scholarly journals The role of echocardiography in the assessment of hemodynamics in patients with connective tissue dysplasia

2015 ◽  
Vol 14 (2) ◽  
pp. 16-25
Author(s):  
E. V. Fomenko ◽  
S. B. Tkachenko ◽  
N. F. Beresten ◽  
E. S. Pavochkina

The article describes the features of ultrasound diagnostics and central hemodynamics in patients with minor heart anomalies. In vivo visualization of these anomalies has become possible after the introduction of echocardiography. The working classification of minor heart anomalies, as well as the description of clinically significant syndromes and abnormalities are considered. The role of connective tissue dysplasia in the development of cardiac pathology is highlighted, and its place in the structure of connective tissue dysplasia syndrome of the heart and heritable disorders of connective tissue is described.

2016 ◽  
pp. 149-154
Author(s):  
Oleksandr Piantkovskiy

In recent years medical practitioners more often pay their attention on role of pathology of different organs and systems of human’s body which are associated with connective tissue dysplasia. The importance of this problem is caused by the great prevalence of connective tissue dysplasia, systemacity of damage, high probability of different diseases’ formation. Connective tissue dysplasia is the violation of the connective tissue structure during embryonic and postnatal periods because of genetically modified fibrillogenesis of extracellular matrix, leading to homeostasis disorder on tissue and organ levels with the progressive course. There was held the clinical neurological examination with 120 patients, who had neurological features of vertebral syndrome of lumbosacral spine. Analysis of the clinical examination results demonstrated that patients with vertebral syndrome of lumbosacral spine (p<0,05) more common can occur pathology of joints and varicose veins of the lower extremities. Significantly (p<0,05) (scoliosis, kyphosis, kyphoscoliosis, tendency to dislocation, stretching the ligaments) increase the duration of treatment and the expression of a pain syndrome. The results of examination and monitoring of patients in the dynamics of the treatment showed that patients with vertebral syndrome of lumbosacral spine (p<0,05) more often have anatomical changes in the lumbosacral spine than patients without evidence of DST.


1999 ◽  
Vol 276 (2) ◽  
pp. L269-L279 ◽  
Author(s):  
K. Zay ◽  
S. Loo ◽  
C. Xie ◽  
D. V. Devine ◽  
J. Wright ◽  
...  

Mineral dusts produce emphysema, and administration of dust to rats results in the rapid appearance of desmosine and hydroxyproline in lavage fluid, confirming that dusts directly induce connective tissue breakdown. To examine the role of neutrophils and α1-antitrypsin (α1-AT) in this process, we instilled silica or coal into normal rats or rats that had been pretreated with antiserum against neutrophils. One day after dust exposure, lavage fluid neutrophils and desmosine and hydroxyproline levels were all elevated; treatment with antiserum against neutrophils reduced neutrophils by 75%, desmosine by 40–50%, and hydroxyproline by 25%. By 7 days, lavage fluid neutrophils and desmosine level had decreased, whereas macrophages and hydroxyproline level had increased. By ELISA analysis, lavage fluid α1-AT levels were increased four- to eightfold at both times. On Western blot, some of the α1-AT appeared as degraded fragments, and by HPLC analysis, 5–10% of the methionine residues were oxidized. At both times, lavage fluid exhibited considerably elevated serine elastase inhibitory capacity and also showed elevations in metalloelastase activity. We conclude that, in this model, connective tissue breakdown is initially driven largely by neutrophil-derived proteases and that markedly elevated levels of functional α1-AT do not prevent breakdown, thus providing in vivo support for the concept of quantum proteolysis proposed by Liou and Campbell (T. G. Liou and E. J. Campbell. Biochemistry 34: 16171–16177, 1995). Macrophage-derived proteases may be of increasing importance over time, especially in coal-treated animals.


2019 ◽  
Vol 7 (3) ◽  
pp. 29-34
Author(s):  
R.Yu. Andreev ◽  
◽  
P.I. Rasner ◽  
E.A. Prilepskaya ◽  
I.V. Semenyakin ◽  
...  

2015 ◽  
Vol 2015 ◽  
pp. 1-7 ◽  
Author(s):  
Francesca Ingegnoli ◽  
Roberta Gualtierotti ◽  
Annalisa Orenti ◽  
Tommaso Schioppo ◽  
Giovanni Marfia ◽  
...  

In patients with Raynaud’s phenomenon (RP), the role of medical history, capillaroscopy, and autoantibodies in order to provide an early diagnosis of connective tissue disease (CTD) were examined. 115 consecutive adults with uni-, bi-, or triphasic colour changes of the fingers were studied. RP was bilateral in 92.7% of patients. The middle finger was significantly more affected. A lack of association between fingers affected by RP and fingers with capillary abnormalities was observedOR=0.75(0.34–1.66). RP with the cyanotic phase had a higher risk at capillaroscopy to have hemorrhagesOR=4.46(1.50–13.30) and giant capillariesOR=24.85(1.48–417.44). The thumb and triphasic involvement have an OR of 1.477 and 1.845, respectively. RP secondary to systemic sclerosis (SSc) had greater value of VAS pain (p=0.011). The presence of anti-centromere antibodies was significantly associated with a higher risk of SSc (p<0.001). 44.3% of subjects had uniphasic blanching of the fingers, and among these, 27% was diagnosed as having an overt or suspected CTD. Markers of a potential development of CTDs include severe RP symptoms, positive autoantibodies, and capillary abnormalities. These data support the proposal to not discharge patients with uniphasic blanching of the fingers to avoid missing the opportunity of an early diagnosis.


2013 ◽  
Vol 39 (6) ◽  
pp. 728-741 ◽  
Author(s):  
Daniel Antunes Silva Pereira ◽  
Alexandre de Melo Kawassaki ◽  
Bruno Guedes Baldi

The initial evaluation of patients with interstitial lung disease (ILD) primarily involves a comprehensive, active search for the cause. Autoantibody assays, which can suggest the presence of a rheumatic disease, are routinely performed at various referral centers. When interstitial lung involvement is the condition that allows the definitive diagnosis of connective tissue disease and the classical criteria are met, there is little debate. However, there is still debate regarding the significance, relevance, specificity, and pathophysiological role of autoimmunity in patients with predominant pulmonary involvement and only mild symptoms or formes frustes of connective tissue disease. The purpose of this article was to review the current knowledge of autoantibody positivity and to discuss its possible interpretations in patients with ILD and without clear etiologic associations, as well as to enhance the understanding of the natural history of an allegedly new disease and to describe the possible prognostic implications. We also discuss the proposition of a new term to be used in the classification of ILDs: lung-dominant connective tissue disease.


2005 ◽  
Vol 54 (3) ◽  
pp. 5-11
Author(s):  
D. F. Kostyuchek ◽  
А. S. Gordeladze ◽  
А. S. Klyukovkina

Theclinical-morphological, immunohistochemical examination of the elongation of uterine cervix (EUC) in patients aged of 29-70 years old was conducted. The correlation of the systemic connective tissue dysplasia (SCTD) with the reconstruction of the tissue architectonics of the cervix, disorders of collagen synthesis, redistribution of the I, III and IV types of collagen was established, which testify the role of SCTD in the pathogenesis of the EUC.


2021 ◽  
Vol 10 (1) ◽  
pp. 206-223
Author(s):  
M.V. Yavorskaya ◽  
N.A. Kravtsova

The article presents the results of a study of psychological adaptation in patients with cardiac pathology against the background of connective tissue dysplasia, conducted at the Regional Clinical Center for Specialized Types of Medical Care in Vladivostok. It was hypothesized that patients with concomitant connective tissue dysplasia will have lower adaptive capacity than patients without this syndrome. The study involved 32 girls 16–18 years old (M=16.72; SD=0.41) with cardiovascular pathology on the background of connective tissue dysplasia and 32 girls of the same age (M=16.78; SD=0.40) with cardiovascular disease without connective tissue dysplasia. To assess the severity of adaptive capabilities, the following methods were used: the Questionnaire of socio-psychological adaptation (A.K. Osnitsky, 2004), the Multi-level Personality Questionnaire "Adaptability" developed by А.G. Maklakov and S.V. Chermyanin, the Communicative Tolerance Questionnaire (V.V. Boyko), K. Ryff's scale of Psychological Well-being adapted by N.N. Lepeshinsky, the Oxford Happiness Inventory adapted by A. Lisitsina (2003). The significant intergroup differences (p&lt;0.05) were revealed in terms of neuropsychic and emotional stability, working capacity, self-esteem, need for communication, behavioral features, experiences of psychological well-being, feelings of happiness, socialization and adaptation, motivation for activity, tolerance to adverse factors environment, as well as indicators of the communicative capabilities of the individual. The results obtained indicate reduced indicators of psychological adaptation in adolescent patients with cardiac pathology against the background of connective tissue dysplasia. These results can be used in the development of individual psychocorrectional programs to improve the social adaptation of girls with connective tissue dysplasia syndrome.


Author(s):  
A. Pyantkovsky

In recent decades, increasing attention attracts practitioners role of pathology of different organs and systems of the human body associated with connective tissue dysplasia (DST). The significance of this problem is due to a large prevalence of GOST, system damage, high probability of the formation of different kinds of diseases. Dysplasia of connective tissue (display breach) a violation of the structure of the connective tissue in the embryonic and postnatal periods due to genetically modified extracellular matrix, leading to frustration on tissue homeostasis and organ levels with a progressive course. There have been clinical and neurological examination of 120 patients with neurological manifestations of the syndrome vertebrogenic lumbar spine. Analysis of the results of the clinical study demonstrated that in patients with vertebral syndrome, lumbosacral spine against the background displays GOST significantly (p <0.05) more common protrusion m / s disk changes are more pronounced yellow ties as its thickening. And also more common syndrome joints and vascular dystonia. Significantly (p <0.05) displays GOST (scoliosis, kyphosis, kyphoscoliosis, joints, tendency to dislocation, stretching the ligaments of the joints) - increases the duration of treatment and the severity of pain. The data screening and monitoring of patients in the dynamics of the treatment showed that patients with symptoms GOST significantly (p <0.05) more often aggravation vertebrogenic syndrome lumbosacral spine.


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