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Reports of Biochemistry and Molecular Biology
rbmb.net
Basic Sciences
http://rbmb.net
1
admin
2322-3480
2322-3480
10.61882/rbmb
en
jalali
1396
2
1
gregorian
2017
5
1
5
2
online
1
fulltext
en
Genetic Analysis of Southwestern Iranian Patients with Familial Mediterranean Fever
زیست شناسی ملکولی
Molecular Biology
مقالات اصلی
Original Article
<p style="text-align: justify;"><strong><em>Background:</em></strong> Familial Mediterranean fever (FMF) is an autosomal recessive genetic disorder characterized by recurrent episodes of self-limited fever and serosal tissues inflammation.</p>
<p style="text-align: justify;"><strong><em>Methods:</em></strong> To evaluate clinical symptoms and common genetic mutations in southwestern Iranian patients with FMF, 20 unrelated patients were enrolled in this study based on clinical criteria. A panel of 12 common <em>MEFV</em> gene mutations was tested.</p>
<p style="text-align: justify;"><strong><em>Results:</em></strong> The most frequent clinical presentations of the patients were fever, colicky abdominal pain and arthritis. Eighteen patients responded completely to colchicine therapy.</p>
<p style="text-align: justify;"><em>MEFV </em>gene mutations were detected in only 40% of the patients. The most common mutation was E148Q, detected in five patients (25%). The V726A, M694V and P369S mutations were each observed in one patient.</p>
<p style="text-align: justify;"><strong><em>Conclusions:</em></strong> Although none of the 12 mutations we included in our test panel was detected in 60% of our patients, all of them had FMF symptoms and responded well to colchicine. <em>MEFV</em> full gene sequencing analysis in these patients may lead to finding new mutations in southwestern Iranian FMF patients which would be helpful in designing a local diagnostic kit.</p>
Familial Mediterranean fever, MEFV gene mutations
117
120
http://rbmb.net/browse.php?a_code=A-10-1-76&slc_lang=en&sid=1
Mahmoud
Haghighat
10031947532846006234
10031947532846006234
No
Gastroenterohepatology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran - Department of Pediatrics, Shiraz University of Medical Sciences, Shiraz, Iran.
Mozhgan
Moghtaderi
10031947532846006235
10031947532846006235
No
Allergy Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.
Shirin
Farjadian
farjadsh@sums.ac.ir
10031947532846006236
10031947532846006236
Yes
Allergy Research Center, Shiraz University of Medical Sciences, Shiraz, Iran - Department of Immunology, Shiraz University of Medical Sciences, Shiraz, Iran.