{ "uid": "1006", "generated_at": "2026-07-07T17:36:30.719863+00:00", "n_refs": 21, "n_in_catalog": 15, "n_ersetzt": 4, "n_keine": 2, "refs": [ { "marker": 1, "alt_quelle": "Srivastava A et al. Haemophilia 2020; 26 (Suppl. 6):1-158", "status": "in_catalog", "url_status": null, "satz": "", "belege": [ { "source_id": "haemophilia-2020-srivastava-wfh-guidelines-for-the-managemen", "rolle": "original", "tier": "leitlinie", "confidence": "", "vollzitat": "Srivastava A, Santagostino E, Dougall A, Kitchen S, Sutherland M, Pipe SW, et al. WFH guidelines for the management of hemophilia, 3rd edition. Haemophilia. 2020. doi:10.1111/hae.14046", "url": "https://onlinelibrary.wiley.com/doi/10.1111/hae.14046", "passagen": [] } ] }, { "marker": 2, "alt_quelle": "Zanon E et al. J Clin Med. 2022; 11(7):1969", "status": "in_catalog", "url_status": null, "satz": "", "belege": [ { "source_id": "jcm-11-01969", "rolle": "original", "tier": "peer-review", "confidence": "", "vollzitat": "Zanon E, Pasca S, Demartis F, Tagliaferri A, Santoro C, Cantori I, et al. Intracranial haemorrhage in haemophilia patients is still an open issue: the final results of the Italian EMO.REC registry. J Clin Med. 2022;11(7):1969.", "url": "", "passagen": [] } ] }, { "marker": 3, "alt_quelle": "den Uijl IEM et al. Haemophilia 2011; 17:849–853", "status": "in_catalog", "url_status": null, "satz": "", "belege": [ { "source_id": "haemophilia-2011-den-uijl-clinical-severity-of-haemophilia-a", "rolle": "original", "tier": "peer-review", "confidence": "", "vollzitat": "den Uijl IEM, Mauser Bunschoten EP, Roosendaal G, Schutgens REG, Biesma DH, Grobbee DE, et al. Clinical severity of haemophilia A: does the classification of the 1950s still stand? Haemophilia. 2011;17(6):849–53.", "url": "https://pubmed.ncbi.nlm.nih.gov/21545376/", "passagen": [] } ] }, { "marker": 4, "alt_quelle": "Måseide RJ et al. Haemophilia 2021; 27 (5):793–801", "status": "in_catalog", "url_status": null, "satz": "", "belege": [ { "source_id": "haemophilia-2021-maseide-bleeding-phenotype-of-patients-with", "rolle": "original", "tier": "peer-review", "confidence": "", "vollzitat": "Måseide RJ, Berntorp E, Nummi V, Lassila R, Tjønnfjord GE, Holme PA. Bleeding phenotype of patients with moderate haemophilia A and B assessed by thromboelastometry and thrombin generation. Haemophilia. 2021;27:793–801.", "url": "", "passagen": [] } ] }, { "marker": 5, "alt_quelle": "Raffini L et al. British Journal of Haematology 2007; 136:777–787", "status": "in_catalog", "url_status": null, "satz": "", "belege": [ { "source_id": "br-j-haematol-2007-leslie-modern-management-of-haemophilic-a", "rolle": "original", "tier": "peer-review", "confidence": "", "vollzitat": "Raffini L, Manno C. Modern management of haemophilic arthropathy. Br J Haematol. 2007;136(6):777-87.", "url": "https://pubmed.ncbi.nlm.nih.gov/17341263/", "passagen": [] } ] }, { "marker": 6, "alt_quelle": "Modifiziert nach den Uijl IEM *et al.* Haemophilia 2011; 17:849–853", "status": "ersetzt", "url_status": null, "satz": "Davon treten ca. 80 Prozent in den Sprung-, Knie- und Ellenbogengelenken auf.[6]", "belege": [ { "source_id": "piis1538783622114637", "rolle": "ersatz", "tier": "peer-review", "confidence": "medium", "vollzitat": "Valentino LA. Blood-induced joint disease: the pathophysiology of hemophilic arthropathy. J Thromb Haemost. 2010;8(9):1895-902.", "url": "https://www.jthjournal.org/action/showPdf?pii=S1538-7836%2822%2911463-7", "passagen": [ { "quote": "Eighty per cent of joint bleeding episodes involve the knees, elbows, and ankles [2].", "lang": "en", "note": "Das Zitat belegt direkt, dass 80% der Gelenkblutungen Knie, Ellenbogen und Sprunggelenke betreffen." } ] }, { "source_id": "hematolrep-15-00014", "rolle": "ersatz", "tier": "peer-review", "confidence": "medium", "vollzitat": "Sarmiento Doncel S, Díaz Mosquera GA, Cortes JM, Agudelo Rico C, Meza Cadavid FJ, Peláez RG. Haemophilia A: a review of clinical manifestations, treatment, mutations, and the development of inhibitors. Hematol Rep. 2023;15(1):130-150.", "url": "", "passagen": [ { "quote": "Hemarthroses (70–80% incidence): the most common events occur in the knee, ankle, and elbow joints, and less frequently in the shoulders, wrists, and hips.", "lang": "en", "note": "Das Zitat belegt, dass Hämarthrosen mit 70–80% Inzidenz am häufigsten in Knie-, Sprung- und Ellenbogengelenken auftreten, was der Aussage über ~80% in diesen Gelenken entspricht." } ] } ] }, { "marker": 7, "alt_quelle": "den Uijl IEM et al. Blood Transfus 2014;12(Suppl 1):S330–S336;", "status": "ersetzt", "url_status": null, "satz": "Laut Studien erhalten nur 29 bis 69 Prozent der Menschen mit einer leichten oder mittelschweren Hämophilie eine Prophylaxe.[7][8][9] Findet keine prophylaktische Behandlung statt, kann es zu einer Verschlechterung der krankheitsbedingten Einschränkungen kommen.", "belege": [ { "source_id": "bloodtransfus2014-vol12-suppl-1-s330-s336-2012-0091-12", "rolle": "ersatz", "tier": "peer-review", "confidence": "medium", "vollzitat": "den Uijl IEM, Biesma D, Grobbee D, Fischer K. Outcome in moderate haemophilia. Blood Transfus. 2014;12(Suppl 1):S330-S336.", "url": "https://www.bloodtransfusion.it/public/pre2018archives/2014/BloodTransfus2014_Vol12_Suppl_1_s330-s336_2012.0091-12.pdf", "passagen": [ { "quote": "Twenty-two (29%) patients with moderate haemophilia were treated with prophylaxis at some time in their life.", "lang": "en", "note": "Das Zitat belegt, dass 29% der Patienten mit mittelschwerer Hämophilie eine Prophylaxe erhielten, was dem genannten unteren Bereich von 29% entspricht." } ] } ] }, { "marker": 8, "alt_quelle": "ATHN dataset report 2020", "status": "ersetzt", "url_status": null, "satz": "Laut Studien erhalten nur 29 bis 69 Prozent der Menschen mit einer leichten oder mittelschweren Hämophilie eine Prophylaxe.[7][8][9] Findet keine prophylaktische Behandlung statt, kann es zu einer Verschlechterung der krankheitsbedingten Einschränkungen kommen.", "belege": [ { "source_id": "bloodtransfus2014-vol12-suppl-1-s330-s336-2012-0091-12", "rolle": "ersatz", "tier": "peer-review", "confidence": "medium", "vollzitat": "den Uijl IEM, Biesma D, Grobbee D, Fischer K. Outcome in moderate haemophilia. Blood Transfus. 2014;12(Suppl 1):S330-S336.", "url": "https://www.bloodtransfusion.it/public/pre2018archives/2014/BloodTransfus2014_Vol12_Suppl_1_s330-s336_2012.0091-12.pdf", "passagen": [ { "quote": "Twenty-two (29%) patients with moderate haemophilia were treated with prophylaxis at some time in their life.", "lang": "en", "note": "Das Zitat belegt, dass 29% der Patienten mit mittelschwerer Hämophilie eine Prophylaxe erhielten, was dem genannten unteren Bereich von 29% entspricht." } ] } ] }, { "marker": 9, "alt_quelle": "Scott MJ et al. Haemophilia 2019;25:205–212", "status": "in_catalog", "url_status": null, "satz": "", "belege": [ { "source_id": "haemophilia-2018-scott-treatment-regimens-and-outcomes-in-se", "rolle": "original", "tier": "peer-review", "confidence": "", "vollzitat": "Scott MJ, Xiang H, Hart DP, Palmer B, Collins PW, Stephensen D, et al. Treatment regimens and outcomes in severe and moderate haemophilia A in the UK: The THUNDER study. Haemophilia. 2019;25(2):205-12.", "url": "https://pubmed.ncbi.nlm.nih.gov/30408835/", "passagen": [] } ] }, { "marker": 10, "alt_quelle": "Walsh C et al. Haemophilia. 2021; 27(S1):25–32", "status": "in_catalog", "url_status": null, "satz": "", "belege": [ { "source_id": "haemophilia-2021-walsh-identified-unmet-needs-and-proposed-s", "rolle": "original", "tier": "peer-review", "confidence": "", "vollzitat": "Walsh C, Boggio L, Brown-Jones L, Miller R, Hawk S, Savage B, et al. Identified unmet needs and proposed solutions in mild-to-moderate haemophilia: a summary of opinions from a roundtable of haemophilia experts. Haemophilia. 2021;27(S1):25-32.", "url": "", "passagen": [] } ] }, { "marker": 11, "alt_quelle": "Collins PW et al. Haemophilia, 2021; 27:192–198", "status": "in_catalog", "url_status": null, "satz": "", "belege": [ { "source_id": "haemophilia-2021-collins-clinical-phenotype-of-severe-and-mo", "rolle": "original", "tier": "peer-review", "confidence": "", "vollzitat": "Collins PW, Obaji SG, Roberts H, Gorsani D, Rayment R. Clinical phenotype of severe and moderate haemophilia: who should receive prophylaxis and what is the target trough level? Haemophilia. 2021;27(2):192-198. doi:10.1111/hae.14219", "url": "", "passagen": [] } ] }, { "marker": 12, "alt_quelle": "Nissen F et al. ISTH Virtual Congress; 2020; Abstract OC 09.3", "status": "keine", "url_status": null, "satz": "Ca. 85 Prozent der Betroffenen mit mittelschwerer Hämophilie haben im Laufe ihres Lebens Gelenkblutungen, die zu Gelenkproblemen und somit zu einer Beeinträchtigung der Lebensqualität führen können.[10][11][12]", "belege": [] }, { "marker": 13, "alt_quelle": "Manco-Johnson MJ et al. J Thromb Haemost 2017; 15: 2115–2124", "status": "in_catalog", "url_status": null, "satz": "", "belege": [ { "source_id": "piis1538783622043860", "rolle": "original", "tier": "peer-review", "confidence": "", "vollzitat": "Manco-Johnson MJ, Lundin B, Funk S, Peterfy C, Raunig D, Werk M, et al. Effect of late prophylaxis in hemophilia on joint status: a randomized trial. J Thromb Haemost. 2017;15:2115-24.", "url": "", "passagen": [] } ] }, { "marker": 14, "alt_quelle": "https://www.bundesaerztekammer.de/fileadmin/user_upload/_old-files/downloads/pdf-Ordner/MuE/Querschnitts-Leitlinien_BAEK_zur_Therapie_mit_Blutkomponenten_und_Plasmaderivaten-Gesamtnovelle_2020.pdf", "status": "in_catalog", "url_status": null, "satz": "", "belege": [ { "source_id": "leitlinienreport-qll2020", "rolle": "original", "tier": "leitlinie", "confidence": "", "vollzitat": "Bundesärztekammer. Leitlinienreport der Querschnitts-Leitlinien zur Therapie mit Blutkomponenten und Plasmaderivaten, Gesamtnovelle 2020. Berlin: Bundesärztekammer; 2020.", "url": "https://www.bundesaerztekammer.de/fileadmin/user_upload/_old-files/downloads/pdf-Ordner/MuE/Querschnitts-Leitlinien_BAEK_zur_Therapie_mit_Blutkomponenten_und_Plasmaderivaten-Gesamtnovelle_2020.pdf", "passagen": [] } ] }, { "marker": 15, "alt_quelle": "Soucie JM et al. Blood advances 2018; 16:2136–2144", "status": "in_catalog", "url_status": null, "satz": "", "belege": [ { "source_id": "advances020552", "rolle": "original", "tier": "peer-review", "confidence": "", "vollzitat": "Soucie JM, Monahan PE, Kulkarni R, Konkle BA, Mazepa MA; for the US Hemophilia Treatment Center Network. The frequency of joint hemorrhages and procedures in nonsevere hemophilia A vs B. Blood Adv. 2018;2(16):2136-2144.", "url": "https://ashpublications.org/bloodadvances/article/2/16/2136/15919/The-frequency-of-joint-hemorrhages-and-procedures", "passagen": [] } ] }, { "marker": 16, "alt_quelle": "den Uijl IEM et al. Haemophilia 2011; 17:849–853", "status": "in_catalog", "url_status": null, "satz": "", "belege": [ { "source_id": "haemophilia-2011-den-uijl-clinical-severity-of-haemophilia-a", "rolle": "original", "tier": "peer-review", "confidence": "", "vollzitat": "den Uijl IEM, Mauser Bunschoten EP, Roosendaal G, Schutgens REG, Biesma DH, Grobbee DE, et al. Clinical severity of haemophilia A: does the classification of the 1950s still stand? Haemophilia. 2011;17(6):849–53.", "url": "https://pubmed.ncbi.nlm.nih.gov/21545376/", "passagen": [] } ] }, { "marker": 17, "alt_quelle": "Olivieri M et al. Haemophilia 2012; 18:369–374", "status": "in_catalog", "url_status": null, "satz": "", "belege": [ { "source_id": "haemophilia-2011-olivieri-identification-and-longterm-observ", "rolle": "original", "tier": "peer-review", "confidence": "", "vollzitat": "Olivieri M, Kurnik K, Pfluger T, Bidlingmaier C. Identification and long-term observation of early joint damage by magnetic resonance imaging in clinically asymptomatic joints in patients with haemophilia A or B despite prophylaxis. Haemophilia. 2012;18(3):369–374.", "url": "https://pubmed.ncbi.nlm.nih.gov/22032268/", "passagen": [] } ] }, { "marker": 18, "alt_quelle": "Valentino LA. Thromb Haemost 2010; 8:1895–1902", "status": "in_catalog", "url_status": null, "satz": "", "belege": [ { "source_id": "piis1538783622114637", "rolle": "original", "tier": "peer-review", "confidence": "", "vollzitat": "Valentino LA. Blood-induced joint disease: the pathophysiology of hemophilic arthropathy. J Thromb Haemost. 2010;8(9):1895-902.", "url": "https://www.jthjournal.org/action/showPdf?pii=S1538-7836%2822%2911463-7", "passagen": [] } ] }, { "marker": 19, "alt_quelle": "Oldenburg J. Blood 2015; 13:2038–2044", "status": "in_catalog", "url_status": null, "satz": "", "belege": [ { "source_id": "2038", "rolle": "original", "tier": "peer-review", "confidence": "", "vollzitat": "Oldenburg J. Optimal treatment strategies for hemophilia: achievements and limitations of current prophylactic regimens. Blood. 2015;125(13):2038-44.", "url": "https://ashpublications.org/blood/article/125/13/2038/33972/Optimal-treatment-strategies-for-hemophilia", "passagen": [] } ] }, { "marker": 20, "alt_quelle": "Noone D et al. 3449; ASH 2020", "status": "ersetzt", "url_status": null, "satz": "Aufgrund der momentan nicht optimalen Therapiesituation vieler Patienten mit leichter und mittelschwerer Hämophilie, ist auch die Lebensqualität der Betroffenen eingeschränkt.[20]", "belege": [ { "source_id": "haemophilia-2021-walsh-identified-unmet-needs-and-proposed-s", "rolle": "ersatz", "tier": "peer-review", "confidence": "high", "vollzitat": "Walsh C, Boggio L, Brown-Jones L, Miller R, Hawk S, Savage B, et al. Identified unmet needs and proposed solutions in mild-to-moderate haemophilia: a summary of opinions from a roundtable of haemophilia experts. Haemophilia. 2021;27(S1):25-32.", "url": "", "passagen": [ { "quote": "patients with haemophilia are often limited in their activities of daily living.10,15 Pain and limited mobility can impair the ability of patients with mild-to-moderate haemophilia to complete tasks in an efficient manner and require the need for assistance with these activities. This could affect a patient's self-esteem and ultimately lead to depression.", "lang": "en", "note": "Das Zitat belegt, dass Patienten mit leichter und mittelschwerer Hämophilie in ihren Alltagsaktivitäten eingeschränkt sind, Schmerzen und eingeschränkte Mobilität haben und dies zu Selbstwertproblemen und Depression führen kann – was eine eingeschränkte Lebensqualität beschreibt." } ] } ] }, { "marker": 21, "alt_quelle": "Kratzer V et al. T06-P36; GTH 2022", "status": "keine", "url_status": null, "satz": "Die Ergebnisse liegen erstaunlich nah beieinander und lassen vermuten, dass die Auswirkungen der leichten Hämophilie auf den Alltag und die Gesundheit der Betroffenen oft unterschätzt wird.[21]", "belege": [] } ] }