Familial perireticular amyloidosis in a Swedish family

      This paper is only available as a PDF. To read, Please Download here.


      A familial perireticular amyloidosis in a Swedish family, not of the Jewish race, is described. The systemic deposition of amyloid, manifested clinically as nephropathy, was the only finding at autopsy. The pathoanatomic findings were identical with those known to be characteristic of the amyloidosis of familial Mediterranean fever. The mode of inheritance in our family appears, however, to be dominant and the clinical syndrome differs somewhat from that of familial Mediterranean fever. It therefore appears probable that the condition in the family described constitutes a new entity of familial perireticular amyloidosis.
      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'


      Subscribe to The American Journal of Medicine
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect


        • Lubarsch O.
        Zur Kenntnis ungewöhnlicher Amyloidablagerungen.
        Arch. path. Anat. 1929; 271: 367
        • Heller H.
        • Sohar E.
        • Gafni J.
        Classification of amyloidosis with special regard to the genetic types.
        Path. et Microbiol. 1964; 27: 833
        • Missmahl H.P.
        Welche Beziehungen bestehen zwischen den verschiedenen Formen der Amyloidose und den Bindgewebsfasern?.
        Verhandl. deutsch. Gesellsch. inn. Med. 1959; 65: 439
        • Heller H.
        • Missmahl H.P.
        • Sohar E.
        • Gafni J.
        Amyloidosis: its differentiation into perireticulin and peri-collagen types.
        J. Path. & Bact. 1964; 88: 15
        • Sohar E.
        • Pras M.
        • Heller J.
        • Heller H.
        Genetics of familial mediterranean fever (FMF).
        Arch. Int. Med. 1961; 107: 529
        • 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; 43: 227
        • Muckle T.J.
        • Wells M.
        Urticaria, deafness and amyloidosis. A new heredo-familial syndrome.
        Quart. J. Med. 1962; 31: 235
        • Rukavina J.G.
        • Block W.D.
        • Jackson C.E.
        • Falls H.F.
        • Carey J.H.
        • Curtis A.G.
        Primary systemic amyloidosis. A review and an experimental genetic and clinical study of 29 cases with particular emphasis on the familial form.
        Medicine. 1956; 35: 329
        • Andrade C.
        Peculiar form of peripheral neuropathy. Familial atypical generalized amyloidosis with special involvement of peripheral nerves.
        Brain. 1952; 75: 408
        • da S. Horta J.
        • Filipe I.
        • Duarte S.
        Portuguese polyneuritic familial type of amyloidosis.
        Path. et microbiol. 1964; 27: 809
        • Frederiksen T.
        • Gøtzsche H.
        • Harboe N.
        • Kiaer W.
        • Mellemgaard K.
        Familial primary amyloidosis with severe amyloid heart disease.
        Am. J. Med. 1962; 33: 328
        • Nilsson S.E.
        • Floderus S.
        Nine cases of hereditary and non-hereditary periodic diseases.
        Acta med. scandinav. 1964; 175: 341
        • Missmahl H.P.
        Doppelbrechung der reticulären Faser und sich heraus ergebender Nachweis von gerichtet eingelagerten Lipoiden in die reticuläre Faser.
        Ztschr. Zellforsch. 1957; 45: 612
        • Missmahl H.P.
        • Gafni J.
        Peri-collagen and peri-reticular amyloidoses. Their differentiation by polarization microscopy.
        Path. et microbiol. 1964; 27: 826