Atypical hemolytic uremic syndrome in the era of terminal complement inhibition: an observational cohort study

التفاصيل البيبلوغرافية
العنوان: Atypical hemolytic uremic syndrome in the era of terminal complement inhibition: an observational cohort study
المؤلفون: Brocklebank, Vicky, Walsh, Patrick R., Smith-Jackson, Kate, Hallam, Thomas M., Marchbank, Kevin J., Wilson, Valerie, Bigirumurame, Theophile, Dutt, Tina, Montgomery, Emma K., Malina, Michal, Wong, Edwin K. S., Johnson, Sally, Sheerin, Neil S., Kavanagh, David, Holt, Richard, Jones, Caroline, Ashman, Neil, Fan, Stanley, Forbes, Suzanne, Rajakariar, Ravindra, De Freitas, Declan, Magee, Colm, Hanko, Jennifer, Milford, David, Muorah, Mordi, Howie, Alexander J., Baharani, Jyoti, Dasgupta, Indranil, Roberts, Russell, MacDiarmaid-Gordon, Adam, Fry, Andrew, Torpey, Nicholas, Waldron, Mary, Awan, Atif, Raftery, Tara, Ratcliffe, Laura, Vilar, Enric, Newman, Joel, Seviar, Dale, Marks, Stephen D., Rees, Lesley, Veligratli, Faidra, Waters, Aoife, Chowdhury, Paramit, Pattison, James, Booth, Caroline, Williams, Nicole, Corbett, Richard, Amin, Beena, Pickering, Matthew, Bramham, Kate, Al-Hamed, Mohamed, Hill, Anita, Mooney, Andrew, Finlay, Eric, Newnham, Amanda, Yadav, Pallavi, Kaur, Amrit, Plant, Nicholas, Shenoy, Mohan, Choong, Lye Wai, Helps, Aileen, McLemon, Robert, Muniraju, Thalakunte, Khan, Izhar, Kidder, Dana, Padmanabhan, Neal, Cousland, Zoe, Price, Jonathan, Taylor, Alison, Jennings, Claudine, Ahmad, Sohail, Mead, Paul, Aslam, Mona, Bebb, Charlotte, Byrne, Catherine, Ferraro, Alastair, Christian, Martin, Evans, Jonathan, Kim, Jon Jin, Lunn, Andrew, Mallik, Meeta, Mason, Phil, Mole, David, Craze, Janet, Sangala, Nicholas, Uniacke, Mark, McGregor, Ellon, Lambie, Stewart, Bhandary, Nitin, Flossmann, Oliver, Mohteshamzadeh, Mobin, Abeyaratne, Asanga, Bingham, Coralie, Clissold, Rhian, Smyth, Lucy, Connolly, John, Reynolds, Ben, Neary, John, Bell, Joanne, Roxburgh, Sarah, Popoola, Joyce, Donne, Rosie, Fardon, Nicholas, Khwaja, Arif, Siddiqi, Shareen, Kumar, Gurinder, Kardasz, Steve, Moore, Iain, Tez, Didem, Willows, Jamie, Davison, Rachel, Harty, John, Wong, William, Williams, Andrew J., Gale, Daniel, Coward, Richard, Inward, Carol, Mraz, Martin, Ayub, Waqar, Short, Andrew, Lappin, David, Baines, Richard, Bommayya, Girish, Houlberg, Kris, Saif, Imran, Gilbert, Rodney, Griffin, Sian, Smith, Graham, Van Der Voort, Judith, Chandar, Jayanthi, Freundlich, Michael, McCarroll, Frank, Wong, Germaine, Laboi, Paul, Molyneux, Rebekah
المصدر: Blood; 20230101, Issue: Preprints
مستخلص: •We prove in genotype-matched complement-mediated aHUS cohorts that C5 inhibition results in a statistical improvement in ESKD-free survival.•We demonstrate that biallelic pathogenic mutations in EXOSC3cause eculizumab nonresponsive aHUS.
قاعدة البيانات: Supplemental Index
الوصف
تدمد:00064971
15280020
DOI:10.1182/blood.2022018833