Rudakov S S, Verovskiĭ V A, Prozorovskaia N N, Razuvaev M K
Ortop Travmatol Protez. 1990 Nov(11):10-7.
104 patients with progressive forms of funnel chest deformity of the II-III degree with Ehlers-Danlos-Marfan syndrome, Marfan-like phenotype, isolated funnel chest deformity and unclassified funnel chest deformity of the first degree were subjected to synromologic examination. There were detected 10 dysplastic signs, indicating funnel chest deformity progression: anti-Mongol shape of the eyes, arachnodactyly, high palate, Ehlers-Danlos syndrome, floor of the auricle's dysplasia, dolichostenomely, posture disturbance, mitral valve prolapse, umbilical hernia, wide filter. The results of biochemical examination of collagen metabolite--hydroxyproline++ (the first and the second hydroxyproline++ fraction ratio disturbance, decrease of the first hydroxyproline++ fraction percentage, disturbance of direct correlation between total hydroxyproline++ and percentage of the first hydroxyproline++ fraction)--corroborated the clinical data.
对104例患有II - III度进行性漏斗胸畸形的患者进行了综合征学检查,这些患者分别患有埃勒斯 - 当洛 - 马凡综合征、马凡样表型、孤立性漏斗胸畸形以及未分类的I度漏斗胸畸形。检测到10种发育异常体征,提示漏斗胸畸形进展:蒙古样眼型、蜘蛛指、高腭弓、埃勒斯 - 当洛综合征、耳廓底部发育异常、细长指、姿势障碍、二尖瓣脱垂、脐疝、宽滤器。胶原蛋白代谢产物——羟脯氨酸++的生化检查结果(第一和第二羟脯氨酸++组分比例紊乱、第一羟脯氨酸++组分百分比降低、总羟脯氨酸++与第一羟脯氨酸++组分百分比之间直接相关性紊乱)证实了临床数据。