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Long-term follow-up of Czech children with D plus hemolytic-uremic syndrome

Publication at First Faculty of Medicine, Faculty of Physical Education and Sport, Second Faculty of Medicine |
2002

Abstract

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.