scholarly journals rhIGF-1 Treatment Increases Bone Mineral Density and Trabecular Bone Structure in Children with PAPP-A2 Deficiency

2018 ◽  
Vol 89 (3) ◽  
pp. 200-204 ◽  
Author(s):  
Federico G. Hawkins-Carranza ◽  
María T. Muñoz-Calvo ◽  
Gabriel Á. Martos-Moreno ◽  
Gonzalo Allo-Miguel ◽  
Luis Del Río ◽  
...  

Aim: Our objective was to determine changes in bone mineral density (BMD), trabecular bone score (TBS), and body composition after 2 years of therapy with recombinant human insulin-like growth factor-1 (rhIGF-1) in 2 prepubertal children with a complete lack of circulating PAPP-A2 due to a homozygous mutation in PAPP-A2 (p.D643fs25*) resulting in a premature stop codon. Methods: Body composition, BMD, and bone structure were determined by dual-energy X-ray absorptiometry at baseline and after 1 and 2 years of rhIGF-1 treatment. Results: Height increased from 132 to 145.5 cm (patient 1) and from 111.5 to 124.5 cm (patient 2). Bone mineral content increased from 933.40 to 1,057.97 and 1,152.77 g in patient 1, and from 696.12 to 773.26 and 911.51 g in patient 2, after 1 and 2 years, respectively. Whole-body BMD also increased after 2 years of rhIGF-1 from baseline 0.788 to 0.869 g/cm2 in patient 1 and from 0.763 to 0.829 g/cm2 in patient 2. After 2 years of treatment, both children had an improvement in TBS. During therapy, a slight increase in body fat mass was seen, with a concomitant increase in lean mass. No adverse effects were reported. Conclusion: Two years of rhIGF-1 improved growth, with a tendency to improve bone mass and bone microstructure and to modulate body composition.

2018 ◽  
Vol 3 (4) ◽  
pp. 62
Author(s):  
Jose Antonio ◽  
Anya Ellerbroek ◽  
Cassandra Carson

The effects of long-term high-protein consumption (i.e., >2.2 g/kg/day) are unclear as it relates to bone mineral content. Thus, the primary endpoint of this investigation was to determine if consuming a high-protein diet for one year affected various parameters of body composition in exercise-trained women. This investigation is a follow-up to a prior 6-month study. Subjects were instructed to consume a high-protein diet (>2.2 g/kg/day) for one year. Body composition was assessed via dual-energy X-ray absorptiometry (DXA). Subjects were instructed to keep a food diary (i.e., log their food ~three days per week for a year) via the mobile app MyFitnessPal®. Furthermore, a subset of subjects had their blood analyzed (i.e., basic metabolic panel). Subjects consumed a high-protein diet for one year (mean ± SD: 2.3 ± 1.1 grams per kilogram body weight daily [g/kg/day]). There were no significant changes for any measure of body composition over the course of the year (i.e., body weight, fat mass, lean body mass, percent fat, whole body bone mineral content, whole body T-score, whole body bone mineral density, lumbar bone mineral content, lumbar bone mineral density and lumbar T-score). In addition, we found no adverse effects on kidney function. Based on this 1-year within-subjects investigation, it is evident that a diet high in protein has no adverse effects on bone mineral density or kidney function.


2017 ◽  
Vol 176 (2) ◽  
pp. 233-242 ◽  
Author(s):  
Line Cleemann ◽  
Kirsten Holm ◽  
Hanne Kobbernagel ◽  
Bent Kristensen ◽  
Sven Oluf Skouby ◽  
...  

Objective Reduced bone mineral density (BMD) is seen in Turner syndrome (TS) with an increased risk of fractures, and body composition is characterized by increased body fat and decreased lean body mass. To evaluate the effect of two different doses of oral 17B-estradiol in young TS women on bone mineral density (BMD), biochemical markers of bone turnover and body composition with the hypothesis of a positive effect of the higher dose. Design A double-blind 5-year randomized controlled clinical trial. 20 young TS women participated. Inclusion criteria were diagnosis of TS, age 15–25 years and current treatment with 2 mg oral estradiol daily. Methods The low-dose (LD) group was administered 2 mg 17B-estradiol/day orally and placebo, the high-dose (HD) group was administered 2 + 2 mg 17B-estradiol/day orally. Main outcome measures were whole body and regional bone mineral density (BMD), lean body mass (LBM), fat mass (FM) measured yearly by DXA scan and resorptive and formative bone markers in serum. Results BMD, whole body and regional, increased over time with an attenuation toward the end of the study, and bone turnover markers decreased over time, both with no differences between the treatment groups (P = 0.2–0.9). LBM increased significantly more in the HD group (P = 0.02). FM remained stable in both groups. Conclusions A steady increase in BMD over time in TS was found similar to healthy young women. The higher estrogen dose did not differentially affect BMD or bone markers. The positive effect on body composition may have long-ranging health benefits in TS.


2020 ◽  
Vol 9 (7) ◽  
pp. 649-657
Author(s):  
Mojca Zerjav Tansek ◽  
Ana Bertoncel ◽  
Brina Sebez ◽  
Janez Zibert ◽  
Urh Groselj ◽  
...  

Despite recent improvements in the composition of the diet, lower mineral bone density and overweight tendencies are incoherently described in patients with phenylketonuria (PKU). The impact of dietary factors and plasma phenylalanine levels on growth, BMI, body composition, and bone mineral density was investigated in our cohort of patients with hyperphenylalaninemia (HPA) with or without dietary treatment. The anthropometric, metabolic, BMI and other nutritional indicators and bone mineral density were compared between the group of 96 treated patients with PKU (58 classic PKU (cPKU) and 38 patients with moderate-mild PKU defined as non-classic PKU (non-cPKU)) and the untreated group of 62 patients with benign HPA. Having compared the treated and untreated groups, there were normal outcomes and no statistically significant differences in BMI, body composition, and bone mineral density. Lower body height standard deviation scores were observed in the treated as compared to the untreated group (P < 0.001), but the difference was not significant when analyzing patients older than 18 years; however, cPKU adults were shorter compared to non-cPKU treated adults (P = 0.012). Interestingly, the whole-body fat was statistically higher in non-cPKU as compared to cPKU patients. In conclusion, the dietary treatment ensured adequate nutrition without significant consequences in BMI, body composition, and bone mineral density. A low protein diet may have delayed the growth in childhood, but the treated patients gained a normal final height. Mild untreated hyperphenylalaninemia characteristic for benign HPA had no negative physiological effect on bone mineral density.


1996 ◽  
Vol 17 (4) ◽  
pp. 282
Author(s):  
S. Koneru ◽  
J. R. Buscombe ◽  
A. Davenport ◽  
D. J. McCool ◽  
J. E. Agnew ◽  
...  

1998 ◽  
Vol 15 (4) ◽  
pp. 345-356
Author(s):  
Manny Felix ◽  
Jeff McCubbin ◽  
Janet Shaw

Many women with mild to moderate mental retardation (MMR) exhibit low levels of physical activity, muscle strength, and muscle mass, which place these individuals at risk for osteoporosis. Bone mineral density (BMD), the primary index of osteoporosis, of the femoral neck and the whole body was measured in premenopausal women with (M age = 28.14 ± 8.43) and without (M age = 29.64 ± 10.86) mental retardation (MMR and NMR, respectively). Multivariate analyses revealed no differences (p > .05) between groups (MMR = 16, NMR = 16) for BMD values. Significant differences existed (p < .05) between groups on body composition and muscle strength variables. In the MMR group, significant positive relationships (p < .05) were found between lean muscle mass and both femoral neck (r = .74) and whole body (r = .81) BMD. Unaccounted lifestyle factors may have contributed to nonsignificant BMD values between groups.


BMJ Open ◽  
2020 ◽  
Vol 10 (4) ◽  
pp. e032268
Author(s):  
Zeyu Xiao ◽  
Zhiqiang Tan ◽  
Jingjie Shang ◽  
Yong Cheng ◽  
Yongjin Tang ◽  
...  

ObjectivesThis study was an attempt to investigate the variation trend of body composition with ageing and explore the association between regional body composition and bone mineral density (BMD).DesignCross-sectional study.Setting and participantsA total of 5749 healthy adults aged 20–95 years was recruited from 2004 to 2017.Primary outcome measuresWhole-body lean mass (LM), fat mass (FM), android FM, gynoid FM, appendicular lean mass (ALM) and BMD in the lumbar spine, femoral neck and total hip were obtained by dual-energy X-ray absorptiometry (DXA). The android/gynoid fat mass ratio (A/G FMR) based on DXA scan was calculated as an indicator of adipose distribution. Pearson correlation and multiple linear regression analyses were used to determine the associations between body composition, adipose distribution, and BMD of each skeletal site.ResultsWhole-body FM, percentage of whole-body FM, Android FM and A/G FMR consistently increased with age in both genders, especially in women, and ALM began to decrease in the fifth decade for both men and women. In multivariable linear regression models with age, body mass index, A/G FMR and ALM as predictor variables, ALM was associated with the most BMD variance of all skeletal sites in men (standard β ranged from 0.207 to 0.405, p<0.001), although not the largest but still a positive predictor of BMD in women (standard β ranged from 0.074 to 0.186, p<0.05). A/G FMR was an inverse predictor of BMD at all skeletal sites for women (standard β ranged from −249 to −0.052, p<0.01) but not in men.ConclusionsIn this large cohort of Chinese adults, ALM had a strong positive association with BMD in both genders. A/G FMR as an indicator of central adipose accumulation was inversely associated with BMD in women but not in men.


PeerJ ◽  
2021 ◽  
Vol 9 ◽  
pp. e11296
Author(s):  
Valentina Cavedon ◽  
Marco Sandri ◽  
Ilaria Peluso ◽  
Carlo Zancanaro ◽  
Chiara Milanese

Background The impact of the type and the severity of disability on whole-body and regional body composition (BC), and bone mineral density (BMD) must be considered for dietary advice in athletes with a physical impairment (PI). This study aimed to investigate the impact of the type and the severity of disability on BC, the pattern of distribution of fat mass at the regional level, and BMD in athletes with a PI. Methods Forty-two male athletes with spinal cord injury (SCI, n = 24; age = 40.04 ± 9.95 years, Body Mass Index [BMI] = 23.07 ± 4.01 kg/m2) or unilateral lower limb amputation (AMP, n = 18; age = 34.39 ± 9.19 years, BMI = 22.81 ± 2.63 kg/m2) underwent a Dual-Energy X-Ray Absorptiometry scan. Each athlete with a PI was matched by age with an able-bodied athlete (AB, n = 42; age = 37.81 ± 10.31 years, BMI = 23.94 ± 1.8 kg/m2). Results One-Way Analysis of Variance showed significant differences between the SCI, AMP and AB groups for percentage fat mass (%FM) (P < 0.001, eta squared = 0.440). Post-hoc analysis with Bonferroni’s correction showed that athletes with SCI had significantly higher %FM vs. the AMP and AB groups (25.45 ± 5.99%, 21.45 ± 4.21% and 16.69 ± 2.56%, respectively; P = 0.008 vs. AMP and P < 0.001 vs. AB). The %FM was also significantly higher in the AMP vs. the AB group (P < 0.001). Whole-body BMD was negatively affected in SCI athletes, with about half of them showing osteopenia or osteoporosis. In fact, the mean BMD and T-score values in the SCI group (1.07 ± 0.09 g/cm2 and −1.25 ± 0.85, respectively) were significantly lower in comparison with the AB group (P = 0.001 for both) as well as the AMP group (P = 0.008 for both). The type of disability affected BC and BMD in the trunk, android, gynoid and leg regions in SCI athletes and the impaired leg only in AMP athletes. Conclusions In conclusion, the type of disability and, partly, the severity of PI impact on BC and BMD in athletes with a PI. Nutritionists, sports medicine doctors, clinicians, coaches and physical conditioners should consider athletes with SCI or AMP separately. Athletes with a PI would benefit from specific nutrition and training programs taking into account the type of their disability.


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