Milachowski K, Keyl W, Witt T N
Z Orthop Ihre Grenzgeb. 1982 Sep-Oct;120(5):657-61. doi: 10.1055/s-2008-1051372.
This report deals with two brothers suffering from chondrodystrophic myotomy (Schwartz-Jampel syndrome), and presents, through the study of these cases, the orthopedic and neurological problems presented by this condition. In the case of our patients there is no confirmation of the stunted growth mentioned in other reports, nor are there any changes in the vertebral column. In contrast to cases reported to date, pathologically high sero-enzyme amounts were found in both children. The congenital changes in the hip joints worsen during the pre-puberty phase, so that in one case bilateral adduction contraction and in the other complete bilateral dislocation of the hip joints resulted. Early orthopedic treatment to prevent later irreparable consequences is recommended.
本报告涉及两名患有软骨营养障碍性肌强直(施瓦茨 - 扬佩尔综合征)的兄弟,并通过对这些病例的研究,阐述了该病症所呈现的骨科和神经学问题。在我们的患者病例中,并未证实其他报告中提及的生长发育迟缓情况,脊柱也未出现任何变化。与迄今报道的病例不同,两名患儿的血清酶含量在病理上均偏高。髋关节的先天性病变在青春期前阶段会恶化,以至于其中一例出现双侧内收挛缩,另一例则导致双侧髋关节完全脱位。建议尽早进行骨科治疗以防止后期出现无法挽回的后果。