Atypical and secondary hemolytic uremic syndromes have a distinct presentation and no common genetic risk factors
| العنوان: | Atypical and secondary hemolytic uremic syndromes have a distinct presentation and no common genetic risk factors |
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| المؤلفون: | Sophie Limou, Véronique Frémeaux-Bacchi, Noémie Jourde-Chiche, Fadi Fakhouri, David Ribes, Alice Le Clech, François Provôt, Ludivine Lebourg, Moglie Le Quintrec, Alexandre Karras, Jean-Michel Halimi, Paula Vieira-Martins, Eric Rondeau, Noémie Simon-Tillaux, Steven Grangé, Yahsou Delmas |
| المساهمون: | Centre de Recherche en Transplantation et Immunologie (U1064 Inserm - CRTI), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Nantes - UFR de Médecine et des Techniques Médicales (UFR MEDECINE), Université de Nantes (UN)-Université de Nantes (UN), Translational ImmunoGenetic in AutoImmunity and Transplantation (Team 5 - U1064 Inserm - CRTI), Université de Nantes (UN)-Université de Nantes (UN)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Nantes - UFR de Médecine et des Techniques Médicales (UFR MEDECINE), Centre recherche en CardioVasculaire et Nutrition = Center for CardioVascular and Nutrition research (C2VN), Aix Marseille Université (AMU)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE), Immunotherapy in Transplantation And Autoimmunity (Team 3 - U1064 Inserm - CRTI), CCSD, Accord Elsevier |
| المصدر: | Kidney International Kidney International, Nature Publishing Group, 2019, 95 (6), pp.1443-1452. ⟨10.1016/j.kint.2019.01.023⟩ Kidney International, 2019, 95 (6), pp.1443-1452. ⟨10.1016/j.kint.2019.01.023⟩ |
| بيانات النشر: | HAL CCSD, 2019. |
| سنة النشر: | 2019 |
| مصطلحات موضوعية: | 0301 basic medicine, medicine.medical_specialty, Thrombotic microangiopathy, medicine.medical_treatment, 030232 urology & nephrology, Disease, urologic and male genital diseases, Gastroenterology, Organ transplantation, 03 medical and health sciences, 0302 clinical medicine, Internal medicine, medicine, complement, Dialysis, [SDV.MHEP] Life Sciences [q-bio]/Human health and pathology, business.industry, Eculizumab, medicine.disease, 3. Good health, thrombotic microangiopathy, 030104 developmental biology, Nephrology, Cohort, hemolytic uremic syndrome, Pancreatitis, eculizumab, business, [SDV.MHEP]Life Sciences [q-bio]/Human health and pathology, Kidney disease, medicine.drug |
| الوصف: | International audience; Secondary hemolytic uremic syndrome (HUS) is a heterogeneous group of thrombotic microangiopathies associated with various underlying conditions. Whether it belongs to the spectrum of complement-mediated HUS remains controversial. We analysed the presentation, outcome, and frequency of complement gene rare variants in a cohort of 110 patients with secondary HUS attributed to drugs (29%), autoimmune diseases (24%), infections (17%), malignancies (10%), glomerulopathies (9%), extra-renal organ transplantation (8%), and pancreatitis (3%). The frequency of complement gene rare variants was similar in patients with secondary HUS (5%) and in healthy individuals (6% and 8% in French and European controls, respectively). At diagnosis, 40% of patients required dialysis and 18% had neurological manifestations. Fifty percent of patients received plasmatherapy and 35% were treated with eculizumab. Haematological and complete renal remission was achieved in 80% and 24% of patients, respectively. Thirty-nine percent of patients progressed to chronic kidney disease (stages 3-4) and an additional 37% reached end-stage renal disease. Eleven percent of patients died, most often from complications of the underlying cause of HUS. Only one patient experienced an HUS relapse. Patients treated with eculizumab presented with more severe HUS and were more likely to require dialysis at the time of diagnosis as compared to patients not treated with eculizumab. Rates of hematological remission, chronic kidney disease (stages 3-4), and end-stage renal disease were similar in the two groups. Secondary HUS is an acute nonrelapsing form of HUS, not related to complement dysregulation. The efficacy of eculizumab in this setting is not yet established. |
| وصف الملف: | application/pdf |
| اللغة: | English |
| تدمد: | 0085-2538 1523-1755 |
| الوصول الحر: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::624be61dbab26399e3a72968f42bd5b8Test https://www.hal.inserm.fr/inserm-02158644/documentTest |
| حقوق: | OPEN |
| رقم الانضمام: | edsair.doi.dedup.....624be61dbab26399e3a72968f42bd5b8 |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 00852538 15231755 |
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