Clinical gait features are associated with MRI findings in patients with haemophilic ankle arthropathy.

INTRODUCTION: Haemophilic ankle arthropathy due to repeated joint bleeds often leads to altered gait in adult patients with haemophilia. AIM: To investigate the association between clinical gait features and blood-induced ankle joint damage scored using MRI findings in patients with haemophilic ankle arthropathy. METHODS: This observational study investigated 48 ankles of 24 patients with severe haemophilia (median age of 33 years). Blood-induced ankle joint damage was scored by an experienced radiologist using the International Prophylaxis Study Group (IPSG-)MRI score which evaluates the presence or absence of effusion, synovial hypertrophy, haemosiderin, surface erosions, subchondral cysts and cartilage degeneration. Using 3D gait analysis, peak ankle joint power generation and absorption (W/kg) were measured for each ankle since these are surrogate measures for joint loading during walking. Associations between MRI findings and these two clinical gait features were calculated using Spearman's ρ correlation with an α-level correction (α = 0.01) for multiple tests. RESULTS: We found large negative associations between ankle joint peak power generation and IPSG-MRI score (ρ = -0.631; P = <.001), IPSG-MRI osteochondral subscore (ρ = -0.701; P = <.001), severity of synovial hypertrophy (ρ = -0.507; P = <.001) and haemosiderin (ρ = -0.400; P = .005). Associations were also found for ankle joint peak power absorption and IPSG-MRI score (ρ = -0.425; P = .003) and IPSG-MRI osteochondral subscore (ρ = -0.556; P = <.001). CONCLUSION: Severe blood-induced ankle joint damage relates to a lowered tolerance towards ankle joint mechanical loading during walking in patients with haemophilia.

Hemophilia carrier's awareness, diagnosis, and management in emerging countries: a cross-sectional study in Côte d'Ivoire (Ivory Coast).

BACKGROUND: Little data is available on awareness of hemophilia carrier condition or associated bleeding risk and management in Sub-Saharan African countries. This study sought to identify hemophilia carriers in Côte d'Ivoire in order to collect data on demographics, bleeding phenotype, and laboratory results. Another purpose was to provide Ivorian hemophilia carriers with counseling on their risk of bleeding and of having children with hemophilia. A 12-month prospective study was conducted involving Ivorian hemophilia carriers recruited trough pedigree analysis pertaining to 81 hemophilia patients followed-up at the Yopougon Hemophilia Treatment Center in Abidjan. They were assessed using in-depth interviews, pedigree analysis, and laboratory testing. RESULTS: Sixty-one subjects comprising 27 obligate and 34 possible carriers were recruited. None had previously been assessed, with 64% unaware of their carrier status despite a familial history of hemophilia in 69%. The most frequently reported bleeding symptom was menorrhagia (31%). Prolonged bleeding was reported after vaginal delivery in 19.6%, post-surgery in 4.9%, and post-dental extraction in 4.9%. Only one carrier was treated with tranexamic acid, with no other hemostatic therapy recorded. The median (range) clotting FVIII was 0.85 IU/mL (0.24-1.90 IU/mL) and FIX 0.60 IU/mL (0.42-1.76 IU/mL) in hemophilia A and B carriers, respectively. HA carriers had a FVIII < 0.5 IU/mL in 12.5%. CONCLUSIONS: This study highlights the need of implementing care for hemophilia carriers in developing countries, and the high value of pedigree analysis for carrier identification, along with the relevance of diagnosis, treatment, and education of carriers, families, and caregivers.