Park Jihyun, Kang Seong-Woong, Choi Won Ah, Lee Yewon, Cho Han Eol
Department of Rehabilitation Medicine and Rehabilitation Institute of Neuromuscular Disease, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul, Korea.
Pulmonary Rehabilitation Center, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul, Korea.
Ann Rehabil Med. 2020 Apr;44(2):165-170. doi: 10.5535/arm.2020.44.2.165. Epub 2020 Apr 29.
Freeman-Sheldon syndrome (FSS) is a rare distal arthrogryposis syndrome. There are few reports on the respiratory insufficiency of FSS. Additionally, there is no detailed information on pulmonary functional evaluation. A 17-year-old male patient with FSS developed respiratory failure, leading him to be admitted to hospital several times for evaluation and treatment. Of those times he was admitted, two were due to pneumonia. His pulmonary functions were indicative of a restrictive lung disease potentially caused by severe scoliosis. After a non-invasive ventilatorwas applied correctly to the patient, pulmonary hypertension was normalized. His pulmonary function has been maintained for 13 years. Since receiving proper respiratory care, which includes assisted coughing methods, the patient has not developed pneumonia. It is important to properly evaluate the pulmonary function of patients who have FSS and scoliosis to eliminate the risk of long-term respiratory complications.
弗里曼-谢尔顿综合征（FSS）是一种罕见的远端关节挛缩综合征。关于FSS呼吸功能不全的报道很少。此外，也没有关于肺功能评估的详细信息。一名17岁的FSS男性患者出现呼吸衰竭，导致他多次住院进行评估和治疗。其中两次住院是因为肺炎。他的肺功能表明存在一种可能由严重脊柱侧弯引起的限制性肺病。在给患者正确应用无创呼吸机后，肺动脉高压恢复正常。他的肺功能已维持了13年。自从接受了包括辅助咳嗽方法在内的适当呼吸护理后，该患者未再发生肺炎。对患有FSS和脊柱侧弯的患者进行适当的肺功能评估，以消除长期呼吸并发症的风险非常重要。
