This case report describes how eculizumab reversed neurologic impairment and improved renal damage in severe atypical hemolytic uremic syndrome. A 50-year-old female, after presenting with diarrhea and abdominal pain, developed pancolitis, acute renal failure, and thrombocytopenia. The patient underwent total abdominal colectomy. Pathology confirmed ischemic colitis with scattered mesenteric mi...

BACKGROUND The incidence of gemcitabine-induced hemolytic uremic syndrome has already been described in adults. Several approaches have been employed in the treatment of gemcitabine-induced hemolytic uremic syndrome with different outcomes. One of the most promising agents is eculizumab, which is a monoclonal antibody directed against C5 complement protein. CASE PRESENTATION We reported the c...

Atypical hemolytic uremic syndrome (HUS) is a rare disorder in children, therefore it may lead to misdiagnosis, delay in treatment, or acute kidney injury. Patients with atypical HUS present signs and symptoms of hemolytic anemia, thrombocytopenia, and higher lactate dehydrogenase and uric acid levels. The main risk factor highlighted is consanguinity, gene mutations, and viral infections. Ecul...

Thrombotic microangiopathy (TMA) describes a pathological process of microvascular thrombosis, consumptive thrombocytopenia, and microangiopathic hemolytic anemia, leading to end-organ ischemia and infarction, affecting particularly the kidney and brain. TMA is a pathological feature of a number of clinical disorders including thrombotic thrombocytopenic purpura, hemolytic uremic syndrome, and ...

Atypical hemolytic uremic syndrome (aHUS) is a thrombotic microangiopathy (TMA) characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. Most cases of aHUS are caused by uncontrolled complement activation due to genetic mutations in the alternative pathway of complement. More recently, mutations in the gene of coagulation system have also been identified in...

150 words) and a brief biographical sketch. Synopses: Submit concise reviews of infectious diseases or closely related topics. Preference will be given to reviews of new and emerging diseases; however, timely updates of other diseases or topics are also welcome. Synopses should be approximately 3,500 words and should include references, not to exceed 40. The section should begin with an introdu...

Hemolytic uremic syndrome (HUS) is a triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. The atypical form of HUS is a disease characterized by complement overactivation. Inherited defects in complement genes and acquired autoantibodies against complement regulatory proteins have been described. Incomplete penetrance of mutations in all predisposing genes is r...

below), the emphasis for differentiating HUS from TTP has shifted from the pattern of clinical involvement to the presence or absence of the enzyme deficiency. It is now suggested that the diagnosis of TTP should be used to describe only those patients who have this enzyme deficiency; while patients without the enzyme deficiency should be labeled as suffering from HUS.4 ETIOLOGY AND PATHOGENESI...

Paroxysmal nocturnal hemoglobinuria and atypical hemolytic uremic syndrome are diseases of excess activation of the alternative pathway of complement that are treated with eculizumab, a humanized monoclonal antibody against the terminal complement component C5. Eculizumab must be administered intravenously, and moreover some patients with paroxysmal nocturnal hemoglobinuria on eculizumab have s...

CONTEXT Hemolytic uremic syndrome (HUS) is a severe acute disease, sometimes with long-term sequelae. The diarrhoea-unrelated forms are particularly associated with a poor prognosis. The aim of this paper is to review current evidence regarding etiology and management, and explore methods by which the outcome may be optimized. EVIDENCE ACQUISITION An internet search of Medline, Medscape, MDCo...