title: New insights in growth of phenylketonuric patients.
abstract: Treatment of phenylketonuria involves a restriction in the intake of natural proteins. This can lead to growth impairment. Weight, height and body mass index of 109 hyperphenylalaninemic patients (mild hyperphenylalaninemia (HPA) and phenylketonuria (PKU)) were determined from birth until 18 years, every 6 months, and differences to the healthy population, depending on the age, sex and phenotype, were analyzed. Data collection was longitudinal retrospective during 31 years. Statistical analysis of z-score values was performed by advanced statistical tools. Long-term evolution of anthropometric z-scores showed no significant statistical differences between PKU and mild HPA individuals, according to the general population. For PKU individuals, height is slightly lower and weight slightly higher than in the healthy population, but differences are smaller than one standard deviation. Nevertheless, over-time evolutions of female height z-scores are different in each type of pathology, with a crossover between 8 and 12 years (p = 0.0186).
  
  
  CONCLUSIONS
  It is nowadays possible to achieve a long-term normal growth in PKU patients with appropriate dietary treatment. There is however an acceleration of growth up to 8 years old for PKU female patients that leads to a slightly lower final height. Detection of this behaviour was possible by using nonlinear mixed effects models.
authors.affiliation: Unit of Diagnosis and Treatment of Congenital Metabolic Diseases, Department of Pediatrics, Hospital Clínico Universitario de Santiago de Compostela, A Choupana s/n,, 15706, Santiago de Compostela, A Coruña, Spain, maria.luz.couce.pico@sergas.es.
authors.forename: María L
authors.initials: ML
authors.lastname: Couce
authors.forename: Ipek
authors.initials: I
authors.lastname: Guler
authors.forename: Andrés
authors.initials: A
authors.lastname: Anca-Couce
authors.forename: Marta
authors.initials: M
authors.lastname: Lojo
authors.forename: Alicia
authors.initials: A
authors.lastname: Mirás
authors.forename: Rosaura
authors.initials: R
authors.lastname: Leis
authors.forename: Alejandro
authors.initials: A
authors.lastname: Pérez-Muñuzuri
authors.forename: José M
authors.initials: JM
authors.lastname: Fraga
authors.forename: Francisco
authors.initials: F
authors.lastname: Gude
mesh_terms: D000293:Adolescent; D041923:Adolescent Development / Q000502:physiology*; D001827:Body Height / Q000502:physiology; D015992:Body Mass Index; D001835:Body Weight / Q000502:physiology; D002648:Child; D002657:Child Development / Q000502:physiology*; D002675:Child, Preschool; D018753:Diet, Protein-Restricted; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008137:Longitudinal Studies; D008297:Male; D017711:Nonlinear Dynamics; D010661:Phenylketonurias / Q000178:diet therapy*; D012189:Retrospective Studies