Hurler syndrome with special reference to histologic abnormalities of the growth plate.

@article{Silveri1991HurlerSW,
  title={Hurler syndrome with special reference to histologic abnormalities of the growth plate.},
  author={Christopher P. Silveri and Frederick S. Kaplan and Michael D. Fallon and E Bayever and Charles S. August},
  journal={Clinical orthopaedics and related research},
  year={1991},
  volume={269},
  pages={305-11}
}
Hurler syndrome is a mucopolysaccharide disorder resulting from an heritable deficiency in alpha-L-iduronidase, an enzyme required in the catabolism of heparan sulfate and dermatan sulfate glycosaminoglycan (GAGs). The resultant intracellular accumulation of GAG leads to disruption of the intracellular and extracellular environment and dysfunction of multiple organ systems. Among the most noted manifestations of this disease is disproportionate short trunk dwarfism, which develops during the… CONTINUE READING

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