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Published on: November 5, 2019
Occult ischemic bone lesions in children with sickle cell disease: A study of prevalence
Vincenzo Voi1, Silvia Turrini2, Margherita Mattavelli3
1Department of Clinical and Biological Science, School of Medicine, Centre for Hemoglobinopathies, AOU San Luigi Gonzaga, University of Turin, Turin, Italy.
Introduction:
Avascular necrosis (AVN) is a severe complication of sickle cell disease (SCD) and involves principally the femoral head. Few data exist about the prevalence of lesions in other segments.
Methods:
In this cross-sectional study, 42 children (20 males and 22 females) underwent a magnetic resonance imaging (MRI) of the spine, upper arms, and femurs. The primary outcome was to define the prevalence and locations of bone infarcts.
Results:
Forty-two patients completed the study; the total median age was 11.9 years (interquartile range = 9.5-13.9). Eleven patients (26.2%) were positive for altered bone findings for a total of 32 lesions. Most of the lesions were in the humerus 17 (53.1%), 11 (34.4%) in the femurs, and 4 (12.5%) in the vertebrae. The median number of vaso-occlusive crises (VOCs) was two and four for patients without and with bone lesions, respectively (p = .01). The annual rate of VOC is the best marker for the positive MRI (odds ratio = 82.6; p = .03), and it is correlated with the number of sites involved (p = .02).
Conclusions:
Our study highlights that the prevalence of skeletal lesions could be underestimated, and it provides the basis for clinical reasoning and tailored therapy in SCD children.
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