Cargando…

Mechanisms of Bone Impairment in Sickle Bone Disease

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 patho...

Descripción completa

Detalles Bibliográficos
Autores principales: Giordano, Paola, Urbano, Flavia, Lassandro, Giuseppe, Faienza, Maria Felicia
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7918363/
https://www.ncbi.nlm.nih.gov/pubmed/33668588
http://dx.doi.org/10.3390/ijerph18041832
_version_ 1783657905358110720
author Giordano, Paola
Urbano, Flavia
Lassandro, Giuseppe
Faienza, Maria Felicia
author_facet Giordano, Paola
Urbano, Flavia
Lassandro, Giuseppe
Faienza, Maria Felicia
author_sort Giordano, Paola
collection PubMed
description 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.
format Online
Article
Text
id pubmed-7918363
institution National Center for Biotechnology Information
language English
publishDate 2021
publisher MDPI
record_format MEDLINE/PubMed
spelling pubmed-79183632021-03-02 Mechanisms of Bone Impairment in Sickle Bone Disease Giordano, Paola Urbano, Flavia Lassandro, Giuseppe Faienza, Maria Felicia Int J Environ Res Public Health Review 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. MDPI 2021-02-13 2021-02 /pmc/articles/PMC7918363/ /pubmed/33668588 http://dx.doi.org/10.3390/ijerph18041832 Text en © 2021 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/).
spellingShingle Review
Giordano, Paola
Urbano, Flavia
Lassandro, Giuseppe
Faienza, Maria Felicia
Mechanisms of Bone Impairment in Sickle Bone Disease
title Mechanisms of Bone Impairment in Sickle Bone Disease
title_full Mechanisms of Bone Impairment in Sickle Bone Disease
title_fullStr Mechanisms of Bone Impairment in Sickle Bone Disease
title_full_unstemmed Mechanisms of Bone Impairment in Sickle Bone Disease
title_short Mechanisms of Bone Impairment in Sickle Bone Disease
title_sort mechanisms of bone impairment in sickle bone disease
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7918363/
https://www.ncbi.nlm.nih.gov/pubmed/33668588
http://dx.doi.org/10.3390/ijerph18041832
work_keys_str_mv AT giordanopaola mechanismsofboneimpairmentinsicklebonedisease
AT urbanoflavia mechanismsofboneimpairmentinsicklebonedisease
AT lassandrogiuseppe mechanismsofboneimpairmentinsicklebonedisease
AT faienzamariafelicia mechanismsofboneimpairmentinsicklebonedisease