Gurd A R, Thompson T R
J Pediatr Orthop. 1981;1(3):317-20. doi: 10.1097/01241398-198111000-00013.
Children with Prader-Willi syndrome frequently have musculoskeletal problems such as joint hyperlaxity, hypotonia, delayed bone age, and scoliosis. Their musculoskeletal problems are magnified by the extreme obesity most of these patients exhibit. In certain cases, such as scoliosis, the Prader-Willi patient is placed at significant risk for increased morbidity and mortality. Our paper emphasizes the accurate diagnosis of this syndrome, proper dietary management, and some guidelines regarding surgical evaluation and management of a patient with significant scoliosis.
普拉德-威利综合征患儿常伴有肌肉骨骼问题,如关节过度松弛、肌张力减退、骨龄延迟和脊柱侧弯。这些患者大多表现出的极度肥胖会加剧他们的肌肉骨骼问题。在某些情况下,如脊柱侧弯,普拉德-威利综合征患者出现发病率和死亡率增加的风险显著。我们的论文强调了该综合征的准确诊断、适当的饮食管理,以及关于严重脊柱侧弯患者手术评估和管理的一些指导原则。