Search tips
Search criteria 


Logo of archdischArchives of Disease in ChildhoodVisit this articleSubmit a manuscriptReceive email alertsContact usBMJ
Arch Dis Child. 1981 June; 56(6): 464–467.
PMCID: PMC1627456

Amyloidosis in children with familial Mediterranean fever.


The clinical and laboratory findings of 35 children with familial Mediterranean fever who developed amyloidosis are described. The types, frequency, and severity of attacks of familial Mediterranean fever in these children were no different from patients with this disease without amyloidosis. Although amyloid was widely deposited in all tissues, the major clinical manifestations of the amyloidosis were proteinuria, the nephrotic syndrome, and progressive renal failure. Only 20% of the patients were alive 5 years after the first appearance of proteinuria.

Full text

Full text is available as a scanned copy of the original print version. Get a printable copy (PDF file) of the complete article (525K), or click on a page image below to browse page by page. Links to PubMed are also available for Selected References.

Selected References

These references are in PubMed. This may not be the complete list of references from this article.
  • Strauss RG, Schubert WK, McAdams AJ. Amyloidosis in childhood. J Pediatr. 1969 Feb;74(2):272–282. [PubMed]
  • Andrade C, Araki S, Block WD, Cohen AS, Jackson CE, Kuroiwa Y, Nissim J, Sohar E, McKusick VA, Van Allen MW. Hereditary amyloidosis. Arthritis Rheum. 1970 Nov-Dec;13(6):902–915. [PubMed]
  • Sohar E, Gafni J, Pras M, Heller H. Familial Mediterranean fever. A survey of 470 cases and review of the literature. Am J Med. 1967 Aug;43(2):227–253. [PubMed]
  • Dinarello CA, Wolff SM, Goldfinger SE, Dale DC, Alling DW. Colchicine therapy for familial mediterranean fever. A double-blind trial. N Engl J Med. 1974 Oct 31;291(18):934–937. [PubMed]
  • Zemer D, Revach M, Pras M, Modan B, Schor S, Sohar E, Gafni J. A controlled trial of colchicine in preventing attacks of familial mediterranean fever. N Engl J Med. 1974 Oct 31;291(18):932–934. [PubMed]
  • Smith ME, Ansell BM, Bywaters EG. Mortality and prognosis related to the amyloidosis of Still's disease. Ann Rheum Dis. 1968 Mar;27(2):137–145. [PMC free article] [PubMed]
  • Levin M, Franklin EC, Frangione B, Pras M. The amino acid sequence of a major nonimmunoglobulin component of some amyloid fibrils. J Clin Invest. 1972 Oct;51(10):2773–2776. [PMC free article] [PubMed]
  • Glenner GG, Terry WD, Isersky C. Amyloidosis: its nature and pathogenesis. Semin Hematol. 1973 Jan;10(1):65–86. [PubMed]
  • Cohen AS, Cathcart ES, Skinner M. Amyloidosis: current trends in its investigation. Arthritis Rheum. 1978 Jan-Feb;21(1):153–160. [PubMed]
  • Derosena R, Koss MN, Pirani CL. Demonstration of amyloid fibrils in urinary sediment. N Engl J Med. 1975 Nov 27;293(22):1131–1133. [PubMed]
  • Shirahama T, Skinner M, Cohen AS, Benson MD. Uncertain value of urinary sediments in the diagnosis of amyloidosis. N Engl J Med. 1977 Oct 13;297(15):821–823. [PubMed]
  • Jones NF, Hilton PJ, Tighe JR, Hobbs JR. Treatment of "primary" renal amyloidosis with melphalan. Lancet. 1972 Sep 23;2(7778):616–619. [PubMed]
  • Ansell BM, Eghtedari A, Bywaters EG. Chlorambucil in the management of juvenile chronic polyarthritis complicated by amyloidosis. Ann Rheum Dis. 1971 May;30(3):331–331. [PMC free article] [PubMed]
  • Laufer A, Tal C, Kolander N. Experimental amyloidosis and the effect of cortisone treatment. Pathol Microbiol (Basel) 1968;31(2):85–92. [PubMed]
  • GARDNER DL. Amyloidosis in rheumatoid arthritis treated with hormones. Ann Rheum Dis. 1962 Sep;21:298–299. [PMC free article] [PubMed]
  • SOHAR E, GAFNI J, CHAIMOW M, PRASS M, HELLER H. Low-fat diet in familial Mediterranean fever; a therapeutic trial. Arch Intern Med. 1962 Aug;110:150–154. [PubMed]
  • Jones MB, Adams JM, Passer JA. Amyloidosis in a renal allograft in familial Mediterranean fever. Ann Intern Med. 1977 Nov;87(5):579–580. [PubMed]
  • Jacob ET, Bar-Nathan N, Shapira Z, Gafni J. Renal transplantation in the amyloidosis of familial Mediterranean fever. Experience in ten cases. Arch Intern Med. 1979 Oct;139(10):1135–1138. [PubMed]
  • Zemer D, Pras M, Sohar E, Gafni J. Letter: Colchicine in familial Mediterranean fever. N Engl J Med. 1976 Jan 15;294(3):170–171. [PubMed]
  • Kedar I, Ravid M, Sohar E, Gafni J. Colchicine inhibition of casein-induced amyloidosis in mice. Isr J Med Sci. 1974 Jul;10(7):787–789. [PubMed]
  • Shirahama T, Cohen AS. Blockage of amyloid induction by colchicine in an animal model. J Exp Med. 1974 Oct 1;140(4):1102–1107. [PMC free article] [PubMed]
  • Ravid M, Robson M, Kedar I. Prolonged colchicine treatment in four patients with amyloidosis. Ann Intern Med. 1977 Nov;87(5):568–570. [PubMed]

Articles from Archives of Disease in Childhood are provided here courtesy of BMJ Publishing Group