RESUMO
PURPOSE: Corneal involvement in mitochondrial disease is seldom described. Kearns-Sayre syndrome (KSS) is a mitochondrial disorder characterized by retinitis pigmentosa, external ophthalmoplegia, and heart block. We report 2 patients with KSS with corneal lesions involving the endothelium, which improved with Coenzyme Q10 (CoQ10). Based on recent research regarding the role of dysfunctional oxidative metabolism in Fuchs Endothelial Corneal Dystrophy (FECD), we propose that mitochondrial diseases and FECD share a final pathway. METHODS: A chart review was performed and a review of the literature was completed with a PubMed search using the terms "Kearns-Sayre Syndrome", "mitochondria", "endothelium", "Fuchs endothelial corneal dystrophy", and "cornea". RESULTS: There are 19 reports of corneal involvement in clinical phenotypes of mitochondrial disease. Nine of these 19 cases had findings consistent with KSS. Our patients with KSS had microcystic changes throughout the cornea and excrescences on the endothelial surface seen with ultrasound biomicroscopy, similar to the clinical findings in FECD. CoQ10 improved corneal disease in both children. CoQ10 deficiency has been reported in a variety of mitochondrial diseases, and efficacy of supplementation has been demonstrated. It may be beneficial in these patients because of its antioxidant properties and role in oxidative phosphorylation. CONCLUSIONS: The common deletion found in patients with KSS has recently been implicated in FECD, which has recently been shown to be a disease related to dysfunctional oxidative metabolism. Future research should explore the use of antioxidants, such as CoQ10 in patients with FECD.
Assuntos
Edema da Córnea/tratamento farmacológico , Complexo de Proteínas da Cadeia de Transporte de Elétrons/uso terapêutico , Endotélio Corneano/efeitos dos fármacos , Distrofia Endotelial de Fuchs/tratamento farmacológico , Síndrome de Kearns-Sayre/tratamento farmacológico , Ubiquinona/análogos & derivados , Criança , Pré-Escolar , Edema da Córnea/diagnóstico , Endotélio Corneano/patologia , Humanos , Síndrome de Kearns-Sayre/diagnóstico , Masculino , Soluções Oftálmicas , Ubiquinona/uso terapêutico , Acuidade Visual/efeitos dos fármacosRESUMO
OBJECTIVE: The aim of this study is to show the possible positive effect of coenzyme Q10 (Co Q10) on regenerating in facial palsy. MATERIALS AND METHODS: Sixteen female Sprague-Dawley albino rats were randomly divided into 2 groups as Co Q10 and control groups. Group Q10 (n = 8) received Co Q10 of 10 mg/kg/d intraperitoneally for 30 days, and group C (n = 8) received saline solution of 1 mL/d intraperitoneally once daily for 30 days. The right facial nerve stimulation thresholds were determined before crush, immediately after crush, and after 1 month.After determination of the thresholds, the crushed part of the facial nerve was then excised. All specimens were examined by a pathologist using a light microscope. RESULTS: No statistically significant difference in stimulation threshold was found between the Co Q10 and saline groups after crushing (P = 0.645). After 1 month of treatment, stimulation thresholds were significantly lower in both the Co Q10 and saline groups (Ps = 0.028 and 0.016). However, the Co Q10 group showed greater improvement than the saline group (P = 0.050).After 1 month of treatment, neither the Co Q10 group nor the saline group had reached the precrushing amplitude levels (Ps = 0.027 and 0.011).Significant differences were found in vascular congestion, macrovacuolization, and myelin thickness between the Co Q10 and control groups by light microscopy (P < 0.05). CONCLUSIONS: Although many treatment methods have been tried to accelerate facial nerve regeneration after trauma, a definitive method has not been found yet. Co Q for the treatment of acute facial paralysis is promising on both physiologic assessments and pathologic evaluation.
Assuntos
Antioxidantes/uso terapêutico , Traumatismos do Nervo Facial/tratamento farmacológico , Regeneração Nervosa/efeitos dos fármacos , Ubiquinona/análogos & derivados , Animais , Estimulação Elétrica , Complexo de Proteínas da Cadeia de Transporte de Elétrons/uso terapêutico , Traumatismos do Nervo Facial/patologia , Traumatismos do Nervo Facial/fisiopatologia , Paralisia Facial/tratamento farmacológico , Paralisia Facial/fisiopatologia , Feminino , Hiperemia/tratamento farmacológico , Hiperemia/patologia , Bainha de Mielina/efeitos dos fármacos , Bainha de Mielina/patologia , Distribuição Aleatória , Ratos , Ratos Sprague-Dawley , Limiar Sensorial/efeitos dos fármacos , Ubiquinona/uso terapêutico , Vacúolos/efeitos dos fármacosRESUMO
Oxidative stress is known to play a relevant role in Down syndrome (DS) and its effects are documented from embryonic life. Oxidative DNA damage has been shown to be significantly elevated in Down syndrome patients, and this has been indicated as an early event promoting neurodegeneration and Alzheimer type dementia. The aim of this study was to investigate the efficacy of coenzyme Q(10) (CoQ(10)) in delaying the effect of oxidative damage in these patients. In our previous study we demonstrated a mild protective effect of CoQ(10) on DNA, although the treatment was unable to modify the overall extent of oxidative damage at the patient level. Possible limitations of the previous study were: time of treatment (6 months) or spectrum of DNA lesions detected. In order to overcome these limitations we planned a continuation of the trial aimed at evaluating the effects of CoQ(10) following a prolonged treatment. Our results highlight an age-specific reduction in the percentage of cells showing the highest amount of oxidized bases, indicating a potential role of CoQ(10) in modulating DNA repair mechanisms.
Assuntos
Dano ao DNA/genética , Síndrome de Down/terapia , Estresse Oxidativo/genética , Ubiquinona/análogos & derivados , Vitaminas/administração & dosagem , Adolescente , Envelhecimento/metabolismo , Plaquetas/metabolismo , Criança , Pré-Escolar , Síndrome de Down/enzimologia , Síndrome de Down/genética , Complexo de Proteínas da Cadeia de Transporte de Elétrons/administração & dosagem , Complexo de Proteínas da Cadeia de Transporte de Elétrons/sangue , Complexo de Proteínas da Cadeia de Transporte de Elétrons/uso terapêutico , Humanos , Linfócitos/metabolismo , Fatores de Tempo , Resultado do Tratamento , Ubiquinona/administração & dosagem , Ubiquinona/sangue , Ubiquinona/uso terapêutico , Vitaminas/sangue , Vitaminas/uso terapêuticoRESUMO
Coenzyme Q10 (CoQ10) is critical to production of adenosine triphosphate and is an antioxidant that scavenges reactive oxygen species during oxidative stress. The use of CoQ10 in treating oxidative stress in cardiovascular diseases, diabetes, and cancer is reviewed.
Assuntos
Complexo de Proteínas da Cadeia de Transporte de Elétrons/farmacologia , Complexo de Proteínas da Cadeia de Transporte de Elétrons/uso terapêutico , Estresse Oxidativo/efeitos dos fármacos , Ubiquinona/análogos & derivados , Neoplasias da Mama/tratamento farmacológico , Interações Medicamentosas , Feminino , Insuficiência Cardíaca/tratamento farmacológico , Humanos , Inibidores de Hidroximetilglutaril-CoA Redutases/uso terapêutico , Hipertensão/tratamento farmacológico , Estresse Oxidativo/fisiologia , Ubiquinona/farmacologia , Ubiquinona/uso terapêuticoAssuntos
Anticolesterolemiantes/efeitos adversos , Inibidores de Hidroximetilglutaril-CoA Redutases/efeitos adversos , Doenças Musculares/prevenção & controle , Ubiquinona/análogos & derivados , Suplementos Nutricionais , Complexo de Proteínas da Cadeia de Transporte de Elétrons/efeitos dos fármacos , Complexo de Proteínas da Cadeia de Transporte de Elétrons/metabolismo , Complexo de Proteínas da Cadeia de Transporte de Elétrons/uso terapêutico , Humanos , Músculo Esquelético/efeitos dos fármacos , Músculo Esquelético/enzimologia , Doenças Musculares/induzido quimicamente , Doenças Musculares/enzimologia , Ubiquinona/efeitos dos fármacos , Ubiquinona/metabolismo , Ubiquinona/uso terapêuticoAssuntos
Antioxidantes/uso terapêutico , Higiene da Pele , Envelhecimento/fisiologia , Ácido Ascórbico/uso terapêutico , Complexo de Proteínas da Cadeia de Transporte de Elétrons/uso terapêutico , Flavonoides/uso terapêutico , Humanos , Fenóis/uso terapêutico , Polifenóis , Ácido Tióctico/uso terapêutico , Ubiquinona/análogos & derivados , Ubiquinona/uso terapêuticoRESUMO
OBJECTIVE: To determine the short-term effects of coenzyme Q10 (CoQ10) on the antioxidative status and tinnitus expression in patients with chronic tinnitus aurium. STUDY DESIGN: A 16-week prospective nonrandomized clinical trial (n = 20). Tinnitus and Short Form-36 Questionnaires (TQ/SF-36) were evaluated together with the plasma concentrations of CoQ10, malondialdehyde, and the total antioxidant status. RESULTS: The mean plasma CoQ10 concentration rose under external CoQ10 supply and remained elevated after medication stopped without overall effects on the tinnitus score. However, in a subgroup of 7 patients with low initial plasma CoQ10 concentration and significant increase in the plasma CoQ10 level, a clear decrease in the TQ score was observed. CONCLUSION: In patients with a low plasma CoQ10 concentration, CoQ10 supply may decrease the tinnitus expression. SIGNIFICANCE: This is the first study to examine the effect of CoQ10 in chronic tinnitus aurium.