Riccardi V M
Birth Defects Orig Artic Ser. 1977;13(3C):171-84.
The Warkany syndrome is characterized by mental retardation, relatively specific facies, absent or dysplastic patellas, joint contractures, plantar/palmar furrows, distinctively abnormal toe posture, vertebral anomalies, narrow pelvis, ureteral-renal anomalies, or other abnormalities. There is no association with advanced parental age, and birthweight and length are usually commensurate with gestational age. The chromosomal mechanisms accounting for the WS include either literal trisomy (8 (aneuploidy), usually if not always with mosaicism, or translocation leading to partial trisomy 8 (8q2). In addition, some patients with mosaic trisomy 8 may not have the Warkany syndrome.
瓦尔卡尼综合征的特征为智力发育迟缓、相对特殊的面容、髌骨缺如或发育异常、关节挛缩、足底/手掌纹路、明显异常的足趾姿势、脊柱异常、骨盆狭窄、输尿管-肾脏异常或其他异常。该综合征与父母高龄无关,出生体重和身长通常与孕周相符。导致瓦尔卡尼综合征的染色体机制包括单纯三体性(8号染色体三体(非整倍体),通常伴有或不伴有嵌合体),或易位导致部分8号染色体三体(8q2)。此外,一些8号染色体嵌合三体患者可能不患有瓦尔卡尼综合征。