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

2013 ◽  
Vol 132 (2) ◽  
pp. 378-386 ◽  
Author(s):  
J. Pablo Abonia ◽  
Ting Wen ◽  
Emily M. Stucke ◽  
Tommie Grotjan ◽  
Molly S. Griffith ◽  
...  
2020 ◽  
Vol 3 (3) ◽  
pp. 182-188
Author(s):  
I.Yu. Il’ina ◽  
◽  
A.A. Chikisheva ◽  

This paper discusses connective tissue disorders (CTD), in particular, undifferentiated connective tissue disease (UCTD) whose importance is accounted for by a high prevalence of this condition. Generalized connective tissue damage that involves reproductive system significantly affects the course of pregnancy and delivery. The complications of pregnancy, delivery, and postpartum period in women with UCTD which often require surgical procedures (i.e., amniotomy, episiotomy, perineotomy, C-section etc.) are described. The paper highlights the role of magnesium which is of crucial importance for the complex biosynthesis of extracellular matrix during connective tissue formation and fibroblast morphological functional status. The tests for connective tissue metabolic disorders (blood oxyproline and fibronectin, urine pyrilinks-D and glycosaminoglycans etc.) are addressed. Considering the lack of the reliable diagnostic (biochemical and genetic) criteria for UCTD, the authors advocate the need for a complex examination using anamnestic data and the results of clinical, instrumental, and laboratory tests.KEYWORDS: connective tissue disorders, hemostasis, complications of pregnancy and delivery, magnesium deficiency, endothelial dysfunction, markers of collagen degradation, genital prolapse.FOR CITATION: Il’ina I.Yu., Chikisheva A.A. Course of the pregnancy in women with connective tissue disorders. Russian Journal of Woman and Child Health. 2020;3(3):182–188. DOI: 10.32364/2618-8430-2020-3-3-182-188


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

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):  
B. N. Davydov ◽  
D. A. Domenyuk ◽  
S. V. Dmitrienko ◽  
T. A. Kondratyeva ◽  
Yu. S. Harutyunyan

Relevance. The high prevalence of dysplastic disorders involving connective tissue, and its negative effecton the development of dentoalveolar anomalies, carious and non-carious lesions of the teeth, periodontopathy, temporomandibular joint issues in the child population, lay the basis for improving diagnostics algorithms. Enhancing the already available standards is of greatest importance for children at the initial stages of diagnostics when evaluating the external signs of dysplastic disorders.Purpose – improving diagnostics algorithms for connective tissue dysplasia (CTD) in children in primary dental care facilities based on the evaluation of external phenotype signs and maxillofacial morphological features.Materials and methods. Depending on the external phenotype manifestations severity, as well as on laboratory, clinical and instrumental signs, the 92 children with CTD were divided into groups with mild, moderate and severe degrees of undifferentiated dysplasia. Gnathometric and biometric examinations of the maxillofacial area were performed through traditional methods, whereas the diagnosis was set following the generally accepted classifications. The diagnosis confirmation implied evaluation through cone beam computed imaging.Results. The nature and the intensity of morphofunctional disorders in the craniofacial structures (“small” stigmas) depend on the severity of connective tissue dysplastic disorders.Conclusions. The change direction vector in the facial and brain parts of cranium in children with CTD is aimed at increasing hypoplastic tendencies and dolichocephalia, proof to that being the following constitutional and morphological features: the prevalence of the vertical type of face skeleton growth over the horizontal and neutral ones; a convex face profile with a disproportionate general heights of the face skeleton; reduction of latitudinal with an increase in altitude facial parameters; a narrow short branch of the lower jaw; the upper jaw displaced downwards and forward; a decrease in the size of the apical basis of the lower dentition, the lower jaw body, as well as the height and width of the lower jaw branches. 


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.


2021 ◽  
Vol 23 (1) ◽  
Author(s):  
Alejandro Perez-Casares ◽  
Audrey Dionne ◽  
Kimberlee Gauvreau ◽  
Ashwin Prakash

Abstract Background Aortic stiffness has been shown to be abnormal in patients with bicuspid aortic valve (BAV), and is considered a component of the aortopathy associated with this condition. Progressive aortic stiffening associated with aging has been previously described in normal adults. However, it is not known if aging related aortic stiffening occurs at the same rate in BAV patients. We determined the longitudinal rate of decline in segmental distensibility in BAV patients using serial cardiovascular magnetic resonance (CMR) studies, and compared to previously published results from a group of patients with connective tissue disorders (CTD). Methods A retrospective review of CMR and clinical data on children and adults with BAV (n = 49, 73% male; 23 ± 11 years) with at least two CMRs (total 98 examinations) over a median follow-up of 4.1 years (range 1–9 years) was performed to measure aortic distensibility at the ascending (AAo) and descending aorta (DAo). Longitudinal changes in aortic stiffness were assessed using linear mixed-effects modeling. The comparison group of CTD patients had a similar age and gender profile (n = 50, 64% male; 20.6 ± 12 years). Results Compared to CTD patients, BAV patients had a more distensible AAo early in life but showed a steeper decline in distensibility on serial examinations [mean 10-year decline in AAo distensibility (× 10−3 mmHg−1) 2.4 in BAV vs 1.3 in CTD, p = 0.005]. In contrast, the DAo was more distensible in BAV patients throughout the age spectrum, and DAo distensibility declined with aging at a rate similar to CTD patients [mean 10 year decline in DAo distensibility (× 10−3 mmHg−1) 0.3 in BAV vs 0.4 in CTD, p = 0.58]. Conclusions On serial CMR measurements, AAo distensibility declined at significantly steeper rate in BAV patients compared to a comparison group with CTDs, while DAo distensibility declined at similar rates in both groups. These findings offer new mechanistic insights into the differing pathogenesis of the aortopathy seen in BAV and CTD patients.


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