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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.

Hereditarni angioedem (HAE) je rijetka ali potencijalno po život opasna bolest, zbog nepredvidivih napada bezbolnih, asimetričnih, ograničenih, recidivirajućih, o gravitaciji neovisnih otoka supkutanog ili submukoznog, rahlog intersticijskog tkiva u trajanju od par sati do nekoliko dana. Bolest je posljedica nasljednih defekata gena koji kodiraju C1-inhibitor ili druge proteine u kininskom putu.Oboljelih od HAE u RH ima oko 100.Prikazane su smjernice za liječenje HAE u Hrvatskoj.

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 testirati, 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.

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.

Jedna od hitnoća koja može ugroziti stanje pacijenta te izazvati strah i zabrinutost u otorinolaringologiji je epistaksa. Epistaksa označava krvarenje iz nosa. Etiologija može biti različita, a krvarenje se dijeli na prednje ili stražnje. Najčešće popratne bolesti kod epistakse su hipertenzija i koagulopatije. Krvarenje iz nosnica može biti jednostrano ili obostra¬no, a bolesnik može krvariti i na usta. Pri pružanju prve pomoći, bolesnika stavljamo u uspravni sjedeći položaj s glavom nagnutom prema naprijed, čime se smanjuje otjecanje krvi u usta te njeno gutanje i povraćanje. Da bi se postigla kompresija, bolesnik prije tamponade mora dobro ispuhati nos i izbaciti iz nosa sve ugruške. Najčešće nakon prednje tamponade krvarenje stane, bolesnika se promatra te nakon toga otpušta na kućnu njegu. Prije otpusta provjera¬vamo slijeva li se svježa krv iz epifarinksa niz stražnju stijenku ždrijela. Ako nakon prednje tamponade krv nije stala, potrebno je učiniti stražnju tamponadu nakon čega se pacijent hospitalizira te dalje obrađuje i liječi. Po potrebi se pristupa drugim metodama zaustavljanja krvarenja kao što su embolizacija, ligacija te kirurško liječenje. Epistaxis is one of the emergencies that can endanger the patient's condition and cause fear and concern in otorhinolaryngology. Epistaxis means bleeding from the nose. The etiology can be different, and the bleeding is divided into anterior or posterior. The most common comorbidities in epistaxis are hypertension and coagulopathies. Bleeding from the nostrils can be unilateral or bilateral, and the patient may bleed into the mouth. In first aid, the patient is placed in an upright sitting position with the head tilted forward, which reduces the outflow of blood into the mouth and its swallowing and vomiting. In order to achieve compression, the patient must blow his nose well before nasal packing and expel all clots from his nose. Most often, after anterior tamponade, the bleeding stops, the patient is observed and then discharged to home care. Before discharge, we check if fresh blood from the epipharynx flows down the back wall of the pharynx. If the bleeding did not stop after the anterior packing, it is necessary to perform a posterior nasal packing, after which the patient is hospitalized and further processed and treated. If necessary, other methods of stopping bleeding are approached, such as embolization, ligation, and surgical treatment.

U radu je opisana traheotomija, kirurški zahvat koji se izvodi kod različitih stanja koja ugrožavaju disanje. Opisane su indikacije i kontraindikacije te moguće posljedice nakon kirurškoga zahvata. Postoji više načina izvođenja traheotomije; kirurška ili perkutana, može biti trajna ili privremena. Također, postoji više vrsta trahealnih kanila koje stoje u traheostomi. Nakon zahvata je važno kvalitetno provoditi zdravstvenu njegu rane kako ne bi došlo do neželjenih postoperativnih komplikacija. Oporavak je individualan te ovisi o fizičkome, ali i mentalnome stanju pacijenta. The paper describes a tracheotomy, a surgical procedure performed in various conditions that endanger breathing. Indications , contraindications and possible consequences after surgery are described. There are several ways to perform a tracheotomy; surgical or percutaneous. Tracheostomy may be permanent or temporary. After the procedure, it is important to provide quality wound care so that unwanted postoperative complications do not occur. Recovery is individual and depends on the physical and mental condition of the patient.