نتایج جستجو برای: hereditary spherocytosis

تعداد نتایج: 84467  

Journal: :Journal of clinical pathology 1985
D I Evans

Three new cases (two fatal) of postsplenectomy sepsis occurring 14, 25, and 26 years after operation for hereditary spherocytosis are described. There are now 25 cases in the published work in which this complication occurred 10 or more years after operation, 14 of which were fatal. The mean age of onset is 37 years. The features of the disease are similar to those seen in other postsplenectomy...

2002
Hani Hassoun James Murray Scott J. Yi Manjit Hanspal Christine A. Johnson Jiri Palek

Red cell membrane protein analysis by sodium dodecyl sulfate-polyacrylamide gel electrophoresis (SDS-PAGE) and direct quantitation by radioimmunoassay or cytofluorometry defines four distinct subsets of patients with hereditary spherocytosis: Patients with isolated spectrin deficiency, combined spectrin and ankyrin deficiency, band 3 deficiency, and protein 4.2 deficiency. In regard to the firs...

Journal: :Blood reviews 2013
Lydie Da Costa Julie Galimand Odile Fenneteau Narla Mohandas

Hereditary spherocytosis and elliptocytosis are the two most common inherited red cell membrane disorders resulting from mutations in genes encoding various red cell membrane and skeletal proteins. Red cell membrane, a composite structure composed of lipid bilayer linked to spectrin-based membrane skeleton is responsible for the unique features of flexibility and mechanical stability of the cel...

Journal: :Haematologica 2008
Mariagabriella Mariani Wilma Barcellini Cristina Vercellati Anna Paola Marcello Elisa Fermo Paola Pedotti Carla Boschetti Alberto Zanella

BACKGROUND Hereditary spherocytosis is a very heterogeneous form of hemolytic anemia. The aim of this study was to relate the type of molecular defect with clinical and hematologic features and response to splenectomy using information from a large database of patients. DESIGN AND METHODS Data from 300 consecutive patients with hereditary spherocytosis, grouped according to the results of sod...

Journal: :Journal of Clinical Investigation 1966

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