Long-term N-carbamylglutamate treatment of hyperammonemia in patients with classic organic acidemias

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KIYKIM E., Oguz O., DUMAN Ç., ZÜBARİOĞLU T., CANSEVER M. Ş., AKTUĞLU ZEYBEK A. Ç.

MOLECULAR GENETICS AND METABOLISM REPORTS, vol.26, 2021 (SCI-Expanded)

• Publication Type: Article / Article
• Volume: 26
• Publication Date: 2021
• Doi Number: 10.1016/j.ymgmr.2021.100715
• Journal Name: MOLECULAR GENETICS AND METABOLISM REPORTS
• Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus, EMBASE, Directory of Open Access Journals
• Istanbul University Affiliated: No

Abstract

Background: Classic organic acidurias (OAs) usually characterized by recurrent episodes of acidemia, ketonuria, and hyperammonemia leading to coma and even death if left untreated. Acute hyperammonemia episodes can be treated effectively with N-carbamylglutamate (NCG). The effect of the long-term efficacy of N-carbamylglutamate is little known.