Mediterranean fever gene mutations in patients with idiopathic mesangial proliferative glomerulonephritis
Background: Familial Mediterranean Fever (FMF) is the most common inherited autoinflammatory disease. Kidney involvement in FMF is usually attributed to secondary amyloidosis. Non-amyloid glomerular involvement has also been reported. Objectives: We suppose that heterozygous mutation of Mediterranea...
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Society of Diabetic Nephropathy Prevention
2018-04-01
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| Series: | Journal of Nephropathology |
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| Online Access: | https://nephropathol.com/PDF/jnp-7-45.pdf |
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| author | Jalal Etemadi Taraneh Majidi Roza Motavalli Mortaza Bonyadi Sepideh Zununi Vahed Behzad Zaker Mohammadreza Ardalan |
| author_facet | Jalal Etemadi Taraneh Majidi Roza Motavalli Mortaza Bonyadi Sepideh Zununi Vahed Behzad Zaker Mohammadreza Ardalan |
| author_sort | Jalal Etemadi |
| collection | DOAJ |
| description | Background: Familial Mediterranean Fever (FMF) is the most common inherited autoinflammatory disease. Kidney involvement in FMF is usually attributed to secondary amyloidosis. Non-amyloid glomerular involvement has also been reported. Objectives: We suppose that heterozygous mutation of Mediterranean fever (MEFV) gene could be the underlying cause in some cases of mesangial proliferative glomerulonephritis (MePGN) in FMF endemic area. Materials and Methods: This prospective study was done between 2013 and 2015 in NorthWest of Iran among the Azari-Turkish population. A panel of MEFV gene including M680I, R761H, M694V, R408Q, E148Q, A744S, F479L, P369S, V726A, M694I, and E167D were studied in a group of patients with idiopathic MePGN. Clinical characteristics and therapeutic responses were compared between those with and without a mutation. A total of 39 idiopathic MePGN patients and 156 healthy subjects were studied. Results: Heterozygote mutations of MEFV gene were detected in 11/39 (28.2%) of MePGN patients and 46/156 (17.3%) of controls. Clinical response regarding 24 hours urine protein excretion was significant in mutation-negative patients after 6 months of follow-up. Conclusions: This study shows a possible underlying role of heterozygous MEFV gene mutation in the clinical course of some case of idiopathic MePGN, particularly in FMF endemic population. |
| first_indexed | 2024-04-09T12:57:14Z |
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| id | doaj.art-90795cc33c404c11a5b63ee62064d2ed |
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| issn | 2251-8363 2251-8819 |
| language | English |
| last_indexed | 2024-04-09T12:57:14Z |
| publishDate | 2018-04-01 |
| publisher | Society of Diabetic Nephropathy Prevention |
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| series | Journal of Nephropathology |
| spelling | doaj.art-90795cc33c404c11a5b63ee62064d2ed2023-05-13T11:29:42ZengSociety of Diabetic Nephropathy PreventionJournal of Nephropathology2251-83632251-88192018-04-0172455010.15171/jnp.2018.13jnp-20180109124736Mediterranean fever gene mutations in patients with idiopathic mesangial proliferative glomerulonephritisJalal Etemadi0Taraneh Majidi1Roza Motavalli2Mortaza Bonyadi3Sepideh Zununi Vahed4Behzad Zaker5Mohammadreza Ardalan6Kidney Research Center, Tabriz University of Medical Sciences, Tabriz, IranKidney Research Center, Tabriz University of Medical Sciences, Tabriz, IranSchool of Advanced Biomedical Sciences, Tabriz University of Medical Sciences, Tabriz, IranCenter of Excellence for Biodiversity, Faculty of Natural Sciences, Tabriz University of Medical Sciences, Tabriz, IranKidney Research Center, Tabriz University of Medical Sciences, Tabriz, IranKidney Research Center, Tabriz University of Medical Sciences, Tabriz, IranKidney Research Center, Tabriz University of Medical Sciences, Tabriz, IranBackground: Familial Mediterranean Fever (FMF) is the most common inherited autoinflammatory disease. Kidney involvement in FMF is usually attributed to secondary amyloidosis. Non-amyloid glomerular involvement has also been reported. Objectives: We suppose that heterozygous mutation of Mediterranean fever (MEFV) gene could be the underlying cause in some cases of mesangial proliferative glomerulonephritis (MePGN) in FMF endemic area. Materials and Methods: This prospective study was done between 2013 and 2015 in NorthWest of Iran among the Azari-Turkish population. A panel of MEFV gene including M680I, R761H, M694V, R408Q, E148Q, A744S, F479L, P369S, V726A, M694I, and E167D were studied in a group of patients with idiopathic MePGN. Clinical characteristics and therapeutic responses were compared between those with and without a mutation. A total of 39 idiopathic MePGN patients and 156 healthy subjects were studied. Results: Heterozygote mutations of MEFV gene were detected in 11/39 (28.2%) of MePGN patients and 46/156 (17.3%) of controls. Clinical response regarding 24 hours urine protein excretion was significant in mutation-negative patients after 6 months of follow-up. Conclusions: This study shows a possible underlying role of heterozygous MEFV gene mutation in the clinical course of some case of idiopathic MePGN, particularly in FMF endemic population.https://nephropathol.com/PDF/jnp-7-45.pdffamilial mediterranean fevermefv geneazari-turkish population |
| spellingShingle | Jalal Etemadi Taraneh Majidi Roza Motavalli Mortaza Bonyadi Sepideh Zununi Vahed Behzad Zaker Mohammadreza Ardalan Mediterranean fever gene mutations in patients with idiopathic mesangial proliferative glomerulonephritis Journal of Nephropathology familial mediterranean fever mefv gene azari-turkish population |
| title | Mediterranean fever gene mutations in patients with idiopathic mesangial proliferative glomerulonephritis |
| title_full | Mediterranean fever gene mutations in patients with idiopathic mesangial proliferative glomerulonephritis |
| title_fullStr | Mediterranean fever gene mutations in patients with idiopathic mesangial proliferative glomerulonephritis |
| title_full_unstemmed | Mediterranean fever gene mutations in patients with idiopathic mesangial proliferative glomerulonephritis |
| title_short | Mediterranean fever gene mutations in patients with idiopathic mesangial proliferative glomerulonephritis |
| title_sort | mediterranean fever gene mutations in patients with idiopathic mesangial proliferative glomerulonephritis |
| topic | familial mediterranean fever mefv gene azari-turkish population |
| url | https://nephropathol.com/PDF/jnp-7-45.pdf |
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