General anesthesia in a patient with citrullinemia using Precedex as an adjunct to prevent delayed emergence

Patel, Hersh; Kim, Jung; Huncke, Tessa

doi:10.1016/j.jclinane.2016.04.045

Cited by 6 publications

(39 citation statements)

References 11 publications

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“…[10] Therefore, previous studies have emphasized anesthetic techniques and regimens to minimize metabolic burden on the liver and reduce stress response to surgery and meticulous management of perioperative ammonia levels. [6,7,11] Despite her low BMI, our patient remained clinically asymptomatic and maintained ammonia and amino acid levels within the reference range with L-arginine medication and diet therapy. However, an abrupt increase in ammonia level after temporary interruption of nitrogen scavenging medication suggested that she was likely to develop metabolic deterioration and hyperammonemia in response to surgical stress in the perioperative period.…”

Section: Discussionmentioning

confidence: 74%

Desflurane and remifentanil anesthesia in a child with citrin deficiency

Kim¹,

Jung²

2022

Medicine

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Rationale: Hyperammonemia, metabolic derangement, and/or the prolonged effects of anesthetics may lead to delayed emergence from general anesthesia as well as the onset of type 2 citrullinemia, even in compensated patients with citrin deficiency.Patient concern: A 5-year-old girl with citrin deficiency was scheduled for blepharoplasty under general anesthesia. She developed hyperammonemia with temporary interruption of medication for a few days before surgery. Diagnosis:The patient was genetically diagnosed as citrin deficiency with a mutation in the SLC25A13 gene via newborn screening for metabolic disorders. Her citrulline and ammonia levels were well-controlled with arginine medication and protein-rich diet. Her elevated ammonia level by temporary interruption of medication was corrected with resumption of arginine medication and proteinrich diet before surgery.Interventions: We used desflurane and remifentanil for general anesthesia to avoid hyperammonemia and delayed emergence. End-tidal desflurane concentration and anesthetic depth were carefully monitored to avoid excessive anesthesia.Outcomes: She recovered consciousness with slightly increased ammonia level immediately after anesthesia.Lessions: General anesthesia of the shortest duration with the least metabolized drugs using desflurane and remifentanil, would be beneficial for rapid emergence in surgical patients with citrin deficiency. Maintenance of nitrogen scavenging medication, a proteinrich diet, and serial measurement of ammonia levels in the perioperative period are also important for avoiding hyperammonemiarelated neurological dysfunction.Abbreviations: BIS = bispectral index, CTLN2 = type 2 citrullinemia, NICCD = neonatal intrahepatic cholestasis caused by citrin deficiency.

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Section: Discussionmentioning

confidence: 74%

Desflurane and remifentanil anesthesia in a child with citrin deficiency

Kim¹,

Jung²

2022

Medicine

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“…Preoperative management of patients with UCD aims to prevent catabolism. Given the risk of UCDs regarding acute metabolic decompensation during surgery and general anesthesia, elective surgery should only be conducted in centers equipped for handling hyperammonemic decompensation and ideally scheduled as the first case of the list 2,5,16 . Further, there should be verified emergency guidelines for use in the metabolic unit.…”

Section: Discussionmentioning

confidence: 99%

“…Residual effects of opioids and benzodiazepines, as well as metabolic decompensation (ammonia levels of 21, 51, and 33 μmol/L after 2, 24, and 48 hours, respectively), were ruled out (naloxone and anexate were administered), and we confirmed thiopental overdose. Gharavifard 17 and Patel 16 reported delayed awakening for patients with citrullinemia type I; however, it was not associated with thiopental use. In our series, there was no case of delayed emergence among children with citrullinemia type I.…”

Section: Discussionmentioning

confidence: 99%

Perioperative management of children with urea cycle disorders

Rio

Martín‐Hernández

Ruiz

et al. 2020

Pediatric Anesthesia

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Background Urea cycle disorders are congenital metabolism errors that affect ammonia elimination. Clinical signs and prognosis are strongly influenced by peak ammonia levels. Numerous triggers associated with metabolic decompensation have been described with many of them, including fasting or stress, being related to the perioperative period. Aims We aimed to assess perioperative complications in pediatric patients with urea cycle disorders requiring general anesthesia in our center. Methods We reviewed the clinical history of all the pediatric patients with a confirmed urea cycle disorders diagnosis requiring surgery or a diagnostic procedure with anesthesia between January 2002 and June 2018. Results We included 33 operations (major surgery, minor surgery, and diagnostic procedures) carried out on 10 patients via different anesthetic techniques. We observed the following complications: intraoperative hyperglycemia in one case, postoperative vomiting in eight cases, and slightly increased postoperative ammonia levels (54, 59, and 69 µmol/L) with normal preoperative levels in three cases without associated metabolic decompensation. There were two cases of perioperative hyperammonemia (72 and 69 µmol/L) secondary to preoperative metabolic decompensation (137 and 92 µmol/L) with the levels progressively dropping and normalizing in the first 24‐48 hours, respectively. Conclusions Procedures under anesthesia on pediatric patients with urea cycle diseases should be performed by experienced multidisciplinary teams at specialized centers. Perioperative management focused on avoiding catabolism (especially during fasting) and monitoring signs associated with metabolic decompensation to allow for its early treatment should be included in routine anesthetic techniques for children with urea cycle disorders.

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“…Two types exist: type I (neonatal onset) occurs when the defective arginosuccinate synthase enzyme fails to complete the third step of the urea cycle by converting citrulline and aspartate to L-argininosuccinate. Type II (adult onset) develops with the production of malfunctioning citrine proteins, which serve to transport molecules used in the metabolism of simple sugars, the assembly of protein, and the urea cycle [1]. In both types, the pathogenesis involves the build-up of ammonia, which results in neurologic symptoms, including lethargy, ataxia, and seizures.…”

Section: Introductionmentioning

confidence: 99%

“…As in other cases described, we use dexmedetomidine in continuous infusion during the procedure as it reduces the release of pain neurotransmitters by activating the receptors in the primary sensory nerves, which decreases general narcotic requirements. It also reduces sympathetic momentum and release of norepinephrine, which plays a crucial role in reducing nitrogen losses and converting proteins into ammonia [1].…”

Section: Introductionmentioning

confidence: 99%