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Bone Age Delay in X-linked Hypophosphatemia.

Julio Soto1,2,3, Rucha Anant Patki1, Lauren J Ehrlich4

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Journal of the Endocrine Society
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Summary
This summary is machine-generated.

Bone age (BA) is delayed in children with X-linked hypophosphatemia (XLH), particularly in males. Height predictions using standard methods showed variations, with specific methods overestimating adult height in males and females.

Keywords:
X-linked hypophosphatemiabone agepredicted adult heightskeletal maturation

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Area of Science:

  • Pediatric Endocrinology
  • Skeletal Dysplasias
  • Growth and Development

Background:

  • X-linked hypophosphatemia (XLH) is a rare genetic disorder affecting phosphate metabolism and bone mineralization.
  • Bone age (BA) assessment and prediction of adult height (AdHt) are crucial for managing growth in children with chronic conditions.
  • Limited data exists on BA and AdHt prediction accuracy in pediatric XLH patients.

Purpose of the Study:

  • To evaluate bone age (BA) assessment in children with X-linked hypophosphatemia (XLH).
  • To determine the utility of BA in predicting adult height (AdHt) in this cohort.
  • To compare the accuracy of different BA assessment methods for height prediction in XLH.

Main Methods:

  • Retrospective, cross-sectional, and longitudinal analysis of BA using two standard methods.
  • Calculation of predicted adult height (PAH) from BA assessments.
  • Comparison of PAH with final or near-final AdHt in a cohort of 56 children with XLH.

Main Results:

  • Children with XLH exhibited delayed BA, with males showing a significantly greater delay than females (P < .05).
  • Fifty-eight percent of males and 21% of females had a BA delay of 1-2 years.
  • The Bayley-Pinneau method tended to overestimate AdHt in males, while the Tanner-Whitehouse method overestimated AdHt in females.

Conclusions:

  • Bone age is delayed in children with XLH, with a more pronounced effect observed in males.
  • Standard methods for predicting adult height in XLH patients yielded results within the typical range for healthy children.
  • The choice of prediction method (Bayley-Pinneau vs. Tanner-Whitehouse) impacts accuracy, with specific overestimations noted for each in XLH.