Therapeutic Apheresis in the Treatment of Hemolytic Uremic Syndrome in View of Pathophysiological Aspects

Bambauer, Rolf; Latza, Reinhard; Schiel, Ralf

doi:10.1111/j.1744-9987.2010.00903.x

Cited by 15 publications

(18 citation statements)

References 84 publications

(100 reference statements)

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“…Plasmapheresis with fresh frozen plasma is reserved for the most severe cases 10 , although it was proven ineffective in some recent controlled clinical trials 11 . Platelet transfusions are avoided and limited for control of active bleeding, considering some studies have suggested it could contribute to the microthrombosis and worsen outcome 4 .…”

Section: Discussionmentioning

confidence: 99%

Case Report: Severe form of hemolytic-uremic syndrome with multiple organ failure in a child: a case report

2014

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Introduction: Hemolytic-uremic syndrome (HUS) is a leading cause of acute renal failure in infants and young children. It is traditionally defined as a triad of acute renal failure, hemolytic anemia and thrombocytopenia that occur within a week after prodromal hemorrhagic enterocolitis. Severe cases can also be presented by acute respiratory distress syndrome (ARDS), toxic megacolon with ileus, pancreatitis, central nervous system (CNS) disorders and multiple organ failure (MOF). Case presentation: A previously healthy 4-year old Caucasian girl developed acute renal failure, thrombocytopenia and hemolytic anemia following a short episode of abdominal pain and bloody diarrhea. By the end of the first week the diagnosis of the typical HUS was established. During the second week the disease progressed into MOF that included ileus, pancreatitis, hepatitis, coma and ARDS, accompanied by hemodynamic instability and extreme leukocytosis. Nonetheless, the girl made a complete recovery after one month of the disease. She was successfully treated in the intensive care unit and significant improvement was noticed after plasmapheresis and continuous veno-venous hemodialysis. Conclusions: Early start of plasmapheresis and meticulous supportive treatment in the intensive care unit, including renal placement therapy, may be the therapy of choice in severe cases of HUS presented by MOF. Monitoring of prognostic factors is important for early performance of appropriate diagnostic and therapeutical interventions.

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Section: Discussionmentioning

confidence: 99%

Case Report: Severe form of hemolytic-uremic syndrome with multiple organ failure in a child: a case report

2014

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“…It is postulated that some unknown substances from placenta, or the bioincompatibility of the fetus triggers ADAMST 13, which finally homed by fibrinogen deposition, and consequently acts as the deleterious factor in the vessel. This devastating effect can cause organ damage and certainly harm red blood cells, leading to severe hemolysis and then hemolytic uremic syndrome (22,23). The treatment selected was DFPP using the plasma fractionator EC‐20A or EC‐30A, in order to clear fibrinogen, which had a molecular weight 370 kDa.…”

Section: Discussionmentioning

confidence: 99%

“…The most important point was the rapidity of fibrinogen deposition in vascular endothelium, which is one of the most life threatening pathogenic factors that cause generalized vascular occlusion, ischemic process, and finally organ failure. Therefore, the point of clearing fibrinogen as fast as possible is the target of DFPP therapy (23,24), which also is very new in toxemia of pregnancy. These kinds of patients have been lost for years without any rescue treatment, which is so severe and always life‐threatening.…”

Section: Discussionmentioning

confidence: 99%

Double Filtration Plasmapheresis in Different Diseases in Thailand

et al. 2012

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Double filtration plasmapheresis (DFPP) was applied to the treatment of two different categories from 100 cases that had been collected over a 5 year period (2007-2011). These categories were allocated into groups by size of toxic substances, which were classified as two different kinds of diseases. Group I comprised diseases that were caused by alloimmunity in transplantation, autoimmune diseases, complicated nephrotic syndrome, pure red cell aplasia, and toxemia of pregnancy. This group was treated with a plasma separator (plasmaflow-05, Asahi Kasei) and plasma fractionators, EC-20W. The second group, which included hyperviscosity syndrome, was treated by the same plasma separator, but with different plasma fractionators using EC-40W. This group included diabetes nephropathy, hyperlipidemia, peripheral arterial diseases, and neurosensory hearing loss. Both groups used 1.5 plasma volumes in each treatment for three sessions in two consecutive weeks. The result of treatment in group I showed that plasma immunoglobulin G (IgG) was decreased substantially by 66% in either transplant or lupus nephritis patients after the third session. In the second group, IgM, fibrinogen, and lipid markedly responded to the treatment. Two diabetes nephropathy patients showed stable renal function for more than 12 months. Peripheral arterial disease was shown to benefit from significantly decreasing fibrinogen and IgM, which resulted in clinical tissue oxygenation. Neither bleeding diathesis nor membrane anaphylaxis were reported from the treatment. In summary, apheresis patients were shown to benefit in hypersensitized and hyperviscosity syndrome.

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“…The etiology and pathogenesis of HUS are not completely understood, and the therapy of HUS is complicated. The pathophysiologic aspects of the different pathogenic types of HUS and the therapeutic modalities are discussed by Bambauer et al .…”

Section: Hemolytic‐uremic Syndromementioning

confidence: 99%

Therapeutic Apheresis in Immunologic Renal and Neurological Diseases

Bambauer¹,

Latza²,

Burgard³

et al. 2017

Ther Apher Dial

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Since the mid 1970s, when membrane modules became available, plasma separation techniques have gained in importance especially in the past few years. The advantages of this method are a complete separation of the corpuscular components from the plasma and due to increased blood flow rate and higher efficacy. Systemic autoimmune diseases based on an immune pathogenesis produce autoantibodies and circulating immune complexes, which cause inflammation in the tissues of various organs. In most cases, these diseases have a poor prognosis without treatment. Therapeutic apheresis (TA) in combination with immunosuppressive therapies has led to a steady increase in survival rates over the last 40 years. The updated information on immunology and molecular biology of different immunologic diseases are discussed in relation to the rationale for apheresis therapy and its place in combination with other modern treatments. The different diseases can be treated by various apheresis methods such as therapeutic plasma exchange (TPE) with substitution solution, or with online plasma or blood purification using adsorption columns, which contain biological or non-biological agents. Here, the authors provide an overview of the most important pathogenic aspects indicating that TA can be a supportive therapy in systemic autoimmune diseases such as renal and neurological disorders. For the immunological diseases that can be treated with TA, the guidelines of the German Working Group of Clinical Nephrology and of the Apheresis Committee of the American Society for Apheresis are cited.

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Therapeutic Apheresis in the Treatment of Hemolytic Uremic Syndrome in View of Pathophysiological Aspects

Cited by 15 publications

References 84 publications

Case Report: Severe form of hemolytic-uremic syndrome with multiple organ failure in a child: a case report

Case Report: Severe form of hemolytic-uremic syndrome with multiple organ failure in a child: a case report

Double Filtration Plasmapheresis in Different Diseases in Thailand

Therapeutic Apheresis in Immunologic Renal and Neurological Diseases

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