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Arch Pediatr. 2008 Sep;15(9):1464-73. doi: 10.1016/j.arcped.2008.04.023. Epub 2008 Jun 16.

[Hereditary spherocytosis: guidelines for the diagnosis and management in children].

[Article in French]

Author information

  • 1Service de pédiatrie générale, hôpital de Bicêtre, AP-HP, Le-Kremlin-Bicêtre, France. corinne.guitton@bct.aphp.fr

Abstract

Hereditary spherocytosis (HS) is the commonest inherited disorder of the erythrocyte membrane in Northern Europe and North America. It is marked by a regenerative anemia which varies widely from asymptomatic patients to severe hemolysis. In 75% of HS patients, inheritance is autosomal dominant. The diagnosis of HS is easily made when there are a family history, hemolytic anemia, reticulocytosis, spherocytes and increased hyperdense cells. Specialized testing to clarify the nature of membrane disorder is required when the film appearance is atypical without a positive family history, in the absence of a family history, in the newborn and before the splenectomy, to rule out the stomatocytosis which is contraindicated. The indication for splenectomy is dependent on the degree of anemia and its clinical manifestation.

PMID:
18556182
[PubMed - indexed for MEDLINE]
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