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Oxidative Medicine and Cellular Longevity
Volume 2014, Article ID 203512, 9 pages
Clinical Study

L-Arginine/NO Pathway Is Altered in Children with Haemolytic-Uraemic Syndrome (HUS)

1Department of Paediatric Kidney, Liver and Metabolic Diseases, Hannover Medical School, Carl-Neuberg-Straße 1, 30625 Hannover, Germany
2Institute of Clinical Pharmacology, Hannover Medical School, Carl-Neuberg-Straße 1, 30625 Hannover, Germany
3Department of Paediatrics, University of Mainz, Langenbeckstraße 1, 55131 Mainz, Germany
4Institute of Biometry, Hannover Medical School, Carl-Neuberg-Straße 1, 30625 Hannover, Germany
5Department of Medicine III, Klinikum Fulda, Pacelliallee 4, 36043 Fulda, Germany
6Department of Neuropaediatrics, University Children’s Hospital, Ruhr University Bochum, Gudrunstraße 56, 44791 Bochum, Germany

Received 6 January 2014; Accepted 11 February 2014; Published 17 March 2014

Academic Editor: Daniela Giustarini

Copyright © 2014 Nele Kirsten Kanzelmeyer et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


The haemolytic uraemic syndrome (HUS) is the most frequent cause of acute renal failure in childhood. We investigated L-arginine/NO pathway in 12 children with typical HUS and 12 age-matched healthy control subjects. Nitrite and nitrate, the major NO metabolites in plasma and urine, asymmetric dimethylarginine (ADMA) in plasma and urine, and dimethylamine (DMA) in urine were determined by GC-MS and GC-MS/MS techniques. Urinary measurements were corrected for creatinine excretion. Plasma nitrate was significantly higher in HUS patients compared to healthy controls , whereas urine nitrate was borderline lower in HUS patients compared to healthy controls . ADMA plasma concentrations were insignificantly lower, but urine ADMA levels were significantly lower in the HUS patients . Urinary DMA was not significantly elevated. In HUS patients, nitrate but not nitrite, L-arginine, or ADMA concentrations in plasma correlated with free haemoglobin concentration. Our results suggest that both NO production and ADMA synthesis are decreased in children with typical HUS. We hypothesize that in the circulation of children with HUS a vicious circle between the L-arginine/NO pathway and free haemoglobin-mediated oxidative stress exists. Disruption of this vicious circle by drugs that release NO and/or sulphydryl groups-containing drugs may offer new therapeutic options in HUS.