HISTOPATHOLOGY OF THE HUMAN RETINA IN RETINITIS-PIGMENTOSA

The term, 'retinitis pigmentosa', refers to a heterogeneous group of i nherited diseases that cause degeneration of rod and cone photorecepto rs in the human retina. Loss of photoreceptor cells is usually followe d by alterations in the retinal pigment epithelium and retinal glia. U ltimately, degenerative changes occur in the inner retinal neurons, bl ood vessels, and optic nerve head. This chapter provides background in formation on the genetics of retinitis pigmentosa and a summary of the histopathologic alterations in human retinas caused by this disease. Detailed information is provided on the effects of the primary disease process on the rod photoreceptors and changes in the other retinal co mponents, all of which are important considerations for understanding and developing therapies for retinitis pigmentosa. (C) 1998 Elsevier S cience Ltd. All rights reserved.