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Biomedical subjects

Alev Guven

Publications and source records attributed to Alev Guven.

4 recordsLinked to original sources

Refractive errors in neurofibromatosis type 1 and type 2.

OBJECTIVE: To document the prevalence of refractive errors in patients with neurofibromatosis type 1 (NF1) and type 2 (NF2) and to compare it with that of age- and sex-matched controls. METHODS: 82 patients with NF1, 21 patients with NF2 and 103 age- and sex-matched controls were evaluated in this prospective observational case-control study. Cycloplegic autorefraction and dilated fundus examination were performed. Myopia was defined as the spherical equivalent refraction of at least -0.50 diopters (D), hyperopia as the spherical equivalent refraction of at least 2.0 D and astigmatism as the cylinder of at least 1.0 D. Main outcome measures were refractive error, IQ, years of education, height, weight and body mass index (BMI). RESULTS: The prevalence of myopia was 23.1% in patients with NF1, 23.8% in patients with NF2 and 16.5% in age- and sex-matched controls. These differences were significant (p<0.03, p<0.03), and adjusting for intelligence, education, height, weight and BMI increased the significance of this finding (p<0.001, p<0.001). The prevalences of astigmatism and hyperopia were similar in both groups. CONCLUSION: A high prevalence of myopia seems to be an additional feature of NF1 and NF2.

Case-Control Studies↗

Lymphocyte subsets, TNF alpha and interleukin-4 levels in treated and untreated subacute sclerosing panencephalitis patients.

Immunologic studies in relation to clinical status might help to understand the pathogenesis of subacute sclerosing panencephalitis (SSPE) and the effect of treatment. We measured lymphocyte subsets and intracellular TNFalpha and interleukin-4 levels in peripheral blood in SSPE patients. Patients had elevated percentages of CD8+ cells compared to age-matched control children. Rapidly progressive course was associated with increased CD4+ cells. Treatment with interferons and inosiplex altered the percentage of CD3+, CD4+ and CD19+ cells. TNFalpha and interleukin-4 levels had no correlation with course or treatment. The proportions of lymphocyte subsets appear to have a role in the evolution or manifestations of SSPE, if not in the pathogenesis.

Adolescent↗

Optic atrophy and macular degeneration as initial presentations of subacute sclerosing panencephalitis.

PURPOSE: To report a case of subacute sclerosing panencephalitis in which the initial clinical presentations were optic atrophy and macular degeneration. DESIGN: Observational case report. METHODS: A 9-year-old boy with visual loss attributable to optic atrophy and macular degeneration had a diagnostic examination. RESULTS: The visual acuity levels of both eyes were light perception at presentation. Fundus examination revealed bilateral optic atrophy and macular degenerative changes including retinal pigment epithelial atrophy, macular scarring, and epiretinal membrane formation. The antimeasles IgG titers were high both in serum and cerebrospinal fluid samples. Neurologic symptoms of myoclonus, ataxia, motor, and mental disabilities occurred 4 months after the onset of first visual disturbances and 2 weeks after the patient applied to our clinic. CONCLUSION: Children with optic atrophy and macular degenerative lesions should be examined for sclerosing panencephalitis, even if the classical neurologic symptoms of this disease are not present.

Analgesics, Non-Narcotic↗

Retrospective evaluation of interferon-beta treatment in subacute sclerosing panencephalitis.

BACKGROUND: Few effective treatment methods are available for subacute sclerosing panencephalitis (SSPE),an infection associated with the measles virus. Interferons have shown some benefit in previous studies and clinical practice. OBJECTIVE: The purpose of this study was to compare the efficacy of 2 different regimens of interferon-beta(IFN-beta) in the treatment of SSPE in pediatric patients. METHODS: We retrospectively compared the results obtained with 2 regimens of IFN-beta1a: 60 microg administered intramuscularly once weekly (IFN-beta 1/wk), or 22 microg administered subcutaneously 3 times per week (IFN-beta 3/wk). All patients also received oral inosiplex 50 to 100 mg/kg daily, a treatment known to have partial efficacy in SSPE. Patients who continued treatment for at least 3 months and had at least 1 year of follow-up data were evaluated. Clinical parameters included the Neurological Disability Index (NDI), a measurement of mental, motor, and sensory functions; disease stage; and mental status. Data obtained at 6 and 12 months were compared with those at the time of diagnosis, and the percent change from baseline was calculated. A satisfactory clinical response was defined as reduction or stabilization of the NDI or stage improvement at 6 or 12 months. RESULTS: Patients treated with IFN-beta 3/wk had increased survival time (P < 0.02) and higher clinical response rates compared with those treated with IFN-beta 1/wk (P < 0.05). When stage 2 and stage 3 patients were evaluated separately, survival was significantly longer (P = 0.007) and the rate of progression slower in both stage groups with IFN-beta 3/wk. CONCLUSION: The results obtained for this patient sample suggest that IFN-beta administered subcutaneously times per week combined with inosiplex may be an effective treatment option in SSPE. This treatment regimen warrants further study.

Adjuvants, Immunologic↗