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Epidemiology of hemolytic uremic syndrome in childhood


Hemolytic-uremic syndrome (HUS) in childhood is the most prominent cause of acute renal failure. HUS is part of thrombotic microangiopathies that are caused by several different means (N Engl J Med 347:589-600, 2002). In children HUS is mainly associated with foodborne infections by Shiga toxin-producing Escherichia coli (STEC). STEC contaminated food (raw milk, meat, juices), water and human to human transmission are the main causes for infection (Int J Infect Dis 7:42-45, 2003; J Infect Dis 186:493-500, 2002). In order to define the epidemiology, pathophysiology and long term outcome 627 pediatric patients with the clinical diagnosis of HUS were evaluated from January 1997 to December 2002, in a prospective multicenter surveillance study in Germany and Austria (incidence 0.7/100,000 and 0.4/100,000 children <15 years old, respectively). Preceding diarrhea was described in 90% of patients, bloody diarrhea in 56%. STEC infection could be detected in 82% patients either in stool culture (including shiga toxin genes) or in serum samples (antibodies to O157 lipopolysaccharide). 99% of all isolated STEC strains produced Shiga toxin 2 (Stx 2) either alone or in combination with Stx 1 or Stx 2c. Dialysis was required in 63% with a median duration of 10 days. Death occurred in 16/627 patients (3%) mainly in connection with cerebral involvement of HUS. One third of the HUS patients suffers from any kind of sequelae 1 year after the acute disease: arterial hypertension 16%, proteinuria >0.2 g/l 26%, impaired renal function (GFR <80 ml/min/1.73m2) 11%, neurologic residues 5%. Only recently from October to December 2002 an outbreak with 52 affected children caused by sorbitol-fermenting O157:H- STEC could be detected in the South of Germany and parts of Austria, a source could not yet be found. Antibiotic treatment of infections by STEC has been found to increase the risk of development of HUS (N Engl J Med 342:1990-1991, 2000). If antibiotics given to patients that develop HUS alter the course of patients was evaluated in this study prospectively. 40/372 (11%) of the patients in this study received antibiotic treatment 2 weeks prior to diagnosis of HUS.

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