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Growth in X-linked hypophosphatemic rickets
Gema Ariceta1, Craig B Langman
1Division of Pediatric Kidney Diseases, Hospital Cruces, Vizcaya, Spain. gariceta@hcru.osakidetza.net
European Journal of Pediatrics
|December 16, 2006
Summary
Growth failure is common in X-linked hypophosphatemic rickets (XLHR). While standard treatment helps, recombinant human growth hormone (rhGH) showed potential for normalizing stature in some boys with XLHR.
Area of Science:
- Pediatrics
- Endocrinology
- Genetics
Background:
- X-linked hypophosphatemic rickets (XLHR) frequently causes growth failure in children.
- Standard treatment involves phosphate and calcitriol, but growth impairment often persists.
- Recombinant human growth hormone (rhGH) is a potential therapy for short children with XLHR.
Purpose of the Study:
- To evaluate growth outcomes in children with XLHR receiving standard treatment.
- To assess the efficacy and safety of recombinant human growth hormone (rhGH) in XLHR patients with impaired growth.
Main Methods:
- Retrospective study of 27 children with XLHR treated with phosphate and calcitriol.
- Analysis of Z-height changes over a median follow-up of 10.12 years.
- Evaluation of rhGH use in four male patients, with detailed follow-up in two.
Main Results:
- Linear growth failure was observed in a third of XLHR children despite standard treatment.
- No significant improvement in Z-height was noted after 5 years of follow-up.
- Two of four boys treated with rhGH achieved normalized stature without observed side effects.
Conclusions:
- Standard treatment for XLHR does not always resolve growth failure.
- Early diagnosis and improved treatment adherence are crucial for managing XLHR.
- rhGH may be a viable option for children with XLHR experiencing impaired growth, particularly after rickets control.
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