Haemophilia最新文献

{"title":"Effects of resistance training on muscle strength in adults with haemophilia: A systematic review and meta-analysis","authors":"Rodrigo Núñez-Cortés,&nbsp;Sofía Pérez-Alenda,&nbsp;Joaquín Calatayud,&nbsp;Verónica Soto,&nbsp;Ronei S. Pinto,&nbsp;Lars Louis Andersen,&nbsp;Carlos Cruz-Montecinos","doi":"10.1111/hae.15067","DOIUrl":"10.1111/hae.15067","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Introduction</h3>\u0000 \u0000 <p>Although resistance training is frequently prescribed for people with haemophilia (PWH), no previous meta-analyses have quantified the effect of this intervention on muscle strength, nor the implications of the intervention's modality and duration.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Aim</h3>\u0000 \u0000 <p>(1) To determine the effects of resistance training on muscle strength in adults with haemophilia; (2) To determine the most effective duration and modality among the exercise protocols.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>A systematic search from inception until 28 November 2023 was conducted in PubMed, Embase, Web of Science, CENTRAL and CINAHL databases. We included randomised controlled trials or before-after studies that involved resistance training without other physiotherapy co-interventions. Study selection, data extraction and risk of bias assessment were independently performed by two reviewers. Disagreements were resolved in consultation with a third author. The level of evidence was determined according to the GRADE methodology.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Seven studies were included. Measurements of knee extensor strength and elbow extensor strength were included in the meta-analysis. Subgroup analysis showed significant effects for both elastic resistance protocols (SMD: 0.54; 95% CI: 0.02–1.07) and conventional training (isometric and weight-based equipment) (SMD: 0.88; 95% CI: 0.50–1.25), demonstrating small and moderate effect sizes respectively. Additionally, both protocols of duration 5–7 weeks (SMD: 1.16, 95% CI: 0.63–1.69) as well as those of duration ≥8 weeks (SMD: 0.57, 95% CI: 0.20–0.94) showed a significant difference.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>Resistance training is effective in improving muscle strength of the knee and elbow extensors in PWH. Both elastic resistance and conventional training show benefits.</p>\u0000 </section>\u0000 </div>","PeriodicalId":12819,"journal":{"name":"Haemophilia","volume":"30 4","pages":"894-904"},"PeriodicalIF":3.0,"publicationDate":"2024-06-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141283524","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Functional clinical motor performance tests to assess potential fall risks in patients with haemophilia: A case-control study","authors":"Fabian Tomschi,&nbsp;Marius Brühl,&nbsp;Alexander Schmidt,&nbsp;Pia Ransmann,&nbsp;Andreas C. Strauss,&nbsp;Thomas Hilberg","doi":"10.1111/hae.15060","DOIUrl":"10.1111/hae.15060","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Introduction</h3>\u0000 \u0000 <p>Patients with haemophilia (PwH) are at increased risk of falls due to haemophilic arthropathy. Yet, studies on clinical tests associated with the risk of falling are scarce in PwH.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Aims</h3>\u0000 \u0000 <p>(1) To evaluate the feasibility of different clinical motor performance tests associated with the risk of falling in PwH; (2) to evaluate PwH's performance of these tests compared to a control group; (3) to identify possible influencing factors that affect performance.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Twenty-nine severe and moderate PwH (57.0 years, IQR: 48.0–61.5) and 29 healthy age- and BMI-matched control participants (CG) performed 13 different clinical tests (SPPB, timed up and go, push and release, functional reach, single-leg stance, knee and grip strength). Haemophilia joint health score (HJHS), kinesiophobia (TSK-11), subjective physical performance (HEP-Test-Q), falls efficiency (FES-I) and falls were assessed.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>No adverse events occurred. PwH showed impaired performance in all clinical tests, a lower falls efficiency and a higher HJHS than CG. PwH with higher HJHS, lower HEP-Test-Q and higher TSK-11 scores showed higher deficits. Largest discrepancies were observed in the single-leg stance with eyes open and knee extensor strength, where orthopaedically majorly affected PwH showed worse performance compared to minorly affected PwH and the CG, respectively. The prevalence of ≥1 fall in the last year was 27.6% (PwH) and 10.3% (CG).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>These clinical tests are feasible in PwH. Impaired joint status, a high kinesiophobia and low physical performance impair performance. These tests can be used by clinicians for gaining specific information on functional motor abilities of patients.</p>\u0000 </section>\u0000 </div>","PeriodicalId":12819,"journal":{"name":"Haemophilia","volume":"30 4","pages":"1032-1042"},"PeriodicalIF":3.0,"publicationDate":"2024-06-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/hae.15060","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141261649","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Humanistic burden of haemophilia A without inhibitors: A cross-sectional analysis of the HemoLIFE study","authors":"María Teresa Álvarez-Román,&nbsp;Ramiro Jose Nuñez Vazquez,&nbsp;Olga Benitez Hidalgo,&nbsp;Laura Quintana Paris,&nbsp;Laura Entrena Ureña,&nbsp;Francisco Jose Lopez Jaime,&nbsp;Hortensia la De Corte-Rodríguez,&nbsp;María García Dasí,&nbsp;Pau Bosch,&nbsp;María Eva Mingot Castellano,&nbsp;Itziar Guerra Garaeta,&nbsp;Inmaculada Soto-Ortega","doi":"10.1111/hae.15057","DOIUrl":"10.1111/hae.15057","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Aim</h3>\u0000 \u0000 <p>To evaluate the impact of haemophilia A without inhibitors on humanistic outcomes in patients and caregivers. Herein, we report a cross-sectional analysis of the baseline data of persons with haemophilia (PWH) participating in the prospective study HEMOLIFE.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>These data are part of a prospective, observational, and multicentre study currently being conducted in 20 hospitals in Spain by haematologists. We included subjects 12 years or older diagnosed with haemophilia. The evaluations included the Maladjustment Scale, Haemophilia–Specific Quality of Life Questionnaire for Adults (HaemoQol)/HaemoQol Short Form (Children), haemophilia-specific version of the Work Productivity and Impairment Questionnaire plus the Classroom Impairment Questionnaire (WPAI+CIQ:HS), Haemophilia Activity List (HAL)/Paediatric Haemophilia Activities List (pedHAL), visual analogue scale (VAS) for evaluating pain, Coping Pain Questionnaire–Reduced (CAD-R), and Hospital Anxiety and Depression Scale (HADS).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>A total of 81 PWH were recruited at 18 centres; 66 PWH were ≥18 years (i.e., adults), and PWH 15 were &lt;18 years (i.e., paediatric patients). Out of the 79 evaluable subjects, 16 (20%) showed an impact of haemophilia on daily life, and the areas most affected were “leisure time” (58% showed maladjustment) and “work/studies” (47% showed maladjustment). Patients reported a higher impact of haemophilia on quality of life (mean [SD] of the transformed score) in the dimensions of “sport” (49.4 [28.6]), “physical health” (40.5 [25.8]) and “future” (37.7 [28.9]). In adults, according to HAL scores, greater impairment of function was observed in “lying/sitting/kneeling/standing,” “function of legs” and “leisure activities and sports,” with mean normalized scores of 64.7, 65.1 and 69.0, respectively. Productivity was mostly impacted by presenteeism. The pain was infrequent and moderate. According to the HADS scores, nine (11.5%) patients had clinical anxiety and depression.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>PWH without inhibitors exhibited impairments in adjustment, quality of life and functionality, especially related to leisure and sports activities, and exhibit relevant levels of anxiety and depression.</p>\u0000 </section>\u0000 </div>","PeriodicalId":12819,"journal":{"name":"Haemophilia","volume":"30 4","pages":"950-958"},"PeriodicalIF":3.0,"publicationDate":"2024-06-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/hae.15057","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141199721","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Self-conducted sonographic monitoring of the knee in patients with haemophilia—A feasibility study","authors":"Anna Christina Strauss,&nbsp;Natascha Marquardt,&nbsp;Johannes Oldenburg,&nbsp;Claus Christian Pieper,&nbsp;Ulrike Attenberger,&nbsp;Jamil Hmida,&nbsp;Charlotte Rommelspacher,&nbsp;Sebastian Koob,&nbsp;Andreas Christian Strauss","doi":"10.1111/hae.15056","DOIUrl":"10.1111/hae.15056","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Introduction/aim</h3>\u0000 \u0000 <p>To evaluate whether patients with haemophilia (PwH) can be enabled to perform ultrasonography (US) of their knees without supervision according to the Haemophilia Early Arthropathy Detection with Ultrasound (HEAD-US) protocol and whether they would be able to recognize pathologies.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Five PwH (mean age 29.6 years, range 20–48 years) were taught the use of a portable US device and the HEAD-US protocol. Subsequently, the patients performed US unsupervised at home three times a week for a total of 6 weeks with a reteaching after 2 weeks. All images were checked for mapping of the landmarks defined in the HEAD-US protocol by a radiologist. In a final test after the completion of the self-sonography period, participants were asked to identify scanning plane and potential pathology from US images of other PwH.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>On the images of the self-performed scans, 82.7% of the possible anatomic landmarks could be identified and 67.5% of the requested images were unobjectionable, depicting 100% of the required landmarks. There was a highly significant improvement in image quality following reteaching after 2 weeks (74.80 ± 36.88% vs. 88.31 ± 19.87%, <i>p</i> &lt; .001). In the final test, the participants identified the right scanning plane in 85.0% and they correctly identified pathology in 90.0% of images.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>Appropriately trained PwH can perform the HEAD-US protocol of their knee with high quality and are capable to identify pathologic findings on these standardized images. Asynchronous tele-sonography could enable early therapy adjustment and thereby possibly reduce costs.</p>\u0000 </section>\u0000 </div>","PeriodicalId":12819,"journal":{"name":"Haemophilia","volume":"30 4","pages":"1025-1031"},"PeriodicalIF":3.0,"publicationDate":"2024-06-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/hae.15056","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141199724","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Oral surgery in people with inherited bleeding disorder: A retrospective study","authors":"Emma Fribourg,&nbsp;Sabine Castet,&nbsp;Mathilde Fénelon,&nbsp;Yoann Huguenin,&nbsp;Jean-Christophe Fricain,&nbsp;Virginie Chuy,&nbsp;Sylvain Catros","doi":"10.1111/hae.15055","DOIUrl":"10.1111/hae.15055","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Introduction</h3>\u0000 \u0000 <p>The objectives were to describe the peri-operative management of people with inherited bleeding disorders in oral surgery and to investigate the association between type of surgery and risk of developing bleeding complications.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Materials and Methods</h3>\u0000 \u0000 <p>This retrospective observational study included patients with haemophilia A or B, von Willebrand disease, Glanzmann thrombasthenia or isolated coagulation factor deficiency such as afibrinogenemia who underwent osseous (third molar extraction, ortho-surgical traction, dental implant placement) or nonosseous oral surgery between 2014 and 2021 at Bordeaux University Hospital (France). Patients and oral surgery characteristics were retrieved from medical records. Odds ratio (OR) and 95% confidence interval (CI) were estimated using logistic regression.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Of the 83 patients included, general anaesthesia was performed in 16%. Twelve had a bleeding complication (14.5%) including six after osseous surgery. The most serious complication was the appearance of anti-FVIII inhibitor in a patient with moderate haemophilia A. All bleeding complications were managed by a local treatment and factor injections where indicated. No association was observed between type of surgery (osseous vs. nonosseous) and risk of bleeding complications after controlling for sex, age, disease type and severity, multiple extractions, type of anaesthesia and use of fibrin glue (OR: 3.21, 95% CI: .69–14.88).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>In this study, we have observed that bleeding complications after oral surgery in people with inherited bleeding disorders were moderately frequent and easily managed. However, in this study, we observed a serious complication highlighting the necessity of a thorough benefit-risk balance evaluation during the preoperative planning of the surgical and medical protocol.</p>\u0000 </section>\u0000 </div>","PeriodicalId":12819,"journal":{"name":"Haemophilia","volume":"30 4","pages":"943-949"},"PeriodicalIF":3.0,"publicationDate":"2024-06-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/hae.15055","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141199722","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Limited value of testing for factor XIII and α2-antiplasmin deficiency in patients with a bleeding disorder of unknown cause","authors":"Sander Ariëns,&nbsp;Albert Huisman,&nbsp;Idske C. L. Kremer Hovinga,&nbsp;Rolf T. Urbanus,&nbsp;Karin P. M. van Galen,&nbsp;Lize F. D. van Vulpen,&nbsp;Kathelijn Fischer,&nbsp;Roger E. G. Schutgens","doi":"10.1111/hae.15059","DOIUrl":"10.1111/hae.15059","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Introduction</h3>\u0000 \u0000 <p>In patients with an increased bleeding tendency, extensive diagnostic blood testing is often performed. When results of tier 1 assays of primary haemostasis are normal, protocols recommend additional testing to rule out rare disorders including coagulation factor XIII (FXIII) and α2-antiplasmin (α2AP) deficiency.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Aim</h3>\u0000 \u0000 <p>To evaluate the added diagnostic value of FXIII and α2AP levels in patients with a bleeding disorder of unknown cause (BDUC).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>A retrospective monocentre cohort study between August 2011 and August 2023 was conducted. In all patients with bleeding tendencies and normal diagnostic tests for von Willebrand disease and platelet function, FXIII and α2AP were measured.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>We included 158 consecutive patients; mean ISTH-BAT scores were 8.2 (SD ± 3.7) in children, 6.2 (SD ± 2.1) in men and 10.6 (SD ± 3.3) in women. Median age was 37 (range 5–79) years, 88.6% of patients were female. Patients displayed median FXIII activity of 111% (IQR = 97–131) and median α2AP activity of 112% (IQR = 103–119).</p>\u0000 \u0000 <p>Three (1.9%) patients had FXIII levels &lt; 50%, respectively 43%, 45% and 46%. Corresponding ISTH-BAT scores were 7, 12 and 14. No α2AP levels &lt; 60% was observed. No significant association was found between FXIII levels and ISTH-BAT scores.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>In our cohort of BDUC patients, no clinical relevant FXIII deficiencies were detected; absolute values were well above the 30% cutoff considered adequate for normal haemostasis. No α2AP deficiencies were detected. These data suggest that in BDUC patients, measuring FXIII or AP activity is of limited value.</p>\u0000 </section>\u0000 </div>","PeriodicalId":12819,"journal":{"name":"Haemophilia","volume":"30 4","pages":"998-1002"},"PeriodicalIF":3.0,"publicationDate":"2024-05-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/hae.15059","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141174493","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The relevance of MRI findings in joints of persons with haemophilia: Insights from the last decade","authors":"Wouter Foppen,&nbsp;Flora H. P. van Leeuwen,&nbsp;Merel A. Timmer,&nbsp;Kathelijn Fischer","doi":"10.1111/hae.15058","DOIUrl":"10.1111/hae.15058","url":null,"abstract":"&lt;p&gt;Most bleeding episodes in persons with haemophilia (PwH) occur in the large synovial joints (elbows, knees, and ankles). Recurrent joint bleeding eventually leads to irreversible haemophilic arthropathy, which causes pain, reduced functionality, and thus reduced quality of life. Prophylactic treatment prevents most bleeding episodes.&lt;span&gt;&lt;sup&gt;1&lt;/sup&gt;&lt;/span&gt; Even in the absence of clinically overt joint bleeding, long-term progression to arthropathy is observed. Subclinical bleeding and inflammation are therefore thought to contribute to the development of arthropathy.&lt;span&gt;&lt;sup&gt;2-4&lt;/sup&gt;&lt;/span&gt; Detection of these subclinical processes is becoming increasingly important in the prevention of arthropathy in PwH as overt spontaneous joint bleeding is almost completely avoided by prophylaxis with new (non-factor) replacement therapies.&lt;span&gt;&lt;sup&gt;5&lt;/sup&gt;&lt;/span&gt;&lt;/p&gt;&lt;p&gt;Magnetic resonance imaging (MRI) is considered the gold standard for evaluation of early blood-induced joint changes in PwH. In 2005, the International Prophylaxis Study Group (IPSG) published compatible scales for progressive and additive MRI assessment based on the Denver MRI score and the European MRI score.&lt;span&gt;&lt;sup&gt;6&lt;/sup&gt;&lt;/span&gt; These scores were combined in a comprehensive scoring scheme in 2012.&lt;span&gt;&lt;sup&gt;7&lt;/sup&gt;&lt;/span&gt; Based on an appraisal of the original IPSG MRI scale,&lt;span&gt;&lt;sup&gt;8&lt;/sup&gt;&lt;/span&gt; the score is now updated to the &lt;i&gt;IPSG MRI Scale version 2.0&lt;/i&gt; by Lundin and colleagues.&lt;span&gt;&lt;sup&gt;9&lt;/sup&gt;&lt;/span&gt; To provide more insight on the clinical relevance of MRI findings, we provide an overview of research on the clinical relevance of MRI findings evaluated by &lt;i&gt;IPSG MRI Scale version 2.0&lt;/i&gt;.&lt;/p&gt;&lt;p&gt;Since the 1980s, there have been suspicions that chronic microbleeding or subclinical bleeding into the joints can cause arthropathy.&lt;span&gt;&lt;sup&gt;10, 11&lt;/sup&gt;&lt;/span&gt; Manco-Johnson et al. found further evidence for this hypothesis after observing haemosiderin deposits and other joint changes on MRI in joints without any history of prior bleeding in a prospective clinical trial.&lt;span&gt;&lt;sup&gt;2&lt;/sup&gt;&lt;/span&gt; Potentially, prophylaxis may reduce subclinical bleeding and joint deterioration. Worth noting, haemosiderin deposits in joints without a history of bleeds were observed in 26% of Canadian children with severe haemophilia treated with tailored primary prophylaxis,&lt;span&gt;&lt;sup&gt;12&lt;/sup&gt;&lt;/span&gt; in 14% of Dutch adults with non-severe haemophilia (94% treated on demand),&lt;span&gt;&lt;sup&gt;13&lt;/sup&gt;&lt;/span&gt; and in 16% of Dutch adolescents and adults with severe haemophilia on prophylaxis.&lt;span&gt;&lt;sup&gt;14&lt;/sup&gt;&lt;/span&gt; These observations implicate that subclinical bleeding occurs in non-severe haemophilia and is not fully prevented by prophylaxis in severe haemophilia.&lt;/p&gt;&lt;p&gt;The (longer-term) clinical consequences of the observed signs of previous subclinical bleeding are a topic of interest. Haemosiderin accumulates in the synovial membrane and a previous study observed that haemosiderin deposits were","PeriodicalId":12819,"journal":{"name":"Haemophilia","volume":"30 4","pages":"877-879"},"PeriodicalIF":3.0,"publicationDate":"2024-05-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/hae.15058","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141174507","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Association between cognitive impairment and functional limitations in everyday life in patients with haemophilia in Hong Kong","authors":"Yin Ting Cheung,&nbsp;Chung Tin Ma,&nbsp;Deng Weishang,&nbsp;Henry Hon Wai Lam,&nbsp;Siu Cheung Ling,&nbsp;Kevin Kwok,&nbsp;Chak Ho Li,&nbsp;Chung Yin Ha,&nbsp;Sze Fai Yip,&nbsp;Raymond Siu Ming Wong,&nbsp;Winnie Chiu Wing Chu,&nbsp;Chi Kong Li","doi":"10.1111/hae.15035","DOIUrl":"10.1111/hae.15035","url":null,"abstract":"","PeriodicalId":12819,"journal":{"name":"Haemophilia","volume":"30 4","pages":"1084-1088"},"PeriodicalIF":3.0,"publicationDate":"2024-05-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141157940","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Alleviated bleeding phenotypes in a child with severe haemophilia A and thalassemia disease","authors":"Pongpak Pongphitcha,&nbsp;Werasak Sasanakul,&nbsp;Praguywan Kadegasem,&nbsp;Duantida Songdej,&nbsp;Chanin Limwongse,&nbsp;Mattana Amesbutr,&nbsp;Suphaneewan Jaovisidha,&nbsp;Ampaiwan Chuansumrit,&nbsp;Nongnuch Sirachainan","doi":"10.1111/hae.15041","DOIUrl":"10.1111/hae.15041","url":null,"abstract":"","PeriodicalId":12819,"journal":{"name":"Haemophilia","volume":"30 4","pages":"1092-1095"},"PeriodicalIF":3.0,"publicationDate":"2024-05-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141087296","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Inherited coagulation factor VII deficiency in Taiwan: Two novel F7 variants with relevant regional features in 33 patients","authors":"Hsuan-Yu Lin,&nbsp;Ching-Yeh Lin,&nbsp;Su-Feng Kuo,&nbsp;Jen-Shiou Lin,&nbsp;Po-Te Lin,&nbsp;Ying-Chih Huang,&nbsp;Han-Ni Hsieh,&nbsp;Ming-Ching Shen","doi":"10.1111/hae.15054","DOIUrl":"10.1111/hae.15054","url":null,"abstract":"","PeriodicalId":12819,"journal":{"name":"Haemophilia","volume":"30 4","pages":"1096-1101"},"PeriodicalIF":3.0,"publicationDate":"2024-05-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141087298","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}