ESFEROCITOSIS HEREDITARIA PDF

The Spanish Association of Pediatrics has as one of its main objectives the dissemination of rigorous and updated scientific information on the different areas of pediatrics. Annals of Pediatrics is the Body of Scientific Expression of the Association and is the vehicle through which members communicate. The Impact Factor measures the average number of citations received in a particular year by papers published in the journal during the two receding years. CiteScore measures average citations received per document published. Read more.

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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. Read this article at SciELO. There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience. Journal Journal ID publisher : aap. Abbreviated Title: Arch. ISSN: DOI: Comment on this article Sign in to comment.

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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:.

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2012, Número 4

Skip to search form Skip to main content You are currently offline. Some features of the site may not work correctly. La esferocitosis hereditaria es la anemia hemolitica congenita mas frecuente en la poblacion caucasica. Tiene una amplia variabilidad clinica y desde el punto de vista hematologico se caracteriza por anemia y presencia de esferocitos en la lamina periferica. Su base fisiopatologica esta determinada por el defecto de algunas de las proteinas que conforman la membrana eritrocitaria, por el efecto del bazo sobre los hematies anomalos y otros factores.

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Esferocitosis hereditaria: de la biogénesis a la patogénesis.

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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