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Letra I


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INIBIDOR DE C1 - ESTERASE

Material:

Soro

Instruções de coleta:

Coletar em tubo sem anticoagulante, separar o soro e congelar.

Informações:

Avaliação de edema angioneurótico hereditário (doença autossômica dominante). O edema angioneurótico hereditário é uma doença autossômica dominante causada pela deficiência de inibidor de C1 esterase. O defeito permite ativação não controlada do 1o. componente do Complemento e do sistema das cininas, com produção de substâncias vasoativas que causam episódios recorrentes de edema da pele, do trato digestivo, do sistema genito-urinário e do trato respiratório. Estes pacientes apresentam também níveis baixo de C4 e C2, mesmo em períodos fora de crises. Quando o inibidor está presente, e com atividade normal, o mesmo liga-se a C1r com mascaramento de sua antigenicidade, o que pode ser detectado por técnica de imunodifusão. Cerca de 15% dos pacientes com edema angioneurótico hereditário apresentam a proteína numa forma não funcionante. O inibidor de C1 esterase é um inibidor de protease multiespecífico, que está presente no protoplasma humano e soro, regulando enzimas do complemento, coagulação e fibrinólise. Valores diminuídos: edema angioneurótico hereditário, doenças linfóides malignas, doenças autoimunes.

Referência:

15,0 a 34,0 mg/dL

 

Laboratório Bioclinico

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