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W Bernauer

Publications and source records attributed to W Bernauer.

At least 19 recordsLinked to original sources

[Early clinical diagnosis of acanthamoeba keratitis. A study of 70 eyes].

BACKGROUND: Acanthamoeba keratitis is an uncommon condition which is usually associated with contact lens wear. The use of home made saline and poor hygiene are important risk factors. Early diagnosis is crucial since these cases respond well to medical therapy. The purpose of this paper is to describe and demonstrate early clinical signs. METHOD: Between September 1992 and October 1994, 70 cases of acanthamoeba keratitis, one of them bilateral, were prospectively monitored at Moorfields Eye Hospital in London. A database of all patients was set up and the clinical findings, diagnostic methods, therapeutic interventions and the outcome were recorded. RESULTS: 66 patients (96%) were contact lens wearers, 64 of them (97%) wore soft lenses. The mean interval between first symptoms and correct diagnosis was 42%. The most frequent initial diagnoses were "unclear keratoconjunctivitis" and "herpetic keratitis". Early corneal findings included punctate keratopathy (n = 14; 20%), pseudodendrites (n = 4; 6%), epithelial infiltrates (n = 17; 24%), diffuse or focal sub-epithelial infiltrates (n = 36; 51%) and radial keratoneuritis (n = 5; 7%). Ring infiltrates (n = 13; 18%) and corneal ulceration (n = 13) were late signs. CONCLUSION: When the above corneal findings are observed, particularly in contact lens wearers, the diagnosis of acanthamoeba keratitis should be considered. The diagnosis of "herpetic keratitis" in association with contact lens wear should be encountered with scepticism.

Acanthamoeba Keratitis

Progression of disease in ocular cicatricial pemphigoid.

BACKGROUND: Ocular cicatricial pemphigoid (OCP) is a sight threatening autoimmune disease that can lead to severe conjunctival cicatrisation and keratopathy. It has a variable course and little is known about the factors that determine disease progression. This study analysed the factors that have prognostic significance regarding disease outcome, progression, and keratopathy. METHODS: Sixty six patients with OCP were monitored prospectively at Moorfields Eye Hospital. The influence of ocular features, the systemic disease, and the management were analysed to identify factors that influence the outcomes and disease progression. RESULTS: The mean age at presentation was 67 years; 56% were men. The binocular visual acuities were 6/24 or worse in 25%. Extensive cicatrisation at presentation was common but correlated only weakly with the visual prognosis. Systemic manifestations included lesions of the mouth in 44%, pharynx in 30%, oesophagus in 27%, nose/sinus in 18%, and skin in 17%. There was no association between the ocular and systemic manifestations. Persistent corneal epithelial defects and limbitis occurred in 18% and 32%, respectively, and both were associated with a worse visual prognosis. Systemic immunosuppression was ultimately prescribed in 74%, mainly in patients with advanced stages of conjunctival cicatrisation. Of patients with more than 24 months follow up, progression of cicatrisation occurred in 35% of eyes (16/46) all but one of which were associated with episodes of conjunctival inflammation. CONCLUSIONS: Persistent epithelial defects, limbal inflammation, and ongoing conjunctival inflammation are important factors that lead to keratopathy and visual handicap. These require aggressive management, often with systemic immunosuppressive treatment. Close follow up is required in cases with extensive cicatrisation.

Adult

[Vision disorders in inflammatory-rheumatic diseases].

The association of visual disturbances and rheumatic disease has been known for centuries. This review provides a synopsis of the ocular conditions that are associated with inflammatory rheumatic disease. The major ophthalmic manifestations of the rheumatic diseases include keratoconjunctivitis sicca, ulcerative keratitis, scleritis, uveitis, retinal vascular disease, and neuro-ophthalmic lesions. Each of these ocular conditions is most characteristically associated with a few, but not all, of the rheumatic disorders. Scleritis, for example, is most often seen with rheumatoid arthritis or with vasculitis. Acute anterior uveitis is most often seen with the seronegative spondylarthropathies. Retinal vascular and neuro-ophthalmic lesions are seen with disorders having either a vaso-occlusive component, such as systemic lupus erythematosus, or with one of the vasculitides. Important considerations for a successful collaboration between ophthalmologists and physicians/rheumatologists are discussed.

Adult

[The eye and HIV].

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AIDS-Related Opportunistic Infections

Effect of antiarrhythmics on the release of adenosine in rat hearts with coronary occlusion and reperfusion.

In isolated perfused rat hearts the left coronary artery was occluded for 5 min, with subsequent reperfusion for 20 min. During the reperfusion severe tachyarrhythmias were observed, with ventricular fibrillation occurring in all hearts. Simultaneously, large amounts of adenosine and its degradation products inosine, hypoxanthine, xanthine and uric acid were released into the coronary perfusate. The antiarrhythmics quinidine, lidocaine and gallopamil significantly decreased the release. The effect of quinidine and lidocaine was linked with the antifibrillatory action of these drugs. Also the interruption of fibrillation immediately after its appearance by potassium chloride decreased the release of adenosine and its metabolites in a highly significant way. The effect of gallopamil on the release was independent of an antifibrillatory action. The findings indicate that different kinds of antiarrhythmic drugs can affect the release of nucleosides and oxypurines in hearts with ischaemia and reperfusion.

Adenosine

[Inflammatory cellular infiltration in scleritis].

BACKGROUND: Scleritis can be a destructive disease frequently associated with autoimmune disorders. It is believed that primary vasculitis plays an important role in its pathogenesis, but little is known about the cellular effector mechanisms. The purpose of this study was to analyze the inflammatory cellular infiltrate in scleritis. PATIENTS AND METHODS: Two enucleated eyes were studied. In one patient, enucleation was done after perforation occurred in anterior necrotizing scleritis and, in the other, after the misdiagnosis of posterior scleritis as an intraocular tumor. Morphological criteria and immunohistochemical methods were used to characterize the inflammatory cellular infiltrate. RESULTS: The cells infiltrating the scleral fibers in the enucleated eyes consisted predominantly of T cells in both cases; many of the T cells were CD4-positive. Clusters of B cells were found in perivascular areas. In circumscribed areas neutrophils, macrophages, and plasma cells were part of the scleral infiltrate. Signs of a granulomatous process with activated macrophages (epitheloid and giant cells) were present in necrotizing scleritis. Expression of major histocompatibility class II molecules (MHC II) was found on lymphocytes and rarely on macrophages. Signs of primary vasculitis were not found in any of the specimens. CONCLUSION: The cellular infiltrate in scleritis shows, at least at certain stages, features compatible with a T-cell mediated (autoimmune) disorder, which has major therapeutic implications.

Adult

Cell death and disposal in retinoblastoma: an electron microscopic study.

BACKGROUND: Tumor necrosis and cell death are common features of retinoblastoma. In non-malignant retinal cells after ischemia, as well as in many non-retinal tumors, cell death occurs in at least two ways. We investigated whether similar patterns of cell death could be demonstrated in retinoblastoma cells. METHODS: Nine globes with retinoblastoma from eight patients were studied. Paraffin sections stained with HE or the Feulgen method were examined by light microscopy. Several samples from each tumor were selected for electron microscopic study. RESULTS: Ultrastructurally, two main types of cell death were identified. Type I was characterized by progressive lysis of the cytoplasm and karyoplasm. Nuclear chromatin either dissolved or was transformed into compact clumps becoming extracellular dense bodies. Phagocytosis of cell remnants by neighboring tumor cells, or occasional macrophages, was common. Type II was characterized by progressive condensation and shrinkage of the cytoplasm and nucleus. Type II was subdivided in two forms distinguished mainly by characteristic patchy vs crescentic chromatin condensation. Small parts of condensed cytoplasm were engulfed by neighboring tumor cells. Compact cell remnants then underwent either phagocytosis by neighboring retinoblastoma cells or progressive intercellular disaggregation. CONCLUSION: Retinoblastoma cells may undergo at least two types of cell death. Type I fits the definition of necrosis, while both forms of type II exhibited several features consistent with apoptosis. The types of cell death observed in retinoblastoma exhibited similarities to patterns observed in ischemic retina, as well as in other malignant tumors. Type II cell death (apoptosis) may play a role in limiting tumor growth.

Apoptosis

The value of biopsies in the evaluation of chronic progressive conjunctival cicatrisation.

BACKGROUND: Chronic progressive conjunctival cicatrisation is poorly understood, and therapy of this condition remains difficult. This study assessed the value of immunohistochemical investigations in the evaluation of patients who present with chronic cicatrising conjunctivitis similar to cicatricial pemphigoid (CP). METHODS: Bulbar conjunctival biopsies from 36 patients with acute (n = 5), subacute (n = 13) and chronic (n = 18) ocular disease were studied. The biopsy was retaken in 7 patients to evaluate the present immunological findings in comparison with a biopsy more then 5 years ago. All the specimens were investigated for the presence of immunoglobulins and complement at the epithelial basement membrane, and the phenotype of the inflammatory cellular infiltrate was analysed. Twenty-nine patients were evaluated for the presence of circulating IgG-anti-basement membrane zone antibodies. RESULTS: CP was confirmed by immunoglobulins and/or complement deposition at the epithelial basement membrane in 11 patients (31%). IgA was found to be the most frequent deposit. Eleven CP patients, mainly those with active or "burnt-out" disease, showed absence of immunoglobulins and/or complement at the conjunctival basement membrane. In 14 of 36 patients, conjunctival cicatrisation was subsequently felt to be caused by conditions other than CP. The cellular phenotype in the subepithelial conjunctiva was unspecific, but in CP the disease activity was reflected by the number of neutrophils and macrophages. Circulating IgG antibodies were found in none of the patients' serum. CONCLUSION: Immunoglobulin and/or complement deposition at the epithelial basement membrane confirms the diagnosis of mucous membrane pemphigoid. Their absence, however, does not rule it out and is a frequent feature in very active conjunctival disease or after immunosuppressive treatment. The analysis of the cellular phenotype in mucous membrane pemphigoid may be useful in the assessment of disease activity but does not help in determining the underlying disease process causing the cicatrising conjunctivitis.

Acute Disease

Cryotherapy for trichiasis in ocular cicatricial pemphigoid.

Trichiasis is a common and potentially sight threatening complication of ocular cicatricial pemphigoid and is usually secondary to cicatricial entropion. This study aimed to assess the success and complications of eyelid cryotherapy for trichiasis in a group of patients with long term follow up. The case records of all patients with ocular cicatricial pemphigoid that attended the external disease clinic at Moorfields Eye Hospital from 1980 to 1992 were reviewed. Each eyelid was divided into three horizontal zones. Cryotherapy was delivered with an Amoilette cryoprobe for approximately 30 seconds. Failure of the cryotherapy was defined as a regrowth of the eyelashes within the treated zone that either required epilation for symptom control or caused keratopathy. Ninety two lid zones were treated, involving 25 lids of 12 patients. The cumulative chance of success decreased rapidly to 40% over the first year. Thereafter, the chance of success declined slowly to 34% at 4 years. Complications included lid notching (n = 2), tarsal atrophy (n = 1), altered lid contour (n = 1), and temporarily raised intraocular pressure (n = 1). All patients had quiescent disease at the time of the cryotherapy and no patients showed increased conjunctival disease activity after treatment. Six patients were taking systemic immunosuppression medication. When ocular cicatricial pemphigoid is quiescent, lid cryotherapy has an acceptable complication rate. The major reason for recurrence of the trichiasis was attributed to inadequate follicle freezing.

Aged

Cells perpetuating the inflammatory response in scleritis.

Scleritis can be a destructive disease frequently associated with autoimmune disorders. It is believed that primary vasculitis plays an important role in its pathogenesis, but little is known about the cellular effector mechanisms. The purpose of this study was to analyse the inflammatory cellular infiltrate in scleritis. Six episcleral biopsies and two enucleated eyes were studied. The episcleral biopsies were taken from patients with nodular scleritis. In one patient enucleation was done after perforation in anterior necrotising scleritis and, in the other after misdiagnosis of posterior scleritis as intraocular tumour. Morphological criteria and immunohistochemical methods were used to characterise the inflammatory cellular infiltrate. The inflammatory cells infiltrating the episcleral tissue were mainly T lymphocytes and macrophages. There was a predominance of CD4 positive cells, but only few lymphocytes were activated (expressed IL-2 receptor). The cells infiltrating the scleral fibres in the enucleated eyes consisted in both cases predominantly of T cells. Clusters of B cells were found in perivascular areas. In circumscribed areas neutrophils, macrophages, and plasma cells were part of the scleral infiltrate. Signs of a granulomatous process with activated macrophages (epithelioid and giant cells) were present in necrotising scleritis. Expression of major histocompatibility class II molecules (MHC II) was found on lymphocytes and rarely on macrophages. Signs of primary vasculitis were not found in any of the specimens. The cellular infiltrate in scleritis shows, at least at certain stages, features compatible with a T cell mediated (autoimmune) disorder, which may have major therapeutic implications.

Adult

[Immunohistologic findings in chronic cicatricial conjunctivitis].

BACKGROUND: Chronic progressive conjunctival cicatrization is found in some mucocutaneous disorders (cicatricial pemphigoid, linear IgA disease) and after long-term treatment with certain topical medications (pseudo-pemphigoid). Little is known about the mechanisms of conjunctival shrinkage, and therapy for these conditions remains difficult. OBJECTIVES: To elucidate the immune mechanisms and assess the main factors involved in conjunctival scar tissue formation in patients with chronic progressive conjunctival cicatrization. PATIENTS AND METHOD: We examined 14 bulbar conjunctival biopsy specimens from patients with chronic progressive conjunctival cicatrization (8 with benign mucous membrane pemphigoid confirmed by biopsy, 4 with the ocular features of benign mucous membrane pemphigoid and 2 with pseudopemphigoid) and 10 biopsies from matched healthy individuals: 1.5 mm sections of glycol methacrylate embedded tissue were analysed with the aid of a panel of monoclonal antibodies. MAIN RESULTS: Cell counts in the subepithelial substantia propria showed a marked increase in T-cells over normal controls (up to 30-fold). Among the T-cell subsets, there were more CD8 than CD4-positive cells observed. Only about 5% of the T-cells were activated (IL-2 receptor-positive). Macrophages were--as in normal tissues--the second most predominant cell. The absolute number was 2-3 times as high in diseased conjunctiva as in controls. There was increased expression of MHC II molecules on macrophages, lymphocytes und fibroblasts. The numbers of B-cells and NK were not increased. CONCLUSIONS: The analysis of the cellular infiltrate showed nonspecific immunopathological characteristics. Thus, the cellular infiltrate gives no explanation for the progressive cicatrization. There is evidence that soluble factors, especially fibrogenic cytokines, play an important role.

B-Lymphocytes

Cytokines in the conjunctiva of acute and chronic mucous membrane pemphigoid: an immunohistochemical analysis.

The aim of this study was to evaluate the potential role of certain soluble factors in conjunctival scar tissue formation of pemphigoid patients. Epibulbar conjunctival biopsy specimens were taken from patients with acute ulcerative (n = 4), subacute (n = 8) and chronic (n = 8) mucous membrane pemphigoid and from twelve age-matched healthy individuals. The tissues were embedded in glycol methacrylate and analysed by immunohistochemical methods. Interleukin-2 (IL-2), interferon-gamma, transforming growth factor-beta (TGF-beta), platelet-derived growth factor (PDGF), basic fibroblast growth factor (bFGF), tumour necrosis factor-alpha and proliferating cells (as identified with the antibody Ki-67) were found in both pemphigoid patients and normal controls. Interleukin-4 was not found with this method in either normal or diseased conjunctiva. Significant differences between normal and diseased conjunctiva were found for TGF-beta and for proliferating cells, which were both increased in the acute disease group. More intense staining was found in the subacute disease group for IL-2, bFGF and PDGF. Our findings showed that a variety of cytokines were present in normal and diseased bulbar conjunctiva. Acute conjunctival disease in mucous membrane pemphigoid may indicate active scar tissue formation, implied by an increase in TGF-beta and the presence of proliferating fibroblasts.

Acute Disease

The conjunctiva in acute and chronic mucous membrane pemphigoid. An immunohistochemical analysis.

BACKGROUND: The mechanism of chronic progressive conjunctival cicatrization in mucous membrane pemphigoid is not well understood, and current therapy is often of limited use. Rapid progression of cicatrization follows exacerbations of clinical inflammation, and the investigation of immune mechanisms related to disease activity may provide a clue for more effective therapeutic strategies. METHODS: The authors undertook an immunohistochemical study, using monoclonal and polyclonal antibodies in glycol methacrylate-embedded tissues, of epibulbar conjunctival biopsy specimens obtained from 20 patients with ocular cicatricial pemphigoid and from 12 matched healthy controls. The study patients were classified according to the ocular disease activity as acute ulcerative (n = 4), subacute (n = 8), and chronic (n = 8). RESULTS: The composition of the subepithelial cellular infiltrate varied with disease activity. Acute disease was characterized by an abundance of macrophages and neutrophils. The number of T lymphocytes was significantly raised in all the disease groups, but were most marked in subacute disease. Of the T-cell subsets, there were more CD8- than CD4-positive cells observed, except in acute disease where there were equal numbers. Only approximately 5% of the T cells in all disease groups were activated as demonstrated by expression of interleukin-2 receptor. There was increased expression of major histocompatibility complex class II (MHC II) molecules on macrophages, fibroblasts, and other cells in all the groups. The number of B cells and natural killer cells was not increased. Staining for the fibrogenic cytokines, transforming growth factor-beta (TGF-beta), platelet-derived growth factor, and basic fibroblast growth factor was found in both pemphigoid patients and control persons, but the intensity of TGF-beta staining was significantly greater in acute disease. CONCLUSIONS: The composition of the cellular infiltrate in the bulbar conjunctiva depends on clinical disease activity. The numbers of neutrophils and macrophages seem to reflect clinical disease activity. Fibrogenic cytokines, especially TGF-beta, may play an important role in the formation of conjunctival scar tissue.

Acute Disease

Chronic progressive conjunctival cicatrisation.

The aim of this review is to demonstrate the spectrum of conditions encompassed by the term 'chronic progressive cicatrising conjunctivitis', to discuss mechanisms of conjunctival scar tissue formation and to describe the sequelae and therapeutic options in this potentially blinding condition. Chronic progressive cicatrising conjunctivitis is found in association with some mucocutaneous disorders (cicatricial pemphigoid, linear IgA disease), as part of paraneoplastic syndromes and after long-term treatment with certain systemic and topical medications (pseudo-pemphigoid). Recent studies on the conjunctiva of pemphigoid patients indicate that macrophages may play a pivotal role in chronic progressive conjunctival cicatrisation. They mediate the transition from inflammation to scar tissue by secretion of fibrogenic cytokines. There is evidence that similar mechanisms are involved in the other fibrosing conjunctival disorders. Sequelae of chronic conjunctival cicatrisation include the obstruction of lacrimal and meibomian glands, tear film alterations, trichiasis, keratopathy and blindness. Present possibilities and future options for the treatment of this condition are discussed.

Chronic Disease

Failure to control AIDS-related CMV-retinitis with intravenous ganciclovir.

Between January 1988 and May 1991 intravenous ganciclovir (GCV) treatment was administered to eight male AIDS-patients with unilateral cytomegalovirus (CMV)-retinitis. Despite of continuous therapy with at least the recommended dose of GCV, three patients developed slowly progressive CMV-retinitis in the fellow eye after 4 to 13 months. The progression could not be stopped by GCV and thus bilateral blindness resulted after 12 to 22 months. The number of CD4-lymphocytes in the blood was reduced in all patients, but particularly in patients with progressive disease. Treatment failure was partly related to the duration of CMV-retinitis and partly to the degree of immunodeficiency. Intravenous treatment with GCV alone can not stop the progression of CMV-retinitis in long-term survivors and in those with advanced immunodeficiency.

AIDS-Related Opportunistic Infections

[Morphologic aspects of therapy-resistant cytomegalovirus retinitis].

Intravenous ganciclovir treatment was performed in eight male AIDS patients with primary unilateral CMV-retinitis. Three patients developed slowly progressive CMV-retinitis in the fellow eye despite adequate dose of ganciclovir. These different CMV-manifestations are shown in a sequence of fundus pictures. Three types of CMV-lesions were observed in connection with this study. Untreated central lesions showed the aspect of crumbled cheese and ketchup. Untreated lesions in the peripherie were yellowish-white, granular, "dry" and showed in most cases no haemorrhages. Lesions appearing during treatment showed initially "dry" white opaque subretinal areas, turning later on to the typical aspect of untreated lesions. The progression could not be stopped by highdose ganciclovir i.v. and thus bilateral blindness resulted after 12 to 22 months. The level of CD4-lymphocytes in the blood was diminished in all patients, but much more in patients with progressive disease.

Acquired Immunodeficiency Syndrome

Bietti's corneal-retinal dystrophy. A 16-year progression.

Bietti's crystalline corneal-retinal dystrophy is characterized by deposits of crystals in the marginal cornea and the paracentral and peripapillary retina. To date, only three cases with long term follow-up have been reported. The case of another patient, who has been observed for 16 years, is reported here. The most striking morphologic feature during the period of examination was the diminution of the retinal crystals, an optical phenomenon that appears to be due to the advanced atrophy of the retinal pigment epithelium. The progressive course is documented photographically.

Adult