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2018 ; 8
(1
): 25-34
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C3 Glomerulopathy and Atypical Hemolytic Uremic Syndrome: Two Important
Manifestations of Complement System Dysfunction
#MMPMID29594148
Bajwa R
; DePalma JA
; Khan T
; Cheema A
; Kalathil SA
; Hossain MA
; Haroon A
; Madhurima A
; Zheng M
; Nayer A
; Asif A
Case Rep Nephrol Dial
2018[Jan]; 8
(1
): 25-34
PMID29594148
show ga
The advances in our understanding of the alternative pathway have emphasized that
uncontrolled hyperactivity of this pathway causes 2 distinct disorders that
adversely impact the kidney. In the so-called atypical hemolytic uremic syndrome
(aHUS), renal dysfunction occurs along with thrombocytopenia, anemia, and target
organ injury to multiple organs, most commonly the kidney. On the other hand, in
the so-termed C3 glomerulopathy, kidney involvement is not associated with
thrombocytopenia, anemia, or other system involvement. In this report, we present
2 cases of alternative pathway dysfunction. The 60-year-old female patient had
biopsy-proven C3 glomerulopathy, while the 32-year-old female patient was
diagnosed with aHUS based on renal dysfunction, thrombocytopenia, anemia, and
normal ADAMTS-13 level. The aHUS patient was successfully treated with the
monoclonal antibody (eculizumab) for complement blockade. The patient with C3
glomerulopathy did not receive the monoclonal antibody. In this patient,
management focused on blood pressure and proteinuria control with an
angiotensin-converting enzyme inhibitor. This article focuses on the clinical
differences, pathophysiology, and treatment of aHUS and C3 glomerulopathy.