Utilize este identificador para referenciar este registo: http://hdl.handle.net/10400.17/1182
Título: Clinical Dilemma in the Treatment of a Patient with Microangiopathic Haemolytic Anaemia, Thrombocytopaenia and Severe Hypertension
Autor: Gomes, D
Viegas, V
Castro, I
Palavras-chave: Anemia Hemolítica
Trombocitopenia
Hipertensão Grave
ADAMTS13
Criança
HDE NEF PED
Data: 2010
Editora: Sociedade Portguesa de Nefrologia e Hipertensão
Citação: Port J Nephrol Hypert 2010; 24(2): 173-177
Resumo: While haemolytic uraemic syndrome in children is predominantly associated with Shiga toxin -producing Escherichia coli (typically 0157:H7), some cases occur without associated diarrhoea, or as the manifestation of an underlying disorder other than infection. Haemolytic uraemic syndrome is characterised by microangiopathic anaemia, thrombocytopaenia and renal failure, on occasion accompanied by severe hypertension. Malignant hypertension is a syndrome that sometimes exhibits the same laboratory abnormalities as haemolytic uraemic syndrome as it may share the same pathological findings: thrombotic microangiopathy. As clinical features of both entities overlap, the distinction between them can be very difficult. However, differentiation is essential for the treatment decision, since early plasma exchange dramatically reduces mortality in haemolytic uraemic syndrome not associated with diarrhoea. An increasing number of genetic causes of this pathology have been described and may be very useful in differentiating it from thrombotic microangiopathy due to other aetiologies. Despite advances in the understanding of the pathophysiology of haemolytic uraemic syndrome not associated with diarrhoea, the management often remains empirical. We describe a patient with simultaneous microangiopathic haemolytic anaemia, thrombocytopaenia and severe hypertension managed in the acute period of illness with plasma exchange.
URI: http://hdl.handle.net/10400.17/1182
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