HEREDITARY SPHEROCYTOSIS CHARACTERIZED BY INCREASED SPECTRIN/BAND 3 RATIO
نویسندگان
چکیده
منابع مشابه
Hereditary 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...
متن کاملHereditary Spherocytosis
A 12-year-old girl was brought to the Dhaka Hospital of ICDDR,B with diarrhoea. Incidentally, the parents provided a history of repeated episodes of pallor and jaundice since she was two and half years old. Three of her family members had similar problems. History, clinical examination, and laboratory findings of the girl and her family members suggested a case of hereditary spherocytosis. To o...
متن کاملIncreased Cell Membrane Permeability in the Pathogenesis of Hereditary Spherocytosis.
The hemolytic process in hereditary spherocytosis (HS) is presumed to involve an intrinsic defect in the structure or metabolism of the red cell (1-3). The nature of the basic cellular defect remains enigmatic, although its manifestations are well characterized. Distinctive features of the HS red cell are its more spheroidal shape, small surface area, and high hemoglobin concentration and its e...
متن کاملIncreased erythrocyte adhesion in mice and humans with hereditary spherocytosis and hereditary elliptocytosis.
Mice with disruptions of the red blood cell (RBC) cytoskeleton provide severe hemolytic anemia models in which to study multiorgan thrombosis and infarction. The incidence of cerebral infarction ranges from 70% to 100% in mice with alpha-spectrin deficiency. To determine whether mutant RBCs abnormally bind adhesive vascular components, we measured adhesion of mouse and human RBCs to immobilized...
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ژورنال
عنوان ژورنال: British Journal of Haematology
سال: 1992
ISSN: 0007-1048,1365-2141
DOI: 10.1111/j.1365-2141.1992.tb06417.x