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- Title
Canlı donörden akçaağaç şurup idrar hastasına yapılan karaciğer nakli: İki olgu sunumu.
- Authors
Baştürk, Ahmet; Keçeli, Meryem; Erbiş, Halil; Soyucen, Erdoğan; Aliosmanoğlu, İbrahim; Dinçkan, Ayhan; Yılmaz, Aygen; Artan, Reha
- Abstract
Liver transplantation were reported in patients with classic maple syrup urine disease in the literature. Branched chain alpha keto acid dehydrogenase activity can be improved in patients after transplant, and a protein-restricted diet is usually not needed. The first patient was a boy aged 2,5 years who presented with frequent ketosis attacks and epileptic seizures, and the second patient was an 11-month-old boy who also presented with frequent ketosis episodes, both despite adherence to diet therapy. Both patients received liver transplantations from live donors. A low protein diet was no longer required and no decline in cognitive functions was observed in either patient in the follow-up. We wanted to present these cases to show that despite a normal diet, plasma levels of branched- chain amino acids remained normal without any decline in cognitive function after liver transplantation in patients with classic maple syrup urine disease patients.
- Subjects
ACIDOSIS; COGNITION; SEIZURES (Medicine); DRUGS; EPILEPSY; LIVER transplantation; ORGAN donors; OXIDOREDUCTASES; PATIENT compliance; POSTOPERATIVE period; DIETARY proteins; SPASMS; MAPLE syrup urine disease
- Publication
Türk Pediatri Arşivi, 2018, Vol 53, Issue 2, p113
- ISSN
1306-0015
- Publication type
Article
- DOI
10.5152/TurkPediatriArs.2018.3710