Kohn G, Elrayyes E R, Makadmah I, Rösler A, Grünebaum M
J Med Genet. 1987 Jun;24(6):366-9. doi: 10.1136/jmg.24.6.366.
A new variant of spondyloepiphyseal dysplasia tarda with mild to moderate mental retardation is described in three daughters born to healthy, consanguineous parents. The mode of inheritance is compatible with that of an autosomal recessive disorder. The identification of this variant is important, as it enables more precise counselling in families in which sporadic cases with this form of presentation are found.
本文描述了一种迟发性脊椎骨骺发育不良的新变种,三名患有轻度至中度智力障碍的女儿出生于健康的近亲父母。其遗传模式与常染色体隐性疾病相符。识别这种变种很重要,因为它能为发现这种散发病例的家庭提供更精确的遗传咨询。