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.jpg): Failed to open stream: No such file or directory in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 117 Diagn+Pathol
2015 ; 10
(ä): 104
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Diffuse and multifocal nephrogenic adenoma with Familial Mediterranean Fever: a
case report with molecular study
#MMPMID26428868
Ishikawa N
; Amano C
; Taketani T
; Kumori K
; Harada Y
; Hiraiwa H
; Itamura K
; Maruyama R
Diagn Pathol
2015[Jul]; 10
(ä): 104
PMID26428868
show ga
Nephrogenic adenoma, also referred to nephrogenic metaplasia, is a benign
proliferative lesion of urothelium, usually associated with chronic physical
stimuli or inflammation. Familial Mediterranean fever is an inherited autosomal
recessive disease characterized by recurrent short episodes of fever. The site of
mutation is found in MEFV gene which controls inflammatory responses. We have
experienced a case of nephrogenic adenoma in a 16-year-old girl with Familial
Mediterranean Fever, showing proliferative lesions diffusely in the urinary
bladder and multifocally in the other parts of urinary tract. These lesions
disappeared after colchicine treatment. We searched for MEFV gene mutation using
the specimen from the resected urinary bladder and detected heterozygous mutation
of E148Q. There is a possibility that control of inflammation caused by the
surgery for vesicoureteral reflux in the local site didn't work well on the
background of heterozygous mutation of MEFV gene, and as a result, nephrogenic
adenoma appeared. This is the first report of a combination of two rare diseases.
We have to be aware that nephrogenic adenoma can occur in association with
Familial Mediterranean Fever, and the former condition should be taken into
consideration when rendering a correct pathological diagnosis.