Renal and neurological involvement in typical Shiga toxin-associated HUS.
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Renal and neurological involvement in typical Shiga toxin-associated HUS. / Trachtman, Howard; Austin, Catherine; von Lewinski, Maria; Stahl, Rolf A.K.
In: NAT REV NEPHROL, Vol. 8, No. 11, 11, 2012, p. 658-669.Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review
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TY - JOUR
T1 - Renal and neurological involvement in typical Shiga toxin-associated HUS.
AU - Trachtman, Howard
AU - Austin, Catherine
AU - von Lewinski, Maria
AU - Stahl, Rolf A.K.
PY - 2012
Y1 - 2012
N2 - Shiga toxin-producing Escherichia coli-associated haemolytic uraemic syndrome (STEC-HUS) is one of the most important causes of acute kidney injury in patients of all ages, especially in children. It can occur sporadically or in outbreaks. STEC-HUS is a systemic illness caused by toxin-mediated injury to the vascular endothelium and a generalized inflammatory response. The kidney and the brain are the two primary target organs. Nearly 40% of patients with STEC-HUS require at least temporary renal replacement therapy and up to 20% will have permanent residual kidney dysfunction. Neurological injury can be sudden and severe and is the most frequent cause of acute mortality in patients with STEC-HUS. Over the past 30 years, a wide range of inflammatory mediators have been linked to the pathogenesis of STEC-HUS and associated renal and neurological complications. Recently, evidence has accumulated that abnormal activation of the alternative pathway of complement occurs in patients with STEC-HUS. In the large outbreak of STEC-HUS caused by E. coli O104:H4 that occurred in Germany in May 2011, a large number of patients received eculizumab, a monoclonal antibody directed against C5, in an open-label manner. We describe the experience with eculizumab under these emergent circumstances at one large centre.
AB - Shiga toxin-producing Escherichia coli-associated haemolytic uraemic syndrome (STEC-HUS) is one of the most important causes of acute kidney injury in patients of all ages, especially in children. It can occur sporadically or in outbreaks. STEC-HUS is a systemic illness caused by toxin-mediated injury to the vascular endothelium and a generalized inflammatory response. The kidney and the brain are the two primary target organs. Nearly 40% of patients with STEC-HUS require at least temporary renal replacement therapy and up to 20% will have permanent residual kidney dysfunction. Neurological injury can be sudden and severe and is the most frequent cause of acute mortality in patients with STEC-HUS. Over the past 30 years, a wide range of inflammatory mediators have been linked to the pathogenesis of STEC-HUS and associated renal and neurological complications. Recently, evidence has accumulated that abnormal activation of the alternative pathway of complement occurs in patients with STEC-HUS. In the large outbreak of STEC-HUS caused by E. coli O104:H4 that occurred in Germany in May 2011, a large number of patients received eculizumab, a monoclonal antibody directed against C5, in an open-label manner. We describe the experience with eculizumab under these emergent circumstances at one large centre.
KW - Comorbidity
KW - Humans
KW - Germany/epidemiology
KW - Antibodies, Monoclonal, Humanized/therapeutic use
KW - Disease Outbreaks
KW - Plasma Exchange
KW - Shiga-Toxigenic Escherichia coli
KW - Acute Kidney Injury/complications/microbiology
KW - Brain/immunology/microbiology
KW - Brain Diseases/microbiology
KW - Complement Activation
KW - Complement Pathway, Alternative/immunology
KW - Gastroenteritis/complications/epidemiology
KW - Hemolytic-Uremic Syndrome/complications/diagnosis/epidemiology/immunology/microbiology/therapy
KW - Kidney/immunology/microbiology
KW - Nervous System Diseases/microbiology
KW - Renal Replacement Therapy
KW - Comorbidity
KW - Humans
KW - Germany/epidemiology
KW - Antibodies, Monoclonal, Humanized/therapeutic use
KW - Disease Outbreaks
KW - Plasma Exchange
KW - Shiga-Toxigenic Escherichia coli
KW - Acute Kidney Injury/complications/microbiology
KW - Brain/immunology/microbiology
KW - Brain Diseases/microbiology
KW - Complement Activation
KW - Complement Pathway, Alternative/immunology
KW - Gastroenteritis/complications/epidemiology
KW - Hemolytic-Uremic Syndrome/complications/diagnosis/epidemiology/immunology/microbiology/therapy
KW - Kidney/immunology/microbiology
KW - Nervous System Diseases/microbiology
KW - Renal Replacement Therapy
M3 - SCORING: Journal article
VL - 8
SP - 658
EP - 669
JO - NAT REV NEPHROL
JF - NAT REV NEPHROL
SN - 1759-5061
IS - 11
M1 - 11
ER -
