Metabolically Based Liver Damage Pathophysiology in Patients with Urea Cycle Disorders - A New Hypothesis

Overview

Journal World J Gastroenterol

Publisher Baishideng Publishing Group

Specialty Gastroenterology

Date 2017 Dec 7

PMID 29209134

Citations 5

Authors

Ivan Ivanovski

Milos Jesic

Ana Ivanovski

Livia Garavelli

Petar Ivanovski

Affiliations

Soon will be listed here.

Abstract

Conclusion: Providing the hypothesis is correct, neonatal liver damage in patients having UCD can be prevented by the supplementation of pregnant women with fumaric or succinic acid, prepared in the form of iron supplementation pills. After birth, liver damage in patients having UCDs can be prevented by supplementation of these patients with zinc fumarate or zinc succinylate, as well.

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References

Arndt T, Stanzel S, Sewell A . Paediatric age-dependent serum transferrin isoform distribution studied by HPLC. Clin Lab. 2008; 53(9-12):575-82. View

Teufel U, Weitz J, Flechtenmacher C, Prietsch V, Schmidt J, Hoffmann G . High urgency liver transplantation in ornithine transcarbamylase deficiency presenting with acute liver failure. Pediatr Transplant. 2011; 15(6):E110-5. DOI: 10.1111/j.1399-3046.2009.01171.x. View

Mori T, Nagai K, Mori M, Nagao M, Imamura M, Iijima M . Progressive liver fibrosis in late-onset argininosuccinate lyase deficiency. Pediatr Dev Pathol. 2002; 5(6):597-601. DOI: 10.1007/s10024-002-0109-7. View

Smith G, Cameron A . Estimating human fetal blood volume on the basis of gestational age and fetal abdominal circumference. BJOG. 2002; 109(6):721-2. DOI: 10.1111/j.1471-0528.2002.01047.x. View

LaBrecque D, Latham P, Riely C, HSIA Y, KLATSKIN G . Heritable urea cycle enzyme deficiency-liver disease in 16 patients. J Pediatr. 1979; 94(4):580-7. DOI: 10.1016/s0022-3476(79)80014-1. View

Walker V . Ammonia toxicity and its prevention in inherited defects of the urea cycle. Diabetes Obes Metab. 2009; 11(9):823-35. DOI: 10.1111/j.1463-1326.2009.01054.x. View

Tuchman M, Lee B, Lichter-Konecki U, Summar M, Yudkoff M, Cederbaum S . Cross-sectional multicenter study of patients with urea cycle disorders in the United States. Mol Genet Metab. 2008; 94(4):397-402. PMC: 2640937. DOI: 10.1016/j.ymgme.2008.05.004. View

Tamamori A, Okano Y, Ozaki H, Fujimoto A, Kajiwara M, Fukuda K . Neonatal intrahepatic cholestasis caused by citrin deficiency: severe hepatic dysfunction in an infant requiring liver transplantation. Eur J Pediatr. 2002; 161(11):609-13. DOI: 10.1007/s00431-002-1045-2. View

Mhanni A, Chan A, Collison M, Seifert B, Lehotay D, Sokoro A . Hyperornithinemia-hyperammonemia-homocitrullinuria syndrome (HHH) presenting with acute fulminant hepatic failure. J Pediatr Gastroenterol Nutr. 2008; 46(3):312-5. DOI: 10.1097/MPG.0b013e318145a8e5. View

10.

Sundaram S, Alonso E, Narkewicz M, Zhang S, Squires R . Characterization and outcomes of young infants with acute liver failure. J Pediatr. 2011; 159(5):813-818.e1. PMC: 3177978. DOI: 10.1016/j.jpeds.2011.04.016. View

11.

Zamora S, Pinto A, Scott R, Parsons H . Mitochondrial abnormalities of liver in two children with citrullinaemia. J Inherit Metab Dis. 1997; 20(4):509-16. DOI: 10.1023/a:1005341228486. View

12.

Mustafa A, Clarke J . Ornithine transcarbamoylase deficiency presenting with acute liver failure. J Inherit Metab Dis. 2006; 29(4):586. DOI: 10.1007/s10545-006-0303-2. View

13.

de Groot M, Cuppen M, Eling M, Verheijen F, Rings E, Reijngoud D . Metabolic investigations prevent liver transplantation in two young children with citrullinemia type I. J Inherit Metab Dis. 2010; 33 Suppl 3:S413-6. PMC: 3757263. DOI: 10.1007/s10545-010-9207-2. View

14.

Clayton P . Inborn errors presenting with liver dysfunction. Semin Neonatol. 2002; 7(1):49-63. DOI: 10.1053/siny.2001.0086. View

15.

Fleming R, Ponka P . Iron overload in human disease. N Engl J Med. 2012; 366(4):348-59. DOI: 10.1056/NEJMra1004967. View

16.

Feldman A, Whitington P . Neonatal hemochromatosis. J Clin Exp Hepatol. 2015; 3(4):313-20. PMC: 3940210. DOI: 10.1016/j.jceh.2013.10.004. View

17.

Ito S, Kurasawa G, Yamamoto K, Furuta I, Ishihara F, Kobayashi K . A pregnant patient with fulminant hepatic failure was found to carry a novel missense mutation in the argininosuccinate synthetase gene. J Gastroenterol. 2004; 39(11):1115-7. DOI: 10.1007/s00535-004-1455-1. View

18.

Fleming R, Bacon B . Orchestration of iron homeostasis. N Engl J Med. 2005; 352(17):1741-4. DOI: 10.1056/NEJMp048363. View

19.

Jenkins T, Weitzman P . Distinct physiological roles of animal succinate thiokinases. Association of guanine nucleotide-linked succinate thiokinase with ketone body utilization. FEBS Lett. 1986; 205(2):215-8. DOI: 10.1016/0014-5793(86)80900-0. View

20.

Whitington P, Hibbard J . High-dose immunoglobulin during pregnancy for recurrent neonatal haemochromatosis. Lancet. 2004; 364(9446):1690-8. DOI: 10.1016/S0140-6736(04)17356-X. View