Damar Çağrı, Boyunağa Öznur, Derinkuyu Betül Emine, Battaloğlu Nergiz, Ezgü Fatih Süheyl
Faculty of Medicine, Department of Radiology, Gazi University, Beşevler, 06500, Ankara, Turkey,
Skeletal Radiol. 2014 Nov;43(11):1651-4. doi: 10.1007/s00256-014-1957-8. Epub 2014 Jul 20.
We report two sisters who have a rare skeletal abnormality termed Patterson-Lowry rhizomelic dysplasia. The typical findings of these cases on bone survey are isolated shortening and proximal metaphyseal enlargement and cupping of the bilateral humeri. The elder sister also has coxa vara deformity and dysplastic proximal femoral epiphyses on both sides. The younger sister has normal hip joint bones bilaterally, but her proximal femoral epiphyses are smaller than normal. All other bones of the sisters are of normal size and configuration. Our patients are two siblings, and their parents are first degree relatives, suggesting autosomal-recessive (AR) inheritance. The present patients help us to understand the genetic relationships and skeletal variabilities of this rare entity.
我们报告了两名患有罕见骨骼异常疾病——帕特森-洛瑞短肢侏儒症的姐妹。这些病例在骨骼检查中的典型表现为双侧肱骨孤立性缩短、近端干骺端增大及杯口状改变。姐姐还患有双侧髋内翻畸形及发育不良的近端股骨骨骺。妹妹双侧髋关节骨骼正常,但其近端股骨骨骺小于正常。姐妹俩的所有其他骨骼大小和形态均正常。我们的患者为两名同胞姐妹,其父母为一级亲属,提示为常染色体隐性(AR)遗传。目前的这两名患者有助于我们了解这种罕见疾病的遗传关系和骨骼变异性。