PubMed Health⌕ Search

Biomedical subjects

D A Jabs

Publications and source records attributed to D A Jabs.

107 records · Page 6Linked to original sources

Treatment of cytomegalovirus retinitis with ganciclovir.

Eighteen immunocompromised patients with cytomegalovirus (CMV) retinitis were treated with ganciclovir, an investigational antiviral drug. CMV retinitis in association with acquired immune deficiency syndrome (AIDS) developed in 17 patients; CMV retinitis developed in one patient after cardiac transplantation. Fourteen patients responded to ganciclovir treatment with improvement in CMV retinitis. In 11 patients, the response was classified as complete; in three patients, the response was partial. Continued improvement in the retinitis was often seen while the patient was on maintenance treatment. Maintenance ganciclovir therapy was required; relapse occurred in five of seven patients in whom ganciclovir treatment was interrupted. The major limiting toxic side effect of ganciclovir was neutropenia, which necessitated temporary discontinuation of ganciclovir in five patients but was reversible in all cases. Ganciclovir appears to be an effective therapy for CMV retinitis, but chronic maintenance therapy is required.

Acquired Immunodeficiency Syndrome↗

Bullous pemphigoid autoantibodies are markers of corneal epithelial hemidesmosomes.

Sera from patients with bullous pemphigoid (BP) contain autoantibodies that bind to the BP antigen, which is a component of the epithelial-stromal junction of the cornea. Previous studies, employing direct immunoelectron microscopy (IEM) on perilesional skin of patients have localized the BP antigen to the lamina lucida. On this basis, studies of corneal epithelial-stromal adhesion and wound healing have employed BP antigen as a marker of the lamina lucida of the corneal basement membrane zone (BMZ). The authors used indirect IEM with BP autoantibodies on frozen sections of cornea and found that the majority of the BP antigen is intracellular and is closely associated with the corneal epithelial hemidesmosome. Only a small amount of BP antigen appears to be extracellular, limited to the portion of the lamina lucida directly beneath individual hemidesmosomes. When rabbit corneal epithelium is extracted and analyzed by Western immunoblotting, BP autoantibodies recognize two polypeptides of molecular weights of 240 and 180 kilodaltons, which comigrate with BP antigens extracted from epidermis. BP autoantibodies are a specific marker of corneal epithelial hemidesmosomes and can be used as a probe to identify and study the role of hemidesmosomes in epithelial-stromal adhesion.

Animals↗

Presumed varicella zoster retinitis in immunocompromised patients.

The acute retinal necrosis (ARN) syndrome is a morphologically defined necrotizing retinitis, occurring in apparently otherwise healthy patients. It has been shown that the varicella zoster virus is at least one cause of the ARN syndrome; treatment with acyclovir has proven to be effective for the infectious component of ARN. We report three immunocompromised patients who developed cutaneous herpes zoster and a necrotizing retinitis that was morphologically similar to the ARN syndrome. All three patients responded promptly to treatment with acyclovir, an agent highly effective against varicella zoster and herpes simplex viruses, but ineffective in the treatment of cytomegalovirus. While cytomegalovirus retinitis is more common in immunocompromised patients, these patients may occasionally develop an ARN-like retinitis, presumably as a result of the varicella zoster virus, which responds to treatment with acyclovir.

Acyclovir↗

Ocular involvement in chronic sarcoidosis.

Although spontaneous remissions often occur in sarcoidosis, chronic persistent disabling disease is also observed. Of a series of 183 patients with chronic sarcoidosis, 47 (26%) had ophthalmic involvement. In this series, chronic sarcoid was defined as disease for a minimum of five years. Patients were followed primarily for their systemic disease for a mean of 13 years. Uveitis developed in 35 patients (19%) and was an early manifestation in 32 (91%). The course of the ocular disease did not necessarily parallel that of the systemic disease. Despite the chronic nature of the underlying disease, the anterior uveitis did not pursue a chronic course in 15 of 33 patients (45%) and was generally characterized by a single episode at the onset of disease. Chronic uveitis and secondary glaucoma were poor prognostic signs, as eight of 11 patients with uveitis and glaucoma suffered severe visual loss.

Blindness↗

Severe retinal vaso-occlusive disease in systemic lupus erythematous.

Retinopathy in systemic lupus erythematosus generally consists of cotton-wool spots with or without intraretinal hemorrhages. Although rare, a more severe retinal vaso-occlusive disease, termed retinal vasculitis, has been described. We report data from 11 patients with SLE and severe retinal vaso-occlusive disease. Visual outcome was often poor, with 55% of the involved eyes suffering visual loss, frequently to an acuity of worse than 20/200. The systemic feature significantly associated with severe retinal vascular disease was central nervous system involvement (CNS lupus), present in eight (73%) of the 11 patients vs an overall prevalence of 37%. The association of CNS lupus with severe retinal vaso-occlusive disease probably reflects a similar pathogenetic mechanism and microangiopathy.

Adult↗

Optic neuropathy in systemic lupus erythematosus.

We report data from seven cases of optic neuropathy in systemic lupus erythematosus. The visual outcome varied, but improvement occasionally occurred following treatment with corticosteroids. The clinical picture was variable and could present as acute retrobulbar optic neuritis, ischemic optic neuropathy, or slowly progressive visual loss. Visual evoked response testing revealed increased latency in two patients and loss of amplitude in two others; a fifth patient had both findings in the involved eye. Despite the variable presentation, the probable pathogenesis in all cases is vaso-occlusive disease in small vessels of the optic nerves.

Adult↗

Familial abnormalities of lymphocyte function in a large Sjögren's syndrome kindred.

A large kindred (32 members), whose proband had primary Sjögren's syndrome, was investigated to ascertain whether abnormalities of lymphocyte function were present in family members of patients with Sjögren's syndrome. Ten members of the kindred with autoimmune diseases, 17 blood relatives, 5 spouses, and 32 matched controls were studied. Concanavalin A induced suppression of pokeweed mitogen stimulated immunoglobulin synthesis was decreased in patients and blood relatives both with and without autoimmune diseases. Elevations of the mean T4/T8 ratio, due to decreased numbers of suppressor/cytotoxic lymphocytes, were present in both the disease group and the blood relatives. These abnormalities occurred independently of HLA and were not necessarily associated with autoantibodies.

Autoimmune Diseases↗

Familial granulomatous synovitis, uveitis, and cranial neuropathies.

A family is presented that had what is believed to be a previously undescribed syndrome of granulomatous synovitis, bilateral recurrent uveitis, and cranial neuropathies. Affected members included the proband, his brother, father, and probably the decreased paternal grandmother. Disease onset was in childhood. Each had symmetric, boggy polysynovitis of the hands and wrists, resulting in nearly identical boutonniere deformities. Hand radiography in the proband and his brother revealed no erosions or joint destruction despite more than 20 years of disease. Synovectomy specimens in the proband and his brother showed granulomatous inflammation with giant cells. Recurrent, nongranulomatous, acute iridocyclitis with visual impairment afflicted the proband, brother, and father. Apparently corticosteroid-responsive bilateral neurosensory hearing loss occurred in the proband, and a transient sixth cranial nerve palsy in his brother. All members of the family were antinuclear antibody-, rheumatoid factor-, and HLA-B27-negative. Serum angiotensin-converting enzyme levels were within normal limits in all family members. The inheritance pattern of this syndrome is most consistent with an autosomal dominant mode.

Acute Disease↗

Ocular inflammation in autoimmune MRL/Mp mice.

Congenic mice of the MRL/Mp strain spontaneously develop an autoimmune connective tissue disease that shares immunologic and histopathologic features with the human disorders systemic lupus erythematosus, rheumatoid arthritis, and systemic vasculitis. The autoimmune disorder in these mice is markedly accelerated by the recessive gene lpr. Older MRL/Mp-lpr/lpr mice develop significant inflammatory ocular disease, including choroiditis, scleritis, and orbital vasculitis. Animals of both the MRL/Mp-+/+ and MRL/Mp-lpr/lpr substrains develop lacrimal gland inflammatory infiltrates. The MRL/Mp mouse provides a potential model for ocular inflammatory disease and for Sjögren's syndrome.

Animals↗

Microvascular aspects of acquired immune deficiency syndrome retinopathy.

Twelve of 13 patients with the acquired immune deficiency syndrome exhibited ophthalmoscopically detectable retinal findings, including cotton-wool spots, hemorrhages, Roth spots, and microvascular changes. When we used fluorescein angiography, we detected focal nonperfusion and microvascular changes in all 13 of our patients. Most patients had no visual complaints unless they had cytomegalovirus involvement of the optic papilla or the central retina. One patient lost central visual acuity from loss of perfusion in a portion of his perifoveal capillary net. Histopathologic studies demonstrated the loss of retinal capillary cells and focal occlusions of small vessels. Retinal vessel walls were also thickened with PAS-positive material.

Acquired Immunodeficiency Syndrome↗

The eye in bone marrow transplantation. I. Clinical study.

A prospective study of 45 patients undergoing bone marrow transplantation included full ocular examination and pretransplant and posttransplant conjunctival biopsies. The major finding was the development of a severe keratoconjunctivitis sicca syndrome that developed in 20 of the 45 patients and closely correlated with the occurrence of acute graft-v-host disease (GVHD). An acute pseudomembranous conjunctivitis in the fulminant stage of acute GVHD developed in six patients. A further ten patients made up a retrospective assessment of bone marrow transplantation and confirmed these clinical findings.

Adolescent↗

The eye in bone marrow transplantation. II. Histopathology.

The major histopathologic changes seen in the eyes of patients undergoing bone marrow transplantation involve the conjunctiva, cornea, choroid, and lacrimal gland. The major finding in the conjunctiva is keratinization. The major findings in the cornea are epithelial thinning and keratinization. Keratinization of the conjunctiva and cornea is associated with graft-v-host disease (GVHD) and may be either a primary manifestation of GVHD or be secondary to the "dry eye" syndrome that develops in patients with GVHD. Corneal epithelial thinning is probably secondary to the chemotherapy used in the preparative regimen. An unusual histiocytelike infiltrate is present in the choroid and appears to be associated with GVHD. In acute GVHD, lacrimal gland stasis occurs and is the probable cause of the dry eyes seen in these patients.

Adolescent↗

Ischaemic optic neuropathy with painful ophthalmoplegia in diabetes mellitus.

Two patients with mild, adult-onset diabetes mellitus developed a painful ophthalmoplegia and ipsilateral optic neuropathy that was relatively unresponsive to steroids. Histopathological study of the optic nerve of one patient revealed an extensive ischaemic infarct. There was ultimate recovery from the cranial nerve palsies in both patients and the optic neuropathy in one patient. Ischaemic polyneuropathy involving the cranial nerves may cause an orbital apex/cavernous sinus syndrome in patients with diabetes mellitus.

Diabetic Neuropathies↗

Leukemic iris infiltration.

Three patients with acute lymphoblastic leukemia and leukemic infiltration of the iris are presented. The clinical features, diagnostic techniques, and treatment of this condition are described.

Acute Disease↗

Cytomegalovirus retinitis in AIDS: natural history, diagnosis, and treatment.

Cytomegalovirus retinitis is the most common intraocular infection and the leading cause of blindness in patients with AIDS. Diagnosis is made on clinical grounds; a variety of other infectious and neoplastic retinitides should be considered in the differential diagnosis. Treatment with intravenous ganciclovir or foscarnet has been proven effective, but late complications of relapse, viral resistance, retinal detachment, and drug toxicity remain problematic. Approved and investigational drugs for the treatment of CMV retinitis are limited by their virostatic, rather than virocidal, properties. Because the efficacies of ganciclovir and foscarnet are similar, the choice of therapy should be based on systemic considerations such as drug toxicity and patient survival. The cost, toxicity, and limited efficacy of currently available therapy of CMV retinitis make the need for an effective prophylactic drug even more important.

AIDS-Related Opportunistic Infections↗