Medium-chain triglyceride supplementation under a low-carbohydrate formula is a promising therapy for adult-onset type II citrullinemia.

Hayasaka, Kiyoshi; Numakura, Chikahiko; Toyota, Kentaro; Kakizaki, Satoru; Watanabe, Hisayoshi; Haga, Hiroaki; Takahashi, Hiroshi; Takahashi, Yoshimi; Kaneko, Mieko; Yamakawa, Mitsunori; Nunoi, Hiroyuki; Kato, Takeo; Ueno, Yoshiyuki; Mori, Masatomo.

Hayasaka K; Dept. of Pediatrics, Yamagata University School of Medicine, Yamagata, Japan.
Numakura C; Dept. of Pediatrics, Yamagata University School of Medicine, Yamagata, Japan.
Toyota K; Dept. of Pediatrics, Yamagata University School of Medicine, Yamagata, Japan.
Kakizaki S; Dept. of Medicine and Molecular Science, Gunma University Graduate School of Medicine, Maebashi, Gunma, Japan.
Watanabe H; Dept. of Gastroenterology, Yamagata University School of Medicine, Yamagata, Japan.
Haga H; Dept. of Gastroenterology, Yamagata University School of Medicine, Yamagata, Japan.
Takahashi H; Dept. of Internal Medicine, Prefectural Ninohe Hospital, Iwate, Japan.
Takahashi Y; Dept. of Neurology, Hematology, Metabolism, Endocrinology, and Diabetology, Yamagata University School of Medicine, Yamagata, Japan.
Kaneko M; Dept. of Medicine and Molecular Science, Gunma University Graduate School of Medicine, Maebashi, Gunma, Japan.
Yamakawa M; Dept. of Pathological Diagnostics, Yamagata University School of Medicine, Yamagata, Japan.
Nunoi H; Division of Pediatrics, Dept. of Reproductive and Developmental Medicine, University of Miyazaki, Miyazaki, Japan.
Kato T; Dept. of Neurology, Hematology, Metabolism, Endocrinology, and Diabetology, Yamagata University School of Medicine, Yamagata, Japan.
Ueno Y; Dept. of Gastroenterology, Yamagata University School of Medicine, Yamagata, Japan.
Mori M; Dept. of Medicine and Molecular Science, Gunma University Graduate School of Medicine, Maebashi, Gunma, Japan.

Mol Genet Metab Rep ; 1: 42-50, 2014.

Article en En | MEDLINE | ID: mdl-27896073

Palabras clave

ALP, serum alkali phosphatase; ALT, alanine aminotransferase; ASS1, argininosuccinate synthetase 1; AST, aspartate aminotransferase; Adult-onset type II citrullinemia (CTLN2); BMI, body mass index; CTLN2, adult-onset type II citrullinemia; ChE, cholinesterase; Citrin deficiency; LDH, lactate dehydrogenase; MCFA, medium-chain free fatty acids; MCT, medium-chain triglycerides; Malate-aspartate shuttle; Medium-chain triglycerides (MCT); NICCD, neonatal intrahepatic cholestasis; Neonatal intrahepatic cholestasis (NICCD); PPAR, peroxisome proliferator-activated receptor; PSTI, pancreatic secretory trypsin inhibitor; SLC25A13; TIBC, total iron-binding capacity; UIBC, unsaturated iron-binding capacity; Î³-GTP, gamma-glutamyl transpeptidase

Texto completo: 1 Banco de datos: MEDLINE Idioma: En Año: 2014 Tipo del documento: Article

Texto completo: 1 Banco de datos: MEDLINE Idioma: En Año: 2014 Tipo del documento: Article