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Imunodeficiência primária por défice de ZAP-70

2001Journal article Open access
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dc.contributor.authorBarata, LT
dc.contributor.authorHenriques, R
dc.contributor.authorHivroz, C
dc.contributor.authorJouanguy, E
dc.contributor.authorPaiva, A
dc.contributor.authorFreitas, AM
dc.contributor.authorCoimbra, HB
dc.contributor.authorFischer, A
dc.contributor.authorCarmona da Mota, H
dc.date.accessioned2011-10-24T12:03:08Z
dc.date.available2011-10-24T12:03:08Z
dc.date.issued2001
dc.description.abstractThe authors present the case of a child with recurrent infections since the age of 4 months, including bilateral pneumonia by Pneumocystis carinii and protracted varicella. Serum immunoglobulin values (when 10 months old), and B cell values were normal. There was persistent lymphocytic leucocytosis, near absence of CD8+ cells, and an increased CD4/CD8 ratio. The percentage of activated T cells and the expression of HLA class I were normal. Proliferation, activation and IL-2 synthesis studies in T cells showed a TCR/CD3-associated signal transduction deficit. ZAP-70 cDNA sequencing showed a mutation, and no ZAP-70 protein was detected in T cells. ZAP-70 deficiency is associated with a rare immune deficiency with absence of CD8+ T cells as well as a functional deficiency in T cells. Seven months after bone marrow transplantation the child is clinically well and immunologically recoveredpor
dc.identifier.citationActa Med Port. 2001 Jul-Aug;14(4):413-7.por
dc.identifier.urihttp://hdl.handle.net/10400.4/1089
dc.language.isoporpor
dc.peerreviewedyespor
dc.subjectImunodeficiência Primáriapor
dc.titleImunodeficiência primária por défice de ZAP-70por
dc.title.alternativePrimary immunodeficiency secondary to ZAP-70 deficiencypor
dc.typejournal article
dspace.entity.typePublication
rcaap.rightsopenAccesspor
rcaap.typearticlepor

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