Evidence of Reduced Bone Turnover and Disturbed Mineralization Process in a Boy with Stickler Syndrome

@article{Kaissi2009EvidenceOR,
  title={Evidence of Reduced Bone Turnover and Disturbed Mineralization Process in a Boy with Stickler Syndrome},
  author={Ali Al Kaissi and Paul Roschger and Kamilla Nawrot-Wawrzyniak and Andreas Krebs and Franz Grill and Klaus Klaushofer},
  journal={Calcified Tissue International},
  year={2009},
  volume={86},
  pages={126-131}
}
We describe a tall-statured 14-year-old boy who illustrated the full phenotypic and radiographic features of Stickler syndrome type I. A bone biopsy showed evidence of reduced bone mass and bone turnover, such as reduced BV/TV (–43%), TbTh (–29%), and OS/BS (–48%), Ob.S/BS (–27%), and Oc/BS (–47%) compared to “age-matched” controls. Moreover, there was evidence that the mineralization process was severely disturbed. Quantitative backscattered electron imaging revealed that the bone… CONTINUE READING
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