X-linked hypophosphatemia: Effects of treatment with recombinant human growth hormone

G. Reusz, Gabriel Miltényi, Gusztáv Stubnya, András Szabó, C. Horváth, Dennis J. Byrd, Ferenc Péter, T. Tulassay

Research output: Contribution to journalArticle

26 Citations (Scopus)

Abstract

The impact of recombinant human growth hormone (rhGH) treatment on growth, bone mineral metabolism, and bone mineral density (BMD) was evaluated in six children (3 girls, 3 boys) with familial hypophosphatemic rickets (XLH). Five were prepubertal (aged 6-8.8 years), one 15.3-year-old boy had combined XLH and GH deficiency, but had not been treated with rhGH previously. rhGH was administered daily for 1 year, at a dose of 1 IU/kg per week, combined with 1,25-dihydroxyvitamin D3 and oral phosphate therapy. Z scores for growth velocity and height improved significantly (-2.9 vs. 2.5, P <0.01, and -2.2 vs. -1.5, P <0.01, respectively). However, the ratio of Z score for height to that of subischial leg length decreased significantly (0.65 vs. 0.43, P <0.01), indicating disproportionate growth in favor of the trunk. The height-corrected BMD Z increased slightly (-0.99 vs. -0.94, P <0.05). A slight increase in serum phosphate occurred (0.78 vs. 0.88 mmol/l, P <0.02). Tubular reabsorption of phosphate/glomerular filtration rate increased from 0.45 mmol/l to 0.55 mmol at 6 months (P <0.02), but returned to the initial level at 12 months. These results indicate that children with XLH can benefit from the positive effect of rhGH on growth, however treatment could aggravate the already existing tendency to disproportionate growth. GH production should be evaluated in poorly growing patients with XLH, because it can mask GH deficiency. rhGH can be safely combined with conventional treatment in XLH. Further studies are needed to determine the effect of treatment on final height and maximal BMD.

Original languageEnglish
Pages (from-to)573-577
Number of pages5
JournalPediatric Nephrology
Volume11
Issue number5
DOIs
Publication statusPublished - Oct 1997

Fingerprint

Familial Hypophosphatemic Rickets
Human Growth Hormone
Growth Hormone
Bone Density
Phosphates
Growth
Therapeutics
Calcitriol
Bone Development
Masks
Glomerular Filtration Rate
Minerals
Leg
Serum

Keywords

  • Bone mineral density
  • Growth hormone
  • Hypophosphatemic rickets
  • X-linked hypophosphatemia

ASJC Scopus subject areas

  • Nephrology
  • Pediatrics, Perinatology, and Child Health

Cite this

X-linked hypophosphatemia : Effects of treatment with recombinant human growth hormone. / Reusz, G.; Miltényi, Gabriel; Stubnya, Gusztáv; Szabó, András; Horváth, C.; Byrd, Dennis J.; Péter, Ferenc; Tulassay, T.

In: Pediatric Nephrology, Vol. 11, No. 5, 10.1997, p. 573-577.

Research output: Contribution to journalArticle

Reusz, G. ; Miltényi, Gabriel ; Stubnya, Gusztáv ; Szabó, András ; Horváth, C. ; Byrd, Dennis J. ; Péter, Ferenc ; Tulassay, T. / X-linked hypophosphatemia : Effects of treatment with recombinant human growth hormone. In: Pediatric Nephrology. 1997 ; Vol. 11, No. 5. pp. 573-577.
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