Secondary Bone Defect in Neuromuscular Diseases in Childhood: A Longitudinal “Muscle-Bone Unit” Analysis

2018 ◽  
Vol 49 (06) ◽  
pp. 397-400 ◽  
Author(s):  
C. Ribstein ◽  
D. Courteix ◽  
N. Rabiau ◽  
C. Bommelaer ◽  
Y. Bourdeau ◽  
...  
Keyword(s):  
Bone Defect ◽  
Functional Relationship ◽  
Bone Markers ◽  
Neuromuscular Diseases ◽  
Muscular Dystrophies ◽  
Mineral Density ◽  
Z Scores ◽  
Spinal Muscular Atrophies ◽  
Total Body

AbstractTo evaluate the potential bone defect in neuromuscular diseases, we conducted a longitudinal study including three groups of patients: 14 Duchenne muscular dystrophies (DMD) and 2 limb-girdle muscular dystrophies (LGMD); 3 Becker muscular dystrophies (BeMD) and 7 spinal muscular atrophies (SMA). Yearly osteodensitometries assessed body composition and bone mineral density (BMD) associated with bone markers and leptin. Along the 7-year study, 107 osteodensitometries showed that bone status evolved to osteopenia in most patients except BeMD. When analyzing the crude values, BMD improved with age in BeMD and SMA but not in DMD/LGMD. The correlation using the Z-scores displayed a decrease in BMD with age in DMD/LGMD for all regions, in SMA at total body less head, whereas BMD increased in BeMD at lumbar spine. As observed in healthy persons, muscular mass and bone tissue were significantly correlated. Glucocorticoids were deleterious on trabecular and cortical bone. Leptin was high in most patients and correlated to fat mass and bone parameters. This study confirms a secondary bone defect in neuromuscular diseases, further confirming the functional relationship between bone and muscle and arguing for regular bone follow-up in patients to prevent fracture risk. Adipose tissue seems to interfere with bone remodeling in neuromuscular diseases.

scholarly journals Bone mineral density and explanatory factors in children and adults with juvenile dermatomyositis at long term follow-up; a cross sectional study

2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Henriette Schermacher Marstein ◽  
Kristin Godang ◽  
Berit Flatø ◽  
Ivar Sjaastad ◽  
Jens Bollerslev ◽  
...  
Keyword(s):  
Bone Turnover ◽  
Inflammatory Markers ◽  
Juvenile Dermatomyositis ◽  
Inflammatory Myopathy ◽  
Disease Onset ◽  
Whole Body ◽  
Mineral Density ◽  
Z Scores

Abstract Background Juvenile dermatomyositis (JDM) is the most common idiopathic inflammatory myopathy in children and adolescents. Both the disease and its treatment with glucocorticoids may negatively impact bone formation. In this study we compare BMD in patients (children/adolescence and adults) with long-standing JDM with matched controls; and in patients, explore how general/disease characteristics and bone turnover markers are associated with BMD. Methods JDM patients (n = 59) were examined median 16.8y (range 6.6–27.0y) after disease onset and compared with 59 age/sex-matched controls. Dual-energy X-ray absorptiometry (DXA) was used to measure BMD of the whole body and lumbar spine (spine) in all participants, and of ultra-distal radius, forearm and total hip in participants ≥20y only. Markers of bone turnover were analysed, and associations with outcomes explored. Results Reduced BMD Z-scores (<−1SD) were found in 19 and 29% of patients and 7 and 9% of controls in whole body and spine, respectively (p-values < 0.05). BMD and BMD Z-scores for whole body and spine were lower in all patients and for < 20y compared with their respective controls. In participants ≥20y, only BMD and BMD Z-score of forearm were lower in the patients versus controls. In patients, BMD Z-scores for whole body and/or spine were found to correlate negatively with prednisolone use at follow-up (yes/no) (age < 20y), inflammatory markers (age ≥ 20y) and levels of interferon gamma-induced protein 10 (IP-10) (both age groups). In all patients, prednisolone use at follow-up (yes/no) and age ≥ 20y were independent correlates of lower BMD Z-scores for whole body and spine, respectively. Conclusion In long-term JDM, children have more impairment of BMD than adults in spine and whole-body. Associations with BMD were found for both prednisolone and inflammatory markers, and a novel association was discovered with the biomarker of JDM activity, IP-10.

Long-term efficacy and safety of enzalutamide (ENZ) monotherapy in hormone-naïve prostate cancer (HNPC): 3-year, open-label, follow-up results.

2017 ◽  
Vol 35 (6_suppl) ◽  
pp. 220-220
Author(s):  
Bertrand F. Tombal ◽  
Michael Borre ◽  
Per Rathenborg ◽  
Patrick Werbrouck ◽  
Hendrik Van Poppel ◽  
...  
Keyword(s):  
Health Status ◽  
Global Health ◽  
Specific Antigen ◽  
Global Health Status ◽  
Mineral Density ◽  
Hot Flush ◽  
Psa Response ◽  
Total Body

220 Background: A phase 2 study of the androgen receptor inhibitor ENZ as monotherapy in patients with HNPC [NCT01302041] showed a high prostate-specific antigen (PSA) response rate, regardless of baseline metastases, and favorable tolerability. In a 1- and 2-year follow-up, ENZ maintained long-term reductions from baseline in PSA, with minimal impact on total-body bone mineral density (BMD). Herein, results from a pre-specified 3-year follow-up are reported. Methods: A total of67 patients with HNPC and non-castrate testosterone ( ≥ 230 ng/dL) received ENZ 160 mg/day until disease progression or unacceptable toxicity. The primary end point of PSA response ( ≥ 80% decline from baseline) was analyzed at week 25 and 1, 2, and 3 years. Other end points were best overall tumor response, BMD, body composition, quality of life, and safety. Results: At the 3-year visit, 42 (62.7%) patients remained on the study medication. Of those, 38 (90.5%; 95% confidence interval 77.4%, 97.3%) maintained a PSA response. Of the 26 patients with metastases at baseline, 17 (65.4%) had a complete or partial response as best overall response at 3 years. In patients who completed the 3-year visit, minimal changes from baseline were observed in total-body BMD or in BMD of the femoral neck, trochanter, spine L1–L4, or forearm (median and mean changes ranged from –3.6% to 1.3% and –2.7% to –0.1%, respectively). The EORTC QLQ-C30 global health status results showed a small decrease at 3 years versus baseline (–3.96 points), consistent with the 2-year results. At 3 years, measurements for total body fat increased (median, 14.7%; mean, 16.5%) and total body lean decreased (median, –6.3%; mean, –6.5%) from baseline. Physical functioning, fatigue, and dyspnea worsened ( > 10 points) at 3 years, similar to results at 2 years. The most frequently reported adverse events ( > 10%) were gynecomastia, fatigue, hot flush, nipple pain, hypertension, diarrhea, nausea, pain in extremity, back pain, and constipation. Conclusions: In patients with HNPC treated with ENZ for 3 years, the efficacy of ENZ as monotherapy was maintained. Overall, BMD, global health status, and safety results were similar to those at 2 years. Clinical trial information: NCT01302041.

HRT preserves increases in bone mineral density and reductions in body fat after a supervised exercise program

1998 ◽  
Vol 84 (5) ◽  
pp. 1506-1512 ◽  
Author(s):  
Wendy M. Kohrt ◽  
Ali A. Ehsani ◽  
Stanley J. Birge
Keyword(s):  
Bone Mineral Density ◽  
Lumbar Spine ◽  
Bone Mineral ◽  
Exercise Program ◽  
Treatment Phase ◽  
Mineral Density ◽  
Supervised Exercise ◽  
Total Body ◽  
Exercise Induced

The aims of this study were to confirm our previous finding that hormone-replacement therapy (HRT) augments exercise-induced increases in bone mineral density (BMD) in older women and to determine whether HRT preserves the adaptations when exercise is reduced or discontinued. The study included an 11-mo treatment phase and a 6-mo follow-up phase. Participants, aged 66 ± 3 yr, were assigned to control (Con; n = 10), exercise (Ex; n = 18), HRT ( n = 10), and Ex+HRT ( n = 16) groups. HRT was continued during the follow-up. After the treatment phase, changes in total body BMD were −0.5 ± 1.7, 1.5 ± 1.4, 1.2 ± 0.8, and 2.7 ± 1.2% in Con, Ex, HRT, and Ex+HRT, respectively. Ex+HRT was more effective than HRT in increasing BMD of the total body and tended ( P = 0.08) to be more effective at the lumbar spine. Ex+HRT was more effective than Ex in increasing BMD of the total body, lumbar spine, and trochanter. Exercise-induced gains in BMD were preserved during the follow-up only in those individuals on HRT. HRT also attenuated fat accumulation, particularly in the abdominal region, after the exercise program. These findings suggest that HRT is an important adjunct to exercise for the prevention not only of osteoporosis but also of diseases related to abdominal obesity.

Duchenne and Becker muscular dystrophies: A 4-year longitudinal follow-up study of bone mineral density

Bone ◽  
2009 ◽  
Vol 45 ◽  
pp. S60
Author(s):  
A.C. Söderpalm ◽  
A.K. Kroksmark ◽  
P. Magnusson ◽  
A.C. Åhlander ◽  
J. Karlsson ◽  
...  
Keyword(s):  
Bone Mineral Density ◽  
Bone Mineral ◽  
Muscular Dystrophies ◽  
Mineral Density ◽  
Follow Up Study

scholarly journals Depression in Anorexia Nervosa: A Risk Factor for Osteoporosis

2005 ◽  
Vol 90 (9) ◽  
pp. 5382-5385 ◽  
Author(s):  
Jerzy Konstantynowicz ◽  
Halina Kadziela-Olech ◽  
Maciej Kaczmarski ◽  
Roger M. D. Zebaze ◽  
Sandra Iuliano-Burns ◽  
...  
Keyword(s):  
Anorexia Nervosa ◽  
Risk Factor ◽  
Adolescent Girls ◽  
Rating Scale ◽  
Tanner Stage ◽  
Bone Fragility ◽  
Mineral Density ◽  
Cross Sectional ◽  
Z Scores ◽  
Total Body

Abstract Context: Both anorexia nervosa (AN) and depression are associated with osteoporosis. We hypothesized that adolescent girls with AN and depression will have lower bone mineral density (BMD) than anorexic girls without depression. Objective: The objective of this study was to investigate whether depression is an independent risk factor for osteoporosis in anorexic adolescent girls. Design: This study was cross-sectional. Setting: This study was conducted at the University Children’s Hospital (Bialystok, Poland) from October 2002 through September 2003. Participants: Forty-five Caucasian anorexic girls aged 13–23 yr, matched by age, Tanner stage, weight, height, calcium intake, and duration of AN, were studied, including 14 with comorbid depression (based on Hamilton Depression Rating Scale and Montgomery-Asberg Depression Rating Scale) and 31 anorexic girls without depression. Main Outcome Measures: Total body and lumbar spine (LS) BMD, fat mass, and lean mass assessed using dual-energy x-ray absorptiometry were compared between AN girls with and without depression. Results: BMD was reduced in both groups, relative to reference data, but girls with AN and depression had lower BMD than those with AN alone (LS Z-scores, −2.6 ± 0.3 vs. −1.7 ± 0.3; P = 0.02) (mean ± sem). Quantitative assessment of depression correlated independently with total body BMD (r = −0.4; P &lt; 0.05) and LS BMD (r = −0.6; P &lt; 0.001). Conclusion: Anorexic girls with depression are at higher risk of osteoporosis than those without depression. The mechanisms responsible for decreased BMD in depression are not known. Independent treatment of the depressive disorder in AN may partly alleviate the bone fragility.

scholarly journals Associations between serum levels of insulin-like growth factor-I and bone mineral acquisition in pubertal children: a 3-year follow-up study in Hamamatsu, Japan

2019 ◽  
Vol 38 (1) ◽  
Author(s):  
Katsuyasu Kouda ◽  
Masayuki Iki ◽  
Kumiko Ohara ◽  
Harunobu Nakamura ◽  
Yuki Fujita ◽  
...  
Keyword(s):  
Bone Mineral Density ◽  
Bone Mineral ◽  
Serum Levels ◽  
Confounding Factors ◽  
Bone Area ◽  
Mineral Density ◽  
Igf I ◽  
Total Body ◽  
Mineral Acquisition

Abstract Background Epidemiological data regarding the association between serum levels of IGF-I and bone mineral acquisition during childhood are scarce. Here, we investigated the association between serum levels of IGF-I and bone status during puberty. Methods We analyzed prospective 3-year follow-up data of 254 community-dwelling children who completed both baselines (at age 11.2 years) and follow-up (at age 14.2 years) surveys in Hamamatsu, Japan. Total body (TB) bone area and bone mineral parameters were assessed using dual-energy X-ray absorptiometry. Results During the 3-year follow-up period, there were significant (P < 0.05) increases in total body less head (TBLH) areal bone mineral density (aBMD), TBLH bone mineral content (BMC), and TB bone area, and a significant decrease in TB bone mineral apparent density (BMAD, volumetric bone mineral density, vBMD). IGF-I levels showed significant positive relationships with TBLH BMC and TBLH aBMD at both baseline and follow-up. TBLH aBMD in boys and TB BMAD in girls at follow-up showed significant increases from the lowest to highest quartiles of baseline IGF-I levels after adjusting for confounding factors. Similarly, changes in TBLH aBMD in boys and TB BMAD in girls during the 3-year follow-up period showed significant increases from the lowest to highest quartiles of baseline IGF-I levels after adjusting for confounding factors. Conclusions These results suggest that pubertal children with high levels of serum IGF-I tended to have high bone mineral acquisition later on.

scholarly journals Changes in bone mineral density over 10 years in patients with early rheumatoid arthritis

RMD Open ◽  
2020 ◽  
Vol 6 (1) ◽  
pp. e001142
Author(s):  
Lisa Theander ◽  
Minna Willim ◽  
Jan Åke Nilsson ◽  
Magnus Karlsson ◽  
Kristina E Åkesson ◽  
...  
Keyword(s):  
Rheumatoid Arthritis ◽  
Bone Mineral Density ◽  
Lumbar Spine ◽  
Femoral Neck ◽  
Z Score ◽  
Mineral Density ◽  
Early Ra ◽  
Z Scores ◽  
The Mean

ObjectivesTo investigate changes in bone mineral density (BMD) in patients with early rheumatoid arthritis (RA) over a 10-year period.MethodsConsecutive patients with early RA (symptom duration <12 months) were followed according to a structured programme and examined with dual-energy X-ray absorptiometry (DXA) at inclusion and after 2, 5 and 10 years. Mean Z-scores over the study period were estimated using mixed linear effect models. Changes in Z-scores between follow-up visits were analysed using paired T-tests.ResultsAt inclusion, 220 patients were examined with DXA. At the femoral neck, the mean Z-score over 10 years was −0.33 (95 % CI −0.57 to −0.08) in men and −0.07 (−0.22 to 0.08) in women. Men had significantly lower BMD at the femoral neck than expected by age at inclusion (intercept Z-score value −0.35; 95 % CI −0.61 to −0.09), whereas there was no such difference in women. At the lumbar spine, the mean Z-score over the study period for men was −0.05 (−0.29 to 0.19) and for women 0.06 (−0.10 to 0.21). In paired comparisons of BMD at different follow-up visits, femoral neck Z-scores for men decreased significantly from inclusion to the 5-year follow-up. After 5 years, no further reduction was seen.ConclusionsIn this observational study of a limited sample, men with early RA had reduced femoral neck BMD at diagnosis, with a further significant but marginal decline during the first 5 years. Lumbar spine BMD Z-scores were not reduced in men or women with early RA. Data on 10-year follow-up were limited.

scholarly journals Bone mineral density is increased after a 16-week resistance training intervention in elderly women with decreased muscle strength

2016 ◽  
Vol 175 (6) ◽  
pp. 571-582 ◽  
Author(s):  
Ville Huovinen ◽  
Kaisa K Ivaska ◽  
Riku Kiviranta ◽  
Marco Bucci ◽  
Heta Lipponen ◽  
...  
Keyword(s):  
Resistance Training ◽  
Muscle Strength ◽  
Bone Markers ◽  
Elderly Women ◽  
Osteoporotic Fractures ◽  
Training Intervention ◽  
Mineral Density ◽  
Total Hip ◽  
Hip Bmd

ObjectiveNon-pharmacological interventions are important in reducing risk for osteoporotic fractures. We investigated the effects of a 16-week individualized resistance training intervention on bone mineral density (BMD), bone turnover markers and 10-year relative risk (RR) for osteoporotic fracture.DesignInterventional study with a follow-up.MethodsIn total, 37 elderly women (mean age 71.9 ± 3.1 years) with decreased muscle strength participated in the resistance training intervention three times per week with 60 min per session for 16 weeks under the supervision of a licensed physiotherapist. Total hip BMD with quantitative CT, bone markers (sclerostin, osteocalcin, CTX, PINP, IGF-1, 25(OH)-D) and 10-year RR for osteoporotic fracture were measured at baseline, post-intervention and at 1-year follow-up after the end of the intervention. Eleven age- and sex-matched controls did not participate in the intervention but were studied at baseline and at 1-year follow-up.ResultsResistance training seemed to increase total hip BMD by 6% (P= 0.005). Sclerostin (P< 0.001) and total osteocalcin (P= 0.04) increased while other bone markers remained unchanged. A 10-year RR for major osteoporotic and hip fracture remained unchanged. At follow-up total hip BMD (P< 0.001) decreased back to the baseline level with a simultaneous decrease in serum sclerostin (P= 0.045), CTX (P< 0.001) and an increase in 25(OH)-D (P< 0.001), 10-year RR for major osteoporotic (P= 0.002) and hip fracture (P= 0.01).ConclusionsOur findings suggest an important role of continuous supervised resistance training for the prevention of osteoporotic fractures in elderly women with decreased muscle strength.

scholarly journals Case Report: Low Bone and Normal Lean Mass in Adolescents With Complete Androgen Insensitivity Syndrome

2021 ◽  
Vol 12 ◽  
Author(s):  
Aaron Misakian ◽  
Michelle McLoughlin ◽  
Louisa C. Pyle ◽  
Thomas F. Kolon ◽  
Andrea Kelly ◽  
...  
Keyword(s):  
Body Composition ◽  
Bone Mineral ◽  
Lean Mass ◽  
Hormone Replacement ◽  
Androgen Insensitivity Syndrome ◽  
Mineral Density ◽  
Complete Androgen Insensitivity Syndrome ◽  
Androgen Insensitivity ◽  
Z Scores ◽  
Total Body

IntroductionOsteopenia and osteoporosis have been reported in adults with Complete Androgen Insensitivity Syndrome (CAIS). Little is known about changes in bone mineral density (BMD) in adolescents with CAIS and whether it is affected by early gonadectomy. Body composition data have not been reported.MethodsSingle-center, retrospective study of CAIS adolescents who underwent dual-energy x-ray absorptiometry (DXA) (Hologic, Horizon A). Body composition is presented as lean and fat mass indices (LMI, FMI). Z-scores for lumbar spine areal BMD (LBMD), total body less head (TBLH), bone mineral content (BMC), LMI, and FMI were calculated using female normative data. Results are expressed as median and min, max.ResultsSix females with genetically confirmed CAIS were identified—one with intact gonads and five with history of gonadectomy at 2–11 months. In the subject with intact gonads, LBMD-Z and TBLH BMC-Z were −1.56 and −1.26, respectively, at age 16 years. Among those with gonadectomy, LBMD-Z was −1.8 (−3.59 to 0.49) at age 15.6 years (12–16.8) and decreased in all three subjects who had longitudinal follow-up despite hormone replacement therapy (HRT). Adherence to HRT was intermittent. LMI-Z and FMI-Z were 0.1 (−1.39 to 0.7) and 1.0 (0.22 to 1.49), respectively.ConclusionsThese limited data indicate that adolescents with CAIS have bone mass deficit. Further studies are needed to understand the extent of BMD abnormalities and the effect of gonadectomy, especially early in childhood, and to establish the optimal HRT regimen for bone accrual. Data on lean mass are reassuring.

scholarly journals The Association of Thyroid Function With Bone Density During Childhood

2018 ◽  
Vol 103 (11) ◽  
pp. 4125-4134 ◽  
Author(s):  
Karlien Veldscholte ◽  
Mirjana Barjaktarovic ◽  
Katerina Trajanoska ◽  
Vincent W V Jaddoe ◽  
Theo J Visser ◽  
...  
Keyword(s):  
Bone Density ◽  
Bone Mass ◽  
Thyroid Function ◽  
Bone Mineral ◽  
Population Based ◽  
Inverse Association ◽  
Mineral Density ◽  
Free T4 ◽  
Total Body

Abstract Context Although the skeleton is a well-known thyroid hormone target organ, very little data are available on the association of thyroid function with bone outcomes during childhood. Objective To study the association of thyroid function with bone mass during childhood. Design, Setting, and Participants Population-based prospective cohort including 4204 children with TSH and free T4 (FT4) measured at the age of 6 years. Main Outcome Measures Bone density was assessed by a total body dual-energy X-ray absorptiometry scan at the median age of 6 years (95% range, 5.6 to 7.9) and at the age of 10 years (95% range, 9.0 to 10.9) in 4204 and 3404 participants, respectively. Results There was an inverse association of TSH with bone mineral density (BMD) at the age of 6 (β −0.028 ± 0.011, P = 0.009) and with follow-up measurements at the age of 10 (β −0.027 ± 0.011, P = 0.014), but not with bone mineral content (BMC) at the age of 6 (β −0.028 ± 0.015, P = 0.06) or for follow-up measurements of BMC at the age of 10 (β −0.011 ± 0.015, P = 0.47). There was an inverse association of FT4 with BMD (β −0.016 ± 0.006, P = 0.014) and BMC (β −0.023 ± 0.009, P = 0.009) cross-sectionally, and also at the age of 10 years (BMD: β −0.018 ± 0.007, P = 0.007; BMC: β −0.021 ± 0.009, P = 0.020). Conclusion A higher FT4 concentration is associated with lower bone mass at the age of 6 and at the age of 10 years. These data provide insights into the effects of thyroid function on bone physiology during childhood.

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