Anesthetic Hypersensitivity in a Case-Controlled Series of Patients With Mitochondrial Disease.

BACKGROUND: Children with mitochondrial disease undergo anesthesia for a wide array of surgical procedures. However, multiple medications used for their perioperative care can affect mitochondrial function. Defects in function of the mitochondrial electron transport chain (ETC) can lead to a profound hypersensitivity to sevoflurane in children. We studied the sensitivities to sevoflurane, during mask induction and maintenance of general anesthesia, in children presenting for muscle biopsies for diagnosis of mitochondrial disease. METHODS: In this multicenter study, 91 children, aged 6 months to 16 years, presented to the operating room for diagnostic muscle biopsy for presumptive mitochondrial disease. General anesthesia was induced by a slow increase of inhaled sevoflurane concentration. The primary end point, end-tidal (ET) sevoflurane necessary to achieve a bispectral index (BIS) of 60, was recorded. Secondary end points were maximal sevoflurane used to maintain a BIS between 40 and 60 during the case, and maximum and minimum heart rate and blood pressures. After induction, general anesthesia was maintained according to the preferences of the providers directing the cases. Primary data were analyzed comparing data from patients with complex I deficiencies to other groups using nonparametric statistics in SPSS v.27. RESULTS: The median sevoflurane concentration to reach BIS of 60 during inductions (ET sevoflurane % [BIS = 60]) was significantly lower for patients with complex I defects (0.98%; 95% confidence interval [CI], 0.5-1.4) compared to complex II (1.95%; 95% CI, 1.2-2.7; P < .001), complex III (2.0%; 95% CI, 0.7-3.5; P < .001), complex IV (2.0%; 95% CI, 1.7-3.2; P < .001), and normal groups (2.2%; 95% CI, 1.8-3.0; P < .001). The sevoflurane sensitivities of complex I patients did not reach significance when compared to patients diagnosed with mitochondrial disease but without an identifiable ETC abnormality (P = .172). Correlation of complex I activity with ET sevoflurane % (BIS = 60) gave a Spearman's coefficient of 0.505 (P < .001). The differences in sensitivities between groups were less during the maintenance of the anesthetic than during induction. CONCLUSIONS: The data indicate that patients with complex I dysfunction are hypersensitive to sevoflurane compared to normal patients. Hypersensitivity was less common in patients presenting with other mitochondrial defects or without a mitochondrial diagnosis.

Mitochondrial Disease and Anesthesia

Abstract It is increasingly common for children with mitochondrial disease to undergo surgery and anesthesia. Although many different anesthetics have been used successfully for these patients, serious, unexpected complications have occurred during and following anesthetic exposure. This has led to the widespread opinion among anesthesiologists that mitochondrial patients are at increased risk from the stress of surgery and anesthesia. Defects in function of the mitochondrial electron transport chain can lead to striking hypersensitivity to volatile anesthetics in children. Despite this striking finding, the connection between mitochondrial function and response to anesthetics is unknown. We review here the anesthetic considerations for patients with mitochondrial defects. In addition, we present an approach to anesthetic care of these patients at our institutions.

Instillation of liposomes vs dimethyl sulphoxide or pentosan polysulphate for reducing bladder hyperactivity.

OBJECTIVE: To investigate the efficacy of intravesical liposomes against dimethyl sulphoxide (DMSO), and pentosan polysulphate (PPS) in reducing chemically induced bladder hyperactivity in rats. MATERIALS AND METHODS: Bladder reflex activity of female Sprague-Dawley rats was evaluated by continuous cystometry under urethane anaesthesia (1.0 g/kg). After obtaining a control cystometrogram (CMG) with normal saline (0.04 mL/min) for 2 h, bladder hyperactivity was then induced by 1 h infusion of protamine sulphate (10 mg/mL) followed by a 1-h infusion of KCl (500 mm). Six rats each were then infused with KCl-based preparations containing either 50% DMSO, PPS (6 mg/mL), or liposomes (2 mg/mL) for 2 h. The variables measured included the intercontraction interval (ICI), pressure threshold (PT) and baseline pressure (BP). RESULTS: Sequential infusion of protamine sulphate/KCl induced hyperactive bladder with no significant difference in ICI, PT or BP among groups before initiating treatment. ICI was significantly increased after infusion of PPS (58.1% increase) and liposomes (156.8% increase) but there was no increase with DMSO. PT was not significantly affected by liposome infusion but slightly increased with PPS (12.4% increase). There was a large and significant increase in PT and BP with DMSO (116.5% increase) and BP largely remained unchanged after instillation with liposomes or PPS. CONCLUSIONS: Intravesical liposomes and PPS have a beneficial effect in a bladder hyperactivity rat model, while acute instillation of DMSO does not. Intravesical liposomes were effective in doubling the ICI compared with PPS, and might be a new treatment option for bladder hyperactivity.

Development and evaluation of a charge capture program for long-term care providers.

OBJECTIVE: Clinicians often have difficulty determining the appropriate Current Procedural Terminology Evaluation and Management code to assign to the type and intensity of patient care they provide. The purpose of this study was to develop, implement, and evaluate a handheld charge capture program for use by providers in the long-term care setting. DESIGN: Using a pre-post study design, we compared the coding accuracy and user satisfaction of an established paper process with a handheld charge capture program created for this study by means of: (1) preimplementation and postimplementation assessment of coding accuracy, and (2) preimplementation and postimplementation clinician survey. SETTING: We studied an academic division of geriatric medicine. PARTICIPANTS: Participants consisted of six clinicians who currently spend at least 50% of their clinical time practicing in the long-term care setting. INTERVENTION: A handheld charge capture program to replace the current paper-based charge capture process was reviewed. RESULTS: Overall coding accuracy improved by approximately 20% when the handheld program was used instead of a paper coding process. The majority of clinicians found that the handheld program was more widely available, efficient, easier to use, and encouraged the participants to document more completely and accurately in the patient's medical record. CONCLUSION: A handheld billing and coding program used by clinicians who provide care for long-term care residents is not only feasible, but leads to an improvement in coding accuracy when compared with a paper process. In addition, clinician satisfaction toward the billing and coding processes improved with the use of the handheld program.