A case of homocystinuria: anesthetic implications

  • Kewal Krishan Gupta Department of Anesthesiology and Intensive Care, Post Graduate Institute of Medical Education & Research (PGIMER), Chandigarh, Punjab (India)
  • Sammer Sethi Department of Anesthesiology and Intensive Care, Post Graduate Institute of Medical Education & Research (PGIMER), Chandigarh, Punjab (India)
Keywords: Homocystinuria, Thromoboembolism, Dehydration, Nitrous oxide

Abstract

We report a case of 12 yr. old male child who presented with diminshed vision for 3-4 years and was found to have supranasal subluxation of lens. He was suspected to have homocystinuria and confirmed by high serum homocysteine level. Homocystinuria is second most common inherited amino acid metabolic disorder, associated with cystathionine-beta-synthetase deficiency. After reducing his serum homocysteine level with medication, patient was taken up for surgery under general anesthesia. During perioperative period prolonged fasting hours and use of nitrous oxide were avoided and use of pneumatic compressions was taken to avoid the various complications and mortality associated with this syndrome.

Citation: Gupta KK, Sethi S. A case of homocystinuria: anesthetic implications. Anaesth Pain & Intensive Care 2014;18(4):443-45

Published
01-28-2019
How to Cite
Gupta, K. K., & Sethi, S. (2019). A case of homocystinuria: anesthetic implications. Anaesthesia, Pain & Intensive Care, 443-445. Retrieved from https://mail.apicareonline.com/index.php/APIC/article/view/371
Issue
Vol.18 No.4(2014): October-December
Section
Case Reports