Bone Mineral Density Z-score Research Articles

The dose-dependent effect of estrogen on bone mineral density in trans girls.

Treatment in transgender girls can consist of puberty suppression (PS) with a gonadotropin-releasing hormone agonist (GnRHa) followed by gender-affirming hormonal treatment (GAHT) with estrogen. Bone mineral density (BMD) Z-scores decrease during PS and remain relatively low during GAHT, possibly due to insufficient estradiol dosage. Some adolescents receive high-dose estradiol or ethinyl estradiol (EE) to limit growth allowing comparison of BMD outcomes with different dosages. Retrospective study. Adolescents treated with GnRHa for ≥1 year prior to GAHT followed by treatment with a regular estradiol dose (gradually increased to 2 mg), 6 mg estradiol or 100-200 µg EE were included to evaluate height-adjusted BMD Z-scores (HAZ scores) on DXA. Eighty-seven adolescents were included. During 2.3 ± 0.7 years PS, lumbar spine HAZ scores decreased by 0.69 [95% confidence interval (CI) -0.82 to -0.56)]. During 2 years HT, lumbar spine HAZ scores hardly increased in the regular group (0.14, 95% CI -0.01 to 0.28, n = 59) vs 0.42 (95% CI 0.13 to 0.72) in the 6 mg group (n = 13), and 0.68 (95% CI 0.20 to 1.15) in the EE group (n = 15). Compared with the regular group, the increase with EE treatment was higher (0.54, 95% CI 0.05 to 1.04). After 2 years HT, HAZ scores approached baseline levels at start of PS in individuals treated with 6 mg or EE (difference in 6 mg group -0.20, 95% CI -0.50 to 0.09; in EE 0.17, 95% CI -0.16 to 0.50) but not in the regular group (-0.64, 95% CI -0.79 to -0.49). Higher estrogen dosage is associated with a greater increase in lumbar spine BMD Z-scores. Increasing dosage up to 2 mg estradiol is insufficient to optimize BMD and approximately 4 mg may be required for adequate serum concentrations.

Radiological Features of Zoledronic Acid Therapy in Saudi Children with Osteogenesis Imperfecta

Background: The majority of osteogenesis imperfecta (OI) cases have an autosomal dominant pattern of inheritance and are usually caused by mutations in genes encoding type I collagen. Patients with OI may have blue sclerae, dentinogenesis imperfecta, and growth deficiency. Zoledronic acid is a third-generation bisphosphonate which was reported to inhibit osteoclast-mediated bone resorption, improve bone density, and reduce incidence of fractures. Aim: To describe the radiographic features of cyclic Zoledronic acid administration on the growing skeleton in children with OI and to report the efficacy of Zoledronic acid on bone mineral density. Methods: We retrospectively reviewed the radiographs of 11 children treated with Zoledronic acid. The age of these children ranged from one to 13 years. Zoledronic acid was administrated intravenously at 6 months intervals at the Pediatric Endocrinology Day Medical Unit, King Faisal Specialist Hospital and Research Centre, and Security Forces Hospital, Riyadh, Kingdom of Saudi Arabia for 2 years. Results: During the course of treatment, a gradual increase in bone density was observed. The baseline mean lumbar BMDz score was -4.9SD + 1.3, which improved to a mean score of -2.7SD + 1.1, and a mean whole body BMDz score was -2SD +1.2 and improved to a mean score of -1.1SD + 1.3 by the end of therapy. Post treatment, there were multiple sclerotic metaphyseal bands seen in all children in the long bones paralleling to the growth plates and corresponding to the number of treatment cycles. Conclusion: Intravenous Zoledronic acid in children with osteogenesis imperfecta improved the bone mineral density and resulted in permanent sclerotic metaphyseal bands.

Bone mineral density among children living with HIV failing first-line anti-retroviral therapy in Uganda: A sub-study of the CHAPAS-4 trial.

Children living with perinatally acquired HIV (CLWH) survive into adulthood on antiretroviral therapy (ART). HIV, ART, and malnutrition can all lead to low bone mineral density (BMD). Few studies have described bone health among CLWH in Sub-Saharan Africa. We determined the prevalence and factors associated with low BMD among CLWH switching to second-line ART in the CHAPAS-4 trial (ISRCTN22964075) in Uganda. BMD was determined using dual-energy X-ray Absorptiometry (DXA). BMD Z-scores were adjusted for age, sex, height and race. Demographic characteristics were summarized using median interquartile range (IQR) for continuous variables and proportions for categorical variables. Logistic regression was used to determine the associations between each variable and low BMD. A total of 159 children were enrolled (50% male) with median age (IQR) 10 (7-12) years, median duration of first -line ART 5.2(3.3-6.8) years; CD4 count 774 (528-1083) cells/mm3, weight-for-age Z-score -1.36 (-2.19, -0.65) and body mass index Z-score (BMIZ) -1.31 (-2.06, -0.6). Low (Z-score≤ -2) total body less head (TBLH) BMD was observed in 28 (18%) children, 21(13%) had low lumbar spine (LS) BMD, and15 (9%) had both. Low TBLH BMD was associated with increasing age (adjusted odds ratio [aOR] 1.37; 95% CI: 1.13-1.65, p = 0.001), female sex (aOR: 3.8; 95% CL: 1.31-10.81, p = 0.014), low BMI (aOR 0.36:95% CI: 0.21-0.61, p<0.001), and first-line zidovudine exposure (aOR: 3.68; 95% CI: 1.25-10.8, p = 0.018). CD4 count, viral load and first- line ART duration were not associated with TBLH BMD. Low LS BMD was associated with increasing age (aOR 1.42; 95% CI: 1.16-1.74, p = 0.001) and female sex: (aOR 3.41; 95% CI: 1.18-9.8, p = 0.023). Nearly 20% CLWH failing first-line ART had low BMD which was associated with female sex, older age, first-line ZDV exposure, and low BMI. Prevention, monitoring, and implications following transition to adult care should be prioritized to identify poor bone health in HIV+adolescents entering adulthood.

Reductions in Bone Mineral Density Are Apparent Early in Children With Prevalent Osteonecrosis Lesions Following Leukemia Therapy.

Osteonecrosis (ON) is a serious complication of childhood acute lymphoblastic leukemia. We determined the prevalence of osteonecrotic lesions in our patient population by a one-time multisite magnetic resonance imaging (MRI) more than 1 year following leukemia therapy. MRI findings were evaluated in relationship to clinical factors (including longitudinal changes in bone mineral density [BMD]). Eighty-six children enrolled in the Steroid Associated Osteoporosis in the Pediatric Population (STOPP) study were evaluated for ON at 3.1 ± 1.3 years following therapy. Thirty children had a total of 150 confirmed ON lesions (35%). Lumbar spine (LS) BMD Z-scores (mean ± SD) were low at diagnosis and similar between patients with and without ON (-1.09 ± 1.53 versus -1.27 ± 1.25, p = 0.549). LS BMD Z-scores declined from baseline to 12 months in children with ON (-0.31 ± 1.02) but not in those without (0.13 ± 0.82, p = 0.035); the hip BMD Z-scores from baseline to 24 months declined in both groups, but to a greater extent in those with ON (-1.77 ± 1.22) compared to those without (-1.03 ± 1.07, p = 0.045). At the time of the MRI, mean total hip and total body (TB) BMD Z-scores were lower in children with ON (hip -0.98 ± 0.95 versus -0.28 ± 1.06, p = 0.010; TB -1.36 ± 1.10 versus -0.48 ± 1.50, p = 0.018). Pain occurred in 11/30 (37%) with ON versus 20/56 (36%) without, p = 0.841. In multivariable models, older age at diagnosis (odds ratio [OR] 1.57; 95% confidence interval [CI], 1.15-2.13; p = 0.004), and hip BMD Z-score at MRI (OR 2.23; 95% CI, 1.02-4.87; p = 0.046) were independently associated with ON. Overall, one-third of children demonstrated ON after leukemia therapy. Those with ON had greater reductions in spine and hip BMD Z-scores in the first 1 and 2 years of therapy, respectively. Older age and lower hip BMD Z-scores at MRI were significantly associated with prevalent, off-therapy ON. These data assist in identifying children at risk of ON. © 2023 The Authors. Journal of Bone and Mineral Research published by Wiley Periodicals LLC on behalf of American Society for Bone and Mineral Research (ASBMR).

Trabecular bone score and bone mineral density as indices of skeletal fragility in endogenous Cushing's syndrome.

Endogenous Cushing's syndrome (CS) is a known cause of secondary osteoporosis. Vertebral fractures (VFs) in endogenous CS may occur despite normal bone mineral density (BMD). Trabecular bone score (TBS) is a relatively new, non-invasive technique to assess bone microarchitecture. The objective of our study was to analyse the BMD and bone microarchitecture using TBS in endogenous CS and compare it with a group of age and sex-matched healthy controls, and also analyse the factors predicting BMD and TBS. Cross-sectional study of cases and controls. We included 40 female patients with overt endogenous CS, out of which 32 were adrenocorticotropic hormone (ACTH)-dependent CS and 8 were ACTH-independent. We also included 40 healthy, female controls. Both patients and controls were subjected to an assessment of biochemical parameters and BMD and TBS. Patients with endogenous CS had significantly lower BMD at the lumbar spine, femoral neck, and total hip and significantly lower TBS than healthy controls (all p < .001), while no significant difference was noted in the distal radius BMD (p = .055). In endogenous CS, a large proportion of patients, n = 13 (32.5%) had normal BMD for age (BMD Z-score ≥ -2.0) with low TBS (L1 -L4 TBS ≤ 1.34). TBS correlated negatively with HbA1c (p = .006), and positively with serum T4 (p = .027). TBS should be considered an important complementary tool in addition to BMD for the routine assessment of skeletal health in CS.

An association of speed performance with bone mineral density in national-level Kho-Kho players

Purpose: The study investigated the status of bone mineral density (BMD), serum Vitamin D and calcium and their association with performance outcomes in national-level Kho-Kho players. Methods: Kho-Kho players (n = 52; aged 16–31 years) undergoing training in a national camp were recruited. A lumbar spine (L1–L4) scan was performed using dual-energy X-ray absorptiometry to assess BMD. Blood samples were obtained to assess serum calcium and Vitamin D3 status. The 30-m sprint and Illinois tests were used to estimate speed and agility performance, respectively. The Spearman correlation and linear regression were used to ascertain the association between BMD and performance outcomes. Results: T-score was lower in 22.5% (mean ± standard deviation: 0.32 ± 0.96; range: −2 to +3), and serum Vitamin D3 was lower (&lt;20 ng/ml) in 13.7% and insufficient (&gt;20=&lt;30 ng/ml) in 54.9% of players. No significant correlation between BMD (T-score and Z-score), serum Vitamin D3 and calcium was observed. T-score (r = 0.295, P &lt; 0.05) and Z-score (r = 0.321, P &lt; 0.05) were significantly positively correlated with speed but not with agility. An association was also found between Z-score (standardised-β: 1.38, confidence interval [CI]: 0.34–2.44, P &lt; 0.01) and T-score (standardised-β: 1.49, CI: 0.37–2.62, P &lt; 0.01) and speed. Conclusion: Low BMD and Vitamin D3 deficiency were prevalent amongst Kho-Kho players. Speed performance has an association with BMD.

Low bone mineral density is found in low weight female youth with avoidant/restrictive food intake disorder and associated with higher PYY levels

BackgroundAvoidant/restrictive food intake disorder (ARFID) is a restrictive eating disorder commonly associated with medical complications of undernutrition and low weight. In adolescence, a critical time for bone accrual, the impact of ARFID on bone health is uncertain. We aimed to study bone health in low-weight females with ARFID, as well as the association between peptide YY (PYY), an anorexigenic hormone with a role in regulation of bone metabolism, and bone mineral density (BMD) in these individuals. We hypothesized that BMD would be lower in low-weight females with ARFID than healthy controls (HC), and that PYY levels would be negatively associated with BMD.MethodsWe performed a cross-sectional study in 14 adolescent low-weight females with ARFID and 20 HC 10–23 years old. We assessed BMD (total body, total body less head and lumbar spine) using dual x-ray absorptiometry (DXA) and assessed fasting total PYY concentration in blood.ResultsTotal body BMD Z-scores were significantly lower in ARFID than in HC (− 1.41 ± 0.28 vs. − 0.50 ± 0.25, p = 0.021). Mean PYY levels trended higher in ARFID vs. HC (98.18 ± 13.55 pg/ml vs. 71.40 ± 5.61 pg/ml, p = 0.055). In multivariate analysis within the ARFID group, PYY was negatively associated with lumbar BMD adjusted for age (β = -0.481, p = 0.032).ConclusionOur findings suggest that female adolescents with low-weight ARFID may have lower BMD than healthy controls and that higher PYY levels may be associated with lower BMD at some, but not all, sites in ARFID. Further research with larger samples will be important to investigate whether high PYY drives bone loss in ARFID.

Bone mineral density in egyptian children with juvenile idiopathic arthritis: possible correlation to serum RANKL / osteoprotegerin (OPG) ratio and OPG gene polymorphisms

BackgroundChildren with juvenile idiopathic arthritis (JIA) are at higher risk of decreased bone mineral density (BMD) compared with healthy children due to genetic, disease and medication-related causes. This study aims to investigate the possible effects of osteoprotegerin (OPG) gene polymorphisms and serum levels of osteoprotegerin (OPG) and receptor activator of nuclear factor κB-ligand (RANKL) and RANKL/OPG ratio on BMD in children with JIA.MethodsOPG gene rs2073617, rs3134069, serum RANKL, OPG and RANKL/OPG ratio were evaluated in 60 JIA children and 100 matched healthy controls. BMD was evaluated by lumbar dual energy X-ray absorptiometry (DEXA) according to which patients were classified in 2 groups (DEXA z-score above and below − 2). Composite disease activity was measured using the Juvenile Arthritis Disease Activity Score (JADAS) 27-joints. Articular damage was scored using the juvenile arthritis damage index (JADI).ResultsPatients aged 12.05 ± 3.2 years, included 38 females and 31% had BMD z-score below-2. Systemic-onset JIA was the most frequent phenotype (38%). Genotypes and alleles frequencies of the 2 studied polymorphisms did not differ between patients and controls (p > 0.05 for all) while serum RANKL and RANKL/OPG ratio were significantly higher in patients compared to controls (p = < 0.001 and 0.03 respectively). Patients with BMD < -2 had significantly greater frequencies of rs2073617 TT genotype and T allele (p < 0.001), higher serum RANKL, RANKL/OPG ratio (p = 0.01, 0.002), female predominance (p = 0.02), higher articular and extra-articular damage index (p = 0.008,0.009) and more frequent steroid usage (p = 0.02) compared to patients with BMD z-score >-2. Multivariate analysis showed rs2073617 TT genotype, RANKL/OPG ratio, long disease duration (above 36 months) and use of steroid to be associated with decreased BMD (p = 0.03,0.04,0.01,0.01 respectively) in JIA children.ConclusionsEgyptian children with JIA have decreased BMD. rs2073617 TT genotype and T allele, RANKL/OPG ratio are possible determinants of reduced BMD in JIA. Our results underline the importance of frequent monitoring of BMD in JIA children and trying to control disease activity to preserve long term bone health.

#6649 SIGNIFICANT CLINICAL IMPROVEMENT OF PAEDIATRIC AND ADULT PATIENTS WITH DISTAL RENAL TUBULAR ACIDOSIS AFTER 6 YEARS OF TREATMENT WITH SIBNAYAL®

Abstract Background and Aims Distal renal tubular acidosis (dRTA) is a rare disease, inherited or acquired, characterized by hyperchloremic metabolic acidosis leading to negative effects on growth, bone and kidney, with growth retardation, rickets in children and osteomalacia in adults, nephrolithiasis and chronic kidney disease. No alkalizing treatment, necessary to control the metabolic acidosis and its consequences in growth, bone and kidney, has been shown, to date, to control the disease in the long term. The aim of this clinical study is to describe a cohort of adult and pediatric dRTA patients treated during 6 years by Sibnayal®, a new prolonged-release alkalizing formulation, in term of metabolic control, growth and long-term complications on bone mineralization and kidney function. Method Thirty patients with genetic dRTA taking Sibnayal®, previously treated with alkalizing standard of care and enrolled in the short-term B21CS study, were followed up for six years on average in a multicenter open-label extension trial (B22CS) to evaluate long-term impact of treatment on standard deviation score (SDS) of height, SDS weight, body mass index (BMI), Z-score spine bone mineral density (BMD), phosphocalcic metabolism (up to 4 years). glomerular filtration rate (GFR), nephrolithiasis, metabolic acidosis control, safety and compliance were also evaluated. Paired t-tests were done from baseline to end of study (EoS) when appropriate. The covariance ANCOVA test was performed to analyze spine BMD Z-score. Data are presented as mean ± standard error of mean. Results Clinical observations in this cohort, after an average of six years of treatment with Sibnayal®, confirmed the adequate control of metabolic acidosis (plasma bicarbonate from 22.0±0;6 mmol/L at baseline to 22.8±0.56 mmol/L at study end). The phosphocalcic metabolism analysis, from baseline to Month 48, demonstrated no significant change on Z-score for age of blood bone alkaline phosphatases, and a significant decrease of Z-score for age of blood phosphorus level (from to -0.5±0.7 to -1.3±1.0, p = 0.03). From baseline to EoS, SDS of height and weight increased significantly (-0.6±1.0 to -0.3±0.9 p = 0.04 and 0.2±1.5 to 0.7±1.4 p = 0.03, respectively), without significant difference in BMI. The spine BMD Z-score, relevant skeletal area for both pediatric and adult patients, underwent a continuous and significant increase of the change from baseline values over 6 years of treatment (difference [95% CI] = 0.404 [0.170; 0.639]). At EoS, spine BMD Z-score was improved in pre- and post-pubertal patients (mean 0.76±0.54 and 0.56±0.22 respectively), while it was stabilized in pubertal patients (mean -0.01±0.39). There was no significant variation of GFR between baseline and EoS. Nephrolithiasis increased slightly according to the increased age of the patient, without surgical intervention for stones removal. Safety and adherence to treatment remained good throughout the study. Conclusion Our data show the positive effect of the long-term treatment with Sibnayal® on growth and spine BMD, nicely improved, in the dRTA patients who participated to this long-term follow-up clinical study. The kidney function is also stabilized in these patients during the follow-up. This is the first report describing the prevention effect on the long-term complications of the distal Renal Tubular Acidosis patients under 6 years of Sibnayal® treatment.

Effect of long-term medium to high-dose inhaled budesonide on bone mineral density in children with asthma: a cross-sectional study

Objective The objective of this study was to examine the impact of long-term medium to high-dose inhaled budesonide on bone mineral density in children with asthma. Methods We conducted a cross-sectional study in children aged 7–17 years with asthma, who received long-term (≥2 years), medium to high-dose inhaled budesonide (≥400μg/day in 6–11 years old; ≥800 μg/day in >11 years old). We measured bone mineral density (BMD) using dual-energy X-ray absorptiometry and compared it with reference Indian normative values. Results Thirty-five children with moderate to severe asthma receiving long-term medium to high-dose inhaled budesonide, were included in the study. We found a significantly low lumbar-spine BMD in the study population compared to reference Indian values (p-value 0.002). Eight cases had short stature. Despite the adjustment for height-age in these short-stature cases, lumbar-spine BMD remained significantly low in the study population (p-value 0.020). No significant difference was found in 25-hydroxy vitamin D levels between subjects with “low BMD” and “BMD z-score > –2”. Conclusions The findings of this study suggest that long-term medium to high-dose inhaled budesonide treatment in children with asthma is associated with decreased BMD. However, further investigation with a larger sample size is necessary to confirm this relationship.

Support for inadvertent undereating in female adolescent athletes with clinical indicators of low energy availability.

This study evaluated pathways to low energy availability in a sample of female adolescent athletes (n = 464). Cross-sectional. Participants (age 13-18 y) underwent assessments for height, weight, eating attitudes and behaviors, and menstrual function. Bone mineral density and body composition were evaluated by dual-energy x-ray absorptiometry in a subset of participants (n = 209). Athletes were classified with clinical indicators of low energy availability if they met criteria for 1) primary or secondary amenorrhea or 2) clinical underweight status (body mass index-for-age < 5th percentile). Disordered eating was assessed using the Eating Disorder Examination Questionnaire. Thirty (6.5%) athletes exhibited clinical indicators of low energy availability, with higher estimates in leanness than non-leanness sports (10.9% vs. 2.1%, p < 0.005). Among athletes with clinical indicators of low energy availability, 80% (n = 24) did not meet criteria for disordered eating, eating disorder, or report the desire to lose weight. Athletes with (vs. without) clinical indicators of low energy availability exhibited lower lumbar spine (-1.30 ± 1.38 vs. -0.07 ± 1.21, p < 0.001) and total body (-0.30 ± 0.98 vs. 0.53 ± 0.97, p < 0.006) bone mineral density Z-scores. A majority of female adolescent athletes with clinical indicators of low energy availability did not exhibit characteristics consistent with intentional dietary restriction, supporting the significance of the inadvertent pathway to low energy availability and need for increased nutrition education in this population.

Associations of Hormonal and Metabolic Parameters with Bone Mineralization in Young Adult Females.

Osteoporosis is characterized by impaired bone mineralization and microarchitecture. An important protective factor is a high peak bone mass (PBM), attained in the second and third decade of life. The aim of the study was to evaluate the effect of hormonal and metabolic parameters on bone mineralization in young adult female patients. A total of 111 participants qualified for the study. Bone mineral density of the lumbar spine (L1-L4) and whole skeleton was measured using dual-energy X-ray absorptiometry (DXA). Hormonal parameters were determined: the concentrations of androstendione, dihydroepiandrosterone sulphate, testosterone, sex hormone binding protein, 17-OH-progesterone, folliculotropic hormone, estradiol, thyrotropic hormone, free thyroxine and cortisol. Metabolic parameters were also examined. The study showed a statistically significant correlation between bone mineral density and estradiol concentration and a negative relationship between cortisol concentration and the bone mineral density (BMD) Z-score of the lumbar spine. Sclerostin measurements taken during this study were not related to bone mineral density. It has been shown that the concentration of the hormones tested, even within the reference range, may affect bone mineralization. We suggest observing the follow-up of the menstrual cycles, as well as analyzing the results of test patients in an annual examination system. However, each clinical case should be considered individually. The sclerostin test is currently not useful in the clinical evaluation of bone mineralization in young adult women.

Grip strength data for individuals with severe anorexia nervosa

Objective: This retrospective study compiled grip strength data of patients with severe anorexia nervosa to assess associations among grip strength, anorexia nervosa severity, depression, and physiological comorbidities. Design: 475 patients with severe anorexia nervosa admitted to an inpatient hospital and completed a grip strength assessment at admission and discharge. Depression was diagnosed via clinical interview. Independent sample t-tests and Mann-Whitney U tests compared difference in grip strength and anthropometric measures between anorexia nervosa subtypes. Linear and logistic regressions assessed associations among grip strength, depression, and physiological measures. Results: The cohort was 91.8% female (M age: 31.8 ± 11.7 years). At admission, patients had an average body mass index of 13.8 ± 2.4 kg/m2 with 97.7% exhibiting impaired right-hand grip strength for their sex and age. Weaker right grip strength was associated with admission lower body mass index, worsening bone mineral density z-scores, and lower serum prealbumin (all p’s&lt;.05) for both sexes. Patients with binge-purge anorexia nervosa had significantly stronger right grip strength (M:19.0 ± 7.8 kg) compared to patients with restricting anorexia nervosa (M:16.9 ± 7.9 kg), p=0.003. Conclusion: Severe malnutrition from anorexia nervosa was associated with profound deficits in grip strength, medical comorbidities and disease severity, but was not associated with depression.

Bone Mineral Density and Dickkopf-1 in Adolescents with Non-Deletional Hemoglobin H Disease

Background: Low bone mineral density (BMD) is prevalent in individuals with β-thalassemia and is associated with increased circulating dickkopf-1 concentration. These data are limited in α-thalassemia. Therefore, we aimed to determine the prevalence of low BMD and the association between BMD and serum dickkopf-1 in adolescents with non-deletional hemoglobin H disease, a form of α-thalassemia whose severity is comparable to β-thalassemia intermedia. Methodology: The lumbar spine and total body BMD were measured and converted into height-adjusted z-scores. Low BMD was defined as BMD z-score ≤ -2. Participant blood was drawn for measurement of dickkopf-1 and bone turnover marker concentrations. Results: Thirty-seven participants with non-deletional hemoglobin H disease (59% female, mean age 14.6 ± 3.2 years, 86% Tanner stage ≥2, 95% regularly transfused, 16% taking prednisolone) were included. Over one year prior to the study, mean average pretransfusion hemoglobin, ferritin and 25-hydroxyvitamin D concentrations were 8.8 ± 1.0 g/dL, and 958 ± 513 and 26 ± 6 ng/mL, respectively. When participants taking prednisolone were excluded, the prevalence of low BMD at the lumbar spine and total body was 42% and 17%, respectively. BMD at both sites was correlated positively with body mass index z-score, and negatively with dickkopf-1 (all p-values <0.05). There were no correlations among dickkopf-1, 25-hydroxyvitamin D, osteocalcin and C-telopeptide of type-I collagen. Multiple regression analysis showed dickkopf-1 inversely associated with total body BMD z-score adjusting for sex, bone age, body mass index, pre-transfusion hemoglobin, 25-hydroxyvitamin D, history of delayed puberty, type of iron chelator and prednisolone use (p-value = 0.009). Conclusions: We demonstrated a high prevalence of low BMD in adolescents with non-deletional hemoglobin H disease. Moreover, dickkopf-1 inversely associated with total body BMD suggesting it may serve as a bone biomarker in this patient population.

More Prevalent and Severe Low Bone-Mineral Density in Boys with Severe Adolescent Idiopathic Scoliosis Than Girls: A Retrospective Study of 798 Surgical Patients.

A total of 0.1-0.8% of AIS patients progress to severe stages without clear mechanisms, and AIS girls are more prone to curve progression than boys. Recent studies suggest that AIS girls have systemic and persistent low bone-mineral density (BMD), which has been shown to be a significant prognostic factor of curve progression in AIS. The present study aimed to (a) investigate the prevalence of low BMD in patients with severe AIS and (b) assess the sexual dimorphism and independent risk factors of low BMD in severe AIS patients. A total of 798 patients (140 boys vs. 658 girls) with AIS who reached surgical threshold (Cobb ≥ 40°) were recruited. BMD were assessed using BMD Z-scores from dual-energy X-ray absorptiometry (DXA). Demographic, clinical, and laboratory values of the subjects were collected from their medical records. Logistic regression analysis was performed to identify independent risk factors of low BMD. The overall prevalence of BMD Z-score ≤ -2 and ≤ -1 were 8.1% and 37.5%, respectively. AIS boys had significantly lower BMD Z-scores (-1.2 ± 0.96 vs. -0.57 ± 0.92) and higher prevalence of low BMD (Z-score ≤ -2: 22.1% vs. 5.2%, p < 0.001; Z-score ≤ -1: 59.3% vs. 32.8%, p < 0.001) than girls. Sex, BMI, serum alkaline phosphatase, and potassium were independent factors of low BMD in the severe AIS patients. The present large cohort of surgical AIS patients revealed that low BMD is more prevalent and severe in boys than in girls with severe curves. Low BMD may serve as a more valuable predictive factor for curve progression to the surgical threshold in boys than girls with AIS.

Male Runners With Lower Energy Availability Have Impaired Skeletal Integrity Compared to Nonathletes.

Female athletes, particularly runners, with insufficient caloric intake for their energy expenditure [low energy availability (EA) or relative energy deficiency] are at risk for impaired skeletal integrity. Data are lacking in male runners. To determine whether male runners at risk for energy deficit have impaired bone mineral density (BMD), microarchitecture, and estimated strength. Cross-sectional. Clinical research center. 39 men (20 runners, 19 controls), ages 16-30 years. Areal BMD (dual-energy x-ray absorptiometry); tibia and radius volumetric BMD and microarchitecture (high-resolution peripheral quantitative computed tomography); failure load (microfinite element analysis); serum testosterone, estradiol, leptin; energy availability. Mean age (24.5 ± 3.8 y), lean mass, testosterone, and estradiol levels were similar; body mass index, percent fat mass, leptin, and lumbar spine BMD Z-score (-1.4 ± 0.8 vs -0.8 ± 0.8) lower (P < .05); and calcium intake and running mileage higher (P ≤ .01) in runners vs controls. Runners with EA <median had lower lumbar spine (-1.5 ± 0.7, P = .028), while runners with EA ≥median had higher hip (0.3 ± 0.7 vs -0.4 ± 0.5, P = .002), BMD Z-scores vs controls. After adjusting for calcium intake and running mileage, runners with EA <median had lower mean tibial total and trabecular volumetric BMD, trabecular bone volume fraction, cortical porosity, and apparent modulus vs controls (P < .05). Appendicular lean mass and serum estradiol (R ≥ 0.45, P ≤ .046), but not testosterone, were positively associated with tibial failure load among runners. Despite weight-bearing activity, skeletal integrity is impaired in male runners with lower caloric intake relative to exercise energy expenditure, which may increase bone stress injury risk. Lower estradiol and lean mass are associated with lower tibial strength in runners.

Bone health in childhood and adolescence: an overview on dual-energy X-ray absorptiometry scanning, fracture surveillance and bisphosphonate therapy for low-middle-income countries.

Bone accrual in childhood determines bone health in later life. Loss of bone strength in early life can lead to increased morbidity and reduced quality of life in childhood and adolescence. Increased availability of assessment tools and bisphosphonate therapy, together with increased awareness on the significance of fracture history and risk factors, have led to greater opportunities, to improve detection and optimize management of children and adolescents with bone fragility globally, including those in lower resource settings. Bone mineral density z-scores and bone mineral content are surrogate measures of bone strength, which can be measured by dual-energy X-ray absorptiometry (DXA), in growing individuals. DXA can aid in the diagnosis and management of primary and secondary bone fragility disorders in childhood. DXA helps evaluate children with clinically significant fractures, and monitor those with bone fragility disorders, or at high risk for compromised bone strength. Obtaining DXA images can however be challenging, especially in younger children, due to difficulty in positioning and movement artefacts, while paediatric DXA interpretation can be confounded by effects of growth and puberty. Furthermore, access to DXA facilities as well as appropriate paediatric reference norms and expertise for interpretation, may not be easily available especially in lower resource settings. Pediatric bone experts are now placing increasing emphasis on the fracture phenotype and clinical context to diagnose osteoporosis over bone mineral density (BMD) by DXA. Low trauma vertebral fractures are now recognized as a hallmark of bone fragility, and spinal fracture surveillance by either conventional lateral thoracolumbar radiographs or vertebral fracture assessment by DXA is gaining increasing importance in diagnosing childhood osteoporosis, and initiating bone protective therapy. Furthermore, it is now understood that even a single, low-trauma long bone fracture can signal osteoporosis in those with risk factors for bone fragility. Intravenous bisphosphonate therapy is the mainstay of treatment for childhood bone fragility disorders. Other supportive measures to improve bone strength include optimizing nutrition, encouraging weight bearing physical activity within the limits of the underlying condition, and treating any associated endocrinopathies. With this paradigm shift in childhood osteoporosis evaluation and management, lack of DXA facilities to assess BMD at baseline and/or provide serial monitoring is not a major barrier for initiating IV bisphosphonate therapy in children in whom it is clinically indicated and would benefit from its use. DXA is useful, however, to monitor treatment response and optimal timing for treatment discontinuation in children with transient risk factors for osteoporosis. Overall, there is lack of awareness and paucity of guidelines on utilizing and adopting available resources to manage paediatric bone disorders optimally in lower-resource settings. We provide an evidence-based approach to the assessment and management of bone fragility disorders in children and adolescents, with appropriate considerations for lower resource settings including LMIC countries.

The Influence of Anti-Citrullinated Polypeptide Antibodies on Bone Mineral Density Decrease and Incident Major Osteoporotic Fractures in Patients with Rheumatoid Arthritis: A Retrospective Case-Control Study

Background: Effects of anti-citrullinated polypeptide antibodies (ACPA) on the bone mineral density (BMD) reduction and incidence of major osteoporotic fractures (MOF) in patients with rheumatoid arthritis (RA) were evaluated using a retrospective longitudinal case-control study. Methods: Patients with RA who were examined using dual-energy X-ray absorptiometry and simultaneously treated for more than 5 years were recruited. BMD absolute value and Z-scores at initial measurements (baseline) and changes of these values from baseline were assessed, and associations between BMD and candidate risk factors including ACPA positivity and serum titer levels were statistically evaluated. Additional statistical evaluations of ACPA positivity in regard to the incidence of MOF were tested. Results: A total of 222 patients were included. Higher ACPA titers correlated significantly with lower BMD and Z-scores at baseline using a multivariate model (p &lt; 0.05). ACPA positivity correlated significantly with lower values and an annual decrease in the Z-score in total hip at follow-up using a univariate model (p &lt; 0.05), whereas no significant correlation was found using a multivariate model. Z-scores in the ACPA-positive group were significantly lower than those of the ACPA-negative group (p &lt; 0.05). However, ACPA-positivity demonstrated no higher risk for incident MOF. Conclusions: The presence of ACPA is a potential risk of BMD loss however weak.

Diagnosis of Osteogenesis Imperfecta in 23 Years Old Man: A Case Report

Osteogenesis imperfecta is a disorder of the formation of collagen tissue that functions as connective tissue and is caused by a gene mutation that causes disturbances in the formation of type 1 collagen. This study aimed to describe the diagnosis of osteogenesis imperfecta. A 23-year-old man came to the rheumatology polyclinic of Dr. Mohammad Hoesin General Hospital Palembang with a complaint of recurrent fractures since ± 17 years ago. The patient also complained that the hearing in the left ear was slowly decreasing. The patient's right thigh was still pinned 2 years ago, but after being controlled by orthopedics, it was said that the bones were still not fused. Then the patient was referred to the rheumatology polyclinic for further examination and management. On examination, the patient was 145 cm tall and weighed 40 kg. He had blue-gray sclera, triangular facial appearance, right leg length 86 cm, left leg length 78 cm, blue sclera, and scoliosis, while secondary sex growth was within normal limits. From the examinations of bone survey and bone age, it was found osteoporotic bone structure, scoliosis, plate-screw in the middle 1/3 of the right femur, fracture union of the femur, bowing of the left femur, BMD Z-Score -3.0 means very low compared to the same age and gender. Management is more focused on supportive therapy with the aim of minimizing the occurrence of fractures, minimizing disability, and helping people with osteogenesis imperfecta to be independent and maintain overall health. The goal of orthopedic management is to treat osteogenesis imperfecta with fractures and prevent or correct bone deformity. In conclusion, osteogenesis imperfecta is a complex hereditary disease characterized by striking clinical variability necessitating a logical classification system. Disease management requires multidisciplinary experts and further research on therapeutic approaches such as bisphosphonates.

Bone status and HCV infection in thalassemia major patients.

Hepatitis C virus (HCV) infection increases the risk for osteoporosis but this relationship has not been investigated among multi-transfused patients with thalassemia major (TM). We cross-sectionally explored the association of HCV infection with bone mineral density (BMD), vitamin D, and bone turnover biomarkers in TM. We considered 130 TM patients (41.89±5.49years, 67 females) enrolled in the E-MIOT (Extension-Myocardial Iron Overload in Thalassemia) Network. BMD measurements taken at the lumbar spine, femoral neck and total hip were expressed as Z-scores, with a BMD Z-score≤-2.0 indicating low bone mass. Z-scores were not associated with gender, iron overload indices, vitamin D levels, and biochemical bone turnover markers, but decreased with aging and in presence of hypogonadism and were directly correlated with body mass index (BMI). The prevalence of low bone mass was 70.7%. Three groups of patients were identified: 78 who never contracted the infection (group 0), 72 who cleared HCV (group 1), and 29 with chronic HCV infection (CHC) (group 2). All Z-scores progressively decreased according to HCV status from group 0 to group 2. Osteocalcin levels were significantly lower in groups 2 and 1 than in group 0. CHC patients were more likely to have low bone mass compared to HCV naive patients, after adjusting for age, BMI, hypogonadism, and pancreatic iron. In TM, CHC appears as one additive risk factor for low bone mass and osteocalcin may play a role in this association.