Meinecke P
Abt. Medizinische Genetik, Altonaer Kinderkrankenhaus, Hamburg, Germany.
Genet Couns. 1993;4(2):147-51.
Report on a second family. The patients, an 18-year-old boy and his 15-year-old sister, have pre and postnatal short stature, microcephaly, moderate to severe mental retardation, and cutaneous syndactylies of hands and feet. In addition, they show a mildly dysmorphic but apparently characteristic face. Radiologically, hands and feet demonstrate brachydactyly, metacarpals and metatarsals being the most severely affected. This observation confirms that this multiple congenital anomalies/mental retardation pattern is a distinct, probably autosomal recessively inherited entity.
另一个家庭的报告。患者为一名18岁男孩及其15岁的妹妹,有产前和产后身材矮小、小头畸形、中度至重度智力发育迟缓以及手足皮肤并指(趾)畸形。此外,他们有轻度畸形但明显具有特征性的面容。影像学检查显示,手足存在短指(趾)畸形,掌骨和跖骨受影响最为严重。这一观察结果证实,这种多发性先天性畸形/智力发育迟缓模式是一种独特的、可能为常染色体隐性遗传的疾病实体。