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Towards a new classification of hemolytic uremic syndrome

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Complement and Kidney Disease

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References

  1. Calderwood S B, Acheson DWK, Keusch G T and Barrett T J (1996) Proposed new nomencalature for SLT (VT) family. ASM News 62: 118–119

    Google Scholar 

  2. Wong CS, Jelacic S, Habeeb RL, Watkins SL, Tarr PI (2000) The risk of the hemolyticuremic syndrome after antibiotic treatment of Escherichia coli O157:H7 infections. N Engl J Med 342: 1930–1936

    CAS  PubMed  Google Scholar 

  3. Bhimma R, Rollins NC, Coovadia HM, Adhikari M (1997) Post-dysenteric hemolytic uremic syndrome in children during an epidemic of Shigella dysentery in Kwazulu/Natal. Pediatr Nephrol 11:560–564

    CAS  PubMed  Google Scholar 

  4. Louise CB, Obrig TG (1995) Specific interaction of Escherichia coli O157:H7-derived Shiga-like toxin II with human renal endothelial cells. J Infect Dis 172: 1397–1401

    CAS  PubMed  Google Scholar 

  5. Hughes AK, Stricklett PK, Kohan DE (1998) Cytotoxic effect of Shiga toxin-1 on human proximal tubule cells. [see comment]. Kidney Intern 54: 426–437

    CAS  Google Scholar 

  6. Taylor CM, Neild GH (2005) Acute renal failure associated with microangiopathy (haemolytic-uremic syndrome and thrombotic thrombocytopenic purpura). In: AM Davison, JS Cameron, JP GrĂĽnfeld, C Ponticelli, E Ritz, CG Winearls, C van Ypersele (eds): Oxford Textbook of Clinical Nephrology. Oxford University Press, Oxford

    Google Scholar 

  7. Wiener Y, Nakhleh RE, Lee MW, Escobar FS, Venkat KK, Kupin WL, Mozes MF (1987) Prognostic factors and early resumption of cyclosporin A in renal allograft recipients with thrombotic microangiopathy and hemolytic uremic syndrome. Clin Transplant 11: 157–162

    Google Scholar 

  8. Neild GH, Reuben R, Hartley RB, Cameron JS (1985) Glomerular thrombi in renal allografts associated with cyclosporin treatment. J Clin Pathol 38: 253–258

    CAS  PubMed  Google Scholar 

  9. Shulman H, Striker G, Deeg HJ, Kennedy M, Storb R, Thomas ED (1981) Nephrotoxicity of cyclosporin A after allogeneic marrow transplantation: glomerular thromboses and tubular injury. N Engl J Med 305: 1392–1395

    CAS  PubMed  Google Scholar 

  10. Langer RM, Van Buren CT, Katz SM, Kahan BD (2002) De novo hemolytic uremic syndrome after kidney transplantation in patients treated with cyclosporine-sirolimus combination. Transplantation 73: 756–760

    CAS  PubMed  Google Scholar 

  11. Lin CC, King KL, Chao YW, Yang AH, Chang CF, Yang WC (2003) Tacrolimus-associated hemolytic uremic syndrome: a case analysis. J Nephrol 16: 580–585

    PubMed  Google Scholar 

  12. Heering P, Deppe CE, Farokhzad F, Helmchen U, Grabensee B (2002) Hemolytic uremic syndrome after renal transplantation: immunosuppressive therapy with rapamycin. Nephron 91: 177

    Article  CAS  PubMed  Google Scholar 

  13. Franco A, Hernandez D, Capdevilla L, Errasti P, Gonzalez M, Ruiz JC, Sanchez J; HUS-Sirolimus Spanish Study Group (2003) De novo hemolytic-uremic syndrome/thrombotic microangiopathy in renal transplant patients receiving calcineurin inhibitors: role of sirolimus. Transplant Proc 35: 1764–1766

    CAS  PubMed  Google Scholar 

  14. Grino JM, Caralps A, Carreras L, Nogues R, Castelao AM, Romero R, Carreras M, Alsina J (1988) Apparent recurrence of hemolytic uremic syndrome in azathioprine-treated allograft recipients. Nephron 49: 301–304

    CAS  PubMed  Google Scholar 

  15. Goodman DJ, Walker RG, Birchall IE, d’Apice AJ, Powell HR, Kincaid-Smith P (1991) Recurrent haemolytic uraemic syndrome in a transplant recipient on orthoclone (OKT 3). Pediatr Nephrol 5: 240–241

    Article  CAS  PubMed  Google Scholar 

  16. Fung MC, Storniolo AM, Nguyen B, Arning M, Brookfield W, Vigil J (1999) A review of hemolytic uremic syndrome in patients treated with gemcitabine therapy. Cancer 85: 2023–2032

    CAS  PubMed  Google Scholar 

  17. Serke S, Riess H, Oettle H, Huhn D (1999) Elevated reticulocyte count—a clue to the diagnosis of haemolytic-uraemic syndrome (HUS) associated with gemcitabine therapy for metastatic duodenal papillary carcinoma: a case report. Br J Cancer 79: 1519–1521

    Article  CAS  PubMed  Google Scholar 

  18. Gross M, Hiesse C, Kriaa F, Goldwasser F (1999) Severe hemolytic uremic syndrome in an advanced ovarian cancer patient treated with carboplatin and gemcitabine. Anti-Cancer Drugs 10: 533–536

    CAS  PubMed  Google Scholar 

  19. Muller S, Schutt P, Bojko P, Nowrousian MR, Hense J, Seeber S, Moritz T (2005) Hemolytic uremic syndrome following prolonged gemcitabine therapy: report of four cases from a single institution. Ann Hematol 84: 110–114

    CAS  PubMed  Google Scholar 

  20. Venat-Bouvet L, Ly K, Szelag JC, Martin J, Labourey JL, Genet D, Tubiana-Mathieu N (2003) Thrombotic microangiopathy and digital necrosis: two unrecognized toxicities of gemcitabine. Anti-Cancer Drugs 14: 829–832

    CAS  PubMed  Google Scholar 

  21. Walter RB, Joerger M, Pestalozzi BC (2002) Gemcitabine-associated hemolytic-uremic syndrome. Am J Kidney Dis 40: E16

    Article  PubMed  Google Scholar 

  22. Cattell V (1985) Mitomycin-induced hemolytic uremic kidney. An experimental model in the rat. Am J Pathol 121: 88–95

    CAS  PubMed  Google Scholar 

  23. Verweij J, van der Burg ME, Pinedo HM (1987) Mitomycin C-induced hemolytic uremic syndrome. Six case reports and review of the literature on renal, pulmonary and cardiac side effects of the drug. Radiother Oncol 8: 33–41

    CAS  PubMed  Google Scholar 

  24. Andersohn F, Hagmann FG, Garbe E (2004) Thrombotic thrombocytopenic purpura/haemolytic uraemic syndrome associated with clopidogrel: report of two new cases. Heart 90: e57

    Article  CAS  PubMed  Google Scholar 

  25. Manor SM, Guillory GS, Jain SP (2004) Clopidogrel-induced thrombotic thrombocytopenic purpura-hemolytic uremic syndrome after coronary artery stenting. Pharmacotherapy 24: 664–667

    PubMed  Google Scholar 

  26. Moy B, Wang JC, Raffel GD, Marcoux JP (2000) 2nd Hemolytic uremic syndrome associated with clopidogrel: a case report. Arch Intern Med 160: 1370–1372

    Article  CAS  PubMed  Google Scholar 

  27. Chinnakotla S, Leone JP, Fidler ME, Hammeke MD, Tarantolo S (2000) Clopidogrel-associated thrombotic thrombocytopenic purpura/hemolytic uremic syndrome in a kidney/pancreas transplant recipient. Transplantation 70: 550–552

    CAS  PubMed  Google Scholar 

  28. Evens AM, Kwaan HC, Kaufman DB, Bennett CL (2002) TTP/HUS occurring in a simultaneous pancreas/kidney transplant recipient after clopidogrel treatment: evidence of a nonimmunological etiology. Transplantation 74: 885–887

    PubMed  Google Scholar 

  29. Bennett CL, Connors JM, Carwile JM, Moake JL, Bell WR, Tarantolo SR, McCarthy LJ, Sarode R, Hatfield AJ, Feldman MD et al (2000) Thrombotic thrombocytopenic purpura associated with clopidogrel. N Engl J Med 342: 1773–1777

    Article  CAS  PubMed  Google Scholar 

  30. Glynne P, Salama A, Chaudhry A, Swirsky D, Lightstone L (1999) Quinine-induced immune thrombocytopenic purpura followed by hemolytic uremic syndrome. Am J Kidney Dis 33: 133–137

    CAS  PubMed  Google Scholar 

  31. Kojouri K, Vesely SK, George JN (2001) Quinine-associated thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: frequency, clinical features, and long-term outcomes. Ann Intern Med 135: 1047–1051

    CAS  PubMed  Google Scholar 

  32. McMinn JR, George JN (2001) Evaluation of women with clinically suspected thrombotic thrombocytopenic purpura-hemolytic uremic syndrome during pregnancy. J Clin Apheresis 16: 202–209

    Article  CAS  PubMed  Google Scholar 

  33. Kawasaki Y, Suzuki J, Nozawa R, Suzuki S, Suzuki H (2002) A 12-year-old girl with hemolytic uremic syndrome as initial symptom of systemic lupus erythematosus and a literature review. Am J Nephrol 22: 576–580

    Article  CAS  PubMed  Google Scholar 

  34. Tsai HM, Lian EC (1998) Antibodies to von Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpura. N Engl J Med 339: 1585–1594

    Article  CAS  PubMed  Google Scholar 

  35. Rabinowe SN, Soiffer RJ, Tarbell NJ, Neuberg D, Freedman AS, Seifter J, Blake KW, Gribben JG, Anderson KC, Takvorian T et al (1991) Hemolytic-uremic syndrome following bone marrow transplantation in adults for hematologic malignancies. Blood 77: 1837–1844

    CAS  PubMed  Google Scholar 

  36. Warwicker P, Goodship TH, Donne RL, Pirson Y, Nicholls A, Ward RM, Turnpenny P, Goodship JA (1998) Genetic studies into inherited and sporadic hemolytic uremic syndrome. Kidney Int 53: 836–844

    Article  CAS  PubMed  Google Scholar 

  37. Cameron JS, Vick R (1973) Letter: Plasma-C3 in haemolytic-uraemic syndrome and thrombotic thrombocytopenic purpura. Lancet 2: 975

    CAS  PubMed  Google Scholar 

  38. Kaplan BS, Thomson PD, MacNab GM (1973) Letter: Serum-complement levels in haemolytic-uraemic syndrome. Lancet 2: 1505–1506

    CAS  PubMed  Google Scholar 

  39. Monnens L, Molenaar J, Lambert PH, Proesmans W, van Munster P (1980) The complement system in hemolytic-uremic syndrome in childhood. Clin Nephrol 13: 168–171

    CAS  PubMed  Google Scholar 

  40. Pichette V, Querin S, Schurch W, Brun G, Lehner-Netsch G, Delage JM (1994) Familial hemolytic-uremic syndrome and homozygous factor H deficiency. Am J Kidney Dis 24: 936–941

    CAS  PubMed  Google Scholar 

  41. Noris M, Ruggenenti P, Perna A, Orisio S, Caprioli J, Skerka C, Vasile B, Zipfel PF, Remuzzi G (1999) Hypocomplementemia discloses genetic predisposition to hemolytic uremic syndrome and thrombotic thrombocytopenic purpura: role of factor H abnormalities. Italian Registry of Familial and Recurrent Hemolytic Uremic Syndrome/Thrombotic Thrombocytopenic Purpura. J Am Soc Nephrol 10: 281–293

    CAS  PubMed  Google Scholar 

  42. Whaley K, Ruddy S (1976) Modulation of C3b hemolytic activity by a plasma protein distinct from C3b inactivator. Science 193: 1011–1013

    CAS  PubMed  Google Scholar 

  43. Pangburn MK, Schreiber RD, Muller-Eberhard HJ (1977) Human complement C3b inactivator: isolation, characterization, and demonstration of an absolute requirement for the serum protein beta1H for cleavage of C3b and C4b in solution. J Exp Med 146: 257–270

    Article  CAS  PubMed  Google Scholar 

  44. Gordon DL, Kaufman RM, Blackmore TK, Kwong J, Lublin DM (1995) Identification of complement regulatory domains in human factor H. J Immunol 155: 348–356

    CAS  PubMed  Google Scholar 

  45. Kuhn S, Skerka C, Zipfel PF (1995) Mapping of the complement regulatory domains in the human factor H-like protein 1 and in factor H1. J Immunol 155: 5663–5670

    CAS  PubMed  Google Scholar 

  46. Kuhn S, Zipfel PF (1996) Mapping of the domains required for decay acceleration activity of the human factor H-like protein 1 and factor H. Eur J Immunol 26: 2383–2387

    CAS  PubMed  Google Scholar 

  47. Hellwage J, Kuhn S, Zipfel PF (1997) The human complement regulatory factor-H-like protein 1, which represents a truncated form of factor H, displays cell-attachment activity. Biochem J 326: 321–327

    CAS  PubMed  Google Scholar 

  48. DiScipio RG (1992) Ultrastructures and interactions of complement factors H and I. J Immunol 149: 2592–2599

    CAS  PubMed  Google Scholar 

  49. Blackmore TK, Sadlon TA, Ward HM, Lublin DM, Gordon DL (1996) Identification of a heparin binding domain in the seventh short consensus repeat of complement factor H. J Immunol 157: 5422–5427

    CAS  PubMed  Google Scholar 

  50. Blackmore TK, Hellwage J, Sadlon TA, Higgs N, Zipfel PF, Ward HM, Gordon DL (1998) Identification of the second heparin-binding domain in human complement factor H. J Immunol 160: 3342–3348

    CAS  PubMed  Google Scholar 

  51. Sharma AK, Pangburn MK (1996) Identification of three physically and functionally distinct binding sites for C3b in human complement factor H by deletion mutagenesis. Proc Natl Acad Sci USA 93: 10996–11001

    CAS  PubMed  Google Scholar 

  52. Zipfel PF (2001) Complement factor H: physiology and pathophysiology. Semin Thromb Hemost 27: 191–199

    Article  CAS  PubMed  Google Scholar 

  53. Zipfel PF, Skerka C (1994) Complement factor H and related proteins: an expanding family of complement-regulatory proteins? Immunol Today 15: 121–126

    Article  CAS  PubMed  Google Scholar 

  54. Ault BH, Schmidt BZ, Fowler NL, Kashtan CE, Ahmed AE, Vogt BA, Colten HR (1997) Human factor H deficiency. Mutations in framework cysteine residues and block in H protein secretion and intracellular catabolism. J Biol Chem 272: 25168–25175

    Article  CAS  PubMed  Google Scholar 

  55. Jansen JH, Hogasen K, Harboe M, Hovig T (1998) In situ complement activation in porcine membranoproliferative glomerulonephritis type II. Kidney Int 53: 331–349

    Article  CAS  PubMed  Google Scholar 

  56. Meri S, Koistinen V, Miettinen A, Tornroth T, Seppala IJ (1992) Activation of the alternative pathway of complement by monoclonal lambda light chains in membranoproliferative glomerulonephritis. J Exp Med 175: 939–950

    Article  CAS  PubMed  Google Scholar 

  57. Liszewski MK, Leung M, Cui W, Subramanian VB, Parkinson J, Barlow PN, Manchester M, Atkinson JP (2000) Dissecting sites important for complement regulatory activity in membrane cofactor protein (MCP; CD46). J Biol Chem 275: 37692–37701

    Article  CAS  PubMed  Google Scholar 

  58. Dent JA, Berkowitz SD, Ware J, Kasper CK, Ruggeri ZM (1990) Identification of a cleavage site directing the immunochemical detection of molecular abnormalities in type IIA von Willebrand factor. [erratum appears in Proc Natl Acad Sci USA Dec; 87 (23):9508]. Proc Natl Acad Sci USA 87: 6306–6310

    CAS  PubMed  Google Scholar 

  59. Tsai HM, Sussman II, Nagel RL (1994) Shear stress enhances the proteolysis of von Willebrand factor in normal plasma. Blood 83: 2171–2179

    CAS  PubMed  Google Scholar 

  60. Moake JL, Rudy CK, Troll JH, Weinstein MJ, Colannino NM, Azocar J, Seder RH, Hong SL, Deykin D (1982) Unusually large plasma factor VIII: von Willebrand factor multimers in chronic relapsing thrombotic thrombocytopenic purpura. N Engl J Med 307: 1432–1435

    CAS  PubMed  Google Scholar 

  61. Furlan M, Robles R, Galbusera M, Remuzzi G, Kyrle PA, Brenner B, Krause M, Scharrer I, Aumann V, Mittler U, Solenthaler M, Lammle B (1998) von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome. N Engl J Med 339: 1578–1584

    Article  CAS  PubMed  Google Scholar 

  62. Gerritsen HE, Turecek PL, Schwarz HP, Lammle B, Furlan M (1999) Assay of von Willebrand factor (vWF)-cleaving protease based on decreased collagen binding affinity of degraded vWF: a tool for the diagnosis of thrombotic thrombocytopenic purpura (TTP). Thromb Haemost 82: 1386–1389

    CAS  PubMed  Google Scholar 

  63. Bergstein JM, Riley M, Bang NU (1992) Role of plasminogen-activator inhibitor type 1 in the pathogenesis and outcome of the hemolytic uremic syndrome. N Engl J Med 327: 755–759

    CAS  PubMed  Google Scholar 

  64. Farr MJ, Roberts S, Morley AR, Dewar DF, Uldall PR (1975) The haemolytic uraemic syndrome — a family study. Q J Med 44: 161–188

    CAS  PubMed  Google Scholar 

  65. Edelsten AD, Tuck S (1978) Familial haemolytic uraemic syndrome. Arch Dis Child 53: 255–256

    CAS  PubMed  Google Scholar 

  66. Thompson RA, Winterborn MH (1981) Hypocomplementaemia due to a genetic deficiency of beta 1H globulin. Clin Exp Immunol 46: 110–119

    CAS  PubMed  Google Scholar 

  67. Merrill RH, Knupp CL, Jennette JC (1985) Familial thrombotic microangiopathy. Q J Med 57: 749–759

    CAS  PubMed  Google Scholar 

  68. Pirson Y, Lefebvre C, Arnout C, van Ypersele de Strihou C (1987) Hemolytic uremic syndrome in three adult siblings: a familial study and evolution. Clin Nephrol 28: 250–255

    CAS  PubMed  Google Scholar 

  69. Mattoo TK, Mahmood MA, al-Harbi MS, Mikail I (1989) Familial, recurrent hemolytic-uremic syndrome. J Pediatr 114: 814–816

    CAS  PubMed  Google Scholar 

  70. Roodhooft AM, McLean RH, Elst E, Van Acker KJ (1990) Recurrent haemolytic uraemic syndrome and acquired hypomorphic variant of the third component of complement. Pediatr Nephrol 4: 597–599

    Article  CAS  PubMed  Google Scholar 

  71. Kaplan BS, Kaplan P (1992) Hemolytic uremic syndrome in families. In: BS Kaplan, RS Trompeter, JL Moake (eds): Hemolytic uremic Syndrome and Thrombotic Thrombocytopenic Purpura. Marcel Dekker Inc., New York, 213–225

    Google Scholar 

  72. Kaplan BS, Leonard MB (2000) Autosomal dominant hemolytic uremic syndrome: variable phenotypes and transplant results. Pediatr Nephrol 14: 464–468

    CAS  PubMed  Google Scholar 

  73. Kaplan BS, Chesney RW, Drummond KN (1975) Hemolytic uremic syndrome in families. N Engl J Med 292: 1090–1093

    CAS  PubMed  Google Scholar 

  74. Kaplan BS, Papadimitriou M, Brezin JH, Tomlanovich SJ, Zulkharnain (1997) Renal transplantation in adults with autosomal recessive inheritance of hemolytic uremic syndrome. Am J Kidney Dis 30: 760–765

    CAS  PubMed  Google Scholar 

  75. Richards A, Buddles MR, Donne RL, Kaplan BS, Kirk E, Venning MC, Tielemans CL, Goodship JA, Goodship TH (2001) Factor H mutations in hemolytic uremic syndrome cluster in exons 18—20, a domain important for host cell recognition. Am J Human Genet 68: 485–490

    CAS  Google Scholar 

  76. Perkins SJ, Goodship TH (2002) Molecular modelling of the C-terminal domains of factor H of human complement: a correlation between haemolytic uraemic syndrome and a predicted heparin binding site. J Mol Biol 316: 217–224

    Article  CAS  PubMed  Google Scholar 

  77. Caprioli J, Bettinaglio P, Zipfel PF, Amadei B, Daina E, Gamba S, Skerka C, Marziliano N, Remuzzi G, Noris M and Italian Registry of Familial and Recurrent HUS/TTP (2001) The molecular basis of familial hemolytic uremic syndrome: mutation analysis of factor H gene reveals a hot spot in short consensus repeat 20. J Am Soc Nephrol 12: 297–307

    CAS  PubMed  Google Scholar 

  78. Remuzzi G, Ruggenenti P, Codazzi D, Noris M, Caprioli J, Locatelli G, Gridelli B (2002) Combined kidney and liver transplantation for familial haemolytic uraemic syndrome. Lancet 359: 1671–1672

    Article  PubMed  Google Scholar 

  79. Sanchez-Corral P, Perez-Caballero D, Huarte O, Simckes AM, Goicoechea E, Lopez-Trascasa M, de Cordoba SR (2002) Structural and functional characterization of factor H mutations associated with atypical hemolytic uremic syndrome. Am J Human Genet 71: 1285–1295

    CAS  Google Scholar 

  80. Neumann HP, Salzmann M, Bohnert-Iwan B, Mannuelian T, Skerka C, Lenk D, Bender BU, Cybulla M, Riegler P, Konigsrainer A et al (2003) Haemolytic uraemic syndrome and mutations of the factor H gene: a registry-based study of German speaking countries. J Med Genet 40: 676–681

    Article  CAS  PubMed  Google Scholar 

  81. Dragon-Durey MA, Loirat C, Cloarec S, Macher MA, Blouin J, Nivet H, Weiss L, Fridman WH, Fremeaux-Bacchi V (2005) Anti-factor H autoantibodies associated with atypical hemolytic uremic syndrome. J Am Soc Nephrol 16: 555–563

    Article  CAS  PubMed  Google Scholar 

  82. Richards A, Kemp EJ, Liszewski MK, Goodship JA, Lampe AK, Decorte R, Muslumanoglu MH, Kavukcu S, Filler G, Pirson Y et al (2003) Mutations in human complement regulator, membrane cofactor protein (CD46), predispose to development of familial hemolytic uremic syndrome. Proc Natl Acad Sci USA 100: 12966–12971

    CAS  PubMed  Google Scholar 

  83. Noris M, Brioschi S, Caprioli J, Todeschini M, Bresin E, Porrati F, Gamba S, Remuzzi G and International Registry of Recurrent and Familial (2003) Familial haemolytic uraemic syndrome and an MCP mutation. Lancet 362: 1542–1547

    Article  CAS  PubMed  Google Scholar 

  84. Antoine G, Zimmermann K, Plaimauer B, Grillowitzer M, Studt JD, Lammle B, Scheiflinger F (2003) ADAMTS13 gene defects in two brothers with constitutional thrombotic thrombocytopenic purpura and normalization of von Willebrand factor-cleaving protease activity by recombinant human ADAMTS13. Br J Haematol 120: 821–824

    Article  CAS  PubMed  Google Scholar 

  85. Levy GG, Nichols WC, Lian EC, Foroud T, McClintick JN, McGee BM, Yang AY, Siemieniak DR, Stark KR, Gruppo R et al (2001) Mutations in a member of the ADAMTS gene family cause thrombotic thrombocytopenic purpura. Nature 413: 488–494

    Article  CAS  PubMed  Google Scholar 

  86. Fujikawa K, Suzuki H, McMullen B, Chung D (2001) Purification of human von Willebrand factor-cleaving protease and its identification as a new member of the metalloproteinase family. Blood 98: 1662–1666

    Article  CAS  PubMed  Google Scholar 

  87. Gerritsen HE, Robles R, Lammle B, Furlan M (2001) Partial amino acid sequence of purified von Willebrand factor-cleaving protease. Blood 98: 1654–1661

    Article  CAS  PubMed  Google Scholar 

  88. Kentouche K, Budde U, Furlan M, Scharfe V, Schneppenheim R, Zintl F (2002) Remission of thrombotic thrombocytopenic purpura in a patient with compound heterozygous deficiency of von Willebrand factor-cleaving protease by infusion of solvent/detergent plasma. [see comment]. Acta Paediatrica 91: 1056–1059

    Article  CAS  PubMed  Google Scholar 

  89. Kokame K, Matsumoto M, Soejima K, Yagi H, Ishizashi H, Funato M, Tamai H, Konno M, Kamide K, Kawano Y et al (2002) Mutations and common polymorphisms in ADAMTS13 gene responsible for von Willebrand factor-cleaving protease activity. Proc Natl Acad of Sci USA 99: 11902–11907

    CAS  Google Scholar 

  90. Savasan S, Lee SK, Ginsburg D, Tsai HM (2003) ADAMTS13 gene mutation in congenital thrombotic thrombocytopenic purpura with previously reported normal VWF cleaving protease activity. Blood 101: 4449–4451

    Article  CAS  PubMed  Google Scholar 

  91. Assink K, Schiphorst R, Allford S, Karpman D, Etzioni A, Brichard B, van de Kar N, Monnens L, van den Heuvel L (2003) Mutation analysis and clinical implications of von Willebrand factor-cleaving protease deficiency. Kidney Int 63: 1995–1999

    Article  CAS  PubMed  Google Scholar 

  92. Schneppenheim R, Budde U, Oyen F, Angerhaus D, Aumann V, Drewke E, Hassenpflug W, Haberle J, Kentouche K, Kohne E et al. (2003) von Willebrand factor cleaving protease and ADAMTS13 mutations in childhood TTP. Blood 101: 1845–1850

    Article  CAS  PubMed  Google Scholar 

  93. Matsumoto M, Kokame K, Soejima K, Miura M, Hayashi S, Fujii Y, Iwai A, Ito E, Tsuji Y, Takeda-Shitaka M et al (2004) Molecular characterization of ADAMTS13 gene mutations in Japanese patients with Upshaw-Schulman syndrome. Blood 103: 1305–1310

    CAS  PubMed  Google Scholar 

  94. Uchida T, Wada H, Mizutani M, Iwashita M, Ishihara H, Shibano T, Suzuki M, Matsubara Y, Soejima K, Matsumoto M et al and Research Project on Genetics of Thrombosis (2004) Identification of novel mutations in ADAMTS13 in an adult patient with congenital thrombotic thrombocytopenic purpura. Blood 104: 2081–2083

    Article  CAS  PubMed  Google Scholar 

  95. Licht C, Stapenhorst L, Simon T, Budde U, Schneppenheim R, Hoppe B (2004) Two novel ADAMTS13 gene mutations in thrombotic thrombocytopenic purpura/hemolyticuremic syndrome (TTP/HUS). Kidney Int 66: 955–958

    Article  CAS  PubMed  Google Scholar 

  96. Perez-Caballero D, Gonzalez-Rubio C, Gallardo ME, Vera M, Lopez-Trascasa M, Rodriguez de Cordoba S, Sanchez-Corral P (2001) Clustering of missense mutations in the C-terminal region of factor H in atypical hemolytic uremic syndrome. Am J Hum Genet 68: 478–484.

    CAS  PubMed  Google Scholar 

  97. Loirat C, Niaudet P (2003) The risk of recurrence of hemolytic uremic syndrome after renal transplantation in children. Pediatr Nephrol 18: 1095–1101

    Article  PubMed  Google Scholar 

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Salzmann, M., Hoffmann, M., Schluh, G., Riegler, P., Cybulla, M., Neumann, H.P. (2006). Towards a new classification of hemolytic uremic syndrome. In: Zipfel, P.F. (eds) Complement and Kidney Disease. Progress in Inflammation Research. Birkhäuser Basel. https://doi.org/10.1007/3-7643-7428-4_8

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