Atypical hemolytic uremic syndrome (atypical HUS) is characterized by endothelial injury and microvascular thrombosis resulting in microangiopathic hemolytic anemia, thrombocytopenia, and ischemic injury to organs, especially the kidney. Activation of complements is involved in the pathogenesis of atypical HUS. Eculizumab, a neutralizing monoclonal antibody directed against complement C5, has shown salutary effects in patients with atypical HUS. In this report, we present a 23-year-old man with atypical HUSwho was treated with eculizumab. During the first four weeks of treatment, eculizumab failed to achieve a remission. Microangiopathic hemolytic anemia and thrombocytopenia persisted, while renal function deteriorated necessitating initiation of hemodialysis. Continuation of eculizumab therapy, however, led to marked improvement in hemolytic anemia, thrombocytopenia, and renal function. After 10 weeks of eculizumab therapy, hemodialysis was discontinued. At 5-month follow-up, serum creatinine was 1.1 mg/dL with continued eculizumab therapy every other week. In addition, platelet count was normal, while there was no evidence of hemolysis. We conclude that in patients with persistent atypical HUS continued treatment with eculizumab can be helpful in achieving remission.
Renal artery stenosis (RAS) due to atherosclerosis continues to be a major cause of secondary hypertension. It can also lead to renal dysfunction due to ischemic nephropathy. While major clinical trials have emphasized that medical management should be preferred over angioplasty and stenting for the treatment of renal artery stenosis, clinical scenarios continue to raise doubts about the optimal management strategy. Herein, we present two cases that were admitted with hypertensive emergency and renal function deterioration. Medical therapy failed to control the blood pressure and in one patient, renal failure progressed to a point where renal replacement therapy was required. Both patients underwent angioplasty (for >90% stenosis) and stent insertion with successful resolution of stenosis by interventional radiology. Postoperatively, blood pressure gradually decreased with improvement in serum creatinine. Dialysis therapy was discontinued. At 4- and 8-month follow-up, both patients continue to do well with blood pressure readings in the 132-145/70-90 mmHg range. This article highlights the importance of percutaneous interventions in the management of atherosclerotic RAS and calls for heightened awareness and careful identification of candidates who would benefit from angioplasty and stent insertion.
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