Cystinosis: renal glomerular and renal tubular function in relation to compliance with cystine-depleting therapy

@article{Nesterova2014CystinosisRG,
  title={Cystinosis: renal glomerular and renal tubular function in relation to compliance with cystine-depleting therapy},
  author={Galina Nesterova and Caitlyn Williams and Isa M Bernardini and William A Gahl},
  journal={Pediatric Nephrology},
  year={2014},
  volume={30},
  pages={945-951}
}
Nephropathic cystinosis is a lysosomal storage disorder characterized by renal tubular Fanconi syndrome in infancy and glomerular damage leading to renal failure at ∼10 years of age. Therapy with the cystine-depleting agent cysteamine postpones renal failure, but the degree of compliance with this treatment has not been correlated with preservation of kidney function. We assessed leucocyte cystine depletion by cysteamine and created the composite compliance score that incorporates the extent of… CONTINUE READING
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