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...
Autores principales: | , , , |
---|---|
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 |