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Metacarpal measurements in X-linked hypophosphataemic rickets
Helvetica Paediatrica Acta
|October 1, 1986
Summary
Children with hypophosphataemic vitamin D-resistant rickets show wider metacarpal bones but thinner cortices. Bone mass appears normal or increased, with length varying by sex.
Area of Science:
- Pediatric Endocrinology
- Skeletal Biology
- Radiology
Background:
- Hypophosphataemic vitamin D-resistant rickets is a rare genetic disorder.
- It affects bone development and mineralization.
- Understanding its skeletal manifestations is crucial for management.
Purpose of the Study:
- To evaluate metacarpal bone dimensions in children with hypophosphataemic vitamin D-resistant rickets.
- To compare these measurements with age- and height-matched controls.
- To assess bone mass and length characteristics.
Main Methods:
- Radiographic analysis of the second metacarpal bone from the left hand.
- Measurements included midshaft diameter, combined cortical thickness, cortical area, and metacarpal length.
- Comparison with age- and height-matched healthy controls.
- Longitudinal assessment in a subset of patients.
Main Results:
- Patients exhibited increased metacarpal diameter, with onset in early childhood.
- Combined cortical thickness was decreased.
- Cortical area was normal for age and increased for height, suggesting normal or increased bone mass.
- Metacarpal length was normal for age/height in boys and increased for height (within normal range) in girls.
Conclusions:
- Children with this condition present with distinct metacarpal abnormalities.
- Findings suggest normal or elevated bone mass despite altered dimensions.
- Skeletal growth patterns differ between sexes in this condition.