Double-valve replacement for Scheie's syndrome subtype mucopolysaccaridosis type 1-S.

Murashita, Takashi; Kobayashi, Junjiro; Shimahara, Yusuke; Toda, Koichi; Fujita, Tomoyuki; Nakajima, Hiroyuki · Ann Thorac Surg · 2011

case_report · Level V

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Abstract

Scheie's syndrome is a rare hereditary disorder of proteoglycan-degrading enzymes. Deposition of mucopolysaccharide can cause valvular and other tissue abnormalities. Few studies have reported surgical results for Scheie's syndrome, and the perioperative course is complicated. This is a report of a 35-year-old woman with Scheie's syndrome who underwent double-valve replacement with intensive perioperative management.

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