Surgical Treatment for Scheie's Syndrome (Mucopolysaccharidosis Type I-S)

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  • Minakata Kenji
    Department of Cardiovascular Surgery, Japanese Red Cross Society, Wakayama Medical Center
  • Konishi Yutaka
    Department of Cardiovascular Surgery, Japanese Red Cross Society, Wakayama Medical Center
  • Matsumoto Masahiko
    Department of Cardiovascular Surgery, Japanese Red Cross Society, Wakayama Medical Center
  • Miwa Senri
    Department of Cardiovascular Surgery, Japanese Red Cross Society, Wakayama Medical Center

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タイトル別名
  • Report of Two Cases

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説明

Scheie's syndrome (mucopolysaccharidosis type I-S) is a rare genetic lysosomal storage disease affecting mucopolysaccharide metabolism, and is known to include cardiovascular disease. Surgical treatment was carried out in 2 patients with Scheie's syndrome. Patient 1 was a 56-year-old man with triple-vessel coronary artery disease, who successfully underwent coronary artery bypass grafting. Patient 2 was a 52-year-old man with aortic and mitral valve stenosis, who successfully underwent combined aortic and mitral valve replacement. The literature on Scheie's syndrome associated with valvular and coronary artery disease is also reviewed. (Jpn Circ J 1998; 62: 700 - 703)

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