Fourteen cases of sarcomatous degeneration in Paget's disease.

OBJECTIVE: To describe characteristics of cases of sarcomatous degeneration in Paget's disease (SDP) recorded over 10 years in 2 French university hospitals, with particular emphasis on the pattern of this entity on magnetic resonance imaging (MRI). METHODS: Fourteen cases are described. Epidemiological, clinical, radiological, histological, and MRI features (in 3 cases) are compared to others in the literature. RESULTS: Nine men and 5 women (mean age 75.2+/-7.7 yrs) were studied. Paget's disease was monostotic in 5 cases and polyostotic in 9. Neurological complications were present in 10 cases. The mean interval between the first clinical signs and diagnosis was 4.4+/-4.7 months. The locations were: limbs 2, skull 2, pelvis 7, sacrum 3. The radiological pattern was lytic in 9 cases, sclerotic in 3, and mixed in 2. MRI revealed a similar pattern in all 3 cases studied (heterogeneous low signal intensity of the tumor on T1 weighted images increased after intravenous gadolinium administration and high signal intensity on T2 weighted images), whereas the radiological pattern was lytic in 2 cases and sclerotic in one case. The diagnosis was proved by histological investigation in 11/14 patients (osteogenic sarcoma of different appearance in 7 patients, fibroblastic sarcoma in one, fibrous histiocytoma-like sarcoma in one, and undifferentiated sarcoma in 2). Four patients had pulmonary metastases and 4 patients are still alive with followup ranging from 5 months to 5 years. CONCLUSION: Paget sarcomas remain the most threatening sarcomas of bone, their prognosis being far more negative than primary sarcomas. Improvement in therapy strategies including surgery, radiation therapy, and chemotherapy might together provide better prognosis for SDP.

[Emergence of dengue hemorrhagic fever in French Antilles. 3 initial fatal cases in Guadeloupe]. / Emergence de la dengue hémorragique aux antilles françaises. Trois premiers décès en Guadeloupe.

BACKGROUND: Two outbreaks of dengue hemorrhagic fever occurred in Guadeloupe (French West Indies) in successive epidemics in 1994 and 1995. The first outbreak was caused by DEN-2 virus and the second by DEN-1. CASE REPORTS: Seven life-threatening infections (WHO grade 3/4) were identified. Three previously healthy adults (including two brothers) died. Autopsy reports (2 patients) disclosed hemorrhagic serous effusions, disseminated intravascular coagulation, and in one case a spontaneous spleen rupture. DISCUSSION: Dengue fever is an emerging disease. Its severe hemorrhagic form tends to an uprising incidence and can no longer be considered a disease limited to children in Far-Eastern Asia. Fatalities may occur very suddenly and unexpectedly, even in optimal health care settings, in healthy adults living or travelling in endemic areas, notably the Caribbean.

Abdominal angiostrongyliasis: third case in Guadeloupe - abstract

Angiostrongylus costaricencis causes a clinicopathologic disease, abdominal angiostrongyliasis (AA), first observed in Costa Rica by Morera and Cespedes. AA is mainly observed in children, and is characterized by abdominal pain typically localized to the right lower quadrant. The disease is mostly endemic to Central and South America. However, the disease has begun to appear outside of its endemic area and may simulate Crohn's disease, and is observed in old persons. We report 3 cases of AA, all diagnosed in the Department of Pathology in the CHU in Guadeloupe, FWI, since 1987. The 3 cases are instructive of the disease and the physician needs to be aware of it. It is also a priority to identify intermediate hosts to prevent the disease (AU)

Schnitzler's syndrome (urticaria and macroglobulinemia) dramatically improved with corticosteroids.

The authors report the case of a 65-year-old man who had an evolving case of Schnitzler's syndrome with lytic bone lesions which was unresponsive to several drugs, and which dramatically improved with small quantities of corticosteroids. Schnitzler's syndrome, first described in 1974, is defined by chronic non-pruritic urticaria, osteocondensation, and monoclonal IgM dysproteinemia without features of lymphoproliferative disease, and is associated with fever, deterioration of the general health and biological signs of inflammation. Only 22 cases of Schnitzler's syndrome have been reported hitherto and there is no known effective treatment, H1 and H2 blockers, dapsone, colchicine, chloroquine, cyclophosphamide, chlorambucil and azathioprine having proven useless in the few patients treated. Corticosteroids may be effective, however, especially against the urticaria.

Destructive arthritis of the wrist and palmoplantar pustulosis.

The authors report a case of destructive arthritis of the right wrist occurring in a 29-year-old Algerian man, associated with palmoplantar pustulosis and HLA B27 antigen. Joint fluid and synovial biopsy were sterile and the course was favourable with surgical synovectomy and nonsteroidal antiinflammatory drugs. Usually, arthritis associated with palmoplantar pustulosis is of the non-erosive type but cases of destructive arthritis have been reported. This condition is probably a new member of the seronegative spondylarthropathy group.

Three cases of arthritis after BCG therapy for bladder cancer.

The authors report three cases of arthritis occurring after treatment with BCG immunotherapy for bladder disease. This complication is observed in 0.5 to 1% of treated patients and affects the small joints within 1 to 5 months of the first BCG injection. The clinical outcome is favourable with non-steroidal antiinflammatory drugs. This form of arthritis may be attributable to a cross-reaction between antigenic components of cartilaginous proteoglycan and BCG, influenced by a particular HLA-DR pattern, and with the intervention of a heat shock protein.

[Automated percutaneous nucleotomy. Results in 50 patients]. / Nucléotomie percutanée automatisée. Résultats chez 50 patients.

Automated percutaneous discectomy was introduced by Onik et al. in 1985 for the treatment of lumbar disk herniation. Success rates have ranged from 42% to 86%. We evaluated efficacy and looked for factors with a bearing on outcome in a retrospective study of 50 patients. Patients who did not have subsequent surgery were evaluated at least six months after the procedure, using the criteria developed by Mac Nab and by Stauffer and Coventry. The procedure was successful in 31 patients (62%) and failed in 19 (38%). Thirteen patients with failed automated percutaneous discectomy required surgery. Severe disk degeneration was significantly predictive of treatment failure. Lumbar spinal stenosis was also associated with lower success rates. Two patients developed infectious discitis after the procedure. Automated percutaneous discectomy may be less satisfactory than nucleolysis. Further studies are needed to determine the role and efficacy of this method for the treatment of lumbar disk herniation.

Discitis after lumbar disc surgery. Features of "aseptic" and "septic" forms.

Two major types of postoperative discitis have been previously described: septic discitis and "avascular" or "chemical" discitis. Percutaneous discal biopsy is an important way of distinguishing these entities. In a retrospective study of 25 cases of postoperative discitis, three groups have been analyzed with bacteriologic and histologic tests: a group of nine patients (group A) with positive discal bacteriologic cultures; a group of eight patients (group B) with typical septic histologic tests but negative bacteriologic discal procedures; and a group of eight patients (group C) in whom the histologic picture was reminiscent of a mechanical process. No group was unique in any clinical and radiologic parameter. Group A and group B were quite similar in biological features, but group C had erythrocyte sedimentation rate and C-reactive protein serum levels significantly lower than groups A and B (P less than 0.01). After 4 weeks, these differences were still present. This study confirms that there are two main features of postoperative discitis that can be recognized by histologic and biological tests, allowing for different treatments.

[Reprogramming of a pacemaker induced by electrocautery]. / Reprogrammation d'un stimulateur cardiaque induite par le bistouri électrique.

A case is reported of reprogramming of a ventricular unipolar permanent pacemaker induced by electrocautery during biliary surgery. After skin incision and use of the unipolar electrosurgery unit, the CPI model 505 multiprogrammable pulse generator previously set at 70 b X min-1 abruptly fired at 120 b X min-1. Application of a magnet over the pacemaker reduced the heart rate to 100 b X min-1. After surgery, the pulse generator was successfully reprogrammed to a rate of 65 b X min-1. Based on the analysis of this case and of previous reports, it is suggested, so as to avoid such complications, that the unipolar electrocautery be avoided when the surgical field is near the pulse generator or lead: that the bipolar electrocautery be preferred; that a magnet and non-invasive programmer be available during and after surgery; and that a postoperative assessment of the pulse generator be carried out.