[Hereditary spherocytosis in neonates. Review of our caseload].
OBJECTIVE
Review of hereditary spherocytosis diagnosed in infants younger than two months and their follow up.


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 dominant in 94% of the cases. Anaemia was observed in all the patients and hyperbilirubinemia in only 44%, although the latter was the clinical presentation in patients diagnosed 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.


CONCLUSIONS
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.
Aramburu Arriaga
N
N
Departamento de Pediatría. Hospital de Cruces. Baracaldo. Vizcaya.
Fernández Cuesta
M A
MA
Martínez González
M J
MJ
Astigarraga Aguirre
I
I
Fernández-Teijeiro Alvarez
A
A
Navajas Gutiérrez
A
A
Piñán Frances
M A
MA
D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D012189:Retrospective Studies; D013103:Spherocytosis, Hereditary / Q000175:diagnosis*