Abstract:
BACKGROUND/AIMS:Evaluation of the risk factors, and phenotype-genotype correlation of familial Mediterranean fever (FMF) gene (MEFV) and serum amyloid A1 (SAA1) gene polymorphisms in renal amyloidosis. METHODS:We investigated MEFV and SAA1 genotypes (alpha, beta, and gamma isoforms) in 50 FMF patients and 50 healthy children. Tel-Hashomer criteria were used for the diagnosis and severity scoring of FMF. RESULTS:The most common MEFV mutation and SAA1 genotype were M694V/M694V (n = 26/50) and SAA1 alpha/alpha (n = 26/50), respectively. Positive family history for amyloidosis was significantly higher (p < 0.001) with more severe clinical course (p = 0.006) in the amyloidosis group than the non-amyloid group. In M694V/M694V mutation, erysipelas-like skin erythema (p = 0.029), arthritis (p = 0.004), arthralgia (p < 0.001) were significantly more frequent with higher severity scores (p = 0.008) than the patients with other mutations. Comparison of the SAA1 alpha/alpha genotype with other genotypes revealed more frequent arthritis (p = 0.003) in the SAA1 alpha/alpha genotype. In amyloidosis group patients having both M694V/M694V and SAA1 alpha/alpha genotypes were the largest subgroup (n = 14, p < 0.001). Logistic regression analysis for amyloidosis corrected risk revealed a 1.2 times increase in M694V/M694V, a 2.4 times increase in SAA1 alpha/alpha genotypes and a 2.5 times increase when both are together. CONCLUSION:Positive family history for amyloidosis and presence of SAA1 alpha/alpha genotype in M694V/M694V mutation may predispose to amyloidosis by increasing the clinical severity. Therefore, in such children early colchicine treatment might be recommended even if they are asymptomatic.
journal_name
Am J Nephroljournal_title
American journal of nephrologyauthors
Delibaş A,Oner A,Balci B,Demircin G,Bulbul M,Bek K,Erdoğan O,Baysun S,Yilmaz Edoi
10.1159/000087824subject
Has Abstractpub_date
2005-09-01 00:00:00pages
434-40issue
5eissn
0250-8095issn
1421-9670pii
87824journal_volume
25pub_type
杂志文章abstract:BACKGROUND:We determined the familial aggregation of end-stage renal disease (ESRD) in a large, population-based sample of incident ESRD cases to assess the feasibility of developing a targeted screening and prevention program directed at members of families at high risk for kidney disease. METHODS:Between January 1, ...
journal_title:American journal of nephrology
pub_type: 杂志文章
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journal_title:American journal of nephrology
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doi:10.1159/000013326
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journal_title:American journal of nephrology
pub_type: 杂志文章
doi:10.1159/000166711
更新日期:1983-07-01 00:00:00
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journal_title:American journal of nephrology
pub_type: 临床试验,杂志文章,多中心研究,随机对照试验
doi:10.1159/000169049
更新日期:1996-01-01 00:00:00
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pub_type: 杂志文章
doi:10.1159/000443775
更新日期:2015-01-01 00:00:00
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journal_title:American journal of nephrology
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journal_title:American journal of nephrology
pub_type: 杂志文章
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更新日期:2014-01-01 00:00:00
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更新日期:2011-01-01 00:00:00
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journal_title:American journal of nephrology
pub_type: 杂志文章
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更新日期:2011-01-01 00:00:00
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journal_title:American journal of nephrology
pub_type: 杂志文章
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更新日期:2013-01-01 00:00:00
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journal_title:American journal of nephrology
pub_type: 临床试验,杂志文章
doi:10.1159/000109968
更新日期:2008-01-01 00:00:00
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journal_title:American journal of nephrology
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更新日期:1984-01-01 00:00:00
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pub_type: 杂志文章,随机对照试验
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更新日期:2016-01-01 00:00:00
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pub_type: 临床试验,杂志文章,多中心研究
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journal_title:American journal of nephrology
pub_type: 杂志文章
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更新日期:1982-01-01 00:00:00
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pub_type: 杂志文章
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更新日期:2017-01-01 00:00:00