An integrated approach in a case of facioscapulohumeral dystrophy.

BACKGROUND: Muscle fatigue, weakness and atrophy are basilar clinical features that accompany facioscapulohumeral dystrophy (FSHD) the third most common muscular dystrophy.No therapy is available for FSHD. CASE PRESENTATION: We describe the effects of 6mo exercise therapy and nutritional supplementation in a 43-year-old woman severely affected by FSHD. CONCLUSION: A mixed exercise program combined with nutritional supplementation can be safely used with beneficial effects in selected patients with FSHD.

Eber- and LMP-1-expressing pulmonary lymphoepithelioma-like carcinoma in a Caucasian patient.

Primary lymphoepithelioma-like carcinoma (LELC) of the lung is a rare tumor that preferentially affects Asian patients, with only 15 cases described in Caucasians to date. Epstein-Barr virus (EBV) infection is present in most lung LELCs in Asians, but it has never been shown in Caucasians. EBV small nuclear RNA and latent membrane protein-1 were identified in the neoplastic cells of primary LELC of the lung diagnosed by endobronchial biopsy in a 25-year-old Italian male. Despite advanced locoregional disease, with neoplastic infiltration of the main right bronchus, the carina, and the pulmonary artery, which prevented surgical resection, partial response and 1-year symptom-free follow-up were achieved after combined chemotherapy and radiotherapy.

Efficacy of tacrolimus rescue therapy in refractory acute rejection after lung transplantation.

BACKGROUND: Encouraging results in transplantation of other solid organs led to investigation of the use of tacrolimus in lung transplantation as a salvage immunosuppressant in persistent acute rejection. METHODS: The incidence and severity of acute rejection and the number of steroid pulses were analyzed in 20 lung recipients who were converted from a cyclosporine- to a tacrolimus-based immunosuppressive regimen because of refractory biopsy-proven acute rejection. RESULTS: Tacrolimus was started 12.0 +/- 13.0 months after transplantation, and the mean follow-up was 25.0 +/- 13.7 months. After shifting to tacrolimus, a significant decline was observed in both the number of acute rejections per patient (3.0 +/- 1.56 to 0.85 +/- 1.14, p < 0.0001), and the incidence of acute rejection per 100 patient-days (1.52 +/- 0.99 to 0.14 +/- 0.21, p < 0.0001). Furthermore, the average histologic grade of rejection decreased from 1.9 +/- 0.8 to 0.4 +/- 0.5 (p < 0.0001). Methylprednisolone pulses similarly decreased from 1.9 +/- 1.3/patient to 0.3 +/- 0.7/patient (p < 0.0001). During cyclosporine immunosuppression, the mean forced expiratory volume in 1 second decreased to 84.4% +/- 13.3% of individual best value. The average lung function parameters were stable 3 months after the change of medication, and then began to improve. After an average follow-up of 36.5 +/- 19.2 months, 2 patients have developed bronchiolitis obliterans syndrome (one has Stage 1 and one has Stage 3). CONCLUSION: Conversion to a tacrolimus-based immunosuppressive regimen for refractory acute lung rejection is associated with reduced incidence and severity of acute rejection episodes, steroid sparing, and stabilization or improvement of pulmonary function.