Metabolic Disorders and Anesthesia

Yeoh, Cindy; Teng, Howard; Jackson, Jacob; Hingula, Lee; Irie, Takeshi; Legler, Aron; Levine, Corrine; Chu, Iris; Chai, Casey M.; Tollinche, Luis

doi:10.1007/s40140-019-00345-w

Cited by 17 publications

(41 citation statements)

References 111 publications

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“…11 Of the opioids, alfentanil has the most rapid analgesic onset and time to peak effect as well as the shortest distribution and elimination half-lives (►Fig. 1). No cases of opioids impacting the process of oxidative phosphorylation have been reported.…”

Section: Discussionmentioning

confidence: 99%

“…The breakdown of ATP in cellular tissue releases energy used for metabolism. 1 Mitochondrial disorders are defined as a group of conditions caused by the impairment of the electron transport chain leading to decreased ATP production using oxidative phosphorylation. The presentation of mitochondrial disease is extremely variable; energy-dependent organs such as the heart, brain, muscle, liver, and kidney are highly susceptible.…”

Section: Introductionmentioning

confidence: 99%

“…The involvement of at least three organ systems without a unifying diagnosis should raise the suspicion of a mitochondrial disorder. 1 The frequency of mitochondrial disorders in the pediatric population has been estimated as a minimal risk of 1 in 5,000, with complex 1 deficiencies the most commonly observed defect. 2 Children with mitochondrial disorders are at an increased risk of metabolic decompensation that may be exacerbated by illness or decreased oral intake, leading to severe lactic acidosis resulting in metabolic encephalopathy.…”

Section: Introductionmentioning

confidence: 99%

“…2 Children with mitochondrial disorders are at an increased risk of metabolic decompensation that may be exacerbated by illness or decreased oral intake, leading to severe lactic acidosis resulting in metabolic encephalopathy. 1,[3][4][5] The use of glucose-containing solutions while avoiding lactate-containing solutions is necessary to prevent lactic acidosis in these patients by promoting aerobic metabolism. [3][4][5] Here, we describe the challenges and success in selecting an anesthetic induction drug for our patient given her underlying mitochondrial disorder.…”

Section: Introductionmentioning

confidence: 99%

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Endotracheal Intubation Using Alfentanil in a Pediatric Patient with a Mitochondrial Myopathy and Gastroparesis

et al. 2020

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Children with mitochondrial disorders represent a subset of patients who require unique anesthetic considerations. Routinely administered medications for general anesthesia, such as propofol, have been shown to increase the risk of developing metabolic acidosis. In addition, both depolarizing and nondepolarizing neuromuscular blockers are contraindicated due to the risk of hyperkalemic cardiac arrest and worsening of preexisting muscle weakness, respectively. These limitations pose challenges while choosing appropriate medications for induction of general anesthesia, especially when the risk of aspiration is high. We present a novel case of using inhaled sevoflurane and intravenous alfentanil to facilitate intubation in a 4-year-old girl with a complex 1 mitochondrial disorder suffering from severe gastroparesis and esophageal dysmotility.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Endotracheal Intubation Using Alfentanil in a Pediatric Patient with a Mitochondrial Myopathy and Gastroparesis

et al. 2020

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“…Prolonged fasting leads to dehydration, biochemical imbalance, and hypoglycemia, especially in children, and has been discouraged in anesthetic and surgical practice [15][16][17]. Perioperative hypoglycemia would lead to patient morbidity and mortality, and it is a danger in pediatric practice resulting in anesthetic problems including, lethargy, irritability, metabolic acidosis, and seizures [18]. Prolonged fasting times have several negative implications and lead to poor anesthetic outcomes [19,20].…”

Section: Introductionmentioning

confidence: 99%

Preoperative Fasting Time and Its Association with Hypoglycemia during Anesthesia in Pediatric Patients Undergoing Elective Procedures at Tikur Anbessa Specialized Hospital, Addis Ababa, Ethiopia

Assen

Hassen

Abate

et al. 2021

BioMed Research International

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Background. Preoperative fasting is important to reduce the risk of pulmonary aspiration during anesthesia. The influence of prolonged fasting time on glucose levels during anesthesia in children remains uncertain. Therefore, this study is aimed at assessing preoperative fasting time and its association with hypoglycemia during anesthesia in pediatric patients undergoing elective procedures at Tikur Anbessa Specialized Hospital, Addis Ababa, Ethiopia. The research hypothesis of the study is as follows: there is a prolonged preoperative fasting time, and it influences the glucose levels during anesthesia among pediatric patients undergoing elective procedures at Tikur Anbessa Specialized Hospital, Addis Ababa, Ethiopia. Methods. Institutional based cross-sectional study was conducted among 258 pediatric patients who had undergone elective procedures in a tertiary care center. A systematic sampling method was used to select study participants. The data were collected through face-to-face interviews and medical record reviews. Binary logistic regression was used to identify associated factors of hypoglycemia during anesthesia among pediatric patients undergoing elective procedures. All explanatory variables with a p value of ≤0.25 from the bivariable logistic regression model were fitted into the multivariable logistic regression model to control the possible effect of confounders, and finally, the variables which had an independent association with hypoglycemia were identified based on adjusted odds ratio with 95% confidence interval, and a p value less than 0.05 was significant. Results. The mean (standard deviation) fasting hours from breast milk, solid foods, and clear fluids were 7.75 (2.89), 13.25 (3.14), and 12.31 (3.22), respectively. The majority (89.9%, 57.9%, and 100%) of participants had fasted from solid, breast milk, and clear fluids for more than 8, 6, and 4 hours, respectively. More than one-fourth (26.2%) of participants were hypoglycemic immediately after induction. Residence, order of nothing per mouth, source of patient, and duration of fasting from solid foods had a significant association with hypoglycemia during anesthesia in children. Conclusion. Children undergoing elective procedures were exposed to unnecessarily long fasting times which were associated with hypoglycemia during anesthesia.

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Type 1 citrullinemia patient with Brugada pattern undergoing general anesthesia for dental extractions: A case report

Dell’Olio

Lorusso

Siciliani

et al. 2023

Clinical Case Reports

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Key Clinical MessageThe perioperative control of ammonia, reduction of stress, and administration of drugs tolerated in type 1 citrullinemia and Brugada pattern allowed the successful and uneventful management of general anesthesia in the study patient.AbstractThe aim of this study was to report the targeted perioperative management of general anesthesia (GA) adopted for dental extractions in a rare patient with type 1 citrullinemia and Brugada pattern. A male, Caucasian, adult type 1 citrullinemia patient needed dental extractions under GA. The medical history showed neurodevelopmental impairment, growth retardation, epilepsy, and a Type 2 Brugada electrocardiographic pattern in the second precordial lead. The authors focused the anesthesiologic protocol on the prevention of hyperammonemia and fatal arrhythmias. Changes in diet and 10% glucose solution administration prevented protein catabolism due to the fasting period (ammonia was 44 μmol/L preoperatively and 46 μmol/L postoperatively; glycemia was 120 g/dL preoperatively and 153 g/dL postoperatively). The patient received a continuous electrocardiogram, noninvasive blood pressure, pulse oximeter, entropy monitoring, train‐of‐four monitoring, and external biphasic defibrillator pads. Midazolam, remifentanil, and dexamethasone were administered for pre‐anesthesia; thiopental and rocuronium for induction; remifentanil and desflurane for maintenance; sugammadex for decurarization. After the intraligamentary injection of lidocaine 2% with epinephrine 1:100,000 for local anesthesia, the patient developed a transient Type 1 Brugada pattern that lasted a few minutes. The whole procedure lasted 30 min. The patient's discharge to ward occurred 3 h after the end of GA. The perioperative management of ammonia, reduction of stress, and administration of drugs tolerated in Type 1 citrullinemia and Brugada pattern allowed the successful and uneventful administration of GA in the study patient.

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Metabolic Disorders and Anesthesia

Cited by 17 publications

References 111 publications

Endotracheal Intubation Using Alfentanil in a Pediatric Patient with a Mitochondrial Myopathy and Gastroparesis

Endotracheal Intubation Using Alfentanil in a Pediatric Patient with a Mitochondrial Myopathy and Gastroparesis

Preoperative Fasting Time and Its Association with Hypoglycemia during Anesthesia in Pediatric Patients Undergoing Elective Procedures at Tikur Anbessa Specialized Hospital, Addis Ababa, Ethiopia

Type 1 citrullinemia patient with Brugada pattern undergoing general anesthesia for dental extractions: A case report

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