Jove
Visualize
Contact Us
JoVE
x logofacebook logolinkedin logoyoutube logo
ABOUT JoVE
OverviewLeadershipBlogJoVE Help Center
AUTHORS
Publishing ProcessEditorial BoardScope & PoliciesPeer ReviewFAQSubmit
LIBRARIANS
TestimonialsSubscriptionsAccessResourcesLibrary Advisory BoardFAQ
RESEARCH
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchive
EDUCATION
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualFaculty Resource CenterFaculty Site
Terms & Conditions of Use
Privacy Policy
Policies

Related Experiment Videos

[General anesthesia in Prader-Willi syndrome]

F Cavaliere1, S Cormaci, M Cormaci

  • 1Servizio di Anestesia e Rianimazione, IRCCS Oasi Maria SS per la Ricerca e la Prevenzione del Ritardo Mentale, Troina (Enna).

Minerva Anestesiologica
|October 1, 1996
PubMed
Summary
This summary is machine-generated.

Related Concept Videos

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by
Same author

A multiomic framework for predicting laryngo-esophageal dysfunction following induction chemotherapy in hypopharyngeal-laryngeal carcinoma.

ESMO open·2025
Same author

A Sputtered Gig-Lox TiO<sub>2</sub> Sponge Integrated with CsPbI<sub>3</sub>:EuI<sub>2</sub> for Semitransparent Perovskite Solar Cells.

The journal of physical chemistry. C, Nanomaterials and interfaces·2025
Same author

A multicenter retrospective analysis of recurrent/metastatic nasopharyngeal cancer from non-endemic areas: Results in the pre-immunotherapy era.

European journal of cancer (Oxford, England : 1990)·2025
Same author

Microbial players in autoimmunity: multicentric analysis of the association between <i>Mycoplasma hominis</i> serostatus and rheumatoid arthritis.

Microbiology spectrum·2025
Same author

Efficacy and safety of the combination of encorafenib/cetuximab with or without binimetinib in patients with BRAF V600E-mutated metastatic colorectal cancer: an AGEO real-world multicenter study.

ESMO open·2024
Same author

Changes in body composition in early breast cancer patients treated with aromatase inhibitors.

Journal of endocrinological investigation·2024

This study reports on a Prader-Willi syndrome patient undergoing anesthesia for dental surgery. Findings suggest impaired lipolysis contributes to hypoglycemia in Prader-Willi syndrome, despite glucose infusion.

Area of Science:

  • Anesthesiology
  • Endocrinology
  • Genetics

Background:

  • Prader-Willi syndrome (PWS) is a complex genetic disorder characterized by intellectual disability, hormonal deficiencies, and metabolic abnormalities.
  • Patients with PWS often require surgical interventions, necessitating careful anesthetic management due to potential complications like hypoglycemia and airway challenges.

Observation:

  • A 27-year-old woman with PWS underwent general anesthesia for dental surgery, presenting with typical PWS features including hypotonia and obesity.
  • Anesthesia was induced with propofol and maintained with propofol, fentanyl, and nitrous oxide, with muscle paralysis achieved using atracurium.
  • A small glottis was noted, requiring a 6 mm cuffed endotracheal tube. Surgery lasted 75 minutes with prompt recovery.

Findings:

  • Despite glucose infusion, hyperglycemia was observed post-operatively (245-279 mg/dl), contrasting with expected hypoglycemia.

Related Experiment Videos

  • Stable non-esterified fatty acid (NEFA) levels suggested impaired lipolysis, a potential source of energy during fasting or surgery.
  • Progressive insulin decrease and very low growth hormone (GH) levels were noted, indicating GH deficiency which may correlate with airway anomalies.

    • Implications:

    • The findings support the hypothesis that inadequate lipolysis contributes to hypoglycemia in Prader-Willi syndrome, particularly during periods of metabolic stress.
    • Anesthetic management in PWS patients requires vigilance for airway issues and metabolic disturbances, including impaired lipolysis and potential GH deficiency.
    • Further research into the metabolic derangements in PWS is crucial for optimizing perioperative care and understanding the syndrome's pathophysiology.