[A case of Anderson-Fabry disease: a multidisciplinary approach for diagnosis and follow up].
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
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Prognostic_studies
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Screening_studies
Limite:
Humans
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Male
/
Middle aged
Idioma:
It
Revista:
G Ital Nefrol
Assunto da revista:
NEFROLOGIA
Ano de publicação:
2018
Tipo de documento:
Article