ABSTRACT
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.
ABSTRACT
BACKGROUND: Acute kidney injury (AKI) occurs commonly in the setting of orthotopic liver transplantation (OLT). To date, the correlation between AKI post-OLT and pre-operative changes in renal function has not been rigorously examined. METHODS: To determine the impact of pre-OLT changes in renal function on AKI post-OLT, as well as to identify risk factors for AKI, we analyzed the prospectively maintained NIDDK Liver Transplantation Database, which includes patients who received their first OLT between April 15, 1990, and June 30, 1994. We used the AKI Network definition of AKI. RESULTS: Surprisingly, univariate analysis revealed that worsening renal function while awaiting OLT was protective to the development of AKI post-OLT. Independent predictors of AKI were increased body mass index, increased Childs-Pugh-Turcott score, decreased urine output during cross-clamp, improved renal function while awaiting OLT, increased post-operative stroke volume, non-Caucasian race, and post-operative use of tacrolimus. CONCLUSIONS: The correlation between improving renal function pre-OLT and AKI post-OLT may represent true protection (via ischemic pre-conditioning) or, alternatively, a masking of milder forms of AKI (via improved renal perfusion through correction of the cirrhotic milieu). These results highlight the complex interaction between liver and kidney disease, and suggest that not only the etiology but also the course of pre-OLT renal dysfunction may be a critical determinant of renal function post-OLT.
