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Pediatr Blood Cancer. 2013 Oct;60(10):E125-7. doi: 10.1002/pbc.24588. Epub 2013 May 15.

Association between baseline fetal hemoglobin levels and incidence of severe vaso-occlusive pain episodes in children with sickle cell anemia.

Author information

1
McKusick-Nathans Institute of Genetic Medicine, School of Medicine, Johns Hopkins University, Baltimore, MD 21205, USA.

Abstract

The ameliorating effect of high fetal hemoglobin (HbF) levels on the incidence of pain episodes in sickle cell anemia (SCA) is well-known; however, in children this relationship is less clearly established. We hypothesized that higher HbF levels in children with SCA are associated with fewer severe pain episodes. A meta-analysis of data from the Silent Infarct Transfusion Trial (n = 456) and the Cooperative Study of Sickle Cell Disease (n = 764), demonstrated that baseline HbF levels were associated with the incidence of severe pain, commonly defined across studies as an event requiring hospitalization (P-value = 0.02).

KEYWORDS:

fetal hemoglobin; sickle cell anemia; vaso-occlusive pain

PMID:
23677903
PMCID:
PMC4387562
DOI:
10.1002/pbc.24588
[Indexed for MEDLINE]
Free PMC Article

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