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1.
Rev. neurol. (Ed. impr.) ; 60(7): 321-328, 1 abr., 2015. tab
Artigo em Espanhol | IBECS | ID: ibc-135429

RESUMO

Aunque el tratamiento con alglucosidasa alfa ha contribuido a mejorar el pronóstico de los pacientes con enfermedad de Pompe de inicio tardío, es necesario hacer un seguimiento periódico de la evolución de la enfermedad y de la eficacia del tratamiento. Por este motivo, un comité de expertos españoles ha elaborado una guía para el seguimiento de estos pacientes. El comité propone un modelo de pruebas de seguimiento para la enfermedad de Pompe de inicio tardío. En primer lugar, ha de valorarse el estado nutricional y la función deglutoria. En segundo lugar, y debido a la variabilidad del cuadro clínico, el comité recomienda el uso simultáneo de varias escalas que midan distintas funciones y pará- metros. De este modo, la fuerza muscular se evalúa con la escala del Medical Research Council; la función motora, con la prueba de la marcha en seis minutos y pruebas cronometradas; la discapacidad, con la escala de actividad específica de la enfermedad de Pompe construida según el análisis de Rasch; la función respiratoria, con la medida de la capacidad vital forzada y la saturación de oxígeno; y la fatiga, con la escala de intensidad de la fatiga. Por último, la seguridad y la tolerabilidad del tratamiento enzimático sustitutivo se controlan con el registro y tratamiento de los potenciales efectos adversos y la medición de los anticuerpos antialglucosidasa alfa. Se incluyen también diversas recomendaciones generales (AU)


Although treatment with alglucosidase alfa has helped improve the prognosis of patients with late-onset Pompe disease, both the development of the disease and the effectiveness of the treatment need to be monitored on a regular basis. This is the reason that has led a committee of Spanish experts to draw up a series of guidelines on how to follow up these patients. The committee proposes a model of follow-up tests for late-onset Pompe disease. First of all, the nutritional status and swallowing function must be evaluated. Second, and due to the variability of the clinical features, the committee recommends the simultaneous use of several scales to measure different functions and parameters. Thus, muscular force is assessed with the Medical Research Council scale; motor functioning, with the six-minute walk test and timed tests; disability, with the Rasch-built Pompe-specific Activity scale; respiratory functioning, with measurement of the forced vital capacity and oxygen saturation; and fatigue, with the fatigue intensity scale. Lastly, the safety and tolerability of enzyme replacement therapy are controlled by registering and treating the potential side effects and measurement of the anti-alglucosidase alfa antibodies. A number of different general recommendations are also included (AU)


Assuntos
Humanos , Adolescente , Criança , Doença de Depósito de Glicogênio Tipo II/tratamento farmacológico , Terapia de Reposição de Enzimas , Glucana 1,4-alfa-Glucosidase/uso terapêutico , Força Muscular , Idade de Início , Doença de Depósito de Glicogênio Tipo II/epidemiologia , Biomarcadores , Capacidade Vital , Índice de Gravidade de Doença , Doenças do Sistema Digestório/etiologia , Proteínas Recombinantes/uso terapêutico , Monitoramento de Medicamentos
2.
Int. microbiol ; 6(4): 269-273, dic. 2003. ilus, tab, tab, graf
Artigo em Inglês | IBECS | ID: ibc-98747

RESUMO

The effect of several nutritional and environmental parameters on growth and amylase production from Rhizopus microsporus var. rhizopodiformis was analysed. This fungus was isolated from soil of the Brazilian «cerrado» and produced high levels of amylolytic activity at 45 degrees C in liquid medium supplemented with starch, sugar cane bagasse, oat meal or cassava flour. Glucose in the culture medium drastically repressed the amylolytic activity. The products of hydrolysis were analysed by thin layer chromatography, and glucose was detected as the main component. The amylolytic activity hydrolysed several substrates, such as amylopectin, amylase, glycogen, pullulan, starch, and maltose. Glucose was always the main end product detected by high-pressure liquid chromatography analysis. These results indicated that the amylolytic activity studied is a glucoamylase, but there were also low levels of alpha-amylase. As compared to other fungi, R. microsporus var. rhizopodiformis can be considered an efficient producer of thermostable amylases, using raw residues of low cost as substrates. This information is of technological value, considering the importance of amylases for industrial hydrolysis (AU)


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Assuntos
Microbiologia do Solo , Rhizopus/patogenicidade , Amilases/análise , Glucana 1,4-alfa-Glucosidase/análise , Hidrólise
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