Investigation on acute effects of enzyme replacement therapy and influence of clinical severity on physiological variables related to exercise tolerance in patients with late onset Pompe disease.

Sechi, Annalisa; Salvadego, Desy; Da Ponte, Alessandro; Bertin, Nicole; Dardis, Andrea; Cattarossi, Silvia; Devigili, Grazia; Reccardini, Federico; Bembi, Bruno; Grassi, Bruno

Sechi, Annalisa; Salvadego, Desy; Da Ponte, Alessandro; Bertin, Nicole; Dardis, Andrea; Cattarossi, Silvia; Devigili, Grazia; Reccardini, Federico; Bembi, Bruno; Grassi, Bruno.

Afiliação

Sechi A; Regional Coordinator Center for Rare Diseases, Academic Hospital of Udine, Udine, Italy. Electronic address: annalisa.sechi@asuiud.sanita.fvg.it.
Salvadego D; Department of Medical and Biological Sciences, University of Udine, Udine, Italy.
Da Ponte A; Department of Medical and Biological Sciences, University of Udine, Udine, Italy.
Bertin N; Department of Experimental and Clinical Medical Sciences, University of Udine, Udine, Italy.
Dardis A; Regional Coordinator Center for Rare Diseases, Academic Hospital of Udine, Udine, Italy.
Cattarossi S; Regional Coordinator Center for Rare Diseases, Academic Hospital of Udine, Udine, Italy.
Devigili G; Neurological Unit, Academic Hospital of Udine, Udine, Italy.
Reccardini F; Pneumology Unit, Academic Hospital of Udine, Udine, Italy.
Bembi B; Regional Coordinator Center for Rare Diseases, Academic Hospital of Udine, Udine, Italy.
Grassi B; Department of Medical and Biological Sciences, University of Udine, Udine, Italy.

Neuromuscul Disord ; 27(6): 542-549, 2017 Jun.

Article em En | MEDLINE | ID: mdl-28433478

Assuntos

Terapia de Reposição de Enzimas; Tolerância ao Exercício/efeitos dos fármacos; Doença de Depósito de Glicogênio Tipo II/tratamento farmacológico; Doença de Depósito de Glicogênio Tipo II/fisiopatologia; alfa-Glucosidases/uso terapêutico; Adolescente; Adulto; Teste de Esforço; Feminino; Humanos; Masculino; Pessoa de Meia-Idade; Índice de Gravidade de Doença; Resultado do Tratamento; Adulto Jovem

Palavras-chave

Enzyme replacement therapy, exercise tolerance; Late-onset Pompe disease; VO(2) peak

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Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Doença de Depósito de Glicogênio Tipo II / Tolerância ao Exercício / Alfa-Glucosidases / Terapia de Reposição de Enzimas Limite: Adolescent / Adult / Female / Humans / Male / Middle aged Idioma: En Ano de publicação: 2017 Tipo de documento: Article

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