Intratireoidna lokalizacija tireoglosalne ciste

Tireoglosalna cista jedna je od najčešćih benignih razvojnih tvorbi područja medijalne linije vrata. Tireoglosalne ciste nastaju zbog nepotpunog sraštavanja tireoglosalnog duktusa koji se proteže od foramena cekuma, područja korijena jezika do mjesta štitnjače u medijalnoj regiji vrata. U šestom tjednu fetalnog života tireoglosalni duktus nestaje, ali ukoliko zaostanu pojedinačne stanice ili nakupine embrionalnih stanica, iz njih se kasnije mogu razviti tireoglosalne ciste. Najčešće se javljaju u dječjoj dobi. Tireoglosalne ciste mogu biti različitog smještaja,a prema literaturi se opisuje lokalizacija ovisno o jezičnoj kosti – suprahiodno (20%), infrahioidno (65%), transhioidno (15%). U ovom prikazu slučaja želimo prikazati rijedak smještaj tireoglosalne ciste, također u dobi za koju razvojna anomalija nije učestala. U našem slučaju riječ je o bolesnici u dobi od 60 godina s intratireoidnom lokalizacijom tireoglosalne ciste. Intratireoidno smještena tireoglosalna cista u odrasloj dobi je rijetka pojavnost, a u literaturi je prikazano samo nekoliko slučajeva

Intratireoidna lokalizacija tireoglosalne ciste

Tireoglosalna cista jedna je od najčešćih benignih razvojnih tvorbi područja medijalne linije vrata. Tireoglosalne ciste nastaju zbog nepotpunog sraštavanja tireoglosalnog duktusa koji se proteže od foramena cekuma, područja korijena jezika do mjesta štitnjače u medijalnoj regiji vrata. U šestom tjednu fetalnog života tireoglosalni duktus nestaje, ali ukoliko zaostanu pojedinačne stanice ili nakupine embrionalnih stanica, iz njih se kasnije mogu razviti tireoglosalne ciste. Najčešće se javljaju u dječjoj dobi. Tireoglosalne ciste mogu biti različitog smještaja,a prema literaturi se opisuje lokalizacija ovisno o jezičnoj kosti – suprahiodno (20%), infrahioidno (65%), transhioidno (15%). U ovom prikazu slučaja želimo prikazati rijedak smještaj tireoglosalne ciste, također u dobi za koju razvojna anomalija nije učestala. U našem slučaju riječ je o bolesnici u dobi od 60 godina s intratireoidnom lokalizacijom tireoglosalne ciste. Intratireoidno smještena tireoglosalna cista u odrasloj dobi je rijetka pojavnost, a u literaturi je prikazano samo nekoliko slučajeva

Construction of Road Safety Composite Indicator Using Grey Relational Analysis

Aggregation of different variables into one road safety performance index is a popular concept in evaluating road safety and comparing the performance of territories/entities. This paper presents the development of a novel and innovative weighting methodology using grey relational analysis. Based on the proposed model, ten hierarchical road safety indicators were selected in terms of a two-layered model with three categories related to behaviour, safety and system. Grey weights are assigned to the categorized indicators in each layer, and the grey road safety composite indicator for each entity (21 selected territories) is calculated by the weighted sum approach. With relatively high weights, this systematic methodology can serve the policy makers in targeting the risk domains where improvements are needed. The results clearly illustrate effectiveness in addressing a large number of indicators with hierarchical structures

KARAKTERISTIKE BOLESNIKA PODVRGNUTIH OPERACIJI SINUSA: ORL odjel, Opća bolnica Pula, od 2008. do 2013. godine

Functional endoscopic sinus surgery (FESS) is the most commonly used surgical technique to treat pathology of the sinuses. During the years of developing FESS technique, indication has spread from the inflammatory pathology to sinus malignancy. Most common pathology treated with FESS is medically unresponsive chronic sinusitis. We have analyzed characteristics of patients underwent sinus surgery in our ward during five year period, from 2008. to 2013. We have included 177 patients and 171 of them had a form of chronic sinusitis. 125 patients had bilateral sinus pathology and 52 had unilateral sinus pathology. All of the patients with bilateral sinus pathology had a form of chronic sinusitis. All of our patients were preoperatively and postoperatively treated by suggested EPOS guidelines. Postoperative regular check up is keeping the number of revision surgery low. Only four patients previously underwent FESS needed reoperation. One had inverted papilloma and other three of them had Samter syndrome

Metastatski tumori temporalne kosti

Zloćudni tumori temporalne kosti izrazito su rijetki i čine svega 0,2% svih tumora glave i vrata. Najčešći primarni zloćudni tumor temporalne kosti je planocelularni karcinom, a slijede ga adenokarcinom, melanom, rabdomiosarkom, osteosarkom, limfom, adenoid cistični karcinom, karcinom acinusnih stanica i drugi. Osim primarnih tumora, temporalna kost može biti sijelo metastaza solidnih i hematoloških tumora. Iako su istraživanja o metastatskim tumorima temporalne kosti evoluirala posljednjih desetljeća, i dalje govorimo o izuzetno rijetkom entitetu koji se često prezentira kompliciranom kliničkom slikom i zahtijeva individualizirani pristup liječenju. Prema dosadašnjim istraživanjima, najčešća primarna sijela metastaza u temporalnu kost su dojka, pluća, probavni sustav, bubreg i prostata. Put širenja tumora je u najvećem broju slučajeva hematogen, a najčešće sijelo je apeks petrozne kosti. Klinička slika je često nespecifična i trećina bolesnika nema otološke simptome, što otežava dijagnozu. Najčešći otološki simptomi i znakovi su gubitak sluha, pareza ličnog živca, otalgija, otoreja i vrtoglavica. Uglavnom se radi o kasnoj manifestaciji bolesti, nakon što je tumor već metastazirao u druge dijelove tijela. Obzirom na dosadašnja saznanja, navedeni otološki simptomi i znakovi, posebice pareza ličnog živca i gubitak sluha kod onkoloških bolesnika, moraju pobuditi sumnju na sekundarni proces u temporalnoj kosti. U ovom prikazu slučaja prezentirati ćemo bolesnika s metastazom karcinoma urotelnog trakta u temporalnu kost, što je ujedno bila i prva manifestacija primarne onkološke bolesti

Metastatski tumori temporalne kosti

Zloćudni tumori temporalne kosti izrazito su rijetki i čine svega 0,2% svih tumora glave i vrata. Najčešći primarni zloćudni tumor temporalne kosti je planocelularni karcinom, a slijede ga adenokarcinom, melanom, rabdomiosarkom, osteosarkom, limfom, adenoid cistični karcinom, karcinom acinusnih stanica i drugi. Osim primarnih tumora, temporalna kost može biti sijelo metastaza solidnih i hematoloških tumora. Iako su istraživanja o metastatskim tumorima temporalne kosti evoluirala posljednjih desetljeća, i dalje govorimo o izuzetno rijetkom entitetu koji se često prezentira kompliciranom kliničkom slikom i zahtijeva individualizirani pristup liječenju. Prema dosadašnjim istraživanjima, najčešća primarna sijela metastaza u temporalnu kost su dojka, pluća, probavni sustav, bubreg i prostata. Put širenja tumora je u najvećem broju slučajeva hematogen, a najčešće sijelo je apeks petrozne kosti. Klinička slika je često nespecifična i trećina bolesnika nema otološke simptome, što otežava dijagnozu. Najčešći otološki simptomi i znakovi su gubitak sluha, pareza ličnog živca, otalgija, otoreja i vrtoglavica. Uglavnom se radi o kasnoj manifestaciji bolesti, nakon što je tumor već metastazirao u druge dijelove tijela. Obzirom na dosadašnja saznanja, navedeni otološki simptomi i znakovi, posebice pareza ličnog živca i gubitak sluha kod onkoloških bolesnika, moraju pobuditi sumnju na sekundarni proces u temporalnoj kosti. U ovom prikazu slučaja prezentirati ćemo bolesnika s metastazom karcinoma urotelnog trakta u temporalnu kost, što je ujedno bila i prva manifestacija primarne onkološke bolesti

GUIDELINES FOR THE DIAGNOSIS AND TREATMENT OF HEREDITARY ANGIOEDEMA

Hereditarni angioedem (HAE) rijetka je, ali potencijalno za život opasna bolest zbog nepredvidivih napadaja bezbolnih, ograničenih, recidivirajućih otoka supkutanog ili submukoznog, intersticijskog tkiva u trajanju od nekoliko sati do nekoliko dana. Oboljelih od HAE u RH ima oko 100 (ali vjerojatno ima više nedijagnosticiranih). Poseban kvalitativni napredak u usklađivanju dogovora o liječenju HAE jesu Smjernice Svjetske alergološke organizacije koje su donesene 2012. Oslanjajući se na taj dokument, Radna grupa hrvatskih stručnjaka pripremila je prijedlog smjernica za liječenje HAE u Hrvatskoj. Napadaji angioedema u HAE posljedica su mutacije gena za plazmatski protein inhibitor C1. Zbog manjka inhibitora ili njegove disfunkcionalnosti dolazi do okidačem (trigerom) potaknute autoaktivacije C1 i cijele kaskade s konačno povećanom propusnošću krvnih žila i edemom tkiva. Postoje tri tipa hereditarnog angioedema: tip I uzrokovan sniženom razinom C1inh proteina, tip II uzrokovan proizvodnjom nefunkcionalnog C1inh proteina te tip III karakteriziran normalnom funkcijom i razinom C1inh. U svih bolesnika sa sumnjom na HAE mora se odrediti nivo C4 i inhibitora C1, kao i funkcija inhibitora C1. Liječenje akutne atake HAE: Svi napadaji angioedema koji onesposobe dijelove tijela i/ili zahvaćaju lice, vrat, trbuh, a pogotovo gornje dišne putove zahtijevaju liječenje. Terapija mora biti odmah dostupna (On-Demand Treatment). U akutnoj ataci treba odmah primijeniti koncentrat inhibitora C1 (dobiven iz plazme ili rekombinantni), ikatibant ili ekalantid. Ako ovi lijekovi nisu dostupni, akutni napadaji edema mogu se liječiti plazmom obrađenom detergentom. Ako se ovakva plazma ne može dobiti, angioedemi se liječe svježe smrznutom plazmom. Intubacija ili traheotomija moraju se izvesti na vrijeme ako progredira edem gornjih dišnih putova. U napadaju angioedema bolesnik može dobiti i adjuvantnu terapiju (analgetike, infuzije). Preporučuje se da svi bolesnici uvijek nose sa sobom lijekove za samoprimjenu. Preporučljiva je kratkoročna profilaksa edema prije kirurških zahvata (osobito stomatoloških zahvata), zahvata u kojima je potrebna endotrahealna intubacija, zahvata na gornjim dišnim putovima ili farinksu te prije bronhoskopije i endoskopije. Dugoročna profilaksa indicirana je ako se javi jedna ili više težih ataka angioedema na mjesec. Kao dugoročna profilaksa mogu se rabiti koncentrat inhibitora C1 ili androgeni. Probir djece za HAE trebalo bi odgoditi do 12. mjeseca života. Sve potomke bolesnika treba testiratiHereditary angioedema (HAE) is a rare but potentially fatal genetic disorder with nonpitting, nonerythematous, and not pruritic swelling which can affect the hands, feet, face, genitals and visceral mucosa. The type, frequency, and severity of the attacks vary between patients, and over the lifetime of an individual patient. Efforts in Croatian counties have identified approximately 100 patients (but there must be more undiagnosed patients). The first global guideline for the management of HAE was developed by the World Allergy Organization HAE International Alliance and published in 2012. Based on that document the Working group of Croatian experts was assigned to propose guideline for HAE management in Croatia. HAE is is most often related to decreased or dysfunctional C1 inh with autoactivation of C1 and bradykinin accumulation leading to localized dilatation and increased permeability of blood vessels resulting in tissue swelling. A diagnosis of HAE can be confirmed by measuring complement and C1 inh quantitative and functional levels.Three HAE types could be differentiated: HAE type 1 (C1 inh level is low), HAE type 2 (C1 inh level is normal but dysfunctional), and HAE type 3 (normal level and function of C1 inh). All patients suspected to have HAE-1/2 should be assessed for blood levels of C4, C1 inh protein, and C1 inh function. All attacks that result in debilitation/dysfunction and/or involve the face, the neck, or the abdomen should be considered for on-demand treatment. It is recommended that attacks are treated as early as possible. HAE attacks are treated with C1 inh, ecallantide, or icatibant.If these drugs are not available, attacks should be treated with solvent detergent-treated plasma (SDP). If SDP is not available, then attacks should be treated with frozen plasma.Intubation or tracheotomy should be considered early in progressive upper airway edema. Patients with attacks could receive adjuvant therapy when indicated (pain management, intravenous fluids). All patients should have on-demand treatment for two attacks and carry their on-demand treatment at all times. The administration of short-term prophylaxis should be considered before surgeries (dental/intraoral surgery, where endotracheal intubation is required), where upper airway or pharynx is manipulated, and before bronchoscopy or endoscopy. Long-term prophylaxis should be considered in all severely symptomatic HAE-1/2 patients. C1 inh concentrate or androgens can be used. Screening children for HAE-1/2 should be deferred until the age of 12 months, and all offspring of an affected parent should be tested

PERFORMANCE ASSESSMENT TOOL FOR QUALITY IMPROVEMENT IN HOSPITALS (PATH): FIRST EXPERIENCES IN CROATIA

PATH (Performance Assessment Tool for Quality Improvement in Hospitals), projekt Regionalnog ureda Svjetske zdravstvene organizacije (SZO) za Europu pruža bolnicama sveobuhvatan i standardiziran alat za vrednovanje vlastitih rezultata i razvoj mjera za unapređenje kvalitete. Program PATH pokrenut je 2008. godine i u Hrvatskoj, a od 2009. godine provodi se u bolnicama koje su se dobrovoljno odlučile u njega uključiti. U ovom radu prikazujemo prva iskustva probne faze uspostavljanja programa PATH, utemeljena na podacima koji su prikupljani u 22 hrvatske bolnice. Analiza prvih rezultata upozorila je na postojanje izraženih razlika među bolnicama, koje su se na primjeru postotka carskih rezova kretale od najmanje 1,1% do najviše 21,4% zabilježenih carskih rezova u pojedinim bolnicama tijekom razdoblja prikupljanja podataka. Stopa smrtnosti infarkta miokarda kretala se od 1,9 do 21,4%, dok se smrtnost moždanog udara kretala od 12,5 do 45,5%. Najviši postotak prijavljenih ubodnih ozljeda za liječnike iznosio je 16,2% osoblja tijekom jedne godine, 6,1% za medicinske sestre i 4,6% za spremačice. Ovo istraživanje upućuje na postojanje mnogih problema i ograničenja u prikupljanju pokazatelja na bolničkoj razini, njihovoj analizi i stvaranju preporuka za unapređenje kvalitete koje se moraju uzeti u obzir prilikom usporedbe bolnica na nacionalnoj ili međunarodnoj razini.PATH (Performance Assessment Tool for Quality Improvement in Hospitals), a project of the World Health Organization (WHO) for Europe offers hospitals a comprehensive and standardized tool (a set of indicators) to evaluate their own performance and development of measures for quality improvement. PATH Program was launched in Croatia in 2008, and it was conducted in 2009 in hospitals that have voluntarily decided to be involved. Here we present the results of the first phase of pilot experience of establishing the program, based on data collected in 22 Croatian hospitals. Analysis of the first results indicated the existence of marked differences among the hospitals that have taken the example of the percentage of cesarean sections ranging from 1.1% to 21.4%. The mortality rate of myocardial infarction ranged from 1.9 to 21.4%, while the mortality of stroke ranged from 12.5 to 45.5%. The highest percentage of needle-stick injuries reported for physicians was 16.2% of entire hospital staff in one year, 6.1% for nurses and 4.6% for the supportive staff. The result suggests the existence of many problems and limitations in data collection at hospital level, limitations in their analysis and creates recommendations for quality improvements, which must be taken into account when hospitals are compared on the national or international level

Smjernice za dijagnostiku i liječenje hereditarnog angioedema [Guidelines for the diagnosis and treatment of hereditary angioedema]

Hereditary angioedema (HAE) is a rare but potentially fatal genetic disorder with nonpitting, nonerythematous, and not pruritic swelling which can affect the hands, feet, face, genitals and visceral mucosa. The type, frequency, and severity of the attacks vary between patients, and over the lifetime of an individual patient. Efforts in Croatian counties have identified approximately 100 patients (but there must be more undiagnosed patients). The first global guideline for the management of HAE was developed by the World Allergy Organization HAE International Alliance and published in 2012. Based on that document the Working group of Croatian experts was assigned to propose guideline for HAE management in Croatia. HAE is is most often related to decreased or dysfunctional C1 inh with autoactivation of C1 and bradykinin accumulation leading to localized dilatation and increased permeability of blood vessels resulting in tissue swelling. A diagnosis of HAE can be confirmed by measuring complement and C1 inh quantitative and functional levels.Three HAE types could be differentiated: HAE type 1 (C1 inh level is low), HAE type 2 (C1 inh level is normal but dysfunctional), and HAE type 3 (normal level and function of C1 inh). All patients suspected to have HAE-1/2 should be assessed for blood levels of C4, C1 inh protein, and C1 inh function. All attacks that result in debilitation/dysfunction and/or involve the face, the neck, or the abdomen should be considered for on-demand treatment. It is recommended that attacks are treated as early as possible. HAE attacks are treated with C1 inh, ecallantide, or icatibant.If these drugs are not available, attacks should be treated with solvent detergent-treated plasma (SDP). If SDP is not available, then attacks should be treated with frozen plasma.Intubation or tracheotomy should be considered early in progressive upper airway edema. Patients with attacks could receive adjuvant therapy when indicated (pain management, intravenous fluids). All patients should have on-demand treatment for two attacks and carry their on-demand treatment at all times. The administration of short-term prophylaxis should be considered before surgeries (dental/intraoral surgery, where endotracheal intubation is required), where upper airway or pharynx is manipulated, and before bronchoscopy or endoscopy. Long-term prophylaxis should be considered in all severely symptomatic HAE-1/2 patients. C1 inh concentrate or androgens can be used. Screening children for HAE-1/2 should be deferred until the age of 12 months, and all offspring of an affected parent should be tested

GUIDELINES FOR THE DIAGNOSIS AND TREATMENT OF HEREDITARY ANGIOEDEMA

Hereditarni angioedem (HAE) rijetka je, ali potencijalno za život opasna bolest zbog nepredvidivih napadaja bezbolnih, ograničenih, recidivirajućih otoka supkutanog ili submukoznog, intersticijskog tkiva u trajanju od nekoliko sati do nekoliko dana. Oboljelih od HAE u RH ima oko 100 (ali vjerojatno ima više nedijagnosticiranih). Poseban kvalitativni napredak u usklađivanju dogovora o liječenju HAE jesu Smjernice Svjetske alergološke organizacije koje su donesene 2012. Oslanjajući se na taj dokument, Radna grupa hrvatskih stručnjaka pripremila je prijedlog smjernica za liječenje HAE u Hrvatskoj. Napadaji angioedema u HAE posljedica su mutacije gena za plazmatski protein inhibitor C1. Zbog manjka inhibitora ili njegove disfunkcionalnosti dolazi do okidačem (trigerom) potaknute autoaktivacije C1 i cijele kaskade s konačno povećanom propusnošću krvnih žila i edemom tkiva. Postoje tri tipa hereditarnog angioedema: tip I uzrokovan sniženom razinom C1inh proteina, tip II uzrokovan proizvodnjom nefunkcionalnog C1inh proteina te tip III karakteriziran normalnom funkcijom i razinom C1inh. U svih bolesnika sa sumnjom na HAE mora se odrediti nivo C4 i inhibitora C1, kao i funkcija inhibitora C1. Liječenje akutne atake HAE: Svi napadaji angioedema koji onesposobe dijelove tijela i/ili zahvaćaju lice, vrat, trbuh, a pogotovo gornje dišne putove zahtijevaju liječenje. Terapija mora biti odmah dostupna (On-Demand Treatment). U akutnoj ataci treba odmah primijeniti koncentrat inhibitora C1 (dobiven iz plazme ili rekombinantni), ikatibant ili ekalantid. Ako ovi lijekovi nisu dostupni, akutni napadaji edema mogu se liječiti plazmom obrađenom detergentom. Ako se ovakva plazma ne može dobiti, angioedemi se liječe svježe smrznutom plazmom. Intubacija ili traheotomija moraju se izvesti na vrijeme ako progredira edem gornjih dišnih putova. U napadaju angioedema bolesnik može dobiti i adjuvantnu terapiju (analgetike, infuzije). Preporučuje se da svi bolesnici uvijek nose sa sobom lijekove za samoprimjenu. Preporučljiva je kratkoročna profilaksa edema prije kirurških zahvata (osobito stomatoloških zahvata), zahvata u kojima je potrebna endotrahealna intubacija, zahvata na gornjim dišnim putovima ili farinksu te prije bronhoskopije i endoskopije. Dugoročna profilaksa indicirana je ako se javi jedna ili više težih ataka angioedema na mjesec. Kao dugoročna profilaksa mogu se rabiti koncentrat inhibitora C1 ili androgeni. Probir djece za HAE trebalo bi odgoditi do 12. mjeseca života. Sve potomke bolesnika treba testiratiHereditary angioedema (HAE) is a rare but potentially fatal genetic disorder with nonpitting, nonerythematous, and not pruritic swelling which can affect the hands, feet, face, genitals and visceral mucosa. The type, frequency, and severity of the attacks vary between patients, and over the lifetime of an individual patient. Efforts in Croatian counties have identified approximately 100 patients (but there must be more undiagnosed patients). The first global guideline for the management of HAE was developed by the World Allergy Organization HAE International Alliance and published in 2012. Based on that document the Working group of Croatian experts was assigned to propose guideline for HAE management in Croatia. HAE is is most often related to decreased or dysfunctional C1 inh with autoactivation of C1 and bradykinin accumulation leading to localized dilatation and increased permeability of blood vessels resulting in tissue swelling. A diagnosis of HAE can be confirmed by measuring complement and C1 inh quantitative and functional levels.Three HAE types could be differentiated: HAE type 1 (C1 inh level is low), HAE type 2 (C1 inh level is normal but dysfunctional), and HAE type 3 (normal level and function of C1 inh). All patients suspected to have HAE-1/2 should be assessed for blood levels of C4, C1 inh protein, and C1 inh function. All attacks that result in debilitation/dysfunction and/or involve the face, the neck, or the abdomen should be considered for on-demand treatment. It is recommended that attacks are treated as early as possible. HAE attacks are treated with C1 inh, ecallantide, or icatibant.If these drugs are not available, attacks should be treated with solvent detergent-treated plasma (SDP). If SDP is not available, then attacks should be treated with frozen plasma.Intubation or tracheotomy should be considered early in progressive upper airway edema. Patients with attacks could receive adjuvant therapy when indicated (pain management, intravenous fluids). All patients should have on-demand treatment for two attacks and carry their on-demand treatment at all times. The administration of short-term prophylaxis should be considered before surgeries (dental/intraoral surgery, where endotracheal intubation is required), where upper airway or pharynx is manipulated, and before bronchoscopy or endoscopy. Long-term prophylaxis should be considered in all severely symptomatic HAE-1/2 patients. C1 inh concentrate or androgens can be used. Screening children for HAE-1/2 should be deferred until the age of 12 months, and all offspring of an affected parent should be tested