Long-term course and mechanisms of progression of renal disease in hemolytic uremic syndrome

Abstract

In the classic form of hemolytic uremic syndrome associated with toxins of gram-negative enterobacteria, mortality in the acute stage has been lower than 5% since 1978 (data from the Nephrology Committee, Argentine Society of Pediatrics). Children usually die because of severe involvement of the central nervous system, intestine, or myocardium and its complications, or because of intercurrent infection. Treatment in this phase is supportive, and efforts should be put into prevention of infection by Shiga-like toxin-producing enterohemorrhagic Escherichia coli. Of the 95% who survive, approximately one third is at risk for having chronic sequelae. Motor, sensory, or intellectual deficits, intestinal strictures, myocardial infarctions, or diabetes are infrequent. The more-frequent chronic renal lesion is characterized by the hyperfunction of nephrons remaining after the acute necrotizing lesion, which leads to progressive scarring, and not by persistence or recurrence of the microangiopathic process. Three courses of progression to end-stage renal failure have been described. Children with most severe forms do not recover from acute renal failure and enter directly into a dialysis and transplantation program. A second group recovers renal function partially, with persistent proteinuria and frequently hypertension; progression to end-stage renal failure occurs in 2 to 5 years. The third group may recover normal serum creatinine and creatinine clearance, with persistent proteinuria. They are at risk of progressing to chronic renal failure and end-stage renal disease after more than 5 years, and sometimes as late as 20 years, after the acute disease. Treatment should aim at preventing the mechanisms associated with progressive renal scarring. Transplantation is indicated in this form of hemolytic uremic syndrome, because there is little, if any, risk of recurrence, and the prognosis is similar to that of transplantation for other diseases.

Serogroup-Specific Bacterial Engineered Glycoproteins as Novel Antigenic Targets for Diagnosis of Shiga Toxin-Producing-Escherichia coli-Associated Hemolytic-Uremic Syndrome

“…This incidence rate is 10ϫ higher than that in other industrialized countries (7), representing Ͼ400 new cases of HUS annually, with a mortality rate of 2 to 5% (6). In Argentina, postdiarrheal HUS is endemic and constitutes a critical health issue, since it is the leading cause of acute renal failure among children.Because 20 to 30% of children with HUS end up with long-term renal sequelae, HUS is the second cause of chronic renal failure and accounts for 20% of the renal transplants in children and adolescents (8,9). In this country, Ͼ70% of HUS cases are associated with STEC O157 infection, followed by STEC O145 (Ͼ9%) and O121 (Ͼ2%), among those of other serogroups (6).…”

“…Because 20 to 30% of children with HUS end up with long-term renal sequelae, HUS is the second cause of chronic renal failure and accounts for 20% of the renal transplants in children and adolescents (8,9). In this country, Ͼ70% of HUS cases are associated with STEC O157 infection, followed by STEC O145 (Ͼ9%) and O121 (Ͼ2%), among those of other serogroups (6).…”

Inhibition of Water Absorption and Selective Damage to Human Colonic Mucosa Are Induced by Subtilase Cytotoxin Produced by Escherichia coli O113:H21

“…HUS develops in 5 to 10% of children several days after bloody diarrhea and is a systemic disease characterized by thrombotic microangiopathy, hemolytic anemia, thrombocytopenia, and acute renal failure. HUS is the most common cause of acute renal failure in children and the second leading cause of chronic renal failure in children younger than 5 years old (3,4). STEC O157:H7 is by far the most prevalent serotype associated with HUS although non-O157 STEC strains have been also isolated from children with HUS (5,6).…”

The Nephrology Quiz and Questionnaire

“…The typical clinical course begins with ingestion of the toxin followed by abdominal pain, fever, and vomiting and the production of bloody diarrhea. The latter typically improves spontaneously with resolution of the disease or a more virulent expression of the HUS (28,29).…”