==================================BSR37================================== 37. Retinitis pigmentosa: - Immunohistochemistry - Electron mircroscopy - Gliosis. 1 UI - 86308141 AU - Mukherjee TM ; Dixon BR ; Blumbergs PC ; Swift JG ; Hallpike JF TI - The fine structure of the intramitochondrial crystalloids in mitochondrial myopathy. AB - The fine structure of the intramitochondrial crystalloids found in mitochondrial myopathies was investigated using high angle tilting of ultrathin sections and freeze fracture replicas. Observations show the crystalloids to be composed of 8 nm granules in a unique array. Analysis of the arrangement seen in three mutually perpendicular planes has permitted a model of the crystalloid and its relationship to the mitochondrial membranes to be proposed. The biochemical nature and pathophysiological significance of the crystalloids is still undetermined. MH - Biopsy ; Human ; Intracellular Membranes/ULTRASTRUCTURE ; Kearns Syndrome/ PATHOLOGY ; Microscopy, Electron/METHODS ; Mitochondria, Muscle/ *ULTRASTRUCTURE ; Ophthalmoplegia/PATHOLOGY ; Retinitis Pigmentosa/ PATHOLOGY ; Submitochondrial Particles/ULTRASTRUCTURE SO - J Submicrosc Cytol 1986 Jul;18(3):595-604 2 UI - 86290725 AU - Bull TB ; McCartney AC TI - The ribosome-lamellar complex; is it a normal structure? AB - Ribosome-lamellar complexes were seen in mast cells in the iris of a patient with Usher's syndrome (retinitis pigmentosa associated with deafness) and in the neoplastic cells of an alveolar soft part sarcoma of the thigh. These 'organelles', originally described and associated with haematopoietic malignancy, where they have been described as the hallmark, are being increasingly often described in neoplastic and nonneoplastic cells. We believe that their usefulness in diagnosis should be questioned and the concept of their association with malignancy abandoned. MH - Adult ; Case Report ; Child ; Human ; Iris/ULTRASTRUCTURE ; Male ; Mast Cells/*ULTRASTRUCTURE ; Microscopy, Electron ; Mitochondria/ ULTRASTRUCTURE ; Retinitis Pigmentosa/*PATHOLOGY ; Ribosomes/ *ULTRASTRUCTURE ; Sarcoma/*ULTRASTRUCTURE SO - Ultrastruct Pathol 1986;10(4):363-7 3 UI - 86239467 AU - Albert DM ; Pruett RC ; Craft JL TI - Transmission electron microscopic observations of vitreous abnormalities in retinitis pigmentosa. AB - Ultrastructural studies of six vitreous biopsy specimens obtained during cataract surgery on patients with retinitis pigmentosa showed four types of cells. These were ocular pigment epithelium, uveal melanocytes, retinal astrocytes, and macrophage-like cells. The fibrous astrocytes displayed plump cell bodies, large nuclei, and numerous intracytoplasmic filaments. The pigment epithelial cells and uveal melanocytes were round to cuboidal and were heavily pigmented. Macrophage-like cells demonstrated round cell bodies, inclusions of glycogen, and long processes extending from the cell membrane. Also identified in the vitreous material were loose pigment granules. In contrast, vitreous from the control group showed occasional macrophages and loose pigment. These findings explained the clinical observation of material within the vitreous of patients with retinitis pigmentosa. MH - Adult ; Aged ; Astrocytes/ULTRASTRUCTURE ; Cell Nucleus/PATHOLOGY ; Child ; Child, Preschool ; Collagen/ANALYSIS ; Cytoplasmic Granules/PATHOLOGY ; Cytoskeleton/PATHOLOGY ; Human ; Macrophages/ULTRASTRUCTURE ; Melanins ; Melanocytes/ULTRASTRUCTURE ; Microscopy, Electron ; Middle Age ; Pigment Epithelium of Eye/PATHOLOGY ; Retinitis Pigmentosa/*PATHOLOGY ; Support, Non-U.S. Gov't ; Support, U.S. Gov't, P.H.S. ; Vacuoles/PATHOLOGY ; Vitreous Body/*PATHOLOGY SO - Am J Ophthalmol 1986 Jun 15;101(6):665-72 4 UI - 86217472 AU - Rodrigues MM ; Wiggert B ; T'so MO ; Chader GJ TI - Retinitis pigmentosa: immunohistochemical and biochemical studies of the retina. AB - Several immunohistochemical and biochemical characteristics of retinas from two patients with retinitis pigmentosa (RP) were examined. Histochemically, interphotoreceptor retinoid-binding protein (IRBP) was found in the interphotoreceptor space in small areas of preserved peripheral retina, although enzyme-linked immunosorbent assay suggested that little IRBP was present. Antibodies to glial fibrillary acidic protein and factor VIII-related antigen corroborated retinal gliosis and retinal vessel attenuation, respectively, in the RP cases. Fibronectin showed similar staining in normal and affected tissue. MH - Aged ; Antigens/ANALYSIS ; Case Report ; Enzyme-Linked Immunosorbent Assay ; Factor VIII/ANALYSIS/IMMUNOLOGY ; Female ; Fibronectins/ METABOLISM ; Fundus Oculi ; Glial Fibrillary Acidic Protein/METABOLISM ; Histocytochemistry ; Human ; Immunochemistry ; Male ; Retina/*METABOLISM/ PATHOLOGY ; Retinitis Pigmentosa/*METABOLISM/PATHOLOGY ; Retinol-Binding Proteins/METABOLISM SO - Can J Ophthalmol 1986 Apr;21(3):79-83 5 UI - 86221759 AU - Duvall J ; McKechnie NM ; Lee WR ; Rothery S ; Marshall J TI - Extensive subretinal pigment epithelial deposit in two brothers suffering from dominant retinitis pigmentosa. A histopathological study. AB - The eyes of two brothers with retinitis pigmentosa were removed after death and examined by a variety of techniques, including conventional histology, fluorescence microscopy and both scanning and transmission electron microscopy. Their condition was considered to be of an autosomal dominant type but with some atypical clinical features. The outstanding histological feature in both pairs of eyes was a predominantly acellular deposit of amorphous material situated between the retinal pigment epithelium and Bruch's membrane. This material extended from the disc to beyond the ora serrata. In some regions of the retinae of both brothers, there was a cellular infiltrate into the deposit and this included multinucleate cells. In one brother the deposit was lined externally by a fibrovascular membrane in some few locations. All retinae were degenerate, but all showed preservation of abnormally short and sparse photoreceptor cells in both the peripheral and macular areas. There was only patchy loss of the choriocapillaris, which could have been age-dependent rather than disease-dependent, and the remaining choroidal vessels were patent in all cases. The widespread distribution of the deposit is unusual and suggests that it arises from disordered metabolism of the retinal pigment epithelium. We could not determine whether it was a primary disease process or if it arose as a secondary phenomenon. MH - Case Report ; Electron Probe Microanalysis ; Human ; Male ; Microscopy, Electron ; Microscopy, Electron, Scanning ; Middle Age ; Pedigree ; Pigment Epithelium of Eye/*PATHOLOGY ; Retina/ULTRASTRUCTURE ; Retinitis Pigmentosa/*PATHOLOGY/PHYSIOPATHOLOGY ; Support, Non-U.S. Gov't ; Visual Fields SO - Graefes Arch Clin Exp Ophthalmol 1986;224(3):299-309