Compounds > carglumic acid
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carglumic acid and Hyperammonemia

carglumic acid has been researched along with Hyperammonemia in 28 studies

Research

Studies (28)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's0 (0.00)18.2507
2000's8 (28.57)29.6817
2010's16 (57.14)24.3611
2020's4 (14.29)2.80

Authors

AuthorsStudies
Curcio, MR; Pochiero, F; Procopio, E; Tubili, F1
Caldovic, L; Haskins, N; McNutt, M; Morizono, H; Senkevitch, E; Sonaimuthu, P; Tuchman, M; Uapinyoying, P1
Bellinger, A; Berry, GT; Cohen, DE; Frank, NY; Gershanik, EF; Goldberg, AD; Levy, BD; Lin, AP; Lynch, R; McFaline-Figueroa, JR; Merugumala, SK; O'Donnell-Luria, AH; Rohr, F; Samuels, MA; Simon, T; Tchekmedyian, V; Waisbren, SE1
Carella, A; Di Mauro, AM; Fedele, S; Masciopinto, M; Melpignano, L; Ortolani, F; Papadia, F; Piccinno, E; Tummolo, A; Vendemiale, M1
Ah Mew, N; Chakrapani, A; Häberle, J; Longo, N1
Bellantuono, R; Bellino, V; De Palo, T; Favia, V; Morrone, A; Papadia, F; Ranieri, A; Tummolo, A1
Abdul Aziz, F; Abdullah, IS; Hassim, H; Leong, HY; Mohamed, Z; Ngu, LH; Sthaneshwar, P; Teh, SH; Yap, S1
Buchal, P; Gessler, P; Schwenk, HU; Wermuth, B1
Bisset, WM; Galloway, PG; Pieterse, L; Robinson, PH; Schwahn, BC1
Fernández Carrión, F; Fernández de Miguel, S; Gimeno Díaz de Atauri, A; Serrano Ayestarán, O; Torres Peral, R1
Donati, MA; Filippi, L; Fiorini, P; Gozzini, E; la Marca, G; Malvagia, S1
Aydin, A; Demirci, E; Soyucen, E1
Biberoglu, G; Ezgu, FS; Hasanoglu, A; Kasapkara, CS; Okur, I; Tumer, L1
Cabrera-Luque, J; Caldovic, L; Morizono, H; Senkevitch, E; Tuchman, M1
Bachmann, C; Ben-Shalom, E; Elpeleg, O; Schmid, T; Shaag, A1
Bélanger-Quintana, A; García, MJ; Martínez-Pardo, M; Pallarés, E; Torres, J; Ugarte, M; Wermuth, B1
Böhles, H; Fischer, D; Gebhardt, B; Sewell, A; Vlaho, S1
Bohles, H; Dittrich, S; Gebhardt, B; Matsika, O; Parbel, S; Vlaho, S1
Cheillan, D; Guffon, N; Häberle, J; Schiff, M; Vianey-Saban, C; Wermuth, B1
Jones, S; Morris, AA; Reed, CA; Vijay, S; Walter, JH1
Bettocchi, I; Biasucci, G; Bordugo, A; Burlina, A; Gasperini, S; La Spina, L; Maines, E; Meli, C; Menni, F; Paci, S; Procopio, E; Rossi, A; Rubert, L; Spada, M; Tubili, F; Tummolo, A1
Arrieta, F; Belanger-Quintana, A; Gajate, L; Grau, J; Pintor, R1
Abacan, M; Boneh, A1
Coto Cotallo, GD; Fernández Colomer, B; García López, JE; Montes Granda, M; Pérez González, C; Rekarte García, S1
Kiykim, E; Zubarioglu, T1
Baruteau, J; Cano, A; Chakrapani, A; Coker, M; Couce, ML; Del Toro, M; Delgado, MB; Donati, MA; Garcia-Cazorla, A; Gil-Ortega, D; Gomez-de Quero, P; Guffon, N; Hofstede, FC; Kalkan-Ucar, S; Lama-More, R; Le Mouhaer, J; Martinez-Pardo Casanova, M; Molina, A; Papadia, F; Pichard, S; Plisson, C; Rosello, P; Valayannopoulos, V1
Thompson, CA1
Acquaviva, C; Forest, I; Fouilhoux, A; Guffon, N; Levrat, V; Vianey-Saban, C1

Reviews

1 review(s) available for carglumic acid and Hyperammonemia

ArticleYear
Hyperammonaemia in classic organic acidaemias: a review of the literature and two case histories.
    Orphanet journal of rare diseases, 2018, 12-06, Volume: 13, Issue:1

    Topics: Ammonia; Glucose; Glutamates; Humans; Hyperammonemia; Propionic Acidemia

2018

Trials

1 trial(s) available for carglumic acid and Hyperammonemia

ArticleYear
Carglumic acid enhances rapid ammonia detoxification in classical organic acidurias with a favourable risk-benefit profile: a retrospective observational study.
    Orphanet journal of rare diseases, 2016, Mar-31, Volume: 11

    Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Female; Glutamates; Humans; Hyperammonemia; Infant, Newborn; Male; Propionic Acidemia; Retrospective Studies; Treatment Outcome

2016

Other Studies

26 other study(ies) available for carglumic acid and Hyperammonemia

ArticleYear
Management of methylmalonic acidemia (MMA) with N-carbamylglutamate: A case report from Italy.
    Molecular genetics & genomic medicine, 2023, Volume: 11, Issue:1

    Topics: Amino Acid Metabolism, Inborn Errors; Child; Female; Humans; Hyperammonemia; Italy

2023
Gene delivery corrects N-acetylglutamate synthase deficiency and enables insights in the physiological impact of L-arginine activation of N-acetylglutamate synthase.
    Scientific reports, 2021, 02-11, Volume: 11, Issue:1

    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
Brain MRS glutamine as a biomarker to guide therapy of hyperammonemic coma.
    Molecular genetics and metabolism, 2017, Volume: 121, Issue:1

    Topics: Brain; Coma; Female; Gastric Bypass; Glutamates; Glutamine; Humans; Hyperammonemia; Magnetic Resonance Spectroscopy; Middle Aged; Phenylacetates; Sodium Benzoate; Treatment Outcome

2017
Long-term continuous N-carbamylglutamate treatment in frequently decompensated propionic acidemia: a case report.
    Journal of medical case reports, 2018, Apr-22, Volume: 12, Issue:1

    Topics: Administration, Oral; Adolescent; Amino-Acid N-Acetyltransferase; Biomarkers; Chronic Disease; Developmental Disabilities; Dose-Response Relationship, Drug; Glutamates; Humans; Hyperammonemia; Male; Propionic Acidemia; Urea Cycle Disorders, Inborn

2018
Successful early management of a female patient with a metabolic stroke due to ornithine transcarbamylase deficiency.
    Pediatric emergency care, 2013, Volume: 29, Issue:5

    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
N-Carbamylglutamate Is an Effective Treatment for Acute Neonatal Hyperammonaemia in a Patient with Methylmalonic Aciduria.
    Neonatology, 2016, Volume: 109, Issue:4

    Topics: Administration, Oral; Amino Acid Metabolism, Inborn Errors; Ammonia; Glutamates; Humans; Hyperammonemia; Infant, Newborn; Male; Nutritional Support; Treatment Outcome

2016
Favourable long-term outcome after immediate treatment of neonatal hyperammonemia due to N-acetylglutamate synthase deficiency.
    European journal of pediatrics, 2010, Volume: 169, Issue:2

    Topics: Amino-Acid N-Acetyltransferase; DNA; DNA Mutational Analysis; Female; Follow-Up Studies; Glutamates; Humans; Hyperammonemia; Infant, Newborn; Male; Mutation; Siblings; Time Factors

2010
Biochemical efficacy of N-carbamylglutamate in neonatal severe hyperammonaemia due to propionic acidaemia.
    European journal of pediatrics, 2010, Volume: 169, Issue:1

    Topics: Ammonia; Dose-Response Relationship, Drug; Female; Follow-Up Studies; Glutamates; Humans; Hyperammonemia; Infant, Newborn; Propionic Acidemia; Renal Dialysis; Severity of Illness Index

2010
[N-carbamyl glutamate treatment in hyperammoniemia decompensated propionic acidaemia].
    Anales de pediatria (Barcelona, Spain : 2003), 2009, Volume: 71, Issue:6

    Topics: Glutamates; Humans; Hyperammonemia; Infant, Newborn; Male; Propionic Acidemia

2009
N-carbamylglutamate in emergency management of hyperammonemia in neonatal acute onset propionic and methylmalonic aciduria.
    Neonatology, 2010, Volume: 97, Issue:3

    Topics: Acute Disease; Amino Acid Metabolism, Inborn Errors; Ammonia; Emergency Medical Services; Female; Glutamates; Humans; Hyperammonemia; Infant, Newborn; Male; Methylmalonic Acid; Propionic Acidemia; Vitamin B 12 Deficiency

2010
Outpatient treatment of propionic acidemia-associated hyperammonemia with N-carbamoyl-L-glutamate in an infant.
    Clinical therapeutics, 2010, Volume: 32, Issue:4

    Topics: Carnitine; Diet; Female; Glutamates; Humans; Hyperammonemia; Infant; Propionic Acidemia

2010
N-carbamylglutamate treatment for acute neonatal hyperammonemia in isovaleric acidemia.
    European journal of pediatrics, 2011, Volume: 170, Issue:6

    Topics: Acute Disease; Amino Acid Metabolism, Inborn Errors; Ammonia; Glutamates; Humans; Hyperammonemia; Infant, Newborn; Isovaleryl-CoA Dehydrogenase; Male; Treatment Outcome

2011
A novel biochemically salvageable animal model of hyperammonemia devoid of N-acetylglutamate synthase.
    Molecular genetics and metabolism, 2012, Volume: 106, Issue:2

    Topics: Amino-Acid N-Acetyltransferase; Animals; Breeding; Disease Models, Animal; Female; Gene Order; Gene Targeting; Genotype; Glutamates; Humans; Hyperammonemia; Male; Mice; Mice, Inbred C57BL; Mice, Knockout; Phenotype

2012
N-acetylglutamate synthase deficiency and the treatment of hyperammonemic encephalopathy.
    Annals of neurology, 2002, Volume: 52, Issue:6

    Topics: Acetyltransferases; Amino Acid Sequence; Amino-Acid N-Acetyltransferase; Animals; Brain Diseases, Metabolic, Inborn; Child, Preschool; Female; Glutamates; Humans; Hyperammonemia; Male; Mice; Molecular Sequence Data; Mutation; Sequence Homology, Amino Acid

2002
Hyperammonaemia as a cause of psychosis in an adolescent.
    European journal of pediatrics, 2003, Volume: 162, Issue:11

    Topics: Adolescent; Ammonia; Follow-Up Studies; Glutamates; Humans; Hyperammonemia; Male; Psychotic Disorders; Risk Assessment; Severity of Illness Index; Treatment Outcome

2003
N-carbamylglutamate enhances ammonia detoxification in a patient with decompensated methylmalonic aciduria.
    Molecular genetics and metabolism, 2003, Volume: 79, Issue:4

    Topics: Amino Acid Metabolism, Inborn Errors; Glutamates; Humans; Hyperammonemia; Inactivation, Metabolic; Male; Methylmalonic Acid

2003
N-carbamylglutamate protects patients with decompensated propionic aciduria from hyperammonaemia.
    Journal of inherited metabolic disease, 2005, Volume: 28, Issue:2

    Topics: Glutamates; Humans; Hyperammonemia; Infant, Newborn; Infant, Newborn, Diseases; Male; Metabolism, Inborn Errors; Propionates; Severity of Illness Index

2005
Neonatal hyperammonemia: the N-carbamoyl-L-glutamic acid test.
    The Journal of pediatrics, 2005, Volume: 147, Issue:2

    Topics: Acetyltransferases; Amino-Acid N-Acetyltransferase; Fatal Outcome; Female; Glutamates; Humans; Hyperammonemia; Infant; Infant, Newborn; Male

2005
N-carbamylglutamate for neonatal hyperammonaemia in propionic acidaemia.
    Journal of inherited metabolic disease, 2008, Volume: 31 Suppl 2

    Topics: Ammonia; Biomarkers; Female; Glutamates; Humans; Hyperammonemia; Infant, Newborn; Intubation, Gastrointestinal; Male; Propionic Acidemia; Time Factors; Treatment Outcome

2008
Long-term use of carglumic acid in methylmalonic aciduria, propionic aciduria and isovaleric aciduria in Italy: a qualitative survey.
    European review for medical and pharmacological sciences, 2022, Volume: 26, Issue:14

    Topics: Amino Acid Metabolism, Inborn Errors; Glutamates; Humans; Hyperammonemia; Propionic Acidemia

2022
Carglumic acid (Carbaglu®) treatment in hyperammonemia post liver transplantation.
    Endocrinologia, diabetes y nutricion, 2020, Volume: 67, Issue:2

    Topics: Glutamates; Humans; Hyperammonemia; Liver Transplantation; Male; Middle Aged; Postoperative Complications

2020
Use of carglumic acid in the treatment of hyperammonaemia during metabolic decompensation of patients with propionic acidaemia.
    Molecular genetics and metabolism, 2013, Volume: 109, Issue:4

    Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Carnitine; Child, Preschool; Female; Glutamates; Humans; Hyperammonemia; Infant; Infant, Newborn; Male; Methylmalonyl-CoA Decarboxylase; Propionic Acidemia

2013
[Valproate-induced hyperammonemic encephalopathy in a neonate: treatment with carglumic acid].
    Anales de pediatria (Barcelona, Spain : 2003), 2014, Volume: 81, Issue:4

    Topics: Anticonvulsants; Glutamates; Humans; Hyperammonemia; Infant, Newborn; Male; Neurotoxicity Syndromes; Seizures; Valproic Acid

2014
Low dose of carglumic acid for treatment of hyperammonemia due to N-acetylglutamate synthase deficiency.
    Indian pediatrics, 2014, Volume: 51, Issue:9

    Topics: Amino-Acid N-Acetyltransferase; Child, Preschool; Glutamates; Humans; Hyperammonemia; Infant; Infant, Newborn; Male; Urea Cycle Disorders, Inborn

2014
Carglumic acid approved to treat genetic hyperammonemia.
    American journal of health-system pharmacy : AJHP : official journal of the American Society of Health-System Pharmacists, 2010, May-01, Volume: 67, Issue:9

    Topics: Drug Approval; Glutamates; Hyperammonemia

2010
Carglumic acid: an additional therapy in the treatment of organic acidurias with hyperammonemia?
    Orphanet journal of rare diseases, 2008, Jan-30, Volume: 3

    Topics: Amino Acid Metabolism, Inborn Errors; Female; Glutamates; Humans; Hyperammonemia; Infant, Newborn; Methylmalonic Acid; Propionates

2008