Gianluigi Ardissino1, Francesca Tel2, Sara Testa2, Fabio Paglialonga2, Selena Longhi2, Laura Martelli2, Silvia Consolo2, Damiano Picicco3, Antonella Dodaro3, Laura Daprai3, Rosaria Colombo3, Milena Arghittu3, Michela Perrone4, Giovanna Chidini5, Stefano Scalia Catenacci5, Isabella Cropanese6, Dario Consonni7. 1. Center for HUS Prevention Control and Management, Pediatric Nephrology, Dialysis and Transplantation Unit, IRCCS Foundation Ca' Granda, Ospedale Maggiore Policlinico, Milan, Italy. ardissino@centroseu.org. 2. Center for HUS Prevention Control and Management, Pediatric Nephrology, Dialysis and Transplantation Unit, IRCCS Foundation Ca' Granda, Ospedale Maggiore Policlinico, Milan, Italy. 3. Center for HUS Prevention Control and Management, Laboratory of Microbiology, IRCCS Foundation Ca' Granda, Ospedale Maggiore Policlinico, Milan, Italy. 4. Center for HUS Prevention Control and Management, Neonatal Intensive Care Unit, IRCCS Foundation Ca' Granda, Ospedale Maggiore Policlinico, Milan, Italy. 5. Center for HUS Prevention Control and Management, Maternal-Child Anesthesia and Intensive Care Departement, IRCCS Foundation Ca' Granda, Ospedale Maggiore Policlinico, Milan, Italy. 6. Center for HUS Prevention Control and Management, Child and Adolescent Neuropsychiatric Unit, IRCCS Foundation Ca' Granda, Ospedale Maggiore Policlinico, Milano, Italy. 7. Center for HUS Prevention Control and Management at the Epidemiology Unit, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Milano, Italy.
Abstract
Shigatoxin Escherichia coli-related hemolytic uremic syndrome (eHUS) is a severe thrombotic microangiopathy (TMA) burdened by life-threatening complications and long-term sequelae. Since hemoconcentration is associated with worse outcome, we tried to develop a reliable and easy-to-calculate index for predicting complications and sequelae based on hemoglobin (Hb) at presentation. The first laboratory examinations with signs of TMA in eHUS patients were analyzed in relation to the outcomes with the receiver operating characteristic curves and their areas under the curve (AUC) for Hb and creatinine (sCr). A total of 197 eHUS patients were identified of whom 24% did not have anemia at presentation. Hb level was the best predictor of a poor outcome (AUC 0.67) but the combination of Hb with sCr, in the formula [(Hb in g/dL + (sCr in mg/dL × 2)], showed an even better AUC of 0.75. The described scoring system was also strongly associated and predictive of all complications and health care needs (8% of patients with scoring > 13 died or entered a permanent vegetative state compared with 0% of those with ≤ 13). Conclusion: The presented score is a simple and early predictor of both short- and long-term outcomes and identifies patients who should undergo rapid volume expansion to counteract hemoconcentration, the spreading of microvascular thrombosis, and the consequent increased organ damage. What is Known: • In eHUS, hemoconcentration is associated with worse short- and long-term outcome. • A prognostic index to identify patients at higher risk for complications at presentation is not available. What is New: • We developed a simple and early prognostic index for eHUS outcome with the combination of Hb and sCr at onset, in the following formula [(Hb in g/dL + (sCr in mg/dL × 2)]. • The proposed HUS Severity Score can promptly identify patients with good outcome and those with high risk of worse short- and long-term outcome.
Shigatoxin Escherichia coli-related hemolytic uremic syndrome (eHUS) is a severe thrombotic microangiopathy (TMA) burdened by life-threatening complications and long-term sequelae. Since hemoconcentration is associated with worse outcome, we tried to develop a reliable and easy-to-calculate index for predicting complications and sequelae based on hemoglobin (Hb) at presentation. The first laboratory examinations with signs of TMA in eHUS patients were analyzed in relation to the outcomes with the receiver operating characteristic curves and their areas under the curve (AUC) for Hb and creatinine (sCr). A total of 197 eHUS patients were identified of whom 24% did not have anemia at presentation. Hb level was the best predictor of a poor outcome (AUC 0.67) but the combination of Hb with sCr, in the formula [(Hb in g/dL + (sCr in mg/dL × 2)], showed an even better AUC of 0.75. The described scoring system was also strongly associated and predictive of all complications and health care needs (8% of patients with scoring > 13 died or entered a permanent vegetative state compared with 0% of those with ≤ 13). Conclusion: The presented score is a simple and early predictor of both short- and long-term outcomes and identifies patients who should undergo rapid volume expansion to counteract hemoconcentration, the spreading of microvascular thrombosis, and the consequent increased organ damage. What is Known: • In eHUS, hemoconcentration is associated with worse short- and long-term outcome. • A prognostic index to identify patients at higher risk for complications at presentation is not available. What is New: • We developed a simple and early prognostic index for eHUS outcome with the combination of Hb and sCr at onset, in the following formula [(Hb in g/dL + (sCr in mg/dL × 2)]. • The proposed HUS Severity Score can promptly identify patients with good outcome and those with high risk of worse short- and long-term outcome.
Authors: Sebastian Loos; Thurid Ahlenstiel; Brigitta Kranz; Hagen Staude; Lars Pape; Christoph Härtel; Udo Vester; Laura Buchtala; Kerstin Benz; Bernd Hoppe; Ortraud Beringer; Martin Krause; Dominik Müller; Martin Pohl; Johanna Lemke; Georg Hillebrand; Martin Kreuzer; Jens König; Marianne Wigger; Martin Konrad; Dieter Haffner; Jun Oh; Markus J Kemper Journal: Clin Infect Dis Date: 2012-06-05 Impact factor: 9.079
Authors: Robert S Oakes; Justin K Kirkham; Justin K Kirkhamm; Raoul D Nelson; Richard L Siegler Journal: Pediatr Nephrol Date: 2008-05-09 Impact factor: 3.714
Authors: Chu Yang Lin; Jianling Xie; Stephen B Freedman; Ryan S McKee; David Schnadower; Phillip I Tarr; Yaron Finkelstein; Neil M Desai; Roni D Lane; Kelly R Bergmann; Ron L Kaplan; Selena Hariharan; Andrea T Cruz; Daniel M Cohen; Andrew Dixon; Sriram Ramgopal; Elizabeth C Powell; Jennifer Kilgar; Kenneth A Michelson; Martin Bitzan; Kenneth Yen; Garth D Meckler; Amy C Plint; Fran Balamuth; Stuart Bradin; Serge Gouin; April J Kam; James A Meltzer; Tracy E Hunley; Usha Avva; Robert Porter; Daniel M Fein; Jeffrey P Louie; Gillian A M Tarr Journal: J Pediatr Date: 2021-01-05 Impact factor: 4.406
Authors: Sebastian Loos; Jun Oh; Laura van de Loo; Markus J Kemper; Martin Blohm; Raphael Schild Journal: Pediatr Nephrol Date: 2021-05-27 Impact factor: 3.714