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.jpg): Failed to open stream: No such file or directory in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 117 J+Nephrol
2017 ; 30
(3
): 347-362
Nephropedia Template TP
gab.com Text
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Atypical hemolytic uremic syndrome in the setting of complement-amplifying
conditions: case reports and a review of the evidence for treatment with
eculizumab
#MMPMID27848226
Asif A
; Nayer A
; Haas CS
J Nephrol
2017[Jun]; 30
(3
): 347-362
PMID27848226
show ga
Atypical hemolytic uremic syndrome (aHUS) is a rare, genetic, progressive,
life-threatening form of thrombotic microangiopathy (TMA) predominantly caused by
dysregulation of the alternative pathway of the complement system.
Complement-amplifying conditions (CACs), including pregnancy complications
[preeclampsia, HELLP (hemolysis, elevated liver enzymes, low platelet count)
syndrome], malignant hypertension, autoimmune diseases, transplantation, and
others, are associated with the onset of TMA in up to 69?% of cases of aHUS. CACs
activate the alternative pathway of complement and may be comorbid with aHUS or
may unmask a previously undiagnosed case. In this review, three case reports are
presented illustrating the onset and diagnosis of aHUS in the setting of
different CACs (pregnancy complications, malignant hypertension, renal
transplantation). The report also reviews the evidence for a variety of CACs,
including those mentioned above as well as infections and drug-induced TMA, and
the overlap with aHUS. Finally, we introduce an algorithm for diagnosis and
treatment of aHUS in the setting of CACs. If TMA persists despite initial
management for the specific CAC, aHUS should be considered. The terminal
complement inhibitor eculizumab should be initiated for all patients with
confirmed diagnosis of aHUS, with or without a comorbid CAC.
|Adult
[MESH]
|Algorithms
[MESH]
|Antibodies, Monoclonal, Humanized/adverse effects/*therapeutic use
[MESH]