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Perioperative care of the patient with nephropathic cystinosis.

Authors
  • Ray, Theresa L
  • Tobias, Joseph D
Type
Published Article
Journal
Paediatric anaesthesia
Publication Date
Oct 01, 2004
Volume
14
Issue
10
Pages
878–885
Identifiers
PMID: 15385020
Source
Medline
License
Unknown

Abstract

Cystinosis is an autosomal recessive disease that results in a defective integral membrane protein responsible for the transport of cystine out of lysosomes. This results in the accumulation of cystine in the lysosomes of almost every tissue, with subsequent formation of damaging crystals. The cystinotic process may affect every system of the body, but is particularly damaging to the kidneys and eyes. We discuss the perioperative care of a cystinotic patient with renal insufficiency, Fanconi's syndrome, and photophobia. Other organ systems affected by the cystinotic process are also discussed, with suggestions for the perioperative management of each. Issues of primary concern during perioperative care include preservation of renal function and maintenance of fluid and electrolyte homeostasis.

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