Huq A H, Braverman R M, Greenberg F, Bacino C A, Rimoin D L, Lachman R S, Levin M L
Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, Texas, USA.
Am J Med Genet. 1997 Jan 20;68(2):225-30. doi: 10.1002/(sici)1096-8628(19970120)68:2<225::aid-ajmg21>3.0.co;2-v.
We describe a brother and sister with a unique combination of skeletal findings including camptodactyly (phalangeal dislocations), facial anomalies, neonatal respiratory problems, and feeding problems due to poor suck. Metaphyseal splaying, osteopenia, endosteal bone apposition, campomelia, and multiple fractures characterize the other skeletal abnormalities. The parents are first cousins once removed and are unaffected. These cases appear to represent a previously undescribed autosomal recessive disorder.
我们描述了一对患有独特骨骼异常组合的兄妹,包括屈曲指(指骨脱位)、面部畸形、新生儿呼吸问题以及因吸吮无力导致的喂养问题。干骺端增宽、骨质减少、骨内膜骨附着、弓形腿和多处骨折是其他骨骼异常的特征。父母是隔了一代的表亲,未受影响。这些病例似乎代表了一种此前未被描述的常染色体隐性疾病。