Dataset Information

Desflurane and remifentanil anesthesia in a child with citrin deficiency: A case report.

ABSTRACT:

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 protein-rich 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 protein-rich diet, and serial measurement of ammonia levels in the perioperative period are also important for avoiding hyperammonemia-related neurological dysfunction.

SUBMITTER: Kim K

PROVIDER: S-EPMC8896430 | biostudies-literature | 2022 Mar

REPOSITORIES: biostudies-literature

ACCESS DATA

Publications

Desflurane and remifentanil anesthesia in a child with citrin deficiency: A case report.

Kim Kanghui K Jung Sung Mee SM

Medicine 20220301 9

<h4>Rationale</h4>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.<h4>Patient concern</h4>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.<h4>Diagnosis</h4>The ...[more]

PMID: 35244055

Similar Datasets

Project description:BackgroundOne-lung ventilation during thoracic surgery frequently disturbs normal systemic oxygenation. However, the effect of anesthetics on arterial oxygenation during one-lung ventilation has not been well established in human study. In this clinical trial, we investigated whether a difference between desflurane-remifentanil and propofol-remifentanil anesthesia can be observed with regard to oxygenation during one-lung ventilation for thoracoscopic surgery.MethodsAdult patients with lung cancer, scheduled for video-assisted thoracoscopic lobectomy without preoperative oxygen support, were screened and randomized to receive desflurane or propofol, with remifentanil continuous infusion in both groups. Mechanical ventilation was performed with tidal volume of 8 ml/kg and FIO2 0.5 during two-lung ventilation, and 6 ml/kg and 1.0 during one-lung ventilation, both with positive end-expiratory pressure of 5 cmH2O. Arterial blood gas analysis was performed preoperatively, during two-lung ventilation, and after 15, 30, 45, and 60 min of one-lung ventilation. The primary endpoint was PaO2 at 30 min after initiating one-lung ventilation. Statistical analyses included the independent t-test for the primary endpoint and a mixed model with a post-hoc analysis to evaluate the serial changes in values.ResultsPatients were recruited between July 9 and December 2, 2014. In total, 103 patients were analyzed (n = 52 in desflurane group and n = 51 in propofol group). The primary endpoint, PaO2 at 30 min of one-lung ventilation was lower in the desflurane group than the propofol group (170 ± 72 vs. 202 ± 82 mmHg; p = 0.039). Serial changes in PaO2 during one-lung ventilation showed lower levels during desflurane anesthesia compared with propofol anesthesia (mean difference, 45 mmHg; 95% confidence interval, 16-75 mmHg; p = 0.003).ConclusionsIn conclusion, desflurane-remifentanil anesthesia resulted in decreased arterial oxygenation compared with that of propofol-remifentanil anesthesia during one-lung ventilation for thoracoscopic surgery in patients with lung cancer.Trial registrationClinicalTrials.gov identifier: NCT02191371 , registered on July 7, 2014.

Project description:BackgroundDesflurane is not recommended during anesthesia induction because of its sympathetic stimulation effect, particularly in patients with myocardial ischemic disease. To date, the hemodynamic response to 1 MAC desflurane inhalation in combination with remifentanil infusion during anesthesia induction has rarely been reported.MethodsThis investigation was designed to compare hemodynamic responses to 1 MAC desflurane (group D, n = 200) with sevoflurane (group S, n = 200) during anesthesia induction and endotracheal intubation in adult patients undergoing elective spine surgery. Subgroup analysis of the different age subgroups was also performed. With continuous infusion of remifentanil 0.1 μg/kg/min, anesthesia was induced with propofol bolus, and endotracheal intubation was performed after muscle relaxation. Heart rate (HR) and mean arterial blood pressure (MAP) were measured every minute for 5 min after anesthesia induction (T1-5) and after endotracheal intubation (T6-10).ResultsHR was significantly higher in group D (n = 182) than in group S (n = 173) at T3-10 except at T6 (1 min after intubation) (all P < 0.05). In the age-based subgroup analyses, which subdivided the group D and S into four subgroups based on patient's age, the changes in HR from baseline values were significantly different between the coeval subgroups of patients in their 20-29 years and 30-39 years of age (all P < 0.05). MAP was reduced from baseline value, irrespective of group and age.ConclusionInhalation of 1 MAC desflurane during anesthesia induction with propofol bolus and remifentanil continuous infusion and during endotracheal intubation was more likely to induce elevations in HR more likely than 1 MAC sevoflurane, especially in younger patients.Trial registrationThis study was registered in the Clinical Research Information Service (CRIS, http://cris.nih.go.kr ) of the Republic of Korea on Feb 12, 2016 (Registration No. KCT 0,001,813).

Dataset Information

Desflurane and remifentanil anesthesia in a child with citrin deficiency: A case report.

Rationale

Patient concern

Diagnosis

Interventions

Outcomes

Lessions

Publications

Desflurane and remifentanil anesthesia in a child with citrin deficiency: A case report.

Similar Datasets

OmicsDI is part of the ELIXIR infrastructure

Tweets