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Titolo:
GROWTH AND SKELETAL MATURATION IN CHILDREN WITH PHENYLKETONURIA
Autore:
SCHAEFER F; BURGARD P; BATZLER U; RUPP A; SCHMIDT H; GILLI G; BICKEL H; BREMER HJ;
Indirizzi:
UNIV HEIDELBERG,CHILDRENS HOSP,NEUENHEIMER FELD 150 D-69120 HEIDELBERG GERMANY GERMAN COLLABORAT STUDY CHILDREN TREATED PHENYLKE STUTTGART GERMANY BADEN WURTTEMBERG STATE HLTH OFF STUTTGART GERMANY OLGA HOSP STUTTGART GERMANY
Titolo Testata:
Acta paediatrica
fascicolo: 5, volume: 83, anno: 1994,
pagine: 534 - 541
SICI:
0803-5253(1994)83:5<534:GASMIC>2.0.ZU;2-0
Fonte:
ISI
Lingua:
ENG
Soggetto:
PHENYLALANINE-FREE PRODUCT; LOW-BIRTH-WEIGHT; PHYSICAL GROWTH; COGNITIVE-ABILITIES; SCHOOL AGE;
Keywords:
BONE AGE; GROWTH; HEAD CIRCUMFERENCE; PHENYLKETONURIA;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Citazioni:
37
Recensione:
Indirizzi per estratti:
Citazione:
F. Schaefer et al., "GROWTH AND SKELETAL MATURATION IN CHILDREN WITH PHENYLKETONURIA", Acta paediatrica, 83(5), 1994, pp. 534-541

Abstract

Growth and skeletal maturation was evaluated in 82 children participating in the German Collaborative Study of Children Treated for Phenylketonuria (PKU). Height, weight, head circumference and bone age were recorded at regular intervals for the first 6 years of life. The mean SD score (SDS) for height was not significantly different from zero at study entry, but decreased mainly during the second year of life to a nadir of -0.78 in boys and -0.54 in girls at 2.5 years. During the subsequent years, a significant trend towards a regain of height SDS was noted in both sexes. Weight-for-height SDS was close to zero in both sexes, with a significant continuous increasing trend throughout the observation period. Head circumference SDS decreased in boys during the first year of life from -0.28 to -0.68, whereas girls showed only a minor change. During the further follow-up period, head circumference SDS remained at approximately -0.3 in boys and 0.0 in girls. While the mean verbal and performance IQ of the total study population at 5 and 6years of age did not differ from a group of 212 healthy non-PKU children, patients with a head circumference SDS less than the population median at 2 years of age exhibited poorer cognitative abilities at school age than those patients with a relative head size greater than the population median. The children with a head circumference less than the median at 2 years had smaller head sizes already at birth; in addition, the change in relative head size during the first 2 years was correlated significantly with cognitive abilities at school age in boys. Mean bone age was identical to chronological age at each time point of observation. The rate of maturation was one year of bone age per year of chronological age. No correlation between phenylalanine intake or phenylalanine concentrations and the rates of body or head growth or skeletal maturation could be established. We conclude that despite adequate weight gain, moderate growth retardation occurred during the first2 years of life in this group of children treated for PKU. Growth wasmore compromised in boys than in girls and tended to be compensated during later follow-up. Early infantile head circumference and growth appear to be predictors of cognitive development.

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Documento generato il 04/04/20 alle ore 12:19:06