scholarly journals Pathogenesis, Diagnosis, and Clinical Implications of Hereditary Hemochromatosis—The Cardiological Point of View

Diagnostics ◽  
2021 ◽  
Vol 11 (7) ◽  
pp. 1279
Author(s):  
Ludmiła Daniłowicz-Szymanowicz ◽  
Michał Świątczak ◽  
Katarzyna Sikorska ◽  
Rafał R. Starzyński ◽  
Alicja Raczak ◽  
...  

Hereditary hemochromatosis (HH) is a genetic disease leading to excessive iron absorption, its accumulation, and oxidative stress induction causing different organ damage, including the heart. The process of cardiac involvement is slow and lasts for years. Cardiac pathology manifests as an impaired diastolic function and cardiac hypertrophy at first and as dilatative cardiomyopathy and heart failure with time. From the moment of heart failure appearance, the prognosis is poor. Therefore, it is crucial to prevent those lesions by upfront therapy at the preclinical phase of the disease. The most useful diagnostic tool for detecting cardiac involvement is echocardiography. However, during an early phase of the disease, when patients do not present severe abnormalities in serum iron parameters and severe symptoms of other organ involvement, heart damage may be overlooked due to the lack of evident signs of cardiac dysfunction. Considerable advancement in echocardiography, with particular attention to speckle tracking echocardiography, allows detecting discrete myocardial abnormalities and planning strategy for further clinical management before the occurrence of substantial heart damage. The review aims to present the current state of knowledge concerning cardiac involvement in HH. In addition, it could help cardiologists and other physicians in their everyday practice with HH patients.

Chelovek RU ◽  
2020 ◽  
pp. 18-53
Author(s):  
Sergei Avanesov ◽  

Abstract. The article analyzes the autobiography of the famous Russian philosopher, theologian and scientist Pavel Florensky, as well as those of his texts that retain traces of memories. According to Florensky, the personal biography is based on family history and continues in children. He addresses his own biography to his children. Memories based on diary entries are designed as a memory diary, that is, as material for future memories. The past becomes actual in autobiography, turns into a kind of present. The past, from the point of view of its realization in the present, gains meaning and significance. The au-thor is active in relation to his own past, transforming it from a collection of disparate facts into a se-quence of events. A person can only see the true meaning of such events from a great distance. Therefore, the philosopher remembers not so much the circumstances of his life as the inner impressions of the en-counter with reality. The most powerful personality-forming experiences are associated with childhood. Even the moment of birth can decisively affect the character of a person and the range of his interests. The foundations of a person's worldview are laid precisely in childhood. Florensky not only writes mem-oirs about himself, but also tries to analyze the problems of time and memory. A person is immersed in time, but he is able to move into the past through memory and into the future through faith. An autobi-ography can never be written to the end because its author lives on. However, reaching the depths of life, he is able to build his path in such a way that at the end of this path he will unite with the fullness of time, with eternity.


Author(s):  
R. R. Palmer

In 1792, the French Revolution became a thing in itself, an uncontrollable force that might eventually spend itself but which no one could direct or guide. The governments set up in Paris in the following years all faced the problem of holding together against forces more revolutionary than themselves. This chapter distinguishes two such forces for analytical purposes. There was a popular upheaval, an upsurge from below, sans-culottisme, which occurred only in France. Second, there was the “international” revolutionary agitation, which was not international in any strict sense, but only concurrent within the boundaries of various states as then organized. From the French point of view these were the “foreign” revolutionaries or sympathizers. The most radical of the “foreign” revolutionaries were seldom more than advanced political democrats. Repeatedly, however, from 1792 to 1799, these two forces tended to converge into one force in opposition to the French government of the moment.


Upravlenie ◽  
2020 ◽  
Vol 8 (4) ◽  
pp. 116-122
Author(s):  
Sadeghi Elham Mir Mohammad ◽  
Ahmad Vakhshitekh

The article considers and analyses the basic principles and directions of Russian foreign policy activities during the presidency of V.V. Putin from the moment of his assumption of the post of head of state to the current presidential term. The authors determine the basic principles of Russia's foreign policy in the specified period and make the assessment to them. The study uses materials from publications of both Russian and foreign authors, experts in the field of political science, history and international relations, as well as documents regulating the foreign policy activities of the highest state authorities. The paper considers the process of forming the priorities of Russia's foreign policy both from the point of view of accumulated historical experience and continuity of the internal order, and in parallel with the processes of transformation of the entire system of international relations and the world order. The article notes the multi-vector nature of Russia's foreign policy strategy aimed at developing multilateral interstate relations, achieving peace and security in the interstate arena, actively countering modern challenges and threats to interstate security, as well as the formation of a multipolar world. The authors conclude that at present, Russia's foreign policy activity is aimed at strengthening Russia's prestige, supporting economic growth and competitiveness, ensuring security and implementing national interests. Internal political reforms contribute to strengthening the political power of the President of the Russian Federation and increasing the efficiency of foreign policy decision-making.


2012 ◽  
pp. 66-80
Author(s):  
Michał Mrozowicki

Michel Butor, born in 1926, one of the leaders of the French New Novel movement, has written only four novels between 1954 and 1960. The most famous of them is La Modification (Second thoughts), published in 1957. The author of the paper analyzes two other Butor’s novels: L’Emploi du temps (Passing time) – 1956, and Degrés (Degrees) – 1960. The theme of absence is crucial in both of them. In the former, the novel, presented as the diary of Jacques Revel, a young Frenchman spending a year in Bleston (a fictitious English city vaguely similar to Manchester), describes the narrator’s struggle to survive in a double – spatial and temporal – labyrinth. The first of them, formed by Bleston’s streets, squares and parks, is symbolized by the City plan. During his one year sojourn in the city, using its plan, Revel learns patiently how to move in its different districts, and in its strange labyrinth – strange because devoid any centre – that at the end stops annoying him. The other, the temporal one, symbolized by the diary itself, the labyrinth of the human memory, discovered by the narrator rather lately, somewhere in the middle of the year passed in Bleston, becomes, by contrast, more and more dense and complex, which is reflected by an increasinly complex narration used to describe the past. However, at the moment Revel is leaving the city, he is still unable to recall and to describe the events of the 29th of February 1952. This gap, this absence, symbolizes his defeat as the narrator, and, in the same time, the human memory’s limits. In Degrees temporal and spatial structures are also very important. This time round, however, the problems of the narration itself, become predominant. Considered from this point of view, the novel announces Gerard Genette’s work Narrative Discourse and his theoretical discussion of two narratological categories: narrative voice and narrative mode. Having transgressed his narrative competences, Pierre Vernier, the narrator of the first and the second parts of the novel, who, taking as a starting point, a complete account of one hour at school, tries to describe the whole world and various aspects of the human civilization for the benefit of his nephew, Pierre Eller, must fail and disappear, as the narrator, from the third part, which is narrated by another narrator, less audacious and more credible.


2020 ◽  
Vol 15 (1-3) ◽  
pp. 44-59
Author(s):  
Lidia Peneva

Crimes against marriage and family are a particular group of social relation­ships that the law has defended properly in view of the high public significance and value they enjoy. At the moment they are regulated in Chapter VI, Section I, of the specific part of the Penal Code the Repub­lic of Bulgaria. The subject matter of this Statement will, however, be the legisla­tive provisions concerning these criminal­ized acts in retrospect. The purpose of the study is to show by historical method and through the comparatively legal method the development of these criminal groups during the periods of various criminal laws in Bulgaria. This will also provide a basis for reflection on possible de lege ferenda proposals. This report from a structural point of view will be divided into three distinct points, marking each of the penal laws in the Republic of Bulgaria, which were in force before 1968.


2007 ◽  
Vol 555 ◽  
pp. 177-182 ◽  
Author(s):  
Snezana Pašalić ◽  
P.B. Jovanić ◽  
B. Bugarski

There are many developed strategies for evaluating emulsion stability, aimed at determining the life circle of emulsions. Most of them are based on rheological properties of emulsions. There are, however, very few based on direct emulsion observations. In this paper we present a developed method for the emulsion stability evaluation by direct observation of optical emulsion properties. We propose the fractal dimension approach as a stability quantification measure. The method is based on the measure of emulsion transmittance properties, which are directly dependent on the emulsion stability at the moment of measurement. The oil in water emulsion was used as a test emulsion. The system is classified as stable emulsion and our intention was to find the moment when it starts to break. Emulsion transmittance properties were measure applying a system for acquisition of visual information, which is based on a CCD camera and a fast PC configuration equipped with the capturing software. The acquired sets of visual information were analyzed by the OZARIA software package. The fractal dimensions were determined by the box counting method. For these experiments, 100 boxes of different sizes were used. Experimental emulsions were measured after 7, 14, 21 and 28 days from the moment of creation. A slight increase in fractal dimensions was observed, which indicates that the emulsions are still in the stable region, or from the fractal point of view emulsion are still regular and no significant irregularities were observed. From the first experiments the applied methodology proved to be sensitive enough to be used for emulsions stability evaluation.


2001 ◽  
Vol 11 (4) ◽  
pp. 311-321
Author(s):  
DN Carmichael ◽  
Michael Lye

Heart failure has been defined in many ways and definitions change over time. The multiplicity of definitions reflect the paucity of our understanding of the primary underlying physiology of heart failure and the many diseases for which heart failure is the common end-point. Fundamentally, heart failure represents a failure of the heart to meet the body’s requirement for blood supply for whatever reason. It is thus a clinical syndrome with characteristic features – not a single disease in its own right. The syndrome includes symptoms and signs of organ underperfusion, fluid retention and neuroendocrine activation. The syndrome arises from a range of possible causes of which ischaemic heart disease is the commonest. From the point of view of a clinician, the underlying pathology will determine treatment options and prognosis. The extensive range of possible aetiologies present a diagnostic challenge both to correctly identify the syndrome amongst all other causes of dyspnoea and to identify the aetiology, allowing optimization of treatment.


2020 ◽  
Vol 22 (4) ◽  
pp. 183-191
Author(s):  
O. T. Kotsoeva ◽  
A. V. Koltsov ◽  
V. V. Tyrenko ◽  
A. A. Ialovets

This review discusses a number of aspects of surgical methods for treating severe chronic heart failure: resynchronizing therapy, mechanical circulatory support systems, and heart transplantation. Surgical methods for the treatment of heart failure are a rapidly developing field of modern cardiology and cardiac surgery. The main surgical method of treatment was and remains orthotopic transplantation of a donor heart. The advent of implantable systems has affected the problem of heart transplantation. Over the past decade, the use of mechanical circulatory support systems has grown significantly. At the moment, there are 3 main directions: creating devices for auxiliary blood circulation, various modes and methods of electrical stimulation of the myocardium, creating devices that mechanically remodel the heart chambers (left ventricle). All of these directions to some extent (depending on the evidence base) have found their place in modern recommendations for the treatment of chronic heart failure. The use of mechanical left ventricular remodeling shows good results in patients suffering from symptomatic heart failure, which leads to a significant and persistent decrease in the volume of the left ventricle and improvement of its function, symptoms and quality of life. Despite the fact that at the moment the geography and prevalence of their use is small, the number of implanted devices will only grow. Thus, given the need for frequent hospitalizations and high treatment costs, it is necessary to improve modern methods of surgical treatment of severe and terminal heart failure, make them more accessible, which will affect the duration and quality of life of these patients.


2021 ◽  
pp. 107815522110351
Author(s):  
Atakan Tekinalp ◽  
Taha U Kars ◽  
Hatice Z Dikici ◽  
Pınar D Yılmaz ◽  
Sinan Demircioğlu ◽  
...  

Introduction Cardiac involvement in diffuse large B-cell lymphoma is a rare entity in non-Hodgkin lymphomas. Symptoms are usually related to heart failure. Patients who are severely symptomatic due to cardiac mass could be considered treatment as soon as possible. In this report, we present a patient diagnosed with diffuse large B-cell lymphoma with cardiac involvement. Case Report A 61-year-old female patient was admitted to our unit with gastric biopsy diffuse large B-cell lymphoma. Computerized tomography of the chest and positron emission tomography/computed tomography demonstrated a neoplastic mass in the intra-atrial septum extended to inferior vena cava (5 × 4 cm in size and standardized uptake value maximum 24.6). She was in stage III and in the high-risk group. Because of pronounced heart failure findings associated with the mass-specific chemotherapy was planned early. Management & Outcome Although a fraction of ejection was 60% by echocardiography before the treatment, she had a cardiac risk for doxorubicin due to being over 60 years old and hypertension. Complete remission was achieved after three cycles of rituximab–cyclophosphamide–doxorubicin–vincristine and methylprednisolone protocol including doxorubicin. Treatment was completed with six cycles and she was followed up for three months. Discussion Because of the cardiotoxicity of doxorubicin-based protocols, patients should be evaluated according to cardiac functions before and during the chemotherapy.


Author(s):  
Palmira Cupo ◽  
Mauro Jurca ◽  
Marisa M. Azevedo-Marques ◽  
João Samuel M. Oliveira ◽  
Sylvia E. Hering

Scorpion stings in Brazil are important not only because of their incidence but also for their potential ability to induce severe, and often fatal, clinical situations, especially among children. In this report we present the clinical and laboratory data of 4 patients victims of scorpion stings by T. serrulatus, who developed heart failure and pulmonary edema, with 3 of them dying within 24 hours of the sting. Anatomopathologic study of these patients revealed diffuse areas of myocardiocytolysis in addition to pulmonary edema. The surviving child presented enzymatic, electrocardiographic and echocardiographic changes compatible with severe cardiac involvement, which were reversed within 5 days. These findings reinforce the need for continuous monitoring of patients with severe scorpion envenoming during the hours immediately following the sting.


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