Orthopaedic evaluation in children with severe haemophilia A or B submitted to primary prophylaxis therapy in a coagulopathy treatment centre.

There is a lack of publications concerning the use of primary prophylaxis in developing countries. The aim of this study was to evaluate the effectiveness of primary prophylaxis therapy in preventing the development of arthropathy in children with severe haemophilia A or B. From January 1999 to April 2009, a prospective study was carried out involving 39 patients with severe haemophilia A or B. These haemophilia A and haemophilia B patients received 20-40 UI kg(-1) of factors VIII and IX, three and two times per week, respectively. The patients were followed up by a multidisciplinary team. The analysis was carried out in 23 patients who had been on prophylaxis therapy for at least 12 months. The orthopaedic evaluation was performed according to the recommendations of the Orthopedic Advisory Committee of the World Federation of Hemophilia, by evaluating pain and bleeding, and by conducting physical examination and radiological assessment (Pettersson's Joint Score and magnetic resonance): 82.6% of patients who had used the factor regularly did not present any clinical or radiographic changes in the studied joints; 17.4% used the factor irregularly at the beginning of the treatment and of those, most patients presented mild changes in the joints; and 4.3% presented transient knee and ankle pain in spite of regular factor use. The preliminary results of primary prophylaxis confirm its effectiveness in preventing haemophilic arthropathy. Socioeconomic factors did not play a significant role.

Pneumoperitoneum in association with perforated appendicitis in a Brazilian Amazon woman. Case report.

Radiographic findings of free air in the peritoneal cavity secondary to perforation of a acutely inflamed appendix are extremely rare. It accounts for about 0-7% of all patients with pneumoperitoneum. We report on a 58-years-old Brazilian Amazon woman presenting a 1- week history of abdominal pain, tenderness and distension associated with asthenia and without passage of stool or gas. Abdominal percussion revealed a tympanic sound located on the right hypocondrium. Plain chest radiography revealed a large amount of free air beneath the right leaf of the diaphram. The patient was taken immediately to the operation room and, during surgery, a gangrenous appendix with an apex perforation was verified. Appendectomy was performed as routinely. The patient evolved with pneumonia and septic shock that responded well to intravenous antibiotics and vasoactive drugs. She was discharged to home on the twenty-first post-operative day in good clinical conditions. This case highlights that perforated acute appendicitis is rarely associated with pneumoperitoneum, but it must be considered in the differential diagnosis of patients presenting right abdominal pain and free intraperitoneal air.