The child with glutaric aciduria type I: anesthetic and perioperative management

Abstract

Glutaric aciduria type I (GA-1) is an inborn error of metabolism caused by a deficiency of glutaryl-CoA dehydrogenase. It presents early in life, usually after an episode of fever, dehydration, infection or fasting, and results in metabolic decompensation and neurologic damage. We report the perioperative management of a 5-year-old boy admitted to the hospital for surgery because of neurogenic hip dislocation. Here we present the preoperative preparation, which focused on appropriate fluid administration and therapy intensification, as well as the safe anesthetic management with inhalation anesthesia and remifentanil, taking into consideration the mitochondrial basis of the disease. Furthermore, the role of postoperative care is emphasized in relation to stress response prophylaxis and the avoidance of complications related to the disorder.

Perioperative Management of Acute Appendicitis in a Patient with Glutaric Aciduria Type 1

“…These two steps are essential in treating these patients, in terms of better and many times personalized treatment that they should receive. [9][10][11][12][13] The postoperative course of these patients is almost as important as the pre-and intraoperative course. These patients should be closely monitored for postoperative complications such as hemorrhage or infections, which can again trigger a new crisis.…”

Anesthesia and organic aciduria: is the use of lactated Ringer's solution absolutely contraindicated?

“…We identified all published peer‐reviewed reports of patients with OA where anesthesia management was well described: MMA ( n = 6) , PA ( n = 7) , maple syrup urine disease ( n = 4) , isovaleric aciduria ( n = 1) , glutaric aciduria type 2 ( n = 1) , and 3‐methylcrotonyl‐CoA carboxylase deficiency ( n = 1) . Some reports documented perioperative complications.…”

The Anesthetic Management of a Patient with Type 1 Glutaric Asiduria: Case Report

“…GA-1'li hastalar metabolik dekompanzasyona yatkındır ve dikkatli monitörizasyon gereklidir. 4 Bu yazıda, GA-1'i olan 16 yaşındaki hastaya inhalasyon anesteziği uygulanarak gerçekleştirilen anestezi yön-temi sunulmuştur.…”