Renal Amyloıd- Ourstııcasrı A And Deafness

Abstract

A 48 - year -old patient with progressive bilateral perceptive deafness, bouts ol fever, chronic urticaria, pes cavus and rena! amyloidosis is derseribed. The case was characterized by long duration (14 years) with initial involment of the kidneys and subseguent development of deafness. This represents the third (sporadic) instance of the clinical syndrome initialliy described by Muckle and Wells.

Keywords

• Chronic urticaria
• Renal amyloidosis
• Progressive hearing loss
• Muckle-Wells syndrome
• Autoinflammatory syndrome

BÖBREK AMİLOİDOZİSİ, ÜRTİKER VE SAĞIRLIK Bir olgu ve literatürün Gözden Geçirilmesi

Abstract

ift yanlı perseptif sağırlık, ateş nöbetleri, kronik ürtiker, pes kavus ve böbrek amiloidozisi gösteren 48 yaşında bir erkek hasta takdim edildi. Bu olgu son görüldüğünde 14 yıldır yaşıyordu. Önce böbrek bozukluğu, sonradan sağırlık ortaya çıkmıştır. Bu, Muckle ve Wells'in ileri sürdüğü sendromun belirtilerini gösteren üçüncü sporadik olguyu oluşturmaktadır.

Keywords

• Muckle-Wells sendromu
• Progresif işitme kaybı
• Renal amiloidoz
• Kronik ürtiker
• Otoinflamatuar sendrom

References

1. 1. Gafni Ü, Sohar E, Heller H : The inherilidel amyloidosis, Their clinical and theoretical significance : Lancet 1 : 71-74, 1964
2. 2. Cohen AS ;: Amyloidosis. New Eng 1. Med 277 : 574-580, 1967
3. 3. Mahloudji M, Teasdall RD, Adamkiewicz JJ, Hartmann WH, Lambird PA, McKusick VA : The genetic amyloidosis. With particular reference to hereditary ncuropathic amyloidosis, type Il (Indiana or Rukavina type). Medicine 48 : 1-37, 1969.
4. 4. Muckle TI, Wells M : Urticaria, deafness, and amyloidosis : A new heredo-familinl syndrome. Ouart I Med (N.S) 31 ; 235-250, 1962
5. 5. Kennedy DD, Rosenthal FD, Sneddon IB : Amyloidosis presenting as urticaria. Brit MedJ:31-37, 1966.
6. 6. Anderson V, Buch NH, Jensen MK, Killman SA : Deafness, urticaria and amyloidosis. A sporadiç case With a chromosomal aberration. Amer J Med 42 : 449 - 456, 1967
7. 7. Black JT : Amyloidosis, deafness, urticarla, and limb pains : a hereditary syndrome. Ann Intern Med 70 : 989-994, 1969
8. 8. Lagrue G, Vernant JP, Revuz J, Touraine R, Weil B : Syndrome de Muckle et Wells, Cinguieme observation familite. Nouv Presse Med 1 ; 2223-2226, 1972

9. 9. Sökmen C, Özdemir Aİ : The spectrum of renal diseases foundy by kidney biopsy in Turkey. Ann Intern Med 67 : 603-605, 1967
10. 10. Özdemir Aİ, Sökmen C : Familisl Mediterranean fever among the Turkish people. Amer J Gastroent 51 : 311-316, 1969
11. 11. Özdemir Aİ : 150 renaj amyloidosis diagnosed by percutaneous needle biopsy. In preparatlon.