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MICROANGIOPATIAS TROMBÓTICAS EM PEDIATRIA

MICROANGIOPATIAS TROMBÓTICAS EM PEDIATRIA

Maalej Bayene / Weli Manel

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Editorial:
KS OmniScriptum Publishing
Año de edición:
2025
Materia
Medicina clínica e interna
ISBN:
9786206814832
64,18 €
IVA incluido
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As microangiopatias trombóticas (MAT) são doenças caracterizadas por uma combinação de anemia hemolítica microangiopática mecânica, trombocitopenia e lesões em órgãos-alvo de gravidade variável que requerem tratamento urgente. Em crianças, a etiologia mais comum das MAT é a síndrome hemolítico-urémica (SHU) de Escherichia coli produtora de shigatoxina, anteriormente conhecida como SHU típica. Outras SHU são designadas SHU atípicas e incluem a SHU secundária a infecções por Streptococcus pneumoniae, a SHU secundária a infecções por Haemophilus influenzae e a desregulação da via alternativa do complemento. Outras etiologias incluem a púrpura trombocitopénica trombótica (PTT) devido à deficiência de ADAMTS 13 e outras causas menos comuns, como as induzidas por fármacos, pós-transplante, auto-imunes e genéticas. O curso da doença pode ser marcado pela ocorrência de complicações renais e extra-renais, que podem ser fatais. O tratamento baseia-se na correção de distúrbios hidroelectrolíticos, depuração extra-renal, plasmaferese e anticorpos monoclonais.

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