women with different types of von Willebrand's disease, Obstet Gynaecol, 68, 282",{},{"id":23,"text":1586,"url":23,"identifiers":2368},{"doi":1586},{"id":23,"text":901,"url":23,"identifiers":2370},{"doi":901},{"id":23,"text":2372,"url":23,"identifiers":2373},"Noller KL., 1973, von Willebrand's discase in pregnancy, Obstet Gynaecol, 41, 865",{},{"id":23,"text":2375,"url":23,"identifiers":2376},"Krishnamurthy M., 1977, von Willebrand's disease and pregnancy, Obstet Gynaecol, 49, 244",{},{"id":23,"text":2378,"url":23,"identifiers":2379},"Hanna W., 1981, disease and pregnancy, Blood, 58, 873, 10.1182\u002Fblood.V58.5.873.873",{"doi":1550},{"id":23,"text":2381,"url":23,"identifiers":2382},"Lipton RA., 1982, Severe von Willebrand's disease during labour and delivery, J Am Med Assoc, 248, 1355, 10.1001\u002Fjama.1982.03330110051027",{"doi":2383},"10.1001\u002Fjama.1982.03330110051027",{"id":23,"text":1130,"url":23,"identifiers":2385},{"doi":1130},{"id":23,"text":2387,"url":23,"identifiers":2388},"Sadler JE., 1994, A revised classification of von Wilebrand disease, Thrombos Haemostas, 71, 520",{},{"id":23,"text":2390,"url":23,"identifiers":2391},"Beischer NA., 1986, Obstetrics and the Newborn",{},{"id":23,"text":2393,"url":23,"identifiers":2394},"10.1016\u002F0049-3848(83)90331-6",{"doi":2393},{"id":23,"text":1589,"url":23,"identifiers":2396},{"doi":1589},{"id":23,"text":2398,"url":23,"identifiers":2399},"10.1136\u002Fbmj.4.5832.114-c",{"doi":2398},{"id":23,"text":2401,"url":23,"identifiers":2402},"Rochelson B., 1991, The use of prophylactic desmopression (DDAVP) in labour to prevent haemorrhange in a patient with Ehlers‐DAnlos syndrome, NY State J Med, 91, 268",{},{"id":23,"text":2404,"url":23,"identifiers":2405},"Mannucci PM., 1988, Desmopressin: a non‐transfusional form of treatment for congenital and acquired bleeding disorders, Blood, 72, 1449, 10.1182\u002Fblood.V72.5.1449.1449",{"doi":2406},"10.1182\u002Fblood.V72.5.1449.1449",{"id":2408,"createTime":2409,"updateTime":2409,"relativeEntities":2410,"slug":2411,"properties":2412,"entityType":117,"verifyStatus":118,"verifyTime":2425,"verifyNote":120,"languages":2426,"translateLanguages":23,"viewCount":24,"primaryUrl":2427,"fullTextUrl":23,"authors":2428,"publicationType":180,"publisherRelationship":2612,"citationCount":2665,"citationInfo":2666,"publishDate":2681,"publishYear":2667,"citationAnalyzeStatus":22,"lastCitationAnalyze":23,"indexDatabases":2682,"openAccess":23,"references":2683,"isForceReanalyzing":274},"9f373ad2-bf88-40fb-8958-c814381815a0","2025-02-02T15:31:28.635+00:00",[],"von-Willebrand-disease-VWD-evidence-based-diagnosis-and-management-guidelines-the-National-Heart-Lung-and-Blood-Institute-NHLBI-Expert-Panel-report-USA-sup-1-sup-",{"openalex":2413,"mag":2415,"abstract":2417,"title":2419,"pm":2421,"doi":2423},{"VOID":2414},"W2169344158",{"VOID":2416},"2169344158",{"EN":2418},"\u003Cjats:p>\u003Cjats:bold>Summary. \u003C\u002Fjats:bold> von Willebrand disease (VWD) is a commonly encountered inherited bleeding disorder affecting both males and females, causing mucous membrane and skin bleeding symptoms, and bleeding with surgical or other haemostatic challenges. VWD may be disproportionately symptomatic in women of child‐bearing age. It may also occur less frequently as an acquired disorder (acquired von Willebrand syndrome). VWD is caused by deficiency or dysfunction of von Willebrand factor (VWF), a plasma protein that mediates platelet haemostatic function and stabilizes blood coagulation factor VIII. The pathophysiology, classification, diagnosis and management of VWD are relatively complex, but understanding them is important for proper diagnosis and management of patients with VWD. These evidence‐based guidelines for diagnosis and management of VWD from the National Heart, Lung, and Blood Institute (NHLBI) Expert Panel (USA) review relevant publications, summarize current understanding of VWD pathophysiology and classification, and present consensus diagnostic and management recommendations based on analysis of the literature and expert opinion. They also suggest an approach for clinical and laboratory evaluation of individuals with bleeding symptoms, history of bleeding or conditions associated with increased bleeding risk. This document summarizes needs for further research in VWF, VWD and bleeding disorders, including clinical research to obtain more objective information about bleeding symptoms, advancements in diagnostic and therapeutic tools, and enhancement in the education and training of clinicians and scientists in bleeding and thrombotic disorders. 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[package insert]. King of Prussia PA:CSL Behring 2007.",{},{"id":23,"text":3514,"url":23,"identifiers":3515},"Alphanate SD\u002FHT [package insert]. Los Angeles CA:Grifols 2007.",{},{"id":23,"text":3517,"url":23,"identifiers":3518},"10.1055\u002Fs-0037-1613227",{"doi":3517},{"id":23,"text":3520,"url":23,"identifiers":3521},"10.1055\u002Fs-0037-1613225",{"doi":3520},{"id":23,"text":3523,"url":23,"identifiers":3524},"10.1182\u002Fblood.V99.2.450",{"doi":3523},{"id":23,"text":3526,"url":23,"identifiers":3527},"10.1053\u002Fj.seminhematol.2004.09.007",{"doi":3526},{"id":23,"text":3529,"url":23,"identifiers":3530},"10.1055\u002Fs-2006-949668",{"doi":3529},{"id":23,"text":3532,"url":23,"identifiers":3533},"10.1111\u002Fj.1365-2516.2004.00893.x",{"doi":3532},{"id":23,"text":3535,"url":23,"identifiers":3536},"10.1055\u002Fs-0038-1656010",{"doi":3535},{"id":23,"text":3538,"url":23,"identifiers":3539},"US Food and Drug Administration, Additional Standards for Human Blood and Blood Products",{},{"id":23,"text":3541,"url":23,"identifiers":3542},"10.1097\u002F00062752-200311000-00003",{"doi":3541},{"id":23,"text":3544,"url":23,"identifiers":3545},"National Hemophilia Foundation, 2006, MASAC Document 177. 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510, 10.1055\u002Fs-0038-1651857",{"doi":3576},"10.1055\u002Fs-0038-1651857",{"id":23,"text":3578,"url":23,"identifiers":3579},"10.1016\u002F0049-3848(79)90282-2",{"doi":3578},{"id":23,"text":3581,"url":23,"identifiers":3582},"10.1111\u002Fj.1365-2141.1979.tb03798.x",{"doi":3581},{"id":23,"text":3584,"url":23,"identifiers":3585},"10.1182\u002Fblood.V92.8.2707",{"doi":3584},{"id":23,"text":3587,"url":23,"identifiers":3588},"Arkel YS, 1994, Treatment of acquired von Willebrand syndrome with intravenous immunoglobulin, Thromb Haemost, 72, 643, 10.1055\u002Fs-0038-1648928",{"doi":3589},"10.1055\u002Fs-0038-1648928",{"id":23,"text":3591,"url":23,"identifiers":3592},"Macik BG, 1988, The use of high‐dose intravenous gamma‐globulin in acquired von Willebrand syndrome, Arch Pathol Lab Med, 112, 143",{},{"id":23,"text":3594,"url":23,"identifiers":3595},"Van Genderen PJ, 1995, High‐dose intravenous immunoglobulin delays clearance of von Willebrand factor in acquired von Willebrand disease, Thromb 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Force for Epidemiological Research on Reproductive Health, United Nations Development Programme\u002FUnited Nations Population Fund\u002FWorld Health Organization\u002FWorld Bank Special Programme of Research, Development, Research Training in Human Reproduction, World Health Organization, Geneva, Switzerland, 1998, Effects of contraceptives on hemoglobin and ferritin, Contraception, 58, 262",{},{"id":23,"text":3660,"url":23,"identifiers":3661},"10.1016\u002F0002-9378(92)91394-P",{"doi":3660},{"id":23,"text":3663,"url":23,"identifiers":3664},"10.1592\u002Fphco.23.4.472.32120",{"doi":3663},{"id":23,"text":3666,"url":23,"identifiers":3667},"10.1016\u002FS0029-7844(00)01029-2",{"doi":3666},{"id":23,"text":901,"url":23,"identifiers":3669},{"doi":901},{"id":23,"text":865,"url":23,"identifiers":3671},{"doi":865},{"id":23,"text":2047,"url":23,"identifiers":3673},{"doi":2047},{"id":23,"text":3675,"url":23,"identifiers":3676},"10.1097\u002F00006250-200206000-00006",{"doi":3675},{"id":23,"text":3678,"url":23,"identifiers":3679},"10.1016\u002FS0029-7844(02)01954-3",{"doi":3678},{"id":23,"text":3681,"url":23,"identifiers":3682},"10.1016\u002FS1074-3804(05)60082-5",{"doi":3681},{"id":23,"text":3684,"url":23,"identifiers":3685},"10.1016\u002FS0029-7844(01)01380-1",{"doi":3684},{"id":23,"text":3687,"url":23,"identifiers":3688},"10.1111\u002Fj.1471-0528.1998.tb10236.x",{"doi":3687},{"id":23,"text":3690,"url":23,"identifiers":3691},"10.1001\u002Fjama.291.12.1447",{"doi":3690},{"id":23,"text":3693,"url":23,"identifiers":3694},"10.1016\u002FS0002-9378(96)70086-8",{"doi":3693},{"id":23,"text":3696,"url":23,"identifiers":3697},"Foster PA, 1995, The reproductive health of women with von Willebrand disease unresponsive to DDAVP: results of an international survey, Thromb Haemost, 74, 784, 10.1055\u002Fs-0038-1649813",{"doi":1041},{"id":23,"text":3699,"url":23,"identifiers":3700},"Bottini E, 1991, Prevention of hemoperitoneum during ovulation by oral contraceptives in women with type III von Willebrand disease and afibrinogenemia: case reports, Haematologica, 76, 431",{},{"id":23,"text":924,"url":23,"identifiers":3702},{"doi":924},{"id":23,"text":921,"url":23,"identifiers":3704},{"doi":921},{"id":23,"text":892,"url":23,"identifiers":3706},{"doi":892},{"id":23,"text":1121,"url":23,"identifiers":3708},{"doi":1121},{"id":23,"text":972,"url":23,"identifiers":3710},{"doi":972},{"id":23,"text":1052,"url":23,"identifiers":3712},{},{"id":23,"text":3714,"url":23,"identifiers":3715},"10.1053\u002Fbeha.2001.0140",{"doi":3714},{"id":23,"text":1191,"url":23,"identifiers":3717},{"doi":1191},{"id":23,"text":3719,"url":23,"identifiers":3720},"10.1182\u002Fblood.V97.7.1915",{"doi":3719},{"id":23,"text":3722,"url":23,"identifiers":3723},"10.3109\u002F00016347009157244",{"doi":3722},{"id":23,"text":3725,"url":23,"identifiers":3726},"10.1136\u002Fbmj.1.6115.756-a",{"doi":3725},{"id":23,"text":1598,"url":23,"identifiers":3728},{"doi":1600},{"id":23,"text":3730,"url":23,"identifiers":3731},"Storm O, 1976, Prolonged treatment with tranexamic acid (Cyclocapron) during pregnancy [Danish], Ugeskr Laeger, 138, 1781",{},{"id":23,"text":3733,"url":23,"identifiers":3734},"10.1111\u002Fj.1600-0609.1981.tb00467.x",{"doi":3733},{"id":23,"text":1602,"url":23,"identifiers":3736},{"doi":1602},{"id":23,"text":1595,"url":23,"identifiers":3738},{"doi":1595},{"id":23,"text":3740,"url":23,"identifiers":3741},"Howorka E, 1970, Effect of EACA administered to female rabbits during pregnancy on the fetuses [Polish], Patol Pol, 21, 311",{},{"id":23,"text":3743,"url":23,"identifiers":3744},"Kassam SH, 1984, Sickle‐cell‐induced hematuria in pregnancy: the current diagnostic and therapeutic approach, J Reprod Med, 29, 117",{},{"id":23,"text":1033,"url":23,"identifiers":3746},{"doi":1033},{"id":23,"text":1036,"url":23,"identifiers":3748},{"doi":1036},{"id":23,"text":1136,"url":23,"identifiers":3750},{"doi":1136},{"id":23,"text":1133,"url":23,"identifiers":3752},{"doi":1133},{"id":23,"text":1130,"url":23,"identifiers":3754},{"doi":1130},{"id":23,"text":3756,"url":23,"identifiers":3757},"10.1046\u002Fj.1365-2044.2000.01798-24.x",{"doi":3756},{"id":23,"text":3759,"url":23,"identifiers":3760},"Ross MG, 2002, Obstetrics: Normal and Problem Pregnancies, 37",{},{"id":23,"text":3762,"url":23,"identifiers":3763},"Benedetti TJ, 2005, Obstetrics: Normal and Problem Pregnancies, 503",{},{"id":23,"text":911,"url":23,"identifiers":3765},{"doi":911},{"id":23,"text":1064,"url":23,"identifiers":3767},{"doi":1064},{"id":23,"text":1073,"url":23,"identifiers":3769},{"doi":1073},{"id":23,"text":1076,"url":23,"identifiers":3771},{"doi":1076},{"id":23,"text":1079,"url":23,"identifiers":3773},{"doi":1079},{"id":23,"text":1082,"url":23,"identifiers":3775},{"doi":1082},{"id":23,"text":957,"url":23,"identifiers":3777},{"doi":957},{"id":23,"text":1070,"url":23,"identifiers":3779},{"doi":1070},{"id":23,"text":1067,"url":23,"identifiers":3781},{},{"id":23,"text":975,"url":23,"identifiers":3783},{"doi":975},{"id":23,"text":1085,"url":23,"identifiers":3785},{"doi":1085},{"id":23,"text":3787,"url":23,"identifiers":3788},"10.1111\u002Fj.1538-7836.2004.0562e.x",{"doi":3787},{"id":23,"text":3790,"url":23,"identifiers":3791},"10.1182\u002Fblood-2004-01-0349",{"doi":3790},{"id":23,"text":3793,"url":23,"identifiers":3794},"10.1097\u002F00001721-199412000-00013",{"doi":3793},{"id":23,"text":3796,"url":23,"identifiers":3797},"10.1111\u002Fj.1365-2141.1988.tb07602.x",{"doi":3796},{"id":23,"text":3799,"url":23,"identifiers":3800},"10.1136\u002Fadc.78.3.257",{"doi":3799},{"id":23,"text":3802,"url":23,"identifiers":3803},"Ruggeri ZM, 1982, Type IIB von Willebrand’s disease: differential clearance of endogenous versus transfused large multimer von Willebrand factor, Blood, 60, 1453, 10.1182\u002Fblood.V60.6.1453.1453",{"doi":3804},"10.1182\u002Fblood.V60.6.1453.1453",{"id":23,"text":3806,"url":23,"identifiers":3807},"10.1002\u002Fajh.2830400105",{"doi":3806},{"id":23,"text":3809,"url":23,"identifiers":3810},"10.1046\u002Fj.1365-2516.1998.0040s3048.x",{"doi":3809},{"id":23,"text":3812,"url":23,"identifiers":3813},"10.1073\u002Fpnas.92.17.7739",{"doi":3812},{"id":23,"text":3815,"url":23,"identifiers":3816},"10.1002\u002F(SICI)1521-2254(199903\u002F04)1:2\u003C144::AID-JGM18>3.0.CO;2-5",{"doi":3815},{"id":23,"text":3818,"url":23,"identifiers":3819},"10.1126\u002Fscience.298.5601.2116",{"doi":3818},{"id":23,"text":3821,"url":23,"identifiers":3822},"10.1089\u002Foli.1.1998.8.531",{"doi":3821},{"id":23,"text":1552,"url":23,"identifiers":3824},{},{"id":23,"text":3826,"url":23,"identifiers":3827},"10.1182\u002Fblood-2003-11-3986",{"doi":3826},{"id":23,"text":3829,"url":23,"identifiers":3830},"Department of Health and Human Services, Clinical Hematology Research Career Development Program [Homepage on the Internet]",{},{"id":23,"text":3832,"url":23,"identifiers":3833},"National Hemophilia Foundation, 2006, The National Hemophilia Foundation Clinical Fellowship Program",{},{"id":23,"text":3835,"url":23,"identifiers":3836},"Woods AI, 2001, Clinical features and laboratory patterns in a cohort of consecutive Argentinian patients with von Willebrand’s disease, Haematologica, 86, 420",{},{"id":23,"text":3838,"url":23,"identifiers":3839},"10.1111\u002Fj.1365-2516.2003.00871.x",{"doi":3838},{"id":23,"text":3841,"url":23,"identifiers":3842},"10.1067\u002Fmoe.2002.121431",{"doi":3841},{"id":23,"text":3844,"url":23,"identifiers":3845},"Marder VJ, 1985, Standard nomenclature for factor VIII and von Willebrand factor: a recommendation by the International Committee on Thrombosis and Haemostasis, Thromb Haemost, 54, 871, 10.1055\u002Fs-0038-1660151",{"doi":3846},"10.1055\u002Fs-0038-1660151",{"id":23,"text":3848,"url":23,"identifiers":3849},"Mazurier C, 2001, Recommended abbreviations for von Willebrand factor and its activities, Thromb Haemost, 86, 712, 10.1055\u002Fs-0037-1616109",{"doi":3850},"10.1055\u002Fs-0037-1616109",{"id":23,"text":3852,"url":23,"identifiers":3853},"10.1016\u002FS0049-3848(02)00007-5",{"doi":3852},{"id":23,"text":3855,"url":23,"identifiers":3856},"10.1007\u002FBF00191794",{"doi":3855},{"id":23,"text":3858,"url":23,"identifiers":3859},"10.1097\u002F00001721-199204000-00005",{"doi":3858},{"id":23,"text":3861,"url":23,"identifiers":3862},"10.1073\u002Fpnas.88.9.3857",{"doi":3861},{"id":23,"text":3864,"url":23,"identifiers":3865},"10.1046\u002Fj.1351-8216.2003.00816.x",{"doi":3864},{"id":23,"text":2026,"url":23,"identifiers":3867},{"doi":2026},{"id":23,"text":1967,"url":23,"identifiers":3869},{"doi":1967},{"id":23,"text":1963,"url":23,"identifiers":3871},{"doi":1965},{"id":23,"text":3873,"url":23,"identifiers":3874},"10.1023\u002FA:1025013213192",{"doi":3873},{"id":3876,"createTime":3877,"updateTime":3877,"relativeEntities":3878,"slug":3879,"properties":3880,"entityType":117,"verifyStatus":118,"verifyTime":3892,"verifyNote":120,"languages":3893,"translateLanguages":23,"viewCount":24,"primaryUrl":3894,"fullTextUrl":23,"authors":3895,"publicationType":180,"publisherRelationship":4078,"citationCount":4132,"citationInfo":4133,"publishDate":4136,"publishYear":4134,"citationAnalyzeStatus":22,"lastCitationAnalyze":23,"indexDatabases":4137,"openAccess":23,"references":4138,"isForceReanalyzing":274},"890fb9ae-2463-4033-86e6-797dca33e45b","2025-02-02T15:31:28.258+00:00",[],"Management-of-von-Willebrand-disease-a-guideline-from-the-UK-Haemophilia-Centre-Doctors-Organization",{"openalex":3881,"mag":3883,"abstract":3885,"title":3887,"pm":3889,"doi":3891},{"VOID":3882},"W2067346959",{"VOID":3884},"2067346959",{"EN":3886},"\u003Cjats:p>\u003Cjats:bold>Summary. \u003C\u002Fjats:bold> von Willebrand disease (VWD) is the commonest inherited bleeding disorder. The aim of therapy for VWD is to correct the two defects of haemostasis in this disorder, impaired primary haemostasis because of defective platelet adhesion and aggregation and impaired coagulation as a result of low levels of factor VIII. The objective of this guideline is to inform individuals making choices about the treatment and management of VWD including the use of therapeutic products. 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This review summarizes the current literature for disorders of fibrinogen, and deficiencies of prothrombin, factor V, FV + VIII, FVII, FX, the combined vitamin K‐dependent factors, FXI and FXIII. Based on both collective clinical experience and the literature, guidelines for management of bleeding complications are suggested with specific advice for surgery, spontaneous bleeding, management of pregnancy and the neonate. 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512",{},{"id":23,"text":5027,"url":23,"identifiers":5028},"10.1182\u002Fblood.V77.9.1942.1942",{"doi":5027},{"id":23,"text":5030,"url":23,"identifiers":5031},"10.1182\u002Fblood.V99.7.2448",{"doi":5030},{"id":23,"text":5033,"url":23,"identifiers":5034},"10.1111\u002Fj.1538-7836.2004.00723.x",{"doi":5033},{"id":23,"text":1629,"url":23,"identifiers":5036},{"doi":1629},{"id":23,"text":1632,"url":23,"identifiers":5038},{"doi":1632},{"id":23,"text":1626,"url":23,"identifiers":5040},{"doi":1626},{"id":23,"text":5042,"url":23,"identifiers":5043},"Rapaport S, 1961, The mode of inheritance of PTA deficiency: evidence for the existence of major PTA deficiency and minor PTA deficiency, Blood, 18, 149, 10.1182\u002Fblood.V18.2.149.149",{"doi":1624},{"id":23,"text":5045,"url":23,"identifiers":5046},"Tavori S, 1990, The effect of combined factor XI deficiency with von Willebrand factor abnormalities on haemorrhagic diathesis, Thromb Haemost, 63, 36, 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F., 1965, The therapy of factor 13 deficiency, Bibl Haematol, 23, 1354",{},{"id":23,"text":5086,"url":23,"identifiers":5087},"10.1542\u002Fpeds.109.2.e32",{"doi":5086},{"id":23,"text":5089,"url":23,"identifiers":5090},"10.1046\u002Fj.1365-2141.1999.01648.x",{"doi":5089},{"id":23,"text":5092,"url":23,"identifiers":5093},"10.1055\u002Fs-2007-999041",{"doi":5092},{"id":23,"text":5095,"url":23,"identifiers":5096},"10.1016\u002F0049-3848(94)90010-8",{"doi":5095},{"id":23,"text":5098,"url":23,"identifiers":5099},"10.1046\u002Fj.1538-7836.2003.00469.x",{"doi":5098},{"id":23,"text":5101,"url":23,"identifiers":5102},"10.1055\u002Fs-0038-1648185",{"doi":5101},{"id":23,"text":5104,"url":23,"identifiers":5105},"Kohler HP, 1998, A common coding polymorphism in the FXIII A‐subunit gene (FXIIIVal34Leu) affects cross‐linking activity, Thromb Haemost, 80, 704",{},{"id":23,"text":5107,"url":23,"identifiers":5108},"10.1016\u002F0268-960X(93)90010-2",{"doi":5107},{"id":23,"text":996,"url":23,"identifiers":5110},{"doi":996},{"id":23,"text":2086,"url":23,"identifiers":5112},{"doi":2086},{"id":23,"text":1109,"url":23,"identifiers":5114},{"doi":1109},{"id":23,"text":5116,"url":23,"identifiers":5117},"Lorand L, 1980, Human factor XIII: fibrin‐stabilizing factor, Prog Hemost Thromb, 5, 245",{},{"id":23,"text":5119,"url":23,"identifiers":5120},"Parapia LA, 1988, Proceedings of the XVIII International Congress of the World Federation of Haemophilia, 115",{},{"id":23,"text":5122,"url":23,"identifiers":5123},"Danlos H., 1907, Un cas de cutis laxa et avec tumeurs par contusion chronique des coudes et des genoux (xanthome juvenile pseudodiabetique de MM Hallopeau et Mace de Lapinay), Bull Soc Derm Syph, 19, 70",{},{"id":23,"text":5125,"url":23,"identifiers":5126},"10.1111\u002Fj.1365-2133.1936.tb10006.x",{"doi":5125},{"id":23,"text":5128,"url":23,"identifiers":5129},"10.1002\u002F(SICI)1096-8628(19980428)77:1\u003C31::AID-AJMG8>3.0.CO;2-O",{"doi":5128},{"id":23,"text":5131,"url":23,"identifiers":5132},"10.1172\u002FJCI12881",{"doi":5131},{"id":23,"text":5134,"url":23,"identifiers":5135},"10.1097\u002F00019605-199504000-00006",{"doi":5134},{"id":23,"text":5137,"url":23,"identifiers":5138},"10.1111\u002Fj.1365-2133.1991.tb06063.x",{"doi":5137},{"id":23,"text":5140,"url":23,"identifiers":5141},"Steinmann B., 1993, Connective Tissue and its Heritable Disorders, 351",{},{"id":23,"text":5143,"url":23,"identifiers":5144},"Beighton P., 1993, McKusick's Heritable Disorders of Connective Tissue, 189",{},{"id":23,"text":5146,"url":23,"identifiers":5147},"SchaeferGB.Ehler‐Danlos syndrome. In:http:\u002F\u002Fwww.eMedicine.com\u002Fped\u002Ftopic654.htm;26 January 2003.",{},{"id":23,"text":5149,"url":23,"identifiers":5150},"10.1055\u002Fs-2007-999756",{"doi":5149},{"id":23,"text":5152,"url":23,"identifiers":5153},"10.1097\u002F00001721-200110000-00055",{"doi":5152},{"id":23,"text":5155,"url":23,"identifiers":5156},"10.1097\u002F00043426-199703000-00012",{"doi":5155},{"id":23,"text":5158,"url":23,"identifiers":5159},"Rochelson B, 1991, The use of prophylactic desmopressin (DDAVP) in labor to prevent hemorrhage in a patient with Ehlers‐Danlos syndrome, N Y State J Med, 91, 268",{},{"id":5161,"createTime":5162,"updateTime":5162,"relativeEntities":5163,"slug":5164,"properties":5165,"entityType":117,"verifyStatus":118,"verifyTime":5162,"verifyNote":120,"languages":5178,"translateLanguages":23,"viewCount":24,"primaryUrl":5179,"fullTextUrl":23,"authors":5180,"publicationType":180,"publisherRelationship":5242,"citationCount":5295,"citationInfo":5296,"publishDate":5299,"publishYear":5297,"citationAnalyzeStatus":22,"lastCitationAnalyze":23,"indexDatabases":5300,"openAccess":23,"references":5301,"isForceReanalyzing":274},"3feda757-1558-4d60-a93c-7f696d8c0f2f","2025-02-02T15:31:15.111+00:00",[],"The-prevalence-of-disorders-of-haemostasis-in-adolescents-with-menorrhagia-referred-to-a-haemophilia-treatment-centre",{"openalex":5166,"mag":5168,"abstract":5170,"title":5172,"pm":5174,"doi":5176},{"VOID":5167},"W2067614643",{"VOID":5169},"2067614643",{"EN":5171},"\u003Cjats:p>\u003Cjats:bold>Summary. \u003C\u002Fjats:bold> Menorrhagia at the time of menarche is relatively common and historically attributed primarily to immaturity of the pituitary–ovarian–uterine axis. Intuitively, a proportion of these patients should have an underlying disorder of haemostasis, given the 5–20% prevalence of von Willebrand’s disease and the ≥20% prevalence of platelet dysfunction in light of recent epidemiological studies in menorrhagia, although the average age of the patients in those studies has been ∼35 years. However, there are a few comprehensive studies in the adolescent population determining whether widespread haemostasis evaluation should be carried out in adolescents presenting with menorrhagia. A retrospective chart review study of disorders of haemostasis was carried out in 61 consecutive adolescent patients, ages 11–19 at the time of evaluation referred to the Hemophilia Treatment Center (HTC)\u002FHematology unit. The mean and median ages were 15 ± 2.2 and 14 years (11, 19), respectively. Standard evaluation included complete blood count, prothrombin time, partial thromboplastin time, von Willebrand factor (VWF) levels and platelet aggregation. The proportion of patients with VWF deficiency was 22\u002F61 (36%) [95% confidence interval (CI), 24–49%]; the proportion of patients with platelet aggregation abnormalities was 4\u002F61 (7%) (95% CI, 2–16%). There was no difference in the frequency of additional muco‐cutaneous bleeding symptoms. A relatively high proportion of adolescents are identified with an underlying disorder of haemostasis when referred to an HTC for evaluation of menorrhagia. This involves in part a selective referral bias, but underscores the role of the HTC in evaluating adolescents referred with menorrhagia for an underlying bleeding disorder, given the relatively high yield of haemostatic disorders detected in this setting.\u003C\u002Fjats:p>",{"EN":5173},"The prevalence of disorders of haemostasis in adolescents with menorrhagia referred to a haemophilia treatment centre",{"VOID":5175},"17880454",{"VOID":5177},"10.1111\u002Fj.1365-2516.2007.01496.x",[122],"https:\u002F\u002Fonlinelibrary.wiley.com\u002Fdoi\u002F10.1111\u002Fj.1365-2516.2007.01496.x",[5181,5200,5217],{"id":5182,"sortIndex":24,"researcher":23,"roles":5183,"affiliations":5184,"properties":5193,"displayName":5197,"givenName":23,"familyName":23},"67c5ab05-08ca-49b1-904a-55142cd50654",[],[5185],{"id":5186,"sortIndex":24,"affiliation":5187,"properties":23},"d6ab6ac5-5022-431d-9279-d0cea442d0a7",{"id":5186,"createTime":23,"updateTime":23,"relativeEntities":5188,"slug":23,"properties":5189,"entityType":23,"verifyStatus":23,"verifyTime":23,"verifyNote":23,"languages":23,"translateLanguages":23,"viewCount":23,"url":23,"parentIds":5192,"statistic":23},[],{"title":5190},{"EN":5191},"Rochester General Hospital Department of Medicine",[],{"orcid":5194,"title":5196,"openalex":5198},{"VOID":5195},"https:\u002F\u002Forcid.org\u002F0000-0003-3408-871X",{"EN":5197},"Sameh Mikhail",{"VOID":5199},"A5010739819",{"id":5201,"sortIndex":146,"researcher":23,"roles":5202,"affiliations":5203,"properties":5210,"displayName":5214,"givenName":23,"familyName":23},"36c784fe-f6cb-4536-b580-8fa3d90fbda1",[],[5204],{"id":5186,"sortIndex":24,"affiliation":5205,"properties":23},{"id":5186,"createTime":23,"updateTime":23,"relativeEntities":5206,"slug":23,"properties":5207,"entityType":23,"verifyStatus":23,"verifyTime":23,"verifyNote":23,"languages":23,"translateLanguages":23,"viewCount":23,"url":23,"parentIds":5209,"statistic":23},[],{"title":5208},{"EN":5191},[],{"orcid":5211,"title":5213,"openalex":5215},{"VOID":5212},"https:\u002F\u002Forcid.org\u002F0000-0002-4042-5869",{"EN":5214},"Ramya Varadarajan",{"VOID":5216},"A5030590594",{"id":5218,"sortIndex":164,"researcher":23,"roles":5219,"affiliations":5220,"properties":5235,"displayName":5239,"givenName":23,"familyName":23},"e3231d2c-60bf-4bf4-bc57-221c129ca3f4",[],[5221,5229],{"id":5222,"sortIndex":24,"affiliation":5223,"properties":23},"e3fd5fe7-ec3b-4195-8540-37288a40817c",{"id":5222,"createTime":23,"updateTime":23,"relativeEntities":5224,"slug":23,"properties":5225,"entityType":23,"verifyStatus":23,"verifyTime":23,"verifyNote":23,"languages":23,"translateLanguages":23,"viewCount":23,"url":23,"parentIds":5228,"statistic":23},[],{"title":5226},{"EN":5227},"Mary M. Gooley Hemophilia Center of Rochester, Rochester General Hospital, Rochester, NY, USA",[],{"id":5186,"sortIndex":146,"affiliation":5230,"properties":23},{"id":5186,"createTime":23,"updateTime":23,"relativeEntities":5231,"slug":23,"properties":5232,"entityType":23,"verifyStatus":23,"verifyTime":23,"verifyNote":23,"languages":23,"translateLanguages":23,"viewCount":23,"url":23,"parentIds":5234,"statistic":23},[],{"title":5233},{"EN":5191},[],{"orcid":5236,"title":5238,"openalex":5240},{"VOID":5237},"https:\u002F\u002Forcid.org\u002F0000-0002-3857-8313",{"EN":5239},"Peter A. 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Willebrand EA, 1926, Hereditar pseudohemofili, Finska Lakarsallskapets Handl, 67, 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Evidence‐based guidelines are presented on the selection and use of therapeutic products to treat haemophilia and other hereditary bleeding disorders. 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