Unilateral facial palsy and hearing loss as initial manifestation of Granulomatosis with polyangiitis: a case report

Granulomatosis with polyangiitis (GPA) is a systematic autoimmune disorder characterized by necrotizing vasculitis of small to medium-sized-vessels and granuloma formation. GPA is commonly initially presented with respiratory and renal symptoms. Although involvement of the temporal bone is not unusual, an otologic disorder is rarely the presenting symptom. We present a case of a 36-year-old patient complaining of severe ear pain, hearing loss and unilateral facial palsy. After comprehensive clinical diagnostics and temporal bone and chest imaging, a multiorgan involving form of GPA was diagnosed. The patient was treated with cyclophosphamide and methylprednisolone which resulted in amelioration of ear pain, improvement of hearing loss, partial resolution of facial palsy and altogether clinical improvement of the patient. Although infrequently, systemic GPA can initially present with otologic symptoms and should not be overlooked as a possible cause of an otologic disorder resilient to usual pragmatic treatment

Unilateral facial palsy and hearing loss as initial manifestation of Granulomatosis with polyangiitis: a case report

Granulomatosis with polyangiitis (GPA) is a systematic autoimmune disorder characterized by necrotizing vasculitis of small to medium-sized-vessels and granuloma formation. GPA is commonly initially presented with respiratory and renal symptoms. Although involvement of the temporal bone is not unusual, an otologic disorder is rarely the presenting symptom. We present a case of a 36-year-old patient complaining of severe ear pain, hearing loss and unilateral facial palsy. After comprehensive clinical diagnostics and temporal bone and chest imaging, a multiorgan involving form of GPA was diagnosed. The patient was treated with cyclophosphamide and methylprednisolone which resulted in amelioration of ear pain, improvement of hearing loss, partial resolution of facial palsy and altogether clinical improvement of the patient. Although infrequently, systemic GPA can initially present with otologic symptoms and should not be overlooked as a possible cause of an otologic disorder resilient to usual pragmatic treatment

A Battery in the Stenotic Esophagus of a Child with a Congenital Tracheoesophageal Fistula

A case of a three-year-old male child who was admitted to our hospital with the suspicion that he had swallowed a battery approximately one hour before admittance. The parents believed that it was a button-shaped lithium battery approximately 12 mm in diameter. A chest X-ray was taken immediately, and a battery was identified in the esophagus at the fifth thoracic vertebra. By reviewing the child’s medical history, we found that the child had had surgery the day after birth due to congenital atresia of the esophagus and a tracheoesophageal fistula type III b. An esophagoscopy was performed one hour after admittance, and the battery was found to be partially past the scar from the first surgery. Because of that, the battery was pushed further toward the stomach, out of fear that retrieving the battery through the scarred section of the child’s esophagus could damage the stenotic wall. Upon the next X-ray of the abdomen, the battery was observed in the stomach. The child was monitored, and X-rays were taken over the next several days. The battery was evacuated in stool eight days after it had been ingested

The association of allergy and otitis media with eff usion in children

Upala srednjeg uha s izljevom (OME) česta je bolest u djece s prevalencijom koja doseže do 20% i s utjecajem na provodni gubitak sluha, zakašnjeli razvoj govora i oštećenje sluznice srednjeg uha. Cilj ove studije je utvrditi povezanost alergija i perzistirajućih upala srednjeg uha s izljevom (OME) u djece. U istraživanje je uključeno sedamdeset šestero (76) djece između 2 i 14 godina s dijagnozom perzistirajuće upale srednjeg uha s izljevom, koja su podvrgnuta operativnom zahvatu postavljanja aerizacijskih cjevčica. Sedamdeset šestero (76) zdrave djece slične dobi koja su podvrgnuta različitim neotorinolaringološkim operativnim zahvatima uključena su u kontrolnu skupinu. Pozitivna anamneza alergijskog rinitisa, rezultati intradermalnih kožnih testova i vrijednosti ukupnog i specifi čnog IgE-a su dokumentirani.U skupini s dijagnozom upale srednjeg uha s izljevom 58-ero djece (76,3%) imalo je pozitivnu anamnezu alergijskog rinitisa, a njih 45-ero (59,2%) imalo je pozitivne nalaze intradermalnog kožnog testiranja. Vrijednosti ukupnog IgE-a bile su povišene u 46-ero djece (60.5%), a vrijednosti specifi čnog IgE-a bile su povišene u njih 39-ero (51,3%) u OME skupini. U kontrolnoj skupini 12-ero djece (15,8%) imalo je pozitivnu anamnezu alergijskog rinitisa, njih 10-ero (13,2%) imalo je pozitivne nalaze intradermalnog kožnog testiranja, vrijednosti ukupnog IgE-a bile su povišene u 17-ero djece (22,4%) iz kontrolne skupine, a njih 9-ero (11,8%) imalo je povišene vrijednosti specifi čnog IgE-a. Rad pokazuje značajno veću prevalenciju alergija u skupini djece s upalom srednjeg uha s izljevom u usporedbi s kontrolnom skupinom. Značajno veći broj pozitivnih nalaza alergoloških testiranja nalazimo u skupini djece s upalom srednjeg uha s izljevom u odnosu na kontrolnu skupinu.Otitis media with eff usion (OME) is a common disease in children with a prevalence of up to 20% and great impact on conductive hearing loss, delayed speech development and destruction of middle ear mucosa lining. The aim of the study was to evaluate the association of allergy and persistent OME in children. Seventy-six children aged 2 to 14 years diagnosed with OME were included in the study. Control group included 76 age-matched healthy children attending pediatric surgical unit for diff erent pediatric surgical procedures other than ENT surgery. Fifty-eight (76.3%) OME children had positive history of allergic rhinitis and 45 (59.2%) had positive results of intradermal skin tests. Total IgE levels were elevated in 46 (60.5%) children and specifi c IgE levels were elevated in 39 (51.3%) children in OME group. In control group, 12 (15.8) children had positive history of allergic rhinitis and only 10 (13.2%) had positive results of intradermal skin tests. Total IgE levels were elevated in 17 (22.4%) children and specifi c IgE levels were elevated in 9 (11.8%) control group children. This study showed the prevalence of allergic history to be higher in children with OME as compared with control group. The prevalence of positive results of diff erent allergy tests was also higher in the OME group as compared with control group

Smjernice za dijagnostiku i liječenje akutne vrtoglavice i gubitka ravnoteže

Dijagnostičko-terapijske smjernice za akutnu vrtoglavicu i gubitak ravnoteže, namijenjene su ponajprije otorinolaringolozima, specijalistima hitne medicine, liječnicima obiteljske medicine, te ostalim liječnicima u Republici Hrvatskoj koji skrbe o bolesnicima s vrtoglavicom i gubitkom ravnoteže, u primarnoj i bolničkoj zdravstvenoj zaštiti. U izradi smjernica sudjelovali su predstavnici Hrvatskog društva za otorinolaringologiju i kirurgiju glave i vrata (HD ORL), te predstavnica Hrvatskog neurološkog društva. Prepoznati uzrok akutne vrtoglavice u praksi otežava činjenica da se simptomi oštećenja u različitim organskim sustavima preklapaju, nejasni su i djeluju dramatično. Cilj ovih smjernica je pomoć pri postavljanju dijagnoze kod bolesnika s izraženim simptomima vrtoglavice i pravodobno razlikovanje benigne od životno ugrožavajuće vrtoglavice koja zahtijeva hitno, bolničko liječenje. Smjernice sadrže i detaljne algoritme koji preporučuju postupanja s bolesnikom s napadajem akutne vrtoglavice i gubitkom ravnoteže

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

Smjernice za vestibularnu rehabilitaciju jednostranih i obostranih vestibularnih oštećenja*

Vestibularna rehabilitacija jest planirani, individualno prilagođen sustav vježbi kojima se ubrzava proces središnje kompenzacije oštećenoga vestibularnog sustava. U medicinu ju uvode Cawthorne i Cooksey sredinom prošloga stoljeća. Godinama su postojali prijepori o značenju i učinkovitosti ovoga načina liječenja. Provedene su brojne randomizirane prospektivne kliničke studije, koje su dokazale važnost vestibularnih vježbi, kao i njihov povoljan učinak na brzinu i stupanj oporavka bolesnika, tako da one posljednjih desetljeća zauzimaju sve značajnije mjesto u liječenju vrtoglavica. Vestibularna hipofunkcija, bilo da je riječ o akutnome ili kroničnom poremećaju, ili pak o jednostranome ili obostranom oštećenju, predstavlja dosta čest problem za liječnika praktičara, osobito onoga koji se bavi problemima vrtoglavica. Premda je vestibularna rehabilitacija za većinu poremećaja ravnoteže metoda izbora u liječenju, ona je kod nas još uvijek podosta zapostavljena i neopravdano podcijenjena. Cilj ovih Smjernica za kliničku praksu jest pomoći liječnicima i fizioterapeutima pri ujednačavanju pristupa pacijentima s jednostranim i obostranim vestibularnim oštećenjima, izbjegavajući nepotrebne varijacije u primjeni vestibularnih vježbi. One nisu udžbenik niti jedini i neupitni izvor znanja, već pružaju okvir za strategiju kod donošenja odluka, ne zamjenjujući pritom značenje kliničke prosudbe. Vestibularnim vježbama značajno se ubrzava oporavak bolesnika s vestibularnom hipofunkcijom i njihov povratak uobičajenim životnim aktivnostima. Svrha ovih Smjernica jest utvrditi koga, kada, kako i koliko dugo liječiti, kao i kako otkloniti nepotrebne varijacije u pristupu rehabilitaciji pacijenata s vestibularnom hipofunkcijom. One se zasnivaju na dokazima dobivenim metaanalizom podataka iz dostupne medicinske literature, oslanjajući se pored toga i na naše dugogodišnje iskustvo u radu s vertiginoznim pacijentima

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