Hipermobilidade articular em pacientes com prolapso da valva mitral

Cavenaghi, Simone; Marino, Lais Helena Carvalho; Oliveira, Patrícia Pantaleão; Lamari, Neuseli Marino

doi:10.1590/s0066-782x2009000900016

Cited by 6 publications

(1 citation statement)

References 19 publications

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“…При пролапсе митрального клапана, как и при других ННСТ, имеются системные проявления дефекта соединительной ткани. Так при ПМК значимо чаще встречаются деформация грудной клетки -воронковидная [29][30][31][32] и килевидная [33][34][35], сколиоз [36,37], синдром прямой спины [38], долихостеномелия и арахнодактилия [39], гипермобильность суставов [40,41] и изменения кожи [42].…”

Section: Mitral Valve Prolapse: a Small Anomaly Of The Heart A Nonspe...unclassified

Mitral Valve Prolapse: A Small Anomaly of the Heart, a Nonspecific Manifestation of Hereditary Connective Tissue Disorders or an Independent Syndrome?

et al. 2022

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The review article presents modern concepts of the mitral valve prolapse. The issues of pathogenesis, diagnostic criteria of primary and probable prolapse are reflected, the issues of epidemiology are considered in detail. The problems associated with significant overdiagnosis of this condition in our country (up to 36.8%) are noted, which is due to ignoring internationally agreed algorithms and incorrect interpretation of the results obtained during echocardiography. When using a parasternal two-chamber position and choosing a diagnostic threshold of 3 mm or more, mitral valve prolapse is detected in 4.3% of practically healthy young people, which is comparable to population-based world studies, in the absence of sex differences. The place of mitral valve prolapse in the algorithms for diagnosing hereditary syndromes - Marfan and Ehlers-Danlos, its pleiotropy, which should be taken into account when stratifying the risk of clinically significant events and in case of probable prolapse, is discussed. The situations that allow to interpret mitral valve prolapse as an independent hereditary syndrome or a small anomaly of the heart are indicated. The article presents current views on the pathogenesis of mitral valve prolapse, the role of transforming growth factor-β in the progression of myxomatous degeneration of the valves, the development of manifestations of cardiomyopathy. The prognostic value of primary prolapse in its natural course is shown - the progression of myxomatosis of the valves, mitral insufficiency, dilation of the main vessels.

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Section: Mitral Valve Prolapse: a Small Anomaly Of The Heart A Nonspe...unclassified

Mitral Valve Prolapse: A Small Anomaly of the Heart, a Nonspecific Manifestation of Hereditary Connective Tissue Disorders or an Independent Syndrome?

et al. 2022

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The impairment of lysyl oxidase in keratoconus and in keratoconus-associated disorders

Ďuďáková

Jirsová

2013

J Neural Transm

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Keratoconus (KC) is an eye disease characterized by the progressive thinning and protrusion of the cornea, which results in the loss of visual acuity. This disorder remains poorly understood, although recent studies indicate the involvement of genetic and environmental factors. Recently, we have found that the distribution of the cross-linking enzyme lysyl oxidase (LOX) is markedly decreased in about 63 % of keratoconic specimens. Similarly, LOX activity is significantly reduced by 38 % compared to control tissue. Nearly 70 systemic disorders have been reported in association with KC, most of them affecting the extracellular matrix. In this review we attempted to ascertain whether any KC-associated diseases exhibit signs that may reflect LOX impairment. We hypothesized that very similar changes in the extracellular matrix, particularly at the level of collagen metabolism, including LOX impairment in mitral leaflets, may reflect an association between KC and mitral valve prolapse. Moreover, this putative association is supported by the high frequency of Down syndrome in both diseases. Among other disorders that have been found to coincide with KC, we did not find any in which the LOX enzyme may be directly or indirectly impaired. On the other hand, in cases where KC is present along with other connective tissue disorders (Marfan syndrome, Ehlers-Danlos syndrome and others), KC may not arise as a localized manifestation, but rather may be induced as the result of a more complex connective tissue disorder.

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