Egyptian Journal of Anaesthesia (Oct 2017)

Anaesthetic management of a child with cystinosis

  • Shilpi Verma,
  • Vandana Sharma,
  • Pradeep K. Bhatia,
  • Nikhil Kothari

Journal volume & issue
Vol. 33, no. 4
pp. 381 – 382

Abstract

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Cystinosis is a lysosomal storage disorder which is characterized by abnormal accumulation of amino acid cysteine. Cystinosis affects various tissues of the body and has several anesthetic implications. We discuss successful management of a 5 years old child with infantile nephropathic cystinosis and Fanconi‘s syndrome who underwent osteotomy for rickets. Keywords: Anesthesia, Lysosomal Storage Diseases, Fanconi Syndrome, Polyuria, Osteomalacia, Acidosis