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Aka: Hereditary Spherocytosis , Minkowski-Chauffard syndrome. These images are a random sampling from a Bing search on the term "Hereditary Spherocytosis. Search Bing for all related images.
Started in , this collection now contains interlinked topic pages divided into a tree of 31 specialty books and chapters. Content is updated monthly with systematic literature reviews and conferences. Although access to this website is not restricted, the information found here is intended for use by medical providers.
Patients should address specific medical concerns with their physicians. Toggle navigation. Hematology and Oncology. Hemoglobinopathies Chapter. From Related Chapters. Pharmacology Hydroxyurea. Page Contents Page Contents Pathophysiology Most common inherited membranopathy Results from membrane protein gene mutations Autosomal Dominant.
Management Splenectomy Indicated in moderate to severe cases. Complications Hemolytic Anemia. Images: Related links to external sites from Bing. Related Studies. Trip Database TrendMD. Ontology: Hereditary spherocytosis C Definition MSH A group of familial congenital hemolytic anemias characterized by numerous abnormally shaped erythrocytes which are generally spheroidal. The erythrocytes have increased osmotic fragility and are abnormally permeable to sodium ions.
Definition NCI An autosomal dominant inherited disorder characterized by the presence of smaller than normal, sphere-shaped erythrocytes. It results in hemolytic anemia and splenomegaly. Definition CSP autosomal dominant trait marked by splenomegaly, jaundice, and fragile, spheroid erythrocytes which are prematurely cleared by the spleen; anemia is usually mild; treatment is splenectomy.
Related Topics in Hemoglobinopathies. Hematology and Oncology Chapters. Hematology and Oncology - Hemoglobinopathies Pages. Back Links pages that link to this page. Search other sites for 'Hereditary Spherocytosis'. Page Contents A group of familial congenital hemolytic anemias characterized by numerous abnormally shaped erythrocytes which are generally spheroidal.
An autosomal dominant inherited disorder characterized by the presence of smaller than normal, sphere-shaped erythrocytes. Disease or Syndrome T Anemia sferocitica , Sferocitosi ereditaria.
Anemia en el recién nacido
Gallagher PG, Glader B. Hereditary spherocytosis, hereditary elliptocytosis, and other disorders associated with abnormalities of the erythrocyte membrane. En: Wintrobe's Clinical Hematology. The red blood cell membrane and its disorders: Hereditary spherocytosis, elliptocytosis, and related diseases. New York: McGraw-Hill;
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Red cell membrane protein deficiencies in Mexican patients with hereditary spherocytosis. Blood Cells Mol Dis ; 31 3 : Additional erythrocytic and reticulocytic parameters helpful for diagnosis of hereditary spherocytosis: results of a multicentre study. Ann Hematol ; 90 7 : Guidelines for the diagnosis and management of hereditary spherocytosis update. Br J Haematol ; 1 : Delaunay J.
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Publication date Print : April Hereditary spherocytosis must always be suspected in children with anemia, hyperbilirubinemia, splenomegaly or cholelithiasis, in the asymptomatic individual with an affected relative, and in the neonate with hyperbilirubinemia with no blood group incompatibility; its early detection is key to avoid kernicterus. Follow-up of these patients is based on periodical control and supply of information on the adequate management of hemolytic or aplastic crisis, and early detection of cholelithiasis. The decision to perform splenectomy is usually associated with quality of life rather than life-threatening risk, and it should result from a consensus between patient, parents and physicians. The postsplenectomy follow-up is based on control of compliance with the prophylactic antibiotic therapy and the early diagnosis of infectious disorders. Data availability:. ScienceOpen disciplines: Pediatrics.
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Revista Cubana Hematol Inmunol Hemoter ;18 1 The Italian survey on hereditary spherocytosis. Int J Pediatr Hematol Oncol ; 2: 43— Blood Cells Mol Dis ; — Erythroid membrane protein defects in hereditary spherocytosis.