scholarly journals The impact of degenerative connective tissue disorders on outcomes following endovascular aortic intervention in the Global Registry for Endovascular Aortic Treatment

Author(s):  
Christopher L Delaney ◽  
Ross Milner ◽  
Jack Loa
Author(s):  
Z. V. Nesterenko

Review article on the problem of celiac disease, which is widespread with diverse range of clinical manifestations and inadequate diagnosis, is presented. The conciderable prevalence of connective tissue disorders in the pediatric population, the important role of connective tissue in the function of all organ systems, including the gastrointestinal system, necessitates studying the impact of the impaired structure and function of connective tissue on the development of celiac symptoms. The paper describes the historical development of the concept of “celiac disease”, the process of studying the pattern of the onset and development of symptoms of the disease; modern studies explaining the varied complex mechanisms of the disease.The article cites the researchers who studied the role of connective tissue in the structure and function of all body systems with identifying the impact of connective tissue disorders on the development of the gastrointestinal pathology (abnormal motor- tonic activity, reflux disease, dysbiosis, disturbance of autonomic homeostasis) and the manifestation of celiac disease associated with connective tissue disorders.The conclusion stresses the need to consider the problem of celiac disease as a manifestation of the pathology of the whole organism with the obligatory diagnosis of comorbid diseases, including those associated with connective tissue disorders, which will provide a more successful therapy for celiac disease and an improved prognosis.


2013 ◽  
Vol 12 (3) ◽  
pp. 29-34 ◽  
Author(s):  
A. A. Semenkin ◽  
O. V. Drokina ◽  
G. I. Nechaeva ◽  
L. A. Zhivilova ◽  
A. B. Zhenatov

Aim. To assess the impact of non-specific congenital connective tissue disorders (CCTD) on the changes in arterial stiffness and endothelial function; to study the role of CCTD as an independent predictor of structural and functional arterial changes.Material and methods. The study included 147 18–50-year-old men and women with CCTD and 85 healthy volunteers, comparable by age and gender (controls). All participants underwent a screening assessment of cardiovascular risk factors (RFs) and the assessment of heart rate variability (HRV), pulse wave velocity (PWV) at baseline (PWVbas) and after sublingual administration of nitroglycerin (PWVntg), and endothelium-dependent vasodilatation (EDVD), assessed by photopletismography.Results. Compared to the control group, CCTD patients had lower values of body mass index (p<0,001), waist circumference, WC (p<0,001), hip circumference, HC (p<0,001), WC/HC ratio (p<0,005), systolic blood pressure (p<0,05), total cholesterol (p<0,001), low-density lipoprotein cholesterol (p<0,005), atherogenicity index (p<0,05), and triglycerides (p<0,005). Moreover, CCTD patients were characterized by sympathetic activation, increased PWVbas (p<0,001), PWVntg (p<0,05), and EDVD (p<0,001). According to the results of stepwise multivariate analyses of the whole sample data, CCTD was a significant independent predictor of PWVbas (p<0,001), PWVntg (p<0,005), and EDVD (p<0,005 and <0,05 in men and women, respectively).Conclusion. Patients with CCTD demonstrated increased arterial stiffness and endothelium-dependent vasodilatation, compared to healthy controls. Independently from cardiovascular RFs and autonomic dysregulation, CCTD is a significant predictor of structural and functional arterial changes and can also have a prognostic value. These findings should be taken into account in the studies which assess vascular parameters.


2021 ◽  
Vol 160 (6) ◽  
pp. S-263
Author(s):  
Zeyun Xue ◽  
Lior Abramson ◽  
Talya Miller ◽  
Kisan Thakkar ◽  
Corey J. Ketchem ◽  
...  

Author(s):  
Aleksandra Lopuszko ◽  
Cian Tan ◽  
Wahaj Munir ◽  
Mohamad Bashir

Background Aortic Aneurysm (AA) is a common atherosclerotic condition, accounting for nearly 6,000 deaths in England and up to 175,000 deaths globally each year. The pathological outward bulging of the aorta typically results from atherosclerosis or hereditary connective tissue disorders. AAs are usually asymptomatic until spontaneous rupture or detected on incidental screening. 8 in 10 patients do not survive the rupture and die either before reaching hospital or from complications following surgery. Similar to other cardiovascular pathologies (CVPs), AA is thought to be subject to chronobiological patterns of varying incidence. Methods We performed a literature review of the current literature to evaluate the association between circadian rhythms, seasonal variations, and genetic factors and the pathogenesis of AA, reviewing the impact of chronobiology. Results The incidence of AA is found to peak in the early morning (6 AM – 11 AM) and colder months, and conversely troughs towards the evening and warmer months, exhibiting a similar pattern of chronobiological rhythm as other CVPs such as myocardial infarcts, or cerebrovascular strokes. Conclusion Literature suggests there exists a clear relationship between chronobiology and the incidence and pathogenesis of ruptured AA; incidence increases in the morning (6am - 11am), and during colder months (December – January). This is more pronounced in patients with Marfan Syndrome, or vitamin D deficiency. The underlying pathophysiology and implications this has for chronotherapeutics, are also discussed. Our review shows a clear need for further research into the chronotherapeutic approach to preventing ruptured AA in the journey towards precision medicine.


VASA ◽  
2010 ◽  
Vol 39 (2) ◽  
pp. 175-179
Author(s):  
Hakimi ◽  
Geisbüsch ◽  
Gross ◽  
Hyhlik-Dürr ◽  
Hausser ◽  
...  

We want to report and discuss the indication for open surgery for an asymptomatic penetrating aortic ulcer (PAU) in the era of thoracic endovascular aortic repair (TEVAR). A 31-year-old female presented with the diagnosis of an aneurysm in the distal aortic arch. With respect to the patient’s young age, the controversial status of connective tissue disorders and in the absence of concomitant disease, open repair was indicated. There was no proof of a mycotic plaque or connective tissue disease in the microbiological-, pathological analysis and at electron-microscopy. The patient was discharged on the thirteenth postoperative day. In spite of good preliminary results of TEVAR in PAU, in selective cases there is still an indication for open surgery.


2012 ◽  
Vol 3 (6) ◽  
pp. 19-21
Author(s):  
Dr.Ashish Patela ◽  
◽  
Dr.Dipika Sathvara ◽  
Dr.Himanshu Patel ◽  
Dr.C.Chakrabarti Dr.C.Chakrabarti

Author(s):  
L.M. Karamova ◽  
V.O. Krasovskiy ◽  
D.M. Vagapova ◽  
N.V. Vlasova ◽  
A.S. Khafizova ◽  
...  

Relevance. The importance of studying and analyzing contribution of occupational risk factors in musculoskeletal disorders among emergency medical personnel is related to high disease incidence rates and work specifics. The objective of our research envisaged formalization of information obtained for the analysis and assessment of occupational risks of developing musculoskeletal and connective tissue disorders in ambulance personnel. Results. According to professional medical examination results, musculoskeletal and connective tissue disorders ranked second among all diagnosed diseases. The relative risk of developing those disorders was 70% indicating the link between them and transport vibration (category 1) affecting the backbone during long trips in ambulance cars.


Author(s):  
Andrea Zanichelli ◽  
Henriette Farkas ◽  
Laurance Bouillet ◽  
Noemi Bara ◽  
Anastasios E. Germenis ◽  
...  

AbstractHereditary angioedema (HAE) is a rare condition, mostly due to genetic deficiency of complement C1 inhibitor (C1-INH). The rarity of HAE impedes extensive data collection and assessment of the impact of certain factors known to affect the course of this disabling and life-threatening disease. Establishing a global registry could assist to overcome such issues and provides valuable patient data from different countries. The HAE Global Registry is a disease-specific registry, with web-based electronic support, where data are provided by physicians and patients through a dedicated application. We collected data between January 1, 2018, and August 31, 2020. Data on 1297 patients from 29 centers in 5 European countries were collected. At least one attack was recorded for 497 patients during the study period. Overall, 1182 patients were diagnosed with HAE type 1 and 115 with type 2. At the time of database lock, 389 patients were taking long-term prophylactic medication, 217 of which were on danazol. Most recorded attacks affected the abdomen, were generally moderate in severity, and occurred in patients who were not on prophylactic treatment (70.6%, 6244/8848). The median duration of attacks was 780 min (IQR 290–1740) in patients on prophylactic medication and 780 min (IQR 300–1920) in patients not on continuous prophylactic medication. In conclusion, the establishment of a registry for C1-INH-HAE allowed collection of a large amount of data that may help to better understand the clinical characteristics of this disease. This information may enhance patient care and guide future therapeutic decisions.


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