Treatment Options for Pediatric Psoriasis

Psoriasis is a multifactorial inflammatory papulosquamous disease affecting 0.5% to 2% of paediatric population. Paediatric psoriasis, presenting similarly to adult psoriasis, significantly reduces patients’ quality of life, often requiring individualized treatment approach for each patient. Combination and rotational therapy are helpful in reducing toxicity and maximizing efficacy. Patients with mild and limited disease respond well to topical treatment with steroids or vitamin D analogues, unlike moderate and severe psoriasis where sufficient remission is rarely achieved. Therefore phototherapy, systemic immunomodulators, or biologic agents are next line of treatment to be considered. There is a limited data available on the use and long-term safety of biologics in the paediatric population. Biologic agents must be administered by experienced dermatologists, only in patients with moderate-to-severe plaque psoriasis, intolerant or refractory to other systemic conventional disease-modifying treatment or phototherapy, or if those agents are contraindicated. Psoriasis is a multifactorial inflammatory papulosquamous disease affecting 0.5% to 2% of paediatric population. Paediatric psoriasis, presenting similarly to adult psoriasis, significantly reduces patients’ quality of life, often requiring individualized treatment approach for each patient. Combination and rotational therapy are helpful in reducing toxicity and maximizing efficacy. Patients with mild and limited disease respond well to topical treatment with steroids or vitamin D analogues, unlike moderate and severe psoriasis where sufficient remission is rarely achieved. Therefore phototherapy, systemic immunomodulators, or biologic agents are next line of treatment to be considered. There is a limited data available on the use and long-term safety of biologics in the paediatric population. Biologic agents must be administered by experienced dermatologists, only in patients with moderate-to-severe plaque psoriasis, intolerant or refractory to other systemic conventional disease-modifying treatment or phototherapy, or if those agents are contraindicated. </div

Kearns-Sayre syndrome – rare cause of growth hormone deficiency

Kearns – Sayre syndrome (KSS) is a rare mitochondriopathy. Among chronic progressive external ophthalmoplegia, pigmentary retinal degeneration and ataxia that are present in most patients before age of 20, majority of patients also have various endocrine deficiencies

McCune - Albright syndrome

McCune-Albrightov sindrom (MAS) je rijedak genetski poremećaj kojeg, u većini slučajeva, odlikuje somatska missense mutacija heterotrimernog G proteina kodiranog na GNAS lokusu. Navedena mutacija u 95% slučajeva dovodi do Arg201Cys ili Arg201His supstitucije, koja pak uzrokuje abnormalnu aktivnost Gαs transkripcijskog faktora i povećanu produkciju cAMP-a s posljedičnom hiperaktivacijom brojnih tkiva i organa. Sindrom se nasljeđuje mozaično, a do sada nije opisana vertikalna transmisija. U kliničkoj slici opisuje se klasična trijada simptoma, uključujući cafe-au-lait hiperpigmentacije kože, fibroznu displaziju kostiju i periferni preuranjeni pubertet. Bolest može pogađati i druge organe poput hipofize, štitnjače, nadbubrežne žlijezde, probavne organe i dr. S obzirom na mozaičan način nasljeđivanja, mutirane stanice raspoređuju se po organizmu u različitim omjerima, što konačno dovodi do varijabilne težine kliničke slike u svakog oboljelog- od asimptomatskih slučajeva do slučajeva nespojivih sa životom. Dijagnoza se postavlja klinički, a popis diferencijalnih dijagnoza je širok. Bolesnike se najčešće dijagnosticira u ranoj dječjoj dobi, češće u ženskog spola. Ne postoji mogućnost liječenja uzročnog stanja, već se provodi simptomatsko liječenje s farmakološkim i kirurškim pristupom. Praćenje i terapijski pristup u ovih bolesnika iziskuje multidisciplinarni tim liječnika raznih specijalnosti.McCune-Albright syndrome (MAS) is a rare genetic disorder most commonly caused by a somatic missense mutation of a heterotrimeric G protein encoded at the GNAS locus. This mutation leads to Arg201Cys or Arg201His substitution in 95% of the cases, which causes abnormal Gαs transcription factor activity and increased cAMP production with consequent hyperactivation of numerous tissues and organs. The clinical expression describes a classic triad of symptoms, including cafe-au-lait hyperpigmentation of the skin, fibrous bone dysplasia, and peripheral premature puberty. The disease can affect other organs such as the pituitary gland, thyroid gland, adrenals, pancreas, etc. Due to the mosaic nature of the condition, mutated cells distribute throughout the body in different proportions, which leads to variable severity of the clinical presentations, from asymptomatic to severe. The diagnosis is made clinically, and the list of differential diagnoses is extensive. Patients are most often diagnosed in early childhood, more often in females. There is no definitive cure, but symptomatic treatment is performed with a pharmacological and surgical approach. Monitoring and therapeutic approach in these patients requires a multidisciplinary team of physicians of various subspecialties

Comparative analysis of the most famous accounting frauds in the world

Računovodstvene prijevare su vrlo aktualna tema kako u Hrvatskoj tako i u svijetu. Danas čovječanstvo svjedoči velikom broju i raznim oblicima računovodstvenih prijevara, a da toga u većini nisu niti svjesni. Glavni cilj ovog rada je unaprijediti znanje o načinima i signalima računovodstvenih prijevara. Financijske prijevare i razni oblici manipulacije u računovodstvu pojavljuju se vrlo često i kao takve veliki su problem za poslovanja poduzeća, ali i države. Forenzično računovodstvo i poslovna forenzika predstavljaju područje koje želi djelovati na prijevare. U velikom dijelu slučajeva, počinitelji prijevare žele prikazati što bolju sliku poslovanja poduzeća, tj. prikazivanje laže dobiti ili skrivanje velikih gubitaka poslovanja. U Republici Hrvatskoj u borbi protiv računovodstvenih prijevara sudjeluju nadležne institucije USKOK (Ured za suzbijanje korupcije i organiziranog kriminaliteta) i DORH (Državno odvjetništvo Republike Hrvatske). Statistički podaci prikazuju kako se sve više javljaju prijevara s navedenom problematikom pa im se iz tih razloga treba posvetiti veća pažnja. Rizik od računovodstvene prijevare uvijek je veći kada postoji pritisak na poduzeća da ispune određene ciljeve. Kad se poduzeće nalazi u gospodarskom padu, uvijek postoji povećani rizik od prijevare. Kada se osoba upusti u računovodstvenu prijevaru, ona namjerno manipulira računovodstvenim evidencijama kako bi financijska slika poduzeća izgledala boljom nego što zapravo jest. Kada se netko proglasi krivim za počinjenje računovodstvene prijevare, podliježe kaznenom gonjenju. U nekim situacijama menadžment može doći u iskušenje da počini direktnu prijevaru. Budući da se gotovina ne prikuplja kada se prodaja vrši „na račun“, korumpirana uprava može zabilježiti lažnu dodatnu prodaju jednostavnim stvaranjem fiktivnih kupaca i/ili bilježenjem fiktivne prodaje. Predmet diplomskog rada je komparativna analiza najpoznatijih računovodstvenih prijevara u svijetu.Accounting fraud is a very current topic both in Croatia and in the world. Today, humanity witnesses a large number and various forms of accounting fraud, without most of them being aware of it. The main goal of this paper is to advance knowledge about the ways and signals of accounting fraud. Financial frauds and various forms of manipulation in accounting appear very often and as such are a big problem for the business of companies, as well as the state. Forensic accounting and business forensics represent an area that wants to act on fraud. In most cases, fraud perpetrators want to present the best possible image of the company's operations, i.e. showing false profits or hiding large business losses. In the Republic of Croatia, the competent institutions USKOK (Office for Suppression of Corruption and Organized Crime) and DORH (State Attorney's Office of the Republic of Croatia) participate in the fight against accounting fraud. Statistical data show that more and more frauds are occurring with the above-mentioned problem, so for these reasons, more attention should be paid to them. The risk of accounting fraud is always higher when there is pressure on companies to meet certain targets. When a company is in an economic downturn, there is always an increased risk of fraud. When a person engages in accounting fraud, they intentionally manipulate accounting records to make a company's financial picture look better than it actually is. When someone is found guilty of committing accounting fraud, they are subject to criminal prosecution. In some situations, management may be tempted to commit outright fraud. Since cash is not collected when sales are made „on account, a corrupt administration can record fraudulent additional sales simply by creating fictitious customers and/or recording fictitious sales. The subject of the thesis is a comparative analysis of the most famous accounting frauds in the world

McCune - Albright syndrome

McCune-Albrightov sindrom (MAS) je rijedak genetski poremećaj kojeg, u većini slučajeva, odlikuje somatska missense mutacija heterotrimernog G proteina kodiranog na GNAS lokusu. Navedena mutacija u 95% slučajeva dovodi do Arg201Cys ili Arg201His supstitucije, koja pak uzrokuje abnormalnu aktivnost Gαs transkripcijskog faktora i povećanu produkciju cAMP-a s posljedičnom hiperaktivacijom brojnih tkiva i organa. Sindrom se nasljeđuje mozaično, a do sada nije opisana vertikalna transmisija. U kliničkoj slici opisuje se klasična trijada simptoma, uključujući cafe-au-lait hiperpigmentacije kože, fibroznu displaziju kostiju i periferni preuranjeni pubertet. Bolest može pogađati i druge organe poput hipofize, štitnjače, nadbubrežne žlijezde, probavne organe i dr. S obzirom na mozaičan način nasljeđivanja, mutirane stanice raspoređuju se po organizmu u različitim omjerima, što konačno dovodi do varijabilne težine kliničke slike u svakog oboljelog- od asimptomatskih slučajeva do slučajeva nespojivih sa životom. Dijagnoza se postavlja klinički, a popis diferencijalnih dijagnoza je širok. Bolesnike se najčešće dijagnosticira u ranoj dječjoj dobi, češće u ženskog spola. Ne postoji mogućnost liječenja uzročnog stanja, već se provodi simptomatsko liječenje s farmakološkim i kirurškim pristupom. Praćenje i terapijski pristup u ovih bolesnika iziskuje multidisciplinarni tim liječnika raznih specijalnosti.McCune-Albright syndrome (MAS) is a rare genetic disorder most commonly caused by a somatic missense mutation of a heterotrimeric G protein encoded at the GNAS locus. This mutation leads to Arg201Cys or Arg201His substitution in 95% of the cases, which causes abnormal Gαs transcription factor activity and increased cAMP production with consequent hyperactivation of numerous tissues and organs. The clinical expression describes a classic triad of symptoms, including cafe-au-lait hyperpigmentation of the skin, fibrous bone dysplasia, and peripheral premature puberty. The disease can affect other organs such as the pituitary gland, thyroid gland, adrenals, pancreas, etc. Due to the mosaic nature of the condition, mutated cells distribute throughout the body in different proportions, which leads to variable severity of the clinical presentations, from asymptomatic to severe. The diagnosis is made clinically, and the list of differential diagnoses is extensive. Patients are most often diagnosed in early childhood, more often in females. There is no definitive cure, but symptomatic treatment is performed with a pharmacological and surgical approach. Monitoring and therapeutic approach in these patients requires a multidisciplinary team of physicians of various subspecialties

Comparative analysis of the most famous accounting frauds in the world

Računovodstvene prijevare su vrlo aktualna tema kako u Hrvatskoj tako i u svijetu. Danas čovječanstvo svjedoči velikom broju i raznim oblicima računovodstvenih prijevara, a da toga u većini nisu niti svjesni. Glavni cilj ovog rada je unaprijediti znanje o načinima i signalima računovodstvenih prijevara. Financijske prijevare i razni oblici manipulacije u računovodstvu pojavljuju se vrlo često i kao takve veliki su problem za poslovanja poduzeća, ali i države. Forenzično računovodstvo i poslovna forenzika predstavljaju područje koje želi djelovati na prijevare. U velikom dijelu slučajeva, počinitelji prijevare žele prikazati što bolju sliku poslovanja poduzeća, tj. prikazivanje laže dobiti ili skrivanje velikih gubitaka poslovanja. U Republici Hrvatskoj u borbi protiv računovodstvenih prijevara sudjeluju nadležne institucije USKOK (Ured za suzbijanje korupcije i organiziranog kriminaliteta) i DORH (Državno odvjetništvo Republike Hrvatske). Statistički podaci prikazuju kako se sve više javljaju prijevara s navedenom problematikom pa im se iz tih razloga treba posvetiti veća pažnja. Rizik od računovodstvene prijevare uvijek je veći kada postoji pritisak na poduzeća da ispune određene ciljeve. Kad se poduzeće nalazi u gospodarskom padu, uvijek postoji povećani rizik od prijevare. Kada se osoba upusti u računovodstvenu prijevaru, ona namjerno manipulira računovodstvenim evidencijama kako bi financijska slika poduzeća izgledala boljom nego što zapravo jest. Kada se netko proglasi krivim za počinjenje računovodstvene prijevare, podliježe kaznenom gonjenju. U nekim situacijama menadžment može doći u iskušenje da počini direktnu prijevaru. Budući da se gotovina ne prikuplja kada se prodaja vrši „na račun“, korumpirana uprava može zabilježiti lažnu dodatnu prodaju jednostavnim stvaranjem fiktivnih kupaca i/ili bilježenjem fiktivne prodaje. Predmet diplomskog rada je komparativna analiza najpoznatijih računovodstvenih prijevara u svijetu.Accounting fraud is a very current topic both in Croatia and in the world. Today, humanity witnesses a large number and various forms of accounting fraud, without most of them being aware of it. The main goal of this paper is to advance knowledge about the ways and signals of accounting fraud. Financial frauds and various forms of manipulation in accounting appear very often and as such are a big problem for the business of companies, as well as the state. Forensic accounting and business forensics represent an area that wants to act on fraud. In most cases, fraud perpetrators want to present the best possible image of the company's operations, i.e. showing false profits or hiding large business losses. In the Republic of Croatia, the competent institutions USKOK (Office for Suppression of Corruption and Organized Crime) and DORH (State Attorney's Office of the Republic of Croatia) participate in the fight against accounting fraud. Statistical data show that more and more frauds are occurring with the above-mentioned problem, so for these reasons, more attention should be paid to them. The risk of accounting fraud is always higher when there is pressure on companies to meet certain targets. When a company is in an economic downturn, there is always an increased risk of fraud. When a person engages in accounting fraud, they intentionally manipulate accounting records to make a company's financial picture look better than it actually is. When someone is found guilty of committing accounting fraud, they are subject to criminal prosecution. In some situations, management may be tempted to commit outright fraud. Since cash is not collected when sales are made „on account, a corrupt administration can record fraudulent additional sales simply by creating fictitious customers and/or recording fictitious sales. The subject of the thesis is a comparative analysis of the most famous accounting frauds in the world

McCune - Albright syndrome

McCune-Albrightov sindrom (MAS) je rijedak genetski poremećaj kojeg, u većini slučajeva, odlikuje somatska missense mutacija heterotrimernog G proteina kodiranog na GNAS lokusu. Navedena mutacija u 95% slučajeva dovodi do Arg201Cys ili Arg201His supstitucije, koja pak uzrokuje abnormalnu aktivnost Gαs transkripcijskog faktora i povećanu produkciju cAMP-a s posljedičnom hiperaktivacijom brojnih tkiva i organa. Sindrom se nasljeđuje mozaično, a do sada nije opisana vertikalna transmisija. U kliničkoj slici opisuje se klasična trijada simptoma, uključujući cafe-au-lait hiperpigmentacije kože, fibroznu displaziju kostiju i periferni preuranjeni pubertet. Bolest može pogađati i druge organe poput hipofize, štitnjače, nadbubrežne žlijezde, probavne organe i dr. S obzirom na mozaičan način nasljeđivanja, mutirane stanice raspoređuju se po organizmu u različitim omjerima, što konačno dovodi do varijabilne težine kliničke slike u svakog oboljelog- od asimptomatskih slučajeva do slučajeva nespojivih sa životom. Dijagnoza se postavlja klinički, a popis diferencijalnih dijagnoza je širok. Bolesnike se najčešće dijagnosticira u ranoj dječjoj dobi, češće u ženskog spola. Ne postoji mogućnost liječenja uzročnog stanja, već se provodi simptomatsko liječenje s farmakološkim i kirurškim pristupom. Praćenje i terapijski pristup u ovih bolesnika iziskuje multidisciplinarni tim liječnika raznih specijalnosti.McCune-Albright syndrome (MAS) is a rare genetic disorder most commonly caused by a somatic missense mutation of a heterotrimeric G protein encoded at the GNAS locus. This mutation leads to Arg201Cys or Arg201His substitution in 95% of the cases, which causes abnormal Gαs transcription factor activity and increased cAMP production with consequent hyperactivation of numerous tissues and organs. The clinical expression describes a classic triad of symptoms, including cafe-au-lait hyperpigmentation of the skin, fibrous bone dysplasia, and peripheral premature puberty. The disease can affect other organs such as the pituitary gland, thyroid gland, adrenals, pancreas, etc. Due to the mosaic nature of the condition, mutated cells distribute throughout the body in different proportions, which leads to variable severity of the clinical presentations, from asymptomatic to severe. The diagnosis is made clinically, and the list of differential diagnoses is extensive. Patients are most often diagnosed in early childhood, more often in females. There is no definitive cure, but symptomatic treatment is performed with a pharmacological and surgical approach. Monitoring and therapeutic approach in these patients requires a multidisciplinary team of physicians of various subspecialties

Epigenetically inactivated RASSF1A as a tumor biomarker

RASSF1A, one of the eight isoforms of the RASSF1 gene, is a tumor suppressor gene that influences tumor initiation and development. In cancer, RASSF1A is frequently inactivated by mutations, loss of heterozygosity, and, most commonly, by promoter hypermethylation. Epigenetic inactivation of RASSF1A was detected in various cancer types and led to significant interest; current research on RASSF1A promoter methylation focuses on its roles as an epigenetic tumor biomarker. Typically, researchers analyzed genomic DNA (gDNA) to measure the amount of RASSF1A promoter methylation. Cell-free DNA (cfDNA) from liquid biopsies is a recent development showing promise as an early cancer diagnostic tool using biomarkers, such as RASSF1A. This review discusses the evidence on aberrantly methylated RASSF1A in gDNA and cfDNA from different cancer types and its utility for early cancer diagnosis, prognosis, and surveillance. We compared methylation frequencies of RASSF1A in gDNA and cfDNA in various cancer types. The weaknesses and strengths of these analyses are discussed. In conclusion, although the importance of RASSSF1A methylation to cancer has been established is included in several diagnostic panels, its diagnostic utility is still experimental