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References
Warwicker P, Goodship THJ, 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
Ohali M, Shalev H, Schlesinger M, Katz Y, Kachko L, Carmi R, Sofer S, Landau D (1998) Hypocomplementemic autosomal recessive hemolytic uremic syndrome with decreased factor H. Pediatr Nephrol 12: 619–624
Pichette V, Quérin S, Schürch W, Brun G, Lehner-Netsch G, Deläge JM (1994) Familial hemolytic-uremic syndrome and homozygous factor H deficiency. Am J Kidney Dis 24: 936–941
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
Thompson RA, Winterborn MH (1981) Hypocomplementaemia due to a genetic deficiency of beta 1H globulin. Clin Exp Immunol 46:110–119
Goodship TH, Liszewski MK, Kemp EJ, Richards A, Atkinson JP (2004) Mutations in CD46, a complement regulatory protein, predispose to atypical HUS. Trends Mol Med 10: 226–231
Noris M, Brioschi S, Caprioli J, Todeschini M, Bresin E, Porrati F, Gamba S, Remuzzi G (2003) Familial haemolytic uraemic syndrome and an MCP mutation. Lancet 362: 1542–1547
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
Ault BH (2000) Factor H and the pathogenesis of renal disease. Pediatr Nephrol 14: 1045–1053
Brai M, Misiano G, Maringhini S, Cutaja I, Hauptmann G (1988) Combined homozygous factor H and heterozygous C2 deficiency in an Italian family. J Clin Immunol 8: 50–56
Fijen C, Kuijper E, Holdrinet A, Daha M, Dankert J (1992) Factor H deficiency in a Dutch family. Immunobiology 184: 427
Lopez-Larrea C, Dieguez M, Enguix A, Dominguez O, Marin B, Gomez F (1987) A familial deficiency of complement factor H. Biochem Soc Trans 15: 648–649
Nielsen HE, Christensen KC, Koch C, Thomas BS, Heegard NH, Tranum-Jensen J (1989) Hereditary, complete deficiency of complement factor H associated with recurrent meningococcal disease. Scand J Immunol 30: 711–718
Rougier N, Kazatchkine MD, Rougier JP, Fremeaux-Bacchi V, Blouin J, Deschenes G, Soto B, Baudouin V, Pautard B, Proesmans W et al (1998) Human complement factor H deficiency associated with hemolytic uremic syndrome. J Am Soc Nephrol 9: 2318–2326
Friese MA, Hellwage J, Jokiranta TS, Meri S, Müller-Quernheim HJ, Peter HH, Eibel H, Zipfel PF (2000) Different regulation of factor H and FHL-1/reconectin by inflammatory mediators and expression of the two proteins in rheumatoid arthritis (RA). Clin Exp Immunol 121: 406–415
Friese MA, Manuelian T, Junnikkala S, Hellwage J, Meri S, Peter HH, Gordon DL, Eibel H, Zipfel PF (2003) Release of endogenous anti-inflammatory complement regulators FHL-1 and factor H protects synovial fibroblasts during rheumatoid arthritis. Clin Exp Immunol 132: 485–495
Junnikkala S, Hakulinen J, Jarva H, Manuelian T, Bjorge L, Butzow R, Zipfel PF, Meri S (2002) Secretion of soluble complement inhibitors factor H and factor H-like protein (FHL-1) by ovarian tumor cells. Br J Cancer 87: 1119–1127
Junnikkala S, Jokiranta TS, Friese MA, Jarva H, Zipfel PF, Meri S (2000) Exceptional resistance of human H2 glioblastoma cells to complement-mediated killing by expression and utilization of factor H and factor H-like protein 1. J Immunol 164: 6075–6081
Devine DV, Siegel RS, Rosse WF (1987) Interactions of the platelets in paroxysmal nocturnal hemoglobinuria with complement. Relationship to defects in the regulation of complement and to platalet survival in vivo. J Clin Invest 79: 131–137
DiScipio RG, Daffern PJ, Schraufstatter IU, Sriramarao P (1998) Human polymor-phonuclear leukocytes adhere to complement factor H through an interaction that involves alphaMbeta2 (CD11b/CD18). J Immunol 160: 4057–4066
Manuelian T, Hellwage J, Meri S, Caprioli J, Noris M, Heinen S, Jozsi M, Neumann HPG, Remuzzi G, Zipfel PF (2003) Mutations in factor H reduce binding affinity to C3b and heparin and surface attachment to endothelial cells in hemolytic uremic syndrome. J Clin Invest 111: 1181–1190
Berner R, Krause MF, Gordjani N, Zipfel PF, Boehm N, Krueger M, Brandis M, Zimmerhackl LB (2002) Hemolytic uremic syndrome due to an altered factor H triggered by neonatal pertussis. Pediatr Nephrol 17: 190–192
Filler G, Radhakrishnan S, Strain L, Hill A, Knoll G, Goodship TH (2004) Challenges in management of infantile factor H associated haemolytic uremic syndrome. Pediatr Nephrol 19: 908–911
Gerber A, Kirchhoff-Moradpour AH, Obieglo S, Brandis M, Kirschfink M, Zipfel PF, Goodship JA, Zimmerhackl LB (2003) Successful (?) therapy of hemolytic-uremic syndrome with factor H abnormality. Pediatr Nephrol 18: 952–955
Olie KH, Florquin S, Groothoff JW, Verlaak R, Strain L, Goodship THJ, Weening J, Davin JC (2004) Atypical relapse of hemolytic uremic syndrome after transplantation. Pediatr Nephrol 19: 1173–1176
Licht C, Heinen S, Józsi M, Löschmann I, Saunders RE, Perkins SJ, Waldherr R, Skerka C, Kirschfink M, Hoppe B, Zipfel PF (2005) Deletion of a single amino acid (K224) in the regulatory domain of factor H reveals a novel pathomechanism for membranoproliferative glomerulonephritis type II. Submitted
Dragon-Durey MA, Loirat C, Cloarec S, Macher MA, Blouin J, Nivet H, Weiss L, Fridman WH, Frémeaux-Bacchi V (2005) Anti-factor H autoantibodies associated with atypical hemolytic uremic syndrome. J Am Soc Nephrol 16: 555–563
Kavanagh D, Kemp EJ, Mayland E, Winney RJ, Duffield JS, Warwick G, Richards A, Ward R, Goddship JA, Goddship TH (2005) Mutations in complement factor I predispose to development of atypical hemolytic uremic syndrome. J Am Soc Nephrol 16: 2150–2155
Abbas AK, Lichtman AH, Pober JS (2000) Effector mechanisms of humoral immunity. In: Cellular and Molecular Immunology, 4th edition. WB Saunders Company, Philadelphia, 309–339
Walport MJ (2001) Complement (first of two parts). N Engl J Med 344: 1058–1066
Walport MJ (2001) Complement (second of two parts). N Engl J Med 344: 1140–1144
Young NS (2005) Paroxysmal nocturnal hemoglobinuria: current issues in pathophysiology and treatment. Curr Hematol Rep 4: 103–109
Zipfel PF (2001) Complement factor H: physiology and pathophysiology. Semin Thromb Hemost 27: 191–199
Zipfel PF (2001) Hemolytic uremic syndrome: how do factor H mutants mediate endothelial damage? Trends Immunol 22: 345–348
Zipfel PF, Jokiranta TS, Hellwage J, Koistinen V, Meri S (1999) The factor H protein family. Immunopharmacology 42: 53–60
Zipfel PF, Skerka C (1999) FHL-1/reconectin: a human complement and immune regulator with cell-adhesive function. Immunol Today 20: 135–140
Jószi M, Manuelian T, Heinen S, Oppermann M, Zipfel PF (2004) Attachment of the soluble complement regulator factor H to the cell and tissue surfaces: relevance for pathology. Histol Histopathol 19: 251–258
Quigg RJ (2003) Complement and the kidney. J Immunol 171: 3319–3324
Sánchez-Corral P, Pérez-Caballero D, Huarte O, Simckes AM, Goicoechea E, López-Trascasa M, Rodríguez de Córdoba S (2002) Structural and functional characterization of factor H mutations associated with atypical hemolytic uremic syndrome. Am J Hum Genet 71: 1285–1295
Daha MR, Fearon DT, Austen KF (1976) C3nephritic factor (C3NeF): stabilization of fluid phase and cell-bound alternative pathway convertase. J Immunol 116: 1–7
Mathieson PW, Würzner R, Oliviera DBG, Lachmann PJ, Peters DK (1993) Complement-mediated adipocyte lysis by nephritic factor sera. J Exp Med 177: 1827–1831
Ruggenenti P, Noris M, Remuzzi G (2001) Thrombotic microangiopathy, hemolytic uremic syndrome, and thrombotic thrombocytopenic purpura. Kidney Int 60: 831–846
Symmers WS (1952) Thrombotic microangiopathic haemolytic anemia (thrombotic microangiopathy). Br Med J 2: 897–903
Gasser C, Gautier E, Steck A, Siebenmann RE, Oechslin R (1955) Hemolytic-uremic syndrome: bilateral necrosis of the renal cortex in acute acquired hemolytic anemia. Schweiz Med Wochenschr 85: 905–909
Moschcowitz E (1924) Hyaline thrombosis of the terminal arterioles and capillaries: a hitherto undescribed disease. Proc NY Pathol Soc 24: 21–24
Moake JL (2002) Thrombotic microangiopathies. N Engl J Med 347: 589–600
Donne RL, Abbs I, Barany P, Elinder CG, Little M, Conlon P, Goodship THJ (2002) Recurrence of hemolytic uremic syndrome after live related renal transplantation associated with subsequent de novo disease in the donor. Am J Kidney Dis 40: E22
Loirat C, Niaudet P (2003) The risk of recurrence of hemolytic uremic syndrome after renal transplantation in children. Pediatr Nephrol 18: 1095–1101
Neuman HPH, Salzmann M, Bohnert-Iwan B, Mannuelian T, Skerka C, Lenk D, Bender BU, Cybulla M, Riegler P, Königsrainer 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
Taylor CM, Chua C, Howie AJ, Ridson RA (2004) Clinico-pathological findings in diarrhea negative haemolytic uraemic syndrome. Pediatr Nephrol 19: 419–425
Hughes AK, Ergonul Z, Stricklett PK, Kohan DE (2002) Molecular basis for high renal cell sensitivity to the cytotoxic effects of shigatoxin-1: upregulation of globotriaosylceramide expression. J Am Soc Nephrol 13:2239–2245
Hughes AK, Stricklett PK, Schmid D, Kohan DE (2000) Cytotoxic effect of Shiga toxin-1 on human glomerular epithelial cells. Kidney Int 57:2350–2359
Tsai HM (2003) Advances in the pathogenesis, diagnosis, and treatment of thrombotic thrombocytopenic purpura. J Am Soc Nephrol 14: 1072–1081
Mathieson P (2002) Complement factor H and haemolytic uraemic syndrome. Lancet 359: 801–802
Richards A, Goodship JA, Goodship TA (2002) The genetics and pathogenesis of haemolytic uraemic syndrome and thrombotic thrombocytopenic purpura. Curr Opin Nephrol Hypertens 11: 431–435
Taylor CM (2001) Complement factor H and the haemolytic uraemic syndrome. Lancet 358: 1200–1202
Taylor CM (2001) Hemolytic-uremic syndrome and complement factor H deficiency: clinical aspects. Semin Thromb Hemost 27: 185–190
Zipfel PF, Hellwage J, Friese MA, Hegasy G, Jokiranta ST, Meri S (1999) Factor H and disease: a complement regulator affects vital body function. Mol Immunol 36: 241–248
Zipfel PF, Skerka C, Caprioli J, Manuelian T, Neumann HH, Noris M, Remuzzi G (2001) Complement factor H and hemolytic uremic syndrome. Int Immunopharmacol 1: 461–468
Kaplan BS, Chesney RW, Drummond KN (1975) Hemolytic uremic syndrome in families. N Engl J Med 292: 1090–1093
Kaplan BS, Meyers KE, Schulman SL (1998) The pathogenesis of haemolytic uremic syndrome. J Am Soc Nephrol 9: 1126–1133
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. J Am Soc Nephrol 10: 281–293
Ying L, Katz Y, Schlesinger M, Carmi R, Shalev H, Haider N, Beck G, Sheffield VC, Landau D (1999) Complement factor H gene mutation associated with autosomal recessive atypical haemolytic uremic syndrome. Am J Hum Genet 65: 1538–1546
Buddles MRH, Donne RL, Richards A, Goodship J, Goodship THJ (2000) Complement factor H gene mutation associated with autosomal recessive atypical hemolytic uremic syndrome. Am J Hum Genet 66: 1721–1722
Caprioli J, Bettinaglio P, Zipfel PF, Amadei B, Daina E, Gamba S, Skerka C, Marziliano N, Remuzzi G, Noris M (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
Dragon-Durey MA, Frémeaux-Bacchi V, Loirat C, Blouin J, Niaudet P, Deschenes G, Coppo P, Fridman WH, Weiss L (2004) Heterozygous and homozygous factor H deficiencies associated with hemolytic uremic syndrome or membranoproliferative glomerulonephritis: report and genetic analysis of 16 cases. J Am Soc Nephrol 15: 787–795
Landau D, Shalev H, Levy-Finer G, Polonsky A, Segev Y, Katchko L (2001) Familial hemolytic uremic syndrome associated with complement factor H deficiency. J Pediatr 138: 412–417
Licht C, Weyersberg A, Heinen S, Stapenhorst L, Devenge J, Beck B, Waldherr R, Kirschfink M, Zipfel PF, Hoppe B (2005) Successful plasma therapy for atypical hemolytic uremic syndrome (aHUS) caused by factor H deficiency due to a mutation in the complement cofactor protein (CCP) domain 15. Am J Kidney Dis 45: 415–421
Pérez-Caballero D, González-Rubio C, Gallardo ME, Vera M, López-Trascasa M, Rodríguez de Córdoba S, Sánchez-Corral P (2001) Clustering of missense mutations in the C-terminal region of factor H in atypical haemolytic uremic syndrome. Am J Hum Genet 68: 478–484
Richards A, Buddles MR, Donne RL, Kaplan BS, Kirk E, Venning MC, Tielemans CL, Goodship JA, Goodship THJ (2001) Factor H mutations in hemolytic uremic syndrome cluster in exons 18-20, a domain important for host cell recognition. Am J Hum Genet 68:485–490
Warwicker P, Donne RL, Goodship JA, Goodship THJ, Howie AJ, Kumararatne DS, Thompson RA, Taylor CM (1999) Familial relapsing haemolytic uraemic syndrome and complement factor H deficiency. Nephrol Dial Transplant 14: 1229–1233
Wyatt RJ, Julian BA, Weinstein A, Rothfield NF, McLean RH (1982) Partial H (beta 1H) deficiency and glomerulonephritis in two families. J Clin Immunol 2: 110–117
Zipfel PF, Neumann HPH, Jozsi M (2003) Genetic screening in haemolytic uraemic syndrome. Curr Opin Nephrol Hypertens 12: 653–657
Zipfel PF, Skerka C, Hellwage J, Jokiranta ST, Meri S, Brade V, Kraiczy P, Noris M, Remuzzi G (2002) Factor H family proteins: on complement, microbes and human diseases. Biochem Soc Trans 30: 971–978
Caprioli J, Castelletti F, Bucchioni S, Bettinaglio P, Bresin E, Pianetti G, Gamba S, Brioschi S, Daina E, Remuzzi G, Noris M (2003) Complement factor H mutations and gene polymorphisms in haemolytic uraemic syndrome: the C-257T, the A2089G and the G2881T polymorphisms are strongly associated with the disease. Human Molecular Genetics 12: 3385–3395
Pangburn MK (2002) Localization of the host recognition functions of complement factor H at the carboxyl-terminal: implications for hemolytic uremic syndrome. J Immunol 169: 4702–2706
Hellwage J, Jokiranta TS, Friese MA, Wolk TU, Kampen E, Zipfel PF, Meri S (2002) Complement C3b/C3d and cell surface polyanions are recognized by overlapping binding sites on the most carboxyl-terminal domain of complement factor H. J Immunol 169: 6935–6944
Sánchez-Corral P, González-Rubio C, Rodríguez de Córdoba S, López-Trascasa M (2004) Functional analysis in serum from atypical hemolytic uremic syndrome patients reveals impaired protection of host cells associated with mutations in factor H. Mol Immunol 41: 81–84
Perkins SJ, Goodship THJ (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
Furlan M, Laemmle B (2000) Haemolytic-uraemic syndrome and thrombotic thrombocytopenic purpura — new insights into underlying biochemical mechanisms. Nephrol Dial Transplant 15: 1112–1114
Furlan M, Robles R, Galbusera M, Remuzzi G, Kyrle PA, Brenner B, Krause M, Scharrer I, Aumann V, Mittler U et al (1998) Von Willebrand factor-cleaving protease in thrombotic-thrombocytopenic purpura and the hemolytic-uremic syndrome. N Engl J Med 339: 1578–1584
Furlan M, Robles R, Solenthaler M, Wassmer M, Sandoz P, Lammle B (1997) Deficient activity of von Willebrand factor-cleaving protease in chronic relapsing thrombotic thrombocytopenic purpura. Blood 89: 3097–3103
Schneppenheim R, Budde U, Oyen F, Angerhaus D, Aumann V, Drewke E, Hassenpflug W, Häberle J, Kentouche K, Kohne E et al (2003) Von Willebrand factor cleaving protease and ADAMTS13 mutations in childhood TTP. Blood 101: 1845–1850
Furlan M, Robles R, Solenthaler M, Lammle B (1998) Acquired deficiency of von Willebrand factor-cleaving protease in a patient with thrombotic thrombocytopenic purpura. Blood 91: 2839–2846
Rieger M, Mannucci PM, Hovinga JA, Herzog A, Gerstenbauer G, Konetschny C, Zimmermann K, Scharrer I, Peyvandi F, Galbusera M et al (2005) ADAMTS13 autoantibodies in patients with thrombotic microangiopathies and other immunomediated diseases. Blood May 17 [Epub ahead of print]
Tsai HM, Lian ECY (1998) Antibodies to von Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpura. N Engl J Med 339: 1585–1594
Levy GG, Nichols WC, Lian EC, Foroud T, McClintick JN, Mc Gee 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
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
Noris M, Bucchioni S, Galbusera M, Donadelli R, Bresin E, Castelletti F, Caprioli J, Brioschi S, Scheiflinger F, Remuzzi G (2005) Complement factor H mutation in familial thrombotic thrombocytopenic purpura with ADAMTS13 deficiency and renal involvement J Am Soc Nephrol 16: 1177–1183
Schärer K (2002) Membranoproliferative Glomerulonephritis. In: K Schärer, O Mehls (eds): Pädiatrische Nephrologie. Springer-Verlag, Berlin, 230–232
Levy Y, George J, Yona E, Shoenfeld Y (1998) Partial lipodystrophy, mesangiocapillary glomerulonephritis, and complement dysregulation. An autoimmune phenomenon. Immunol Res 18: 55–60
Schwertz R, de Jong R, Gretz N, Kirschfink M, Anders D, Schärer K and Arbeitsgemeinschaft Pädiatrische Nephrologie (1996) Outcome of idiopathic membranoproliferative glomerulonephritis in children. Acta Paediatr 85: 308–312
Fijen CA, Kuijper EJ, Te Bulte M, van de Heuvel MM, Holdrinet AC, Sim RB, Daha MR, Dankert J (1996) Heterozygous and homozygous factor H deficiency states in a Dutch family. Clin Exp Immunol 105: 511–516
Levy M, Halbwachs-Mecarelli L, Gubler MC, Kohout G, Bensenouci A, Niaudet P, Hauptmann G, Lesavre P (1986) H deficiency in two brothers with atypical dense intramembranous deposit disease. Kidney Int 30: 949–956
Vogt BA, Wyatt RJ, Burke BA, Simonton SC, Kashtan CE (1995) Inherited factor H deficiency and collagen type III glomerulopathy. Pediatr Nephrol 9: 11–15
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
West CD (1994) Nephritic factors predispose to chronic glomerulonephritis. Am J Kidney Dis 24: 956–963
West CD, McAdams AJ (1999) The alternative pathway C3 convertase and glomerular deposits. Pediatr Nephrol 13: 448–453
Marder HK, Coleman TH, Forristal J, Beischel L, West CD (1983) An inherited defect in the C3 convertase, C3b,Bb, associated with glomerulonephritis. Kidney Int 23: 749–758
Hogasen K, Jansen JH, Mollnes TE, Hovdenes J, Harboe M (1995) Hereditary porcine membranoproliferative glomerulonephritis type II is caused by factor H deficiency. J Clin Invest 95: 1054–1061
Jansen JH, Hogasen K, Grondahl AM (1995) Porcine membranoproliferative glomerulonephritis type II: an autosomal recessive deficiency of factor H. Veterinary Record 137: 240–244
Pickering MC, Cook HT, Warren J, Bygrave AE, Moss J, Walport MJ, Botto M (2002) Uncontrolled C3 activation causes membranoproliferative glomerulonephritis in mice deficient in complement factor H. Nat Genet 31: 424–428
Hegasy GA, Manuelian T, Hogasen K, Jansen JH, Zipfel PF (2002) The molecular basis for hereditary porcine membranoproliferative glomerulonephritis type II. Am J Pathol 161: 2027–2034
Schwertz R, Rother U, Anders D, Gretz N, Schärer K, Kirschfink M (2001) Complement analysis in children with idiopathic membranoproliferative glomerulonephritis: a longterm follow-up. Pediatr Allergy Immunol 12: 166–172
Spitzer RE, Stitzel AE, Tsokos GC (1990) Human antiidiotypic antibody responses to autoantibody against alternative pathway C3 convertase. Clin Immunol Immunopathol 57: 19–32
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
Remuzzi G, Ruggenenti P, Colledan M, Gridelli B, Bertani A, Bettinaglio P, Bucchioni S, Sonzogni A, Bonanomi E, Sonzogni V et al (2005) Hemolytic uremic syndrome: a fatal outcome after kidney and liver transplantation performed to correct factor H gene mutation. Am J Transplant 5: 1146–1150
Nathanson S, Frémeaux-Bacchi V, Deschênes G (2001) Successful plasma therapy in hemolytic uremic syndrome with factor H deficiency. Pediatr Nephrol 16: 554–556
Özcakar ZB, Yalcinkaya F, Derelli E, Acar B, Yüksel S, Tulunay Ö, Ekim M (2004) Favorable outcome of haemolytic uremic syndrome with factor H deficiency. Pediatr Nephrol 19: 815–816
Stratton JD, Warwicker P (2002) Successful treatment of factor H-related haemolyticuraemic syndrome. Nephrol Dial Transplant 17: 684–685
George JN (2000) How I treat patients with thrombotic thrombocytopenic purpurahemolytic uremic syndrome. Blood 96: 1223–1229
Hayward CP, Sutton DM, Carter WH, Campbell ED, Scott JG, Francombe WH, Shumak KH, Baker MA (1994) Treatment outcomes in patients with adult thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. Arch Intern Med 154: 982–987
Jones G, Juszczak M, Kingdon E, Harber M, Sweny P, Burns A (2004) Treatment of idiopathic membranoproliferative glomerulonephritis with mycophenolate mofetil and steroids. Nephrol Dial Transplant 19: 3160–3164
Levin A (1999) Management of membranoproliferative glomerulonephritis: evidence-based recommendations. Kidney Int 55: S41–S46
Gross O, Beirowski B, Koepke ML, Kuck J, Reiner M, Addicks K, Smyth N, Schulze-Lohoff E, Weber M (2003) Preemptive ramipril therapy delays renal failure and reduces renal fibrosis in COL4A3-knockout mice with Alport syndrome. Kidney Int 63: 438–446
Gross O, Schulze-Lohoff E, Koepke ML, Beirowski B, Addicks K, Bloch W, Smyth N, Weber M (2004) Antifibrotic, nephroprotective potential of ACE inhibitor vs AT1 antagonist in a murine model of renal fibrosis. Nephrol Dial Transplant 19: 1716–1723
Appel GB, Cook HT, Hageman G, Jennette JC, Kashgarian M, Kirschfink M, Lambris JD, Lanning L, Lutz HU, Meri S et al (2005) Membranoproliferative glomerulonephritis type II (dense deposit disease): an update. J Am Soc Nephrol 16: 1392–1403
Cunningham PN, Quigg RJ (2005) Contrasting roles of complement activation and its regulation in membranous nephropathy. J Am Soc Nephrol 16: 1214–1222
Alexander J, Pickering MC, Haas M, Osawe I, Quigg RJ (2005) Complement factor H limits immune complex deposition and prevents inflammation and scarring in glomeruli of mice with chronic serum sickness. J Am Soc Nephrol 16: 52–57
Kurtz KA, Schlueter AJ (2002) Management of membranoproliferative glomerulonephritis type II with plasmapheresis. J Clin Apheresis 17: 135–137
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Licht, C., Hoppe, B. (2006). Complement defects in children which result in kidney diseases: diagnosis and therapy. In: Zipfel, P.F. (eds) Complement and Kidney Disease. Progress in Inflammation Research. Birkhäuser Basel. https://doi.org/10.1007/3-7643-7428-4_10
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