지원사업
학술연구/단체지원/교육 등 연구자 활동을 지속하도록 DBpia가 지원하고 있어요.
커뮤니티
연구자들이 자신의 연구와 전문성을 널리 알리고, 새로운 협력의 기회를 만들 수 있는 네트워킹 공간이에요.
논문 기본 정보
- 자료유형
- 학술저널
- 저자정보
- 발행연도
- 2020.1
- 수록면
- 165 - 170 (6page)
이용수
초록· 키워드
오류제보하기
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.
목차
등록된 정보가 없습니다.
참고문헌 (10)
참고문헌 신청
Toydemir RM / 2006 / Mutations in embryonic myosin heavy chain(MYH3)cause Freeman-Sheldon syndrome and Sheldon-Hall syndrome / Nat Genet 38:561~565
Attia A / 2008 / Freeman–Sheldon syndrome with respiratory failure : a case report / Respir Med CME 1:274~277
Ferrari D / 2008 / Freeman-Sheldon syndrome : a case report and review of the literature / Chir Organi Mov 92:127~131
Koumbourlis AC / 2006 / Scoliosis and the respiratory system / Paediatr Respir Rev 7:152~160
Stevenson DA / 2006 / Clinical characteristics and natural history of Freeman-Sheldon syndrome / Pediatrics 117:754~762
Attia A / 2008 / Freeman–Sheldon syndrome with respiratory failure : a case report / Respir Med CME 1:274~277
Ferrari D / 2008 / Freeman-Sheldon syndrome : a case report and review of the literature / Chir Organi Mov 92:127~131
Koumbourlis AC / 2006 / Scoliosis and the respiratory system / Paediatr Respir Rev 7:152~160
Stevenson DA / 2006 / Clinical characteristics and natural history of Freeman-Sheldon syndrome / Pediatrics 117:754~762
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