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In the hemophilia population, obesity has an adverse effect on health care cost, chronic complications, and joint disease. Although staffs of federally-funded Hemophilia Treatment Centers in the United States (HTCs) anecdotally recognize these outcomes, practices to promote healthy weights have not been reported. This evaluation identifies routine practices among HTCs in body mass index (BMI) assessment, perceptions about need to address obesity, and roles in offering evidence-based strategies to promote healthy weights. A telephone survey was developed to assess HTCs practices including patient BMI assessment and counseling, perceptions about the importance healthy patient weights, and HTCs roles in weight management. Ninety of the 130 federally-funded HTCs contacted elected to participate and completed the telephone survey. Of these, 67% routinely calculated BMI and 48% provided results to patients. Approximately one third classified obesity correctly for children (30%) and adults (32%), using the Centers for Disease Control and Prevention (CDC)’s BMI cutoffs. Most HTCs (87%) reported obesity as an issue of “big” or “moderate” concern and 98% indicated HTC responsibility to address this issue. Most centers (64%) address patient weight during comprehensive visits. One third (33%) of centers include a nutritionist; of those without, 61% offer nutrition referrals when needed. Most (89%) HTCs do not have a protocol in place to address healthy weights; 53% indicated guidelines are needed. HTCs offer services to help improve weight outcomes. Training programs for calculating and interpreting BMI as well as identification of appropriate guidelines to apply to the HTC patient population are needed.

Purpose Although intracranial hemorrhage remains a leading cause of death in hemophilia, spinal epidural hematoma is seen rarely. Decompressive laminectomy has a high associated morbidity, and recent reports have suggested that patients can be treated conservatively without surgical intervention. Patients and methods We present a case of spontaneous spinal epidural hematoma diagnosed by MRI scan in a 6-month-old hemophilic infant. Immediate treatment with factor VIII replacement was instituted. Results There was rapid and complete clinical and radiographic resolution. Conclusion This case shows that selected hemophilic patients with spinal epidural hematoma can be spared surgical decompression by prompt medical attention.

Chronic proliferative synovitis secondary to haemathroses is a major complication in patients with severe haemophilia. Current management strategies include prophylactic infusions of the missing coagulation factor, corticosteroids, synoviorthesis and/or synovectomy with variable degrees of benefit. In addition, patients with coagulation factor inhibitors are not amenable to the invasive therapeutic modalities. The gross and microscopic findings of the synovitis in haemophilic arthritis are remarkably similar to those seen in patients with rheumatoid arthritis, although the pathophysiology of these two conditions are quite different. Haemophilic arthropathy, in the later stages, resembles degenerative rather than inflammatory joint disease. Oral D-penicillamine, a drug effective in the proliferative synovitis of rheumatoid arthritis, was evaluated in 16 patients. Ten patients had an unequivocal response, while three had a reduction in palpable synovium and three had no response. Thus 81% of the patients had a beneficial response. Minor reversible drug side-effects occurred in two patients (proteinuria in one and a rash in the second). The results of this study suggest that D-penicillamine is an effective and safe drug for the treatment of haemophilic chronic synovitis.

The experience with immune tolerance (IT) induction therapy for haemophilia patients with inhibitors, instituted during 1990-97 at 17 haemophilia treatment centres in the western United States, was reviewed. IT was instituted in 104 of 139 (75%) of all identified haemophilia A and haemophilia B patients with inhibitors. Doses and schedules for IT varied but most patients were treated with a daily administration of 25-200 units kg-1 day-1. Successful immune tolerance (defined as Bethesda Unit titres1.0) was achieved in 57 of 81 (78%) patients who completed therapy. IT success was significantly greater for those patients with historic titres100 BU (P0.0003) and those with titres of10 BU at initiation of IT (P0.0001). IT success was uniformly achieved in 16 patients with low to moderate responding inhibitors. IT in infants2 years of age was problematic due to the presence of high titre inhibitors and complications of venous access. Frequent complications of IT included increased bleeding during intravenous access device insertion (20%) and infections associated with venous access devices (64%). IT is widely accepted as a treatment modality for haemophilia patients with inhibitors. Patient participation in IT regimens and management during therapy is facilitated greatly by the efforts of treatment centre nurse coordinators who are knowledgeable about haemophilia and its complications. Additional clinical trials will be necessary to define better initial dose, schedule, dose adjustment and success/failure criteria for IT.

Danazol was given orally at a dose of 600 mg/day to six hemophiliacs for eight to 14 weeks. All patients showed a significant decrease in activated partial thromboplastin time (APTT) beginning with the first measurement (two weeks) and persisting until use of the drug was discontinued. However, a corresponding increase in the deficient factor activity could not be consistently demonstrated. Despite the shortened APTT, bleeding episodes continued in the severe hemophiliacs and the patient with Christmas disease. In four patients, bleeding appeared to increase in severity or change in pattern, and in two cases the bleeding manifestations did not respond to usual factor infusions but responded to discontinuation of the drug therapy and further factor replacement. Euglobulin lysis times were measured in five patients (one hemophiliac and four with nonhemophilic conditions) who were receiving danazol. The lysis times were markedly shortened. Increased fibrinolytic activity may be responsible for the increased bleeding manifestations in danazol-treated hemophiliacs.