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

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 m², or moderate renal insufficiency with slightly decreased GFR to 60–80 ml/min/1.73 m² with or without residual renal symptoms), and Chronic renal insufficiency/failure (CRI/F, dialysis, transplantation – GFR <60 ml/min/ 1.73 m²). 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.