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AggiornAmenti in riAnimAzione e terApiA intensivA - Pacini Editore

AggiornAmenti in riAnimAzione e terApiA intensivA - Pacini Editore

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12<br />

thrombotic microangiopathy after kidney<br />

transplantation<br />

M. Noris<br />

G. Remuzzi<br />

Cl<strong>in</strong>ical Research Center<br />

for Rare Diseases<br />

“Aldo e Cele Daccò”,<br />

Mario Negri Institute<br />

for Pharmacological Research,<br />

Ranica, Bergamo, Italy<br />

Thrombotic microangiopathy (TMA) is a severe complication of kidney<br />

transplantation that often causes graft failure. TMA may occur de novo,<br />

often triggered by immunosuppressive drugs and acute antibody-mediated<br />

rejection, or recur <strong>in</strong> patients with previous history of hemolytic uremic<br />

syndrome (HUS). Recurrent TMA is very rare <strong>in</strong> patients who had developed<br />

end-stage renal failure follow<strong>in</strong>g HUS caused by Shiga-tox<strong>in</strong> produc<strong>in</strong>g<br />

Escherichia coli, whereas disease recurrence is common <strong>in</strong> patients<br />

with atypical HUS (aHUS). The underly<strong>in</strong>g genetic defect greatly impacts<br />

the risk of posttransplant recurrence <strong>in</strong> aHUS. Indeed recurrence is almost<br />

the rule <strong>in</strong> patients with mutations <strong>in</strong> genes encod<strong>in</strong>g factor H or factor<br />

I, whereas patients with a mutation <strong>in</strong> membrane-cofactor-prote<strong>in</strong> gene<br />

have a good transplant outcome. Prophylactic and therapeutic options<br />

for posttransplant TMA, <strong>in</strong>clud<strong>in</strong>g plasma therapy, comb<strong>in</strong>ed kidney and<br />

liver transplantation and targeted complement <strong>in</strong>hibitors are discussed <strong>in</strong><br />

this review.<br />

Am j Transplant 2010;10(7):1517-23

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