Childhood Kidney Diseases

Korean Society of Pediatric Nephrology (대한소아신장학회)
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A Case of Severe Hyperammonemic Encephalopathy Caused by Urinary Tract Infection in Obstructive Uropathy

  • Mun, Bo Gyung (Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine) ;
  • Lee, Joo Hoon (Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine) ;
  • Park, Young Seo (Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine) ;
  • Jung, Jiwon (Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine)
  • Received : 2021.09.03
  • Accepted : 2021.11.16
  • Published : 2021.12.31
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Abstract

Hyperammonemia is mainly caused by diseases related to liver failure. However, there are also non-hepatic causes of hyperammonemia, such as urinary tract infection (UTI) due to urease-producing organisms. Urease production by these bacteria induces a hydrolysis of urinary urea into ammonia that can cross the urothelial cell membrane and diffuse into blood vessels, leading to hyperammonemia. Delayed diagnosis and treatment of hyperammonemia can lead to lethal encephalopathy that can cause brain damage and life-threatening conditions. In the presence of obstructive uropathy, UTI by urease-producing bacteria can lead to more severe hyperammonemia due to enhanced resorption of ammonia into the systemic circulation. In this report, we present a case of acute severe hyperammonemic encephalopathy leading to brain death due to accumulation of ammonia in blood caused by Morganella morganii UTI in a 10-year-old girl with cloacal anomaly, causing obstructive uropathy even after multiple corrections.

Keywords

Acknowledgement

This study was approved by the institutional review board (IRB), and the consent was waived due to the nature of the retrospective study [IRB number 2021-0979].

References

  1. Burton BK. Urea cycle disorders. Clin Liver Dis 2000;4:815-30. https://doi.org/10.1016/s1089-3261(05)70143-4
  2. Savy N, Brossier D, Brunel-Guitton C, Ducharme-Crevier L, Du Pont-Thibodeau G, Jouvet P. Acute pediatric hyperammonemia: current diagnosis and management strategies. Hepatic Med Evid Res 2018;10:105-15. https://doi.org/10.2147/HMER.S140711
  3. Kaveggia FF, Thompson JS, Schafer EC, Fischer JL, Taylor RJ. Hyperammonemic encephalopathy in urinary diversion with urea-splitting urinary tract infection. Arch Intern Med 1990;150:2389-92. https://doi.org/10.1001/archinte.1990.00390220121025
  4. Warne S, Chitty LS, Wilcox DT. Prenatal diagnosis of cloacal anomalies. BJU Int 2002;89:78-81. https://doi.org/10.1046/j.1464-410X.2002.02556.x
  5. Ozanne B, Nelson J, Cousineau J, Lambert M, Phan V, Mitchell G, et al. Threshold for toxicity from hyperammonemia in critically ill children. J Hepatol 2012;56:123-8. https://doi.org/10.1016/j.jhep.2011.03.021
  6. Braude A.I., Siemienski J. Role of bacterial urease in experimental pyelonephritis. J Bacteriol 1960;80:171-9. https://doi.org/10.1128/jb.80.2.171-179.1960
  7. Kiyokawa H, Igawa Y, Nishizawa O. Hyperammonaemic encephalopathy associated with retention of urine in multiple large diverticula. BJU Int 2001;88:122-3. https://doi.org/10.1046/j.1464-410x.2001.02282.x
  8. Goda T, Watanabe K, Kobayashi J, Nagai Y, Ohara N, Takahashi D. A case of hyperammonemia with obstructive urinary tract infection by urease-producing bacteria. Rinsho Shinkeigaku (Clin Neurol) 2017;57:130-3. https://doi.org/10.5692/clinicalneurol.cn-001002
  9. Emura M, Tsuchihashi K, Shimizu Y, Kanamaru S, Matoba S, Ito N. A Case of Hyperammonemia Caused by Urinary Tract Infection Due to Urease-Producing Bacteria. Hinyokika Kiyo. Acta Urologica Japonica 2016;62:421-5.
  10. McEwan P, Simpson D, Kirk JM, Barr DG, McKenzie KJ. Short report: Hyperammonaemia in critically ill septic infants. Arch Dis Child 2001;84:512-3. https://doi.org/10.1136/adc.84.6.512
  11. Sinha B, Gonzalez R. Hyperammonemia in a boy with obstructive ureterocele and proteus infection. J Urol 1984;131:330-1. https://doi.org/10.1016/S0022-5347(17)50366-3