Anesthetic Approach for a Patient with Jeune Syndrome
Jeune syndrome (JS) is an autosomal recessive disease also known as asphyxiating thoracic dystrophy. A narrow bell-shaped thoracic wall and short extremities are the most typical features of the syndrome. Prognosis in JS depends on the severity of the pulmonary hypoplasia caused by the chest wall de...
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| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
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Wiley
2015-01-01
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| Series: | Case Reports in Anesthesiology |
| Online Access: | http://dx.doi.org/10.1155/2015/509196 |
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| _version_ | 1832565878826205184 |
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| author | Mehmet I. Buget Emine Ozkan Ipek S. Edipoglu Suleyman Kucukay |
| author_facet | Mehmet I. Buget Emine Ozkan Ipek S. Edipoglu Suleyman Kucukay |
| author_sort | Mehmet I. Buget |
| collection | DOAJ |
| description | Jeune syndrome (JS) is an autosomal recessive disease also known as asphyxiating thoracic dystrophy. A narrow bell-shaped thoracic wall and short extremities are the most typical features of the syndrome. Prognosis in JS depends on the severity of the pulmonary hypoplasia caused by the chest wall deformity. Most patient deaths are due to respiratory problems at early ages. Herein, we report a case of JS patient, who was scheduled for femoral extension under general anesthesia. The severity of respiratory problems in JS patients is thought to diminish with age. Our case supported this theory, and we managed the anesthetic process uneventfully. |
| format | Article |
| id | doaj-art-b280b78de2684997933a6ae4aa568332 |
| institution | Kabale University |
| issn | 2090-6382 2090-6390 |
| language | English |
| publishDate | 2015-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Anesthesiology |
| spelling | doaj-art-b280b78de2684997933a6ae4aa5683322025-02-03T01:06:34ZengWileyCase Reports in Anesthesiology2090-63822090-63902015-01-01201510.1155/2015/509196509196Anesthetic Approach for a Patient with Jeune SyndromeMehmet I. Buget0Emine Ozkan1Ipek S. Edipoglu2Suleyman Kucukay3Department of Anesthesiology, Istanbul Medical Faculty, Istanbul University, Fatih, 34093 Istanbul, TurkeyDepartment of Anesthesiology, Istanbul Medical Faculty, Istanbul University, Fatih, 34093 Istanbul, TurkeyDepartment of Anesthesiology, Suleymaniye Obstetrics & Pediatrics Training and Research Hospital, Zeytinburnu, 34116 Istanbul, TurkeyDepartment of Anesthesiology, Istanbul Medical Faculty, Istanbul University, Fatih, 34093 Istanbul, TurkeyJeune syndrome (JS) is an autosomal recessive disease also known as asphyxiating thoracic dystrophy. A narrow bell-shaped thoracic wall and short extremities are the most typical features of the syndrome. Prognosis in JS depends on the severity of the pulmonary hypoplasia caused by the chest wall deformity. Most patient deaths are due to respiratory problems at early ages. Herein, we report a case of JS patient, who was scheduled for femoral extension under general anesthesia. The severity of respiratory problems in JS patients is thought to diminish with age. Our case supported this theory, and we managed the anesthetic process uneventfully.http://dx.doi.org/10.1155/2015/509196 |
| spellingShingle | Mehmet I. Buget Emine Ozkan Ipek S. Edipoglu Suleyman Kucukay Anesthetic Approach for a Patient with Jeune Syndrome Case Reports in Anesthesiology |
| title | Anesthetic Approach for a Patient with Jeune Syndrome |
| title_full | Anesthetic Approach for a Patient with Jeune Syndrome |
| title_fullStr | Anesthetic Approach for a Patient with Jeune Syndrome |
| title_full_unstemmed | Anesthetic Approach for a Patient with Jeune Syndrome |
| title_short | Anesthetic Approach for a Patient with Jeune Syndrome |
| title_sort | anesthetic approach for a patient with jeune syndrome |
| url | http://dx.doi.org/10.1155/2015/509196 |
| work_keys_str_mv | AT mehmetibuget anestheticapproachforapatientwithjeunesyndrome AT emineozkan anestheticapproachforapatientwithjeunesyndrome AT ipeksedipoglu anestheticapproachforapatientwithjeunesyndrome AT suleymankucukay anestheticapproachforapatientwithjeunesyndrome |