Halal F, Preus M
Am J Med Genet. 1979;3(4):317-23. doi: 10.1002/ajmg.1320030402.
The hand pattern profiles of 18 previously published and 10 new cases of de Lange syndrome were compared to those of cases referred as suspects, but judged clinically and by numerical taxonomic methods not to have de Lange syndrome (non-de Lange). Based on a Poznanski metacarpophalangeal profile of the 2 groups of patients, a simple scoring system using 11 measurements was devised using the 16 most marked differences within and between metacarpals and phalanges. Of the metacarpals, the first is shorter than the second, third, fourth, or fifth; and the second and fifth are shorter than the third or fourth. Of the middle phalanges, the third and fourth are shorter than the respective metacarpal and than the second and fifth middle phalanx. Of the distal phalanges, the fifth is shorter than the second. The mean Z score for these 11 measurements is further below normal in the de Lange group than in the non-de Lange group. All 11 non-de Lange patients had a score less than 17, and all 28 de Lange patients had a score of 18 or more.
将18例先前已发表的德朗热综合征病例和10例新病例的手部形态特征，与那些被视为疑似病例但经临床判断和数值分类学方法判定无德朗热综合征（非德朗热综合征）的病例进行了比较。基于两组患者的波兹南斯基掌指骨形态，利用掌骨和指骨内部及之间的16个最显著差异，设计了一个使用11项测量指标的简单评分系统。在掌骨中，第一掌骨短于第二、第三、第四或第五掌骨；第二和第五掌骨短于第三或第四掌骨。在中指骨中，第三和第四中指骨短于各自对应的掌骨，也短于第二和第五中指骨。在远节指骨中，第五远节指骨短于第二远节指骨。德朗热综合征组这11项测量指标的平均Z评分比非德朗热综合征组更低于正常水平。所有11例非德朗热综合征患者的评分均低于17分，而所有28例德朗热综合征患者的评分均为18分或更高。
