title: Effects of Short-Term Calcium Supplementation in Children and Adolescents with Phenylketonuria.
abstract: Reduction of bone mineral density and the risk of osteopenia have been reported to occur in phenylketonuria (PKU) patients. This study aimed to evaluate the short-term effects of calcium supplementation in phenylketonuric children and adolescents. The study included 18 patients with PKU aged 5-18 yr (61% male) under clinical and nutritional treatment. Evaluation of food intake, anthropometry, and biochemical and phalangeal quantitative ultrasound were performed before (phase 1) and after (phase 2) calcium supplementation (1000 mg/d) for 34 d. Statistical analysis was performed using t test for paired samples, Wilcoxon's test, and McNemar's test (p <0.05). There was an inadequate intake of phosphorus and vitamin D, the same occurring with serum concentrations of these nutrients. About 50% of the patients had an accumulation of adipose tissue measures, with a negative correlation between Z-score, body mass index, and phalangeal quantitative ultrasound (amplitude-dependent speed of sound [AD-SoS]). There was a significant difference in urinary phosphorus excretion with higher values before supplementation. Comparison of the two phases revealed significantly higher AD-SoS values after the supplementation (p = 0.017). The reduction in phosphorus excretion associated with increased AD-SoS between the two phases suggested increased bone formation, and showed no negative effects in relation to short-term calcium supplementation in children and in adolescents with PKU.
authors.affiliation: Faculty of Medicine of Ribeirao Preto, University of São Paulo, Ribeirão Preto, São Paulo, Brazil. Electronic address: nancyyyt@hotmail.com.
authors.forename: Nancy Y Y
authors.initials: NYY
authors.lastname: Tanaka
authors.affiliation: Faculty of Medicine of Ribeirao Preto, University of São Paulo, Ribeirão Preto, São Paulo, Brazil.
authors.forename: Marlene F
authors.initials: MF
authors.lastname: Turcato
authors.affiliation: Faculty of Medicine of Ribeirao Preto, University of São Paulo, Ribeirão Preto, São Paulo, Brazil.
authors.forename: Carolina F
authors.initials: CF
authors.lastname: Nicoletti
authors.affiliation: Faculty of Medicine of Ribeirao Preto, University of São Paulo, Ribeirão Preto, São Paulo, Brazil.
authors.forename: Carla B
authors.initials: CB
authors.lastname: Nonino
authors.affiliation: Faculty of Medicine of Ribeirao Preto, University of São Paulo, Ribeirão Preto, São Paulo, Brazil.
authors.forename: Luciana D
authors.initials: LD
authors.lastname: Martins
authors.affiliation: Faculty of Medicine of Ribeirao Preto, University of São Paulo, Ribeirão Preto, São Paulo, Brazil.
authors.forename: Odilon
authors.initials: O
authors.lastname: Iannetta
authors.affiliation: Faculty of Medicine of Ribeirao Preto, University of São Paulo, Ribeirão Preto, São Paulo, Brazil.
authors.forename: Carlos T
authors.initials: CT
authors.lastname: Guerreiro
authors.affiliation: Faculty of Medicine of Ribeirao Preto, University of São Paulo, Ribeirão Preto, São Paulo, Brazil.
authors.forename: Gisele G
authors.initials: GG
authors.lastname: Santos
authors.affiliation: Faculty of Medicine of Ribeirao Preto, University of São Paulo, Ribeirão Preto, São Paulo, Brazil.
authors.forename: Júlio S
authors.initials: JS
authors.lastname: Marchini
mesh_terms: D050154:Adiposity; D000293:Adolescent; D015992:Body Mass Index; D015519:Bone Density; D002118:Calcium / Q000008:administration & dosage / Q000097:blood* / Q000627:therapeutic use* / Q000652:urine; D002648:Child; D002675:Child, Preschool; D004032:Diet; D019587:Dietary Supplements; D005260:Female; D050278:Finger Phalanges / Q000000981:diagnostic imaging; D006801:Humans; D008297:Male; D010649:Phenylalanine / Q000097:blood; D010661:Phenylketonurias / Q000150:complications / Q000188:drug therapy* / Q000378:metabolism*; D010758:Phosphorus / Q000097:blood / Q000652:urine*; D013997:Time Factors; D014463:Ultrasonography; D014807:Vitamin D / Q000097:blood
chemical_list: D014807:Vitamin D; D010758:Phosphorus; D010649:Phenylalanine; D002118:Calcium
keywords: Bone mass density; calcium; dietary supplementation; phenylketonuria