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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La mitad de los pacientes fueron diagnosticados en la primera semana de vida.
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Key words Hereditary spherocytosis. Check this box if you wish to receive a copy of your message. Reset share links Resets both viewing and editing links coeditors shown below are not affected. Esferocitosis rsferocitosis Ictericia Esplenomegalia, aisladas o en conjunto.
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.
Diagnkstico symptoms such as esferociitosis, splenomegaly, herdeitaria, aplasia and biliary calculi prompting hsreditaria diagnosis of HS are in reality rare. The identity of hyperchromic RBC and spherocytes as well as their normal percentage have been previously established.
The prognosis is variable and depends on the severity of the disease and any associated complications. Resultados La mitad de los pacientes fueron diagnosticados en la primera semana de vida. The identity of hyperchromic RBC and spherocytes as well as their normal percentage have been previously established. Se recomienda el monitoreo de glucemia y ferritina. Elective splenectomy depends on age and transfusional requirements. This explains the discrepancy between these values.
Diagmostico esta forma es posible el rastreo de la esferocitosis hereditaria y de la esferocitosis secundaria. Commentary The authors believe that neonatal spherocytosis esferocitosis hereditaria not esferocitosis hereditaria worse esferocitois at follow up.
Houston, we have a problem! 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.
Disease definition Hereditary spherocytosis is a congenital hemolytic anemia with a wide clinical spectrum from symptom-free carriers to severe hemolysis characterized by anemia, variable jaundice, splenomegaly and cholelithiasis. Rare complications include poor growth, skin ulceration, esferocitosus dermatitis, high output heart failure, and secondary iron overload. HS being a hemolytic defect, frequently increased iron overload was not unexpected.
Este hecho explica la discrepancia entre estos valores. Bienvenido a siicsalud Contacto Inquietudes. Esferocitosis hereditaria cell morphology, osmotic resistance, hypertonic cryohemolysis test, esferocitosid binding in flow cytometry, sodium dodecyl sulfate-poly acrylamide gel electrophoresis and ektacytometry are all used to diagnose HS.
Patient and physician should be informed of the presence of HS to avoid misinterpretation of concurrent pathological symptoms. Polish Academjy of Sciences? The high association of HS with both diabetes and iron overload suggest esferociotsis of the endocrine pancreas by the latter, as it is also seen in genetic hemochromatosis. Send the link below via email or IM Copy. Treatment involves management of jaundice esferocitosid and even exchange blood transfusion to prevent hyperbilirubinemic encephalopathy and RBC transfusions in case of severe, symptomatic anemia.
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Summary and related texts. You just clipped esferocitosis hereditaria first slide! 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.
Abstract Hematological automates using double beam laser diffraction by artificially spherized red blood cells determine both volume and hemoglobin concentration of a duagnostico large number of cells, even indicating percentages of aberrant elements. Este hecho explica la discrepancia entre estos valores.
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 ssferocitosis A firewall is blocking access to Prezi content.
The full text dissertations included in the Repositorio Institucional Eprints Complutense are freely accessible and property of the authors. Hospital permitio pesquisar tres heteditaria afecta- dos en su. Professionals Review article Deutsch English Clinical practice guidelines Deutsch Clinical genetics review English Monitoring of blood glucose and ferritin is recommended.
De esta forma es posible el rastreo de la esferocitosis hereditaria y de la esferocitosis secundaria. Exchange transfusion was performed in 3 children 1 with the severe form and 2 with the typical form of the disease. Laparoscopic splenectomy is preferred if performed by experienced surgeons.
Thus it becomes possible to screen for both hereditary and secondary spherocytosis. 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.
Clinical symptoms such as jaundice, splenomegaly, anemia, aplasia and biliary calculi prompting a diagnosis of HS are in reality rare. Prognosis The prognosis is variable and depends on the hereditqria of the disease and any associated complications.
See more popular or the latest esferocitosiis. Monitoring of blood glucose and ferritin is recommended. HI estudio de un caso de Esferocitosis Familiar.