Nephrology News & Articles

1 Two patients with history of STEC-HUS, posttransplant recurrence and complement gene mutations American Journal of Transplantation, June 12, 2013
Alberti M et al. - Hemolytic uremic syndrome (hus) is a disease of microangiopathic hemolytic anemia, thrombocytopenia and acute renal failure. About 90% of cases are secondary to infections by Escherichia coli strains producing Shiga-like toxins (STEC-hus), while 10% are associated with mutations in genes encoding proteins of complement system (ahus). The authors describe two patients with a clinical history of STEC-hus, who developed end-stage renal disease ...

2 Update on Streptococcus pneumoniae associated hemolytic uremic syndrome Current Opinion in Pediatrics, March 22, 2013
Spinale JM et al. – Sphus accounts for 5–15% of all hus cases. The majority of Sphus patients have pneumonia and a low mortality rate in contrast to those with meningitis, who have a more severe clinical course. Although the pathogenesis of Sphus remains unknown, the Thomsen–Friedenreich antigen seems to play a central role. S. pneumoniae produces neuraminidase, thereby exposing the Thomsen–Friedenreich antigen on the surface of cell membranes. Thomsen–Friedenreich antigen exposure can ...

3 Complement disorders and hemolytic uremic syndrome Current Opinion in Pediatrics, March 22, 2013
Joseph C et al. – Complement mediated hus (ahus) has a worse prognosis compared with shiga toxin mediated hus, often resulting in end stage renal disease. Early identification of ahus is crucial so that plasma therapy can be initiated. After renal transplantation, there is very high risk of disease recurrence and graft loss. Eculizumab and combined liver–kidney transplantation offer promise for improved prognosis. Methods Complement mediated hemolytic uremic syndrome (ahus) accounts ...

4 Usefulness of routine head ultrasound scans before surgery for congenital heart disease Pediatrics, June 3, 2013
Rios DR et al. – Preoperative brain injury on MRI was present in 26% of infants with CHD, but only 3% had any evidence of brain injury on hus. Among positive hus, 80% were false–positive results. The authors findings suggest that routine hus is not indicated in asymptomatic term or near–term neonates undergoing surgery for CHD, and MRI may be a preferable tool when the assessment of these infants is warranted. Methods A total of 167 infants diagnosed with CHD were included in this ...

5 Atypical hemolytic uremic syndrome: From the rediscovery of complement to targeted therapy European Journal of Internal Medicine , June 13, 2013
Fakhouri F et al. - Atypical hemolytic uremic syndrome (ahus) is a devastating form of renal thrombotic microangiopathy. In the last five years, authors have finally witnessed a dramatic improvement in the management of ahus patients, and three breakthroughs in the understanding of ahus have led to such an improvement. These breakthroughs have prompted the French working group on ahus to propose a new algorithm for the management of ahus in children and in adults. This algorithm will ...

6 A Novel Strategy for Hemolytic Uremic Syndrome: Successful Treatment With Thrombomodulin alpha Pediatrics, March 14, 2013
Honda T et al. - Hemolytic uremic syndrome (hus) is a life-threatening infectious disease in childhood for which there is no confirmed therapeutic strategy. Endothelial inflammation leading to microthrombosis formation via complement activation is the main pathology of hus. This is the first report of rTM being used to treat hus. These case reports show the positive effect of rTM in patients with hus. Randomized controlled studies should be performed to assess the efficacy and ...

7 Eculizumab in the treatment of atypical haemolytic uraemic syndrome and other complement-mediated renal diseases Current Opinion in Pediatrics, March 22, 2013
Nester CM et al. – The cumulative published data establish the effectiveness of eculizumab in a select group of renal diseases that have at the centre of their disease either abnormal complement control or maladaptive complement activation. Methods This review considers the use of eculizumab in the treatment of atypical haemolytic uraemic syndrome (ahus) as well as the other complement-mediated renal diseases, including dense deposit disease (DDD) and C3 glomerulonephritis (C3GN). ...

8 Atypical hemolytic uremic syndrome with MCP mutations preceded by respiratory infection Clinical and Experimental Nephrology Case Reports, May 30, 2013
Yasuda K et al. - A 14-year-old boy was referred to the hospital with general fatigue and sore throat. A chest X-ray and computed tomography revealed diffuse bilateral bronchitis. A laboratory examination showed anemia, thrombocytopenia, and renal insufficiency. He had a past medical history of hemolytic uremic syndrome (hus) without diarrhea at the age of 3; moreover, his elder brother suffered from hus at the age of 12. These findings indicated that the patient had a ...

9 Genetics and Outcome of Atypical Hemolytic Uremic Syndrome: A Nationwide French Series Comparing Children and Adults Clinical Journal of the American Society of Nephrology, April 12, 2013
Fremeaux-Bacchi V et al. - Atypical hemolytic uremic syndrome (ahus) is a rare complement-mediated kidney disease that was first recognized in children but also affects adults. This study assessed the disease presentation and outcome in a nationwide cohort of patients with ahus according to the age at onset and the underlying complement abnormalities. Mortality rate was higher in children than adults with ahus, but renal prognosis was worse in adults than children. In ...

10 Brain magnetic resonance imaging pattern and outcome in children with haemolytic-uraemic syndrome and neurological impairment treated with eculizumab Developmental Medicine & Child Neurology, May 28, 2013
Gitiaux C et al. - The aim of this study was to describe the magnetic resonance imaging (MRI) findings and the neurological and neuropsychological outcomes in paediatric, diarrhoea-associated haemolytic–uraemic syndrome (D+hus) with central nervous system impairment treated with eculizumab, a monoclonal antibody. This specific brain MRI pattern consisting of an ADC decrease in basal ganglia and white matter without major T2/fluid-attenuated inversion recovery (FLAIR) injury ...