Basic characteristics of most common cardiac heart defects

Cilj rada je prikazati osnovna obilježja prirođenih srčanih grješaka. Rad je pregledni te mu je cilj prikazati dosadašnje spoznaje koje se odnose na etiologiju bolesti, prikazati suvremene patogenetske podjele prirođenih srčanih grješaka, opisati kliničku sliku najčešćih prirođenih srčanih grješaka te navesti smjernice u liječenju pojedinih skupina bolesnika. Od osobitog je značaja naglasiti važnost timskog rada u zbrinjavanju ove skupine bolesnika. Jednako tako treba naglasiti i važnost kontinuiranog praćenja djece koja sve više prelaze u odraslu dob te formiraju skupinu odraslih bolesnika koja poprima sve veći javnozdravstveni značaj. Prirođene srčane grješke su etiološki i fenotipski raznolika skupina bolesti, koja se javlja u oko 0,7 do 1% živorođene djece. S obzirom na veliki broj različitih prirođenih srčanih grješaka, postoje i brojne podjele istih, prema anatomskim, hemodinamskim ili pak kliničkim kriterijima. Prema hemodinamskim karakteristikama dijelimo ih na dvije velike skupine: 1. srčane grješke bez patološkog spoja između sistemskog i pulmonalnog optoka (pulmonalna stenoza, koarktacija aorte, aortna stenoza) te 2. srčane grješke s patološkim spojem između sistemskog i pulmonalnog optoka: grješke s arterijsko-venskim pretokom (defekt ventrikularnogseptuma, defekt atrijskog septuma, otvoreni arterijski duktus Botalli) i grješke s vensko-arterijskim pretokom ili s dvosmjernim pretokom (tetralogija Fallot, trikuspidalna atrezija, transpozicija velikih krvnih žila, arterijski trunkus). Nakon dijagnostičke obrade i postavljanja egzaktne dijagnoze, počinje se s liječenjem, koje je uglavnom kirurško. Naravno, daljnji postupak ovisi o stanju djeteta, dogovoru s roditeljima te o ostalim trenutnim mogućnostima. Revoluciju u liječenju kompleksnih srčanih mana donijeli su kardiokirurški zahvati kojima je omogućeno premoštenje desne, odnosno lijeve strane srca. Na taj je način omogućeno liječenje i sindroma hipoplastičnog lijevog srca. Osim svega navedenoga, pacijentima s prirođenim srčanim grješkama potrebno je kontinuirano praćenje, pravovremeno prepoznavanje mogućih komplikacija i zbrinjavanje istih. Uz to je također izuzetno važna liječnička podrška i dobra komunikacija kako s pacijentima tako i s njihovim obiteljima.The aim of the diploma thesis is to show the basic features of congenital heart disease. The thesis is a review and aims to show the existing knowledge concerning the etiology of the disease, shows the modern pathogenetic classification of the congenital heart disease, describes the clinical picture of the most common congenital heart diseases and states guidelines in the treatment of certain patient groups. It is crucial to emphasize the importance of the teamwork in the care of this group of patients. The continuous monitoring of children moving towards an adulthood and form a group of adult patients, that assumes increasing public health significance, should also be highlighted. Congenital heart diseases are etiologic and phenotypically heterogeneous group of diseases, which occurs in about 0.7 to 1% of live births. Due to the large number of different congenital heart malformations, there are numerous divisions and classifications, according to anatomic, hemodynamic or clinical criteria. According to the hemodynamic characteristics, congenital heart diseases are divided into two major groups: 1. Cardiac malformation without pathological connection between the systemic and pulmonary circulation (pulmonary stenosis, coarctation of the aorta, aortic stenosis); and 2. Cardiac malformation with abnormal connection between the systemic and pulmonary circulation: malformation with arterial-venous flow (ventricular septal defect, atrial septal defect, persistent ductus arteriosus Botalli) and malformation with venous-arterial flow or two-way flow (tetralogy of Fallot, tricuspid atresia, transposition of the great vessels). Diagnostic work and precise diagnosis are followed by the treatment, which is mainly surgical. Of course, the further procedure depends on the child's condition, consultation with parents and another possible options. The surgical interventions which enabled bridging the right and left sides of the heart brought a revolution in the treatment of complex heart malformation. In this way, the treatment of the hypoplastic left heart syndrome is possible. In addition to the above, patients with congenital heart disease have to be continuously monitored. There is also a huge importance of timely recognition and treatment of possible complications as well as a good communication with patients and with their families

Basic characteristics of most common cardiac heart defects

Cilj rada je prikazati osnovna obilježja prirođenih srčanih grješaka. Rad je pregledni te mu je cilj prikazati dosadašnje spoznaje koje se odnose na etiologiju bolesti, prikazati suvremene patogenetske podjele prirođenih srčanih grješaka, opisati kliničku sliku najčešćih prirođenih srčanih grješaka te navesti smjernice u liječenju pojedinih skupina bolesnika. Od osobitog je značaja naglasiti važnost timskog rada u zbrinjavanju ove skupine bolesnika. Jednako tako treba naglasiti i važnost kontinuiranog praćenja djece koja sve više prelaze u odraslu dob te formiraju skupinu odraslih bolesnika koja poprima sve veći javnozdravstveni značaj. Prirođene srčane grješke su etiološki i fenotipski raznolika skupina bolesti, koja se javlja u oko 0,7 do 1% živorođene djece. S obzirom na veliki broj različitih prirođenih srčanih grješaka, postoje i brojne podjele istih, prema anatomskim, hemodinamskim ili pak kliničkim kriterijima. Prema hemodinamskim karakteristikama dijelimo ih na dvije velike skupine: 1. srčane grješke bez patološkog spoja između sistemskog i pulmonalnog optoka (pulmonalna stenoza, koarktacija aorte, aortna stenoza) te 2. srčane grješke s patološkim spojem između sistemskog i pulmonalnog optoka: grješke s arterijsko-venskim pretokom (defekt ventrikularnogseptuma, defekt atrijskog septuma, otvoreni arterijski duktus Botalli) i grješke s vensko-arterijskim pretokom ili s dvosmjernim pretokom (tetralogija Fallot, trikuspidalna atrezija, transpozicija velikih krvnih žila, arterijski trunkus). Nakon dijagnostičke obrade i postavljanja egzaktne dijagnoze, počinje se s liječenjem, koje je uglavnom kirurško. Naravno, daljnji postupak ovisi o stanju djeteta, dogovoru s roditeljima te o ostalim trenutnim mogućnostima. Revoluciju u liječenju kompleksnih srčanih mana donijeli su kardiokirurški zahvati kojima je omogućeno premoštenje desne, odnosno lijeve strane srca. Na taj je način omogućeno liječenje i sindroma hipoplastičnog lijevog srca. Osim svega navedenoga, pacijentima s prirođenim srčanim grješkama potrebno je kontinuirano praćenje, pravovremeno prepoznavanje mogućih komplikacija i zbrinjavanje istih. Uz to je također izuzetno važna liječnička podrška i dobra komunikacija kako s pacijentima tako i s njihovim obiteljima.The aim of the diploma thesis is to show the basic features of congenital heart disease. The thesis is a review and aims to show the existing knowledge concerning the etiology of the disease, shows the modern pathogenetic classification of the congenital heart disease, describes the clinical picture of the most common congenital heart diseases and states guidelines in the treatment of certain patient groups. It is crucial to emphasize the importance of the teamwork in the care of this group of patients. The continuous monitoring of children moving towards an adulthood and form a group of adult patients, that assumes increasing public health significance, should also be highlighted. Congenital heart diseases are etiologic and phenotypically heterogeneous group of diseases, which occurs in about 0.7 to 1% of live births. Due to the large number of different congenital heart malformations, there are numerous divisions and classifications, according to anatomic, hemodynamic or clinical criteria. According to the hemodynamic characteristics, congenital heart diseases are divided into two major groups: 1. Cardiac malformation without pathological connection between the systemic and pulmonary circulation (pulmonary stenosis, coarctation of the aorta, aortic stenosis); and 2. Cardiac malformation with abnormal connection between the systemic and pulmonary circulation: malformation with arterial-venous flow (ventricular septal defect, atrial septal defect, persistent ductus arteriosus Botalli) and malformation with venous-arterial flow or two-way flow (tetralogy of Fallot, tricuspid atresia, transposition of the great vessels). Diagnostic work and precise diagnosis are followed by the treatment, which is mainly surgical. Of course, the further procedure depends on the child's condition, consultation with parents and another possible options. The surgical interventions which enabled bridging the right and left sides of the heart brought a revolution in the treatment of complex heart malformation. In this way, the treatment of the hypoplastic left heart syndrome is possible. In addition to the above, patients with congenital heart disease have to be continuously monitored. There is also a huge importance of timely recognition and treatment of possible complications as well as a good communication with patients and with their families

THE ACCEPTANCE OF COSMETIC SURGERY SCALE (ACSS) AND ITS CORRELATIONS WITH PSYCHOLOGICAL CHARACTERISTICS AMONG THE CROATIAN POPULATION

Background: This study aimed to examine the results of Acceptance of Cosmetic Surgery Scale among the Croatian population and its correlations with other scales and demographic data. Subject and methods: The sample consisted of 420 people who voluntarily filled out an online questionnaire. They completed demographic questions and four scales: Acceptance of Cosmetic Surgery Scale (ACSS), Rosenberg Self-Esteem Scale (RSES), Satisfaction With Life Scale (SWLS), and Body Appreciation Scale-2 (BAS-2). Results: ACSS scale showed five statistically significant differences between genders and a higher overall score in women, but no significant differences were recorded in three ACSS subscales and the overall ACSS score. In contrast to men, women respondents recorded a significant negative correlation between ACSS subscales score (Social, Consider) and BAS-2 and Satisfaction with life score, while a positive correlation was recorded with BMI. Conclusion: The ACSS score among the Croatian population was higher than the results among the Italian and Serbian population, and similar to the original American study, which tells us that the Croatian population accepts and considers cosmetic surgery a lot. Furthermore, our results are important for practitioners and patients because they revealed correlations between ACSS scores and the self-thinking scale

THE ACCEPTANCE OF COSMETIC SURGERY SCALE (ACSS) AND ITS CORRELATIONS WITH PSYCHOLOGICAL CHARACTERISTICS AMONG THE CROATIAN POPULATION

Background: This study aimed to examine the results of Acceptance of Cosmetic Surgery Scale among the Croatian population and its correlations with other scales and demographic data. Subject and methods: The sample consisted of 420 people who voluntarily filled out an online questionnaire. They completed demographic questions and four scales: Acceptance of Cosmetic Surgery Scale (ACSS), Rosenberg Self-Esteem Scale (RSES), Satisfaction With Life Scale (SWLS), and Body Appreciation Scale-2 (BAS-2). Results: ACSS scale showed five statistically significant differences between genders and a higher overall score in women, but no significant differences were recorded in three ACSS subscales and the overall ACSS score. In contrast to men, women respondents recorded a significant negative correlation between ACSS subscales score (Social, Consider) and BAS-2 and Satisfaction with life score, while a positive correlation was recorded with BMI. Conclusion: The ACSS score among the Croatian population was higher than the results among the Italian and Serbian population, and similar to the original American study, which tells us that the Croatian population accepts and considers cosmetic surgery a lot. Furthermore, our results are important for practitioners and patients because they revealed correlations between ACSS scores and the self-thinking scale

Long non-coding RNA PCAT19 safeguards DNA in quiescent endothelial cells by preventing uncontrolled phosphorylation of replication protein A2

In healthy vessels, endothelial cells maintain a stable, differentiated, and growth-arrested phenotype for years. Upon injury, a rapid phenotypic switch facilitates proliferation to restore tissue perfusion. Here we report the identification of the endothelial cell-enriched long non-coding RNA (lncRNA) PCAT19, which contributes to the proliferative switch and acts as a safeguard for the endothelial genome. PCAT19 is enriched in confluent, quiescent endothelial cells and binds to the full replication protein A (RPA) complex in a DNA damage- and cell-cycle-related manner. Our results suggest that PCAT19 limits the phosphorylation of RPA2, primarily on the serine 33 (S33) residue, and thereby facilitates an appropriate DNA damage response while slowing cell cycle progression. Reduction in PCAT19 levels in response to either loss of cell contacts or knockdown promotes endothelial proliferation and angiogenesis. Collectively, PCAT19 acts as a dynamic guardian of the endothelial genome and facilitates rapid switching from quiescence to proliferation

Basic characteristics of most common cardiac heart defects

Cilj rada je prikazati osnovna obilježja prirođenih srčanih grješaka. Rad je pregledni te mu je cilj prikazati dosadašnje spoznaje koje se odnose na etiologiju bolesti, prikazati suvremene patogenetske podjele prirođenih srčanih grješaka, opisati kliničku sliku najčešćih prirođenih srčanih grješaka te navesti smjernice u liječenju pojedinih skupina bolesnika. Od osobitog je značaja naglasiti važnost timskog rada u zbrinjavanju ove skupine bolesnika. Jednako tako treba naglasiti i važnost kontinuiranog praćenja djece koja sve više prelaze u odraslu dob te formiraju skupinu odraslih bolesnika koja poprima sve veći javnozdravstveni značaj. Prirođene srčane grješke su etiološki i fenotipski raznolika skupina bolesti, koja se javlja u oko 0,7 do 1% živorođene djece. S obzirom na veliki broj različitih prirođenih srčanih grješaka, postoje i brojne podjele istih, prema anatomskim, hemodinamskim ili pak kliničkim kriterijima. Prema hemodinamskim karakteristikama dijelimo ih na dvije velike skupine: 1. srčane grješke bez patološkog spoja između sistemskog i pulmonalnog optoka (pulmonalna stenoza, koarktacija aorte, aortna stenoza) te 2. srčane grješke s patološkim spojem između sistemskog i pulmonalnog optoka: grješke s arterijsko-venskim pretokom (defekt ventrikularnogseptuma, defekt atrijskog septuma, otvoreni arterijski duktus Botalli) i grješke s vensko-arterijskim pretokom ili s dvosmjernim pretokom (tetralogija Fallot, trikuspidalna atrezija, transpozicija velikih krvnih žila, arterijski trunkus). Nakon dijagnostičke obrade i postavljanja egzaktne dijagnoze, počinje se s liječenjem, koje je uglavnom kirurško. Naravno, daljnji postupak ovisi o stanju djeteta, dogovoru s roditeljima te o ostalim trenutnim mogućnostima. Revoluciju u liječenju kompleksnih srčanih mana donijeli su kardiokirurški zahvati kojima je omogućeno premoštenje desne, odnosno lijeve strane srca. Na taj je način omogućeno liječenje i sindroma hipoplastičnog lijevog srca. Osim svega navedenoga, pacijentima s prirođenim srčanim grješkama potrebno je kontinuirano praćenje, pravovremeno prepoznavanje mogućih komplikacija i zbrinjavanje istih. Uz to je također izuzetno važna liječnička podrška i dobra komunikacija kako s pacijentima tako i s njihovim obiteljima.The aim of the diploma thesis is to show the basic features of congenital heart disease. The thesis is a review and aims to show the existing knowledge concerning the etiology of the disease, shows the modern pathogenetic classification of the congenital heart disease, describes the clinical picture of the most common congenital heart diseases and states guidelines in the treatment of certain patient groups. It is crucial to emphasize the importance of the teamwork in the care of this group of patients. The continuous monitoring of children moving towards an adulthood and form a group of adult patients, that assumes increasing public health significance, should also be highlighted. Congenital heart diseases are etiologic and phenotypically heterogeneous group of diseases, which occurs in about 0.7 to 1% of live births. Due to the large number of different congenital heart malformations, there are numerous divisions and classifications, according to anatomic, hemodynamic or clinical criteria. According to the hemodynamic characteristics, congenital heart diseases are divided into two major groups: 1. Cardiac malformation without pathological connection between the systemic and pulmonary circulation (pulmonary stenosis, coarctation of the aorta, aortic stenosis); and 2. Cardiac malformation with abnormal connection between the systemic and pulmonary circulation: malformation with arterial-venous flow (ventricular septal defect, atrial septal defect, persistent ductus arteriosus Botalli) and malformation with venous-arterial flow or two-way flow (tetralogy of Fallot, tricuspid atresia, transposition of the great vessels). Diagnostic work and precise diagnosis are followed by the treatment, which is mainly surgical. Of course, the further procedure depends on the child's condition, consultation with parents and another possible options. The surgical interventions which enabled bridging the right and left sides of the heart brought a revolution in the treatment of complex heart malformation. In this way, the treatment of the hypoplastic left heart syndrome is possible. In addition to the above, patients with congenital heart disease have to be continuously monitored. There is also a huge importance of timely recognition and treatment of possible complications as well as a good communication with patients and with their families

USP28: oncogene or tumor suppressor? a unifying paradigm for squamous cell carcinoma

Squamous cell carcinomas are therapeutically challenging tumor entities. Low response rates to radiotherapy and chemotherapy are commonly observed in squamous patients and, accordingly, the mortality rate is relatively high compared to other tumor entities. Recently, targeting USP28 has been emerged as a potential alternative to improve the therapeutic response and clinical outcomes of squamous patients. USP28 is a catalytically active deubiquitinase that governs a plethora of biological processes, including cellular proliferation, DNA damage repair, apoptosis and oncogenesis. In squamous cell carcinoma, USP28 is strongly expressed and stabilizes the essential squamous transcription factor ΔNp63, together with important oncogenic factors, such as NOTCH1, c-MYC and c-JUN. It is presumed that USP28 is an oncoprotein; however, recent data suggest that the deubiquitinase also has an antineoplastic effect regulating important tumor suppressor proteins, such as p53 and CHK2. In this review, we discuss: (1) The emerging role of USP28 in cancer. (2) The complexity and mutational landscape of squamous tumors. (3) The genetic alterations and cellular pathways that determine the function of USP28 in squamous cancer. (4) The development and current state of novel USP28 inhibitors

Macrolactonolides: a novel class of anti-inflammatory compounds

A new concept in design of safe glucocorticoid therapy was introduced by conjugating potent glucocorticoid steroids with macrolides (macrolactonolides). These compounds were synthesized from various steroid 17β-carboxylic acids and 9a-N-(3-aminoalkyl) derivatives of 9-deokso-9a-aza-9a-homoeritromicin A and 3-descladinosyl-9-deokso-9a-aza-9a-homoeritromicin A using stable alkyl chain. Combining property of macrolides to preferentially accumulate in immune cells, especially in phagocyte cells, with anti-inflammatory activity of classic steroids, we designed molecules which showed good anti-inflammatory activity in ovalbumin (OVA) induced asthma in rats. The synthesis, in vitro and in vivo anti-inflammatory activity of this novel class of compounds are described

Long non-coding RNA PCAT19 safeguards DNA in quiescent endothelial cells by preventing uncontrolled phosphorylation of RPA2

In healthy vessels, endothelial cells maintain a stable, differentiated, and growth-arrested phenotype for years. Upon injury, a rapid phenotypic switch facilitates proliferation to restore tissue perfusion. Here we report the identification of the endothelial cell-enriched long non-coding RNA (lncRNA) PCAT19, which contributes to the proliferative switch and acts as a safeguard for the endothelial genome. PCAT19 is enriched in confluent, quiescent endothelial cells and binds to the full replication protein A (RPA) complex in a DNA damage- and cell-cycle-related manner. Our results suggest that PCAT19 limits the phosphorylation of RPA2, primarily on the serine 33 (S33) residue, and thereby facilitates an appropriate DNA damage response while slowing cell cycle progression. Reduction in PCAT19 levels in response to either loss of cell contacts or knockdown promotes endothelial proliferation and angiogenesis. Collectively, PCAT19 acts as a dynamic guardian of the endothelial genome and facilitates rapid switching from quiescence to proliferation