Prophylaxis therapy in haemophilia A: current situation in Spain.

The Spanish Epidemiological Study in Haemophilia carried out in 2006 enrolled 2400 patients [2081-86.7% with haemophilia A (HA) and 319-13.3% with haemophilia B]; 465 of them (19.4%) were on prophylaxis. These rates were higher in patients with severe haemophilia (45.4%) and severe paediatric cases (72.5%). On the basis of information recorded in this study, we analysed the current situation of prophylaxis therapy administered to patients with HA in Spain, as well as their orthopaedic status. Prophylaxis was used in 399 (19.2%) patients with HA; such prophylaxis was primary (PP) in 20.3% and secondary (SP) in 75.9% of cases. Among severe HA patients, 313 (45.9%) were on prophylaxis (22.3% on PP and 74.7% on SP). Taking into account the patients' age, 34.7% of severe HA adults were on prophylaxis (6% PP and 92.1% SP), whereas 71.5% of severe HA paediatric patients (40.5% PP and 55.4% SP) received this kind of treatment. Established haemophilic arthropathy (EHA) was detected in 142 from 313 severe HA patients (45.3%) on prophylaxis, but only in 2.9% of patients under PP vs. 59% of patients receiving SP. There was no EHA in adult severe HA patient on PP, whereas 70.4% on SP had joint damage (P < 0.00001). Among paediatric severe HA patients, EHA was detected in 3.3% under PP and 37.8% under SP (P < 0.00001). In conclusion, our data suggest that an early initiation of prophylaxis avoids EHA in the long-term in patients with severe HA. We should emphasize the early onset of prophylaxis regimens.

Haemophilia in Spain.

To determine the prevalence of haemophilia A and B and their complications in Spain, and to characterize the health care network providing support to haemophiliac patients. The study examines clinical and genetic characteristics, treatment options, and complications observed during the course of the disease. Cross-sectional multi-centre study. The study population were patients with HA and HB in active follow-up at any Spanish hospital by December 2006. We studied 2400 haemophiliacs, 2081 (86.7%) HA and 319 (13.3%) HB patients. Illness was severe in 32.3% of patients, moderate in 16.4%, and mild in 51.3%. Genetic screening was carried out in 32.6% of the patients. Treatment administered in 2006 consisted of coagulation factor concentrates in 60% of patients. Until December 2006, 45.8% of severely ill patients were taking prophylaxis. The mean number of bleeding episodes in 2006 was four for patients not receiving primary prophylaxis and 1.3 for those taking primary prophylaxis. Thirty percent of patients had established haemophiliac arthropathy in at least one joint; 16.8% of patients were HIV-infected and 34.8% HCV-infected. Inhibitors were detected in 10% of severe HA patients and in 6.5% of severe HB patients. Immune tolerance induction therapy was started in 34 patients. This is the first comprehensive study on the epidemiology of haemophilia in Spain. It will enable us to draw comparisons with neighbouring countries, to assess the quality of care provided to haemophiliacs in Spain, and to provide evidence-based guidance for the even provision and improvement of such care.

Bursitis: análisis clínico de 52 casos / Bursitis: clinical analysis of 52 patients

Estudio retrospectivo longitudinal que evalúa las características clínicas de los pacientes diagnosticados de bursitis durante un año en una sección de reumatología de un hospital terciario, comparándolos con resultados previos de la bibliografía. Se incluyó a 52 pacientes, diagnosticados todos de bursitis según criterios clínicos y ecográficos. De ellos, 19 pacientes (36,5 por ciento) presentaban bursitis superficial, y 33 (63,5 por ciento) bursitis profunda. La mayoría de las bursitis superficiales ocurrieron en varones (84,2 por ciento), con una media de 49,2 años de edad. En cambio, las bursitis profundas se dieron predominantemente en mujeres (78,8 por ciento) con una edad media de 53,8 años. Entre los factores predisponentes para las bursitis superficiales encontramos diferentes profesiones (albañil, fontanero, carpintero y ama de casa), frente a las profundas, en que los pacientes eran predominantemente sedentarios (el 100 por ciento de las bursitis glúteas). Las radiografías simples fueron normales, excepto en un 18,8 por ciento de las bursitis superficiales, en las que se observó un aumento de partes blandas. El diagnóstico clínico fue confirmado ecográficamente en todos los casos. Se obtuvo líquido de las bursas en 17 de las bursitis superficiales (89,4 por ciento), y en todos ellos se practicó examen en fresco y cultivo. En ningún caso se observaron cristales. El cultivo fue positivo en 8 pacientes (47 por ciento), y Staphylococcus aureus fue el germen aislado con más frecuencia (75 por ciento). Otros microorganismos aislados fueron: Streptoccus piogenes y Staphylococcus epidermidis. Sólo en cinco (15,1 por ciento) de las bursitis profundas se obtuvo líquido bursal: el cultivo fue negativo en todos los casos, detectándose la presencia de cristales de hidroxiapatita en un caso. El tratamiento más empleado en las bursitis superficiales consistió en la administración de antibióticos (89,5 por ciento); en cambio, en las bursitis profundas, predominó el uso de la infiltración local de esteroides (57,5 por ciento).Conclusiones: Las diferencias etiológicas encontradas entre las bursitis superficiales y las profundas pueden ser de utilidad a la hora de escoger el tratamiento correcto desde el inicio (AU)