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Atypical Hemolytic–Uremic Syndrome

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https://www.readbyqxmd.com/read/29152520/a-case-of-escherichia-coli-hemolytic-uremic-syndrome-in-a-10-year-old-male-with-severe-neurologic-involvement-successfully-treated-with-eculizumab
#1
Malia Rasa, James Musgrave, Keith Abe, Len Tanaka, Konstantine Xoinis, Bruce Shiramizu, Gretchen Foskett, Rhiana Lau
Hemolytic uremic syndrome (HUS) can be classified as typical and atypical, and the treatment recommendations currently differ between the 2 types. Eculizumab is recommended as first-line treatment for atypical HUS; however, its use in typical HUS has been controversial. We report a case of a 10-year-old male with severe neurologic impairment who was successfully treated with eculizumab, which was started 4 days after onset of neurologic symptoms. Our case supports the use of eculizumab in typical HUS with neurologic involvement, even when given later in the course, as the pathophysiology of typical HUS has been shown to involve activation of the complement pathway, similar to atypical HUS...
October 2017: Journal of Investigative Medicine High Impact Case Reports
https://www.readbyqxmd.com/read/29151252/dominant-c3-glomerulopathy-new-roles-for-an-old-actor-in-renal-pathology
#2
REVIEW
Nicola Pirozzi, Antonella Stoppacciaro, Paolo Menè
Recently, a number of reports have described dominant C3 deposits in renal biopsies of patients with infection-related glomerulonephritis (GN). While acute post-infectious GN and membranoproliferative GN are commonly characterized by immune deposits containing C3 and/or C4, the absence of immunoglobulin (Ig) and/or immune complexes at light or electron microscopy is a rather unusual observation. Dominant C3 deposition is believed to result from the alternative pathway of complement activation via the C3bBb "tickover" convertase...
November 18, 2017: Journal of Nephrology
https://www.readbyqxmd.com/read/29148534/genetic-testing-of-complement-and-coagulation-pathways-in-patients-with-severe-hypertension-and-renal-microangiopathy
#3
Christopher P Larsen, Jon D Wilson, Alejandro Best-Rocha, Marjorie L Beggs, Randolph A Hennigar
A diagnosis of thrombotic microangiopathy on kidney biopsy in a patient presenting with hypertensive emergency has historically elicited the diagnosis of malignant hypertension-associated thrombotic microangiopathy. Recent studies, however, have raised awareness that a number of these patients may actually represent atypical hemolytic uremic syndrome. To further investigate this premise, we performed next-generation sequencing to interrogate the coding regions of 29 complement and coagulation cascade genes associated with atypical hemolytic uremic syndrome in 100 non-elderly patients presenting with severe hypertension, renal failure and a kidney biopsy showing microangiopathic changes limited to the classic accelerated hypertension-associated lesion of arterial intimal edema ('mucoid intimal hyperplasia') in isolation and without accompanying glomerular microthrombi...
November 17, 2017: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/29142942/complement-activation-induces-neutrophil-adhesion-and-neutrophil-platelet-aggregate-formation-on-vascular-endothelial-cells
#4
Magdalena Riedl, Damien G Noone, Meraj A Khan, Fred G Pluthero, Walter H A Kahr, Nades Palaniyar, Christoph Licht
Introduction: Atypical hemolytic uremic syndrome is a thrombotic microangiopathy, which is linked to hereditary or autoimmune defects in complement activators or regulators present in blood and on vascular endothelial cells. Acute thrombotic microangiopathy episodes are typically preceded by infections, which by themselves would not be expected to manifest HUS. Thus, it is possible that the host immune response contributes to the precipitation of aHUS. However, the mechanisms involved are not fully understood...
January 2017: KI Reports
https://www.readbyqxmd.com/read/29136640/long-term-outcomes-of-the-atypical-hemolytic-uremic-syndrome-after-kidney-transplantation-treated-with-eculizumab-as-first-choice
#5
Luis Gustavo Modelli de Andrade, Mariana Moraes Contti, Hong Si Nga, Ariane Moyses Bravin, Henrique Mochida Takase, Rosa Marlene Viero, Trycia Nunes da Silva, Kelem De Nardi Chagas, Lilian Monteiro Pereira Palma
INTRODUCTION: The treatment of choice for Atypical Hemolytic Uremic Syndrome (aHUS) is the monoclonal antibody eculizumab. The objective of this study was to assess the efficacy and safety of eculizumab in a cohort of kidney transplant patients suffering from aHUS. METHODS: Description of the prospective cohort of all the patients primarily treated with eculizumab after transplantation and divided into the therapeutic (onset of aHUS after transplantation) and prophylactic use (patients with previous diagnosis of aHUS undergoing kidney transplantation)...
2017: PloS One
https://www.readbyqxmd.com/read/29071054/enterococcus-raffinosus-infection-with-atypical-hemolytic-uremic-syndrome-in-a-multiple-myeloma-patient-after-autologous-stem-cell-transplant
#6
Pankaj Mathur, Blake Hollowoa, Nupur Lala, Sharmilan Thanendrarajan, Aasiya Matin, Atul Kothari, Carolina Schinke
No abstract text is available yet for this article.
September 26, 2017: Hematology Reports
https://www.readbyqxmd.com/read/29068997/atypical-hemolytic-uremic-syndrome-induced-by-cblc-subtype-of-methylmalonic-academia-a-case-report-and-literature-review
#7
REVIEW
Minguang Chen, Jieqiu Zhuang, JianHuan Yang, Dexuan Wang, Qing Yang
RATIONALE: Methylmalonic acidemia (MMA) is a common organic acidemia, mainly due to methylmalonyl-CoA mutase (MCM) or its coenzyme cobalamin (VitB12) metabolic disorders. Cobalamin C (CblC) type is the most frequent inborn error of cobalamin metabolism; it can develop symptoms in childhood and often combine multisystem damage, which leads to methylmalonic acid, propionic acid, methyl citrate, and other metabolites abnormal accumulation, causing nerve, liver, kidney, bone marrow, and other organ damage...
October 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29058539/belgian-consensus-statement-on-the-diagnosis-and-management-of-patients-with-atypical-hemolytic-uremic-syndrome
#8
Kathleen J Claes, Annick Massart, Laure Collard, Laurent Weekers, Eric Goffin, Jean-Michel Pochet, Karin Dahan, Johann Morelle, Brigitte Adams, Nilufer Broeders, Patrick Stordeur, Daniel Abramowicz, Jean-Louis Bosmans, Koen Van Hoeck, Peter Janssens, Lissa Pipeleers, Patrick Peeters, Steven Van Laecke, Elena Levtchenko, Ben Sprangers, Lambertus van den Heuvel, Nathalie Godefroid, Johan Van de Walle
No abstract text is available yet for this article.
October 23, 2017: Acta Clinica Belgica
https://www.readbyqxmd.com/read/29046944/complement-functional-tests-for-monitoring-eculizumab-treatment-in-patients-with-atypical-hemolytic-uremic-syndrome-an-update
#9
Gianluigi Ardissino, Francesca Tel, Martina Sgarbanti, Donata Cresseri, Antenore Giussani, Samantha Griffini, Elena Grovetto, Ilaria Possenti, Michela Perrone, Sara Testa, Fabio Paglialonga, Piergiorgio Messa, Massimo Cugno
BACKGROUND: Atypical hemolytic uremic syndrome (aHUS) is a thrombotic microangiopathy (TMA) characterized by platelet consumption, hemolysis, and organ damage. Eculizumab (ECU), a humanized antibody that blocks complement activity, has been successfully used in aHUS, but the best treatment schedule is not yet clear. METHODS: Here, we report our experience with ECU maintenance treatment and the interval between subsequent doses being extended based on global classical complement pathway (CCP) activity aimed at <30% for maintaining aHUS into remission...
October 18, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28993505/factor-h-competitor-generated-by-gene-conversion-events-associates-with-atypical-hemolytic-uremic-syndrome
#10
Elena Goicoechea de Jorge, Agustín Tortajada, Sheila Pinto García, Sara Gastoldi, Héctor Martín Merinero, Jesús García-Fernández, Emilia Arjona, Mercedes Cao, Giuseppe Remuzzi, Marina Noris, Santiago Rodríguez de Córdoba
Atypical hemolytic uremic syndrome (aHUS), a rare form of thrombotic microangiopathy caused by complement pathogenic variants, mainly affects the kidney microvasculature. A retrospective genetic analysis in our aHUS cohort (n=513) using multiple ligation probe amplification uncovered nine unrelated patients carrying a genetic abnormality in the complement factor H related 1 gene (CFHR1) that originates by recurrent gene conversion events between the CFH and CFHR1 genes. The novel CFHR1 mutants encode an FHR-1 protein with two amino acid substitutions, L290S and A296V, converting the FHR-1 C terminus into that of factor H (FH)...
October 9, 2017: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/28991131/recurrent-atypical-hemolytic-uremic-syndrome-in-children-with-acute-lymphoblastic-leukemia-undergoing-maintenance-chemotherapy
#11
Geoffrey Cheng, Bulent Ozgonenel, Kanta Bhambhani, Gaurav Kapur, Richard J Smith, Süreyya Savaşan
Chemotherapy-associated myelosuppression and renal dysfunction is not uncommon during childhood acute lymphoblastic leukemia (ALL) therapy. Here we report 2 cases of atypical hemolytic uremic syndrome (aHUS) presenting with pancytopenia and renal dysfunction that developed during maintenance chemotherapy characterized by hypocomplementemia. Both cases experienced recurrence after resolution of the initial aHUS episode upon resumption of chemotherapy, raising a possible contributory role for chemotherapy in the disease pathogenesis...
October 4, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28975130/multiple-myeloma-as-the-underlying-cause-of-thrombotic-microangiopathy-leading-to-acute-kidney-injury-revisiting-a-very-rare-entity
#12
Savneek Chugh, Asim Kichloo, Firas Jafri, Liga Yusvirazi, Robert Lerner
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. Rare but important, TMA may also occur in malignancy, connective tissue disease, malignant hypertension, and renal transplantation (rejection or drug toxicity)...
July 2017: Journal of Investigative Medicine High Impact Case Reports
https://www.readbyqxmd.com/read/28947259/-hemolytic-and-uremic-syndrome-and-related-thrombotic-microangiopathies-treatment-and-prognosis
#13
C Rafat, P Coppo, F Fakhouri, V Frémeaux-Bacchi, C Loirat, J Zuber, E Rondeau
Major achievements in the understanding of thrombotic microangiopathies (TMA) have not only resulted in a reclassification of TMA but most of all they have culminated in the design of new treatments and have enabled clinicians to better delineate their prognosis. Recent multicenter studies have improved our understanding of the prognosis of atypical hemolytic and uremic syndromes (aHUS). More specifically, they have highlighted the role of genetic testing on predicting the recurrence of aHUS, the risk of chronic kidney disease and the recurrence following kidney transplantation...
September 22, 2017: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/28941939/complete-functional-characterization-of-disease-associated-genetic-variants-in-the-complement-factor-h-gene
#14
Héctor Martín Merinero, Sheila Pinto García, Jesús García-Fernández, Emilia Arjona, Agustín Tortajada, Santiago Rodríguez de Córdoba
Genetic analyses in atypical hemolytic uremic syndrome (aHUS) and C3-glomerulopathy (C3G) patients have provided an excellent understanding of the genetic component of the disease and informed genotype-phenotype correlations supporting an individualized approach to patient management and treatment. In this context, a correct categorization of the disease-associated gene variants is critical to avoid detrimental consequences for patients and their relatives. Here we describe a comprehensive procedure to measure levels and functional activity of complement regulator factor H (FH) encoded by CFH, the commonest genetic factor associated with aHUS and C3G, and present the results of the analysis of 28 uncharacterized, disease-associated, FH variants...
September 20, 2017: Kidney International
https://www.readbyqxmd.com/read/28939980/targeted-exome-sequencing-in-anti-factor-h-antibody-negative-hus-reveals-multiple-variations
#15
R W Thergaonkar, Ankita Narang, Bahadur Singh Gurjar, Pradeep Tiwari, Mamta Puraswani, Himanshi Saini, Aditi Sinha, Binuja Varma, Mitali Mukerji, Pankaj Hari, Arvind Bagga
BACKGROUND: Genetic susceptibility to atypical hemolytic uremic syndrome (aHUS) may lie within genes regulating or activating the alternate complement and related pathways converging on endothelial cell activation. METHODS: We tested 32 Indian patients of aHUS negative for antibodies to complement factor H for genetic variations in a panel of 15 genes, i.e., CFH, CFHR1-5, CFI, CFB, C3, CD46, MASP2, DGKE, ADAMTS13, THBD and PLG using next-generation DNA sequencing and for copy number variation in CFHR1-3...
September 22, 2017: Clinical and Experimental Nephrology
https://www.readbyqxmd.com/read/28932128/a-suspected-case-of-autoinduction-of-voriconazole-metabolism-in-a-patient-with-cerebral-aspergillosis
#16
Martin J Ferguson, Maria L Randles, Declan G de Freitas
OBJECTIVE: This study aims to report a case of accelerated metabolism of voriconazole in a patient with cerebral aspergillosis. CASE SUMMARY: A 36-year-old woman developed cerebral aspergillosis after immunosuppressive treatment for suspected atypical hemolytic uremic syndrome/thrombotic thrombocytopenic purpura. She was treated with voriconazole using therapeutic drug monitoring to guide dosing. After an initial high level, her dose was reduced, but over the following weeks, she required several dose increases in order to achieve a voriconazole level within the target range...
2017: Drug, Healthcare and Patient Safety
https://www.readbyqxmd.com/read/28911789/a-retrospective-study-of-pregnancy-associated-atypical-hemolytic-uremic-syndrome
#17
Ana Huerta, Emilia Arjona, Jose Portoles, Paula Lopez-Sanchez, Cristina Rabasco, Mario Espinosa, Teresa Cavero, Miquel Blasco, Mercedes Cao, Joaquin Manrique, Virginia Cabello-Chavez, Marta Suñer, Manuel Heras, Xavier Fulladosa, Lara Belmar, Amparo Sempere, Carmen Peralta, Lorena Castillo, Alvaro Arnau, Manuel Praga, Santiago Rodriguez de Cordoba
Pregnancy-associated atypical hemolytic uremic syndrome (aHUS) refers to the thrombotic microangiopathy resulting from uncontrolled complement activation during pregnancy or the postpartum period. Pregnancy-associated aHUS is a devastating disease for which there is a limited clinical understanding and treatment experience. Here we report a retrospective study to analyze the clinical and prognostic data of 22 cases of pregnancy-associated aHUS from the Spanish aHUS Registry under different treatments. Sixteen patients presented during the first pregnancy and as many as nine patients required hemodialysis at diagnosis...
September 11, 2017: Kidney International
https://www.readbyqxmd.com/read/28905254/use-of-eculizumab-in-a-systemic-lupus-erythemathosus-patient-presenting-thrombotic-microangiopathy-and-heterozygous-deletion-in-cfhr1-cfhr3-a-case-report-and-systematic-review
#18
REVIEW
Maria Izabel de Holanda, Luis Cristóvão Pôrto, Teresa Wagner, Luis Fernando Christiani, Lilian M P Palma
The association of thrombotic microangiopathy (TMA) with systemic lupus erythematosus (SLE) has been described in 0.5 to 10% of cases, and patients present worse outcome. TMA is described as the association of microangiopathic hemolytic anemia, thrombocytopenia, and an organ injury, frequently the kidney. This study describes a successful case of use of eculizumab in a patient with SLE and TMA refractory to standard therapy, and provides a literature review. Case description and search in PubMed and MEDLINE using systemic lupus erythemathous and/or antiphospholipid syndrome (APS) and eculizumab retrieved 15 case reports...
December 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28894081/eculizumab-in-renal-transplantation-a-2017-update
#19
Ryszard Grenda, Magdalena Durlik
Despite ongoing progress in renal transplantation, there are still emerging challenges in this field, including consequences of ischemia-reperfusion injury (IRI), pre-existing and produced de novo anti-HLA donor-specific antibodies (DSA), and acute/chronic humoral rejection (AMR), as well as the recurrence of atypical hemolytic-uremic syndrome (aHUS) in genetically predisposed patients. All these conditions are related to the prominent role of the complement system and are deleterious to the fate of the renal graft...
September 12, 2017: Annals of Transplantation: Quarterly of the Polish Transplantation Society
https://www.readbyqxmd.com/read/28864711/meningococcal-b-vaccine-failure-with-a-penicillin-resistant-strain-in-a-young-adult-on-long-term-eculizumab
#20
Sydel R Parikh, Jay Lucidarme, Coralie Bingham, Paul Warwicker, Tim Goodship, Ray Borrow, Shamez N Ladhani
We describe a case of invasive meningococcal disease due to a vaccine-preventable and penicillin-resistant strain in a fully immunized young adult on long-term complement inhibitor therapy and daily penicillin chemoprophylaxis. Eculizumab is a humanized monoclonal antibody that binds human complement C5 protein and inhibits the terminal complement pathway. It is currently recommended for the treatment of complement-mediated thrombotic microangiopathies. An unwanted complication of inhibiting complement, however, is an increased risk of invasive meningococcal disease...
September 2017: Pediatrics
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