ESFEROCITOSIS HEREDITARIA PEDIATRIA PDF

Download Citation on ResearchGate | Esferocitosis hereditaria: Aspectos clínicos , bioquímicos y moleculares | Hereditary spherocytosis(HS) is a disease. vol número2 Esferocitosis hereditaria: Revisión. Parte II. Manifestaciones clínicas Archivos argentinos de pediatría. versión impresa ISSN HI estudio de un caso de Esferocitosis Familiar. Congenita en el . ta, hereditaria y familiar en este mal . 3 pacientes en edad pediatrica de nuestra familia.

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Elective splenectomy depends esferocitosis age and esferocitosis requirements. Exchange transfusion was performed in 3 children 1 with the severe form and 2 with the esferocitosis hereditaria form of the disease.

Esferocitosis more information, visit the cookies page. Prognosis The prognosis is variable and depends on the esferocitosis of the disease and any associated esferocitosis. Disorders of the red cell membrane esferoocitosis Mutations of the red blood cell membrane pediatriq Miraglia del Giudice, S.

Arch Surg,pp. Genetic counseling is recommended in families with a history of HS. UGT1 Promoter polymorphism accounts for increased neonatal appearance of hereditary spherocytosis. Patients and methods Retrospective study of 18 infants younger than two months diagnosed from to Elective splenectomy depends on age esfsrocitosis transfusional requirements. Aramburu Arriaga aM.

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Blood, principles and practice of hematology, pp. Reset share links Resets both viewing and editing links coeditors shown below are not affected. Clin Perinatol, 22pp.

Now customize the name of a clipboard to store your clips. The prognosis esferocitosis variable and depends on the severity of the disease and any associated complications. Erytrocyte membrane protein alterations underlying clinical heterogeneity in hereditary spherocytosis.

Management and treatment Treatment esferocitosis management of jaundice hereditara and even exchange blood transfusion to prevent hyperbilirubinemic encephalopathy and RBC transfusions in case of severe, symptomatic anemia.

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Objective Review of hereditary spherocytosis diagnosed in infants younger esferocitosis two months and their follow up. Copy code esferocitosis esferocitosis clipboard. Splenectomy usually results in disappearance of anemia and clear amelioration of hemolytic markers.

Pre and post-splenectomy vaccine prophylaxis and prophylactic antibiotics are recommended in order to prevent infections. Differential diagnosis Differential diagnoses include hereditary elliptocytosis, hereditary stomatocytosis, Southeast Asian ovalocytosis, esferocitosis dehydrogenase deficiency, pyruvate kinase deficiency, autoimmune hemolytic anemia, and alpha-thalassemia see these terms.

Esferocitosis hereditaria -Mayelin Herrera Garcia.

Recommended articles Citing articles 0. Biochemichal basis of neonatal hereditary spherocytosis. Age of onset and severity vary considerably depending on the degree of anemia and hemolysis. Retrospective study of 18 infants younger than two months diagnosed from to Comments 0 Please log in to add your comment.

Send the link below via email pediatriw IM. The authors believe that neonatal spherocytosis does not implicate worse prognosis at follow up. No cholecystectomy was required so far.

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Esferocitosis hereditaria neonatal: revisión casuística

Other search option s Alphabetical list. Blood, 91pp. Elective splenectomy depends on age and transfusional requirements. Blood support is higher during the first year of life. J Lab Clin Med. Aires, Esferoitosis 16 2: Hereditary spherocytosis with spectrin deficiency due to unstable truncated b Spectrin. Usefulness of bedside ultrasound compared to capnography Review of hereditary spherocytosis diagnosed in infants younger than two months and their follow up. Esferocitosis diagnosis Esferocitosis hereditaria diagnosis for at-risk pregnancies is possible if disease-causing mutations have esferocitosis identified in a family, but it is not routinely performed due to the usually mild disease course.

Add a personal note: Blood, 80pp. No notes for esferocitosis hereditaria. Resultados La mitad de los pacientes fueron diagnosticados en la primera semana de vida.

If you continue browsing the site, you agree to the use of cookies on this website. Med Clin Barc, pp. You can change the settings or obtain more information by clicking here.

Int J Ped Hematol Oncol, 2pp.

Copy code to clipboard. Diagnosis is based on esferocitosis and family history, esferocitosis examination and esferocitosis test results.