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https://www.readbyqxmd.com/read/29157988/de-novo-thrombotic-microangiopathy-after-kidney-transplantation
#1
REVIEW
Neetika Garg, Helmut G Rennke, Martha Pavlakis, Kambiz Zandi-Nejad
Thrombotic microangiopathy (TMA) is a serious complication of transplantation that adversely affects kidney transplant recipient and allograft survival. Post-transplant TMA is usually classified into two categories: 1) recurrent TMA and 2) de novo TMA. Atypical hemolytic uremic syndrome (aHUS) resulting from dysregulation and over-activation of the alternate complement pathway is a rare disease but the most common diagnosis associated with recurrence in the allografts. De novo TMA, on the other hand, represents an overwhelming majority of the cases of post-transplant TMA and is a substantially more heterogeneous entity than recurrent aHUS...
November 4, 2017: Transplantation Reviews
https://www.readbyqxmd.com/read/29147603/age-estrogen-and-immune-response-in-breast-adenocarcinoma-and-adjacent-normal-tissue
#2
David A Quigley, Andliena Tahiri, Torben Lüders, Margit H Riis, Allan Balmain, Anne-Lise Børresen-Dale, Ida Bukholm, Vessela Kristensen
Chronic inflammation promotes breast tumor growth and invasion by accelerating angiogenesis and tissue remodeling in the tumor microenvironment. There is a complex relationship between inflammation and estrogen, which drives the growth of 70 percent of breast tumors. While low levels of estrogen exposure stimulate macrophages and other inflammatory cell populations, very high levels are immune suppressive. Breast tumor incidence is increased by obesity and age, which interact to influence inflammatory cell populations in normal breast tissue...
2017: Oncoimmunology
https://www.readbyqxmd.com/read/29142942/complement-activation-induces-neutrophil-adhesion-and-neutrophil-platelet-aggregate-formation-on-vascular-endothelial-cells
#3
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
#4
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/29106598/safety-and-effectiveness-of-restrictive-eculizumab-treatment-in-atypical-haemolytic-uremic-syndrome
#5
Kioa L Wijnsma, Caroline Duineveld, Elena B Volokhina, Lambertus P van den Heuvel, Nicole C A J van de Kar, Jack F M Wetzels
Background: Atypical haemolytic uremic syndrome (aHUS) is a rare but severe form of thrombotic microangiopathy as a consequence of complement dysregulation. aHUS has a poor outcome with high mortality and >50% of patients developing end-stage renal disease. Since the end of 2012, these outcomes have greatly improved with the introduction of eculizumab. Currently the duration of treatment is debated. Most guidelines advise lifelong treatment. However, there is no hard evidence to support this advice...
July 4, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/29068997/atypical-hemolytic-uremic-syndrome-induced-by-cblc-subtype-of-methylmalonic-academia-a-case-report-and-literature-review
#6
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/29046944/complement-functional-tests-for-monitoring-eculizumab-treatment-in-patients-with-atypical-hemolytic-uremic-syndrome-an-update
#7
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
#8
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
#9
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/28980670/complement-c5-inhibiting-therapy-for-the-thrombotic-microangiopathies-accumulating-evidence-but-not-a-panacea
#10
Vicky Brocklebank, David Kavanagh
Thrombotic microangiopathy (TMA), characterized by organ injury occurring consequent to severe endothelial damage, can manifest in a diverse range of diseases. In complement-mediated atypical haemolytic uraemic syndrome (aHUS) a primary defect in complement, such as a mutation or autoantibody leading to over activation of the alternative pathway, predisposes to the development of disease, usually following exposure to an environmental trigger. The elucidation of the pathogenesis of aHUS resulted in the successful introduction of the complement inhibitor eculizumab into clinical practice...
October 2017: Clinical Kidney Journal
https://www.readbyqxmd.com/read/28947259/-hemolytic-and-uremic-syndrome-and-related-thrombotic-microangiopathies-treatment-and-prognosis
#11
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
#12
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
#13
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/28918528/signaling-of-the-complement-cleavage-product-anaphylatoxin-c5a-through-c5ar-cd88-contributes-to-pharmacological-hematopoietic-stem-cell-mobilization
#14
Kamila Bujko, Sylwia Rzeszotek, Kai Hoehlig, Jun Yan, Axel Vater, Mariusz Z Ratajczak
Several mechanisms have been postulated for orchestrating the mobilization of hematopoietic stem/progenitor cells (HSPCs), and we previously proposed that activation of the complement cascade plays a crucial role in the initiation and execution of the egress of HSPCs from bone marrow (BM) into peripheral blood (PB). In support of this notion, we demonstrated that mice deficient in the mannan-binding lectin (MBL) pathway, which activates the proximal part of the complement cascade, as well as mice deficient in the fifth component of the complement cascade (C5), which is part of the distal part of the complement cascade, are poor mobilizers...
September 16, 2017: Stem Cell Reviews
https://www.readbyqxmd.com/read/28911789/a-retrospective-study-of-pregnancy-associated-atypical-hemolytic-uremic-syndrome
#15
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
#16
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/28899876/treatment-and-management-of-children-with-haemolytic-uraemic-syndrome
#17
REVIEW
Patrick R Walsh, Sally Johnson
Haemolytic uraemic syndrome (HUS), comprising microangiopathic haemolytic anaemia, thrombocytopaenia and acute kidney injury, remains the leading cause of paediatric intrinsic acute kidney injury, with peak incidence in children aged under 5 years. HUS most commonly occurs following infection with Shiga toxin-producing Escherichia coli (STEC-HUS). Additionally, HUS can occur as a result of inherited or acquired dysregulation of the alternative complement cascade (atypical HUS or aHUS) and in the setting of invasive pneumococcal infection...
September 12, 2017: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/28894081/eculizumab-in-renal-transplantation-a-2017-update
#18
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/28863792/genetics-of-immune-mediated-glomerular-diseases-focus-on-complement
#19
REVIEW
Marina Noris, Giuseppe Remuzzi
The spectrum of immune-mediated glomerular diseases is wide, ranging from rare diseases with well-recognized genetic origins to more common and multifactorial diseases. Immune-mediated glomerular injury is complex and involves both the innate and the adaptive immune systems. In the past 20 years a huge effort has been undertaken to unravel the genetic basis of immune-mediated glomerular diseases. The discovery of abnormalities in genes encoding proteins of the alternative pathway of complement in more than 50% of patients with atypical hemolytic uremic syndrome (aHUS), and in approximately 20% of patients with membranoproliferative glomerulonephritis (MPGN), has highlighted the role of this complement pathway in the pathogenesis of immune-mediated glomerular diseases...
September 2017: Seminars in Nephrology
https://www.readbyqxmd.com/read/28858176/midterm-outcomes-of-12-renal-transplant-recipients-treated-with-eculizumab-to-prevent-atypical-hemolytic-syndrome-recurrence
#20
Charlène Levi, Véronique Frémeaux-Bacchi, Julien Zuber, Marion Rabant, Magali Devriese, Renaud Snanoudj, Anne Scemla, Lucile Amrouche, Arnaud Mejean, Christophe Legendre, Rebecca Sberro-Soussan
BACKGROUND: Atypical hemolytic uremic syndrome (aHUS) is an orphan disease with a high rate of recurrence after kidney transplantation. However, reports of successful prevention of posttransplant aHUS recurrence with eculizumab emerged a few years ago. To further delineate its optimal use, we describe the largest series of kidney transplant recipients treated with prophylactic eculizumab. METHODS: Twelve renal transplant recipients with aHUS-related end stage renal disease received eculizumab: 10 from day 0 and 2 at the time of recurrence (days 6 and 25)...
August 25, 2017: Transplantation
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