arginine has been researched along with Hyperammonemia in 63 studies
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 3 (4.76) | 18.7374 |
| 1990's | 0 (0.00) | 18.2507 |
| 2000's | 21 (33.33) | 29.6817 |
| 2010's | 29 (46.03) | 24.3611 |
| 2020's | 10 (15.87) | 2.80 |
| Authors | Studies |
|---|---|
| Jung, SM; Kim, K | 1 |
| Bechter, M; Cederbaum, SD; Diaz, GA | 1 |
| Li, Y; Liu, XF; Lu, JJ; Qiang, JW; Yang, XY | 1 |
| Allegri, G; Burke, KE; Cederbaum, SD; Häberle, J; Lipshutz, GS; Liu, XB; Martini, PGV; Truong, B; Zhu, X | 1 |
| Fukui, K; Hanai, T; Ishibashi, H; Matsufuji, M; Nakashima, M; Takashima, S; Takeshita, E; Watanabe, Y | 1 |
| Alhasan, K; Bagga, A; Basu, RK; Bedoyan, JK; Bunchman, T; Chakraborty, R; Filler, G; Grewal, MK; Jouvet, P; Krishnappa, V; Lichter-Konecki, U; Machado, MC; McCulloch, M; Mew, NA; Picca, S; Raina, R; Schaefer, F; Sethi, SK; Vemuganti, M; Warady, BA | 1 |
| Cai, YN; Jiang, MY; Li, XZ; Lin, YT; Liu, L; Mei, HF; Peng, MZ; Shao, YX; Sheng, HY; Su, L; Yin, X | 1 |
| Amalakanti, S; Daggumati, R; Nagarjunakonda, S | 1 |
| Ando, Y; Arisaka, O; Imataka, G; Ishii, J; Nitta, A; Takagi, Y; Yoshihara, S | 1 |
| Bannick, A; Batzios, S; Bubb, G; Diaz, GA; Enns, GM; Leão-Teles, E; McNutt, MC; Merritt, JL; Potts, SL; Quinn, AG; Schulze, A; Sloan, LS; Zori, RT | 1 |
| Caldovic, L; Haskins, N; McNutt, M; Morizono, H; Senkevitch, E; Sonaimuthu, P; Tuchman, M; Uapinyoying, P | 1 |
| Deans, K; McIntosh, S; Medjoub, K; Sexton, S | 1 |
| Ono, H; Shigematsu, Y; Tamada, T | 1 |
| Harada, M; Hiura, M; Honma, Y; Ishii, M; Koya, Y; Matsumoto, S; Senju, M; Shibata, M | 1 |
| Antipov, E; Brennan, AM; Charbonneau, MR; Dagon, Y; Degar, AJ; Denney, WS; Isabella, VM; Kotula, JW; Kurtz, CB; Miller, PF; Millet, YA; Perreault, M; Puurunen, MK; Wagner, DA; West, KA | 1 |
| Finnegan, J; Giva, S; Ihidero, P; Knerr, I; Maguire, G; Monavari, A; Power, B | 1 |
| Nagesh, NK; Raturi, S; Venkatagiri, P; Venkatesh, IH | 1 |
| Bellantuono, R; Bellino, V; De Palo, T; Favia, V; Morrone, A; Papadia, F; Ranieri, A; Tummolo, A | 1 |
| DU, K; Jin, HY; Liu, WP; Luan, Z; Qu, SQ; Wang, ZY; Yang, H; Yang, YX | 1 |
| Davis, MP; Sharma, P; Shinde, SS | 1 |
| D'Onofrio, V; Enea, A; Lovera, C; Poma, F; Santarelli, F; Spada, M | 1 |
| Walker, V | 2 |
| Calvello, R; Marobbio, CM; Palmieri, F; Palmieri, L; Panaro, MA; Pierri, CL; Punzi, G | 1 |
| Ensenauer, R; Häussinger, D; Mayatepek, E; Schlune, A; Vom Dahl, S | 1 |
| Ding, Y; Li, M; Li, X; Liu, Y; Qin, Y; Song, J; Wang, Q; Wu, T; Yang, Y | 1 |
| Bain, KB; Byers, DE; Chen, C; Hachem, RR; Iuppa, JA; Patterson, GA; Trulock, EP; Witt, CA; Yusen, RD | 1 |
| Alters, SE; Burrage, LC; Elsea, SH; Frankel, AE; Georgiou, G; Jiang, MM; Johnson, DE; Lee, BH; Nagamani, SC; Rowlinson, SW; Stone, E; Sun, Q | 1 |
| Chang, CK; Chen, IF; Wu, CL; Yang, CC | 1 |
| Beblo, S; Burlina, A; Das, A; de Lonlay, P; Derbinski, J; Häberle, J; Hoffmann, GF; Konstantopoulou, V; Mayorandan, S; Rennecke, J; Thimm, E; Unsinn, C; Valayannopoulos, V | 1 |
| de Koning, L; Gifford, JL; Khan, A; Orton, DJ; Seiden-Long, I | 1 |
| Eyer, F; Felgenhauer, N; Fernando, M; Rabe, C; Schrettl, V | 1 |
| Eichinger-Öttl, U; Ertl, C; Haberlandt, E; Häberle, J; Heinz-Erian, P; Karall, D; Rostásy, K; Scholl-Bürgi, S; Sigl, SB | 1 |
| Fukushima, K; Ikeda, S; Kobayashi, K; Nakamura, M; Saheki, T; Takei, H; Tanaka, N; Yazaki, M | 1 |
| Fujisawa, T; Hayashi, H; Inui, A; Kobayashi, K; Komatsu, H; Miida, T; Nagasaka, H; Takatani, T; Takikawa, H; Tsukahara, H; Yorifuji, T | 1 |
| Jalan, R; Noiret, L; Olde Damink, SW; Wright, G | 1 |
| Aubin, I; Barnhart, KF; Benavides, F; Brandon, JL; Conti, CJ; DiGiovanni, J; Gimenez-Conti, I; Giulivi, C; Guénet, JL; Jaubert, J; Kusewitt, DF; Otto, NW; Perez, CJ; Quintanilla, VC; Ross-Inta, CM | 1 |
| Erez, A; Lee, B; Nagamani, SC | 2 |
| Mhanni, AA; Prasad, C; Rockman-Greenberg, C | 1 |
| Choi, DE; Lee, KW; Na, KR; Shin, YT | 1 |
| Craigen, WJ; Davis, EC; Pan, Y; Reid Sutton, V | 1 |
| David, L; Dionisi-Vici, C; Dolce, V; Fiermonte, G; Palmieri, F; Santorelli, FM; Walker, JE | 1 |
| BIANCHINI, HM; FONGI, EG; HERRERO, H; RIVADULLA, II | 1 |
| BRETTE, R; CILLE, Y; FRANCOIS, B | 1 |
| MOLINAROLI, P; RIGAMONTI, PP; ZECCA, C | 1 |
| Bachmann, C | 1 |
| Baumgartner, MR; Dufier, JL; Kamoun, P; Nassogne, MC; Padovani, JP; Rabier, D; Saudubray, JM; Valle, D | 1 |
| Guffon, N; Häberle, J; Koch, HG; Nuoffer, JM; Pauli, S; Schmidt, E; Vianey-Saban, C; Wermuth, B | 1 |
| Berger, R; Besley, GT; Cleary, MA; de Koning, TJ; Dorland, L; Duran, M; Mandell, R; Olpin, SE; Poll-The, BT; Shih, VE | 1 |
| Osada, H; Seki, K | 1 |
| Hara, T; Kanazawa, N; Kira, R; Sakai, Y; Sanefuji, M; Takemoto, M; Torisu, H; Tsujino, S | 1 |
| Andria, G; Carbone, MT; Correra, A; Della Casa, R; Dionisi-Vici, C; Fecarotta, S; Parenti, G; Riva, S; Santorelli, FM; Torre, G; Vajro, P; Zuppaldi, A | 1 |
| Adachi, M; Kanazawa, M; Kobayashi, K; Kubota, M; Kurokawa, K; Murakami, T; Murayama, K; Nagasaka, H; Ogawa, A; Ogawa, E; Takatani, T; Takayanagi, M; Yamamoto, S; Yorifuji, T | 1 |
| Deputy, S; Gereighty, H; Keats, B; Mannick, E; Marble, M; McGoey, RR; Ng, SS; Schmidt-Sommerfeld, E | 1 |
| Blanke, CD; Chan, JS; Harding, CO | 1 |
| Harms, E; Homberger, A; Koch, HG; Linnebank, M; Marquardt, T; Rapp, B; Winter, C | 1 |
| Almashanu, S; Aral, B; Baumgartner, MR; Hu, CA; Kamoun, P; Obie, C; Rabier, D; Saudubray, JM; Steel, G; Valle, D | 1 |
| Summar, M | 1 |
| Batshaw, ML; MacArthur, RB; Tuchman, M | 1 |
| Berry, GT; Steiner, RD | 1 |
| Bauer, MF; Bieger, I; Gempel, K; Gerbitz, KD; Hofmann, S; Pontz, BF; Stadler, S | 1 |
| Jayakumar, AR; Vanaja, P | 1 |
9 review(s) available for arginine and Hyperammonemia
| Article | Year |
|---|---|
The role and control of arginine levels in arginase 1 deficiency.
Topics: Arginase; Arginine; Child, Preschool; Humans; Hyperammonemia; Hyperargininemia; Urea Cycle Disorders, Inborn | 2023 |
Consensus guidelines for management of hyperammonaemia in paediatric patients receiving continuous kidney replacement therapy.
Topics: Arginine; Carnitine; Child; Child, Preschool; Continuous Renal Replacement Therapy; Delphi Technique; Diet, Protein-Restricted; Humans; Hybrid Renal Replacement Therapy; Hyperammonemia; Infant; Infant, Newborn; Parenteral Nutrition; Peritoneal Dialysis; Phenylacetates; Phenylbutyrates; Practice Guidelines as Topic; Renal Dialysis; Sodium Benzoate; Urea Cycle Disorders, Inborn; Vitamin B Complex | 2020 |
Ammonia metabolism and hyperammonemic disorders.
Topics: Ammonia; Animals; Arginine; Biological Transport; Brain; Cell Membrane; Humans; Hyperammonemia; Hyperinsulinism; Liver; Liver Diseases; Pyruvate Carboxylase Deficiency Disease; Syndrome; Urea; Urologic Diseases; Valproic Acid | 2014 |
Hyperargininemia due to arginase I deficiency: the original patients and their natural history, and a review of the literature.
Topics: Arginase; Arginine; Child, Preschool; Female; Guanidine; Humans; Hyperammonemia; Hyperargininemia; Infant; Liver Diseases; Paraplegia; Seizures | 2015 |
Ammonia toxicity and its prevention in inherited defects of the urea cycle.
Topics: Adult; Ammonia; Animals; Arginine; Genetic Therapy; Humans; Hyperammonemia; Ornithine Carbamoyltransferase Deficiency Disease; Urea; Urea Cycle Disorders, Inborn | 2009 |
Interorgan ammonia metabolism in liver failure: the basis of current and future therapies.
Topics: Adipose Tissue; Amino Acids; Ammonia; Arginine; Brain; Dipeptides; Glutamate-Ammonia Ligase; Glutaminase; Hepatic Encephalopathy; Humans; Hyperammonemia; Intestinal Mucosa; Kidney; Liver; Liver Failure; Lung; Muscles; Phenylbutyrates; Sodium Benzoate | 2011 |
Argininosuccinate lyase deficiency-argininosuccinic aciduria and beyond.
Topics: Arginase; Arginine; Argininosuccinate Lyase; Argininosuccinic Acid; Argininosuccinic Aciduria; Carboxy-Lyases; Humans; Hyperammonemia; Infant, Newborn; Liver Diseases; Neonatal Screening; Nitric Oxide Synthase; Ornithine; Urea Cycle Disorders, Inborn | 2011 |
Argininosuccinate lyase deficiency.
Topics: Arginine; Argininosuccinate Lyase; Argininosuccinic Acid; Argininosuccinic Aciduria; Child, Preschool; Citrulline; Cognition Disorders; Diet, Protein-Restricted; Fumarates; Genetic Testing; Glucose; Humans; Hyperammonemia; Hypertension; Infant; Infant, Newborn; Lipids; Liver Diseases; Liver Transplantation; Neonatal Screening; Phenylbutyrates; Sodium Benzoate | 2012 |
Alternative pathway therapy for urea cycle disorders: twenty years later.
Topics: Amino Acid Metabolism, Inborn Errors; Animals; Arginine; Humans; Hyperammonemia; Phenylacetates; Phenylbutyrates; Sodium Benzoate; Urea | 2001 |
4 trial(s) available for arginine and Hyperammonemia
| Article | Year |
|---|---|
Clinical effect and safety profile of pegzilarginase in patients with arginase 1 deficiency.
Topics: Adolescent; Adult; Arginase; Arginine; Child; Child, Preschool; Disease Management; Female; Humans; Hyperammonemia; Hyperargininemia; Male; Recombinant Proteins; United States; Vomiting; Young Adult | 2021 |
An engineered
Topics: Ammonia; Animals; Arginine; Biosynthetic Pathways; Disease Models, Animal; Escherichia coli; Feces; Female; Genetic Engineering; Healthy Volunteers; Humans; Hyperammonemia; Macaca fascicularis; Male; Mice; Nitrates; Stress, Physiological; Survival Analysis | 2019 |
Prevention of perceptual-motor decline by branched-chain amino acids, arginine, citrulline after tennis match.
Topics: Adult; Amino Acids, Branched-Chain; Arginine; Citrulline; Dietary Supplements; Fatigue; Humans; Hyperammonemia; Male; Psychomotor Performance; Single-Blind Method; Sports Nutritional Physiological Phenomena; Tennis | 2017 |
Effects of arginine treatment on nutrition, growth and urea cycle function in seven Japanese boys with late-onset ornithine transcarbamylase deficiency.
Topics: Age of Onset; Amino Acids; Ammonia; Analysis of Variance; Arginine; Biomarkers; Blood Proteins; Body Height; Body Weight; Child, Preschool; Cholesterol, HDL; Cholesterol, LDL; Diet, Protein-Restricted; Growth; Growth Hormone; Humans; Hyperammonemia; Insulin-Like Growth Factor Binding Protein 3; Insulin-Like Growth Factor I; Japan; Male; Nutritional Physiological Phenomena; Ornithine Carbamoyltransferase Deficiency Disease; Orotic Acid; Thyrotropin; Time Factors; Treatment Outcome; Triglycerides; Urea | 2006 |
50 other study(ies) available for arginine and Hyperammonemia
| Article | Year |
|---|---|
Desflurane and remifentanil anesthesia in a child with citrin deficiency: A case report.
Topics: Ammonia; Anesthesia, General; Arginine; Blepharoplasty; Calcium-Binding Proteins; Child, Preschool; Citrullinemia; Desflurane; Endotoxins; Female; Humans; Hyperammonemia; Mitochondrial Membrane Transport Proteins; Organic Anion Transporters; Remifentanil | 2022 |
The interaction of ammonia and manganese in abnormal metabolism of minimal hepatic encephalopathy: A comparison metabolomics study.
Topics: Alanine; Ammonia; Animals; Arginine; Brain; Citrulline; gamma-Aminobutyric Acid; Glutamic Acid; Glutamine; Hepatic Encephalopathy; Hyperammonemia; Inositol; Isoleucine; Lactic Acid; Leucine; Manganese; Metabolomics; Rats; Rats, Sprague-Dawley; Taurine | 2023 |
Lipid nanoparticle-targeted mRNA therapy as a treatment for the inherited metabolic liver disorder arginase deficiency.
Topics: Ammonia; Animals; Arginase; Arginine; Codon; Disease Models, Animal; Glutamine; Hyperammonemia; Hyperargininemia; Lipids; Liver; Liver Diseases; Male; Mice; Mice, Inbred C57BL; Nanoparticles; RNA, Messenger; Urea | 2019 |
Sodium phenylbutyrate improved the clinical state in an adult patient with arginase 1 deficiency.
Topics: Adult; Arginase; Arginine; Female; Humans; Hyperammonemia; Hyperargininemia; Phenylbutyrates | 2020 |
Clinical and biochemical characteristics of patients with ornithine transcarbamylase deficiency.
Topics: Adolescent; Adult; Ammonia; Arginine; Child; Child, Preschool; China; Creatine; Female; Humans; Hyperammonemia; Lysine; Male; Ornithine; Ornithine Carbamoyltransferase Deficiency Disease; Urea; Young Adult | 2020 |
Hyperargininemic Encephalopathy with Unique Clinical Presentation and Novel Genetic Mutations.
Topics: Adult; Arginase; Arginine; Humans; Hyperammonemia; Hyperargininemia; Male; Mutation | 2020 |
Long-term survival of a patient with acute neonatal-onset metabolic encephalopathy with carbamoyl phosphate synthetase 1 deficiency.
Topics: Arginine; Brain Diseases, Metabolic; Carbamoyl-Phosphate Synthase I Deficiency Disease; Carnitine; Female; Humans; Hyperammonemia; Infant, Newborn; Phenylbutyrates; Renal Dialysis; Sodium Benzoate | 2020 |
Gene delivery corrects N-acetylglutamate synthase deficiency and enables insights in the physiological impact of L-arginine activation of N-acetylglutamate synthase.
Topics: Amino-Acid N-Acetyltransferase; Animals; Arginine; Citrulline; Dependovirus; Disease Models, Animal; Gene Transfer Techniques; Glutamates; Humans; Hyperammonemia; Mice; Mice, Knockout; Mutant Proteins; Urea; Urea Cycle Disorders, Inborn | 2021 |
Hyperammonaemic encephalopathy following an uncomplicated surgery.
Topics: Arginine; Brain Diseases; Diagnosis, Differential; Female; Humans; Hyperammonemia; Laparoscopy; Middle Aged; Parenteral Nutrition, Total; Postoperative Complications | 2017 |
Lactate/pyruvate in hyperornithinemia-hyperammonemia-homocitrullinuria syndrome.
Topics: Arginine; Diet, Protein-Restricted; Humans; Hyperammonemia; Infant; Lactates; Male; Ornithine; Pyruvates; Urea Cycle Disorders, Inborn | 2018 |
Hyperammonemia in a Woman with Late-onset Ornithine Transcarbamylase Deficiency.
Topics: Adolescent; Amino Acids; Ammonia; Arginine; Coma; Female; Humans; Hyperammonemia; Male; Middle Aged; Mutation; Ornithine Carbamoyltransferase; Ornithine Carbamoyltransferase Deficiency Disease; Pedigree; Vomiting | 2019 |
Hyperammonaemia in Neonates and Young Children: Potential Metabolic Causes, Diagnostic Approaches and Clinical Consequences
Topics: Ammonia; Arginine; Biomarkers; Brain Diseases, Metabolic, Inborn; Carnitine; Critical Illness; Diet, Protein-Restricted; Early Diagnosis; Emergencies; Female; Humans; Hyperammonemia; Infant; Infant, Newborn; Male; Phenylbutyrates; Sodium Benzoate; Treatment Outcome | 2019 |
Use of continuous hemodiafiltration for the treatment of a neonate with hyperammonemia secondary to argininosuccinate lyase (ASAL) deficiency.
Topics: Ammonia; Arginine; Argininosuccinate Lyase; Argininosuccinic Aciduria; Breast Feeding; Combined Modality Therapy; Diet, Protein-Restricted; DNA Mutational Analysis; Female; Hemofiltration; Humans; Hyperammonemia; Infant Formula; Infant, Newborn; Lactic Acid; Sodium Benzoate | 2013 |
Successful early management of a female patient with a metabolic stroke due to ornithine transcarbamylase deficiency.
Topics: Adolescent; Arginine; Brain; Carnitine; Case Management; Citrulline; Coma; Combined Modality Therapy; Drug Therapy, Combination; Electroencephalography; Female; Glutamates; Hemofiltration; Humans; Hyperammonemia; Lethargy; Ornithine Carbamoyltransferase Deficiency Disease; Phenylbutyrates; Renal Dialysis; Tomography, Emission-Computed, Single-Photon; Vomiting | 2013 |
[Clinical effect of stem cell transplantation via hepatic artery in the treatment of type II hyperammonemia: a report on 6 cases].
Topics: AC133 Antigen; Ammonia; Antigens, CD; Arginine; Citrulline; Female; Glycoproteins; Hepatic Artery; Humans; Hyperammonemia; Infant; Male; Peptides; Stem Cell Transplantation | 2013 |
Acute hyperammonemic encephalopathy in a non-cirrhotic patient with hepatocellular carcinoma reversed by arginine therapy.
Topics: Antineoplastic Agents; Arginine; Brain Diseases, Metabolic; Carcinoma, Hepatocellular; Humans; Hyperammonemia; Indoles; Liver Neoplasms; Male; Pyrroles; Sunitinib; Young Adult | 2014 |
Hyperammonemic coma in a patient with late-onset OTC deficiency.
Topics: Arginine; Child; Coma; Diagnosis, Differential; Fluid Therapy; Humans; Hyperammonemia; Lethargy; Male; Ornithine Carbamoyltransferase Deficiency Disease; Phenotype; Rehydration Solutions; Treatment Outcome; Vomiting | 2014 |
Pathogenic potential of SLC25A15 mutations assessed by transport assays and complementation of Saccharomyces cerevisiae ORT1 null mutant.
Topics: Amino Acid Transport Systems, Basic; Arginine; Biological Transport; Culture Media; Escherichia coli; Genetic Complementation Test; Humans; Hyperammonemia; Liposomes; Mitochondrial Membrane Transport Proteins; Mutation, Missense; Ornithine; Recombinant Proteins; Saccharomyces cerevisiae; Saccharomyces cerevisiae Proteins; Urea Cycle Disorders, Inborn | 2015 |
[Seven patients of argininemia with spastic tetraplegia as the first and major symptom and prenatal diagnosis of two fetuses with high risk].
Topics: Abortion, Induced; Amniocentesis; Arginase; Arginine; Asian People; Child; Child, Preschool; Diet, Protein-Restricted; DNA Mutational Analysis; Exons; Female; Fetus; Genotype; Homozygote; Humans; Hyperammonemia; Hyperargininemia; Infant; Infant, Newborn; Male; Mutation; Phenotype; Pregnancy; Prenatal Diagnosis; Quadriplegia; Seizures | 2015 |
Hyperammonemia Syndrome After Lung Transplantation: A Single Center Experience.
Topics: Aged; Ammonia; Arginine; Biomarkers; Carnitine; Combined Modality Therapy; Decontamination; Female; Humans; Hyperammonemia; Immunosuppressive Agents; Lung Transplantation; Male; Middle Aged; Missouri; Phenylacetates; Protective Agents; Renal Dialysis; Retrospective Studies; Sodium Benzoate; Syndrome; Time Factors; Treatment Outcome; Up-Regulation | 2016 |
Human recombinant arginase enzyme reduces plasma arginine in mouse models of arginase deficiency.
Topics: Animals; Arginase; Arginine; Brain; Child; Child, Preschool; Cohort Studies; Disease Models, Animal; Female; Humans; Hyperammonemia; Hyperargininemia; Longitudinal Studies; Macaca fascicularis; Male; Mice; Mice, Inbred C57BL; Recombinant Proteins; Seizures | 2015 |
Clinical course of 63 patients with neonatal onset urea cycle disorders in the years 2001-2013.
Topics: Arginine; Child, Preschool; Female; Humans; Hyperammonemia; Infant; Kaplan-Meier Estimate; Liver Transplantation; Male; Ornithine Carbamoyltransferase Deficiency Disease; Prognosis; Retrospective Studies; Sodium Benzoate; Urea Cycle Disorders, Inborn | 2016 |
Critically High Plasma Ammonia in an Adolescent Girl.
Topics: Ammonia; Arginine; Child; Clinical Chemistry Tests; Diagnostic Errors; Fatigue; Female; Fever; Headache; Humans; Hyperammonemia; Phenylacetates; Reference Values; Sodium Benzoate | 2016 |
L-Arginine in the treatment of valproate overdose - five clinical cases.
Topics: Acyl Coenzyme A; Adult; Amino-Acid N-Acetyltransferase; Ammonia; Arginine; Carnitine; Coma; Drug Overdose; Female; Humans; Hyperammonemia; Male; Renal Dialysis; Valproic Acid | 2017 |
Amino acids in CSF and plasma in hyperammonaemic coma due to arginase1 deficiency.
Topics: Adult; Amino Acids; Ammonia; Arginine; Biomarkers; Chromatography, Ion Exchange; Coma; Glutamine; Humans; Hyperammonemia; Hyperargininemia; Lysine; Male; Ornithine | 2008 |
Conventional diet therapy for hyperammonemia is risky in the treatment of hepatic encephalopathy associated with citrin deficiency.
Topics: Arginine; Citrullinemia; Diet, Protein-Restricted; Dietary Supplements; Gene Deletion; Hepatic Encephalopathy; Humans; Hyperammonemia; Male; Middle Aged; Mitochondrial Membrane Transport Proteins | 2010 |
Favorable effect of 4-phenylacetate on liver functions attributable to enhanced bile salt export pump expression in ornithine transcarbamylase-deficient children.
Topics: Adenosine Triphosphatases; Adolescent; Arginine; ATP Binding Cassette Transporter, Subfamily B, Member 11; ATP-Binding Cassette Transporters; Bile Acids and Salts; Child, Preschool; Diet, Protein-Restricted; Female; Humans; Hyperammonemia; Liver; Ornithine Carbamoyltransferase Deficiency Disease; Phenylacetates; Receptors, Cytoplasmic and Nuclear; Retrospective Studies; RNA, Messenger; Sodium Benzoate | 2010 |
Two hypomorphic alleles of mouse Ass1 as a new animal model of citrullinemia type I and other hyperammonemic syndromes.
Topics: Alleles; Animals; Arginine; Argininosuccinate Synthase; Blotting, Western; Cell Movement; Cerebellum; Citrullinemia; Developmental Disabilities; Disease Models, Animal; Female; Growth Disorders; Humans; Hyperammonemia; Immunoenzyme Techniques; Male; Mice; Mice, Inbred C57BL; Mice, Knockout; Mice, Mutant Strains; Mutation, Missense; Nitric Oxide; Phenotype; Sodium Benzoate; Syndrome | 2010 |
Ornithine transcarbamylase deficiency presenting as recurrent abdominal pain in childhood.
Topics: Abdominal Pain; Alkalosis, Respiratory; Arginine; Carbamoyl-Phosphate Synthase I Deficiency Disease; Child, Preschool; Citrulline; Consciousness Disorders; Diagnosis, Differential; Diet, Protein-Restricted; Emergencies; Exons; Female; Glutamine; Humans; Hyperammonemia; Language Development Disorders; Liver Transplantation; Mutation, Missense; Ornithine Carbamoyltransferase; Ornithine Carbamoyltransferase Deficiency Disease; Phenylbutyrates; Recurrence | 2011 |
Hyperammonemia in a patient with late-onset ornithine carbamoyltransferase deficiency.
Topics: Age of Onset; Ammonia; Arginine; Citrulline; Humans; Hyperammonemia; Male; Middle Aged; Ornithine; Ornithine Carbamoyltransferase Deficiency Disease; Pedigree; Renal Dialysis; Sodium Benzoate | 2012 |
A mouse model of argininosuccinic aciduria: biochemical characterization.
Topics: Amino Acid Metabolism, Inborn Errors; Animals; Arginase; Arginine; Argininosuccinate Lyase; Argininosuccinic Acid; Argininosuccinic Aciduria; Carbamoyl-Phosphate Synthase (Ammonia); Cyclic GMP; Disease Models, Animal; Female; Genotype; Humans; Hyperammonemia; Male; Mice; Mice, Inbred Strains; Mice, Knockout; Nitric Oxide; Ornithine Carbamoyltransferase; Urea | 2003 |
The mitochondrial ornithine transporter. Bacterial expression, reconstitution, functional characterization, and tissue distribution of two human isoforms.
Topics: Amino Acid Transport Systems, Basic; Arginine; Biological Transport; Carrier Proteins; Citrulline; DNA, Complementary; Electrophoresis, Polyacrylamide Gel; Humans; Hyperammonemia; Kinetics; Liposomes; Liver; Lysine; Malates; Membrane Transport Proteins; Mitochondria; Models, Biological; Mutation; Ornithine; Phosphates; Protein Folding; Protein Isoforms; Recombinant Proteins; Reverse Transcriptase Polymerase Chain Reaction; Substrate Specificity; Time Factors; Tissue Distribution; Transcription, Genetic | 2003 |
[Glutamate of arginine as medication of hyperammoniemia in grave hepatic insufficiency].
Topics: Ammonia; Arginine; Glutamates; Glutamic Acid; Hepatic Insufficiency; Humans; Hyperammonemia; Liver Diseases | 1960 |
[The association of malic acid and arginine in the preventive treatment of hyperammonemia. Experimental study].
Topics: Ammonia; Arginine; Humans; Hyperammonemia; Malates | 1963 |
[EXPERIMENTAL STUDY OF ACTION OF ARGININE GLUCOSE-L-PHOSPHATE ON HYPERAMMONIEMIA IN THE COURSE OF RESPIRATORY ACIDOSIS].
Topics: Acidosis; Acidosis, Respiratory; Ammonia; Animals; Arginine; Blood; Dogs; Glucose; Hexosephosphates; Hyperammonemia; Organophosphorus Compounds; Phosphates; Research | 1965 |
Long-term outcome of patients with urea cycle disorders and the question of neonatal screening.
Topics: Amino Acid Metabolism, Inborn Errors; Amino Acids; Arginine; Argininosuccinic Acid; Child; Citrulline; Diet; False Positive Reactions; Humans; Hyperammonemia; Infant, Newborn; Intellectual Disability; Metabolism, Inborn Errors; Neonatal Screening; Retrospective Studies; Treatment Outcome; Urea | 2003 |
Delta1-pyrroline-5-carboxylate synthase deficiency: neurodegeneration, cataracts and connective tissue manifestations combined with hyperammonaemia and reduced ornithine, citrulline, arginine and proline.
Topics: Amino Acid Metabolism, Inborn Errors; Arginine; Cataract; Child; Child, Preschool; Citrulline; delta-1-Pyrroline-5-Carboxylate Reductase; Female; Humans; Hyperammonemia; Male; Mutation, Missense; Ornithine; Phenotype; Proline; Pyrroline Carboxylate Reductases | 2005 |
Identification of novel mutations of the human N-acetylglutamate synthase gene and their functional investigation by expression studies.
Topics: Acetyltransferases; Amino-Acid N-Acetyltransferase; Arginine; DNA Mutational Analysis; Enzyme Activation; Escherichia coli; Humans; Hyperammonemia; Mitochondrial Proteins; Mutation, Missense; Protein Sorting Signals | 2005 |
Ornithine aminotransferase deficiency: diagnostic difficulties in neonatal presentation.
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Arginine; Citrulline; Diagnosis, Differential; Female; Fibroblasts; Glutamine; Humans; Hyperammonemia; Infant, Newborn; Male; Mutation; Neonatal Screening; Ornithine; Ornithine-Oxo-Acid Transaminase; Orotic Acid | 2005 |
Amino acid changes during successful pregnancy in a case of lysinuric protein insufficiency.
Topics: Adult; Amino Acid Metabolism, Inborn Errors; Arginine; Biological Transport; Biomarkers; Diet, Protein-Restricted; Female; Humans; Hyperammonemia; Lysine; Ornithine; Pregnancy; Pregnancy Complications | 2006 |
A novel R275X mutation of the SLC25A15 gene in a Japanese patient with the HHH syndrome.
Topics: Adult; Amino Acid Metabolism, Inborn Errors; Amino Acid Transport Systems, Basic; Arginine; DNA Mutational Analysis; Female; Humans; Hyperammonemia; Japan; Male; Mitochondrial Diseases; Mitochondrial Membrane Transport Proteins; Mutation; Proteins | 2006 |
HHH syndrome (hyperornithinaemia, hyperammonaemia, homocitrullinuria), with fulminant hepatitis-like presentation.
Topics: Alanine Transaminase; Amino Acid Transport Systems, Basic; Arginine; Aspartate Aminotransferases; Child, Preschool; Citrulline; Diet, Protein-Restricted; Hepatitis; Humans; Hyperammonemia; Liver; Male; Metabolism, Inborn Errors; Mitochondrial Membrane Transport Proteins; Ornithine; Proteins; Syndrome | 2006 |
Living related liver transplant in a patient with argininosuccinic aciduria and cirrhosis: metabolic follow-up.
Topics: Amino Acid Metabolism, Inborn Errors; Arginine; Argininosuccinic Acid; Follow-Up Studies; Humans; Hyperammonemia; Infant; Liver Cirrhosis; Liver Transplantation; Male | 2008 |
Postchemotherapy hyperammonemic encephalopathy emulating ornithine transcarbamoylase (OTC) deficiency.
Topics: Adult; Arginine; Carcinoma, Hepatocellular; Citrulline; Female; Glutamine; Hepatic Encephalopathy; Humans; Hyperammonemia; Liver Neoplasms; Ornithine; Ornithine Carbamoyltransferase Deficiency Disease; Orotic Acid; Polymorphism, Single-Stranded Conformational; Syndrome | 2008 |
Two novel mutations (E86A, R113W) in argininosuccinate lyase deficiency and evidence for highly variable splicing of the human argininosuccinate lyase gene.
Topics: Amino Acid Metabolism, Inborn Errors; Arginine; Argininosuccinate Lyase; Argininosuccinic Acid; Argininosuccinic Aciduria; Child, Preschool; Hepatomegaly; Humans; Hyperammonemia; Point Mutation; Psychomotor Disorders; RNA Splicing; Sodium Benzoate | 2000 |
Hyperammonemia with reduced ornithine, citrulline, arginine and proline: a new inborn error caused by a mutation in the gene encoding delta(1)-pyrroline-5-carboxylate synthase.
Topics: Adult; Amino Acid Metabolism, Inborn Errors; Animals; Arginine; Catalytic Domain; Child; CHO Cells; Citrulline; Cricetinae; DNA Mutational Analysis; Female; Fibroblasts; France; Humans; Hyperammonemia; Male; Mutation; Ornithine; Ornithine-Oxo-Acid Transaminase; Pedigree; Phenotype; Proline; RNA, Messenger; Transfection | 2000 |
Current strategies for the management of neonatal urea cycle disorders.
Topics: Algorithms; Amino Acid Metabolism, Inborn Errors; Antimetabolites, Antineoplastic; Arginine; Dialysis; Extracorporeal Membrane Oxygenation; Humans; Hyperammonemia; Infant, Newborn; Phenylacetates; Sodium Benzoate; Urea | 2001 |
Long-term management of patients with urea cycle disorders.
Topics: Amino Acid Metabolism, Inborn Errors; Arginine; Carbamoyl-Phosphate Synthase (Ammonia); Citrulline; Dietary Proteins; Humans; Hyperammonemia; Ornithine Carbamoyltransferase Deficiency Disease; Phenylbutyrates; Urea | 2001 |
Detection of neonatal argininosuccinate lyase deficiency by serum tandem mass spectrometry.
Topics: Amino Acid Sequence; Amino Acids; Ammonia; Arginine; Argininosuccinate Lyase; Argininosuccinic Acid; Argininosuccinic Aciduria; Base Sequence; Citrulline; Coma; Frameshift Mutation; Humans; Hyperammonemia; Infant, Newborn; Mass Spectrometry; Molecular Sequence Data; Neonatal Screening; Ornithine | 2001 |
Evidence for an involvement of the ammonia-decreasing action of L-arginine in suppressing picrotoxin-induced convulsions in rats and its additive action with diazepam.
Topics: Ammonia; Animals; Arginine; Brain; Diazepam; Drug Interactions; Epilepsy; GABA Antagonists; GABA Modulators; gamma-Aminobutyric Acid; Hyperammonemia; Male; Neural Inhibition; Picrotoxin; Rats; Rats, Wistar; Reaction Time; Seizures; Synaptic Transmission | 2001 |