Evoked Potentials in Patients With Wilson Disease

INTRODUCTION Wilson disease (WD) is an inherited disorder of copper metabolism presenting with a variety of symptoms but commonly as a liver or neuropsychiatric disease. Abnormal evoked responses are constantly found among patients with neurologic manifestations and sometimes in patients with hepatic presentation or in presymptomatic siblings. The aim of our study was to assess visual and brainstem auditory evoked potentials (BAEP) in patients with various presentation of WD. METHODS Visual evoked potentials (VEP) were performed in 36 WD patients and BAEP were done in 37 WD patients. RESULTS Brainstem auditory evoked potentials were normal in patients with isolated hepatic presentation, whereas they were abnormal in 93.5% of patients with neurologic symptoms. There was significant prolongation of the latencies of the III and V waves and of the interpeak III-V and I-V latencies in comparison with the healthy controls (T-test P = 0). Abnormal VEP were observed in 81% of the patients including six of seven neurologically asymptomatic patients. The values of N75, P100, and N145 latencies were significantly longer in all patients than in healthy controls (T-test). CONCLUSIONS The data showed that VEP and BAEP are more frequently abnormal in WD than previously reported. The abnormal VEP and BAEP even without clinical signs and brain MRI abnormalities point to subclinical involvement of visual and auditory pathways caused by copper toxicity. Because VEP and BAEP are noninvasive and widely available, they should be performed in all patents with WD

Optimization of the immobilization process of β-galatosidade by combined entrapment-cross-linking and the kinetics of lactose hydrolysis

The immobilization of Aspergillus oryzae β-galactosidase was achieved by entrapment in sodium alginate and gelatin and cross-linking with glutaraldehyde. The optimal concentrations of the aforementioned variables in the immobilization process were determined using an orthogonal central composite design with an orthogonal axial value of 1.35313. The concentrations of alginate, gelatin and glutaraldehyde that provided the greatest enzymatic activity were 6.60%, 4.05% and 3.64% (w/v), respectively. The stability of the immobilized enzyme under the optimal conditions was evaluated through daily activity assays. After 25 uses, a 20% decrease in the enzymatic activity was observed, indicating that the immobilization process could be used to produce a stable biocatalyst. This study investigates the influence of lactose and product concentrations on kinetic reaction hydrolysis. The concentration ranges for the studied variables were 10 to 56 g/L for lactose and 0 to 11.5 g/L for glucose and galactose. Only galactose presented a competitive inhibitory effect

Recuperação de compostos bioativos a partir do bagaço de uva.

As indústrias de frutas destacam-se pelo volume de resíduo gerado e, principalmente, pela composição dos mesmos. O Brasil vem-se consolidando como um importante produtor mundial de suco de uva, gerando grande quantidade de resíduos diversos, como o bagaço, que corresponde a aproximadamente 20% da fruta processada. O bagaço constitui-se de casca e semente, e apresenta uma composição rica e heterogênea em compostos fenólicos (flavonoides e não flavonoides), razão pela qual foi empregado como matéria-prima no presente trabalho para a obtenção de um extrato bioativo. As extrações enzimática e etanólica foram avaliadas para a recuperação destes compostos para fins alimentícios. Dentre as condições avaliadas nos dois planejamentos experimentais, a extração hidroetanólica foi mais eficiente na recuperação dos compostos bioativos (p< 0,01), cujo extrato apresentou uma capacidade antioxidante de 1.965 µmol Trolox por 100 g de bagaço, 25% superior à do extrato obtido com a melhor condição enzimática (1.580 µmol Trolox por 100 g de bagaço)