This paper classifies the abnormalities of the anterior chamber cleavage syndrome (mesodermal dysgenesis of the iris and cornea). The anatomic findings are arranged in a tabular stepladder fashion which builds from simple to more complex combinations, most of which have been previously known by eponyms. There are three groups of anomalies: 1) peripheral, 2) central, and 3) combinations of the two. 1) The peripheral anomalies consist of a prominent Schwalbe's ring, iris strands to Schwalbe's ring, and hypoplasia of the anterior iris stroma. Developmental glaucoma is commonly present. 2) The essential feature of the central anomalies is a defect in the corneal endothelium and Descemet's membrane with an overlying corneal opacity. Additional components include central iridocorneal adhesions, keratolenticular approximation with cataract, and scleralizaiton of the cornea. Chorioretinal anomalies, developmental glaucoma, and systemic malformations may be present. 3) Central and peripheral combinations may exist in the same eye, in both eyes of the same patient, or within the same family.

Abnormalities, Multiple↗

PubMedDate not supplied

Corneal dystrophies. I. Dystrophies of the epithelium, Bowman's layer and stroma.

Most corneal dystrophies are autosomal dominant, bilateral disorders that primarily affect one layer of an otherwise normal cornea, progress slowly after their appearance in the first or second decade, and are not associated with a systemic disease. Epithelial basement membrane dystrophy and Fuchs' endothelial dystrophy are seen commonly by the general ophthalmologist; fleck, posterior polymorphous, granular or lattice dystrophies are seen more rarely, and others may never be seen in general office practice. While the distinctive clinical appearance of most corneal dystrophies allows accurate diagnosis, the integration of slitlamp findings with histopathologic and biochemical findings aids in the understanding of the clinical observations and provides a more rational basis for therapy. Transmission electtron microscopy is the most accurate method of histopathologic diagnosis. Epithelial dystrophies usually manifest intraepithelial cysts and abnormal basement membrane. In stromal dystrophies, an abnormal substance accumulates within the keratocytes or among the collagen fibrils; it may be an excess normal metabolite (like glycosaminoglycans in macular dystrophy), a material not usually present (like amyloid in lattice dystrophy), or a substance of unknown composition (like hyaline in granular dystrophy). Each dystrophy is illustrated with a composite drawing. Endothelial dystrophies will be reviewed separately in a second article.

Adolescent↗

PubMedDate not supplied

Corneal dystrophies. II. Endothelial dystrophies.

In general, endothelial dystrophies present three types of clinical manifestations: 1) production of collagenous tissue posterior to Descemet's membrane which appears as cornea guttata, polymorphic excrescences or gray sheets; 2) a disrupted endothelial mosaic in specular reflection; and 3) corneal edema as a reflection of decreased endothelial barrier and pump functions. In this review, the authors discuss three endothelial dystrophies -- Fuchs', posterior polymorphous and congenital hereditary. They describe the clinical, histopathologic and biochemical features, and illustrate each dystrophy with a composite drawing. Dystrophies of the epithelium, Bowman's layer, and stroma were reviewed separately in the September-October 1978 issue of this journal.

Adult↗

PubMedDate not supplied

Patterns of pathologic response in the cornea.

The cornea, like most tissues, manifests only a limited number of responses to the wide variety of diseases and insults that assault it. To facilitate a systematic understanding of these responses, we have divided the cornea into four zones: the epithelium; the epithelial basement membrane, Bowman's layer and anterior stroma; the stroma; and the endothelium and Descemet's membrane. We describe six pathologic processes or responses in each zone: defects; fibrosis and vascularization; edema and cysts; inflammation and immune response; deposits; and proliferation. We illustrate this paradigm with a chart that contains examples of each pathologic process in each zone.

Basement Membrane↗

PubMedDate not supplied

Failure of argon laser to halt cytomegalovirus retinitis.

This report describes the experience with Argon laser used in an attempt to slow the advance of CMV retinitis in two eyes of two patients with AIDS, who were not able to be treated with antiviral chemotherapy. The treatment was performed to create a retinal scar to attempt to slow cell-to-cell spread of the virus through retinal tissue. In both patients, treatment was not successful in preventing spread of the CMV infection. Foci of retinitis, beyond the treated areas, became apparent within 2 weeks of treatment. Autopsy histopathology confirmed the diagnosis of CMV retinitis in both patients and necrosis due to CMV retinitis was seen in laser treated retina, and on both sides of laser treated retina.

Acquired Immunodeficiency Syndrome↗

PubMedDate not supplied

Cellular immune response of humans to the circumsporozoite protein of Plasmodium vivax.

The cellular immune response to the circumsporozoite (CS) protein of Plasmodium vivax of individuals from malaria-endemic areas of Brazil was studied. We examined the in vitro proliferative response of the peripheral blood mononuclear cells (PBMC) of 22 individuals when stimulated with a CS recombinant protein (rPvCS-2) and two other synthetic peptides based on the sequence of the P. vivax CS protein. Seven of the individuals from malaria-endemic area displayed an antigen-specific in vitro proliferative response to the recombinant protein PvCS-2 and one out of 6, proliferative response to the peptide 308-320. In contrast, none of the individuals displayed a proliferative response when stimulated with the D/A peptide which represent some of the repeated units present in this CS protein. Our study, therefore, provides evidence for the presence, within the major surface antigen of P. vivax sporozoites, of epitopes capable to induce proliferation of human PBMC.

Adult↗

PubMedDate not supplied

C-reactive protein in human lattice corneal dystrophy.

Lattice corneal dystrophy (LCD), an autosomal dominantly inherited disease, is characterized by a branching network of subepithelial and stromal amyloid deposits (1). Due to their small size and close association with stromal components and epithelial cells, their chemical composition is as yet undetermined. Amyloid deposits in other types of diseases have been found to contain amyloid P protein (AP). Serum amyloid P component (SAP) and C-reactive protein (CRP) resemble each other in molecular structure and amino acid sequence, but appear to be antigenically distinct (2-6). A humoral mediator most likely stimulates CRP release by hepatocytes and could be related to Interleukin-I synthesis from macrophages (4-6). Rabbit corneal epithelial cells also produced an Interleukin-I-like activity and contain a thymocyte activating cytokine (7). In this study, corneas from normal controls, primary LCD and recurrent LCD were fixed in formalin with lmM CaCl2 and tested with antibodies to CRP, AP and AA (non-immunoamyloid), using the immunoperoxidase technique. The stroma of LCD and normal corneas did not stain with antibodies to AP, AA or CRP. However, we now report that antibodies to CRP show immunospecific binding to the corneal epithelium in primary and recurrent LCD.

C-Reactive Protein↗

PubMedDate not supplied

Congenital fibrosis of the extraocular muscles.

Congenital fibrosis of the extraocular muscles is characterized by the replacement of normal contractile muscle tissue by fibrous tissue or fibrous bands in varying degrees. The clinical entities which result from the fibrous replacement can be classified under the following headings: general fibrosis syndrome, congenital fibrosis of the inferior rectus muscle with blepharoptosis strabismus fixus, vertical retraction syndrome and congenital unilateral fibrosis, enophthalmos, and blepharoptosis. Genetic factors may or may not be apparent. One pedigree with general fibrosis syndrome was traced through five generations. Light and electron microscopy demonstrated replacement of normal muscles by collagen and dense fibrous tissue with occasional areas of degenerated skeletal muscle. The surgical management attempts to achieve some functional readjustment of the ocular and lid position as well as the abnormal head posture. The surgical results were considered satisfactory when compared with the original position of the eyes and the backward head tilt.

Adolescent↗