Chapter 70. Cystinosis

  1. Donald A. Molony MD2,3 and
  2. Jonathan C. Craig MD, PhD4,5
  1. William G. van't Hoff BSc, MD, FRCPCH, FRCP

Published Online: 4 MAR 2009

DOI: 10.1002/9781444303391.ch70

Evidence-Based Nephrology

Evidence-Based Nephrology

How to Cite

van't Hoff, W. G. (2008) Cystinosis, in Evidence-Based Nephrology (eds D. A. Molony and J. C. Craig), Wiley-Blackwell, Oxford, UK. doi: 10.1002/9781444303391.ch70

Editor Information

  1. 2

    Division of Renal Disease and Hypertension, University of Texas, USA

  2. 3

    Houston Medical School, Houston, Texas, USA

  3. 4

    School of Public Health, University of Sydney, Australia

  4. 5

    Department of Nephrology, The Children's Hospital at Westmead, Sydney, Australia

Author Information

  1. Nephro-Urology Unit, Great Ormond Street Hospital for Children, London, United Kingdom

Publication History

  1. Published Online: 4 MAR 2009
  2. Published Print: 5 DEC 2008

ISBN Information

Print ISBN: 9781405139755

Online ISBN: 9781444303391



  • cystinosis - disorder characterized biochemically by excess accumulation of cystine;
  • treatment of cystinosis - general and specific measures;
  • recommendations for optimal management;
  • kidney transplantation - successful and cystinosis does not recur;
  • evidence-based therapy for conditions;
  • effects of Fanconi syndrome;
  • renal Fanconi syndrome;
  • evidence ratings and recommendations for cystinosis studies


This chapter contains sections titled:

  • Introduction and clinical course

  • Treatment

  • Recommendations for optimal management

  • References