Prophylaxis in 10 patients with severe haemophilia A and inhibitor: different approaches for different clinical situations.

The effect of bypassing agents is not as predictable as replacement therapy with the deficient factor in inhibitor patients. Consequently, these patients have more levels of arthropathy than patients without inhibitors. Prophylaxis for inhibitor patients has gained attention over the last decade and some papers have reported that bypassing agents could work in the prevention of arthropathy. However, there is a lack data to support any specific agent or regimen or even to recommend their use in different clinical conditions. We report ten patients with haemophilia A and inhibitors treated prophylacticaly with bypassing agents (5 with FEIBA and 5 with NovoSeven). The variable conditioning the choice of one agent or the other was the intention to initiate of immune tolerance induction therapy (ITI) in the future. In 8/10 patients (4 in FEIBA group and 4 in rFVIIa group) there was a decrease of bleeding episodes while 9/10 maintained or increased their joint range of motion (ROM). In the rFVIIa prophylaxis group, prophylaxis can be considered primary since all of them had had less than one joint bleed before prophylaxis. Economic analysis showed that prophylaxis is an expensive treatment. In our experience both agents seem to be safe and effective in reducing the number of bleeds in patients with inhibitors. The anamnestic response provoked by FEIBA could be an issue while awaiting a decline in titres before ITI can be initiated and so rFVIIa may be the best option for prophylaxis in patients with inhibitors who have not yet begun ITI.

[Surgical management of cutaneous and musculoskeletal complications in fulminant sepsis]. / Tratamiento quirúrgico de las complicaciones cutáneas y musculoesqueléticas de los pacientes con sepsis.

INTRODUCTION: Surgical management of purpura fulminans is required as in the acute as in the sequelae period. A retrospective case review of these sequelae, procedures which have been performed in the different moments and the outcomes was undertaken. METHODS AND MATERIALS: We reviewed retrospectively 13 patients treated in our hospital from 1993 to 2003. 8 males and 5 females, with a median age of 2 years old (4 days-14 years) were managed. 70% were meningococcical. The most common areas were low extremities (92%), upper extremities (76%), lips (n=1), nose (n=1) and genitalia (n=1). Most of cases were bilateral (n=11) although two patients presented isolated affectation in one of the extremities. The procedures were classificated depending on the surgical moment. Clinical follow-up was attempted on all surgical patients; we evaluated the aesthetic, functional and psychosocial outcomes. RESULTS: One child died along the first 24 hours; 11 required early surgery and all of them required late interventions. 50% in the serie had necrosis over 15%. In Intensive Care Unit, it was necessary to do 6 fasciotomies (extirpating muscle in 2) and escharectomies and debridements in 8. Early amputations were made in 2 patients: both of them required ampliation in the following days. The cutaneous defects were covered with skin allografts and artificial dermis. During the sequelae period, 8 amputations were necessary (the four extremities in one case). Other procedures were coverage with autologous skin grafting (n=7), Zplasties, Wplasties (n=5), myocutaneous flaps (n=3), release of contractures (n=3) and osseus resection (n=5). There were skeletal growth disturbances which were managed with osteotomies (n=3), realineations(n=3), application of Ilizarov (n=2) and Hoffman (n=2) frames; Nowadays, 6 patients have a complete independent functionning with the use of orthotics (n=3) and prosthetics (n=3) CONCLUSION: The pediatric surgeon has an important role in management and prevention of complications in patients with purpura fuminans. Urgent actuation and close follow-up decreases the morbidity; furthermore, the early reparation of sequelae improves their adaptation in school, in the family and in the society despite the physical disability.

Tratamiento quirúrgico de las complicaciones cutáneas y musculoesqueléticas de los pacientes con sepsis / Surgical management of cutaneous, muscle and skeletal complications in fulminant meningococcemia

Introducción. El cirujano pediátrico participa en fase aguda y en el período de secuelas de la sepsis. El objetivo de este trabajo es evaluar estas secuelas, las técnicas utilizadas en las diversas fases de la enfermedad y los resultados obtenidos en nuestra serie. Material y métodos. Estudiamos retrospectivamente 13 pacientes tratados en nuestro centro por sepsis en los últimos 10 años. Fueron 8 niños y 5 niñas, con una edad mediana de 2 años (4 días-14 años); el 70% fueron meningocócicas. Las regiones afectadas fueron miembros inferiores (92%), superiores (76%), labios (n=1), nariz (n=1) y genitales (n=1). Las lesiones fueron bilaterales en la mayoría (n=11) aunque dos casos presentaron afectación aislada de un miembro. Los procedimientos se clasificaron según el momento de su realización. Evaluamos los resultados estéticos, funcionales y psicosociales a largo plazo. Resultados. Un niño falleció durante las primeras 24 horas; 11 requirieron actuación precoz y todos precisaron intervenciones tardías. El 50% de los casos presentó necrosis en más del 15% de la superficie corporal. Durante su estancia en UCIP, 6 casos precisaron fasciotomías (con extirpación de masa muscular en 2) y en 8 se realizaron importantes (..) (AU)

Introduction. Surgical management of purpura fulminans is required as in the acute as in the sequelae period. A retrospective case review of these sequelae, procedures which have been performed in the different moments and the outcomes was undertaken. Methods and materials. We reviewed retrospectively 13 patients treated in our hospital from 1993 to 2003. 8 males and 5 females, with a median age of 2 years old (4 days-14 years) were managed. 70% were meningococcical. The most common areas were low extremities (92%), upper extremities (76%), lips(n=1), nose (n=1) and genitalia (n=1). Most of cases were bilateral (n=11) although two patients presented isolated affectation in one of the extremities. The procedures were classificated depending on the surgical moment. Clinical follow-up was attempted on all surgical patients; We (..) (AU)

Yttrium-90 synoviorthesis for chronic haemophilic synovitis: Madrid experience.

Between 1994 and 1999 we performed 66 Yttrium-90 (90Y) synoviortheses on 44 persons with haemophilia (45 knees, 12 elbows, nine ankles). At the time of injection 23 patients were HIV+ and two had inhibitors. The average age was 21.1 years (range: 9-39). Five mCi of 90Y were injected into the knees, and 3 mCi into the elbows and ankles. The average follow-up was 3.5 years (range: 1-6). Of the 45 knees, there were eight excellent, 10 good, 15 fair and 12 poor results. Of the 12 elbows there were three excellent results, five good, three fair and two poor. Of the nine ankles there were no excellent results, four good, three fair and two poor. The elbows had better results than the knees and ankles. The best results were obtained in the youngest patients, and in those with a moderate degree of synovitis, regardless of their HIV status and the presence of inhibitor. 90Y synoviorthesis should be performed in very young patients, when the amount of synovium is still moderate. Once the degree of synovitis has become severe, the expected results of radioactive synoviorthesis are worse.

Post-synoviorthesis rehabilitation in haemophilia.

The efficacy of radiosynoviorthesis in the management of chronic haemophilic synovitis has been proven. Like replacement therapy, however, this procedure has no effect on muscle strength or endurance, range of movement, coordination, or the patient's physical condition in general. All the factors just enumerated are essential for the recovery and maintenance of the haemophiliac's quality of life. That is why rehabilitation and physiotherapy are so important, since no medical therapy or surgical procedure are by themselves capable of preventing disabilities.

Hemophilic synovitis of the knee and the elbow.

A prospective study from 1974 to 1996 was done to determine optimal treatment for chronic hemophilic synovitis of the knee and synovitis of the elbow. Sixty-five patients with synovitis affecting 65 knee joints and 40 patients who had synovitis of the elbow (44 elbows), despite a 3-month trial of prophylactic substitution therapy, were treated by synovectomy. Radiation synovectomies (Au-198 synoviorthesis) were done on 38 knees, open surgical synovectomy on 18, and nine had an arthroscopic procedure. Radioactive gold synoviorthesis was performed on 29 elbows, and 15 had a resection of the radial head and partial open synovectomy. Synovectomy (by any method) significantly reduced bleeding episodes, but did not halt the radiographic deterioration of the joints. It is thought that radiation synovectomy is the best choice for patients with persistent synovitis of the knee and synovitis of the elbow unresponsive to a 3-month trial of prophylactic factor replacement. If two to three consecutive synoviortheses with 3 to 6 months intervals had been ineffective, or when the radiographic score is more than two points, an open synovectomy is indicated.

Hamstring release for fixed knee flexion contracture in hemophilia.

Sixteen hamstring tenotomies and posterior capsulotomies were assessed retrospectively in 10 patients with hemophilia. The average age of the patients was 17 years (range, 16-24 years). The main indication for surgery was a fixed knee flexion contracture of 30 degrees to 45 degrees, associated with repeated hemarthroses, and failure of conservative treatment after 6 months. A posterior transverse capsulotomy and a Z shaped hamstring tenotomy were performed. The postoperative treatment consisted of repeated stretching exercises for a 6-month period. An average decrease of 25 degrees in the amount of fixed knee flexion contracture was obtained (range, 10 degrees-40 degrees). Followup for an average of 9.5 years showed 11 good, four fair, and one poor result regarding their joint scores. It is concluded that hamstring release is an effective surgical procedure for fixed knee flexion contracture in hemophilia. It seems to reduce the incidence of hemarthrosis relating to such a deformity.

[Psychological aspects of rheumatoid arthritis. Attempt at a better understanding of the problem]. / Aspects psychologiques de l'arthrite rhumatoïde. Essai pour une meilleure compréhension du problème.

This study is an attempt to identify the principle causes of stress in patients affected with rheumatoid arthritis. It is based on personal experience with 62 patients treated in a multidisciplinary manner over a time period of two years. The psychological impact of these stress factors on the patients were evaluated in an effort to further the treatment methodologies in this area. Among the factors considered are: the disease as a chronic process and as a result the progressive destruction of bodily function, the limitations produced by a disease of the joints, the presence of pain and the side effects produced by the medications. As a step to lessen the mental distress caused by RA, it is proposed that the physician team should seek to inform the patient in a clear and concise manner about RA to establish a working doctor-patient line of communication, and to encourage activities that promote the maintenance of patient self esteem.