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      Growth and Metabolic Control during Puberty in Girls with X-Linked Hypophosphataemic Rickets

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          Objective: X-linked hypophosphataemic rickets (XLH) results in defective bone mineralization and impaired growth. Treatment with oral phosphate (Pi) and calcitriol improves but does not normalize growth. This study assessed whether pubertal growth and metabolic control contribute to the height deficit. Methods: Study included patients with XLH who were treated with Pi-calcitriol from diagnosis to adult height; their hospital records, biochemistry and radiographs were reviewed. Results: Six females with XLH were included. Their mean peak height velocity and total height gain during puberty were nearly normal despite deteriorating metabolic control. Conclusions: In treated girls with XLH, the pubertal growth is nearly normal despite suboptimal metabolic control. The major height loss occurs prior to puberty and is not recovered during the pubertal growth spurt.

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          Effect of growth hormone treatment on final height, phosphate metabolism, and bone mineral density in children with X-linked hypophosphatemic rickets.

          To evaluate the effect of growth hormone treatment on final height, phosphate metabolism, bone markers, and bone mineral density in children with X-linked hypophosphatemic rickets. Six patients (aged 7.8 +/- 1.8 years; height z score, -3.4 +/- 0.5) received conventional treatment (1,25-dihydroxyvitamin D(3) plus phosphate salts) combined with growth hormone (0.6-0.9 IU/kg per week, subcutaneously) (group A); 6 patients (aged 7.9 +/- 2.5 years; height z score, -2.1 +/- 0.6, P <.01 with respect to group A) received only conventional treatment (group B). Final height z score significantly improved in group A (-2.4 +/- 0.5, P <.03 with respect to the value at entry), whereas it did not change in group B (-2.8 +/- 0.5, P = NS). At final height, degree of body disproportion was similar between the groups (group A, 1.3 +/- 0.2; group B, 1.2 +/- 0.1; P = NS). Phosphate retention, bone markers, and radial bone mineral density increased only in group A. No adverse effects were observed. In poorly growing patients with X-linked hypophosphatemic rickets, growth hormone therapy combined with conventional treatment improves final height, phosphate retention, and radial bone mineral density, but it does not influence degree of body disproportion.

            Author and article information

            Horm Res Paediatr
            Hormone Research in Paediatrics
            S. Karger AG
            April 2004
            23 April 2004
            : 61
            : 5
            : 252-256
            aDivision of Endocrinology and bDiagnostic Imaging, Hospital for Sick Children, University of Toronto, Toronto, Ont., Canada; cDivision of Endocrinology, Hospital for Children and Adolescents, University of Helsinki, Helsinki, Finland
            77401 Horm Res 2004;61:252–256
            © 2004 S. Karger AG, Basel

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            Page count
            Figures: 1, Tables: 2, References: 21, Pages: 5
            Original Paper


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