scholarly journals Growth and Body Composition in PKU Children—A 3 Year Prospective Study Comparing the Effects of L-Amino Acid to Glycomacropeptide Protein Substitutes

2021 ◽  
Author(s):  
Anne Daly ◽  
Wolfgang Hogler ◽  
Nicola Crabtree ◽  
Nick Shaw ◽  
Sharon Evans ◽  
...  
Keyword(s):  
Body Composition ◽  
Prospective Study ◽  
Protein Quality ◽  
Blood Concentrations ◽  
Dxa Scan ◽  
X Ray ◽  
Z Scores ◽  
The Impact

Abstract Background: Protein quality and quantity are important factors in determining lean body (muscle) mass (LBM). In phenylketonuria (PKU), protein substitutes provide most of the nitrogen either as amino acids (AA) or glycomacropeptide (CGMP-AA). Body composition and growth are important indicators of long-term health. In a 3-year prospective study comparing the impact of AA and CGMP AA on body composition and growth in PKU, 48 children were recruited. N=19, (median age 11.1y, range 5-15y) took AA only, n=16 (median age 7.3y, range 5-15y) took a combination of CGMP-AA and AA (CGMP50), and 13 children (median age 9.2y, range 5-16y) took CGMP-AA only (CGMP100). A dual energy X ray absorptiometry (DXA) scan at enrolment and 36 months measured LBM, % body fat (%BF) and fat mass (FM). Height was measured at enrolment, 12, 24 and 36 months. Results: No correlation or statistically significant differences were found between the three groups for LBM, FM and height compared with age, gender, puberty or phenylalanine blood concentrations. The change in height z scores: AA 0, CGMP50 +0.4 and CGMP100 +0.7 showed a trend that children in the CGMP100 group were taller, had improved LBM with decreased FM and % BF but this was not statistically significant. Conclusion: There appeared to be no advantage of AA compared to CGMP-AA protein substitutes on body composition after 3 years follow up of usage. Although statistical differences were not reached, a trend towards improved body composition was observed with CGMP when it provided the entire protein substitute requirement.Trial registry: IRAS 129497 (13/WM/10435) registered 10th November 2013 (hppt// www.hra.nhs)

scholarly journals Growth and Body Composition in PKU Children—A Three-Year Prospective Study Comparing the Effects of L-Amino Acid to Glycomacropeptide Protein Substitutes

Nutrients ◽  
2021 ◽  
Vol 13 (4) ◽  
pp. 1323
Author(s):  
Anne Daly ◽  
Wolfgang Högler ◽  
Nicola Crabtree ◽  
Nick Shaw ◽  
Sharon Evans ◽  
...  
Keyword(s):  
Amino Acids ◽  
Body Composition ◽  
Prospective Study ◽  
Protein Quality ◽  
Blood Concentrations ◽  
Dxa Scan ◽  
Z Scores ◽  
The Impact

Protein quality and quantity are important factors in determining lean body (muscle) mass (LBM). In phenylketonuria (PKU), protein substitutes provide most of the nitrogen, either as amino acids (AA) or glycomacropeptide with supplementary amino acids (CGMP-AA). Body composition and growth are important indicators of long-term health. In a 3-year prospective study comparing the impact of AA and CGMP-AA on body composition and growth in PKU, 48 children were recruited. N = 19 (median age 11.1 years, range 5–15 years) took AA only, n = 16 (median age 7.3 years, range 5–15 years) took a combination of CGMP-AA and AA, (CGMP50) and 13 children (median age 9.2 years, range 5–16 years) took CGMP-AA only (CGMP100). A dual energy X-ray absorptiometry (DXA) scan at enrolment and 36 months measured LBM, % body fat (%BF) and fat mass (FM). Height was measured at enrolment, 12, 24 and 36 months. No correlation or statistically significant differences (after adjusting for age, gender, puberty and phenylalanine blood concentrations) were found between the three groups for LBM, %BF, FM and height. The change in height z scores, (AA 0, CGMP50 +0.4 and CGMP100 +0.7) showed a trend that children in the CGMP100 group were taller, had improved LBM with decreased FM and % BF but this was not statistically significant. There appeared to be no advantage of CGMP-AA compared to AA on body composition after 3-years of follow-up. Although statistically significant differences were not reached, a trend towards improved body composition was observed with CGMP-AA when it provided the entire protein substitute requirement.

Treatment of acromegaly has substantial effects on body composition. A long-term follow-up study.

2021 ◽  
Author(s):  
Peter Wolf ◽  
Sylvie Salenave ◽  
Emmanuel Durand ◽  
Jacques Young ◽  
Peter Kamenicky ◽  
...  
Keyword(s):  
Body Composition ◽  
Fat Mass ◽  
Lean Mass ◽  
Individual Therapy ◽  
Sensitivity Index ◽  
Igf I ◽  
The Individual ◽  
The Impact

  Background: Acromegaly is associated with changes in body composition. Long-term changes following acromegaly treatment and the impact of different treatments have been less investigated. Methods: We performed a retrospective study in 201 patients with acromegaly. Body composition was assessed by dual-energy X-ray absorptiometry (DXA). To investigate specific effects of treatment vs ageing, changes in body composition were compared in a group of patients evaluated both at the time of active and controlled disease (A>C; n=31) and in another group of patients evaluated two times while the disease was controlled (C>C; n=32). Results: In the whole cohort, IGF-I correlated with fat (r=-0.369;p<0.001) and lean mass (r=0.383;p<0.001). Patients from A>C and C>C groups were comparable for age, sex, BMI and follow-up duration (p=n.s.). Reduction in IGF-I levels was associated with an increase in fat mass and a decrease in lean mass in the A>C group, which was four and eight times more pronounced compared to the C>C group (fat mass: +39±34 vs +10±15%, p<0.001; lean mass: -8±8 vs -0.2±6%, p<0.001, respectively). Changes in fat mass were negatively associated with IGF-I (r=-0.450; p=0.011) and independent of the individual therapy. The daily dose of pegvisomant correlated with fat mass (r=0.421;p=0.002) and insulin sensitivity index (r=-0.466;p<0.001). Conclusions: Treatment of acromegaly strongly impacts body composition until biochemical disease remission, characterized by an increase in fat mass and a decrease in lean mass. These changes are closely associated with the normalization of IGF-I. Thereafter, body composition changes are similar to what is observed with ageing.

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.

scholarly journals Long-term effects of a modified, low-protein infant formula on growth and body composition: Follow-up of a randomized, double-blind, equivalence trial

2021 ◽  
Author(s):  
Stefanie M.P. Kouwenhoven ◽  
Nadja Antl ◽  
Martijn J.J. Finken ◽  
Jos W.R. Twisk ◽  
Eline M. van der Beek ◽  
...  
Keyword(s):  
Body Composition ◽  
Infant Formula ◽  
Long Term Effects ◽  
Double Blind ◽  
Equivalence Trial ◽  
Low Protein
Sign in / Sign up

Export Citation Format

Share Document