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Published on: May 7, 2012
Long-term follow-up of Czech children with D+ hemolytic-uremic syndrome
Kveta Bláhová1, Jan Janda, Jiri Kreisinger
1First Department of Pediatrics, Second Medical School, V Uvalu 84, 150 00, Prague 5, Motol, Czech Republic. blaha.martin@volny.cz
Insights
Children surviving diarrhea and hemolytic uremic syndrome (HUS) often experience long-term kidney damage. Early HUS onset improves prognosis, and specific human leukocyte antigen (HLA) types are associated with increased risk.
Area of Science:
- Nephrology
- Pediatrics
- Immunogenetics
Background:
- Diarrhea and hemolytic uremic syndrome (HUS) is a serious condition in children.
- Long-term sequelae, particularly renal damage, require further investigation.
Purpose of the Study:
- To evaluate the prevalence and nature of late renal damage in children who survived diarrhea and HUS.
- To identify factors influencing prognosis, including age of onset and human leukocyte antigen (HLA) associations.
Main Methods:
- Retrospective evaluation of 57 children who survived diarrhea and HUS, with follow-up 1-27 years post-illness.
- Classification of patients into recovery, residual renal symptoms, and chronic renal insufficiency/failure groups.
- Human leukocyte antigen (HLA) class I and II typing performed on 64 patients.
Main Results:
- A high prevalence of renal damage was observed in patients surviving more than 10 years post-HUS.
- Only 6 out of 18 long-term survivors had complete recovery; 7 had residual symptoms, and 5 had chronic renal insufficiency/failure.
- Early HUS onset (0-2 years) was associated with a significantly better prognosis (P=0.009).
- Significantly higher frequency of HLA DR9 antigen (P=0.0037) and lower frequency of DQ1 antigen (P=0.009) were found in D+HUS patients.
Conclusions:
- Survivors of diarrhea and HUS exhibit a high prevalence of late-onset renal damage.
- The presence of HLA DR9 haplotypes is significantly associated with D+HUS in the studied Czech population.
Unlabelled:
Fifty-seven children (f/m=31/26) who survived diarrhea (D) + hemolytic uremic syndrome (HUS) were evaluated. The examinations were performed 1-27 years (median 7 years) from the onset of the acute disease. Patients aged 2.3-27 years (median 10 years) were allocated to three groups: Recovery (R, complete recovery), Residual renal symptoms (RRS, hematuria and/or proteinuria and/or hypertension with glomerular filtration rate (GFR) >80 ml/min/1.73 m(2), or moderate renal insufficiency with slightly decreased GFR to 60-80 ml/min/1.73 m(2) with or without residual renal symptoms), and Chronic renal insufficiency/failure (CRI/F, dialysis, transplantation - GFR <60 ml/min/ 1.73 m(2)). Results from 18 patients who survived more than 10 years after HUS demonstrated a high prevalence of renal damage. Only 6/18 patients were in group R, 7/18 patients were in group RRS and 5/18 patients were in group CRI/F. An early onset of HUS (36 patients between 0 and 2 years) was associated with a better prognosis when compared with late onset (21 patients aged more than 2 years), P=0.009. Serology typing of Human leukocyte antigens (HLA) classes I and II in 64 patients revealed a significantly higher frequency of DR9 antigen ( P=0.0037) and a lower frequency of DQ1 antigen ( P=0.009) in D+HUS patients compared with healthy Czech blood donors.
Conclusion:
Our study demonstrates a high prevalence of late renal damage in Czech patients surviving after D+HUS. The HLA typing in our group revealed a significantly higher rate of HLA DR9 haplotypes in D+HUS patients.
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