Uremic Syndrome

BackgroundThe aim of this study was to investigate the histopathological findings in kidney biopsies in children with atypical hemolytic uremic syndrome (aHUS) and to determine whether specific pathological findings in aHUS have a prognostic value.MethodsRenal biopsy specimens of 29 patients who were recorded in the national Turkish aHUS registry database were available for review. Histopathological findings were compared with the clinical and laboratory features at the presentation and the final outcome.ResultsThe mean age at presentation and follow-up period was 4.9 +/- 3.9 and 3.9 +/- 3.0 years, respectively. The median time interval from the first symptom to biopsy was 10 days. Vascular thrombosis and interstitial fibrosis were significantly related to chronic kidney disease (CKD) requiring dialysis or kidney transplantation during follow-up (5.6-fold, for both). Glomerular necrosis, cortical necrosis, and glomerular sclerosis were markedly associated with CKD without dialysis (6.2-fold, 13.3-fold, and 8.8-fold, respectively). However, presence of endothelial swelling, subendothelial widening, and fragmented erythrocytes was found to be correlated with a favorable final outcome.ConclusionsPresence of vascular thrombosis, cortical necrosis, and glomerular sclerosis in histopathological evaluation correlated with developing CKD. Chronic changes in the interstitial compartment were also related to poor prognosis, a finding that has been shown for the first time in pediatric aHUS cases.C1 [Yuksel, Selcuk] Pamukkale Univ, Dept Pediat Nephrol, Sch Med, Mavi Bina 1 Kat, TR-20070 Denizli, Turkey.[Gonul, Ipek Isik] Gazi Univ, Dept Pathol, Sch Med, Ankara, Turkey.[Canpolat, Nur] Istanbul Univ, Dept Pediat Nephrol, Cerrahpa Fac Med, Istanbul, Turkey.[Gokce, Ibrahim] Marmara Univ, Dept Pediat Nephrol, Sch Med, Istanbul, Turkey.[Ozlu, Sare Gulfem] Minist Hlth, Dept Pediat Nephrol, Sami Ulus Children Hosp, Ankara, Turkey.[Ozcakar, Zeynep Birsin] Ankara Univ, Dept Pediat Nephrol, Sch Med, Ankara, Turkey.[Ozaltin, Fatih] Hacettepe Univ, Dept Pediat Nephrol, Sch Med, Ankara, Turkey.[Ozaltin, Fatih] Hacettepe Univ, Nephrogenet Lab, Sch Med, Ankara, Turkey.[Soylemezoglu, Oguz] Gazi Univ, Dept Pediat Nephrol, Sch Med, Ankara, Turkey