title: Prognosis and pathological characteristics of five children with non-Shiga toxin-mediated hemolytic uremic syndrome.
abstract: BACKGROUND
  The three major signs of hemolytic uremic syndrome (HUS) are hemolytic anemia, thrombopenia and acute renal failure. HUS is classified into Shiga toxin-mediated HUS (Stx-HUS) and non-Shiga toxin-mediated HUS (nStx-HUS). The prognosis of nStx-HUS is reported to be less favorable than that of Stx-HUS. Although the association between the prognosis and pathological characteristics of HUS have been reported such that the prognosis was considered to be poor for thrombotic microangiopathy (TMA) with predominant arterial involvement (arterial TMA), good for TMA with predominant glomerular involvement (glomerular TMA) and dependent on the extent of necrosis in cases of renal cortical necrosis, it is not yet clear whether pathological findings are also related to the renal prognosis of nStx-HUS cases. Therefore the purpose of the present paper was to analyze renal biopsy findings and prognosis for five children with nStx-HUS.
  
  
  METHODS
  Clinical records of five cases of nStx-HUS among 74 cases of diagnosed HUS were reviewed, and information and data were summarized.
  
  
  RESULTS
  Histological examination of the kidney led to the diagnosis of arterial TMA in three cases, and glomerular TMA and severe renal cortical necrosis in one case each. Analysis of the relationship between renal histological findings and the prognosis found that three patients with arterial TMA and one patient with severe renal cortical necrosis later developed end-stage renal failure while one patient with glomerular TMA has continued to show normal renal function.
  
  
  CONCLUSIONS
  These findings indicate that pathological findings are closely related to the prognosis in cases of nStx-HUS.
authors: Department of Pediatrics, Kobe University Graduate School of Medicine, Kobe, Japan. ichirok@estate.ocn.ne.jp
authors: Ichiro
authors: I
authors: Kamioka
authors: Kandai
authors: K
authors: Nozu
authors: Teruo
authors: T
authors: Fujita
authors: Hiroshi
authors: H
authors: Kaito
authors: Ryojiro
authors: R
authors: Tanaka
authors: Kunihiko
authors: K
authors: Yoshiya
authors: Kazumoto
authors: K
authors: Iijima
authors: Koichi
authors: K
authors: Nakanishi
authors: Norishige
authors: N
authors: Yoshikawa
authors: Masafumi
authors: M
authors: Matsuo
mesh_terms: D000328:Adult; D002169:Campylobacter Infections / Q000150:complications / Q000473:pathology; D002648:Child; D005260:Female; D006463:Hemolytic-Uremic Syndrome / Q000150:complications / Q000382:microbiology / Q000473:pathology*; D006801:Humans; D007223:Infant; D007668:Kidney / Q000473:pathology; D007673:Kidney Cortex Necrosis / Q000209:etiology / Q000473:pathology; D007676:Kidney Failure, Chronic / Q000209:etiology / Q000473:pathology; D007678:Kidney Glomerulus / Q000473:pathology; D008297:Male; D008833:Microcirculation; D011379:Prognosis; D012008:Recurrence; D013927:Thrombosis / Q000209:etiology / Q000473:pathology