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Revista chilena de pediatría

versión impresa ISSN 0370-4106


VALERO J, MIGUEL; VERDUGO L, PATRICIA; TORDECILLA C, JUAN  y  RODRIGUEZ Z, NATALIE. Microespherocytosis in Pediatrics. Rev. chil. pediatr. [online]. 2010, vol.81, n.4, pp.319-325. ISSN 0370-4106.

Microespherocitosis is the most common hereditary anemia. Clinically it is characterized for hemolytic anemia, with jaundice and transfusion requirements, symptoms can be more intense in newborn period. With the purpose of review this disease, we studied the patients of the pediatric hematology policlinic in Roberto del Río Hospital, controlled between 1990 to 2005, we check the patient's records and registered the clinic and family background evaluating through the Eber's severity classification the indication of splenectomy in each case. We registered 44 patients. The 68.2%) had family background. The 11.3% presented symptoms during the newborn period, in which 91.2% had jaundice. The 38.6%> needed at least one transfusion after the newborn period. The Eber's severity classification was different according to the variable evaluated, which made its application difficult. Only one of the nine splenectomized patient had this indication. From the year 2000, the inquiry of the disease as well as the correct indication of splenectomy has improved. This coincides with the appearance of the Eber's publication about this disease. Conclusion: Microespherocitosis usually appears in the newborn period as jaundice and most patients have familial antecedents. Eber's severity classification and molecular study allows a rational splenectomy indication and predicts the disease evolution.

Palabras clave : Anemia; microespherocitosis; splenectomy; transfusion.

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