Haemophilic Arthropathy Surveyed with whole-body Gamma-Camera Scintigraphy

A number of joints suffering from haemophilic arthropathy was studied. They were surveyed clinically, radiologically, and with a new system of whole-body gamma-camera scintigraphy. This radio-isotopic technique provides advantages such as sensitivity, speed and safety. The results of the survey are presented and discussed.     

ARTICULAR MANIFESTATIONS OF ACROMEGALY

Forty-five patients with acromegaly or gigantism were reviewed for musculoskeletal abnormalities. Abnormalities of peripheral joints occurred in 74% of the patients and spinal involvement in 47%, leading to significant morbidity. Joint abnormalities most frequently affected the large joints (hips, knees and shoulders) but the wrist and hand were also involved. The radiological features of acromegalic arthropathy are described, including vertical widening of the hip joint, enthesopathy and osteophytosis. A favourable response to treatment is associated with a less severe arthropathy and a good functional outcome.     

先天发育性（骨）关节病——关节病的新概念

目的：关节病的传统概念其病因是获得性的或由后天继发因素所致，本文提出先天发育性（骨）关节病的概念。材料与方法：本组通过对成骨不全（６例），Ｍａｒｆａｎ综合征（４例），粘多糖病（８例），多发骨骺发育异常（２２例），致密性骨发育不全（２例），干骺软骨发育异常（４例），石骨症（３例），遗传性多发外生骨疣（６例），内生软骨瘤病（４例），Ｍａｆｆｕｃｃｉ病（２例），Ｍａｆｆｕｃｃｉ病（２例），系统性骨纤维发     

Intra-articularcalcergy and its arthropathic sequelae

Local calcergy is an ectopic calcification reaction (due to hydroxyapatite formation) induced in connective tissue sites by the application of dilute solutions of certain metallic salts (calcergens). The phenomenon is usually produced in the subcutaneous tissues of the experimental animal. The single intra-articular 66injection of lead acetate (PbAc) solution (a known calcergen) into the knee joint of the rat is followed by opacity of the synovial membrane and para-articular tissues with the aggregation of macrophages and multinucleated giant cells and the formation of some granulation tissue. This lesion ultimately resolves and there is no cartilaginous degeneration. Repeated intra-articular injections of PbAc produce pronounced changes in the synovium and para-articular tissues. There is extensive calcific deposition with exuberant macrophage and giant cell accumulation and fibroblastic proliferation. The proliferated synovial membrance becomes adherent to articular cartilage. There is thinning and fragmentation of the latter with focal loss and replacement by fibrous tissue. Therefore, in contrast the effects of a single injection, repeated calcergenic stimulation of the knee joint is associated with a destructive arthropathy.     

Behçet's disease

Behçet's disease is characterized by three primary components: iridocyclitis (historically with hypopyon), aphthous lesions in the mouth, and ulceration of the genitalia. Erythema nodosum, arthropathy and thrombophlebitis often accompany these manifestations, but the ocular symptoms may be the most important and serious manifestations of the disease. Central nervous system involvement, most often due to necrotizing vasculitis, may be the most protean manifestation of the disease, leading to death. The frequency of ocular manifestations is 70–85% in patients with the disease; the underlying disease mechanism in all organ systems is an occlusive vasculitis. Although the most common ocular symptom is that of anterior uveitis, often with hypopyon as a very late sign, the presence of necrotizing retinal vascular lesions is well known and often obscured by the severity of the anterior reaction. Definitions, incidence, clinical characteristics, differential diagnosis, and management of Behçet's ocular disease are discussed, as are the interrelationships of the different organ manifestations. The ophthalmologist should be familiar with the full spectrum of disease presentation since he or she may be the first physician to encounter the Behçet patient.     

Hemiarthroplasty in rotator cuff tear arthropathy

Rotator cuff tear arthropathy results in a painful glenohumeral joint with poor function. Loss of the stabilizing effect of the rotator cuff allows superior subluxation of the humeral head. The poor mechanical advantage of the deltoid muscle creates a weak, poorly functioning shoulder. Hemiarthroplasty with a large humeral head component can relieve pain and retain some of the mechanical advantage of the deltoid by lateralizing the center of rotation of the humeral head and regaining deltoid length. A larger-than-anatomic humeral head decreases the risk of further superior subluxation of the humeral head by decreasing wear. Stability is regained by retensioning the deltoid muscle envelope. This article reviews the technique of large head hemiarthroplasty in this difficult reconstructive problem.     

Outpatient treatment for haemophilic arthropathy with radiosynovectomy: Radiation dose to family members

One of the key features of good practice in medicine is the doctor‐patient communication. Radiation protection standards for radiosynovectomy (RS) is limited. Yttrium‐90 is a beta‐emitting radioisotope used in RS to treat joint pain from haemophilic arthritis. ICRP 94 states that if a patient is treated with up to 200 MBq, there is no need for further precautions when it comes to public exposure, however, activities can go up to 370 MBq in RS for the knee. This study analysed 119 family members’ safety (16.7% pregnant women). The ambient dose equivalent rate was measured within four distances. A survey was carried analysing risk groups and time spent next to patients. Results showed that family members should be advised to remain at 1.0 m from the patient to decrease accumulated dose by 97.6%. The dose per activity factors estimated in this study is also a useful tool during the risk assessment and doctor/patient communication. Pamphlets were distributed with radiation protection recommendations. Ambient dose equivalent was low enough to show that RS is a safe procedure for family members, which is essential to promote adherence to RS in countries where it is needed but not performed due to lack of information on radiation safety.     

Hemophilic arthropathy of the elbow

Hemophilia is a hereditary disease in which circulating levels of coagulation factors are lacking, resulting in a propensity toward bleeding. Intra-articular hemorrhages are a hallmark of hemophilia and may lead a cascade of cytokine elaboration and?inflammatory-mediated changes, which ultimately result in cartilage loss and arthropathy. Diarthrodial joints, such as the knee, elbow, and ankle, are most commonly affected. This article highlights issues surrounding hemophilic arthropathy of the elbow and focuses on preventive measures, management strategies of the hemophilic elbow, and treatment options for established arthropathy.     

Successful angiographic embolization of recurrent elbow and knee joint bleeds in seven patients with severe haemophilia

Summary.  In haemophilic joints with high-grade arthropathy, bleeds occur that do not respond to replacement therapy of the deficient coagulation factor. The reason may be pathologically reactive angiogenesis in chronic synovitis. Seven patients with severe haemophilia A or haemophilia B experienced recurrent massive bleeds of one elbow joint or knee joint in the absence of trauma. After initial application of factor VIII or IX (fVIII/fIX; 50 IU kg⁻¹ bodyweight), there was only slow and never complete relief of symptoms. Despite intensive secondary prophylaxis maintaining the plasma level of factor concentrate at minimum 50%, new massive bleeds at the same location occurred. Vascular bleeding was suspected. Angiography of the arteries was performed via the femoral artery. Vessels identified as potential bleeding sources were embolized with embolization fluid (ONYX) in eight joints (six elbow and two knee joints). Under low-dose prophylactic treatment (15 IU fVIII or fIX per kg bodyweight for three times per week), no recurrent severe bleed unresponsive to coagulation factor replacement occurred after a mean observation time of 16 months after embolization. The consumption of factor concentrate decreased to one-third of the amount consumed before embolization. In conclusion, angiographic embolization with a non-adhesive liquid embolic agent might be considered as a promising therapeutic and coagulation factor saving option in joint bleeds not responding to replacement of coagulation factor to normal levels.