Hyperammonemia as a cause of non-renal dialysis
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liver neoplasm

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Wancjer Nelds B, Molano Triviño A, Yucuma Gutiérrez S, Martínez Montalvo CM. Hyperammonemia as a cause of non-renal dialysis: Hiperamonemia como causa de diálisis no renal. Rev. Colomb. Nefrol. [Internet]. 2020 Nov. 11 [cited 2024 Apr. 20];8(1):e385. Available from: https://revistanefrologia.org/index.php/rcn/article/view/385


Hyperammonemia is a common condition in cirrhotic patients. In the context of a non-cirrhotic cause, this is related to the increase in its production of ammonium or alteration in its elimination. The clinical presentation is nonspecific, from the alteration of the behavior to the coma, being one of the causes of non-renal dialysis due to certain values or refractoriness to medical management. The case of a 35-year-old woman is presented, who is admitted to the emergency department with altered state of consciousness, elevated ammonium levels and hypervascular mass in the liver. Biopsy of the liver lesion reports fibrolamellar liver carcinoma. The presence of a portosystemic shunt that causes hyperammonemia is attributed to this pathology. Dialysis is indicated by persistence of ammonium elevations with little response to medical treatment. In its evolution, it required a restart of renal replacement due to a reappearance of the encephalopathic state when it was suspended. This therapy is maintained until liver transplantation performed as a carcinoma treatment, with subsequent stabilization of ammonium levels and dialysis suspension.

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Allampati S, Mullen KD. Hepatic encephalopathy. Handb Liver Dis. 2017;207-16. https://doi.org/10.1016/B978-0-323-47874-8.00015-8

Téllez Villajos L, Aicart Ramos M, Moreira Vicente V. Hiperamonemia en pacientes adultos sin cirrosis. Med Clin (Barc). 2013;141(11):494-500. https://doi.org/10.1016/j.medcli.2013.04.040

Role of Dialysis in Acute Toxic Encephalopathy Secondary to Hyperammonemia. Am JKidney Dis. 2017;69(4):A69. https://dx.doi.org/10.1053/j.ajkd.2017.02.217

Laish I, Ben Ari Z. Noncirrhotic hyperammonaemic encephalopathy. Liver Int. 2011;31(9):1259-70. https://doi.org/10.1111/j.1478-3231.2011.02550.x

Chapuy CI, Sahai I, Sharma R, Zhu AX, Kozyreva ON. Hyperammonemic Encephalopathy Associated With Fibrolamellar Hepatocellular Carcinoma: Case Report, Literature Review, and Proposed Treatment Algorithm. Oncologist. 2016;21(4):514-20. https://doi.org/10.1634/theoncologist.2015-0267

Hawkes ND, Thomas GA, Jurewicz A, Williams OM, Hillier CE, McQueen IN,et al.Non-hepatic hyperammonaemia: A potentially reversible cause of encephalopathy. PostgradMed https://dx.doi.org/10.1136/pmj.77.913.717

Brusilow SW, Koehler RC, Traystman RJ, Cooper AJL. Astrocyte Glutamine Synthetase: Importance in Hyperammonemic Syndromes and Potential Target for Therapy. Neurotherapeutics. 2010;7(4):452-70. https://doi.org/10.1016/j.nurt.2010.05.015

Surjan RC, dos Santos ES, Basseres T, Makdissi FF, Machado MA. A proposed physiopathological pathway to hyperammonemic encephalopathy in a non-cirrhotic patient with fibrolamellar hepatocellular carcinoma without ornithine transcarbamylase (OTC) mutation. Am J Case Rep. 2017;18:234-41. https://doi.org/10.12659/AJCR.901682

Summar ML, Mew NA. Inborn Errors of Metabolism with Hyperammonemia: Urea Cycle Defects and Related Disorders. Pediatr Clin North Am [Internet]. 2018;65(2):231-46. https://doi.org/10.1016/j.pcl.2017.11.004

Riggle KM, Turnham R, Scott JD, Yeung RS, Riehle KJ. Fibrolamellar Hepatocellular Carci-noma: Mechanistic Distinction From Adult Hepatocellular Carcinoma. Pediatr Blood Can-cer. 2016;63(7):1163-7. https://dx.doi.org/10.1002/pbc.25970

Honeyman JN, Simon EP, Robine N, Chiaroni-Clarke R, Darcy DG, Lim IIP,et al.Detectionof a recurrent DNAJB1-PRKACA chimeric transcript in brolamellar hepatocellular carci-noma. Science. 2014;343(6174):1010-4. https://dx.doi.org/10.1126/science.1249484

Sethi S, Tageja N, Singh J, Arabi H, Dave M, Badheka A, et al. Hyperammonemic encephalopathy: A rare presentation of fibrolamellar hepatocellular carcinoma. Am J Med Sci [Internet]. 2009;338(6):522-4. https://doi.org/10.1097/MAJ.0b013e3181bccfb4

Alsina AE, Franco E, Nakshabandi A, Albers C, Kemmer N, Berry AC, et al. Successful Liver Transplantation for Hyperammonemic Fibrolamellar Hepatocellular Carcinoma. ACG Case Reports J. 2016;3(4):e106. https://doi.org/10.14309/crj.2016.79

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