Pediatric Nephrology

, Volume 26, Issue 8, pp 1215–1227 | Cite as

Renal amyloidosis in children

Educational Review


Renal amyloidosis is a detrimental disease caused by the deposition of amyloid fibrils. A child with renal amyloidosis may present with proteinuria or nephrotic syndrome. Chronic renal failure may follow. Amyloid fibrils may deposit in other organs as well. The diagnosis is through the typical appearance on histopathology. Although chronic infections and chronic inflammatory diseases used to be the causes of secondary amyloidosis in children, the most frequent cause is now autoinflammatory diseases. Among this group of diseases, the most frequent one throughout the world is familial Mediterranean fever (FMF). FMF is typically characterized by attacks of clinical inflammation in the form of fever and serositis and high acute-phase reactants. Persisting inflammation in inadequately treated disease is associated with the development of secondary amyloidosis. The main treatment is colchicine. A number of other monogenic autoinflammatory diseases have also been identified. Among them cryopyrin-associated periodic syndrome (CAPS) is outstanding with its clinical features and the predilection to develop secondary amyloidosis in untreated cases. The treatment of secondary amyloidosis mainly depends on the treatment of the disease. However, a number of new treatments for amyloid per se are in the pipeline.


Renal amyloidosis Children Autoinflammatory diseases 



  1. 1.
    Perfetto F, Moggi-Pignone A, Livi R, Tempestini A, Bergesio F, Matucci-Cerinic M (2010) Systemic amyloidosis: a challenge for the rheumatologist. Nat Rev Rheumatol 6:417–429PubMedCrossRefGoogle Scholar
  2. 2.
    Röcken C, Shakespeare A (2002) Pathology, diagnosis and pathogenesis of AA amyloidosis. Virchows Arch 440:111–122PubMedCrossRefGoogle Scholar
  3. 3.
    Picken MM (2007) New insights into systemic amyloidosis: the importance of diagnosis of specific type. Curr Opin Nephrol Hypertens 16:196–203PubMedCrossRefGoogle Scholar
  4. 4.
    Rysavá R (2007) AL amyloidosis with renal involvement. Kidney Blood Press Res 30:359–364PubMedCrossRefGoogle Scholar
  5. 5.
    Drüeke TB, Massy ZA (2009) Beta2-microglobulin. Semin Dial 22:378–380PubMedCrossRefGoogle Scholar
  6. 6.
    Lachmann HJ, Booth DR, Booth SE, Bybee A, Gilbertson JA, Gillmore JD, Pepys MB, Hawkins PN (2002) Misdiagnosis of hereditary amyloidosis as AL (primary) amyloidosis. N Engl J Med 6(346):1786–1791CrossRefGoogle Scholar
  7. 7.
    Westermark P, Bergström J, Solomon A, Murphy C, Sletten K (2003)Transthyretin-derived senile systemic amyloidosis: clinicopathologic and structural considerations. Amyloid 10 Suppl 1:48-54Google Scholar
  8. 8.
    Ozen S (2004) Renal amyloidosis in familial Mediterranean fever. Kidney Int 65:1118–1127PubMedCrossRefGoogle Scholar
  9. 9.
    Falk RH, Comenzo RL, Skinner M (1997) The systemic amyloidoses. N Engl J Med 337:898–909PubMedCrossRefGoogle Scholar
  10. 10.
    Ben Chetritt R (2003) FMF and renal amyloidosis. Phenotype- genotype correlation, treatment and prognosis. J Nephrol 16:431–434Google Scholar
  11. 11.
    Tunca M, Akar S, Onen F, Ozdogan H, Kasapcopur O, Yalcınkaya F, Tutar E, Ozen S, Topaloglu R, Yılmaz E, Arici N, Bakkaloglu A, Besbas N, Akpolat T, Dinc A, Erken E; Turkish FMF study group (2005) Familial Mediterranean fever in Turkey. Results of a nationwide multicenter study. Medicine 84:1-11Google Scholar
  12. 12.
    Tuglular S, Yalcinkaya F, Paydas S, Oner A, Utas C, Bozfakioglu S, Ataman R, Akpolat T, Ok E, Sen S, Düsünsel R, Evrenkaya R, Akoglu E (2002) A retrospective analysis for aetiology and clinical findings of 287 secondary amyloidosis cases in Turkey. Nephrol Dial Transplant 17:2003–2005PubMedCrossRefGoogle Scholar
  13. 13.
    Uda H, Yokota A, Kobayashi K, Miyake T, Fushimi H, Maeda A, Saiki O (2006) Two distinct clinical courses of renal involvement in rheumatoid patients with AA amyloidosis. J Rheumatol 33:1482–1487PubMedGoogle Scholar
  14. 14.
    Nishi S, Alchi B, Imai N, Gejyo F (2008) New advances in renal amyloidosis. Clin Exp Nephrol 12:93–101PubMedCrossRefGoogle Scholar
  15. 15.
    Neugarten J, Gallo GR, Buxbaum J, Katz LA, Rubenstein J, Baldwin DS (1986) Amyloidosis in subcutaneous heroin abusers ("skin poppers' amyloidosis"). Am J Med 81:635–640PubMedCrossRefGoogle Scholar
  16. 16.
    Orfila C, Lepert JC, Modesto A, Bernadet P, Suc JM (1991) Fanconi's syndrome, kappa light-chain myeloma, non-amyloid fibrils and cytoplasmic crystals in renal tubular epithelium. Am J Nephrol 11:345–349PubMedCrossRefGoogle Scholar
  17. 17.
    Dubrey SW, Cha K, Simms RW, Skinner M, Falk RH (1996) Electrocardiography and Doppler echocardiography in secondary (AA) amyloidosis. Am J Cardiol 77:313–315PubMedCrossRefGoogle Scholar
  18. 18.
    Dubrey SW, Cha K, Anderson J, Chamarthi B, Reisinger J, Skinner M, Falk RH (1998) The clinical features of immunoglobulin light-chain (AL) amyloidosis with heart involvement. QJM 91:141–157PubMedCrossRefGoogle Scholar
  19. 19.
    Benson MD (2003) The hereditary amyloidoses. Best Pract Res Clin Rheumatol 17:909–927PubMedCrossRefGoogle Scholar
  20. 20.
    Sattianayagam P, Hawkins P, Gillmore J (2009) Amyloid and the GI tract. Expert Rev Gastroenterol Hepatol 3:615–630PubMedCrossRefGoogle Scholar
  21. 21.
    Ebert EC, Nagar M (2008) Gastrointestinal manifestations of amyloidosis. Am J Gastroenterol 103:776–787PubMedCrossRefGoogle Scholar
  22. 22.
    Chopra S, Rubinow A, Koff RS, Cohen AS (1984) Hepatic amyloidosis. A histopathologic analysis of primary (AL) and secondary (AA) forms. Am J Pathol 115:186–193PubMedGoogle Scholar
  23. 23.
    Looi LM, Sumithran E (1988) Morphologic differences in the pattern of liver infiltration between systemic AL and AA amyloidosis. Hum Pathol 19:732–735PubMedCrossRefGoogle Scholar
  24. 24.
    Renzulli P, Schoepfer A, Mueller E, Candinas D (2009) Atraumatic splenic rupture in amyloidosis. Amyloid 16:47–53PubMedCrossRefGoogle Scholar
  25. 25.
    Elvy A, Harbach L, Bhangu A (2010) Atraumatic splenic rupture: a 6-year case series. Eur J Emerg Med. doi:10.1097/MEJ.0b013e32833ddeb5
  26. 26.
    Renzulli P, Schoepfer A, Mueller E, Candinas D (2009) Atraumatic splenic rupture in amyloidosis. Amyloid 16:47–53PubMedCrossRefGoogle Scholar
  27. 27.
    Sungur C, Sungur A, Ruacan S, Arik N, Yasavul U, Turgan C, Caglar S (1993) Diagnostic value of bone marrow biopsy in patients with renal disease secondary to familial Mediterranean fever. Kidney Int 44:834–836PubMedCrossRefGoogle Scholar
  28. 28.
    Sucker C, Hetzel GR, Grabensee B, Stockschlaeder M, Scharf RE (2006) Amyloidosis and bleeding: pathophysiology, diagnosis, and therapy. Am J Kidney Dis 47:947–955PubMedCrossRefGoogle Scholar
  29. 29.
    Zangari M, Elice F, Fink L, Tricot G (2007) Hemostatic dysfunction in paraproteinemias and amyloidosis. Semin Thromb Hemost 33:339–349PubMedCrossRefGoogle Scholar
  30. 30.
    Benson MD, Kincaid JC (2007) The molecular biology and clinical features of amyloid neuropathy. Muscle Nerve 36:411–423PubMedCrossRefGoogle Scholar
  31. 31.
    Keven K, Oztas E, Aksoy H, Duman N, Erbay B, Ertürk S (2001) Polyglandular endocrine failure in a patient with amyloidosis secondary to familial Mediterranean fever. Am J Kidney Dis 38:E39PubMedCrossRefGoogle Scholar
  32. 32.
    Herrera GA, Turbat-Herrera EA (2010) Renal diseases with organized deposits: an algorithmic approach to classification and clinicopathologic diagnosis. Arch Pathol Lab Med 134:512–531PubMedGoogle Scholar
  33. 33.
    Sen S, Sarsik B (2010) A proposed histopathologic classification, scoring, and grading system for renal amyloidosis: standardization of renal amyloid biopsy report. Arch Pathol Lab Med 134:532–544PubMedGoogle Scholar
  34. 34.
    Hawkins PN (2002) Serum amyloid P component scintigraphy for diagnosing and monitoring amyloidosis. Curr Opin Nephrol Hypertens 11:649–655PubMedCrossRefGoogle Scholar
  35. 35.
    Petty RE, Soutwood TR, Manners P, Baum J, Glass DN, Goldenberg J, He X, Maldonado-Cocco J, Orozco-Alcala J, Prieur A, Suarez Almazor ME, Woo P (2004) International League of Associations for Rheumatology classification of juvenile idiopathic arthritis: second revision, Edmonton, 2001. J Rheumatol 31:390–392PubMedGoogle Scholar
  36. 36.
    Besbas N, Saatci U, Bakkaloğlu A, Ozen S (1992) Amyloidosis of juvenile chronic arthritis in Turkish children. Scand J Rheumatol 21:257–259PubMedCrossRefGoogle Scholar
  37. 37.
    Stojanov S, Kastner DL (2005) Familial autoinflammatory diseases: genetics, pathogenesis and treatment. Curr Opin Rheumatol 17:586–599PubMedCrossRefGoogle Scholar
  38. 38.
    Masters SL, Simon A, Ksentijevic I, Kastner DL (2009) Horror autoinflammaticus: the molecular pathophysiology of autoinflammatory disease. Annu Rev Immunol 27:621–628PubMedCrossRefGoogle Scholar
  39. 39.
    Ozen S, Karaaslan Y, Ozdemir O, Saatci U, Bakkaloglu A, Koroglu E, Tezcan S (1998) Prevalence of juvenile chronic arthritis and familial Mediterranean fever in Turkey. J Rheumatol 25:2445–2449PubMedGoogle Scholar
  40. 40.
    Sohar E, Gafni J, Pras M, Heller H (1967) Familial Mediterranean fever. A survey of 470 cases and review of literature. Am J Med 43:227–253PubMedCrossRefGoogle Scholar
  41. 41.
    Majeed HA, El-Shanti H, Al-Khateeb MS, Rabia ZA (2002) Genotype/phenotype correlations in Arab patients with FMF. Semin Arthritis Rheum 31:371–376PubMedCrossRefGoogle Scholar
  42. 42.
    Chae JJ, Wood G, Richard K, Jaffe H, Colburn NT, Masters SL, Gumucio DL, Shoham NG, Kastner DL (2008) The familial Mediterranean fever protein, pyrin is cleaved by caspase-1 and activates NF-kappa B through its N-terminal fragment. Blood 11:1794–1803CrossRefGoogle Scholar
  43. 43.
    Samuels J, Ozen S (2006) Familial Mediterranean fever and the other auto inflammatory syndromes: evaluation of the patient with recurrent fever. Curr Opin Rheumatol 18:108–117PubMedCrossRefGoogle Scholar
  44. 44.
    International FMF Consortium (1997) Ancient missense mutations in a new number of the Roret gene family are likely to cause FMF. Cell 90:797–807CrossRefGoogle Scholar
  45. 45.
    French FMF consortium (1997) A candidate gene for familial Mediterranean fever. Nat Genet 17:25–31CrossRefGoogle Scholar
  46. 46.
    Ozen S, Balci B, Ozkara S, Ozcan A, Yilmaz E, Besbas N, Ozguc M, Kastner DL, Bakkaloglu A (2002) Is there a heterozygote advantage for familial Mediterranean fever carriers against tuberculous infections? Clin Exp Rheumatol 20:S57–S58PubMedGoogle Scholar
  47. 47.
    Ozen S (2009) Mutations/polymorphisms in a monogenic auto inflammatory disease is a susceptibility marker for certain rheumatic diseases. Lessons from the bedside for the benchside. Curr Opin Rheumatol 27:S29–S31Google Scholar
  48. 48.
    Booth DR, Lachman JJ, Gillmor JD, Booth SE, Hawkins PN (2002) Prevalence and significance of the familial Mediterranean gene mutation encoding pyrin Q148. QJM 95:332–333CrossRefGoogle Scholar
  49. 49.
    Touitou I, Sarkisian T, Medlej-Hashim M, Tunca M, Livneh A, Cattan D, Yalcınkaya F, Ozen S, Majeed H, Ozdogan H, Kastner D, Booth D, Ben-Chetrit E, Pugnere D, Michelon C, Seguret F, Gershoni-Baruch R (2007) Country as the primary risk factor for renal amyloidosis in familial Mediterranean fever. Arthritis Rheum 56:1706–1712PubMedCrossRefGoogle Scholar
  50. 50.
    Ozen S, Aktay N, Lainka E, Duzova A, Bakkaloglu A, Kallinich T (2009) Disease severity in children and adolescents with familial Mediterranean fever: a comparative study to explore environmental effects on a monogenic disease. Ann Rheum Dis 68:246–248PubMedCrossRefGoogle Scholar
  51. 51.
    Ben Chetrit E, Levy M (1998) Familial Mediterranean fever. Lancet 351:659–664PubMedCrossRefGoogle Scholar
  52. 52.
    Lidar M, Livneh A (2007) Familial Mediterranean fever: clinical, molecular and management advances. Neth J Med 65:318–324PubMedGoogle Scholar
  53. 53.
    Ozen S (2010) The "other" vasculitis syndromes and kidney involvement. Pediatr Nephrol 25:1633–1639PubMedCrossRefGoogle Scholar
  54. 54.
    Lachmann HJ, Sengul B, Yavuzsen TU, Booth DR, Booth SE, Bybee A, Gallimore JR, Soytürk M, Akar S, Tunca M, Hawkins PN (2006) Clinical and subclinical inflammation in patients with familial Mediterranean fever and in heterozygous carriers of MEFV mutations. Rheumatology 45:746–750PubMedCrossRefGoogle Scholar
  55. 55.
    Zemer D, Pras M, Sohar E, et Modan M, Cabili S, Gafni J (1986) Colchicine in the prevention and treatment of amyloidosis of familial Mediterranean fever. N Engl J Med 314:1001–1005PubMedCrossRefGoogle Scholar
  56. 56.
    Saatci U, Ozen S, Ozdemir S, Bakkaloglu A, Besbas N, Topaloglu R, Arslan S (1997) FMF in children: report of a large series and discussions of the risk and prognostic factors of amyloidosis. Eur J Pediatr 156:619–623PubMedCrossRefGoogle Scholar
  57. 57.
    Sohar E, Gafni J, Pras M, Heller H (1967) Familial Mediterranean fever. A survey of 470 cases and review of literature. Am J Med 43:227–253PubMedCrossRefGoogle Scholar
  58. 58.
    Shohat M, Magal N, Shohat T, Chen X, Dagan T, Mimouni A, Danon Y, Laron R, Ogur G, Schlezinger M, Halpern GJ, Schwabe A, Kastner D, Rotter JI, Fischel-Ghodslan N (1999) Phenotype-genotype correlation in FMF: evidence for an association between M694V and amyloidosis. Eur J Hum Genet 7:287–292PubMedCrossRefGoogle Scholar
  59. 59.
    Cakar N, Yalcınkaya F, Ozkaya N, Tekin M, Akar N, Koçak H, Mısırlıoğlu M, Akar E, Tümer N (2001) FMF associated amyloidosis in childhood. Clinical features, course and outcome. Clin Exp Rheumatol 19:S63–S67PubMedGoogle Scholar
  60. 60.
    Cazeneuve C, Ajrapetyan H, Papin S, Rpudot-Thoraval F, Genevieve D, Mindjoyan E, Papazian M, Sarkisian A, Babloyan A, Boissier B, Duquesnay P, Kouyoumdjian JC, Girodon-Boulandet E, Grateau G, Sarkisian T, Amselem S (2000) Identification of MEFV independent modifying genetic factors for FMF. Am J Hum Genet 67:1136–1143PubMedGoogle Scholar
  61. 61.
    Gershoni-Baruch R, Brik R, Zacks N, Shinawi M, Lidar M, Livneh A (2003) The contribution of genotypes at the MEFV and SAA1 loci to amyloidosis and disease severity in patients with FMF. Arthritis Rheum 48:1149–1155PubMedCrossRefGoogle Scholar
  62. 62.
    Ozen S, Ben-Chetrit E, Bakkaloglu A, Gur H, Tınaztepe K, Calguneri M, Turgan C, Turkman A, Akpolat I, Danaci M, Besbas N, Akpolat T (2001) Polyarteritis nodosa in patients with FMF: a concomitant disease or a feature of FMF? Semin Arthritis Rheum 30:281–287PubMedCrossRefGoogle Scholar
  63. 63.
    Goldinger SE (1972) Colchicine for familial Mediterranean fever. N Engl J Med 287:1302Google Scholar
  64. 64.
    Kallinich T, Haffner D, Niehues T, Huss K, Lainka E, Neudorf U, Schaefer C, Stajanov S, Timmann C, Keitzer R, Ozdogan H, Ozen S (2007) Colchicine use in children and adolescents with familial Mediterranean fever: literature review and consensus statement. Pediatrics 119:e474–e483PubMedCrossRefGoogle Scholar
  65. 65.
    Oner A, Erdogan O, Demircin G, Bulbul M, Memis L (2003) Efficacy of colchicine therapy in amyloid nephropathy of familial Mediterranean fever. Pediatr Nephrol 18:521–526PubMedGoogle Scholar
  66. 66.
    Livneh A, Zemer D, Langevitz P, Laor A, Sohar E, Pras M (1994) Colchicine treatment of AA amyloidosis of familial Mediterranean fever: an analysis of factors affecting outcome. Arthritis Rheum 37:1804–1811PubMedCrossRefGoogle Scholar
  67. 67.
    Schattner A (1991) Colchicine-expanding horizons. Postgrad Med J 67:223–226PubMedCrossRefGoogle Scholar
  68. 68.
    Bohle A, Wehrmann M, Fissele R, Gise HV, Mackensen-Haen S, Müller C (1993) The long-term prognosis of AA and al amyloidosis and the pathogenesis of chronic renal failure in renal amyloidosis. Pathol Res Pract 89:316–331Google Scholar
  69. 69.
    Bauer EZ, Valle KJ (1982) Colchicine-induced modulation of collagenase in human skin fibroblast cultures. Stimulation enzyme synthesis normal cells. J Invest Dermatol 79:398–402PubMedCrossRefGoogle Scholar
  70. 70.
    Montseny JJ, Meyrier A, Gherardi RK (1996) Colchicine toxicity in patients with chronic renal failure. Nephrol Dial Transplant 11:2055–2058PubMedGoogle Scholar
  71. 71.
    Wallace SL, Singer JZ, Duncan GJ, Wigley FM, Kunel RW (1991) Renal function predicts colchicine toxicity: guidelines for the prophylactic use of colchicine in gout. J Rheumatol 18:264–269PubMedGoogle Scholar
  72. 72.
    Anderson-Haag T, Patel B (2003) Safety of colchicine in dialysis patients. Semin Dial 16:412–413PubMedCrossRefGoogle Scholar
  73. 73.
    Hoffmann HM, Mueller JL, Broide DH, Wanderer AA, Kolodner RD (2001) Mutation of a new gene encoding a putative pyrin like protein causes familial cold autoinflammatory syndrome and Muckle Wells syndrome. Nat Genet 29:301–305CrossRefGoogle Scholar
  74. 74.
    Feldmann J, Prieur AM, Quartier P, Berquin P, Certain S, Cortis E, Teilac-Hamel D, Fischer A, de Saint BG (2002) Chronic infantile neurological cutaneous and articular syndrome is caused by mutations in CIAS1, a gene highly expressed in polymorphonuclear cells and chondrocytes. Am J Hum Genet 71:198–203PubMedCrossRefGoogle Scholar
  75. 75.
    Hoffman HM, Wanderer AA, Broide DH (2001) Familial cold autoinflammatory syndrome: phenotype and genotype of an autosomal dominant periodic fever. J Allergy Clin Immunol 108:615–620PubMedCrossRefGoogle Scholar
  76. 76.
    Toker O, Hashkes PJ (2010) Critical appraisal of canakinumab in the treatment of adults and children with cryopyrin-associated periodic syndrome (CAPS). Biologics 25:131–138Google Scholar
  77. 77.
    Hawkins PN, Lachmann HJ, Aganna E, McDermott MF (2004) Spectrum of clinical features in Muckle-Wells syndrome and response to anakinra. Arthritis Rheum 50:607–612PubMedCrossRefGoogle Scholar
  78. 78.
    Lachmann HJ, Kone-Paut I, Kuemmerle-Deschner JB, Leslie KS, Hachulla E, Quartier P, Gitton X, Widmer A, Patel N, Hawkins PN (2009) Use of canakinumab in the cryopyrin associated periodic syndrome. N Engl J Med 360:2416–2425PubMedCrossRefGoogle Scholar
  79. 79.
    Drenth JP, Cuisset L, Grateau G, Vasseur C, van de Velde-Visser SD, de Jong JG, Beckmann JS, van der Meer JW, Delpech M (1999) Mutations in the gene encoding mevolanate kinase cause hyper-IgD and periodic fever syndrome. Nat Genet 22:178–181PubMedCrossRefGoogle Scholar
  80. 80.
    Goldbach-Mansky R, Kastner DL (2009) Autoinflammation: the prominent role of IL-1 in monogenic autoinflammatory diseases and implications for common illnesses. J Allergy Clin Immunol 124:1141–1149PubMedCrossRefGoogle Scholar
  81. 81.
    McDermott MF, Aksentijevich I, Galon J, McDermott EM, Ogunkolade BW, Centola M, Mansfield E, Godina M, Karenko L, Petterson T, McCarthy J, Frucht DM, Aringer M, Torosyan Y, Teppo AM, Wilson M, Karaaslan HM, Wan Y, Todd I, Wood G, Schlimgen R, Kumarajeewa TR, Cooper SM, Vella JP, Amos EI, Mulley J, Quane KA, Mollay MG, Ranki A, Powell RJ, Hitman GA, Shea JJ O, Kastner DL (1999) Germline mutations in the extracellular domains of the 55-kDa TNF receptor, TNFR1, define a family of dominantly inherited autoinflammatory syndromes. Cell 97:133–144PubMedCrossRefGoogle Scholar
  82. 82.
    Hull KM, Drewe E, Aksentijevich I, Singh HK, Wong K, McDermott EM, Dean J, Powell RJ, Kastner DL (2002) The TNF receptor–associated periodic syndrome (TRAPS): emerging concepts of an auto inflammatory disorder. Medicine 81:349–368PubMedCrossRefGoogle Scholar
  83. 83.
    Aksentijevich I, Galon J, Soares M, Mansfield E, Hull K, Oh HH, Goldbach-Mansky R, Dean J, Athreya B, Regianato AJ, Henrickson M, Pons-Estel B, O’Shea JJ, Kastner DL (2001) The tumor necrosis factor receptor associated periodic syndrome: new mutations in TNFRSF1A, ancestral origins, genotype-phenotype studies, and evidence for further heterogeneity of periodic fevers. Am J Hum Genet 69:301–314PubMedCrossRefGoogle Scholar
  84. 84.
    Reddy P, Slack JL, Davis R, Cerretti DP, Kozlosky CJ, Blanton RA, Shows D, Peschon JJ, Black RA (2000) Functional analysis of the domain structure of tumor necrosis factor–alpha converting enzyme. J Biol Chem 275:14608–14614PubMedCrossRefGoogle Scholar
  85. 85.
    Toro JR, Aksentijevich I, Hull K, Dean J, Kastner DL (2000) Tumor necrosis factor receptor associated periodic syndrome: a novel syndrome with cutaneous manifestations. Arch Dermatol 136:1487–1494PubMedCrossRefGoogle Scholar
  86. 86.
    Gottorno M, Pelagatti MA, Meini A, Obici L, Barcellona R, Federici S, Buoncompagni A, Plebani A, Merlini G, Martini A (2008) Persistent efficacy of anakinra in patients with tumor necrosis factor receptor associated periodic syndrome. Arthritis Rheum 58:1516–1520CrossRefGoogle Scholar
  87. 87.
    Akpolat T, Akkoyunlu M, Akpolat I, Dilek M, Odabas AR, Ozen S (2002) Renal Behçet’s disease: a cumulative analysis. Semin Arthritis Rheum 31:317–337PubMedCrossRefGoogle Scholar
  88. 88.
    Penza R, Brunetti L, Francioso G, Tricarico A, Lospalluti M (1983) Renal amyloidosis in a child with Behçet's syndrome. Int J Pediatr Nephrol 4:35–37PubMedGoogle Scholar
  89. 89.
    Kaltenis P, Mudeniené V, Maknavicius S, Seinin D (2008) Renal amyloidosis in a child with chronic granulomatous disease and invasive aspergillosis. Pediatr Nephrol 23:831–834PubMedCrossRefGoogle Scholar
  90. 90.
    Simşek B, Bayazit AK, Ergin M, Soran M, Dursun H, Kilinc Y (2006) Renal amyloidosis in a child with sickle cell anemia. Pediatr Nephrol 21:877–879PubMedCrossRefGoogle Scholar
  91. 91.
    Büyükpamukçu M, Hazar V, Tinaztepe K, Bakkaloğlu A, Akyüz C, Kutluk T (2000) Hodgkin's disease and renal paraneoplastic syndromes in childhood. Turk J Pediatr 42:109–114PubMedGoogle Scholar
  92. 92.
    Kyle RA, Rajkumar SV (2007) Epidemiology of the plasma-cell disorders. Best Pract Res Clin Haematol 20:637–664PubMedCrossRefGoogle Scholar
  93. 93.
    Mor A, Pillinger MH, Kishimoto M, Abeles AM, Livneh A (2007) Familial Mediterranean fever successfully treated with etanercept. J Clin Rheumatol 13:38–40PubMedCrossRefGoogle Scholar
  94. 94.
    Gottenberg JE, Merle-Vincent F, Bentaberry F, Allanore Y, Berenbaum F, Fautrel B, Combe B, Durbach A, Sibilia J, Dougados M, Mariette X (2003) Anti-tumor necrosis factor alpha therapy in fifteen patients with AA amyloidoses secondary to inflammatory arthritis. Arthritis Rheum 48:2019–2024PubMedCrossRefGoogle Scholar
  95. 95.
    Ozen S, Bilginer Y, Ayaz NA, Calguneri M (2010) Anti IL1 treatment for FMF patients resistant to colchicines: reasoning treatment from laboratory experience. J Rheumatol. doi:10.3899/jrheumatol.100718
  96. 96.
    Bilginer Y, Ayaz NA, Ozen S (2009) Anti-IL-1 treatment for secondary amyloidosis in an adolescent with FMF and Behçet's disease. Clin Rheumatol 29:209–210PubMedCrossRefGoogle Scholar
  97. 97.
    Dember LM (2009) Modern treatment of amyloidosis: unresolved questions. J Am Soc Nephrol 20:469–472PubMedCrossRefGoogle Scholar
  98. 98.
    Sekijima Y, Kelly JW, Ikeda S (2008) Pathogenesis of and therapeutic strategies to ameliorate the transthyretin amyloidoses. Curr Pharm Des 14:3219–3230PubMedCrossRefGoogle Scholar
  99. 99.
    Dember LM, Hawkins PN, Hazenberg BP, Gorevic PD, Merlini G, Butrimiene I, Livneh A, Lesnyak O, Puéchal X, Lachmann HJ, Obici L, Balshaw R, Garceau D, Hauck W, Skinner M (2007) Eprodisate for AA Amyloidosis Trial Group. Eprodisate for the treatment of renal disease in AA amyloidosis. N Engl J Med 356:2349–2360PubMedCrossRefGoogle Scholar
  100. 100.
    Rysavá R, Merta M, Spicka I, Tesar V (2003) Current therapeutic possibilities in primary and secondary amyloidosis and our experience with 31 patients. Nephrol Dial Transplant 18(Suppl 5):v38–v40PubMedCrossRefGoogle Scholar
  101. 101.
    Keven K, Sengul S, Kutlay S, Ekmekci Y, Anadol E, Nergizoglu G, Ates K, Erturk S, Erbay B (2004) Long-term outcome of renal transplantation in patients with familial Mediterranean fever amyloidosis: a single-center experience. Transplant Proc 36:2632–2634PubMedCrossRefGoogle Scholar
  102. 102.
    Altiparmak MR, Pamuk ON, Ataman R, Serdengeçti K (2004) Continuous ambulatory peritoneal dialysis in familial Mediterranean fever amyloidosis patients with end-stage renal failure: a single-centre experience from Turkey. Nephron Clin Pract 98:c119–c123PubMedCrossRefGoogle Scholar
  103. 103.
    Ari JB, Zlotnik M, Oren A, Berlyne GM (1976) Dialysis in renal failure caused by amyloidosis of familial Mediterranean fever. A report of ten cases. Arch Intern Med 136:449–451PubMedCrossRefGoogle Scholar
  104. 104.
    Sahin S, Sahin GM, Ergin H, Kantarci G (2007) The effect of dialytic modalities on clinical outcomes in ESRD patients with familial Mediterranean fever. Ren Fail 29:315–319PubMedCrossRefGoogle Scholar
  105. 105.
    Tan SY, Irish A, Winearls CG, Brown EA, Gower PE, Clutterbuck EJ, Madhoo S, Lavender JP, Pepys MB, Hawkins PN (1996) Long-term effect of renal transplantation on dialysis-related amyloid deposits and symptomatology. Kidney Int 50:282–289PubMedCrossRefGoogle Scholar
  106. 106.
    Gillmore JD, Lachmann HJ, Rowczenio D, Gilbertson JA, Zeng CH, Liu ZH, Li LS, Wechalekar A, Hawkins PN (2009) Diagnosis, pathogenesis, treatment, and prognosis of hereditary fibrinogen A alpha-chain amyloidosis. J Am Soc Nephrol 20:444–451PubMedCrossRefGoogle Scholar
  107. 107.
    Saglam F, Celik A, Cavdar C, Sifil A, Atila K, Kaya GC, Bora S, Gulay H, Camsari T (2008) A renal transplant recipient with delayed gastric emptying in amyloidosis due to familial Mediterranean fever improved with erythromycin: a case report. Transplant Proc 40:308–309PubMedCrossRefGoogle Scholar
  108. 108.
    Terkeltaub RA (2009) Colchicine update: 2008. Semin Arthritis Rheum 38:411–419PubMedCrossRefGoogle Scholar
  109. 109.
    Tweezer-Zaks N, Rabinovich E, Lidar M, Livneh A (2008) Interferon-alpha as a treatment modality for colchicine-resistant familial Mediterranean fever. J Rheumatol 35:1362–1365PubMedGoogle Scholar
  110. 110.
    Fabrizi F, Messa P, Basile C, Martin P (2010) Hepatic disorders in chronic kidney disease. Nat Rev Nephrol 6:395–403PubMedCrossRefGoogle Scholar
  111. 111.
    Moser C, Pohl G, Haslinger I, Knapp S, Rowczenio D, Russel T, Lachmann HJ, Lang U, Kovarik J (2009) Successful treatment of familial Mediterranean fever with Anakinra and outcome after renal transplantation. Nephrol Dial Transplant 24:676–678PubMedCrossRefGoogle Scholar

Copyright information

© IPNA 2011

Authors and Affiliations

  1. 1.Hacettepe University Faculty of Medicine, Pediatric Nephrology and Rheumatology UnitAnkaraTurkey
  2. 2.Department of NephrologyOndokuz Mayis University School of MedicineSamsunTurkey

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