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[A case of Anderson-Fabry disease: a multidisciplinary approach for diagnosis and follow up].
Zito, Anna; De Pascalis, Antonio; Armeni, Annarita; Ria, Paolo; Barbarini, Leonardo; Caggiula, Marcella; My, Filomena; Barbarini, Silvia; Trianni, Giorgio; Napoli, Marcello.
Afiliação
  • Zito A; Unità Operativa Nefrologia e Dialisi, Ospedale Vito Fazzi, Lecce.
  • De Pascalis A; Unità Operativa Nefrologia e Dialisi, Ospedale Vito Fazzi, Lecce.
  • Armeni A; Unità Operativa Nefrologia e Dialisi, Ospedale Vito Fazzi, Lecce.
  • Ria P; Unità Operativa Nefrologia e Dialisi, Ospedale Vito Fazzi, Lecce.
  • Barbarini L; Unità Operativa Neurologia, Ospedale Vito Fazzi, Lecce.
  • Caggiula M; Unità Operativa Neurologia, Ospedale Vito Fazzi, Lecce.
  • My F; Unità Operativa Neurologia, Ospedale Vito Fazzi, Lecce.
  • Barbarini S; Unità Operativa Nefrologia e dialisi, Fond. Pol. A. Gemelli, Roma.
  • Trianni G; Unità Operativa Neurologia, Ospedale Vito Fazzi, Lecce.
  • Napoli M; Unità Operativa Nefrologia e Dialisi, Ospedale Vito Fazzi, Lecce.
G Ital Nefrol ; 35(5)2018 Sep.
Article em It | MEDLINE | ID: mdl-30234233
Fabry disease (also known as Anderson-Fabry disease, angiocheratoma corporis diffusum, diffuse angiocheratoma) is a rare tesaurismosis linked to the deficiency of the lysosomal enzyme alpha-galactosidase A, required for the physiological catabolism of glycosphingolipids. The related clinical signs show a multisystemic feature and define a degenerative and disabling pathology, whose approach requires a close multidisciplinary specialist collaboration. Currently, the renewed interest in the disease is aimed at the need to provide an early diagnosis, in order to early begin the enzyme replacement therapy and to slow down or avoid the establishment of irreparable organ damage. For this reason, the diagnostic suspicion becomes crucial and arises from the careful observation and research of the symptoms, together with the anamnesis and the overall clinical evaluation of the patient.
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Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Doença de Fabry Tipo de estudo: Diagnostic_studies / Etiology_studies / Prognostic_studies / Screening_studies Limite: Humans / Male / Middle aged Idioma: It Revista: G Ital Nefrol Assunto da revista: NEFROLOGIA Ano de publicação: 2018 Tipo de documento: Article
Buscar no Google
Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Doença de Fabry Tipo de estudo: Diagnostic_studies / Etiology_studies / Prognostic_studies / Screening_studies Limite: Humans / Male / Middle aged Idioma: It Revista: G Ital Nefrol Assunto da revista: NEFROLOGIA Ano de publicação: 2018 Tipo de documento: Article