THROMBOTISCHE
THROMBOTISCHE MIKROANGIOPATHIEN Thrombozytopenie (
THROMBOTISCHE MIKROANGIOPATHIEN bozyten >30.000/µl und/oder Serumkreatinin >1,8 mg/dl ist ein schwerer ADAMTS13-Mangel allerdings unwahrscheinlich. Interessenkonflikt Prof. Dr. Schröppel erhielt Honorare als Berater und/oder Vorträge von AMGEN, Alexion Pharmaceuticals, Vifor Pharma, Pfizer, Novartis, Sanofi, Astellas Pharma sowie Honorare für Studienunterstützung (Drittmittel) von Sanofi und Pfizer. Referenzen 1. Bommer M, Wolfle-Guter M, Bohl S, Kuchenbauer F. The differential diagnosis and treatment of thrombotic microangiopathies. Dtsch Arztebl Int 2018; 115(19): 327–34. 2. Brocklebank V, Wood KM, Kavanagh D. Thrombotic microangiopathy and the kidney. Clin J Am Soc Nephrol 2018; 13(2): 300–17. 3. Goodship TH, Cook HT, Fakhouri F et al.; Conference Participants. Atypical hemolytic uremic syndrome and C3 glomerulopathy: conclusions from a “Kidney Disease: Improving Global Outcomes” (KDIGO) Controversies Conference. Kidney Int 2017; 91(3): 539–51. 4. Fakhouri F, Zuber J, Frémeaux-Bacchi V, Loirat C. Haemolytic uraemic syndrome. Lancet 2017; 390(10095): 681–96. 5. Schaefer F, Ardissino G, Ariceta G et al.; Global aHUS Registry. Clinical and genetic predictors of atypical hemolytic uremic syndrome phenotype and outcome. Kidney Int 2018; 94(2): 408–18. 6. Schönermarck U, Ries W, Schröppel B et al. Relative incidence pf thrombotic thrombocytopnic purpura and hemolytic uraemic syndrome in clinically suspected cases of thrombotic mircroangiopathy. Clincal Kidney Journal 2019; 1–9. 7. Gerber A, Karch H, Allerberger F et al. Clinical course and the role of shiga toxin-producing Escherichia coli infection in the hemolytic-uremic syndrome in pediatric patients, 1997–2000, in Germany and Austria: a prospective study. J Infect Dis 2002; 186(4): 493–500. 8. Timmermans SAMEG, Abdul-Hamid MA, Vanderlocht J et al.; Limburg Renal Registry. Patients with hypertensionassociated thrombotic microangiopathy may present with complement abnormalities. Kidney Int 2017; 91(6): 1420–5. 9. Laurence J, Haller H, Mannucci PM et al. Atypical hemolytic uremic syndrome (aHUS): essential aspects of an accurate diagnosis. Clin Adv Hematol Oncol 2016; 14 Suppl 11(11): 2–15. 10. Scully M, Hunt BJ, Benjamin S et al.; British Committee for Standards in Haematology. Guidelines on the diagnosis and management of thrombotic thrombocytopenic purpura and other thrombotic microangiopathies. Br J Haematol 2012; 158(3): 323–35. 11. Bendapudi PK, Hurwitz S, Fry A et al. Derivation and external validation of the PLASMIC score for rapid assessment of Prof. Dr. med. Bernd Schröppel bernd.schroeppel@uniklinik-ulm.de adults with thrombotic microangiopathies: a cohort study. Lancet Haematol 2017; 4(4): e157–64. 12. Nester CM, Barbour T, de Cordoba SR et al. Atypical aHUS: State of the art. Mol Immunol 2015; 67(1): 31–42. 13. Taylor CM, Machin S, Wigmore SJ, Goodship TH; working party from the Renal Association, the British Committee for Standards in Haematology and the British Transplantation Society. Clinical practice guidelines for the management of atypical haemolytic uraemic syndrome in the United Kingdom. Br J Haematol 2010; 148(1): 37–47. 14. Fremeaux-Bacchi V, Fakhouri F, Garnier A et al. Genetics and outcome of atypical hemolytic uremic syndrome: a nationwide French series comparing children and adults. Clin J Am Soc Nephrol 2013; 8(4): 554–62. 15. Noris M, Caprioli J, Bresin E et al. Relative role of genetic complement abnormalities in sporadic and familial aHUS and their impact on clinical phenotype. Clin J Am Soc Nephrol 2010; 5(10): 1844–59. 16. Bruel A, Kavanagh D, Noris M et al. Hemolytic uremic syndrome in pregnancy and postpartum. Clin J Am Soc Nephrol 2017;12(8): 1237–47. 17. Alberti M, Valoti E, Piras R et al. Two patients with history of STEC-HUS, posttransplant recurrence and complement gene mutations. Am J Transplant 2013; 13(8): 2201–6. Prof. Dr. med. Bernd Schröppel Klinik für Innere Medizin I, Sektion Nephrologie Universitätsklinikum Ulm Albert-Einstein-Allee 23, 89081 Ulm CONFERENCES 25
