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Abstract
BackgroundThrombotic microangiopathies (TMA) in adults such as thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS) are life-threatening disorders if untreated. Clinical presentation is highly variable and prognostic factors for clinical course and outcome are not well established.MethodsWe performed a retrospective observational study of 62 patients with TMA, 22 males and 40 females aged 16 to 76 years, treated with plasma exchange at one center to identify clinical risk factors for the development of renal insufficiency.ResultsOn admission, 39 of 62 patients (63%) had acute renal failure (ARF) with 32 patients (52%) requiring dialysis treatment. High systolic arterial pressure (SAP, p = 0.009) or mean arterial pressure (MAP, p = 0.027) on admission was associated with acute renal failure. Patients with SAP>140 mmHg on admission had a sevenfold increased risk of severe kidney disease (OR 7.464, CI 2.097–26.565). MAP>100 mmHg indicated a fourfold increased risk for acute renal failure (OR 4.261, CI 1.400–12.972). High SAP, diastolic arterial pressure (DAP), and MAP on admission were also independent risk factors for persistent renal insufficiency with the strongest correlation for high MAP. Moreover, a high C-reactive protein (CRP) level on admission correlated with renal failure in the course of the disease (p = 0.003). At discharge, renal function in 11 of 39 patients (28%) had fully recovered, 14 patients (23%) remained on dialysis, and 14 patients (23%) had non-dialysis-dependent chronic kidney disease. Seven patients (11%) died. We identified an older age as risk factor for death.ConclusionsHigh blood pressure as well as high CRP serum levels on admission are associated with renal insufficiency in TMA. High blood pressure on admission is also a strong predictor of sustained renal insufficiency. Thus, adult TMA patients with high blood pressure may require special attention to prevent persistent renal failure.
Highlights
Thrombotic microangiopathies (TMA) such as hemolytic uremic syndrome (HUS) or thrombocytopenic purpura (TTP) are rare microangiopathic thrombotic disorders characterized by hyaline thrombi in the arterial microvasculature, Coombs-negative hemolytic anemia, and thrombocytopenia
According to epidemiologic studies ADAMTS13 activity was reduced below 5% in only 15% of patients with TMA [9], suggesting the presence of additional unrecognized risk factors predisposing for the onset and clinical course of TMA
Most of our patients had no history of renal insufficiency prior to their first TMA episode
Summary
Thrombotic microangiopathies (TMA) such as HUS or TTP are rare microangiopathic thrombotic disorders characterized by hyaline thrombi in the arterial microvasculature, Coombs-negative hemolytic anemia, and thrombocytopenia. With the advent of plasma exchange in TMA mortality rate could be drastically decreased, but TMA can still be viewed as a life-threatening disorder with the outcome depending on the underlying disease, the age of the patient, severity of organ damage (e.g. kidney, heart, brain), and on the time lapse between the onset of symptoms and initiation of plasma therapy. According to epidemiologic studies ADAMTS13 activity was reduced below 5% in only 15% of patients with TMA [9], suggesting the presence of additional unrecognized risk factors predisposing for the onset and clinical course of TMA. Patients with atypical HUS may have a deficiency of complement factor H or auto-antibodies directed against this protein. Thrombotic microangiopathies (TMA) in adults such as thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS) are life-threatening disorders if untreated. Clinical presentation is highly variable and prognostic factors for clinical course and outcome are not well established
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