Zobrazeno 1 - 10
of 127
pro vyhledávání: '"Acute hyperammonemia"'
Autor:
Leandro R. Soria, Gerald S. Lipshutz, Angela De Angelis, Philip Ng, Sergio Attanasio, Suhail Khoja, Patrizia Annunziata, Nicola Brunetti-Pierri, Matthew Nitzahn, Donna Palmer
Publikováno v:
J Inherit Metab Dis
The urea cycle and glutamine synthetase (GS) are the two main pathways for waste nitrogen removal and their deficiency results in hyperammonemia. Here, we investigated the efficacy of liver-specific GS overexpression for therapy of hyperammonemia. To
Publikováno v:
The Journal of Emergency Medicine. 56:e5-e8
Background Inborn errors of metabolism (IEM) commonly present in infancy and, less commonly, later in life. Case Report This case describes an IEM, specifically, ornithine transcarbamylase deficiency, in a previously healthy 7-year-old boy who presen
Autor:
David Brossier, Bruno Ozanne, Christopher Marquis, Grant A. Mitchell, Philippe Jouvet, Isabelle Goyer, Lydia Ziani
Publikováno v:
Journal of Inherited Metabolic Disease. 42:77-85
The purpose of the study was to evaluate the influence of establishing a protocol for the use of combined sodium benzoate and sodium phenylacetate (SBSP) (Ammonul®) to treat acute hyperammonemia. This was a retrospective, single-center study in a 24
Autor:
Laura Sevilla-Tapia, Ana R. Pastor-Flores, Laura A. Palomares, Plácido Espíritu-Ramírez, Nancy Y. Ortega-Balderas, Miguel A. Torres-Vega, Ana G. Montiel-Martínez
Publikováno v:
Annals of Hepatology, Vol 17, Iss 6, Pp 1026-1034 (2018)
Introduction and aim. Hepatic encephalopathy (HE), caused by hyperammonemia resulting from liver disease, is a spectrum of neuropsychiatric and motor disorders that can lead to death. Existing therapies are deficient and alternative treatments are ne
Publikováno v:
Clinical chemistry. 67(11)
Long-term N-carbamylglutamate treatment of hyperammonemia in patients with classic organic acidemias
Autor:
Ayse Cigdem Aktuglu Zeybek, Ertugrul Kiykim, Mehmet Serif Cansever, Ozge Oguz, Cisem Duman, Tanyel Zubarioglu
Publikováno v:
Molecular Genetics and Metabolism Reports
Molecular Genetics and Metabolism Reports, Vol 26, Iss, Pp 100715-(2021)
Molecular Genetics and Metabolism Reports, Vol 26, Iss, Pp 100715-(2021)
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-carbam
Autor:
Catherine Brunel-Guitton, David Brossier, Geneviève Du Pont-Thibodeau, Nadia Savy, Philippe Jouvet, Laurence Ducharme-Crevier
Publikováno v:
Hepatic Medicine: Evidence and Research
Hepatic Medicine: Evidence and Research, 2018, Volume 10, pp.105-115. ⟨10.2147/HMER.S140711⟩
Hepatic Medicine: Evidence and Research, 2018, Volume 10, pp.105-115. ⟨10.2147/HMER.S140711⟩
Acute hyperammonemia may induce a neurologic impairment leading to an acute life-threatening condition. Coma duration, ammonia peak level, and hyperammonemia duration are the main risk factors of hyperammonemia-related neurologic deficits and death.
Publikováno v:
Clinical Toxicology. 57:375-377
Dear Editor,Deferasirox (DFR) is an oral chelator used in transfusion-dependent patients with hematological diseases [1]. Recommended dosage is 20–30 mg/kg/d, but some patients require doses >30 mg...
Autor:
Nobuya Harayama, Shun-ichi Nihei, Mitsumasa Saito, Keiji Aibara, Hideaki Arai, Ayako Kanazawa, Ken Otsuji, Satoko Simizu, Keiji Nagata, Takeru Endo, Masayuki Kamochi
Publikováno v:
Journal of UOEH. 39:271-276
Case reports of hyperammonemia due to urease-producing bacteria are found occasionally, but most of them are associated with urinary tract infections. We experienced a case of infectious enterocolitis with hyperammonemia in which the causative bacter
Autor:
S Bali, A Mallappa
Publikováno v:
BRITISH INHERITED METABOLIC DISEASE GROUP.
Background We report a clinical-biochemical findings in a child who presented with acute hyperammonemic encephalopathy. Methods Case notes and electronic record was used to collect data Results A 21 month old male child presented to accident and emer