Mechanisms of Bone Impairment in Sickle Bone Disease

Paola Giordano, Flavia Urbano, Giuseppe Lassandro and Maria Felicia Faienza
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Paola Giordano: Paediatric Unit, Department of Biomedical Sciences and Human Oncology, University of Bari “A. Moro”, 70124 Bari, Italy
Flavia Urbano: Paediatric Unit, Department of Biomedical Sciences and Human Oncology, University of Bari “A. Moro”, 70124 Bari, Italy
Giuseppe Lassandro: Paediatric Unit, Department of Biomedical Sciences and Human Oncology, University of Bari “A. Moro”, 70124 Bari, Italy
Maria Felicia Faienza: Paediatric Unit, Department of Biomedical Sciences and Human Oncology, University of Bari “A. Moro”, 70124 Bari, Italy

IJERPH, 2021, vol. 18, issue 4, 1-11

Abstract: Sickle bone disease (SBD) is a chronic and invalidating complication of Sickle cell disease (SCD), a multisystem autosomal recessive genetic disorder affecting millions of people worldwide. Mechanisms involved in SBD are not completely known, especially in pediatric age. Among the hypothesized pathogenetic mechanisms underlying SBD are bone marrow compensatory hyperplasia and bone ischemic damage, both secondary to vaso-occlusive crisis (VOC), which leads to cell sickling, thus worsening local hypoxia with a negative impact on osteoblast recruitment. Furthermore, the hypoxia is a strong activator of erythropoietin, which in turn stimulates osteoclast precursors and induces bone loss. Hemolysis and iron overload due to a chronic transfusion regimen could also contribute to the onset of bone complications. Vitamin D deficiency, which is frequently seen in SCD subjects, may worsen SBD by increasing the resorptive state that is responsible for low bone mineral density, acute/chronic bone pain, and high fracture risk. An imbalance between osteoblasts and osteoclasts, with a relative decrease of osteoblast recruitment and activity, is a further possible mechanism responsible for the impairment of bone health in SCD. Moreover, delayed pubertal growth spurt and low peak bone mass may explain the high incidence of fracture in SCD adolescents. The aim of this review was to focus on the pathogenesis of SBD, updating the studies on biochemical, instrumental, and biological markers of bone metabolism. We also evaluated the growth development and endocrine complications in subjects affected with SCD.

Keywords: sickle cell disease; bone; metabolism; growth; endocrine complications (search for similar items in EconPapers)
JEL-codes: I I1 I3 Q Q5 (search for similar items in EconPapers)
Date: 2021
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