Literature DB >> 1750755

Physical growth of children treated for phenylketonuria.

M A McBurnie1, R A Kronmal, V E Schuett, R Koch, C G Azeng.   

Abstract

The growth of 133 children participating in the Collaborative Study of Children Treated for Phenylketonuria (PKU) was compared to growth data from the National Center for Health Statistics (NCHS) to determine whether the growth patterns of the children with PKU were the same as those of unaffected children. Height and weight by age, and weight by height, were analysed for ages 2-10 years. Head circumference by age was analysed for ages 2-7 years. Median height by age of the PKU children was consistently near the 50th percentile of the NCHS growth curves for males and females. However, for both sexes, median weight by height and by age was between the 50th and 75th percentiles for children over 3 years old. Two-sample t-tests showed mean weight was significantly different (p less than 0.05) between the PKU and NCHS groups at most ages for both sexes. Median head circumference for the PKU children tended to be smaller than NCHS standards; however, the maximum difference at any age was less than 0.5 cm. Polynomial growth curves fitted to the PKU and NCHS growth data showed that, on average, the PKU males and females weighed more than their unaffected counterparts, while height and head circumference for both groups were very close. Including median serum phenylalanine (phe) level (mg/dl) in the growth curves suggested that the weight differences between the PKU and NCHS groups are related to degree of diet adherence. Higher phe levels in the PKU group were associated with higher weight levels, more so for girls (p less than 0.001) than for boys (p = 0.08). No relationship was found between phe level and height or head circumference. We conclude that growth in children treated for PKU differs from national standards for weight by age and weight by height, but not for height by age. We speculate that diet adherence may be an important factor in determining which children have a tendency to become overweight.

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Year:  1991        PMID: 1750755     DOI: 10.1080/03014469100001662

Source DB:  PubMed          Journal:  Ann Hum Biol        ISSN: 0301-4460            Impact factor:   1.533


  14 in total

1.  Treatment products and approaches for phenylketonuria: improved palatability and flexibility demonstrate safety, efficacy and acceptance in US clinical trials.

Authors:  A P Prince; M P McMurray; N R Buist
Journal:  J Inherit Metab Dis       Date:  1997-08       Impact factor: 4.982

2.  Accuracy of six anthropometric skinfold formulas versus air displacement plethysmography for estimating percent body fat in female adolescents with phenylketonuria.

Authors:  Teresa D Douglas; Mary J Kennedy; Meghan E Quirk; Sarah H Yi; Rani H Singh
Journal:  JIMD Rep       Date:  2012-12-29

3.  Physical growth in patients with phenylketonuria.

Authors:  J L Dhondt; C Largillière; L Moreno; J P Farriaux
Journal:  J Inherit Metab Dis       Date:  1995       Impact factor: 4.982

4.  NORMAL FATTY ACID CONCENTRATIONS IN YOUNG CHILDREN WITH PHENYLKETONURIA (PKU).

Authors:  Stacey M Lavoie; Cary O Harding; Melanie B Gillingham
Journal:  Top Clin Nutr       Date:  2009-10-01       Impact factor: 0.508

5.  Recommendations for protein and energy intakes by patients with phenylketonuria.

Authors:  P B Acosta
Journal:  Eur J Pediatr       Date:  1996-07       Impact factor: 3.183

6.  Impaired prenatal and postnatal growth in Dutch patients with phenylketonuria. The National PKU Steering Committee.

Authors:  P H Verkerk; F J van Spronsen; G P Smit; R C Sengers
Journal:  Arch Dis Child       Date:  1994-08       Impact factor: 3.791

Review 7.  Growth and body composition in children with classical phenylketonuria: results in 34 patients and review of the literature.

Authors:  M Huemer; C Huemer; D Möslinger; D Huter; S Stöckler-Ipsiroglu
Journal:  J Inherit Metab Dis       Date:  2007-07-11       Impact factor: 4.982

Review 8.  The complete European guidelines on phenylketonuria: diagnosis and treatment.

Authors:  A M J van Wegberg; A MacDonald; K Ahring; A Bélanger-Quintana; N Blau; A M Bosch; A Burlina; J Campistol; F Feillet; M Giżewska; S C Huijbregts; S Kearney; V Leuzzi; F Maillot; A C Muntau; M van Rijn; F Trefz; J H Walter; F J van Spronsen
Journal:  Orphanet J Rare Dis       Date:  2017-10-12       Impact factor: 4.123

9.  Whole body composition analysis by the BodPod air-displacement plethysmography method in children with phenylketonuria shows a higher body fat percentage.

Authors:  Monique Albersen; Marjolein Bonthuis; Nicole M de Roos; Dorine A M van den Hurk; Ems Carbasius Weber; Margriet M W B Hendriks; Monique G M de Sain-van der Velden; Tom J de Koning; Gepke Visser
Journal:  J Inherit Metab Dis       Date:  2010-06-24       Impact factor: 4.982

Review 10.  Phenylketonuria: dietary and therapeutic challenges.

Authors:  M Giovannini; E Verduci; E Salvatici; L Fiori; E Riva
Journal:  J Inherit Metab Dis       Date:  2007-03-08       Impact factor: 4.750

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