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Precise Pulmonary Function Evaluation and Management of a Patient With Freeman-Sheldon Syndrome Associated With Recurrent Pneumonia and Chronic Respiratory Insufficiency

 Jihyun Park  ;  Seong-Woong Kang  ;  Won Ah Choi  ;  Yewon Lee  ;  Han Eol Cho 
 Annals of Rehabilitation Medicine, Vol.44(2) : 165-170, 2020-04 
Journal Title
Annals of Rehabilitation Medicine
Issue Date
Freeman-Sheldon syndrome ; Respiratory function tests ; Respiratory insufficiency
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.
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1. College of Medicine (의과대학) > Dept. of Rehabilitation Medicine (재활의학교실) > 1. Journal Papers
Yonsei Authors
Kang, Seong Woong(강성웅) ORCID logo https://orcid.org/0000-0002-7279-3893
Park, Jihyun(박지현) ORCID logo https://orcid.org/0000-0002-2172-1072
Cho, Han Eol(조한얼) ORCID logo https://orcid.org/0000-0001-5625-3013
Choi, Won Ah(최원아) ORCID logo https://orcid.org/0000-0003-0403-8869
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