Copyright ©2013 Baishideng Publishing Group Co.
World J Nephrol. Aug 6, 2013; 2(3): 56-76
Published online Aug 6, 2013. doi: 10.5527/wjn.v2.i3.56
Table 1 Classification of Hemolytic uremic syndrome, thrombotic thrombocytopenic purpura and related disorders
Etiology identifiedEtiology unknown
HUS infection-inducedHIV infection
(1) Shiga and shiga-like toxin producing bacteria, Enterohemorrhagic Escherichia Coli, Shigella dysenteriae type 1 (2) Streptococcus pneumoniae
HUS induced by disorders of complement regulationMalignancy, cancer chemotherapy, ionizing radiation
(1) Genetic (2) Acquired
ADAMTS13 deficiency (1) Genetic (2) AcquiredCalcineurin inhibitors and transplantation
Defective cobalamin metabolismPregnancy, HELLP syndrome, contraceptive pill
Systemic lupus erythematosus, anti-phospholipid antibody syndrome
Drug induced
Table 2 Development of Shiga-toxin-associated hemolytic uremic syndrome
Pathogenetic stepsClinical
Ingestion of Escherichia Coli through contaminated food or patient to patient transmission or transmission from animal to manColonization of the gut
DiarrhoeaLocal tissue damage
Bloody diarrhoeaSystemic toxinemia
Generation of host cytokines and chemokinesEndothelia cell damage, activation of local thrombosis in kidneys and in other organs
Renal involvement→ HUSDamage to glomerular endothelial cells, arteriolar damage, mesangial cell activation/damage, podocyte injury, tubular damage
Renal InsufficiencyAcute renal failure
Chronic renal failure
Arterial hypertension, proteinuria, hematuria
Table 3 Gene mutation rate of different complement factors according r-hemolytic uremic syndrome registries data
AbnormalityGene locusAtypical HUS rate
Factor HCFH (RCA:1q32)11%-29%
Membrane co-factor proteinMCP (RCA)3%-17%
Factor ICFI (4q25)2%-17%
C3C3 (19p13)2%-17%
Factor BCFB (6p21)0%-5%
ThrombomodulinTHBD (20p11)0%-5%
Anti factor H autoantibodies4%-13%
Table 4 Main clinical characteristics of patients with atypical hemolytic uremic syndrome according complement abnormality
GeneAtypical HUS incidenceRisk of death or renal failure within 1 yrRisk of relapseRelapse after transplantationPlasma-therapy indication
MCP5%-15%0%-6%70%-90%< 20%???
Anti-CFH Ab6%30%-40%40%-60%YesYes