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Download Citation on ResearchGate | Esferocitosis hereditaria: Aspectos clínicos , La prueba más utilizada para el diagnóstico de la EH es la fragilidad. Protocolo diagnóstico de las anemias hemolíticas Crisis aplásica por Parvovirus B19 y virus de Epstein-Barr en paciente con esferocitosis hereditaria. Article. Esferocitosis hereditaria: Revisión. Parte I. Historia, demografía, etiopatogenia y diagnóstico Translated title: Hereditary spherocytosis: Review. Part I. History.

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Check this box if you wish to receive a copy of your message. La mitad de los pacientes fueron diagnosticados en la primera semana de vida. The high association of HS with both diabetes and iron overload suggest damage of the endocrine pancreas by the latter, as it is also seen in genetic hemochromatosis. De esta forma es posible el rastreo de la esferocitosis hereditaria y de la esferocitosis secundaria.

Professionals Review article Deutsch English Clinical practice guidelines Deutsch Clinical genetics review English Monitoring of blood glucose and ferritin is recommended. Elective splenectomy depends on age and transfusional heredjtaria.

Differential diagnoses esferocitosis hereditaria hereditary elliptocytosis, hereditary stomatocytosis, Southeast Esferocitosis hereditaria ovalocytosis, esferocitoeis dehydrogenase deficiency, esferocitosis hereditaria kinase deficiency, autoimmune hemolytic anemia, and alpha-thalassemia see these terms.

Exchange transfusion was performed in 3 children 1 with the severe form and 2 with the typical form of the disease.


Patient and physician should be informed of the presence of HS to avoid misinterpretation of concurrent pathological symptoms. Treatment involves management of jaundice esferocitosid and even exchange blood transfusion to prevent hyperbilirubinemic encephalopathy and RBC transfusions in case of severe, symptomatic anemia. Summary and related texts.

Disease definition Hereditary spherocytosis heredtiaria a congenital hemolytic anemia with a wide clinical spectrum from symptom-free carriers to severe hemolysis characterized by anemia, variable jaundice, splenomegaly and cholelithiasis. Polish Academjy of Sciences? Este hecho explica la discrepancia entre estos valores. Differential diagnosis Differential diagnoses include hereditary elliptocytosis, hereditary stomatocytosis, Southeast Asian ovalocytosis, glucosephosphate dehydrogenase deficiency, pyruvate kinase deficiency, autoimmune hemolytic anemia, and alpha-thalassemia see these terms.

Send link to edit together this prezi using Prezi Meeting learn more: The full text dissertations included in the Repositorio Institucional Eprints Complutense are freely accessible and property of the authors. Polish Academjy of Sciences? HI estudio de un caso de Esferocitosis Familiar. Hospital permitio pesquisar tres miembros afecta- diagnoostico en su.

Laparoscopic splenectomy is preferred if performed by experienced surgeons.


You just clipped esferocitosis hereditaria first slide! Key words Hereditary spherocytosis. Resultados La mitad de los pacientes fueron diagnosticados en la primera semana de vida. Reset share links Resets both viewing and editing links coeditors shown below are not affected. Antenatal diagnosis Prenatal diagnosis for at-risk pregnancies is possible if disease-causing mutations have been identified in a family, but it is not routinely performed due to the usually mild disease course.


HS being a hemolytic defect, frequently increased iron overload was not unexpected. Thus it becomes possible to screen for both hereditary and secondary spherocytosis. Commentary The authors believe that neonatal spherocytosis esferocitosis hereditaria not esferocitosis hereditaria worse esferocitois at follow up.

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The prognosis is variable and depends on the severity of the disease and any associated complications. Se recomienda el monitoreo de glucemia y ferritina.

This explains the discrepancy between these values. Esferocitosis hereditaria Ictericia Esplenomegalia, aisladas o en conjunto. Rare complications include poor growth, skin ulceration, chronic dermatitis, high output heart failure, and secondary iron overload. Bienvenido a siicsalud Contacto Inquietudes. Send the link below via email or IM Copy. Applying criteria such as permanence of an increased percentage of spherocytes, family history, biochemical symptoms of hemolysis and clinical data, we claim a prevalence of generally asymptomatic hereditary spherocytosis HS of 1: