Anestesia generale nella sindrome di Prader-Willi.

Translated title of the contribution: General anesthesia in Prader-Willi syndrome

F. Cavaliere, S. Cormaci, M. Cormaci, A. Alberti, F. Colabucci

Research output: Contribution to journalArticle

Abstract

The case of a woman of 27 affected by the Prader-Willi syndrome who underwent general anaesthesia for dental surgery is reported. The patient presented severe mental retardation, small stature, moderate muscular hypotonia, hyperphagia, obesity, and diabetes mellitus. Premedication consisted of diazepam and atropine; anaesthesia was induced with propofol and maintained with propofol, fentanyl and N2O; muscle paralysis was obtained with atracurium. A small glottis was observed at laryngoscopy so that a 6 mm cuffed tube was inserted. Surgery lasted 75 minutes; the patient recovered promptly a few minutes following the end of propofol infusion; no postoperative complication was recorded. As hypoglycemia can occur during and after surgery in the Prader-Willi syndrome, plasma samples for glucose, NEFA, insulin, cortisol, and growth hormone (GH) were collected prior to the induction of anaesthesia (A), 20 minutes after starting surgery (B), at the end of surgery (C), and 3 hours later (D). In spite of the infusion of glucose, hyperglycemia was observed just in C and D samples (A:77; B:88; C:245; D:279 mg/dl). Stable NEFA values, within the normal range, were observed (A:77; B:88; C:245; D:279 mg/dl) suggesting poor or absent lipolysis. Insulin decreased progressively during surgery (A:10.5; B:8.8; C:5.4; D:7.0 mU/L). Cortisol peaked in B (A:9.5; B:20.9; C:13.4; D:4.8 micrograms/dl), suggesting normal hypothalamic reactivity to the surgical stimulus. Finally very low GH levels were observed (A:0.04; B:0.07; C:0.06; D:0.09 ng/ml) suggesting GH deficiency, which had possibly affected the size of patient's glottis. Our data support the hypothesis that hypoglycemia in the Prader-Willi syndrome originates from inadequate lipolysis during starvation.

Original languageItalian
Pages (from-to)327-332
Number of pages6
JournalMinerva Anestesiologica
Volume62
Issue number10
Publication statusPublished - Oct 1996

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Prader-Willi Syndrome
General Anesthesia
Propofol
Growth Hormone
Glottis
Lipolysis
Hypoglycemia
Nonesterified Fatty Acids
Hydrocortisone
Anesthesia
Insulin
Atracurium
Glucose
Hyperphagia
Laryngoscopy
Muscle Hypotonia
Premedication
Fentanyl
Diazepam
Starvation

ASJC Scopus subject areas

  • Anesthesiology and Pain Medicine

Cite this

Cavaliere, F., Cormaci, S., Cormaci, M., Alberti, A., & Colabucci, F. (1996). Anestesia generale nella sindrome di Prader-Willi. Minerva Anestesiologica, 62(10), 327-332.

Anestesia generale nella sindrome di Prader-Willi. / Cavaliere, F.; Cormaci, S.; Cormaci, M.; Alberti, A.; Colabucci, F.

In: Minerva Anestesiologica, Vol. 62, No. 10, 10.1996, p. 327-332.

Research output: Contribution to journalArticle

Cavaliere, F, Cormaci, S, Cormaci, M, Alberti, A & Colabucci, F 1996, 'Anestesia generale nella sindrome di Prader-Willi.', Minerva Anestesiologica, vol. 62, no. 10, pp. 327-332.
Cavaliere F, Cormaci S, Cormaci M, Alberti A, Colabucci F. Anestesia generale nella sindrome di Prader-Willi. Minerva Anestesiologica. 1996 Oct;62(10):327-332.
Cavaliere, F. ; Cormaci, S. ; Cormaci, M. ; Alberti, A. ; Colabucci, F. / Anestesia generale nella sindrome di Prader-Willi. In: Minerva Anestesiologica. 1996 ; Vol. 62, No. 10. pp. 327-332.
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