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Biomedical subjects

J F Brantus

Publications and source records attributed to J F Brantus.

8 recordsLinked to original sources

Control of severe systemic lupus erythematosus after high-dose immunusuppressive therapy and transplantation of CD34+ purified autologous stem cells from peripheral blood.

A 35 y old woman with severe and progressive systemic lupus erythematosus (SLE) received high-dose chemotherapy followed by a T cell depleted autologous stem cell transplantation. Peripheral blood stem cell were mobilised with Cyclophosphamide 4.5 g/m2 followed by Granulocyte-Colony Stimulating Factor (G-CSF). A CD34 positive selection provided a 3 log T cell depletion. High-dose immunosuppression consisted of the BEAM regimen. The purified autograft was reinfused on day 0. In the post transplant period, hemopoietic growth factors, G-CSF, Granulocyte-Macrophage Colony Stimulating Factor (GM-CSF) and Erythropoietin, were administered, engraftment was rapid. Both the mobilisation and the transplant procedures were easily performed and well tolerated. One year later, the patient is in clinical remission. The ANA and anti-SSA-antibodies were undetectable at 1 and 6 months after intensification, but reappeared at low levels at 9 months. Corticosteroid requirement has gradually decreased. In conclusion, we report here the favourable evolution of a patient with a severe SLE, who clinically improved with high-dose immunosuppressive therapy and autologous stem cell transplantation, and showed a 9 month serological remission.

Adult↗

Effects of intravenous etidronate and oral corticosteroids in fibrodysplasia ossificans progressiva.

There is no known effective therapy for fibrodysplasia ossificans progressiva. Ethane-1-hydroxy-1-diphosphonate has been studied because of its inhibitory effect on bone mineralization and its potential to impair the rapid ossification process observed after acute episodes of fibrodysplasia ossificans progressiva. In an open study, the effects of intravenously administered ethane-1-hydroxy-1-diphosphonate and oral corticosteroids in the treatment of fibrodysplasia ossificans progressiva was evaluated. Thirty-one fibrodysplasia ossificans progressiva attacks were observed in seven patients during the mean followup of 6 years. In 29 attacks, the authors observed a rapid diminution of local inflammation, swelling, and pain during the first 7 days of treatment. However, despite the ethane-1-hydroxy-1-diphosphonate treatment, 10 new ossifications were observed, causing severe deterioration of joint mobility in all affected patients. In 21 attacks, no new ectopic ossification appeared. The radiologic pattern of preexisting ossifications did not change during the treatment. There are no data in the literature describing the percentage of acute episodes leading to an ossification. The results suggest the possibility that intravenous administration of ethane-1-hydroxy-1-diphosphonate and oral steroids may be helpful for the treatment of fibrodysplasia ossificans progressiva, but more control data on the spontaneous resolution of early flareups are needed.

Administration, Oral↗

Sapho syndrome with femoral hyperostosis. Two case-reports.

A characteristic manifestation of SAPHO syndrome is hyperostosis, which most typically affects the sternoclavicular joints. Two cases of SAPHO syndrome with painful hyperostosis of the femur are reported. This misleading localization can lead to delays in the diagnosis and treatment.

Adult↗

[Rheumatic manifestations associated with human immunodeficiency virus infection (HIV)].

The number of subjects presenting articular manifestations associated with HIV-infection is constantly on increase due to the growing number of seropositive patients. The pathophysiological mechanism of this association is still unknown. A recent review of the literature has shown that spondyloarthritis are the most frequently observed articular manifestations in HIV-positive patients. Septic arthritis are rare but severe. The immunodepression due to HIV-infection makes it difficult to use immunosuppressors and renders local therapeutics preferable. The articular manifestations may allow to discover seropositivity that has not been diagnosed before. These data strongly suggest to include serological examination for HIV in the etiological screening for every articular manifestation of unknown origin, even if the patient presents no risk factors.

AIDS-Related Opportunistic Infections↗