How mild is mild haemophilia?

INTRODUCTION: People with mild haemophilia (PWMH) experience sporadic bleeds and are less likely to receive an early diagnosis, appropriate treatment and medical care. Arthropathy is a key determinant of health-related quality of life (QoL), producing pain, limitations in mobility and daily activities. The aim of this study is to evaluate the incidence, risk factors and QoL associated with arthropathy in PWMH. MATERIALS AND METHODS: Observational, cross-sectional cohort study. Data were collected in a single interview and evaluated by a physiotherapist and an orthopaedist and analysed on demographics; baseline factor levels; as well as clinical (Haemophilia Joint Health Score [HJHS]), ultrasound (Haemophilia Early Arthropathy Detection with Ultrasound [HEAD-US]), radiological (Pettersson score [PS]), pain (visual analogue scale [VAS]) and QoL evaluations. We defined arthropathy when at least one of the joints shown with a HEAD-US score ≥ 1. RESULTS: Eighty-five patients and 510 joints were included. Patients' mean age was 35.9 years-old. Median age was 44.2 in patients with arthropathy versus 14.9 in patients without; the difference was statistically significant (p < .001). In patients over 20 years old, 90.5% shown arthropathy. Only 24 (28%) patients had no joint damage (HEAD-US = 0), and 61 (72%) had at least one joint with a HEAD-US ≥ 1. The ankle was the most affected joint. Patient age was found to be the most important risk factor associated with the development of arthropathy. CONCLUSIONS: Joint damage as a result of prior hemarthrosis was the most relevant factor associated with lower QoL, and emphasised the importance of early diagnosis and appropriate management in this particular population.

Clinical and ultrasound evaluation of patients with haemophilia on prophylaxis.

INTRODUCTION: Primary prophylaxis is the current gold standard in haemophilia care for the prevention of bleeding and ensuing joint damage. Early detection of joint bleeding, whether symptomatic or subclinical, preferably during childhood, helps prevent joint deterioration and subsequent disability. The aim of this study is to evaluate the level of agreement between the Haemophilia Joint Health Score and the Haemophilia Early Arthropathy Detection with Ultrasound tools in children with severe haemophilia on primary and secondary prophylaxis. MATERIALS AND METHODS: All patients were followed up regularly at our centre. Elbows, knees and ankles were evaluated by physical examination using the Haemophilia Joint Health Score 2.1 (HJHS 2.1), and by ultrasound with HEAD-US score. RESULTS: A total of 80 children with haemophilia on prophylaxis were included in this study. Mean age was 10.8 years (range 4-18). We evaluated 480 joints, of which 423 (88.1%) were concordant with both tools, whereas 57 (11.9%) were discordant; 377 (78.5%) joints scored 0 on HJHS, 370 (77%) on HEAD-US and 345 (72%) on both tools. The overall Kappa concordance coefficient was .656. For elbows, knees and ankles the respective values were .783, .522 and .589. For HJHS scores greater than 3, all joints scored ≥1 on HEAD-US. CONCLUSION: HJHS and HEAD-US are used to assess joint health in children with haemophilia on prophylaxis. In this study, the level of agreement between both tools was consistent with literature values only for the elbow joint.

Platelet-rich plasma vs platelet-rich plasma plus hyaluronic acid for haemophilic knee arthropathy treatment.

Repeated joint bleeding leads to chronic synovitis, cartilage damage and bone alterations which result in haemophilic arthropathy and are associated with pain, functional impairment and poor quality of life. There are evidence that Hyaluronic Acid (HA) and Platelet-rich Plasma (PRP) have different mechanisms of action in the treatment of arthropathy for this reason we decided to use both components. The aim of this study is to compare, the efficacy, safety and duration of a single intra-articular injection of PRP against PRP+HA for pain, bleeding episodes and joint health, in the same patient with bilateral hemophilic knee arthropathy. Twenty-one men patients (42 knee joints) were treated with intra- articular injections of PRP or PRP+HA. All of them were haemophilia type A severe. The mean age was 36.6 years (21-72). All patients were evaluated for: Haemophilia Joint Health Score (HJHS), pain (VAS), the number of bleeding episodes (BE) in the last 30 days, before treatment, at three and six months after treatment. Statistically significant improvement were shown for both knee joints at three and six months after treatment for VAS and BE (P < 0.00001). The HJHS score did not significantly improve for either knee in the 6-month period after injection. A single PRP or PRP+HA injection is safe and effective in treating haemophilic arthropathy of the knee for up to 6 months follow-up, reducing pain, bleeding episodes and delaying total knee arthroplasty.

Minimally invasive surgery for haemophilic pseudotumour of the limbs: 28 years of experience.

INTRODUCTION: Haemophilic pseudotumour (HP) is an encapsulated haematoma in patients with haemophilia (PWH) which has a tendency to progress and produce clinical symptoms related to its anatomical location. AIM: To show the experience of one surgeon who has been using mini-invasive technique to treat pseudotumours of limbs in PWH with and without inhibitors at one centre for 28 years. MATERIALS AND METHODS: Thirty-three patients with 39 HP were treated. All patients had haemophilia A. Twenty-four patients had no inhibitors (72.8%), and 9 had inhibitors (27.2%). The mean follow-up was 16 years (1-25). All patients had x-rays and MRIs. All of them received Buenos Aires protocol as conservative treatment for 6 weeks. MRIs were repeated after 6 weeks' treatment to assess response to treatment. Surgery was performed in patients who did not respond to conservative treatment. RESULTS: After Buenos Aires protocol, four pseudotumours did not shrink (10.24%), 33 (84.61%) shrank, and two (5.12%) healed. Thirty-seven pseudotumours had surgery, 35 pseudotumours (94.59%) healed with minimally invasive treatment, and two did not heal (5.41%). No infection was observed with this treatment. The mortality rate for the series was 0%. CONCLUSION: The minimally invasive treatment of pseudotumours was effective in 95% of the cases and resulted in no mortality in this series after 28 years.

Inhibition of Fenton reaction is a novel mechanism to explain the therapeutic effect of intra-articular injection of PRP in patients with chronic haemophilic synovitis.

INTRODUCTION AND AIM: Haemarthroses cause major morbidity in haemophilia resulting in chronic haemophilic synovitis (CHS) and arthropathy. Oxidation of haemoglobin-coupled iron released in synovium after haemolysis induces chondrocytes death and cartilage damage, allowing postulate using iron-chelating drugs as potential therapeutic tool for haemophilic joint damage. Considering that albumin, the most abundant plasma protein, is a physiologic iron chelator, we aim to demonstrate that impediment of haemoglobin oxidation is exerted by plasma as a mechanism involved in the therapeutic effect of intra-articular injection of platelet-rich plasma in CHS. METHODS: Oxidation of haemoglobin (Hb) to methaemoglobin (MeHb) through Fenton reaction was induced in vitro by addition of potassium ferricyanide in the presence or absence of peripheral blood-derived platelets-rich or platelets-poor plasma (PRP/PPP) or albumin. The relevance of in vitro findings was analysed in synovial fluid (SF) samples from one patient with CHS obtained before and after 6 months of PRP intra-articular injection. RESULTS: MeHb formation was completely impaired either by of PPP, PRP or albumin indicating that PRP exerts an anti-oxidative effect, probably due by plasma albumin. Analysis of SF samples revealed the presence of MeHb levels and haemosiderin-laden macrophages in SF obtained before PRP treatment. Reduction of synovial MeHb, normalization of cellular composition and improvement of health joint haemophilic score, pain and bleeding episodes were registered after 6 months of PRP intra-articular injection. CONCLUSION: Inhibition of Fenton reaction and the consequent normalization of joint cellular composition is a noncanonical mechanism underlying the therapeutic effect of PRP intra-articular injection in CHS.

The hidden joint in children with haemophilia on prophylaxis.

Prophylaxis is the gold standard treatment for children with haemophilia (CWH). MRI studies revealed joint damage, even with this treatment; this suggests the presence of subclinical bleeding. In the case of children with haemophilia, it is relevant to detect early signs of joint damage, as this allows the medical team to provide the appropriate treatment and follow-up, in order to avoid arthropathy development and its consequences. The aim of this study is to detect the hidden joint in children with haemophilia on prophylaxis (CWHP) and analyse, by age group, which joint is the most affected. We define the hidden joint in CWH on prophylaxis as the joint that presents joint damage secondary to repetitive bleeding episodes and is detected in the joint evaluation, despite being asymptomatic or with mild symptoms. It is most commonly caused by repetitive subclinical bleeding. METHODS: This was an observational, analytical, cross-sectional study of 106 CWH on prophylaxis treated in our centre. Patients were divided according to age and type of treatment. Joint damage was defined as a HEAD-US score ≥ 1. RESULTS: Patients' median age was 12 years. All had severe haemophilia. The median age of onset of prophylaxis was 2.7. Forty-seven (44.3 %) patients received primary prophylaxis (PP) and 59 (55.7 %), secondary prophylaxis. Six hundred and thirty-six joints were analysed. Type of prophylaxis and joint involvement showed statistically significant differences (p < 0.001). However, patients on PP had a greater number of damaged joints at older ages. Twenty-two % (140) of the joints scored ≥1 on HEAD-US. Cartilage was most frequently involved, followed by synovitis, and bone damage. We observed a greater frequency and degree of arthropathy in subjects aged 11 and above. Sixty (12.7 %) joints showed a HEAD-US score ≥ 1, with no history of bleeding. The ankle was the most affected joint, representing the hidden joint according to our definition. CONCLUSION: Prophylaxis is the best treatment for CWH. However, symptomatic or subclinical joint bleeding may occur. The routine evaluation of joint health is relevant, particularly, of the ankle. In our study, early signs of arthropathy according to age and type of prophylaxis were detected by HEAD-US.

Lesion of the hip abductor mechanism.

INTRODUCTION: The disruption of the abductor muscles of the hip after hip revision surgery often causes limping, pain, and instability of the implant. The purpose of our paper is to describe a mesh technique to repair hip abductor mechanism injuries after hip revision. PATIENTS AND METHODS: Forty-six patients with hip abductor damage after prosthetic revision were treated. Inclusion criteria were: patients presenting with prosthetic loosening, complaint of pain, and with a positive Trendelenburg sign due to deficient abductor muscle mechanisms. Thirty-one were women (67.39%) with an average age of 64 years (34-82 years). The number of previous revision surgeries was three (two to seven). The Merle d'Aubigné score and variants before and after treatment were also reported. RESULTS: In the postoperative follow-up after hip revision with the mesh technique, the Merle d'Aubigné score improved and the Trendelenburg sign was negative in 78.3% of the patients (p < 0.001). Also, the Trendelenburg test with the knee flexed was negative in 60.9% (p < 0.001) and the stair-climbing test was negative in 60.9% of cases (p < 0.001). The gluteus medius test in the lateral position was negative in 52.2% of patients, and in the lateral position with the knee flexed it was negative in 47.8% of patients (p < 0.001). DISCUSSION: Repair of the abductor mechanism with the mesh technique has proven effective for both partial and total lesions.

Intertrochanteric partial osteotomy for posterior hip approach.

The posterior dislocation remains one unresolved complication of the posterior approach to the hip joint. In this study, a variation of the posterior hip approach - a partial osteotomy of the greater trochanter - was performed in order to investigate whether it provides better stability to the operated hip joint and reduces the risk of dislocation. We carried out a partial intertrochanteric osteotomy, initially in a cadaveric model and then in 68 patients (30 acute neck of femur fractures and 38 patients with hip osteoarthritis) requiring a total hip replacement. The osteotomised fragment was reattached with wires. All arthroplasties were cemented with Müller acetabular component and Charnley-type stem with a 28-mm interchangeable neck. Intraoperatively, all hips were tested for stability. After the osteosynthesis of the osteotomised fragment, hip dislocation points were increased more than 15% in the flexion and 10% in the internal rotation plane of movement. At one year follow up, no dislocation was observed in the clinical component of the study. This technique was found reliable in providing a stable hip joint.

Mega prótesis a medida para la reconstrucción de defectos óseos masivos en pelvis ¨El cirujano como diseñador del implante¨ / ORTHOPAEDIC RECONSTRUCTION OF MASSIVE PELVIC BONE DEFECTS AFTER PELVIC TUMOUR RESECTION

Despite major developments in the field of orthopaedic reconstruction of pelvic bone defects after pelvic tumour resection, massive pelvic bone loss with bone defect and pelvic discontinuity, still remains a complex problem. Resection of big musculoskeletal sarcomas of the pelvis and the later reconstruction of the residual defect constitutes one of the more exigent challenges of orthopaedic surgery. The authors present a method for reconstruction with a personalized prosthesis by mean of a rapid prototype specially designed for each occasion. The authors describe this individually prefabricated prosthesis based on rapid prototyíng technique with a computer-assisted design in a 3D printing based version. The results obtained are presented and commented.

Osteotomía desrotadora en la necrosis aséptica de la cadera / Distrotative osteotomy for the treatment of aseptic necrosis of the hip

The aseptic necrosis of the hipo is a common pathology, that leaved to its natural evolution can originate a severe discapacity. such pathology involves the femoral epiphysis and leds to the progressive destruction, originating in its final stage, the necroarthrosis. the origin of the disease can be traumatic, secondary to a fracture of the femoral neck or to a luxation or luxofracture of the hip. The autohors present a surgical technique termed disrotative osteotomy of the femur. the results of the experience and details of the surgical procedure, are detailed