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[Hereditary spherocytosis in neonates. Review of our caseload]
N Aramburu Arriaga1, M A Fernández Cuesta, M J Martínez González
1Departamento de Pediatría. Hospital de Cruces. Baracaldo. Vizcaya.
Insights
Hereditary spherocytosis in infants under two months does not worsen prognosis. Blood support needs are highest in the first year, with splenectomy based on age and transfusion needs.
Area of Science:
- Hematology
- Pediatric Medicine
- Genetics
Context:
- Hereditary spherocytosis (HS) is a genetic hemolytic anemia.
- Early diagnosis in infants is crucial for management.
- This study reviews HS cases diagnosed in neonates.
Purpose:
- To review the diagnosis and follow-up of hereditary spherocytosis in infants under two months old.
- To analyze clinical presentation, management, and outcomes.
Summary:
- This retrospective study analyzed 18 infants diagnosed with HS before two months of age.
- Anemia was universal; hyperbilirubinemia was less common but presented earlier.
- Most cases followed an autosomal dominant inheritance pattern.
- Blood support and hemolytic crises were most frequent in the first year.
- Splenectomy was performed in severe/typical forms at a mean age of 8.25 years.
Impact:
- Neonatal HS diagnosis does not predict a worse long-term prognosis.
- Understanding early disease patterns guides timely interventions.
- Management strategies, including splenectomy, are age and transfusion-dependent.
Aim:
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 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.
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