Aortic root dilatation in patients with Alport’s syndrome
A total of 50 patients from 45 unrelated families diagnosed with Alport’s syndrome were examined. The diagnosis was based on molecular genetic testing or kidney biopsy. To assess changes in the aortic root, its volume was examined (calculation methods were given). Most patients with Alport’s syndrom...
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| Format: | Article |
| Language: | Russian |
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Ltd. “The National Academy of Pediatric Science and Innovation”
2017-04-01
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| Series: | Rossijskij Vestnik Perinatologii i Pediatrii |
| Subjects: | |
| Online Access: | https://www.ped-perinatology.ru/jour/article/view/454 |
| _version_ | 1797875247548989440 |
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| author | O. S. Groznova D. I. Toskin D. V. Shentseva M. I. Dovgan |
| author_facet | O. S. Groznova D. I. Toskin D. V. Shentseva M. I. Dovgan |
| author_sort | O. S. Groznova |
| collection | DOAJ |
| description | A total of 50 patients from 45 unrelated families diagnosed with Alport’s syndrome were examined. The diagnosis was based on molecular genetic testing or kidney biopsy. To assess changes in the aortic root, its volume was examined (calculation methods were given). Most patients with Alport’s syndrome were found to have cardiovascular disorders, such as hypertension; dilatation of the ring of the aortic valve; dilatation of the sinuses of Valsalva; some patients had dilatation of the sinotubular junction; dilatation of the ascending aorta; enlargement of the left ventricle; mitral and aortic insufficiency. In the patients with Alport’s syndrome, the median value of aortic root volume is 9.9 Z3, which substantially exceeds the permissible upper normal limit according to the general population data, which is 2.82 Z3. The rate of aortic root dilatation (volume increase) in the patients with Alport’s syndrome rises with an increase in the surface area of the body. In Alport’s syndrome, aortic root dilatation is unrelated to the presence of hypertension that occurs with a relative frequency of 0.52. Dilatation of the ascending aorta in Alport’s syndrome is diagnosed with a relative frequency of 0.37 and significantly more frequently diagnosed in male subjects (the statistical significance of a two-sided test is p=0.056; Fisher’s test is p=0.097; risk posed for men is OR = 7.2; 95% CI, 1.63-31.72. Changes in the cardiovascular system are also detected in female carriers of X-linked Alport’s syndrome; therefore they should be followed up by a cardiologist. |
| first_indexed | 2024-04-10T01:44:37Z |
| format | Article |
| id | doaj.art-8531c5f961ec4f04ad44627e9223b766 |
| institution | Directory Open Access Journal |
| issn | 1027-4065 2500-2228 |
| language | Russian |
| last_indexed | 2024-04-10T01:44:37Z |
| publishDate | 2017-04-01 |
| publisher | Ltd. “The National Academy of Pediatric Science and Innovation” |
| record_format | Article |
| series | Rossijskij Vestnik Perinatologii i Pediatrii |
| spelling | doaj.art-8531c5f961ec4f04ad44627e9223b7662023-03-13T09:12:43ZrusLtd. “The National Academy of Pediatric Science and Innovation”Rossijskij Vestnik Perinatologii i Pediatrii1027-40652500-22282017-04-01621697310.21508/1027-4065-2017-62-1-69-73415Aortic root dilatation in patients with Alport’s syndromeO. S. Groznova0D. I. Toskin1D. V. Shentseva2M. I. Dovgan3ОСП «Научно-исследовательский клинический институт педиатрии имени академика Ю.Е. Вельтищева» ФГБОУ ВО РНИМУ им. Н.И. Пирогова Минздрава РоссииОСП «Научно-исследовательский клинический институт педиатрии имени академика Ю.Е. Вельтищева» ФГБОУ ВО РНИМУ им. Н.И. Пирогова Минздрава РоссииОСП «Научно-исследовательский клинический институт педиатрии имени академика Ю.Е. Вельтищева» ФГБОУ ВО РНИМУ им. Н.И. Пирогова Минздрава РоссииОСП «Научно-исследовательский клинический институт педиатрии имени академика Ю.Е. Вельтищева» ФГБОУ ВО РНИМУ им. Н.И. Пирогова Минздрава РоссииA total of 50 patients from 45 unrelated families diagnosed with Alport’s syndrome were examined. The diagnosis was based on molecular genetic testing or kidney biopsy. To assess changes in the aortic root, its volume was examined (calculation methods were given). Most patients with Alport’s syndrome were found to have cardiovascular disorders, such as hypertension; dilatation of the ring of the aortic valve; dilatation of the sinuses of Valsalva; some patients had dilatation of the sinotubular junction; dilatation of the ascending aorta; enlargement of the left ventricle; mitral and aortic insufficiency. In the patients with Alport’s syndrome, the median value of aortic root volume is 9.9 Z3, which substantially exceeds the permissible upper normal limit according to the general population data, which is 2.82 Z3. The rate of aortic root dilatation (volume increase) in the patients with Alport’s syndrome rises with an increase in the surface area of the body. In Alport’s syndrome, aortic root dilatation is unrelated to the presence of hypertension that occurs with a relative frequency of 0.52. Dilatation of the ascending aorta in Alport’s syndrome is diagnosed with a relative frequency of 0.37 and significantly more frequently diagnosed in male subjects (the statistical significance of a two-sided test is p=0.056; Fisher’s test is p=0.097; risk posed for men is OR = 7.2; 95% CI, 1.63-31.72. Changes in the cardiovascular system are also detected in female carriers of X-linked Alport’s syndrome; therefore they should be followed up by a cardiologist.https://www.ped-perinatology.ru/jour/article/view/454детисиндром альпортасердечно-сосудистые нарушениякорень аортыобъем аортыдилатация аортыартериальная гипертензия |
| spellingShingle | O. S. Groznova D. I. Toskin D. V. Shentseva M. I. Dovgan Aortic root dilatation in patients with Alport’s syndrome Rossijskij Vestnik Perinatologii i Pediatrii дети синдром альпорта сердечно-сосудистые нарушения корень аорты объем аорты дилатация аорты артериальная гипертензия |
| title | Aortic root dilatation in patients with Alport’s syndrome |
| title_full | Aortic root dilatation in patients with Alport’s syndrome |
| title_fullStr | Aortic root dilatation in patients with Alport’s syndrome |
| title_full_unstemmed | Aortic root dilatation in patients with Alport’s syndrome |
| title_short | Aortic root dilatation in patients with Alport’s syndrome |
| title_sort | aortic root dilatation in patients with alport s syndrome |
| topic | дети синдром альпорта сердечно-сосудистые нарушения корень аорты объем аорты дилатация аорты артериальная гипертензия |
| url | https://www.ped-perinatology.ru/jour/article/view/454 |
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