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Ventricular septal defect closure in a neonate with combined methylmalonic aciduria/homocystinuria.

Abstract
Methylmalonic acidemia with associated homocystinuria is a rare inborn error of amino acid metabolism affecting energy supply on the cellular level. Its effects on recovery from surgically induced organ ischemia are largely unknown. We report the successful closure of a nonrestrictive ventricular septal defect by following a normothermic strategy combined with ample metabolic substrate supply.
AuthorsM K Heinemann, M Tomaske, F K Trefz, A Bosk, W Baden, G Ziemer
JournalThe Annals of thoracic surgery (Ann Thorac Surg) Vol. 72 Issue 4 Pg. 1391-2 (Oct 2001) ISSN: 0003-4975 [Print] Netherlands
PMID11603477 (Publication Type: Case Reports, Journal Article)
Chemical References
  • Methylmalonic Acid
Topics
  • Amino Acid Metabolism, Inborn Errors (surgery, urine)
  • Blood Vessel Prosthesis Implantation
  • Female
  • Heart Septal Defects, Ventricular (surgery, urine)
  • Homocystinuria (surgery, urine)
  • Humans
  • Infant, Newborn
  • Methylmalonic Acid (urine)
  • Perioperative Care

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