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Intractable metabolic acidosis in a child with propionic acidemia undergoing liver transplantation -a case report-

Jiyoung Ryu1, Young Hee Shin, Justin Sangwook Ko

  • 1Department of Anesthesiology and Pain Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.

Korean Journal of Anesthesiology
|October 9, 2013
PubMed

Insights

Anesthetic management for propionic acidemia (PA) patients undergoing liver transplantation requires careful attention to metabolic acidosis. Key considerations include maintaining tissue perfusion, preventing hypoglycemia, and administering bicarbonate.

Area of Science:

  • Biochemistry
  • Genetics
  • Medical Science

Background:

  • Propionic acidemia (PA) is a rare metabolic disorder.
  • It results from deficient propionyl-CoA carboxylase activity.
  • Clinical features include metabolic acidosis and developmental issues.

Purpose of the Study:

  • To describe anesthetic considerations for PA patients undergoing liver transplantation.
  • To highlight strategies for managing metabolic acidosis during anesthesia.

Main Methods:

  • This is a case report.
  • Anesthetic management strategies were reviewed.
  • Focus on avoiding acidosis precipitation.

Main Results:

  • Anesthetic management is critical for PA patients.
  • Maintaining optimal tissue perfusion is vital.
  • Preventing hypoglycemia and providing bicarbonate are key.

Conclusions:

  • Anesthesia for PA liver transplant requires minimizing metabolic acidosis.
  • Strategies include maintaining perfusion, preventing hypoglycemia, and bicarbonate administration.
  • Careful anesthetic planning improves patient outcomes.

Keywords:
Liver transplantationPropionic acidemia

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