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 me

SUBMITTER: Kim K 

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

REPOSITORIES: biostudies-literature