scholarly journals A Three-Year Longitudinal Study Comparing Bone Mass, Density, and Geometry Measured by DXA, pQCT, and Bone Turnover Markers in Children with PKU Taking L-Amino Acid or Glycomacropeptide Protein Substitutes

Nutrients ◽  
2021 ◽  
Vol 13 (6) ◽  
pp. 2075
Author(s):  
Anne Daly ◽  
Wolfgang Högler ◽  
Nicola Crabtree ◽  
Nick Shaw ◽  
Sharon Evans ◽  
...  

In patients with phenylketonuria (PKU), treated by diet therapy only, evidence suggests that areal bone mineral density (BMDa) is within the normal clinical reference range but is below the population norm. Aims: To study longitudinal bone density, mass, and geometry over 36 months in children with PKU taking either amino acid (L-AA) or casein glycomacropeptide substitutes (CGMP-AA) as their main protein source. Methodology: A total of 48 subjects completed the study, 19 subjects in the L-AA group (median age 11.1, range 5–6 years) and 29 subjects in the CGMP-AA group (median age 8.3, range 5–16years). The CGMP-AA was further divided into two groups, CGMP100 (median age 9.2, range 5–16years) (n = 13), children taking CGMP-AA only and CGMP50 (median age 7.3, range 5–15years) (n = 16), children taking a combination of CGMP-AA and L-AA. Dual X-ray absorptiometry (DXA) was measured at enrolment and 36 months, peripheral quantitative computer tomography (pQCT) at 36 months only, and serum blood and urine bone turnover markers (BTM) and blood bone biochemistry at enrolment, 6, 12, and 36 months. Results: No statistically significant differences were found between the three groups for DXA outcome parameters, i.e., BMDa (L2–L4 BMDa g/cm2), bone mineral apparent density (L2–L4 BMAD g/cm3) and total body less head BMDa (TBLH g/cm2). All blood biochemistry markers were within the reference ranges, and BTM showed active bone turnover with a trend for BTM to decrease with increasing age. Conclusions: Bone density was clinically normal, although the median z scores were below the population mean. BTM showed active bone turnover and blood biochemistry was within the reference ranges. There appeared to be no advantage to bone density, mass, or geometry from taking a macropeptide-based protein substitute as compared with L-AAs.

2016 ◽  
Vol 62 (5) ◽  
pp. 22-23
Author(s):  
Mariska Caroline Vlot ◽  
Daniel T. Klink ◽  
Martin Den Heijer ◽  
Marinus A. Blankenstein ◽  
Joost Rotteveel ◽  
...  

Background: Puberty is highly important for the accumulation of bone mass. Bone turnover and bone mineral density can be affected in transgender adolescents when puberty is suppressed by gonadotropin-releasing hormone analogues (GnRHa), followed by treatment with cross-sex hormone therapy (CSHT).Objective: To investigate the effect of GnRHa and CSHT on bone turnover markers (BTMs) and bone mineral apparent density (BMAD) in transgender adolescents.Methods: Thirty four female-to-males (FtMs) and 22 male-to-females (MtFs) were divided into a young and old pubertal group, based on the bone age of 14 years in the FtMs and 15 years in the MtFs. All patients received GnRHa triptorelin. CSHT was prescribed in incremental doses from the age of 16 years. FtMs received testosterone ester mixture and MtFs were treated with 17-β estradiol. BTMs P1NP, osteocalcin and ICTP and the BMD of lumbar spine (LS) and femoral neck (FN) were measured at three time points. Furthermore, BMAD and Z-scores were calculated.Results: P1NP and 1CTP decreased during GnRHa treatment, indicating decreased bone turnover. Osteocalcin showed an aberrant pattern. A low BMAD Z-score of both FN and LS was observed in the MtFs at start of GnRHa treatment. The decrease in bone turnover upon GnRHa treatment was accompanied by an unchanged BMAD of both FN and LS, however BMAD Z-scores of predominantly the LS decreased. Twenty-four months after CSHT the BTMs P1NP and ICTP were even more decreased. During CSHT BMAD Z-scores increased and returned towards normal, especially of the LS.Conclusion: Suppressing puberty by GnRHa leads to a decrease of BTMs in transgender adolescents. The increase of BMAD and BMAD Z-scores predominantly in the LS as a result of treatment with CSHT is accompanied by decreasing BTM concentrations after 24 months of CSHT. Therefore, the added value of evaluating BTMs seems to be limited and DEXA-scans remain important in follow-up of transgender adolescents. 


2017 ◽  
Vol 99 (7) ◽  
pp. 559-562 ◽  
Author(s):  
P Rajeev ◽  
A Movseysan ◽  
A Baharani

Introduction Involvement of the bone is common in primary hyperparathyroidism. The aim of the study was to assess bone turnover markers in response to surgery for primary hyperparathyroidism. Methods This was a retrospective study of patients diagnosed and treated for parathyroid disease between 2005 and 2012. Interventions studied were surgery and medical treatment. The main outcome measures studied were serum levels of calcium, intact parathyroid hormone (iPTH), bone-specific alkaline phosphatase, N-terminal cross-linking propeptide of type 1 procollagen (P1NP) and C-terminal cross-linking telopeptides of type I collagen (CTX), both pre- and postoperatively at 6 months and 1 year; bone mineral density (at the spine and hip assessed by dual-energy x-ray absorptiometry after 1 year of treatment. Results A total of 122 (110 female, 12 male) patients (age range 25–91 years) underwent treatment for parathyroid disease during the study period; 30 patients were treated conservatively and 92 proceeded to surgery following localisation studies. Following surgical intervention, P1NP dropped significantly from a mean of 64.68 ng/ml (standard deviation, SD ± 68.07 ng/ml) preoperatively to 26.37 ng/ml (SD ± 20.94 ng/ml) and CTX from 0.69 pg/ml (SD ± 0.44 pg/ml) to 0.15 pg/ml (SD ± 0.16 pg/ml) at 6–12 months (P < 0.0001). This change was reflected in improvement in bone mineral density (T scores) of the hip and spine by 43% (P < 0.03) and 38% (P < 0.01), respectively, following surgery. In patients treated conservatively (n = 30), there was no improvement either in the bone turnover markers or bone densitometry scans. Conclusions Surgery improves bone density in patients with parathyroid disease. Improvement in serum bone turnover markers is seen following parathyroidectomy. The association with bone density needs further evaluation in larger studies.


2013 ◽  
Vol 2013 ◽  
pp. 1-7 ◽  
Author(s):  
Wei-Wei Hu ◽  
Zeng Zhang ◽  
Jin-Wei He ◽  
Wen-Zhen Fu ◽  
Chun Wang ◽  
...  

The reference ranges of bone turnover markers (BTMs) were important during the treatment of osteoporosis, and the associations with bone mineral density (BMD) were controversial. The aim of this study was to establish the reference ranges of N-terminal procollagen of type l collagen (P1NP), osteocalcin (OC), and beta C-terminal cross-linked telopeptides of type I collagen (β-CTX) in Shanghai area and to investigate the relationships between BTMs and BMD in postmenopausal women. 2,799 subjects recruited in Shanghai City were measured BTMs to establish the reference ranges. Additional 520 healthy postmenopausal women were also measured BTMs, these women measured BMD in addition. BTMs were measured using the Roche electrochemiluminescence system. We used the age range of 35 to 45-year-olds to calculate reference intervals. The reference range of OC was 4.91 to 13.90 ng/mL for women and 5.58 to 16.57 ng/mL for men, P1NP was 13.72 to 32.90 ng/mL for women and 16.89 to 42.43 ng/mL for men, andβ-CTX was 0.112 to 0.210 ng/mL for women and 0.100 to 0.378 ng/mL for men. BTMs significantly negatively correlated with lumbar spine and femoral and total hip in postmenopausal women ( = −0.157~−0.217,P< 0.001). We established the normal reference ranges of P1NP, OC, andβ-CTX in the Shanghai area. This study also found that BTMs correlated with BMD and suggested that BTMs were the key determining factors of early BMD decreases.


2014 ◽  
Author(s):  
Mingo Dominguez Maria Luisa de ◽  
Sonsoles Guadalix Iglesias ◽  
Maria Begona Lopez Alvarez ◽  
Guillermo Martinez Diaz-Guerra ◽  
Federico Hawkins Carranza

2019 ◽  
Vol 17 (4) ◽  
pp. 102-106
Author(s):  
M. Yu. Smetanin ◽  
◽  
S. Yu. Nurgalieva ◽  
N. Yu. Kononova ◽  
L. T. Pimenov ◽  
...  

2016 ◽  
Vol 2016 ◽  
pp. 1-6 ◽  
Author(s):  
A. Sánchez ◽  
L. R. Brun ◽  
H. Salerni ◽  
P. R. Costanzo ◽  
D. González ◽  
...  

The aim of this study was to evaluate the effect of denosumab (Dmab) on bone mineral density (BMD) and bone turnover markers after 1 year of treatment. Additionally, the effect of Dmab in bisphosphonate-naïve patients (BP-naïve) compared to patients previously treated with bisphosphonates (BP-prior) was analyzed. This retrospective study included 425 postmenopausal women treated with Dmab for 1 year in clinical practice conditions in specialized centers from Argentina. Participants were also divided according to previous bisphosphonate treatment into BP-naïve and BP-prior. A control group of patients treated with BP not switched to Dmab matched by sex, age, and body mass index was used. Data are expressed as mean ± SEM. After 1 year of treatment with Dmab the bone formation markers total alkaline phosphatase and osteocalcin were significantly decreased (23.36% and 43.97%, resp.), as was the bone resorption marker s-CTX (69.61%). Significant increases in BMD were observed at the lumbar spine, femoral neck, and total hip without differences between BP-naïve and BP-prior. A better BMD response was found in BP-prior group compared with BP treated patients not switched to Dmab.Conclusion. Dmab treatment increased BMD and decreased bone turnover markers in the whole group, with similar response in BP-naïve and BP-prior patients. A better BMD response in BP-prior patients versus BP treated patients not switched to Dmab was observed.


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