scholarly journals Pathology of the Aorta and Aorta as Homograft

2021 ◽  
Vol 8 (7) ◽  
pp. 76
Author(s):  
Gaetano Thiene ◽  
Cristina Basso ◽  
Mila Della Barbera
Keyword(s):  
Inflammatory Diseases ◽  
Dissecting Aneurysm ◽  
Ascending Aorta ◽  
Congenital Defects ◽  
Elastic Fibers ◽  
Aortic Dilatation ◽  
Structural Deterioration ◽  
History Of ◽  
Medial Necrosis ◽  
Genetically Determined

The aorta is not a rigid tube, it is an “organ” with lamellar units, consisting of elastic fibers, extracellular matrix and smooth muscle cells in between as parenchyma. Several diseases may occur in the natural history of the aorta, requiring replacement of both semilunar cusps and ascending aorta. They may be congenital defects, such as bicuspid aortic valve and isthmal coarctation with aortopathy; genetically determined, such as Marfan and William syndromes; degenerative diseases, such as atherosclerosis and medial necrosis with aortic dilatation, valve incompetence and dissecting aneurysm; inflammatory diseases such as Takayasu arteritis, syphilis, giant cell and IgM4 aortitis; neoplasms; and trauma. Aortic homografts from cadavers, including both the sinus portion with semilunar cusps and the tubular portion, are surgically employed to replace a native sick ascending aorta. However, the antigenicity of allograft cells, in the lamellar units and interstitial cells in the cusps, is maintained. Thus, an immune reaction may occur, limiting durability. After proper decellularization and 6 months’ implantation in sheep, endogenous cell repopulation was shown to occur in both the valve and aortic wall, including the endothelium, without evidence of inflammation and structural deterioration/calcification in the mid-term. The allograft was transformed into an autograft.

Genetic diseases of the aorta: aortic diseases associated with bicuspid aortic valves

ESC CardioMed ◽  
2018 ◽  
pp. 2613-2615
Author(s):  
Folkert Meijboom ◽  
Gert-Jan Sieswerda
Keyword(s):  
Aortic Wall ◽  
Structural Integrity ◽  
Genetic Diseases ◽  
Basic Research ◽  
Ascending Aorta ◽  
Flow Profile ◽  
Aortic Diseases ◽  
Cystic Medial Necrosis ◽  
Medial Necrosis ◽  
Genetically Determined

There is a genetic cause of bicuspid aortic valve. Dilatation of the aortic root and ascending aorta has long been considered genetically determined too and treated accordingly. In recent years, basic research combined with advanced cardiac imaging has made a strong case for another cause of this dilatation: an altered flow profile in the ascending aorta due to the abnormal geometry of the bicuspid valve, leading to an area of altered wall shear stress, which in turn leads to remodelling of the aortic wall, with apoptosis of vascular smooth muscle cells and disruption of media layer as a result. These histological changes, previously referred to as cystic medial necrosis, form the basis of a loss of structural integrity of the aortic wall, which makes it prone to dilatation, dissection, and rupture.

scholarly journals Hémodissection Pariétale Oblitérante des Vaisseaux Intracrâniens, une Forme Particulière d’Anévrysme Disséquant

1977 ◽  
Vol 4 (1) ◽  
pp. 63-65 ◽  
Author(s):  
F. Gagne ◽  
M. Lemay ◽  
S. Verret
Keyword(s):  
Anterior Cerebral Artery ◽  
Dissecting Aneurysm ◽  
Congenital Defects ◽  
Internal Elastic Lamina ◽  
Medial Necrosis ◽  
The Media ◽  
Young Subjects ◽  
Nosologic Entity ◽  
The Right ◽  
Elastic Lamina

SummaryA six year old girl complained of sudden severe headache, became hemiplegic and unconscious. A right carotid arteriogram revealed an obstruction of the right anterior cerebral artery and many sylvian branches. Death occurred four days later. At autopsy, a recent softening of nearly all the right middle cerebral arterial territory was found. Thrombus filled the sylvian artery and its main branches. Histologic examination of the vessel walls showed a dissecting infiltration of blood between the internal elastic lamina and the media. This particular form of dissecting aneurysm, occurring in young subjects, in the absence of atherosclerosis, high blood pressure and idiopathic medial necrosis, represents a distinct nosologic entity that has been called “Obstructive parietal hemodissection of intracranial vessels.” The pathogenesis of the disease is unknown: trauma has been mentioned, also congenital defects in the elastic lamina or other morphologic abnormalities ofthat lamina.

RISK FACTORS IN THE DEVELOPMENT OF LIVER ABSCESSES AND DIAGNOSTIC STRATEGY IN THEIR SUSPECTED PRESENCE

2016 ◽  
Vol 2 (1) ◽  
pp. 31
Author(s):  
Josiah Iju WILSON ◽  
Vladimir Egorovich MEDVEDEV
Keyword(s):  
Risk Factors ◽  
Clinical Laboratory ◽  
Inflammatory Diseases ◽  
Bacterial Overgrowth ◽  
Diagnostic Strategy ◽  
Hydrogen Breath Test ◽  
Severe Condition ◽  
Liver Ultrasound ◽  
Liver Abscesses ◽  
History Of

Introduction: The main risk aetiological factors of liver abscesses and development of precision liver ultrasound recommendations to detect signs of possible abscess formation were studied.Material and methods: 248 patients of both sexes aged 4-81 years with liver abscesses were analyzed. Medical history, physical examination, clinical laboratory tests, hydrogen breath test with, ultrasound examination, if necessary - computed tomography and fine needle diagnostic biopsy under ultrasound guidance were carried out..Results and discussion: It was established that liver abscesses are aetiologically heterogeneous, in which the largest in the group was pylephlebitic (64.1%), posttraumatic (14.5%), cholangiogenic (12.5%) and contact abscesses (1.2 %). In connection with the effacement or nonspecific clinical picture, often severe condition of the patient, the prevalence of symptoms in some cases of other diseases, liver abscesses may not be promptly diagnosed.Conclusion: The presence of clinical and laboratory signs of suppurate inflammatory processes, risk factors such as the presence of bacterial overgrowth syndrome, inflammatory diseases of the intestines, history of the use of proton pump inhibitors, diseases in association with cholestasis, surgery, history of trauma, abscesses of other locations, it is recommended that precision liver ultrasound should be carried out to detect possible echo signs of liver abscesses.

scholarly journals Influenza Vaccination in Psoriatic Patients—Epidemiology and Patient Perceptions: A German Multicenter Study (Vac-Pso)

Vaccines ◽  
2021 ◽  
Vol 9 (8) ◽  
pp. 843
Author(s):  
Christian Kromer ◽  
Phoebe Wellmann ◽  
Ralf Siemer ◽  
Selina Klein ◽  
Johannes Mohr ◽  
...  
Keyword(s):  
Influenza Vaccination ◽  
Inflammatory Diseases ◽  
Influenza Virus Infection ◽  
Multivariate Regression Analysis ◽  
Cross Sectional Study ◽  
Patient Perceptions ◽  
Cross Sectional ◽  
Vaccination Rates ◽  
Disease Characteristics ◽  
History Of

The risk of developing severe complications from an influenza virus infection is increased in patients with chronic inflammatory diseases such as psoriasis (PsO) and atopic dermatitis (AD). However, low influenza vaccination rates have been reported. The aim of this study was to determine vaccination rates in PsO compared to AD patients and explore patient perceptions of vaccination. A multicenter cross-sectional study was performed in 327 and 98 adult patients with PsO and AD, respectively. Data on vaccination, patient and disease characteristics, comorbidity, and patient perceptions was collected with a questionnaire. Medical records and vaccination certificates were reviewed. A total of 49.8% of PsO and 32.7% of AD patients were vaccinated at some point, while in season 2018/2019, 30.9% and 13.3% received an influenza vaccination, respectively. There were 96.6% and 77.6% of PsO and AD patients who had an indication for influenza vaccination due to age, immunosuppressive therapy, comorbidity, occupation, and/or pregnancy. Multivariate regression analysis revealed higher age (p < 0.001) and a history of bronchitis (p = 0.023) as significant predictors of influenza vaccination in PsO patients. Considering that most patients had an indication for influenza vaccination, the rate of vaccinated patients was inadequately low.

scholarly journals Genetic Background of Congenital Erythrocytosis

Genes ◽  
2021 ◽  
Vol 12 (8) ◽  
pp. 1151
Author(s):  
Mary Frances McMullin
Keyword(s):  
Receptor Gene ◽  
Cell Mass ◽  
Oxygen Affinity ◽  
Young Age ◽  
Erythropoietin Receptor ◽  
Congenital Defects ◽  
History Of ◽  
Genetic Mechanisms ◽  
High Oxygen Affinity ◽  
Hemoglobin Variants

True erythrocytosis is present when the red cell mass is greater than 125% of predicted sex and body mass, which is reflected by elevated hemoglobin and hematocrit. Erythrocytosis can be primary or secondary and congenital or acquired. Congenital defects are often found in those diagnosed at a young age and with a family history of erythrocytosis. Primary congenital defects mainly include mutations in the Erythropoietin receptor gene but SH2B3 has also been implicated. Secondary congenital erythrocytosis can arise through a variety of genetic mechanisms, including mutations in the genes in the oxygen sensing pathway, with high oxygen affinity hemoglobin variants and mutations in other genes such as BPMG, where ultimately the production of erythropoietin is increased, resulting in erythrocytosis. Recently, mutations in PIEZ01 have been associated with erythrocytosis. In many cases, a genetic variant cannot be identified, leaving a group of patients with the label idiopathic erythrocytosis who should be the subject of future investigations. The clinical course in congenital erythrocytosis is hard to evaluate as these are rare cases. However, some of these patients may well present at a young age and with sometimes catastrophic thromboembolic events. There is little evidence to guide the management of congenital erythrocytosis but the use of venesection and low dose aspirin should be considered.

scholarly journals Dill Extract Induces Elastic Fiber Neosynthesis and Functional Improvement in the Ascending Aorta of Aged Mice with Reversal of Age-Dependent Cardiac Hypertrophy and Involvement of Lysyl Oxidase-Like-1

Biomolecules ◽  
2020 ◽  
Vol 10 (2) ◽  
pp. 173 ◽  
Author(s):  
Wassim Fhayli ◽  
Quentin Boëté ◽  
Nadjib Kihal ◽  
Valérie Cenizo ◽  
Pascal Sommer ◽  
...  
Keyword(s):  
Cardiac Hypertrophy ◽  
Lysyl Oxidase ◽  
Elastic Fiber ◽  
Ascending Aorta ◽  
Functional Improvement ◽  
Elastic Fibers ◽  
Aged Mice ◽  
Age Related ◽  
And Function

Elastic fibers (90% elastin, 10% fibrillin-rich microfibrils) are synthesized only in early life and adolescence mainly by the vascular smooth muscle cells through the cross-linking of its soluble precursor, tropoelastin. Elastic fibers endow the large elastic arteries with resilience and elasticity. Normal vascular aging is associated with arterial remodeling and stiffening, especially due to the end of production and degradation of elastic fibers, leading to altered cardiovascular function. Several pharmacological treatments stimulate the production of elastin and elastic fibers. In particular, dill extract (DE) has been demonstrated to stimulate elastin production in vitro in dermal equivalent models and in skin fibroblasts to increase lysyl oxidase–like-1 (LOXL-1) gene expression, an enzyme contributing to tropoelastin crosslinking and elastin formation. Here, we have investigated the effects of a chronic treatment (three months) of aged male mice with DE (5% or 10% v/v, in drinking water) on the structure and function of the ascending aorta. DE treatment, especially at 10%, of aged mice protected pre-existing elastic lamellae, reactivated tropoelastin and LOXL-1 expressions, induced elastic fiber neo-synthesis, and decreased the stiffness of the aging aortic wall, probably explaining the reversal of the age-related cardiac hypertrophy also observed following the treatment. DE could thus be considered as an anti-aging product for the cardiovascular system.

Congenital Defects of the Vomer

2000 ◽  
Vol 109 (5) ◽  
pp. 497-499 ◽  
Author(s):  
Mitsuhiro Mohri ◽  
Mutsuo Amatsu
Keyword(s):  
Infectious Disease ◽  
Drug Abuse ◽  
Nasal Septum ◽  
Past History ◽  
University Hospital ◽  
Ossification Center ◽  
Congenital Defects ◽  
Nasal Surgery ◽  
History Of ◽  
Nasal Trauma

Six cases of congenital defect of the vomer, a rare nasal anomaly, are reported. All 6 patients visited Kobe University Hospital with other complaints, and the anomaly was incidentally detected. In all cases, the nasal septum showed a defect at the posteroinferior portion that appeared to coincide with the location of the vomer. None of the patients had a past history of nasal trauma, nasal surgery, drug abuse, or infectious disease. This anomaly may be attributable to an embryological disorder based on an immature ossification center of the vomer.

Sudden cardiac arrest in 15-year-old girl. Could it be avoided?

2018 ◽  
Vol 1 (46) ◽  
pp. 43-48
Author(s):  
Maria Posadowska ◽  
Maria Miszczak-Knecht ◽  
Alicja Mirecka-Rola ◽  
Katarzyna Bieganowska
Keyword(s):  
Cardiac Arrest ◽  
Hypertrophic Cardiomyopathy ◽  
Cardiac Death ◽  
Sudden Cardiac Arrest ◽  
Postmortem Examination ◽  
Cardiac Care ◽  
Related Family ◽  
Asymptomatic Patients ◽  
History Of ◽  
Genetically Determined

We present a case of a 15-year-old girl after sudden cardiac arrest because of ventricular fibrillation. Hypertrophic cardiomyopathy was diagnosed. Family history of patient was positive – her uncle (mother’s brother) died suddenly at the age of 21, postmortem examination showed hypertrophic cardiomyopathy. The deceased man’s family was not under cardiac care. The presented case proves, that due to genetically determined cardiac diseases such as cardiomyopathies and channelopathies, all related family members should be examined cardiologically. Diagnosis of the disease in asymptomatic patients would allow the implementation of treatment and reduce the risk of a sudden cardiac arrest / sudden cardiac death.

GENETIC ASPECTS OF PEDIATRIC ENDOCRINOLOGY

PEDIATRICS ◽  
1959 ◽  
Vol 24 (2) ◽  
pp. 330-337
Author(s):  
Lytt I. Gardner ◽  
Ira M. Rosenthal ◽  
Richard J. Feinberg
Keyword(s):  
Relative Number ◽  
Round Table ◽  
Well Being ◽  
Congenital Defects ◽  
Inherited Disease ◽  
Pediatric Endocrinology ◽  
Number Of Children ◽  
German Revolution ◽  
Genetically Determined ◽  
Revolution Of 1848

Dr. Gardner: Historically pediatricians have been in the vanguard of the social and scientific forces improving the health and well-being of their patients—the children of this country. Indeed, we have the heavy responsibility of carrying on the proud tradition of our professional great-grandfather, Dr. Abraham Jacobi, who came to these shores in the aftermath of the German Revolution of 1848, and who eventually rose to the presidency of the American Medical Association. As the pioneer in American pediatrics, Jacobi never failed to let his position be known on controversial issues. His intuitive Jeffersonian grasp of the democratic process facilitated his rôle in the early development of pediatrics here. Jacobi's coat has, in a sense, fallen upon our shoulders, and American pediatrics must continually be on the alert to live up to what he would have expected of us. Therefore let me come directly to the problem at hand. As we know, the relative number of children with congenital defects in our hospitals is very much greater than 25 years ago. Recently in our hospital we tabulated the cases over a 5-month period, and found that 30% of the pediatric inpatients were there because of congenital defects. This apparent increase is almost certainly due in large part to the reduction in patients with infectious disease, but the figure of 30% still must remind us that the care of children with congenital defects is a field of major importance in modern pediatrics. How many of these defects are genetically determined is not known for sure, but certainly a considerable part of this group of patients represents inherited disease. As is obvious from the syndromes we will take up in this endocrine round table, nearly every one of these conditions is genetically determined, that is to say, the result of a mutation which has taken place in the human hereditary material.

Sign in / Sign up

Export Citation Format

Share Document