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[Cogan I syndrome: clinical aspects, therapy and prognosis].

BACKGROUND: Cogan I syndrome is a rare, inflammatory, systemic disease that is typically characterized by severe audiovestibular dysfunction and various inflammatory eye changes. PATIENTS AND METHODS: Between 1978 and 1996 ten patients with Cogan I syndrome were treated at the eye hospital, ENT clinic and medical clinic of the University of Tübingen. Retrospectively, the clinical picture, course of the disease and outcome were investigated by reviewing the charts of these patients. RESULTS: Patient ages ranged from 18 to 78 years (mean 37 years) at the time of disease manifestation. Eight patients were female. Inflammatory ocular changes were observed in nine patients in both eyes. Uveitis was found in six patients, keratitis in five, episcleritis in three and scleritis in two patients. Both patients with scleritis showed a severe, partially sight-threatening course of the disease despite immunosuppressive therapy. Severe hearing loss was seen in all patients. Further manifestations of Cogan's syndrome included pericarditis associated with arthritis, and polyserositis in one patient, and fibromyalgia in two patients. CONCLUSION: In Cogan I syndrome, typically both eyes are affected by the disease process. Various ocular inflammatory changes may occur including uveitis, scleritis, keratitis and episcleritis. The visual prognosis is mostly good, although severe sight-threatening scleritis may occur.

Adolescent↗

Folk medicine in Tonga. A study of the use of herbal medicines for obstetric and gynaecological conditions and disorders.

We report on a study of the role of traditional medicinal practices in two communities in the South Pacific Kingdom of Tonga, with particular emphasis on obstetric and gynaecological complaints of women and the traditional practitioners consulted by them. Data on the nature and frequency of obstetric and gynaecological complaints, patterns of consultations and preferences for the type of practitioner, Western or traditional, were collected from housewives. The nature, range and extent of expertise possessed by traditional practitioners were also assessed. The results indicate that reliance on traditional medicine is rapidly declining in favour of Western medicine. This seems to be due to urbanisation, increased acceptance of Western values and a lack of availability of traditional practitioners in sufficient numbers. However, self-administration of herbal remedies was still common, especially in rural areas, and might even be on the increase. The practitioners treated a variety of obstetric and gynaecological complaints using 60 plant species which were collected and identified.

Back Pain↗

Alpha-1-antitrypsin phenotypes and HLA-B27 typing in uveitis patients in southeast Iran.

OBJECTIVES: Uveitis is an eye disease that affects humans worldwide. Inflammation of the uveal tract is termed uveitis. Alpha-1-antitrypsin (AAT) deficiency is one of many factors that may be involved in abnormalities such as liver and lung disease, inflammatory joint diseases, and inflammatory eye diseases. In this study, the role of AAT in uveitis is analyzed. DESIGN AND METHODS: AAT phenotyping and serum-trypsin inhibitory capacity (S-TIC) experiments were performed on 103 patients who were referred to the ALZAHRA eye center in Zahedan (southeast of Iran). The same experiments were performed on 167 people who did not suffer from any eye or systemic diseases and served as a control group. RESULTS: The results revealed that the frequency of M1S, M2S, M1Z, and MV phenotypes were significantly higher in uveitis patients (P < 0.001). There was no difference in AAT phenotype frequencies between various types of uveitis (P = 0.1). CONCLUSION: AAT deficiency appears to be a risk factor for uveitis in southeast Iran. More investigation is needed to establish potential benefits of AAT phenotyping tests and AAT therapy in the diagnosis and treatment of uveitis cases with unclear etiology.

Case-Control Studies↗

Episodic acute hypotonia after Nd:YAG laser capsulotomy--retinal function and choroidal swelling.

In a patient with uveitis who had been treated with Nd:YAG laser capsulotomy after cataract surgery, several episodes of acute hypotonia occurred which were associated with changes in clinical tests of the eye and of visual function. Immunosuppressive and immunomodulating treatment appeared to reverse the changes in intraocular pressure and normalise the test results. The significance of these observations is discussed.

Acute Disease↗

Evaluation of laser flare-cell photometry in the appraisal and management of intraocular inflammation in uveitis.

BACKGROUND: Laser flare-cell photometry enables objective and quantitative measurement of anterior chamber inflammation. Systematic data currently are used mainly for clinical research; few are yet available in uveitis. The authors prospectively studied the amount, duration, and pattern of inflammation for well-defined uveitic conditions and evaluated the potential usefulness of laser flare-cell photometry in uveitis. METHODS: Mean initial flare was calculated in HLA-B27-positive acute anterior uveitis, acute herpes zoster uveitis, acute retinal necrosis (ARN), Fuchs heterochromic cyclitis, intermediate uveitis (pars planitis-type), posterior sarcoidosis, posterior pole toxoplasmosis, and birdshot chorioretinopathy. Evolution of aqueous flare and cells was analyzed for acute anterior uveitis, ARN, and pars planitis treated for cystoid macular edema (CME), all of which received a standardized therapy. RESULTS: Blood-aqueous barrier disruption was very pronounced in acute anterior uveitis (170.2 +/- 33 photons/msecond), ARN (177.4 +/- 88 photons/msecond), moderate in posterior sarcoidosis (38.1 +/- 11 photons/msecond), acute zoster uveitis (25.8 +/- 6.1 photons/msecond), and pars planitis (19.1 +/- 2.9 photons/msecond) but only minimal in Fuchs heterochromic cyclitis (10.2 +/- 3.5 photons/msecond), toxoplasmosis (9.0 +/- 1.2 photons/msecond) and birdshot chorioretinopathy (5.7 +/- 1.1 photons/msecond). For acute anterior uveitis, ARN, and pars planitis with CME, the inflammatory patterns were determined. The potential of laser flare-cell photometry for precise follow-up and adjustment of therapy was illustrated in cases of anterior and posterior uveitis. CONCLUSION: The authors' findings show that laser flare-cell photometry allows quantitative assessment of inflammation in uveitis and contributes to improved management of patients with uveitis.

Adolescent↗

Intravitreal sustained-release cyclosporine in the treatment of experimental uveitis.

OBJECTIVE: Uveitis often is a chronic disease requiring long-term medical therapy. Despite treatment, the disease may be difficult to control and may produce serious, vision-threatening ocular complications. In this study, the authors determined whether an intravitreal cyclosporine-sustained delivery device was effective in the treatment of ocular inflammation in a rabbit model of uveitis. METHODS: New Zealand White rabbits were immunized subcutaneously with Mycobacterium tuberculosis H37Ra antigen. Fourteen days later, sustained-release cyclosporine devices were implanted into the vitreous cavity of the right eye of experimental rabbits. Control animals received sham devices. Seven days after device implantation, rabbits were challenged with an intravitreal injection of tuberculin antigen. To simulate chronic inflammation with exacerbations, some animals were rechallenged with intravitreal antigen on day 21 after device implantation. Inflammation was assessed clinically by a masked observer who graded anterior chamber cells, flare, corneal neovascularization, iris congestion, and vitreous opacity daily until day 7 and on day 13 after the initial intravitreous challenge, and on days 1 and 2 after the rechallenge. Retinal function was evaluated by electroretinography. Animals were killed 3, 6, 8, and 14 days after the initial intravitreal challenge and on the second day after rechallenge for aqueous leukocyte count, protein measurement, and histologic examination. The number of aqueous and peripheral blood proliferating lymphocytes and the subset of CD4+ T cells were determined by flow cytometry. High-performance liquid chromatography was used to measure cyclosporine A levels in vitreous and peripheral blood. Light microscopy was used to evaluate the eyes histopathologically. RESULTS: By clinical criteria, treated eyes had significantly less inflammation than untreated eyes. The number of aqueous cells and protein concentration determined quantitatively paralleled the clinical assessment of anterior chamber cells and flare. The electroretinography B-wave was depressed significantly in untreated eyes compared with that of treated eyes (P < 0.02). Histopathologic examination results showed marked inflammation and tissue disorganization in untreated eyes, whereas cyclosporine-treated eyes had preserved architecture and greatly reduced inflammatory cells. Intravitreal cyclosporine remained at therapeutic levels for at least 6 months after intravitreal device implantation, whereas blood levels were low to nondetectable. CONCLUSIONS: The intravitreal cyclosporine A device effectively suppresses ocular inflammation in a rabbit model of uveitis. This device may be useful in the treatment of patients with severe chronic uveitis who are intolerant to currently available therapies.

Animals↗

Mycophenolate mofetil. A useful immunosuppressive in inflammatory eye disease.

OBJECTIVE: To assess the usefulness of mycophenolate mofetil (MMF) (Cellcept, Roche), a potent selective uncompetitive and reversible inhibitor of ionisine monophosphate dehydrogenase involved in purine synthesis, as an immunosuppressive and steroid-sparing agent in the management of ocular inflammatory disease. DESIGN: Open-label, prospective, uncontrolled pilot study. PARTICIPANTS: Eleven patients with uncontrolled ocular inflammation. INTERVENTION: Mycophenolate mofetil, at a dosage of 1 g twice daily, was given in conjunction with steroids, as a steroid-sparing agent, or as an additional agent with cyclosporine (CsA), or instead of CsA or azathioprine. MAIN OUTCOME MEASURES: The inflammatory response, side effects, and toxicity were monitored. RESULTS: The addition of MMF to immunosuppressive regimens led to the improvement in symptoms and the ability to reduce the dose of prednisone in most patients. Ten of 11 patients showed a favorable response to MMF, with few side effects noted. CONCLUSION: These findings suggest that MMF is a useful immunosuppressive drug for controlling ocular inflammation with minimal side effects.

Adult↗

Molecular analysis of resolving immune responses in uveitis.

To identify the cellular immune processes underlying intra-ocular inflammation, aqueous humour was obtained at cataract surgery from 22 patients with clinically inactive uveitis and 24 patients with age-related cataract. mRNA expression for the cytokines IL-1beta, IL-2, IL-4, IL-6, IL-10, IL-12, interferon-gamma (IFN-gamma), transforming growth factor-beta (TGF-beta); T cell subsets CD3, CD4, CD8; monocytes and macrophages (CD14); and B cells (CD19) was measured using reverse transcriptase-polymerase chain reaction (RT-PCR) and radiometric analysis. The majority of uveitis patients demonstrated a T cell-mediated inflammatory response, predominately involving a Th1-like cytokine profile with expression of IL-2 and IFN-gamma in 16/22 and 18/22 samples, respectively. These cytokines were present in only a small number of patients with age-related cataract. This Th1-like polarization was supported by an increased expression of CD8 in a number of patients. IL-1beta was expressed in only six uveitic eyes. Only four patients expressed either IL-4 or IL-10 and no patient expressed both. TGF-beta mRNA could be detected in 18/22 uveitis patients and 15/24 controls. IL-12, the paradigmatic Th1-inducing cytokine, was absent in all samples but CD14 was expressed in the majority of patients and controls. CD19 could not be detected in any sample. The cellular infiltrate in the uveitic eyes showed clear evidence of low IL-1 and absent IL-12 expression despite a Th1-like profile and high expression of macrophages. This strongly suggests that the systemic immunosuppressive therapy used prior to surgery in some patients and/or the chronicity of the uveitis had actively suppressed/switched off macrophage function, leading to resolution of T cell activity.

Adolescent↗

[Pars plana vitrectomy in uveitis].

A vitrectomy was performed in 30 eyes suffering from different forms of uveitis. An improvement of visual acuity was obtained in 29 cases and was explained by removal of vitreous haze and/or of the cataract, but not by the improvement of the inflammatory process.

Adolescent↗