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Titolo:
BODY PROTEIN IN PREPUBERTAL CHILDREN WITH PHENYLKETONURIA
Autore:
ALLEN JR; BAUR LA; WATERS DL; HUMPHRIES IR; ALLEN BJ; ROBERTS DCK; GASKIN KJ;
Indirizzi:
ROYAL ALEXANDRA HOSP CHILDREN,JAMES FAIRFAX INST PAEDIAT NUTR,POB 34 CAMPERDOWN NSW 2050 AUSTRALIA ROYAL ALEXANDRA HOSP CHILDREN,JAMES FAIRFAX INST PAEDIAT NUTR CAMPERDOWN NSW 2050 AUSTRALIA UNIV NEWCASTLE,FAC HLTH SCI,DISCIPLINE NUTR & DIABET NEWCASTLE NSW 2308 AUSTRALIA
Titolo Testata:
European journal of clinical nutrition
fascicolo: 3, volume: 50, anno: 1996,
pagine: 178 - 186
SICI:
0954-3007(1996)50:3<178:BPIPCW>2.0.ZU;2-M
Fonte:
ISI
Lingua:
ENG
Soggetto:
FUNDAMENTAL PRINCIPLES; ENERGY PHYSIOLOGY; PHYSICAL GROWTH; NITROGEN; ACID; PKU; BIRTH; AGE;
Keywords:
TOTAL BODY NITROGEN; DIETARY PROTEINS; NUTRITIONAL STATUS; GROWTH; BODY COMPOSITION; NEUTRON CAPTURE ANALYSIS;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Citazioni:
50
Recensione:
Indirizzi per estratti:
Citazione:
J.R. Allen et al., "BODY PROTEIN IN PREPUBERTAL CHILDREN WITH PHENYLKETONURIA", European journal of clinical nutrition, 50(3), 1996, pp. 178-186

Abstract

Objective: To assess body protein and protein deposition in prepubertal children with phenylketonuria (PKU). Design: Cross-sectional study with nested longitudinal cohort. Setting: A tertiary referral paediatric hospital. Subjects: 37 PKU patients (3.9-11.0 years) and 27 unselected healthy controls (4.0-11.5 years) of whom 29 PKU patients and 17 controls were followed longitudinally. Interventions: All had measurements of height, weight, body fat and total body nitrogen (TEN) by neutron capture analysis; PKU patients and their unaffected siblings (n = 16) also had measurements of four day weighed food record and plasma amino acids by HPLC. Results: The children with PKU compared with the controls were significantly shorter (height SD score -0.42+/-O.89 vs 0.17+/-0.94, respectively, P < 0.02) and had a lower TEN (575 +/- 200 vs 710 +/- 215 g, respectively, P < 0.02). TBN in the controls was significantly correlated with lean body mass (LBM), weight, height and age (r = 0.97, 0.95, 0.95, 0.88, respectively, P < 0.001). The children with PKU had significantly lower TEN when predicted from LBM, weight and age (93%, 92%, 92% of predicted, respectively), but normal TEN predicted from height (102% of expected). The annual accretion of nitrogen was similar for the PKU and controls (86 +/- 45 and 77 +/- 58g/y, respectively). There was no difference between the two groups in protein intake or plasma amino acids except for phenylalanine. Conclusion: The children with PKU had a deficit in height and body protein despite a normal to higher accretion of protein. If the deficit occurs early in life, amino acid supplementation and other nutritional practices used at this time need to be reviewed.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 18/09/20 alle ore 10:30:23