What the Croatian Doctors Want to be Learning About?

The aim of the study was to examine doctor’s attitude about topics in continuing medical education (CME) using anonymous questionnaire that was given to the members of the Croatian Medical Association in Rijeka. The questions concerned doctor’s interest of certain medical fields, influence of CME to their everyday practice, and importance of getting credits for re-licensure as a motive to participate in CME. The highest interest was shown for CME in emergency/intensive medicine and the lowest for transplantation medicine. The doctors in primary care showed statistically significantly higher interest for CME in family medicine, pulmology, rheumatology and rehabilitation medicine than hospital doctors. The influence of CME in everyday practice and the importance of getting credits for re-licensure as a reason to participate in CME, in the most cases, have been graded with medium grade 3. The results indicated the existence of specific needs in CME and stressed the importance of having CME with topics from clinical practice

Early diagnosis of neuronal ceroid lipofuscinosis type 2 – a myth or reality?

Neuronska ceroidna lipofuscinoza-tip 2 (CLN2) najčešća je dječja progresivna neurodegenerativna bolest. Glavna obilježja bolesti CLN2 su usporen razvoj govora koji prethodi epileptičkim napadajima, motoričkim poremećajima, progresivnom propadanju vida i kognitivnih funkcija te smrti u dobi rane adolescencije. Prosječno vrijeme od pojave prvih simptoma do postavljenja dijagnoze iznosi oko dvije godine. Kašnjenje u postavljanju dijagnoze odgađa terapijski pristup koji uključuje multidisciplinsku zdravstvenu skrb i učinkovitu primjenu enzimske nadomjesne terapije cerliponazom alfa (Brineura®). Dostupnost liječenja enzimskom nadomjesnom terapijom cerliponazom alfa svrstava CLN2 u skupinu lječivih neurodegenerativnih bolesti. U svake lječive i/ili potencijalno izlječive progresivne neurodegenrativne bolesti pravodobna dijagnoza je ključna, jer omogućuje liječenje u ranom stadiju bolesti. Prepoznavanje ranih simptoma od bitnog je značenja za usmjeravanje kliničara na odgovarajuće pretrage ili traženje dodatnog stručnog mišljenja. U sve djece s prvim neprovociranim epileptičkim napadajem u dobi između druge i četvrte godine, a izričito u one djece koja dodatno u anamnezi imaju podatak o usporenom razvoju govora i/ili motoričke smetnje prvi korak u cilju rane i pravodobne dijagnoze je mjerenje aktivnosti enzima TTP1 putem suhe kapi krvi na fi ltarskom papiru, koji je dostupan u našoj sredini. Dodatna genska analiza i nalaz patogenih mutacija u genu CLN2 potvrđuje dijagnozu. Podizanje svjesnosti o bolesti CLN2 kao lječivoj i potencijalno izlječivoj, te poštivanje predloženih smjernica u kliničkom pristupu zasigurno će pridonijeti tome da rana dijagnoza bolesti CLN2 postane naša stvarnost, a sve manje bude mit. Pravovremena i ispravna dijagnoza prvi je korak u poboljšanju cjelokupne skrbi za djecu oboljelu od bolesti CLN2 i njihove roditelje.Neuronal ceroid lipofuscinosis type 2 (CLN2) is the most common childhood progressive neurodegenerative disease. The main features of CLN2 disease are language delay that precedes epileptic seizures, motor disorders, progressive deterioration of vision and cognition with death in early adolescence. The average time from the onset of fi rst symptoms to diagnosis is about two years. Delay in diagnosis postpones therapeutic approach involving multidisciplinary health care and effi cacious enzyme replacement therapy with cerliponase alpha (Brineura®). The availability of enzyme replacement therapy classifi es CLN2 disease in the group of treatable neurodegenerative diseases. Recognition of early symptoms is crucial to direct clinicians to appropriate examinations or seek additional expert opinion. Timely diagnosis is imperative because it allows treatment at an early stage of the disease. In all children with the fi rst unprovoked epileptic seizures between the ages of two and four, and particularly in those children with a history of language delay and motor impairment, the fi rst step is to measure the TTP1 enzyme activity by dried blood spot testing, which is available in our setting. Genetic testing and a fi nding of pathogenic mutations in the CLN2 gene confi rm the diagnosis. Raising awareness of CLN2 disease as a treatable and potentially curable disease and following the proposed guidelines will certainly contribute to early diagnosis of CLN2 disease to become our reality rather than a myth. Timely and accurate diagnosis is the fi rst step in improving the overall care of children with CLN2 disease and their parents

EXERGY ANALYSIS OF THE MAIN PROPULSION STEAM TURBINE FROM MARINE PROPULSION PLANT

The paper presents exergy analysis of main propulsion steam turbine from LNG carrier steam propulsion plant. Measurement data required for turbine exergy analysis were obtained during the LNG carrier exploitation at three different turbine loads. Turbine cumulative exergy destruction and exergy efficiency are directly proportional - they increase during the increase in propulsion propeller speed (steam turbine load). Cumulative exergy destruction and exergy efficiency amounts 2041 kW and 66.01 % at the lowest (41.78 rpm), up to the 5923 kW and 80.72 % at the highest (83.00 rpm) propulsion propeller speed. Increase in propulsion propeller speed resulted with an increase in analyzed turbine developed power from 3964 kW at 41.78 rpm to 24805 kW at 83.00 rpm. Analyzed turbine lost power at the highest propulsion propeller speed is the highest and amounts 3339 kW. Steam content at the main propulsion turbine outlet decreases during the increase in propulsion propeller speed. Exergy flow streams can vary considerably, even for a small difference in propulsion propeller speed. Steam turbine in land-based power plant (high power steam turbine) or in marine steam plant (low power steam turbine) is not the component which exergy destruction or exergy efficiency is significantly influenced by the ambient temperature change

Cerebral palsy

Cerebral palsy (CP), a range of non-progressive syndromes of posture and motor impairment, is a common cause of disability in childhood. The disorder results from various insults to different areas within the developing nervous system. Additional disabilities as mental retardation, epilepsy, vision, hearing, behaviour and language impairments are very common. We presented new details regarding aetiology, diagnostics and managements options of the CP

Cerebral palsy

Cerebral palsy (CP), a range of non-progressive syndromes of posture and motor impairment, is a common cause of disability in childhood. The disorder results from various insults to different areas within the developing nervous system. Additional disabilities as mental retardation, epilepsy, vision, hearing, behaviour and language impairments are very common. We presented new details regarding aetiology, diagnostics and managements options of the CP