Utilize este identificador para referenciar este registo: http://hdl.handle.net/10198/510
Título: Protein deficiency balance as a predictor of clinical outcome in hereditary spherocytosis
Autor: Rocha, Susana
Rebelo, Irene
Costa, Elísio
Catarino, Cristina
Belo, Luís
Castro, Elisabeth
Cabeda, José
Barbot, José
Quintanilha, Alexandre
Santos-Silva, Alice
Palavras-chave: Hereditary spherocytosis
Erythrocyte membrane proteins
Data: 2005
Editora: Blackwell
Citação: Rocha, Susana; Rebelo, Irene; Costa, Elísio; Catarino, Cristina; Belo, Luís; Castro, Elisabeth; Cabeda, José; Barbot, José; Quintanilha, Alexandre; Santos-Silva, Alice (2005) - Protein deficiency balance as a predictor of clinical outcome in hereditary spherocytosis. European Journal of Haematology. ISSN 1600-0609. 74:5, p. 374–380
Resumo: Vertical and horizontal interactions between membrane constituents account for integrity, strength and deformability of the erythrocyte. Disruption of vertical interactions caused by membrane protein deficiencies in hereditary spherocytosis (HS), favor membrane vesiculation with development of spherocytic cells. Our aim was to evaluate the hematological and clinical presentation of HS according to the type and amount of protein deficiency. We studied 81 Portuguese individuals, 71 belonging to 21 families plus 10 unrelated subjects, and found that 51 of them were HS patients. Patients were classified as presenting mild, typical or severe HS, according to laboratory results and clinical follow-up. We performed screening tests and the standardized electrophoretic membrane protein analysis to identify and quantify protein deficiencies. We found band 3 and ankyrin deficiencies as the major causes for HS. The ratios between the value of the primary and/or secondary protein deficiencies showed significantly different values according to the severity of HS, and a significant inverse correlation with the severity of HS was observed. In mild HS, the ratios between protein deficiencies reflected equivalent protein deficiencies, while an unbalance was observed in typical HS, which was enhanced in severe HS. Our data suggest that the relative quantification of each major membrane protein and of the ratios between the values of protein deficiencies may be helpful in providing additional data about the clinical outcome of HS.
URI: http://hdl.handle.net/10198/510
ISSN: 1600-0609
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