Fabry Nephropathy

Registro completo de metadados
MetadadosDescriçãoIdioma
Autor(es): dc.contributorUniversidade de São Paulo (USP)-
Autor(es): dc.contributorUniversidade Estadual Paulista (UNESP)-
Autor(es): dc.contributorSenhor do Bonfim Group-
Autor(es): dc.contributorUniversidade Federal do Paraná (UFPR)-
Autor(es): dc.contributorFederal University of Triângulo Mineiro-
Autor(es): dc.creatorVaisbich, Maria Helena-
Autor(es): dc.creatorde Andrade, Luis Gustavo Modelli-
Autor(es): dc.creatorSilva, Cassiano Augusto Braga-
Autor(es): dc.creatorBarreto, Fellype Carvalho-
Autor(es): dc.creatordos Reis, Marlene Antonia-
Data de aceite: dc.date.accessioned2025-08-21T21:38:21Z-
Data de disponibilização: dc.date.available2025-08-21T21:38:21Z-
Data de envio: dc.date.issued2025-04-29-
Data de envio: dc.date.issued2022-12-31-
Fonte completa do material: dc.identifierhttp://dx.doi.org/10.1007/978-3-031-17759-0_27-
Fonte completa do material: dc.identifierhttps://hdl.handle.net/11449/308132-
Fonte: dc.identifier.urihttp://educapes.capes.gov.br/handle/11449/308132-
Descrição: dc.descriptionOne of the main features of Fabry disease (FD) is insidious and progressive Fabry Nephropathy. These patients should have the same general measures as all patients with proteinuric kidney disease, such as control of proteinuria and hypertension, salt restriction, and modification of lifestyle, in addition to specific therapy. Specific therapy should be individualized for each patient in a sharing decision with the patient and his family. Enzyme replacement therapy (ERT) is appropriate for all patients with FD, regardless of the type of the GLA variant. Chaperone therapy is a choice only for patients with amenable variants and should not be used in patients with eGFR < 30 mL/min/1.73m2 and/or those under 16 years old. Finally, emerging therapies are under research, including next-generation ERT (pegunigalsidase alfa, moss-derived α-galactosidase A), substrate reduction therapy (lucerastat, venglustat), mRNA therapy, and gene therapy, which may provide better control of Fabry disease in the future.-
Descrição: dc.descriptionPediatric Nephrology Unit University of Sao Paulo-
Descrição: dc.descriptionDepartamento de Medicina Interna Universidade Estadual Paulista, São Paulo-
Descrição: dc.descriptionNephrology Service Senhor do Bonfim Group, BA-
Descrição: dc.descriptionNephrology Service Departamento de Medicina Interna Universidade Federal do Paraná, PR-
Descrição: dc.descriptionNephropathology Service General Pathology Federal University of Triângulo Mineiro-
Descrição: dc.descriptionDepartamento de Medicina Interna Universidade Estadual Paulista, São Paulo-
Formato: dc.format325-347-
Idioma: dc.languageen-
Relação: dc.relationAmyloidosis and Fabry Disease: a Clinical Guide-
???dc.source???: dc.sourceScopus-
Palavras-chave: dc.subjectChronic kidney disease-
Palavras-chave: dc.subjectFabry disease-
Palavras-chave: dc.subjectNephropathy-
Palavras-chave: dc.subjectRare diseases-
Título: dc.titleFabry Nephropathy-
Tipo de arquivo: dc.typelivro digital-
Aparece nas coleções:Repositório Institucional - Unesp

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