Aseptic necrosis of the femoral head in the course of haemophilia; a different clinical and radiological course compared to typical Legg-Calve-Perthes disease (twenty years of follow-up) – case report

The authors analyse the course of aseptic necrosis of the femoral head in a 7-year-old boy with haemophilia A (factor VIII level - 0%) caused by massive haemarthrosis into the hip joint (joint tamponade). The patient’s clinical and radiological symptoms differed from those in classic LCP disease, and now, after 20 years, the consequences of necrosis do not confirm typical Perthes disease (LCP). Significant shortening (about 5 cm) of the limb and the X-ray image of the proximal end of the femur resemble the consequences of severe necrosis of the femoral head in the treatment of an infant with developmental dysplasia of the hip (DDH). The study presents necrosis of the femoral head in the course of haemophilia due to its extremely rare occurrence as opposed to haemophilic arthropathy typical for this disease. This description may also contribute to considerations on the etiopathology of LCP disease.

Quality of life and its predictors among adult patients with haemophilic arthropathy. An observational study

Background Recurrent hemarthrosis that begin in childhood lead to progressive joint deterioration. Patients with haemophilia have chronic pain, functional disability and a reduced perception of health-related quality of life. Purpose To analyse the perceived quality of life of adult patients with haemophilic arthropathy and its relationship with pain, joint condition, kinesiophobia and catastrophism. Methods Eighty-three adult patients with haemophilia were included in this multicentre, cross-sectional, descriptive study. Perceived quality of life (36-Item Short Form Health Survey), perceived usual and maximum pain (visual analogue scale), joint condition (Haemophilia Joint Health Score), kinesiophobia (Tampa Scale of Kinesiophobia) and catastrophism (Pain Catastrophizing Scale) were assessed. Sociodemographic, clinical and therapeutic variables and drug consumption for pain control were collected. Descriptive statistics used means and standard deviations. The correlation of quality of life with the dependent variables was calculated with the Pearson correlation test. The differences in quality of life as a function of the binomial variables were calculated with Student’s t-test for independent samples. Results Physical component of quality of life perceived by patients with hemophilia is lower than Spanish population (30.51 VS 48.85). Regarding the mental component, patients with hemophilia showed higher values (56.07 VS 49.97). Catastrophism correlated (p < .05) with all items of quality of life questionnaire. Kinesiophobia correlated (p < .05) with all items of quality of life except to role-emotional (r = -.18; p > .05). Habitual and maximal joint pain correlated with all items except to role-emotional (r = − .19 and r = − .09, respectively) and mental component score (r = − .16 and r = − .07, respectively). Catastrophism and weekly drug intake were inversely correlated with quality of life. Age was positively correlated with perceived quality of life. There were differences in quality of life as a function of the severity of haemophilia and the intake of drugs for pain control. Conclusions The perceived quality of life of adult patients with haemophilia is worse than that of the Spanish population. Pain, kinesiophobia, catastrophism, haemophilia severity and the intake of pain-control medication influence the quality of life of these patients.

Magnetic Resonance Imaging of Hemophilic Joints: Correlations with the Bleeding Phenotype and Physical Examination

Introduction: Blood-induced joint damage as a hallmark of haemophilia continues to occur despite the widespread prophylaxis. Pre-cise assessment and follow-up of joint status are crucial for tailoring their treatment.&nbsp; Aim: To study the correlation between the bleeding phenotype, the functional joint status, and the magnetic resonance imaging score in pediatric patients with haemophilia. Materials and methods: Eighty-six joints (ankles, knees, and elbows) in patients aged 10.7&plusmn;0.5 (range 4 - 20) years with severe/moderate haemophilia A, severe haemophilia B and haemophilia A with inhibitors were included in the study. The joints were assessed by Haemophilia Joint Health Score 2.1 (HJHS2.1) one month after the last hemarthrosis in a non-bleeding state. The magnetic reso-nance imaging was performed on 40 (46.5%) of the examined hemophilic joints (16 ankles, 11 knees and 13 elbows).&nbsp; Results: Joint bleeds were present in 37 (38.9%) of the joints with ankles being the most commonly affected. Sixty joints (69.8%) had normal HJHS2.1 score. Only the loss of flexion score differed significantly between the joints and the ankles had highest score. The cumulative number of hemarthrosis in the joint correlated moderately with hemosiderin deposition and strongly with the formation of subchondral cysts on magnetic resonance imaging. The magnetic resonance imaging scores for soft tissue and osteochondral domains correlated moderately with the cumulative number of hemarthrosis in the joint and only with the presence of pain and crepitus of mo-tion from the physical examination. Conclusions: Magnetic resonance imaging is more sensitive than the bleeding phenotype and physical examination in detecting early signs of haemophilic arthropathy.