Case report no. 9. Recurrence of amyloid in a kidney allograft. Eva Honsová Institute for Clinical and Experimental Medicine Prague, Czech Republic. Clinical history. A 55-year-old man was admitted to the hospital for severe hypertension in 1989.
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Institute for Clinical and Experimental Medicine
Prague, Czech Republic
Both parents dead (mother: 40, abdominal tumor;
father: 77, DM, IHD; sister: 7, car accident)
2 children, a son and a daughter: healthy
No evidence of kidney disease
GGraft biopsy sample, IF & IH detection fibrinogen A rGenetic studies were performed and mutation in the fibrinogen gene was confirmed (Glu526Val)
The most common types: AA+AL
AA (commercial antibodies)
AL(low reactivityof commercial
Abs in paraffinsections)
Hereditary amyloidoses: more frequently recognized, are gaining in clinical importance. Autosomal dominant, variable penetrance, family history is lacking, clinical manifestation at older ages, therefore the correct diagnosis is challenging
Each type requires different therapy, dg must be
based on identification of the protein
animal experimental models
(ducks, geese: pate de foie gras)
patients at risk of amyloidosis?