title: Long-term growth hormone treatment in children with renal hypophosphatemic rickets: effects on growth, mineral metabolism, and bone density.
abstract: OBJECTIVE
  To evaluate the effects of treatment with recombinant human growth hormone (rhGH) on growth, mineral metabolism, and bone density in children with renal hypophosphatemic rickets (RHR).
  
  
  METHODS
  Long-term rhGH treatment combined with conventional therapy with 1,25-dihydroxyvitamin D3 plus inorganic phosphate salts.
  
  
  METHODS
  Endocrine unit, department of pediatrics, university hospital.
  
  
  METHODS
  Twelve patients (5 boys; age range 4.6 to 12.5 years, median 7.0 years) were subdivided into two groups of six patients on the basis of the median of height z score (-2.41) and the median bone age/statural age (BA/SA) ratio (1.23). Group A included patients with a severe degree of short stature (height z score -3.4 +/- 0.5) (mean +/- SD) and altered BA/SA ratio (1.26 +/- 0.08); group B included patients with a lesser degree of short stature (height z score -2.1 +/- 0.6, p < 0.001 vs group A) and more normal BA/SA ratio (1.04 +/- 0.15, p < 0.01 vs group A).
  
  
  METHODS
  Group A received rhGH treatment (0.6 IU/kg per week subcutaneously) combined with conventional therapy; group B received conventional therapy alone.
  
  
  METHODS
  Height, growth velocity, predicted adult height, serum values of calcium, phosphate, bone alkaline phosphatase isoenzyme, osteocalcin, propeptides of type I and type III procollagen, intact parathyroid hormone, 25-hydroxyvitamin D, 1,25-dihydroxyvitamin D, and urinary calcium/urinary creatinine ratio and tubular maximum for phosphate reabsorption normalized to the glomerular filtration rate (TmP/GFR), as well as radial bone density, were measured at baseline and for 3 years.
  
  
  RESULTS
  Height z score, growth velocity z score, predicted adult height, serum values of phosphate, bone alkaline phosphatase isoenzyme, osteocalcin, propeptides of type I and type III procollagen, intact parathyroid hormone 1,25-dihydroxyvitamin D, and TmP/GFR, as well as radial bone density, improved significantly only in group A. Serum calcium and 25-hydroxyvitamin D, and urinary calcium/urinary creatinine ratio did not change in either group.
  
  
  CONCLUSIONS
  Long-term rhGH administration may benefit growth, phosphate retention, and bone density in patients with RHR, without evidence of side effects.
authors.affiliation: Department of Pediatrics, University of Pisa, Italy.
authors.forename: G
authors.initials: G
authors.lastname: Saggese
authors.forename: G I
authors.initials: GI
authors.lastname: Baroncelli
authors.forename: S
authors.initials: S
authors.lastname: Bertelloni
authors.forename: G
authors.initials: G
authors.lastname: Perri
mesh_terms: D015519:Bone Density / Q000187:drug effects*; D001842:Bone and Bones / Q000187:drug effects* / Q000378:metabolism; D002117:Calcitriol / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D004359:Drug Therapy, Combination; D005260:Female; D006128:Growth / Q000187:drug effects*; D013006:Growth Hormone / Q000009:adverse effects / Q000627:therapeutic use*; D006801:Humans; D007015:Hypophosphatemia, Familial / Q000188:drug therapy* / Q000378:metabolism; D016014:Linear Models; D008297:Male; D008903:Minerals / Q000378:metabolism*; D010710:Phosphates / Q000627:therapeutic use; D011446:Prospective Studies; D011994:Recombinant Proteins / Q000009:adverse effects / Q000627:therapeutic use; D018709:Statistics, Nonparametric; D013997:Time Factors
chemical_list: D008903:Minerals; D010710:Phosphates; D011994:Recombinant Proteins; D013006:Growth Hormone; D002117:Calcitriol