Thrombotic Microangiopathy and the Kidney

Brocklebank, Vicky; Wood, Katrina; Kavanagh, David

doi:10.2215/cjn.00620117

Cited by 288 publications

(341 citation statements)

References 130 publications

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“…Haemolytic uraemic syndrome (HUS) was previously the most common cause of acute kidney injury in childhood . It is characterised by microangiopathic haemolytic anaemia, thrombocytopenia and acute renal insufficiency . HUS has historically been classified into two forms: (i) typical following a diarrhoeal illness and (ii) atypical, which often has an inherited and/or acquired complement abnormality .…”

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confidence: 99%

“…HUS has historically been classified into two forms: (i) typical following a diarrhoeal illness and (ii) atypical, which often has an inherited and/or acquired complement abnormality . The classification of HUS is constantly evolving as greater understanding of the molecular basis of disease has been achieved …”

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confidence: 99%

“…The primary histopathological lesion in the kidney exhibits extensive glomerular capillary and arteriole endothelial cell damage with vascular lumina occlusion by microthrombi . This is the pathological description of thrombotic microangiopathy (TMA) . TMA can manifest with a diverse range of signs and symptoms; however, acute kidney injury is a prominent feature due to the propensity of glomerular circulation to endothelial damage and occlusion .…”

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confidence: 99%

“…TMA can manifest with a diverse range of signs and symptoms; however, acute kidney injury is a prominent feature due to the propensity of glomerular circulation to endothelial damage and occlusion . HUS caused by the shiga toxin‐producing Escherichia coli (most frequently E. coli serotype O157:H7) is defined by the presence of shiga toxin‐producing bacteria (STEC‐HUS) . There is evidence of complement activation via an alternate pathway in STEC‐HUS patients .…”

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“…There is evidence of complement activation via an alternate pathway in STEC‐HUS patients . HUS is also associated with Streptococcus pneumoniae …”

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confidence: 99%

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Follow‐up of children with infection‐associated haemolytic uraemic syndrome 1979–1995: Would eculizumab have improved prognosis?

Aldridge

Burke

2019

J Paediatrics Child Health

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The late outcome in 55 children with infection-mediated haemolytic uremic syndrome (Shiga Toxin E Coli (STEC)-HUS and pneumococcal HUS) observed in 1979-1995 was followed up 23 years after disease onset. Of these, two were later confirmed to have atypical HUS (aHUS). Furthermore, of this population, five children had impaired kidney function at 3-months follow-up, which continued to deteriorate. These children had significant oliguria/anuria and hypertension during their illness requiring early dialysis and antihypertensive therapy. At 23 years post-disease onset, all five (100%) of these children have developed end-stage kidney disease or chronic kidney disease. Eculizumab is a monoclonal antibody that binds with high affinity to the C5 protein of the complement pathway, a major component of the pathophysiology of infection-mediated HUS. There are no long-term randomised controlled trials in the literature to support its use in such cases. Our 23-year follow-up of a population of severely affected children with infection-mediated HUS demonstrates a high percentage of chronic kidney disease and end-stage kidney disease (19%). Randomised controlled trials with eculizumab are now being conducted in this affected cohort.

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“…There is evidence of complement activation via an alternate pathway in STEC‐HUS patients . HUS is also associated with Streptococcus pneumoniae …”

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confidence: 99%