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


CONCLUSIONS
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.
Bláhová
Kveta
K
First Department of Pediatrics, Second Medical School, V Uvalu 84, 150 00, Prague 5, Motol, Czech Republic. blaha.martin@volny.cz
Janda
Jan
J
Kreisinger
Jiri
J
Matejková
Eva
E
Sedivá
Anna
A
D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D002648:Child; D002675:Child, Preschool; D018153:Czech Republic; D003967:Diarrhea / Q000209:etiology*; D005260:Female; D005500:Follow-Up Studies; D006683:HLA-DQ Antigens / Q000032:analysis; D006684:HLA-DR Antigens / Q000032:analysis; D059810:HLA-DR Serological Subtypes; D006463:Hemolytic-Uremic Syndrome / Q000150:complications* / Q000453:epidemiology; D006801:Humans; D007674:Kidney Diseases / Q000453:epidemiology / Q000209:etiology; D007676:Kidney Failure, Chronic / Q000453:epidemiology / Q000209:etiology; D008297:Male; D015995:Prevalence; D011379:Prognosis; D012016:Reference Values
D006683:HLA-DQ Antigens; C051568:HLA-DQ1 antigen; D006684:HLA-DR Antigens; D059810:HLA-DR Serological Subtypes; C038988:HLA-DR9 antigen