The first case with FBXL4 mutation successfully treated with a parenteral ketogenic diet for lactic acidosis

İNCİ A., Aktas E., Cengiz Ergin F. B., OKUR İ., BİBEROĞLU G., EZGÜ F. S., ...Daha Fazla

JOURNAL OF PARENTERAL AND ENTERAL NUTRITION, cilt.45, sa.8, ss.1788-1792, 2021 (SCI-Expanded) identifier identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 45 Sayı: 8
  • Basım Tarihi: 2021
  • Doi Numarası: 10.1002/jpen.2121
  • Dergi Adı: JOURNAL OF PARENTERAL AND ENTERAL NUTRITION
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus, BIOSIS, CAB Abstracts, CINAHL, EMBASE, International Pharmaceutical Abstracts, MEDLINE, Veterinary Science Database
  • Sayfa Sayıları: ss.1788-1792
  • Anahtar Kelimeler: enteral ketogenic diet, lactic acidosis, mitochondrial depletion syndrome, parenteral ketogenic diet
  • Gazi Üniversitesi Adresli: Evet

Özet

Background The ketogenic diet (KD) is a low-carbohydrate, high-fat diet that has been used as an effective nonpharmacological treatment in many neurological and metabolic disorders for a long time. The effectiveness of the KD is revealed in mitochondrial disorders, mainly in pyruvate dehydrogenase deficiency.