Search

Your search keyword '"Fakhouri, F."' showing total 217 results
Start Over Author "Fakhouri, F." Publication Year Range Last 10 years
217 results on '"Fakhouri, F."'

5. Glomerular diseases in pregnancy: pragmatic recommendations for clinical management.

Author

Fakhouri, F., Schwotzer, N., Cabiddu, G., Barratt, J., Legardeur, H., Garovic, V., Orozco-Guillen, A., Wetzels, J., Daugas, E., Moroni, G., Noris, M., Audard, V., Praga, M., Llurba, E., Wuerzner, G., Attini, R., Desseauve, D., Zakharova, E., Luders, C., Wiles, K., Leone, F., Jesudason, S., Costedoat-Chalumeau, N., Kattah, A., Soto-Abraham, V., Karras, A., Prakash, J., Lightstone, L., Ronco, P., Ponticelli, C., Appel, G., Remuzzi, G., Tsatsaris, V., Piccoli, G.B., Fakhouri, F., Schwotzer, N., Cabiddu, G., Barratt, J., Legardeur, H., Garovic, V., Orozco-Guillen, A., Wetzels, J., Daugas, E., Moroni, G., Noris, M., Audard, V., Praga, M., Llurba, E., Wuerzner, G., Attini, R., Desseauve, D., Zakharova, E., Luders, C., Wiles, K., Leone, F., Jesudason, S., Costedoat-Chalumeau, N., Kattah, A., Soto-Abraham, V., Karras, A., Prakash, J., Lightstone, L., Ronco, P., Ponticelli, C., Appel, G., Remuzzi, G., Tsatsaris, V., and Piccoli, G.B.

Abstract

01 februari 2023, Item does not contain fulltext, Our understanding of the various aspects of pregnancy in women with kidney diseases has significantly improved in the last decades. Nevertheless, little is known about specific kidney diseases. Glomerular diseases are not only a frequent cause of chronic kidney disease in young women, but combine many challenges in pregnancy: immunologic diseases, hypertension, proteinuria, and kidney tissue damage. An international working group undertook the review of available current literature and elicited expert opinions on glomerular diseases in pregnancy with the aim to provide pragmatic information for nephrologists according to the present state-of-the-art knowledge. This work also highlights areas of clinical uncertainty and emphasizes the need for further collaborative studies to improve maternal and fetal health.

Published
2023

8. The Rational Use of Complement Inhibitors in Kidney Diseases

Author

Fakhouri, F., Schwotzer, N., Golshayan, D., and Frémeaux-Bacchi, V.

Subjects
complement, complement inhibitors, glomerular diseases
Abstract

The development of complement inhibitors represented one of the major breakthroughs in clinical nephrology in the last decade. Complement inhibition has dramatically transformed the outcome of one of the most severe kidney diseases, the atypical hemolytic uremic syndrome (aHUS), a prototypic complement-mediated disorder. The availability of complement inhibitors has also opened new promising perspectives for the management of several other kidney diseases in which complement activation is involved to a variable extent. With the rapidly growing number of complement inhibitors tested in a rapidly increasing number of indications, a rational use of this innovative and expensive new therapeutic class has become crucial. The present review aims to summarize what we know, and what we still ignore, regarding complement activation and therapeutic inhibition in kidney diseases. It also provides some clues and elements of thoughts for a rational approach of complement modulation in kidney diseases.

Published
2022

9. Histological predictors of outcome in C3 glomerulopathy and idiopathic immunoglobulin-associated membranoproliferative glomerulonephritis

Author

Medjeral-Thomas, N, Barbour, S, Gisby, J, Han, H, Bomback, A, Fervenza, F, Cairns, T, Szydlo, R, Tan, S-J, Marks, S, Waters, A, Appel, G, D'Agati, V, Sethi, S, Nast, C, Bajema, I, Alpers, C, Fogo, A, Licht, C, Fakhouri, F, Cattran, D, Peters, J, Terence, C, Lomax-Browne, H, Pickering, M, and Alexion Pharmaceuticals Inc.

Subjects
urogenital system, 1103 Clinical Sciences, Urology & Nephrology
Abstract

Background and objectives C3 glomerulopathy and idiopathic immunoglobulin-associated membranoproliferative glomerulonephritis (Ig-MPGN) are kidney diseases characterised by abnormal glomerular complement C3 deposition. These conditions are heterogeneous in outcome, but approximately 50% of patients reach end stage kidney disease within 10 years. Design, setting, participants and measurements To improve identification of patients with poor prognosis we performed a detailed analysis of percutaneous kidney biopsies in a large cohort of patients. Using a validated histological scoring system, we analysed 156 native diagnostic kidney biopsies from a retrospective cohort of 123 patients with C3 glomerulopathy and 33 patients with Ig-MPGN. We used linear regression, survival analysis and Cox proportional hazards models to assess the relationship between histological and clinical parameters with outcome. Results Frequent biopsy features were mesangial expansion and hypercellularity, glomerular basement membrane (GBM) double contours and endocapillary hypercellularity. Multivariable analysis showed negative associations between estimated glomerular filtration rate (eGFR) and crescents, interstitial inflammation, and interstitial fibrosis and tubular atrophy (IFTA). Proteinuria positively associated with endocapillary hypercellularity and GBM double contours. Analysis of second native biopsies did not demonstrate associations between immunosuppression treatment and improvement in histology. Using a composite outcome, risk of progression to kidney failure associated with eGFR and proteinuria at time of biopsy, and cellular/fibrocellular crescents, segmental sclerosis and IFTA scores. Conclusions Our detailed assessment of kidney biopsy data indicated that cellular/fibrocellular crescents and IFTA scores were significant determinants of deterioration in kidney function.

Published
2022

12. POS-048 PHASE 3, RANDOMIZED, MULTICENTER STUDY TO EVALUATE THE EFFICACY AND SAFETY OF PEGCETACOPLAN IN TREATMENT OF C3G OR IC-MPGN

Author

Remuzzi, G., primary, Dixon, B., additional, Fakhouri, F., additional, Pickering, M., additional, Cook, T., additional, Kavanagh, D., additional, Walker, P., additional, Licht, C., additional, Appel, G., additional, Vivarelli, M., additional, Zhang, Z., additional, Li, L., additional, and Kocinsky, H., additional

Published
2022
Full Text
View/download PDF

14. Nouveautés dans la pathophysiologie et le traitement de l’anémie rénale [New insights in the pathophysiology and treatment of renal anemia]

Author

Vultaggio, P., Loria, F., Fakhouri, F., Leuenberger, N., and Pruijm, M.

Subjects
Anemia/etiology, Anemia/therapy, Erythropoiesis, Erythropoietin/metabolism, Erythropoietin/therapeutic use, Humans, Hypoxia-Inducible Factor-Proline Dioxygenases/metabolism, Iron/therapeutic use, Renal Insufficiency, Chronic/complications, Renal Insufficiency, Chronic/therapy, hemic and lymphatic diseases
Abstract

Renal anemia is a frequently encountered complication in patients suffering from advanced chronic kidney disease. This is mainly due to the decreased secretion of erythropoietin by the diseased kidneys. The current treatment of renal anemia is based on iron substitution and administration of recombinant erythropoietin. The discovery of HIF (Hypoxia-Inducible Factor) has led to the development of a new class of molecules that block the activity of prolyl-4-hydroxylases and stabilize HIF (Hypoxia-Inducible Factor), a transcription factor that plays an essential role in numerous cellular pathways, including those linked to erythropoiesis and iron metabolism. In this article, we discuss the current understanding of the pathophysiological mechanisms underlying renal anemia and the potential role of the new HIF-stabilizers in its treatment.

Published
2022

15. Hypertension artérielle sévère ou urgence hypertensive : du cabinet à l’hôpital [Severe asymptomatic hypertension and hypertensive emergency : From the ambulatory care to the emergency room]

Author

Berney, M., Fakhouri, F., and Wuerzner, G.

Subjects
Ambulatory Care, Antihypertensive Agents/therapeutic use, Blood Pressure, Emergency Service, Hospital, Humans, Hypertension/diagnosis, Hypertension/drug therapy, Hypertension, Malignant/drug therapy
Abstract

High blood pressure levels are frequently encountered in medical practice, whether in an outpatient or inpatient setting. It is imperative to quickly differentiate severe hypertension associated with target organ damage, from severe hypertension without acute organ damage. In the latter situation, the management can be done on an outpatient basis by prescribing oral antihypertensive treatment with a close follow-up. On the other hand, in severe hypertension with acute target organ damage, patients should be admitted in an intensive care unit for close monitoring and, in most cases, treated with intravenous antihypertensive treatment. This article reviews the new definitions and management of these two entities and is addressed to both general practitioner and emergency doctor.

Published
2021

21. Complément et rein [Complement system and kidney]

Author

Halfon, M., Pascual, M., Sadallah, S., and Fakhouri, F.

Subjects
Drug-Related Side Effects and Adverse Reactions, Humans, Kidney, Kidney Transplantation, Vasculitis
Abstract

In the last few years, there has been a growing interest in the study of complement, fueleld mainly by the design of complement modulators, especially the C5-blocker eculizumab. The latter has significantly improved the prognosis of some nephropathies, such as the atypical hemolytic uremic syndrome. This breakthrough is a perfect example of fundamental translational research leading to clinical applications for patients. Currently, new molecules are being developed and some of them have already demonstrated clinical efficacy, such as avacopan (C5aR blocker) in ANCA vasculitis. As for kidney transplantation, complement modulators may lead to a new perspective in the treatment of some complications, such as humoral rejection. However, complement modulators carry the side effects, especially the infectious, and high costs.

Published
2021

22. Les reins : cibles du réchauffement climatique ? [The kidneys : possible victims of global warming ?]

Author

Ngatchou, N., Martin, P.Y., Fakhouri, F., and Pruijm, M.

Subjects
Central America/epidemiology, Global Warming, Humans, Kidney/pathology, Kidney/physiopathology, Kidney Diseases/epidemiology, Kidney Diseases/etiology, Kidney Diseases/prevention & control, Kidney Failure, Chronic/epidemiology, Kidney Failure, Chronic/etiology, Kidney Failure, Chronic/prevention & control, Sri Lanka/epidemiology, Switzerland/epidemiology
Abstract

Over the last decades, an increasing number of cases of chronic and end-stage kidney disease has been observed in Central America and Asia. This kidney disease mainly affects young farmers without classic renal risk factors. The clinical presentation includes a progressive decrease of the glomerular filtration rate, minimal proteinuria and the presence of tubulo-interstitial nephritis at renal biopsy. A close link with global warming is suspected for this disease, called (according to its location) meso-american nephropathy, Sri Lanka nephropathy or chronic kidney disease of unknown etiology. Others have suggested that intake of water contaminated with pesticides may be responsible. This article provides an overview of this new kidney disease. Measures to prevent acute kidney injury during heat waves in Switzerland are also discussed.

Published
2020

25. Microangiopathies thrombotiques (MAT) associées aux infections : particularités et pronostic

Author

Thoreau, B., primary, Bayer, G., additional, Barbet, C., additional, Cloarec, S., additional, Meriau, E., additional, Lachot, S., additional, Garot, D., additional, Bernard, L., additional, Gyan, E., additional, Perrotin, F., additional, Pouplard, C., additional, Maillot, F., additional, Gatault, P., additional, Sautenet, B., additional, Rusch, E., additional, Buchler, M., additional, Fremeaux-Bacchi, V., additional, Vigneau, C., additional, Fakhouri, F., additional, and Halimi, J.M., additional

Published
2019
Full Text
View/download PDF

27. BLOOD PRESSURE IN THROMBOTIC MICROANGIOPATHY

Author

Halimi, J., primary, Von Tokarski, F., additional, Thoreau, B., additional, Bauvois, A., additional, Barbet, C., additional, Cloarec, S., additional, Mérieau, E., additional, Lachot, S., additional, Garot, D., additional, Lemaignen, A., additional, Gyan, E., additional, Perrotin, F., additional, Pouplard, C., additional, Maillot, F., additional, Gatault, P., additional, Sautenet, B., additional, Rusch, E., additional, Buchler, M., additional, Frémeaux-Bacchi, V., additional, Vigneau, C., additional, Fakhouri, F., additional, and Bayer, G., additional

Published
2019
Full Text
View/download PDF

29. C3 glomerulopathy - understanding a rare complement-driven renal disease

Author

Smith, R.J., Appel, G.B., Blom, A.M., Cook, H.T., D'Agati, V.D., Fakhouri, F., Fremeaux-Bacchi, V., Jozsi, M., Kavanagh, D., Lambris, J.D., Noris, M., Pickering, M.C., Remuzzi, G., Cordoba, S.R. de, Sethi, S., Vlag, J. van der, Zipfel, P.F., Nester, C.M., Smith, R.J., Appel, G.B., Blom, A.M., Cook, H.T., D'Agati, V.D., Fakhouri, F., Fremeaux-Bacchi, V., Jozsi, M., Kavanagh, D., Lambris, J.D., Noris, M., Pickering, M.C., Remuzzi, G., Cordoba, S.R. de, Sethi, S., Vlag, J. van der, Zipfel, P.F., and Nester, C.M.

Abstract

Item does not contain fulltext, The C3 glomerulopathies are a group of rare kidney diseases characterized by complement dysregulation occurring in the fluid phase and in the glomerular microenvironment, which results in prominent complement C3 deposition in kidney biopsy samples. The two major subgroups of C3 glomerulopathy - dense deposit disease (DDD) and C3 glomerulonephritis (C3GN) - have overlapping clinical and pathological features suggestive of a disease continuum. Dysregulation of the complement alternative pathway is fundamental to the manifestations of C3 glomerulopathy, although terminal pathway dysregulation is also common. Disease is driven by acquired factors in most patients - namely, autoantibodies that target the C3 or C5 convertases. These autoantibodies drive complement dysregulation by increasing the half-life of these vital but normally short-lived enzymes. Genetic variation in complement-related genes is a less frequent cause. No disease-specific treatments are available, although immunosuppressive agents and terminal complement pathway blockers are helpful in some patients. Unfortunately, no treatment is universally effective or curative. In aggregate, the limited data on renal transplantation point to a high risk of disease recurrence (both DDD and C3GN) in allograft recipients. Clinical trials are underway to test the efficacy of several first-generation drugs that target the alternative complement pathway.

Published
2019

34. Nutritional potential of dehydrated residues from rice milk production

Author

Coordenaçao de Aperfeiçoamento de Pessoal de Nível Superior, Brasil, Silva, Luan, Casari, A., Velasco, J., Fakhouri, F., Coordenaçao de Aperfeiçoamento de Pessoal de Nível Superior, Brasil, Silva, Luan, Casari, A., Velasco, J., and Fakhouri, F.

Abstract

[EN] The aim of this work was to verify the influence of drying on physical and chemical characteristics of residues from rice milk production. Residues were from the production of white, red and black rice. They were dehydrated in an oven with air circulation at 60ºC for 8h. Characterization of them were carried out by physical and chemical analyses. Residues have presented statistical difference for all parameters measured. Its characterization is necessary to justify its use in other process or application on new products.The residues from rice milk production have high content of nutrients and phenolic compounds, even after dehydration.

Published
2018

36. SAT0029 B cell depletion affects CD8 T cells in anca-associated vasculitis

Author

Néel, A, primary, Bucchia, M, additional, Néel, M, additional, Rimbert, M, additional, Agard, C, additional, Hourmant, M, additional, Tilly, G, additional, Yap, M, additional, Perrin, F, additional, Graveleau, J, additional, Godmer, P, additional, Brouard, S, additional, Bressollette, C, additional, Fakhouri, F, additional, Hamidou, M, additional, and Degauque, N, additional

Published
2017
Full Text
View/download PDF

37. Atypical hemolytic uremic syndrome and C3 glomerulopathy: conclusions from a 'Kidney Disease: Improving Global Outcomes' (KDIGO) Controversies Conference

Author

Goodship, T.H., Cook, H.T., Fakhouri, F., Fervenza, F.C., Fremeaux-Bacchi, V., Kavanagh, D., Nester, C.M., Noris, M., Pickering, M.C., Cordoba, S., Roumenina, L.T., Sethi, S., Smith, R.J., Kar, N.C. van de, et al., Goodship, T.H., Cook, H.T., Fakhouri, F., Fervenza, F.C., Fremeaux-Bacchi, V., Kavanagh, D., Nester, C.M., Noris, M., Pickering, M.C., Cordoba, S., Roumenina, L.T., Sethi, S., Smith, R.J., Kar, N.C. van de, and et al.

Abstract

Contains fulltext : 170623.pdf (Publisher’s version ) (Open Access), In both atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathy (C3G) complement plays a primary role in disease pathogenesis. Herein we report the outcome of a 2015 Kidney Disease: Improving Global Outcomes (KDIGO) Controversies Conference where key issues in the management of these 2 diseases were considered by a global panel of experts. Areas addressed included renal pathology, clinical phenotype and assessment, genetic drivers of disease, acquired drivers of disease, and treatment strategies. In order to help guide clinicians who are caring for such patients, recommendations for best treatment strategies were discussed at length, providing the evidence base underpinning current treatment options. Knowledge gaps were identified and a prioritized research agenda was proposed to resolve outstanding controversial issues.

Published
2017

38. An international consensus approach to the management of atypical hemolytic uremic syndrome in children

Author

Loirat, C., Fakhouri, F., Ariceta, G., Besbas, N., Bitzan, M., Bjerre, A., Coppo, R., Emma, F., Johnson, S., Karpman, D., Landau, D., Langman, C.B., Lapeyraque, A.L., Licht, C., Nester, C., Pecoraro, C., Riedl, M., Kar, N.C.A.J. van de, Walle, J. Vande, Vivarelli, M., Fremeaux-Bacchi, V., Loirat, C., Fakhouri, F., Ariceta, G., Besbas, N., Bitzan, M., Bjerre, A., Coppo, R., Emma, F., Johnson, S., Karpman, D., Landau, D., Langman, C.B., Lapeyraque, A.L., Licht, C., Nester, C., Pecoraro, C., Riedl, M., Kar, N.C.A.J. van de, Walle, J. Vande, Vivarelli, M., and Fremeaux-Bacchi, V.

Abstract

Item does not contain fulltext, Atypical hemolytic uremic syndrome (aHUS) emerged during the last decade as a disease largely of complement dysregulation. This advance facilitated the development of novel, rational treatment options targeting terminal complement activation, e.g., using an anti-C5 antibody (eculizumab). We review treatment and patient management issues related to this therapeutic approach. We present consensus clinical practice recommendations generated by HUS International, an international expert group of clinicians and basic scientists with a focused interest in HUS. We aim to address the following questions of high relevance to daily clinical practice: Which complement investigations should be done and when? What is the importance of anti-factor H antibody detection? Who should be treated with eculizumab? Is plasma exchange therapy still needed? When should eculizumab therapy be initiated? How and when should complement blockade be monitored? Can the approved treatment schedule be modified? What approach should be taken to kidney and/or combined liver-kidney transplantation? How should we limit the risk of meningococcal infection under complement blockade therapy? A pressing question today regards the treatment duration. We discuss the need for prospective studies to establish evidence-based criteria for the continuation or cessation of anticomplement therapy in patients with and without identified complement mutations.

Published
2016

39. Recommandations francophones, internationales et multidisciplinaires d’experts pour l’utilisation de biomédicaments dans le lupus érythémateux systémique : le groupe de travail du CRI-IMIDIATE

Author

Kleinmann, J.F., primary, Tubach, F., additional, Le Guern, V., additional, Mathian, A., additional, Richez, C., additional, Saadoun, D., additional, Sacré, K., additional, Sellam, J., additional, Seror, R., additional, Amoura, Z., additional, Andres, E., additional, Audia, S., additional, Bader-Meunier, B., additional, Blaison, G., additional, Bonnotte, B., additional, Cacoub, P., additional, Caillard, S., additional, Chiche, L., additional, Chosidow, O., additional, Costedoat-Chalumeau, N., additional, Daien, C., additional, Daugas, E., additional, Derdèche, N., additional, Doria, A., additional, Fain, O., additional, Fakhouri, F., additional, Farge, D., additional, Gabay, C., additional, Guillo, S., additional, Hachulla, E., additional, Hajjaj-Hassouni, N., additional, Hamidou, M., additional, Houssiau, F., additional, Jourde-Chiche, N., additional, Kone-Paut, I., additional, Ladjouz-Rezig, A., additional, Lambotte, O., additional, Lipsker, D., additional, Mariette, X., additional, Martin Silva, N., additional, Martin, T., additional, Maurier, F., additional, Meckenstock, R., additional, Mekinian, A., additional, Meyer, O., additional, Mohamed, S., additional, Morel, J., additional, Moulin, B., additional, Mulleman, D., additional, Papo, T., additional, Poindron, V., additional, Puéchal, X., additional, Punzi, L., additional, Quartier, P., additional, Sailler, L., additional, Smail, A., additional, Soubrier, M., additional, Sparsa, A., additional, Tazi Mezalek, Z., additional, Zakraoui, L., additional, Zuily, S., additional, Sibilia, J., additional, and Gottenberg, J., additional

Published
2016
Full Text
View/download PDF

47. Acquired and genetic drivers of C3 and C5 convertase dysregulation in C3 glomerulopathy and immunoglobulin-associated MPGN.

Author

Roquigny J, Meuleman MS, El Sissy C, Martins PV, Meri S, Duval A, Lequintrec M, Fakhouri F, Chauvet S, and Frémeaux-Bacchi V

Abstract

Dysregulation of the alternative pathway of complement plays a central role in the pathophysiology of C3 Glomerulopathy (C3G). Various autoimmune and genetic factors targeting the alternative pathway have been associated to both C3G and primary Immunoglobulin-associated Membranoproliferative Glomerulonephritis (Ig-MPGN), suggesting shared pathophysiological mechanisms. This review highlights the wide range of disease drivers identified that mainly target components or protein complexes of the alternative pathway, both in C3G and Ig-MPGN. Nephritic factors, which constitute a heterogeneous group of autoantibodies targeting the C3 or the C5 convertase, are the most common abnormalities. Monoclonal gammopathies are frequent in aging adults. They may promote complement activation and have in some cases also found to target alternative pathway regulatory proteins. Additionally, some patients with C3G and Ig-MPGN carry rare variants in genes encoding complement activating or regulating proteins of the alternative pathway. This review provides an informative overview of pathogenetic mechanisms associated with each abnormality, acting at different steps in the complement cascade. The diversity of targets involved in the C3G pathophysiology suggests the potential benefit of therapeutical approaches tailored to the underlying disease drivers, with a pivotal impact upstream or at the level of the C3 or C5 convertase activity., (© The Author(s) 2024. Published by Oxford University Press on behalf of the ERA.)

Published
2024
Full Text
View/download PDF

48. Non-plasmapheresis Approaches for Managing Severe Renal Thrombotic Microangiopathy: A Report of 7 Cases.

Author

Frimat M, Maanaoui M, Schwotzer N, Lebas C, Lionet A, Dubucquoi S, Gnemmi V, Fakhouri F, and Provôt F

Abstract

C5-blockers are the established treatment for complement-mediated hemolytic uremic syndrome (CM-HUS). However, CM-HUS, lacking a definitive test, prompts plasma exchanges as a common first-line therapy, pending further assessments, despite complications and limited evidence in this indication. Recent experts' opinion endorses C5-blockers as the initial treatment for severe renal thrombotic microangiopathy (TMA). This retrospective, single-center study reports a series of 7 patients treated with a plasmapheresis-free approach. All patients presented with severe renal TMA symptoms and low French score and received prompt 900mg eculizumab within a median of 9 hours from admission. Hematological recovery was rapid, and renal function improved in 6 patients within 6.5 days, with a median hospital stay of 16 days. No rescue plasmapheresis was used. We report 7 cases of an early C5-blocker and plasmapheresis-free strategy in severe renal TMA suspicious for CM-HUS, demonstrating promising initial results. Clinical trials are needed to confirm the efficacy and safety of this approach. Addressing the high cost of C5-blocking therapies and exploring cost-effective alternatives is also crucial for broader implementation and accessibility in targeted therapies for adult renal TMA., (Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)

Published
2024
Full Text
View/download PDF

49. An expert discussion on the atypical hemolytic uremic syndrome nomenclature-identifying a road map to precision: a report of a National Kidney Foundation Working Group.

Author

Nester CM, Feldman DL, Burwick R, Cataland S, Chaturvedi S, Cook HT, Cuker A, Dixon BP, Fakhouri F, Hingorani SR, Java A, van de Kar NCAJ, Kavanagh D, Leung N, Licht C, Noris M, O'Shaughnessy MM, Parikh SV, Peyandi F, Remuzzi G, Smith RJH, Sperati CJ, Waldman M, Walker P, and Vivarelli M

Subjects
Humans, Consensus, Nephrology standards, Atypical Hemolytic Uremic Syndrome genetics, Atypical Hemolytic Uremic Syndrome diagnosis, Terminology as Topic, Delphi Technique
Abstract

The term atypical hemolytic uremic syndrome has been in use since the mid-1970s. It was initially used to describe the familial or sporadic form of hemolytic uremic syndrome as opposed to the epidemic, typical form of the disease. Over time, the atypical hemolytic uremic syndrome term has evolved into being used to refer to anything that is not Shiga toxin-associated hemolytic uremic syndrome. The term describes a heterogeneous group of diseases of disparate causes, a circumstance that makes defining disease-specific natural history and/or targeted treatment approaches challenging. A working group of specialty-specific experts in the thrombotic microangiopathies was convened to review the validity of this broad term in an era of swiftly advancing science and targeted therapeutics. A Delphi approach was used to define and interrogate some of the key issues related to the atypical hemolytic uremic syndrome nomenclature., (Copyright © 2024 International Society of Nephrology. Published by Elsevier Inc. All rights reserved.)

Published
2024
Full Text
View/download PDF

50. The role of complement in kidney disease: conclusions from a Kidney Disease: Improving Global Outcomes (KDIGO) Controversies Conference.

Author

Vivarelli M, Barratt J, Beck LH Jr, Fakhouri F, Gale DP, Goicoechea de Jorge E, Mosca M, Noris M, Pickering MC, Susztak K, Thurman JM, Cheung M, King JM, Jadoul M, Winkelmayer WC, and Smith RJH

Subjects
Humans, Biomarkers blood, Complement Inactivating Agents therapeutic use, Complement System Proteins immunology, Complement System Proteins metabolism, Congresses as Topic, Disease Progression, Kidney Glomerulus immunology, Kidney Glomerulus pathology, Complement Activation immunology, Kidney Diseases immunology, Kidney Diseases therapy, Kidney Diseases diagnosis
Abstract

Uncontrolled complement activation can cause or contribute to glomerular injury in multiple kidney diseases. Although complement activation plays a causal role in atypical hemolytic uremic syndrome and C3 glomerulopathy, over the past decade, a rapidly accumulating body of evidence has shown a role for complement activation in multiple other kidney diseases, including diabetic nephropathy and several glomerulonephritides. The number of available complement inhibitor therapies has also increased during the same period. In 2022, Kidney Diseases: Improving Global Outcomes (KDIGO) convened a Controversies Conference, "The Role of Complement in Kidney Disease," to address the expanding role of complement dysregulation in the pathophysiology, diagnosis, and management of various glomerular diseases, diabetic nephropathy, and other forms of hemolytic uremic syndrome. Conference participants reviewed the evidence for complement playing a primary causal or secondary role in progression for several disease states and considered how evidence of complement involvement might inform management. Participating patients with various complement-mediated diseases and caregivers described concerns related to life planning, implications surrounding genetic testing, and the need for inclusive implementation of effective novel therapies into clinical practice. The value of biomarkers in monitoring disease course and the role of the glomerular microenvironment in complement response were examined, and key gaps in knowledge and research priorities were identified., (Copyright © 2024 Kidney Disease: Improving Global Outcomes (KDIGO). Published by Elsevier Inc. All rights reserved.)

Published
2024
Full Text
View/download PDF

Catalog

Books, media, physical & digital resources
See catalog results