Fr200 OVERLAP OF EOSINOPHILIC ESOPHAGITIS, AUTOIMMUNE, AND CONNECTIVE TISSUE DISORDERS, AND THE IMPACT ON TREATMENT RESPONSE

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.

Download Full-text

Classification, diagnosis, and pathogenesis of rare heritable connective tissue disorders: The impact of differential ultrastructural aberrations of dermal connective tissue components

Ultrastructural Pathology ◽

10.1080/01913123.2016.1269409 ◽

2017 ◽

Vol 41 (1) ◽

pp. 71-72

Author(s):

Ingrid Hausser

Keyword(s):

Connective Tissue ◽

Connective Tissue Disorders ◽

The Impact ◽

Dermal Connective Tissue ◽

Heritable Connective Tissue Disorders

Download Full-text

High prevalence of eosinophilic esophagitis in patients with inherited connective tissue disorders

Journal of Allergy and Clinical Immunology ◽

10.1016/j.jaci.2013.02.030 ◽

2013 ◽

Vol 132 (2) ◽

pp. 378-386 ◽

Cited By ~ 104

Author(s):

J. Pablo Abonia ◽

Ting Wen ◽

Emily M. Stucke ◽

Tommie Grotjan ◽

Molly S. Griffith ◽

...

Keyword(s):

Connective Tissue ◽

Eosinophilic Esophagitis ◽

Connective Tissue Disorders ◽

High Prevalence

Download Full-text

NON-SPECIFIC CONGENITAL CONNECTIVE TISSUE DISORDERS AS AN INDEPENDENT PREDICTOR OF STRUCTURAL AND FUNCTIONAL ARTERIAL CHANGES

CARDIOVASCULAR THERAPY AND PREVENTION ◽

10.15829/1728-8800-2013-3-29-34 ◽

2013 ◽

Vol 12 (3) ◽

pp. 29-34 ◽

Cited By ~ 3

Author(s):

A. A. Semenkin ◽

O. V. Drokina ◽

G. I. Nechaeva ◽

L. A. Zhivilova ◽

A. B. Zhenatov

Keyword(s):

Connective Tissue ◽

Arterial Stiffness ◽

Independent Predictor ◽

Density Lipoprotein ◽

Control Group ◽

Sublingual Administration ◽

Connective Tissue Disorders ◽

Significant Independent Predictor ◽

Men And Women ◽

The Impact

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.

Download Full-text

Aortic Aneurysm Disease - Make Room for Chronobiology

10.22541/au.161408828.86982396/v1 ◽

2021 ◽

Author(s):

Aleksandra Lopuszko ◽

Cian Tan ◽

Wahaj Munir ◽

Mohamad Bashir

Keyword(s):

Vitamin D ◽

Aortic Aneurysm ◽

Connective Tissue ◽

Literature Review ◽

Genetic Factors ◽

Early Morning ◽

Connective Tissue Disorders ◽

Cardiovascular Pathologies ◽

The Impact ◽

Underlying Pathophysiology

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.

Download Full-text

Treatment of an asymptomatic penetrating aortic ulcer in a young patient

VASA ◽

10.1024/0301-1526/a000024 ◽

2010 ◽

Vol 39 (2) ◽

pp. 175-179

Author(s):

Hakimi ◽

Geisbüsch ◽

Gross ◽

Hyhlik-Dürr ◽

Hausser ◽

...

Keyword(s):

Electron Microscopy ◽

Connective Tissue ◽

Aortic Arch ◽

Open Surgery ◽

Open Repair ◽

Thoracic Endovascular Aortic Repair ◽

Concomitant Disease ◽

Connective Tissue Disorders ◽

Penetrating Aortic Ulcer ◽

Pathological Analysis

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 patients 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.

Download Full-text