Egyptian Journal of Anaesthesia (Oct 2017)
Anaesthetic management of a child with cystinosis
Abstract
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