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September 30, 2004 Vol. 351 No. 14
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This multinational, double-blind trial compared caspofungin, an echinocandin, with liposomal amphotericin B as empirical antifungal therapy in 1095 patients with persistent fever and neutropenia. Caspofungin was as efficacious as the standard therapy and was better tolerated, with less nephrotoxicity and fewer drug-related adverse events.
In 2002, the Journal published a report of 13 cases of pure red-cell aplasia in patients with renal failure who were receiving erythropoietin (epoetin). From 1998 through 2003, 175 cases were associated with the use of Eprex (epoetin alfa), 11 with the use of Neorecormon (epoetin beta), and 5 with the use of Epogen (epoetin alfa). After the promulgation of new regulatory mandates, the incidence of pure red-cell aplasia associated with epoetin therapy was reduced by more than 80 percent.
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The autoimmune lymphoproliferative syndrome (ALPS) is a childhood disorder in which lymphadenopathy, splenomegaly, hypergammaglobulinemia, and autoimmunity can be traced to the resistance of lymphocytes to apoptosis. The failure of cell death is usually due to a germ-line mutation of Fas, a gene that is essential for apoptosis. This article describes a form of the disease involving a somatic mutation in Fas.
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A patient who inherited a mutation in the Fas gene from his father and a mutation in the perforin gene from his mother was found to have autoimmune lymphoproliferative syndrome (ALPS) and a B-cell lymphoma. The mutant Fas gene was probably responsible for the development of ALPS, and the mutant perforin gene probably diminished resistance to the development of a B-cell lymphoma.
Pulmonary arterial hypertension, which is characterized by vascular proliferation and remodeling of small pulmonary vessels, leads to a progressive increase in pulmonary vascular resistance and, ultimately, to right ventricular failure and death. Despite recent major improvements in therapy, no current treatments cure this devastating condition. This article discusses recent progress in developing treatments that prolong patients' lives and improve their quality of life.
In this Health Policy Report, the author describes a major pay-for-performance program that is being implemented in the United Kingdom. Additional government payments to family practitioners will be based on the quality of care they deliver. Family practitioners can earn up to 1050 quality bonus points (expected to be worth more than $75,000 in gross income) for performing well on a complex set of indicators that measure the quality of clinical care, organization of the practice, and experience of the patients.
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