Bone and Joint Disease in Sickle Cell Disease

Aguilar, Christine; Vichinsky, Elliott; Neumayr, Lynne

doi:10.1016/j.hoc.2005.07.001

Cited by 61 publications

(44 citation statements)

References 61 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…The pathology of SCD disturbs the metabolic activity and damages the structural integrity of tissues throughout the body, resulting in sporadic and acute pain most commonly affecting bone and joints [Aguilar et al, 2005]. Through conventional radiology, it is widely accepted that SCD in bone results in widening of the bone marrow cavity, along with a decrease in bone thickness and mineral content; however, the morphology of sickle bone at the microscale and its contribution mechanically is still largely uncharacterized [Almeida and Roberts, 2005].…”

Section: Discussionmentioning

confidence: 99%

Microarchitectural and mechanical characterization of the sickle bone

Green

Akinsami

Lin

et al. 2015

Journal of the Mechanical Behavior of Biomedical Materials

View full text Add to dashboard Cite

Individuals with sickle cell disease often experience acute and chronic bone pain due to occlusive events within the tissue vasculature that result in ischemia, necrosis, and organ degeneration. Macroscopically, sickle bone is identified in clinical radiographs by its reduced mineral density, widening of the marrow cavity, and thinning of the cortical bone due to the elevated erythroid hyperplasia accompanying the disease. However, the microstructural architecture of sickle bone and its role in mechanical functionality is largely unknown. This study utilized micro-CT and biomechanical testing to determine the relationship between the bone morphology, tissue mineral density, and trabecular and cortical microarchitecture of 10-and 21-week-old femurs from transgenic sickle male mice and littermates with sickle trait, as well as a wild-type control. While bone tissue mineral density did not vary among the genotypes at either age, variation in bone microstructure were observed. At 10 weeks, healthy and trait mice exhibited similar morphology within the cortical and trabecular bone, while sickle mice exhibited highly connected trabeculae. Within older femurs, sickle and trait specimens displayed significantly fewer trabeculae, and the remaining trabeculae had a more deteriorated geometry based on the structure model index. Thinning of the cortical region in sickle femurs contributed to the displayed flexibility with a significantly lower elastic modulus than the controls at both 10- and 21-weeks old. Wild-type and trait femurs generally demonstrated similar mechanical properties; however, trait femurs had a significantly higher modulus than sickle and wild-type control at 21-weeks. Overall, these data indicate that the progressive damage to the microvasculature caused by sickle cell disease, results in deleterious structural changes in the bone tissue's microarchitecture and mechanics.

show abstract

Section: Discussionmentioning

confidence: 99%

Microarchitectural and mechanical characterization of the sickle bone

Green

Akinsami

Lin

et al. 2015

Journal of the Mechanical Behavior of Biomedical Materials

View full text Add to dashboard Cite

show abstract

“…Though distinct in presentation, both groups exhibit similar risk factors for low bone mass: delayed growth and pubertal development [10,11], milk avoidance, lactose intolerance, bone forming nutritional deficiencies (Calcium, Vit D, Zinc) [12,13,14], as well as depressed serum 25-OH vitamin D [15,16]. Additionally chronic hemolytic anemia or ineffective erythropoiesis leads to widening of medullary cavities and intratrabecular spaces and decreased cortical thickness [17,18]. Both groups also have a number of factors which may prove protective against fracture: severe anemia leads to decreased physical activity and fewer opportunities for recreational fractures [19]; patients with sickle cell disease who experience hypoxic episodes tend to have more pain and are therefore less apt to exercise; and parental overprotection may decrease exposure of the chronically ill child to fall risk.…”

Section: Introductionmentioning

confidence: 99%

Fracture prevalence and relationship to endocrinopathy in iron overloaded patients with sickle cell disease and thalassemia

Fung¹,

Harmatz²,

Milet³

et al. 2008

Bone

Self Cite

View full text Add to dashboard Cite

Transfusional iron overload leads to gonadal failure and low bone mass in patients with thalassemia (Thal). However, gonadal failure is rarely reported in transfused patients with sickle cell disease (SCD) and the literature regarding fracture prevalence in SCD is limited. The objective of this study was to assess self-reported fracture prevalence and its relationship to endocrinopathy in transfused Thal or SCD subjects and compare to non-transfused subjects with SCD (NonTxSCD). Eligibility was based on age ≥12 years and liver iron concentration ≥ 10 mg/g dry wt or serum ferritin ≥ 2000 ng/mL (Thal or TxSCD) or for NonTxSCD, ferritin < 500 ng/mL. Data were collected by patient interview and chart review at 31 clinical centers in the U.S., Canada and the U.K. 152 subjects with Thal (52% Male; 25.6±0.7 yrs), 203 subjects with TxSCD (44% Male, 24.7 ±0.9 years: Mean ± SE), and 65 NonTxSCD (50% Male, 22.2 ±1.3 yrs) were enrolled. Overall, male subjects with Thal were more likely to have sustained a fracture in their lifetime (51%) compared to TxSCD (28%) or NonTxSCD (32%) (p=0.005). There was no difference in fracture prevalence among women (Thal: 26%, TxSCD 17%, NonTxSCD: 16%). Fracture was most frequently reported in the upper extremities (53.3% of all fractures) while spine and pelvic fractures were relatively common for such a young cohort: 10.6%. Though overall fracture prevalence was not distinctly different from published healthy cohorts, fewer fractures occurred during the adolescent years. In multivariate analysis, the significant predictors of fracture prevalence were Thal diagnosis (Odds Ratio: 2.3; 1.2-4.6; 95%CI), male gender (OR: 2.6; 1.5-4.5), hypothyroidism (OR: 3.3; 1.1-9.8) and age (OR: 1.1; 1.03-1.08). These data suggest that despite similar iron burden, transfused patients with Thal are at greater risk for fracture than subjects with SCD. Male subjects with Thal and hypothyroidism are at particular risk for fracture, in contrast, transfused subjects with SCD had no greater risk of fracture compared to non-transfused SCD. Though ethnic differences in fracture risk cannot be ignored, endocrinopathy is rare in TxSCD which may also provide some protection from fracture.

show abstract

“…In sickle cell disease, ß-globin mutation alters the red blood cell shape to become sickled shaped under low oxygen tension [22]. It is postulated that increased adhesiveness of the sickled red cells make them more likely to become static during the transit through the microvasculature and cause microvascular occlusions.…”

Section: Pathology and Pathophysiology Of Juvenile Fhomentioning

confidence: 99%

Childhood Femoral Head Osteonecrosis

Kim

Larson

Fletcher

et al. 2011

Clinic Rev Bone Miner Metab

View full text Add to dashboard Cite

Femoral head osteonecrosis (FHO) is potentially a devastating condition of the hip joint that can affect children and adults. The most serious outcome of this condition is the development of painful progressive osteoarthritis necessitating a total hip replacement at a relatively young age. This review focuses on childhood FHO with a special attention to the conditions that are more commonly seen in the pediatric population: Legg-CalvePerthes disease, corticosteroid-associated FHO, and sickle cell disease.

show abstract

Bone and Joint Disease in Sickle Cell Disease

Cited by 61 publications

References 61 publications

Microarchitectural and mechanical characterization of the sickle bone

Microarchitectural and mechanical characterization of the sickle bone

Fracture prevalence and relationship to endocrinopathy in iron overloaded patients with sickle cell disease and thalassemia

Childhood Femoral Head Osteonecrosis

Contact Info

Product

Resources

About