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Atypical HUS

Valentina Talarico, Monica Aloe, Alice Monzani, Roberto Miniero, Gianni Bona
Hemolytic uremic syndrome (HUS) is a thrombotic microangiopathy defined by thrombocytopenia, non-immune microangiopathic hemolytic anemia and acute renal failure. HUS is typically classified into two primary types: 1) HUS due to infections, often associated with diarrhea (D+HUS, Shiga toxin-producing Escherichia Coli-HUS), with the rare exception of HUS due to a severe disseminated infection caused by Streptococcus; 2) HUS related to complement, such HUS is also known as "atypical HUS" and is not diarrhea associated (D-HUS, aHUS); but recent studies have shown other forms of HUS, that can occur in the course of systemic diseases or physiopathological conditions such as pregnancy, after transplantation or after drug assumption...
December 2016: Minerva Pediatrica
Diana Karpman, Sebastian Loos, Ramesh Tati, Ida Arvidsson
Haemolytic uraemic syndrome (HUS) is defined by the simultaneous occurrence of nonimmune haemolytic anaemia, thrombocytopenia and acute renal failure. This leads to the pathological lesion termed thrombotic microangiopathy, which mainly affects the kidney, as well as other organs. HUS is associated with endothelial cell injury and platelet activation, although the underlying cause may differ. Most cases of HUS are associated with gastrointestinal infection with Shiga toxin-producing enterohaemorrhagic Escherichia coli (EHEC) strains...
October 10, 2016: Journal of Internal Medicine
Dineke Westra, Elena B Volokhina, Renate G van der Molen, Thea J A M van der Velden, Annelies Jeronimus-Klaasen, Joop Goertz, Valentina Gracchi, Eiske M Dorresteijn, Antonia H M Bouts, Mandy G Keijzer-Veen, Joanna A E van Wijk, Jaap A Bakker, Anja Roos, Lambert P van den Heuvel, Nicole C A J van de Kar
BACKGROUND: The role of complement in the atypical form of hemolytic uremic syndrome (aHUS) has been investigated extensively in recent years. As the HUS-associated bacteria Shiga-toxin-producing Escherichia coli (STEC) can evade the complement system, we hypothesized that complement dysregulation is also important in infection-induced HUS. METHODS: Serological profiles (C3, FH, FI, AP activity, C3d, C3bBbP, C3b/c, TCC, αFH) and genetic profiles (CFH, CFI, CD46, CFB, C3) of the alternative complement pathway were prospectively determined in the acute and convalescent phase of disease in children newly diagnosed with STEC-HUS or aHUS...
October 7, 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Kheir Eddine Kerboua, Fatma Haiba, Djamila Batouche
Treatment of atypical hemolytic uremic syndrome (aHUS) by the complement C5 inhibitor eculizumab (Soliris®) is highly effective but unfortunately, associated with an economic pressure on the health care systems even in high incomes countries. Despite spacing infusions has been proposed as the unique solution to minimize this economic impact, no reliable laboratory assays are available to tailor such therapy optimization. We aimed to propose and evaluate a complement composite marker for eculizumab efficacy monitoring in aHUS...
September 12, 2016: Journal of Immunoassay & Immunochemistry
Aude Servais, Nadège Devillard, Véronique Frémeaux-Bacchi, Aurélie Hummel, Laurent Salomon, Cécile Contin-Bordes, Hélène Gomer, Christophe Legendre, Yahsou Delmas
BACKGROUND: A therapeutic strategy based on complement blockade by eculizumab is widely used to treat atypical haemolytic uraemic syndrome (aHUS). Recent data are available on the administration of eculizumab during pregnancy in patients treated for paroxysmal nocturnal haemoglobinuria but there are very few data for aHUS patients. METHODS: We analysed the use of eculizumab for the treatment of aHUS during five pregnancies in three patients and studied an additional pregnancy without eculizumab...
September 1, 2016: Nephrology, Dialysis, Transplantation
A Mitchell, B Nokay, H Guberina, U Behlen-Wilm, M Nagel, M Kirschfink, T Wiech, A Kribben, A Bienholz
OBJECTIVE: Malignant hypertension as borne out by uncontrolled hypertension with accelerated target organ damage has a variable and often dire prognosis. Loss of kidney function is a hallmark of the condition and is frequently accompanied by thrombotic microangiopathy (TMA). DESIGN AND METHOD: Here we present the case of a patient with malignant hypertension, who was successfully treated using a combination of in-label and off-label medication according to the following pathophysiological concepts: 1) Innate immunity has been identified as a player in blood pressure (BP) control, 2) the complement inhibitor eculizumab has become available for treating atypical hemolytic uremic syndrome (aHUS), which shares many features of malignant hypertension, 3) endothelin (ET) antagonism, although not currently approved for these indications, has been shown to be effective for treating proteinuria and as a possible adjunct for the treatment of resistant hypertension...
September 2016: Journal of Hypertension
Anna Jin, Laleh Boroujerdi-Rad, Gaurang Shah, Joline L T Chen
Thrombotic microangiopathies (TMAs) include thrombotic thromobocytopenic purpura and hemolytic uremic syndrome (HUS). Among these conditions, atypical HUS is now recognized to be a disease of alternative complement pathway dysregulation. Eculizumab is a recombinant humanized monoclonal antibody that binds to the complement protein C5 and prevents the cleavage of C5 to C5a and C5b. Eculizumab has been used as a novel treatment for complement-mediated TMA. We present a case of a patient with human immunodeficiency virus infection who developed TMA and was successfully treated with eculizumab...
August 2016: Clinical Kidney Journal
Kiran P Sathe, Kumud P Mehta
The simultaneous presence of multiple immune-mediated diseases in a single host is rare. The implications of such coexistence relating to the disease pathogenesis and treatment are not well understood. We describe two cases of renal failure with immune-mediated overlap conditions. We believe, this is the first reported case of antineutrophil cytoplasmic antibody (ANCA) associated vasculitis coexisting with atypical hemolytic uremic syndrome (HUS). Two boys aged eight years (Case 1) and 10 years (Case 2) presenting with renal failure secondary to glomerulonephritis are described...
July 2016: Saudi Journal of Kidney Diseases and Transplantation
Tammy M Brady, Cozumel Pruette, Lauren F Loeffler, Darcy Weidemann, John J Strouse, Eleni Gavriilaki, Robert A Brodsky
The clinical manifestations of typical hemolytic uremic syndrome (HUS) encompass a wide spectrum. Despite the potentially severe sequelae from this syndrome, treatment approaches remain supportive. We present the clinical course of a child who contracted Shiga toxin-positive E. coli (STEC) from a daycare center during an outbreak. Utilizing the modified Ham test which is a rapid, serum-based functional assay used to detect activation of the alternative pathway of complement as observed in atypical HUS, patient sera revealed evidence of increased complement activation in the acute phase of the syndrome but not after resolution...
2016: J Clin Exp Nephrol
James M Gray, Rebecca K Ameduri
HUS is a well-known entity primarily associated with bacterial infection and is characterized by a classic triad of anemia, thrombocytopenia, and kidney injury. Its atypical form has been associated with calcineurin inhibitors and has been extensively discussed in renal transplantation. We present a case of tacrolimus-associated HUS in a pediatric heart transplant recipient, which we believe to be previously unreported in the literature.
September 2016: Pediatric Transplantation
Bodo B Beck, FrancJan van Spronsen, Arjan Diepstra, Rolf M F Berger, Martin Kömhoff
Methylmalonic aciduria and homocystinuria, cobalamin C (cblC) type, is the most common genetic type of functional cobalamin (vitamin B12) deficiency. This metabolic disease is characterized by marked heterogeneity of neurocognitive disease (microcephaly, seizures, developmental delay, ataxia, hypotonia) and variable extracentral nervous system involvement (failure to thrive, cardiovascular, renal, ocular) manifesting predominantly early in life, sometimes during gestation. To enhance awareness and understanding of renal disease associated with cblC defect, we studied biochemical, genetic, clinical, and histopathological data from 36 patients...
June 11, 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
M Gaggl, C Aigner, G Sunder-Plassmann, A Schmidt
Thrombotic microangiopathy (TMA) is a clinical syndrome that is characterized by hemolysis, thrombocytopenia, and acute kidney injury, known as atypical hemolytic syndrome (aHUS), thrombotic thrombocytopenic purpura (TTP), and shigatoxin-associated HUS (STEC-HUS) among others. Several diseases, like malignoma, infections, malignant hypertension, or autoimmune disease can result in secondary TMAs. aHUS is caused by a hyperactivated complement system. Identification of the underlying causes of the TMA is the most important issue and directly associated with treatment success...
June 2016: Medizinische Klinik, Intensivmedizin und Notfallmedizin
Kioa L Wijnsma, Sheila A M van Bommel, Thea van der Velden, Elena Volokhina, Michiel F Schreuder, Lambertus P van den Heuvel, Nicole C A J van de Kar
BACKGROUND: In the majority of pediatric patients, the hemolytic-uremic syndrome (HUS) is caused by an infection with Shiga toxin-producing Escherichia coli (STEC), mostly serotype O157. It is important to discriminate between HUS caused by STEC and complement-mediated HUS (atypical HUS) due to differences in treatment and outcome. As STEC and its toxins can only be detected in the patient's stool for a short period of time after disease onset, the infectious agent may go undetected using only fecal diagnostic tests...
November 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Thurid Ahlenstiel-Grunow, Svenja Hachmeister, Franz Christoph Bange, Cyrill Wehling, Michael Kirschfink, Carsten Bergmann, Lars Pape
BACKGROUND: In contrast to atypical haemolytic uraemic syndrome (aHUS), only single case reports and limited data have been published on systemic activation of the complement system and mutations in complement genes in paediatric enterohaemorrhagic Escherichia coli-induced HUS (EHEC-HUS). METHODS: Complement activation (CH50, APH50, C3d, sC5b-9) was analysed at four timepoints (Week 1, Week 2, Month 3 and Month 6 after primary diagnosis of HUS) in 25 children with EHEC-HUS...
July 2016: Nephrology, Dialysis, Transplantation
Alexander Åkesson, Anna M Blom, Jenny Klintman, Eva Zetterberg
BACKGROUND: Complement-mediated atypical hemolytic uremic syndrome (aHUS) is a rare disease with high mortality and morbidity if left untreated. The diagnostic work-up is complicated and the manifestations overlap with other conditions. Therefore, we hypothesize that complement-mediated aHUS is an underdiagnosed disease. METHODS: A cohort of 768 referrals referred to the Coagulation Unit in Malmo, Sweden, for analysis of a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13), 2007-2012, were retrospectively reviewed...
May 13, 2016: Nephrology
Fadi Fakhouri
Pregnancy is a high-risk period for various types of thrombotic microangiopathies (TMA). The improvement of our understanding of the pathophysiology of TMAs has translated into better management of pregnancy-related TMAs. The two main types of TMA, TTP (thrombotic thrombocytopenic purpura) and hemolytic uremic syndrome (HUS), can both occur during pregnancy and postpartum. TTP is related in most cases to acquired or congenital deficiency of ADAMTS13; it tends to develop mainly during the second and third trimesters of pregnancy...
April 2016: Transfusion and Apheresis Science
Shunichiro Yasuda, Masahide Yamamoto, Tetsuya Fukuda, Yasufumi Ohtsuka, Osamu Miura
Atypical hemolytic uremic syndrome (aHUS) is a rare type of HUS associated with dysregulation of the alternative complement pathway. We herein report the findings of a 43-year-old woman who developed aHUS two days after myomectomy. The serum C3 level was low and the sheep erythrocyte hemolytic assay showed a moderate hemolysis. The renal insufficiency progressed despite performing plasma exchange therapy, although a partial hematological response was observed. Thus, the patient was subsequently treated with the anti-C5 antibody eculizumab, which promptly improved and normalized the renal function...
2016: Internal Medicine
Prabesh Bajracharya, Amrish Jain, Rossana Baracco, Tej K Mattoo, Gaurav Kapur
BACKGROUND: Patients negative for Shiga toxin-producing E. coli (STEC) are categorized as having atypical hemolytic uremic syndrome (HUS) and are associated with an increased risk for complement mutations and poorer prognosis compared with typical HUS. However, STEC identification is limited by the natural history of HUS. METHODS: The current study is aimed at identifying HUS patients with poor outcomes based on the presence or absence of diarrhea (D) or Shiga toxin (S)...
October 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Lance A Williams, Marisa B Marques
OBJECTIVES: Pathologists specializing in transfusion medicine, apheresis medicine, and/or coagulation are often consulted by clinicians to reach a diagnosis for patients with thrombotic microangiopathy (TMA), so that disease-specific, often life-saving therapy can be initiated as promptly as possible. METHODS: This article describes how to proceed when treating a patient with TMA. The differential diagnosis is broad and potentially very challenging. Thrombotic thrombocytopenic purpura (TTP), atypical hemolytic uremic syndrome (aHUS), and typical hemolytic uremic syndrome (HUS) are three such TMAs that require timely diagnosis and treatment...
February 2016: American Journal of Clinical Pathology
Catherine Chaudoir, Menchu G Ong, Xin Gu, Diana Veillon, James Cotelingam
Atypical Hemolytic Uremic Syndrome is a triad of microangiopathic hemolytic anemia, thrombocytopenia and acute renal failure not associated with diarrhea. It is a rare condition associated with complement disorders in about 50 percent of cases. The first line of treatment is therapeutic plasma exchange. However, because clinical response to TPE varies, an anti-complement drug, eculizumab has been tried. We report a case of atypical HUS successfully treated with eculizumab.
January 2016: Journal of the Louisiana State Medical Society: Official Organ of the Louisiana State Medical Society
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