Anesthetic management in Joubert syndrome: case report.
Joubert syndrome is a rare autosomal recessive disorder of cerebellum that occurs in 1 of 100.000 live births. The syndrome is characterized by a distinctive cerebellar and brainstem malformation, hypotonic, developmental delays, either hyperpnea or apnea or atypical eye movements or both. Cognitive...
| Publicado en: | Turkiye Klinikleri Journal of Medical Sciences Vol. 30; no. 6; pp. 2045 - 2048 |
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| Autores principales: | , , , |
| Formato: | case study Journal Article |
| Publicado: |
Turkiye Klinikleri
Dec2010
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| Acceso en línea: | Ver este registro en EBSCOhost |
| fields | @attributes: recordID: 1 pdfLink: plink: https://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=104812883&site=ehost-live header: @attributes: shortDbName: ccm uiTerm: 104812883 longDbName: CINAHL Complete uiTag: AN controlInfo: bkinfo: dissinfo: jinfo: jid: 13000292 1VOP jtl: Turkiye Klinikleri Journal of Medical Sciences issn: 13000292 maglogo: N pubinfo: dt: Dec2010 vid: 30 iid: 6 pid: 67298 pub: Turkiye Klinikleri artinfo: ui: 104812883 104812883 2010938032 10.5336/medsci.2008-10159 104812883 ppf: 2045 ppct: 3 formats: fmt: @attributes: type: P tig: atl: Anesthetic management in Joubert syndrome: case report. aug: au: Güclü, Çigdem Yildirim Uluer, Pinar Durak Kökten, Gizem Erdemli, Özcan sug: subj: Anesthesia Cerebellar Diseases Child Male Child: 6-12 years Male ab: Joubert syndrome is a rare autosomal recessive disorder of cerebellum that occurs in 1 of 100.000 live births. The syndrome is characterized by a distinctive cerebellar and brainstem malformation, hypotonic, developmental delays, either hyperpnea or apnea or atypical eye movements or both. Cognitive abilities are variable, ranging from severe to mild mental retardation. A magnetic resonance imaging (MRI) is the most common neuroimaging technique used to establish the diagnosis of Joubert syndrome. 'Molar teeth' sign on MRI is spesific for Joubert Syndrome. There are limited reports on the anesthetic manegement of these patients. These patients may be sensitive to respiratory depression caused by anesthetics. Because of these reasons, anesthetic management of these patients need more attention. These patients are especially susceptible to N2O, opioids and neuromuscular blockers. Ventilation parameters must be monitored closely for at least 24 hours postoperatively. pubtype: Academic Journal doctype: case study Journal Article ougenre: Article language: English refInfo: holdings: @attributes: islocal: N |
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