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A Journal on Anesthesiology, Resuscitation, Analgesia and Intensive Care

Official Journal of the Italian Society of Anesthesiology, Analgesia, Resuscitation and Intensive Care
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Minerva Anestesiologica 2002 October;68(10):783-90

language: English, Italian

Manopharmacologic general anaesthesia with sevoflurane in paediatric patient with Prader-Willi syndrome

Rinaldi S., Rizzo L., Di Filippo A., Secchi S., Paterno-ster G., La Torre M. S., Pascente C., Stanzani M. R.

Sec­tion of Anes­the­sia and Resus­ci­ta­tion Depart­ment of Med­i­cal and Sur­gi­cal Inten­sive ­Care Uni­ver­sity of Flor­ence, Flor­ence


Prad­er-Wil­li syn­drome (PWS) is a genet­ic dis­ease ­caused by a ­loss of pater­nal ­genes locat­ed in chro­mo­some 15. Chil­dren affect­ed by ­this syn­drome ­often ­have pre­term deliv­ery; dur­ing child­hood the hall­marks are: ­severe infan­tile hypo­to­nia and feed­ing prob­lems. After­ward, neu­ro­log­ic man­i­fes­ta­tions, endo­crine ­signs and dys­met­a­bol­ic abnor­mal­ities are usu­al­ly ­seen togeth­er ­with cra­ni­o­fa­cial man­i­fes­ta­tions and mus­cu­los­kel­e­tal abnor­mal­ities. Obes­ity caus­es ­sleep abnor­mal­ities includ­ing ­sleep ­apnea. The ­case we ­present is of a 5 ­year old ­child (CA) sched­uled for stra­bis­mus sur­gery. The ­child has a lot of typ­i­cal (PWS) ­signs. A num­ber of anaesthes­io­log­ic prob­lems are asso­ciat­ed ­with (PWS). ­Some of ­them ­relate to obes­ity, oth­ers to ­facial dys­mor­phism. More­over, the syn­drome may ­give a pro­longed and exag­ger­at­ed ­response to eve­ry sed­a­tive ­drug. P.W.S. is ­also char­ac­ter­ized by ther­mo­reg­u­la­to­ry dis­or­ders. ­Sleep ­apnea ­occurs ­often. Con­sid­er­ing all ­these pro- b­lems, we ­planned a mono­phar­mac­o­log­ic anaesthes­io­log­ic pro­ce­dure ­using sevof­lu­rane.

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