Recent advances in understanding the pathogenesis of the hemolytic uremic syndromes

• Published in: Pediatric nephrology (Berlin, West) Vol. 4; no. 3; pp. 276 - 283
• Main Authors: KAPLAN, B. S, CLEARY, G, OBRIG, T. G
• Format: Journal Article
• Language: English
• Published: Heidelberg Springer 01-05-1990
• Subjects: Bacterial Toxins; Biological and medical sciences; Child; Hemolytic-Uremic Syndrome - microbiology; Hemolytic-Uremic Syndrome - pathology; Humans; Medical sciences; Nephrology. Urinary tract diseases; Nephropathies. Renovascular diseases. Renal failure; Renal failure; Shiga Toxin 1; Shiga Toxin 2; Human; Endothelial cell; Urinary system disease; Hemolytic uremic syndrome; Pathophysiology; Acute; Escherichia coli; Etiopathogenesis; Cytotoxicity; Shigella dysenteriae; Hemopathy; Infection; Toxin; Renal failure; Bacteriosis; Bacteria; Enterobacteriaceae
• ISSN: 0931-041X; 1432-198X
• DOI: 10.1007/bf00857676

One of the requirements for an agent to cause hemolytic uremic syndrome (HUS) is its ability to injure endothelial cells. Shiga-like toxin (SLT) can do this. SLT is produced by Escherichia coli and Shigella dysenteriae serotype 1; both have been implicated as causes of typical HUS. Endothelial cells have receptors (GB3) for SLT and the toxin can inhibit eukaryotic protein synthesis, thereby causing cell death. Glomerular endothelial cell injury or death results in a decreased glomerular filtration rate and many of the perturbations seen in HUS. It is no longer certain that hemolysis is the result of a microangiopathy. Cell injury results in release of von Willebrand multimers; if these are ultra-large, thrombosis may ensue. There is also increasing evidence that neutrophils have a role in the pathogenesis of typical HUS. Streptococcus pneumoniae can also cause HUS and care must be taken to avoid giving plasma to patients with S. pneumoniae-associated HUS. There is compelling evidence that types of HUS are inherited by autosomal recessive and autosomal dominant modes. Patients with autosomal recessive HUS may have recurrent episodes. Mortality and morbidity rates are high for the inherited forms.