Use of propofol anesthesia during outpatient radiographic imaging studies in patients with Lesch-Nyhan syndrome
Identifieur interne : 009D27 ( Main/Exploration ); précédent : 009D26; suivant : 009D28Use of propofol anesthesia during outpatient radiographic imaging studies in patients with Lesch-Nyhan syndrome
Auteurs : Karen Stanley Williams [États-Unis] ; James G. Hankerson [États-Unis] ; Monique Ernst [États-Unis] ; Alan Zametkin [États-Unis]Source :
- Journal of Clinical Anesthesia [ 0952-8180 ] ; 1997.
Descripteurs français
- Wicri :
- topic : Santé mentale.
English descriptors
- KwdEn :
- Abnormality, Airway, Allopurinol, Anesthesia, Anesthesiology, Anesthetic, Anesthetic management, Apnea, Baseline neuropsychologic status, Blood pressure, Bolus dose, Carbon dioxide, Child psychiatry branch, Complete airway obstruction, Diazepam, Elsevier science, Gastric contents, General anesthesia, Georgetown university, Heart rate, Infusion, Involuntary movements, Large cohort, Lesch-Nyhan syndrome, Mental health, Mmhg, Nasal cannula, National institutes, Outpatient, Patient population, Propofol, Pulmonary aspiration, Purine metabolism, Radiographic, Rare disorder, Recessive disorder, Respiratory abnormalities, Respiratory rates, Sedation, Severe bradycardia, Syndrome, Unexplained death, ambulatory, propofol.
- Teeft :
- Abnormality, Airway, Allopurinol, Anesthesia, Anesthesiology, Anesthetic, Anesthetic management, Apnea, Baseline neuropsychologic status, Blood pressure, Bolus dose, Carbon dioxide, Child psychiatry branch, Complete airway obstruction, Diazepam, Elsevier science, Gastric contents, General anesthesia, Georgetown university, Heart rate, Infusion, Involuntary movements, Large cohort, Mental health, Mmhg, Nasal cannula, National institutes, Outpatient, Patient population, Propofol, Pulmonary aspiration, Purine metabolism, Radiographic, Rare disorder, Recessive disorder, Respiratory abnormalities, Respiratory rates, Sedation, Severe bradycardia, Syndrome, Unexplained death.
Abstract
Lesch-Nyhan syndrome is a rare, x-linked, recessive disorder of purine metabolism resulting in hyperuricemia, spasticity, choreoathetosis, dystonia, self-injurious behavior, and aggression, without significant cognitive impairment. Anesthetic management of inpatients who demonstrate classic manifestations of Lesch-Nyhan syndrome and require surgical interventions have been described. There are no guidelines in the literature addressing the anesthetic management of the outpatient with Lesch-Nyhan syndrome. Specifically, sudden, unexplained death, abnormalities in respiration, apnea, severe bradycardia, and an increased incidence of vomiting and chronic pulmonary aspiration may preclude this patient population from receiving anesthesia for outpatient procedures. General anesthesia with spontaneous ventilation was performed for diagnostic, radiographic imaging in 11 outpatients with Lesch-Nyhan syndrome using intravenous propofol. A bolus dose of 1.5 to 2.0 mg/kg propofol was followed by maintenance doses of 60 to 160 mcg/kg/min. Results during and following sedation indicated endtidal carbon dioxide ranges between 34 mmHg and 59 mmHg. Respiratory rates were never below 10 breaths/min and no partial/complete airway obstruction or labored breathing was clinically evident. Hemodynamics were within 30 % of presedation values. No patient demonstrated nausea, vomiting, or pulmonary aspiration. Baseline neuropsychologic status was achieved following sedation, and patients were discharged from the hospital 35 to 90 minutes after sedation was computed. Potential risks and benefits of using propofol in this patient population are discussed.
Url:
DOI: 10.1016/S0952-8180(96)00177-8
Affiliations:
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<front><div type="abstract" xml:lang="en">Lesch-Nyhan syndrome is a rare, x-linked, recessive disorder of purine metabolism resulting in hyperuricemia, spasticity, choreoathetosis, dystonia, self-injurious behavior, and aggression, without significant cognitive impairment. Anesthetic management of inpatients who demonstrate classic manifestations of Lesch-Nyhan syndrome and require surgical interventions have been described. There are no guidelines in the literature addressing the anesthetic management of the outpatient with Lesch-Nyhan syndrome. Specifically, sudden, unexplained death, abnormalities in respiration, apnea, severe bradycardia, and an increased incidence of vomiting and chronic pulmonary aspiration may preclude this patient population from receiving anesthesia for outpatient procedures. General anesthesia with spontaneous ventilation was performed for diagnostic, radiographic imaging in 11 outpatients with Lesch-Nyhan syndrome using intravenous propofol. A bolus dose of 1.5 to 2.0 mg/kg propofol was followed by maintenance doses of 60 to 160 mcg/kg/min. Results during and following sedation indicated endtidal carbon dioxide ranges between 34 mmHg and 59 mmHg. Respiratory rates were never below 10 breaths/min and no partial/complete airway obstruction or labored breathing was clinically evident. Hemodynamics were within 30 % of presedation values. No patient demonstrated nausea, vomiting, or pulmonary aspiration. Baseline neuropsychologic status was achieved following sedation, and patients were discharged from the hospital 35 to 90 minutes after sedation was computed. Potential risks and benefits of using propofol in this patient population are discussed.</div>
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