Disproportionate growth following long-term growth hormone treatment in short children with X-linked hypophosphataemia
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Three short prepubertal children with X-linked hypophosphataemia were treated with 1 IU recombinant human growth hormone (rhGH)/kg per weck sc in addition to calcitriol and phosphate supplementation over a period of 3 years. Improvement of height standard deviation score (SDS) ranged from 1.0–1.7 SD based on an increase in sitting height of 1.5–2.9 SD, whereas subischial leg length improved only slightly by 0.3–0.9 SD. In all three patients, renal phosphate threshold concentration increased slightly and transient hyperparathyroidism was noted.
Treatment of stunted children with X-linked hypophosphataemia is effective in improving growth velocity, but appears to aggravate the pre-existent disporportionate stature of such children.
Key wordsX-linked hypophosphataemia Short stature Growth hormone Disproportionate growth Secondary hyperparathyroidism
glomerular filtration rate
recombinant human growth hormone
renal threshold phosphate concentration
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