Esferocitosis hereditaria neonatal: revisión casuística
- N. Aramburu Arriaga
- M.A. Fernández Cuesta
- M.J. Martínez González
- I. Astigarraga Aguirre
- A. Fernández-TeijeiroÁlvarez
- A. Navajas Gutiérrez
- M.A. Piñán Frances
Year of publication: 2000
Volume: 52
Issue: 6
Pages: 569-572
Type: Article
Sustainable development goals
Abstract
Objective Review of hereditary spherocytosis diagnosed in infants younger than two months and their follow up. Patients and methods Retrospective study of 18 infants younger than two months diagnosed from 1973 to 1995. Results Diagnosis was established in the first week of life in 50% of the patients. Hereditary pattern was autosomic domi-nant in 94% of the cases. Anaemia was observed in all the patients and hyperbilirrubinemia in only 44%, although the latter was the clinical presentation in patients diagno-sed at younger age. Exchange transfusion was performed in 3 children (1 with the severe form and 2 with the typical form of the disease). During the first 6 months of age, 55% of infants presented hemolytic crises that required transfusion in 91% of them. Both periodicity of crises and transfusions decreased to 38 and 44% respectively after the first year. Splenectomy was performed in the 3 children with severe forms and in 6 with typical forms (mean age 8 years and 3 months). No cholecystectomy was required so far. Commentary The authors believe that neonatal spherocytosis does not implicate worse prognosis at follow up. Blood support is higher during the first year of life. Elective splenectomy depends on age and transfusional requirements.