Liver transplantation from a live donor to a patient with maple syrup urine disease: Two case reports

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Basturk A., KEÇELİ M., Erbis H., SOYUÇEN E., Aliosmanoglu I., Dinckan A., ...Daha Fazla

TURK PEDIATRI ARSIVI-TURKISH ARCHIVES OF PEDIATRICS, cilt.53, sa.2, ss.113-116, 2018 (ESCI) identifier identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 53 Sayı: 2
  • Basım Tarihi: 2018
  • Doi Numarası: 10.5152/turkpediatriars.2018.3710
  • Dergi Adı: TURK PEDIATRI ARSIVI-TURKISH ARCHIVES OF PEDIATRICS
  • Derginin Tarandığı İndeksler: Emerging Sources Citation Index (ESCI), Scopus, TR DİZİN (ULAKBİM)
  • Sayfa Sayıları: ss.113-116
  • Anahtar Kelimeler: Child, liver transplantation, maple syrup wine disease patients, TERM-FOLLOW-UP
  • Akdeniz Üniversitesi Adresli: Evet

Özet

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.