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.
Abstract

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