Clinical course of 63 children with hereditary spherocytosis
نویسندگان
چکیده
منابع مشابه
Clinical course of 63 children with hereditary spherocytosis: a retrospective study
BACKGROUND Hereditary spherocytosis (HS) is an inherited hemolytic anemia that is caused by deficiency or dysfunction of erythrocyte cytoskeletal proteins. AIM The aim of this study was to describe the clinical course of hereditary spherocytosis in patients treated in the Pediatric Hematology Unit, Hospital das Clínicas, Universidade Federal de Minas Gerais. METHODS Sixty-three under 16-yea...
متن کاملComments on 'clinical course of 63 children with hereditary spherocytosis: a retrospective study' - with the particular question: 'Should HS be treated the same way throughout the world?'
The authors describe a retrospective study of 63 children with hereditary spherocytosis (HS) in Minas Gerais, Brazil. (1) HS is generally a mild and benign disease which is found in all areas of the world, but most commonly in peoples of Northern European origin. In this study, as elsewhere, more severe forms of the disorder were diagnosed earlier in life. The classification of Eber et al. has ...
متن کاملHereditary spherocytosis: from clinical to molecular defects.
Resistance and elastic deformability of red cells are due to a protein network (cytoskeleton) that laminates the lipid bilayer and to proteins that span the latter. All proteins are interconnected. Their structure as well as the structure of the corresponding genes are now well known. Hereditary spherocytosis (HS) is the most common hemolytic anemia due to a red cell membrane defect. It derives...
متن کاملHereditary spherocytosis.
Hereditary spherocytosis is a common inherited disorder that is characterised by anaemia, jaundice, and splenomegaly. It is reported worldwide and is the most common inherited anaemia in individuals of northern European ancestry. Clinical severity is variable with most patients having a well-compensated haemolytic anaemia. Some individuals are asymptomatic, whereas others have severe haemolytic...
متن کاملHereditary spherocytosis.
Hereditary spherocytosis is a congenital haemolytic anaemia due to defect in spectrin-a RBC membrane protein and is transmitted as autosomal dominant. Due to this defect there is presence of characteristic spherical cell in peripheral blood smear and osmotic fragility is increased. Haemolytic anaemia, reticulocytosis, jaundice and splenomegaly are present. This article reports a case of a 9 yea...
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ژورنال
عنوان ژورنال: Revista Brasileira de Hematologia e Hemoterapia
سال: 2011
ISSN: 1516-8484
DOI: 10.5581/1516-8484.20120006