Page last updated: 2024-09-03

carglumic acid and Acidemia Propionic

carglumic acid has been researched along with Acidemia Propionic in 14 studies

Research

Studies (14)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's0 (0.00)18.2507
2000's2 (14.29)29.6817
2010's9 (64.29)24.3611
2020's3 (21.43)2.80

Authors

AuthorsStudies
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
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
Ah Mew, N; Daikhin, Y; McCarter, R; Nissim, I; Tuchman, M; Yudkoff, M1
Jones, S; Morris, AA; Reed, CA; Vijay, S; Walter, JH1
Ababneh, F; Ahmed, H; Al Mutairi, F; Al Othaim, A; Alasmari, A; Albdah, B; Alfadhel, M; Alfares, A; Aljeraisy, M; Almannai, M; Alohali, L; Alswaid, A; Elamin, M; Eyaid, W; Faqeih, E; Hussein, MA; Nashabat, M; Rahbeeni, Z; Saleh, M; Umair, M1
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
Benenato, KE; Fortucci, S; Frassetto, A; Guey, LT; Hoar, K; Jacquinet, E; Jiang, L; Laureano, R; Liang, S; Lukacs, CM; Martini, PGV; Mihai, C; Park, JS; Presnyak, V; Tunkey, C; Yan, X; Yang, J; Yin, L; Zhu, X; Zhuo, J1
Ababneh, F; Ahmed, H; Al Mutairi, F; Alasmari, A; Alfadhel, M; Aljeraisy, M; Alohali, L; Alswaid, A; Elamin, M; Eyaid, W; Faqeih, E; Hussein, MA; Nashabat, M; Obaid, A; Saleh, M1
Abacan, M; Boneh, A1
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

Reviews

1 review(s) available for carglumic acid and Acidemia Propionic

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

4 trial(s) available for carglumic acid and Acidemia Propionic

ArticleYear
N-carbamylglutamate augments ureagenesis and reduces ammonia and glutamine in propionic acidemia.
    Pediatrics, 2010, Volume: 126, Issue:1

    Topics: Administration, Oral; Adolescent; Ammonia; Blood Chemical Analysis; Child; Child, Preschool; Confidence Intervals; Dose-Response Relationship, Drug; Drug Administration Schedule; Female; Follow-Up Studies; Glutamates; Glutamine; Humans; Infant; Male; Probability; Propionic Acidemia; Prospective Studies; Risk Assessment; Treatment Outcome; Urea

2010
Long-term effectiveness of carglumic acid in patients with propionic acidemia (PA) and methylmalonic acidemia (MMA): a randomized clinical trial.
    Orphanet journal of rare diseases, 2021, 10-11, Volume: 16, Issue:1

    Topics: Amino Acid Metabolism, Inborn Errors; Glutamates; Humans; Methylmalonic Acid; Propionic Acidemia; Prospective Studies

2021
Evaluation of long-term effectiveness of the use of carglumic acid in patients with propionic acidemia (PA) or methylmalonic acidemia (MMA): study protocol for a randomized controlled trial.
    BMC pediatrics, 2019, 06-13, Volume: 19, Issue:1

    Topics: Adolescent; Amino Acid Metabolism, Inborn Errors; Carnitine; Child; Clinical Trials, Phase III as Topic; Diet, Protein-Restricted; Drug Administration Schedule; Early Termination of Clinical Trials; Glutamates; Humans; Metronidazole; Multicenter Studies as Topic; Propionic Acidemia; Prospective Studies; Randomized Controlled Trials as Topic; Sample Size; Saudi Arabia

2019
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

9 other study(ies) available for carglumic acid and Acidemia Propionic

ArticleYear
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
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 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
Dual mRNA therapy restores metabolic function in long-term studies in mice with propionic acidemia.
    Nature communications, 2020, 10-21, Volume: 11, Issue:1

    Topics: Animals; Disease Models, Animal; Enzyme Replacement Therapy; Glutamates; Humans; Kinetics; Lipids; Liver; Methylmalonyl-CoA Decarboxylase; Mice; Mice, Knockout; Mice, Transgenic; Mitochondria; Nanoparticles; Propionic Acidemia; Protein Subunits; RNA, Messenger

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
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