Long-term prognosis of hemolytic uremic syndrome and effective renal plasma flow

Humboldt-Universität zu Berlin, Berlín, Berlin, Germany
Pediatric Nephrology (Impact Factor: 2.86). 11/1999; 13(8):672-7. DOI: 10.1007/s004670050679
Source: PubMed


The long-term prognosis of diarrhea-associated hemolytic uremic syndrome (D+ HUS) was evaluated in a cohort of 127 of 149 children who had survived the acute phase. Glomerular filtration rate (GFR) and effective renal plasma flow (ERPF) were estimated by serial (51)Cr-EDTA and (123)iodine-hippurate clearances. All children had acute renal failure during the initial phase and 74% of patients were dialyzed. During the 1st year, mean GFR and ERPF increased continuously until a plateau was reached. In the 2nd year after the diagnosis of HUS, GFR was below 80 and ERPF below 515 ml/min per 1. 73 m(2) in 16% and 47% of patients, respectively. At the end of a median follow-up of 5.0 (range 2.0-13.2) years, the proportion of children with renal sequelae such as proteinuria >/=300 mg/l, hypertension, or a GFR <80 ml/min per 1.73 m(2) was 23%. Anuria of more than 7 days' duration and hypertension during the acute phase were statistically significant risk factors for an unfavorable outcome. A reduced ERPF in the 2nd year was found in 93% of patients with sequelae. Mean filtration fraction (SD) in these patients was 0. 26 (+/-0.07) versus 0.19 (+/-0.05) in patients without sequelae (P<0. 0001). These data suggest that loss of nephrons during the acute phase may implicate hyperfiltration in the residual functioning kidney mass leading to progressive renal disease. ERPF in the 2nd year after D+ HUS may serve as an excellent parameter to detect patients with a high risk of an unfavorable long-term outcome.

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    • "The long term renal prognosis of VTEC-associated HUS is controversial. Hüsemann et al (31) in 1999 reported a cohort of 127 of 149 children who had survived the acute phase of HUS. The clearance data revealed hemodynamic changes in half of the patients in the second year after HUS. "
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    ABSTRACT: This article describes the birth of the Canadian Pediatric Kidney Disease Research Centre (CPKDRC) in 1985 and the activities that have transpired as a result of collaborative research at paediatric centres across Canada. These include the National Retrospective Study of Childhood Hemolytic Uremic Syndrome (HUS), National Prospective Study of Risk Factors for Developing Escherichia coli O157:H7 Infection, and Intervention Studies for the Prevention of HUS. A look to the future describes possible studies to determine potential factors (surrogate markers) to identify children who are at risk for developing HUS following verotoxin-producing E coli gastroenteritis, other intervention studies and a more accurate understanding of permanent renal insufficiency in children who have had HUS.
    Preview · Article · Oct 2002 · Paediatrics & child health
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    ABSTRACT: Every year in France, about 100 children, most of them less than 3 years old, have typical diarrhea-associated HUS (D + HUS). Evidence of exposure to verotoxin producing E. coli (VTEC), mostly the O157: H7 serotype, is demonstrated in about 85% of cases. A prodromal illness of acute gastroenteritis with diarrhea, often bloody, precedes the HUS by 1 to 15 days. HUS onset is sudden, with the typical association of hemolytic anemia with fragmented red blood cells, thrombocytopenia and acute renal insufficiency. Involvement of other organs than the kidneys may occur, such as severe hemorragic colitis with rectal prolapse, bowell wall necrosis or secondary stenosis, acute pancreatitis, central nervous system involvement which determines the vital outcome. Early accurate supportive treatment allows a current mortality rate below 5%, with most deaths due to central nervous system involvement. Five to 10% of children develop end stage renal disease, rarely directly, more often after having recovered some renal function with chronic renal insufficiency during a few years. After 15 or more years follow-up, at least one third of patients have some degree of proteinuria and/or hypertension, and eventually chronic or end stage renal failure. Predictive features of poor renal outcome at the acute phase are severe gastrointestinal involvement, severe CNS involvement, polynucleosis over 20 000/mm3, and duration of initial anuria longer than one week. The role of VTEC in D + HUS makes the disease a public health problem. Preventive measures are essential.
    No preview · Article · Sep 2001 · Archives de Pédiatrie
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    ABSTRACT: The lipopolysaccharide (LPS) of enterohemorrhagic Escherichia coli (EHEC) and Shiga toxin together substantially contribute to the pathophysiology of typical hemolytic-uremic syndrome (HUS). Both factors have been shown to be immune stimulators and could play a key role in the individual innate immune response, characterized by proinflammatory and anti-inflammatory cytokines. By use of a whole blood stimulation model, we therefore compared the LPS- and superantigen-induced cytokine responses in children who had been having recovering from an acute episode of typical HUS for at least 6 months (group 1) with those in controls, who consisted of patients seen in the pediatric neurology outpatient department for routine examination (group 2). Samples were analyzed for cytokine protein levels and the levels of mRNA production. LPS stimulation revealed lower levels of interleukin 10 (IL-10) (P < 0.05) and increased levels of gamma interferon (P < 0.05) and increased ratios of pro- and anti-inflammatory cytokines (P < 0.05 for the IL-1beta/IL-10 ratio; P < 0.05 for the tumor necrosis factor alpha/IL-10 ratio) in group 1. In addition superantigen stimulation showed decreased IL-2 levels in group 1 (P < 0.01). Our results suggest an alteration of the cytokine response characterized by high proinflammatory cytokine levels and low anti-inflammatory cytokine levels as well as low levels of IL-2 production in children who have experienced an episode of typical HUS. We hypothesize that this altered immune response is not a residual effect of the infection but a preexisting characteristic of the patient. This could be one reason why individuals infected with EHEC are potentially predisposed to a systemic disease (HUS).
    Full-text · Article · Dec 2003 · Clinical and Diagnostic Laboratory Immunology
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