Cysteamine therapy delays the progression of nephropathic cystinosis in late adolescents and adults.

@article{BrodinSartorius2012CysteamineTD,
  title={Cysteamine therapy delays the progression of nephropathic cystinosis in late adolescents and adults.},
  author={Albane Brodin-Sartorius and Marie-Jos{\`e}phe T{\^e}te and Patrick Niaudet and Corinne Antignac and Genevi{\`e}ve Guest and Chris Ottolenghi and Marina Charbit and Dominique Moyse and C. Legendre and Philippe Lesavre and Pierre Cochat and Aude Servais},
  journal={Kidney international},
  year={2012},
  volume={81 2},
  pages={179-89}
}
Nephropathic cystinosis is a multisystem autosomal recessive disease caused by cystine accumulation, which is usually treated by oral cysteamine. In order to determine long-term effects of this therapy, we enrolled 86 adult patients (mean age 26.7 years) diagnosed with nephropathic cystinosis, 75 of whom received cysteamine. Therapy was initiated at a mean age of 9.9 years with a mean duration of 17.4 years. By last follow-up, 78 patients had end-stage renal disease (mean age 11.1 years), 62… CONTINUE READING
43 Extracted Citations
47 Extracted References
Similar Papers

Citing Papers

Publications influenced by this paper.
Showing 1-10 of 43 extracted citations

Referenced Papers

Publications referenced by this paper.
Showing 1-10 of 47 references

Cystinosis: epidemiology in France

  • P Cochat, B Cordier, C Lacôte, MH. Saı̈d
  • In: Michel Broyer (ed). Cystinosis,
  • 1999
Highly Influential
6 Excerpts

692–698

  • Aupetit J Raehm M et al. Measurement of cystine in granulo Chabli A, 40
  • Kidney International
  • 2012
1 Excerpt

effect of CTNS mutations on the transport activity and subcellular localization of cystinosin

  • V Kalatzis, N Nevo, cystinosis Cherqui S et al. Molecular pathogenesis of
  • Hum Mol Genet 2004; 13: 1361–1371. 188 Kidney…
  • 2012
1 Excerpt

Similar Papers

Loading similar papers…