Background: Current research on osteoporosis (OP) in hemophilia is insufficient. The suitability of high-resolution peripheral quantitative computed tomography (HR-pQCT) for evaluating osteoporosis in hemophilia remains unclear.
Aim: To investigate the current status of osteoporosis and the applicability of HR-pQCT in adult hemophilia patients.
Methods: Thirty three hemophilia patients aged 23-49 years were recruited. X-ray examinations were performed on the bleeding joints. Dual energy X-ray absorptiometry (DXA) and HR-pQCT were used to assess bone mineral density (BMD). The HR-pQCT values of the distal tibia and radius were compared between hemophilia patients and healthy controls(HCs).
Results: All bleeding joints showed local osteoporosis on X-ray. Only 33.3% of patients had a hip BMD lower than the expected value according to DXA. The Tb.vBMD(98.5 ± 44.2 mg/cm), Tt.Ar(612.5 ± 163.5mm),Tb.Ar(487.0 ± 175.6mm), Ct.Ar(117.0 ± 25.7mm), Tb.BV/TV(0.2 ± 0.1), Tb.N(0.9 ± 0.3 1/mm), Ct.Pm(96.3 ± 13.8 mm) of the distal tibia and Tt.Ar(248.4 ± 53.1mm),Tb.Ar(186.0 ± 55.1mm), Ct.Ar(66.1 ± 14.4 mm), Ct.Pm(68.1 ± 7.1 mm) of the distal radius in the hemophilia group was significantly lower than the HCs(tibia Tb.vBMD:186.4 ± 44.3mg/cm, Tt.Ar:906.8 ± 135.0mm,Tb.Ar:743.7 ± 137.6mm, Ct.Ar:169.3 ± 21.9mm,Tb.
Bv/tv: 0.3 ± 0.1, Tb.N:1.5 ± 0.2 1/mm,Ct.Pm:117.8 ± 8.2 mm; radius Tt.Ar:285.7 ± 35.6 mm, Tb.Ar:83.8 ± 7.9mm, Ct.Ar:0.3 ± 0.1mm, Ct.Pm:80.2 ± 4.3 mm) with statistically significant differences (p < 0.05). Correlation analysis showed a positive correlation (r = 0.768, p = 0.016) between femoral neck BMD with DXA and total volumetric BMD(Tt.vBMD) at the distal tibia.
Conclusion: The bone health status of adult hemophilia patients in China is worrying. The occurrence of OP may be accompanied by varying degrees of bone loss, bone destruction, and structural abnormalities observed in both trabecular and cortical bones of the upper and lower limbs. The condition of the trabecular bones in the lower limbs is particularly severe. The correlation between BMD measurements obtained from HR-pQCT and DXA is strong.
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http://dx.doi.org/10.1186/s12891-025-08473-7 | DOI Listing |
Anaesthesiologie
March 2025
Klinik für Anästhesiologie, Intensivmedizin und Schmerztherapie, Universitätsklinikum des Saarlandes und Medizinische Fakultät der Universität des Saarlandes, 66421, Homburg (Saar), Deutschland.
Perioperative bleeding is and remains a major complication during and after surgical interventions, resulting in increased morbidity and mortality. The main causes of a non-primary vascular-related bleeding are congenital or multifactorial pre-existing hemostatic disorders that have not yet been diagnosed, the operating procedure itself and acquired hemostatic abnormalities as a secondary phenomenon, e.g.
View Article and Find Full Text PDFBMC Musculoskelet Disord
March 2025
Department of Rehabilitation Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Science, Dongcheng District, Beijing, 100730, China.
Background: Current research on osteoporosis (OP) in hemophilia is insufficient. The suitability of high-resolution peripheral quantitative computed tomography (HR-pQCT) for evaluating osteoporosis in hemophilia remains unclear.
Aim: To investigate the current status of osteoporosis and the applicability of HR-pQCT in adult hemophilia patients.
J Thromb Haemost
March 2025
Unite d'Hemostase Clinique, Centre de Reference de l'Hemophilie, Hopital Louis Pradel, Lyon, France; Universite Claude Bernard Lyon 1, UR4609 Hemostase & Thrombose, Lyon, France. Electronic address:
Background: For every man diagnosed with hemophilia, approximately 1.6 women are expected to be carriers. Carriers are classified based on their Factor VIII (FVIII) levels and symptoms, ranging from asymptomatic to mild, moderate or severe symptoms.
View Article and Find Full Text PDFJ Thromb Haemost
March 2025
Department of Hematology, Radboud university medical center, Nijmegen, The Netherlands; Hemophilia Treatment Center, Nijmegen - Eindhoven - Maastricht, The Netherlands. Electronic address:
Background: Limited data exists on persons with rare bleeding disorders (RBDs) possessing a heterozygous genotype, as most studies focus on bi-allelic genotypes and more severe coagulation factor deficiencies. A growing body of evidence suggests that persons with a heterozygous genotype experience clinically relevant bleeding symptoms.
Objectives: Explore the incidence of bleeding symptoms and postoperative bleeding in persons with a heterozygous genotype.
Haemophilia
March 2025
Division of Hematology and Bone Marrow Transplantation, Azienda Ospedaliero-Universitaria Policlinico G. Rodolico - San Marco, Catania, Italy.
Background: Acquired haemophilia A (AHA) is a rare and severe bleeding disorder generally associated with pregnancy or aging. Spontaneous remission and prompt inhibitor eradication are described more frequently in postpartum cases. We evaluated retrospectively 15 postpartum AHA cases between 2007 and 2023 in order to evaluate response in terms of inhibitor eradication.
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