These tools will no longer be maintained as of December 31, 2024. Archived website can be found here. PubMed4Hh GitHub repository can be found here. Contact NLM Customer Service if you have questions.
5. Clinical findings of patients with hyperammonemia affected by urea cycle disorders with hepatic encephalopathy. Lopes FF; Sitta A; de Moura Coelho D; Ribas GS; Faverzani JL; Dos Reis BG; Wajner M; Vargas CR Int J Dev Neurosci; 2022 Dec; 82(8):772-788. PubMed ID: 36129623 [TBL] [Abstract][Full Text] [Related]
6. Review of Multi-Modal Imaging in Urea Cycle Disorders: The Old, the New, the Borrowed, and the Blue. Sen K; Anderson AA; Whitehead MT; Gropman AL Front Neurol; 2021; 12():632307. PubMed ID: 33995244 [TBL] [Abstract][Full Text] [Related]
7. The utility of EEG monitoring in neonates with hyperammonemia due to inborn errors of metabolism. Wiwattanadittakul N; Prust M; Gaillard WD; Massaro A; Vezina G; Tsuchida TN; Gropman AL Mol Genet Metab; 2018 Nov; 125(3):235-240. PubMed ID: 30197275 [TBL] [Abstract][Full Text] [Related]
8. Corticospinal tract damage in HHH syndrome: a metabolic cause of hereditary spastic paraplegia. Olivieri G; Pro S; Diodato D; Di Capua M; Longo D; Martinelli D; Bertini E; Dionisi-Vici C Orphanet J Rare Dis; 2019 Aug; 14(1):208. PubMed ID: 31443672 [TBL] [Abstract][Full Text] [Related]
9. Infectious precipitants of acute hyperammonemia are associated with indicators of increased morbidity in patients with urea cycle disorders. McGuire PJ; Lee HS; ; Summar ML J Pediatr; 2013 Dec; 163(6):1705-1710.e1. PubMed ID: 24084106 [TBL] [Abstract][Full Text] [Related]
10. Evaluation of oxidative damage to biomolecules and inflammation in patients with urea cycle disorders. Lopes FF; Lamberty Faverzani J; Hammerschmidt T; Aguilar Delgado C; Ferreira de Oliveira J; Wajner M; Regla Vargas C Arch Biochem Biophys; 2023 Mar; 736():109526. PubMed ID: 36702451 [TBL] [Abstract][Full Text] [Related]
11. Feasibility of adjunct therapeutic hypothermia treatment for hyperammonemia and encephalopathy due to urea cycle disorders and organic acidemias. Lichter-Konecki U; Nadkarni V; Moudgil A; Cook N; Poeschl J; Meyer MT; Dimmock D; Baumgart S Mol Genet Metab; 2013 Aug; 109(4):354-9. PubMed ID: 23791307 [TBL] [Abstract][Full Text] [Related]
12. Glycerol phenylbutyrate efficacy and safety from an open label study in pediatric patients under 2 months of age with urea cycle disorders. Longo N; Diaz GA; Lichter-Konecki U; Schulze A; Inbar-Feigenberg M; Conway RL; Bannick AA; McCandless SE; Zori R; Hainline B; Ah Mew N; Canavan C; Vescio T; Kok T; Porter MH; Berry SA Mol Genet Metab; 2021 Jan; 132(1):19-26. PubMed ID: 33388234 [TBL] [Abstract][Full Text] [Related]
13. Mitochondrial targets in hyperammonemia: Addressing urea cycle function to improve drug therapies. Moedas MF; Simões RJM; Silva MFB Biochem Pharmacol; 2024 Apr; 222():116034. PubMed ID: 38307136 [TBL] [Abstract][Full Text] [Related]
14. Perspectives on urea cycle disorder management: Results of a clinician survey. Enns GM; Porter MH; Francis-Sedlak M; Burdett A; Vockley J Mol Genet Metab; 2019; 128(1-2):102-108. PubMed ID: 31377149 [TBL] [Abstract][Full Text] [Related]
16. Ammonia toxicity and its prevention in inherited defects of the urea cycle. Walker V Diabetes Obes Metab; 2009 Sep; 11(9):823-35. PubMed ID: 19531057 [TBL] [Abstract][Full Text] [Related]
17. Oxidative stress in urea cycle disorders: Findings from clinical and basic research. Parmeggiani B; Vargas CR Clin Chim Acta; 2018 Feb; 477():121-126. PubMed ID: 29203429 [TBL] [Abstract][Full Text] [Related]
18. In vivo monitoring of urea cycle activity with (13)C-acetate as a tracer of ureagenesis. Opladen T; Lindner M; Das AM; Marquardt T; Khan A; Emre SH; Burton BK; Barshop BA; Böhm T; Meyburg J; Zangerl K; Mayorandan S; Burgard P; Dürr UH; Rosenkranz B; Rennecke J; Derbinski J; Yudkoff M; Hoffmann GF Mol Genet Metab; 2016 Jan; 117(1):19-26. PubMed ID: 26597322 [TBL] [Abstract][Full Text] [Related]
19. Argininosuccinate lyase deficiency-argininosuccinic aciduria and beyond. Erez A; Nagamani SC; Lee B Am J Med Genet C Semin Med Genet; 2011 Feb; 157C(1):45-53. PubMed ID: 21312326 [TBL] [Abstract][Full Text] [Related]