Abstract | BACKGROUND:
Neuronal ceroid lipofuscinoses (NCLs) are a group of autosomal recessive neurodegenerative diseases characterized by lysosomal accumulation of autofluorescent material in neurons and other cell types. The infantile NCL (INCL) subtype is rare (1 in >100,000 births), the most devastating of childhood subtypes, and is caused by mutations in the gene CLN1, which encodes palmitoyl- protein thioesterase-1. METHODS: To investigate the incidence of hypothermia and bradycardia during general anesthesia in patients with INCL, we conducted a case-control study to examine the perianesthetic course of patients with INCL and of controls receiving anesthesia for diagnostic studies. RESULTS: Eight children with INCL (mean age 25 mo [range, 10-32] at first anesthetic) and 25 controls (mean age 44 mo [range, 18-92]) underwent 62 anesthetics for nonsurgical procedures. Patients with INCL had neurologic deficits including developmental delay, myoclonus, and visual impairment. Patients with INCL had lower baseline temperature (36.4 +/- 0.1 vs 36.8 +/- 0.1, INCL versus controls, P < 0.007), and during anesthesia, despite active warming techniques, had significantly more hypothermia (18 vs 0 episodes, P < 0.001) and sinus bradycardia (10 vs 1, P < 0.001) compared with controls. INCL diagnosis was significantly associated with temperature decreases during anesthesia (P < 0.001), whereas age, sex, and duration of anesthesia were not (P = NS). CONCLUSIONS: We report that patients with INCL have lower baseline body temperature and during general anesthesia, despite rewarming interventions, are at increased risk for hypothermia and bradycardia. This suggests a previously unknown INCL phenotype, impaired thermoregulation. Therefore, when anesthetizing these children, careful monitoring and routine use of warming interventions are warranted.
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Authors | Ning Miao, Sondra W Levin, Eva H Baker, Rafael C Caruso, Zhongjian Zhang, Andrea Gropman, Deloris Koziol, Robert Wesley, Anil B Mukherjee, Zenaide M N Quezado |
Journal | Anesthesia and analgesia
(Anesth Analg)
Vol. 109
Issue 2
Pg. 372-8
(Aug 2009)
ISSN: 1526-7598 [Electronic] United States |
PMID | 19608805
(Publication Type: Journal Article, Research Support, N.I.H., Intramural)
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Chemical References |
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Topics |
- Anesthesia
(adverse effects)
- Anesthetics
- Body Temperature
(drug effects)
- Bradycardia
(epidemiology, etiology)
- Case-Control Studies
- Child, Preschool
- Female
- Humans
- Hypothermia
(epidemiology, etiology)
- Infant
- Intraoperative Complications
(epidemiology)
- Male
- Monitoring, Intraoperative
- Neuronal Ceroid-Lipofuscinoses
(complications, physiopathology)
- Rewarming
- Risk Assessment
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