摘要譯文
漏斗胸可能的原因是結締組織的問題,17位病患中,65%有二尖瓣脫垂,53%在電子顯微鏡檢查發現病人皮膚之膠原纖維及彈性蛋白不正常。所以漏斗胸可視為一種身體結締組織異常的表現。
朱醫師註:漏斗胸常合併心臟瓣膜逆流及脫垂,或許除了胸壁直接的壓迫心臟以外,心臟瓣膜本身的膠原及彈性蛋白成份的異常,也應是原因之一。在臨床病患的觀察中,我發現許多病患除了心臟瓣膜的問題外,也常合併有許多結締組織的疾病,如神經纖維瘤,軟骨組織疾病等等。

[Mitral valve prolapse and pectus excavatum. Expressions of connective tissue dystrophy?]

[Article in French]

Saint-Mézard G, Chanudet X, Duret JC, Larrue J, Bonnet J, Bricaud H.

Pectus excavatum is a common malformation in diseases of elastic tissue (Marfan, Ehlers-Danlos...). When observed apparently alone it may represent a minor form of dystrophy, implying the same risk of a cardiac lesion. Abnormalities of the thoracic skeleton and echocardiographic mitral valve prolapse is a well established association, suggesting a common disorder of connective tissue. However, there is no absolute proof that this is a statistically significant association. Histological connective tissue changes relating these two markers have yet to be found. Clinical and echocardiographic examinations and skin biopsies were performed in 17 patients with pectus excavatum. Mitral valve prolapse was detected in 65% of cases (associated in 1 out of 3 cases with tricuspid valve prolapse). In 53% of cases electron microscopy showed abnormal skin collagen and elastin. Collagen abnormalities were twice as common as those of elastin and could be associated. Mixed changes of thinning of elastin and collagen fibres of irregular calibre were particularly suggestive. Pectus excavatum would therefore seem to be the expression of a minor form of dystrophy of collagen and elastin tissues and a clinical marker of possible mitral valve prolapse.
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