We found a match
Your institution may have rights to this item. Sign in to continue.
- Title
A case of homocystinuria: anesthetic implications.
- Authors
Krishan Gupta, Kewal; Sethi, Sammer
- 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.
- Subjects
HOMOCYSTINURIA; HOMOCYSTEINE in the body; AMINO acids in the blood; METABOLIC disorders in children; CYSTATHIONINE beta-synthase; GENERAL anesthesia; DEHYDRATION in children; NITROUS oxide
- Publication
Anaesthesia, Pain & Intensive Care, 2014, Vol 18, Issue 4, p443
- ISSN
1607-8322
- Publication type
Case Study