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

Expression of matrix metalloproteinase-9 in patients with squamous cell carcinoma of the larynx.

Aim of this study was to investigate the correlation of matrix metalloproteinase-9 (MMP-9) expression with histopathologic and clinical characteristics of laryngeal squamous cell carcinoma, and to assess the role of MMP-9 expression in patient survival. Study included 196 patients with squamous cell carcinoma of the larynx treated at ENT Department, Split University Hospital Centre, from January 1, 2000 till December 31, 2009. The level of MMP-9 expression showed a statistically significant correlation (p < 0.001) with the disease histopathologic grade, stage, metastatic potential, recurrence potential, and survival. Kaplan-Meier curve yielded a statistically significant survival difference (p < 0.001) among the three patient groups with different levels of MMP-9 expression. The survival curve clearly showed the MMP-9 expression as a predictor of survival to be significantly (p < 0.001) associated with survival. In this study, MMP-9 expression as a biological marker showed a potential predictive value in laryngeal squamous cell carcinoma.

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.

Efficacy and safety of inferior turbinate coblation-channeling in the treatment of nasal obstructions.

The aim of this study was to evaluate the safety and effectiveness of coblation-channeling in the treatment of inferior turbinate hypertrophy. The study was conducted in the Department of ENT Head and Neck Surgery, Split University Hospital Center, Split, Croatia. Fifty-two patients with inferior turbinate hypertrophy who were refractory to medical therapy were evaluated for coblation. The procedures were performed under local anesthesia using an ArthroCare ReFlexUltra 45 wand; three submucosal channels were made per turbinate. Clinical examinations, a questionnaire on individual nasal symptoms (hyposmia, nasal drainage and post-nasal drip), a 10-cm visual analog scale (VAS) grading general nasal obstructions, and rhinomanometry before and 8 weeks after the treatment were administered to assess treatment outcomes. No adverse effects were encountered. Nasal breathing was significantly improved in all patients, decreasing the VAS from a median of 7 (range 2-9) to 1 (range 0-3) (p < 0.001). Total nasal resistance decreased from 0.44 Pa +/- 0.50 to 0.24 Pa +/- 0.11 (p = 0.005). Improvement was statistically significant for all three symptoms (hyposmia [p = 0.005], nasal drainage [p = 0.003] and post-nasal drip [p < 0.001]). In this paper, we demonstrate that coblation-channeling of the hypertrophic inferior turbinate is an effective and safe way to reduce nasal obstruction symptoms.

Succinylcholine use in adult anesthesia - A multinational questionnaire survey.

There are no definitive evidence based standards regarding use of succinylcholine (SCh) for anesthesia induction. However, there is a global trend toward eliminating SCh not only in elective, but also in emergency surgery in adults. The aim of the study was to survey the use of SCh in adult elective and emergency anesthesia practice in several European countries and the United States by questionnaire. One hundred and seventy anesthesiologists out of 201 possible, from six institutions in five countries (Croatia, Bosnia and Herzegovina, Hungary, United Kingdom, and the United States) anonymously completed the questionnaire about their use of SCh. The questionnaire was structured to assess the respondents': frequency of use of SCh in adult surgery (elective and emergency), reasons for use or rejection of SCh, positive and negative attributes of SCh, and observed side effects in their practice. Differences in use were tested using X2-test when appropriate. There was a significant difference in the use of SCh between countries for elective surgery with the lower use in UK and Hungary (chi2=45.8, p <0.001). One hundred and seventeen (69%) use it regularly. In emergency surgery 165 (97%) anesthesiologists use it without any significant difference among countries (chi2=2.13, p<0.711). The top indications for SCh use were anticipated difficult intubation/ventilation (74%), caesarean section (54%), and obesity and/or hiatus hernia (49%). The top reasons against SCh use were adequate substitutes (87%), fear of arrhythmias (45%), and anaphylaxis (19%). The most desirable reported drug features were: rapid onset (88%), short duration (64%), and effective relaxation (61%). Forty-six per cent of the surveyed anesthesiologists stated they had never experienced a complication with its use. The most frequently reported side effects were myalgias (47%), bradycardias (42%), and prolonged blockade (39%). Allergic reactions were reported by 13%, and asystole by 12% of physicians. From our survey it is possible to conclude that succinylcholine is still regularly used, at least by surveyed anesthesiologists in Europe and USA, in adult anesthesia practice, especially in elective surgery for which it may be least suited. This reflects the discrepancies between the international guidelines for the use of SCh and the clinical practice of many anesthesiologists in different countries. The regional differences in SCh usage may be considered through anesthesia cultures and practice variations depending on country.

[Succinylcholine use by anesthesiologists in Croatia--is it really abandoned?]. / Uporaba sukcinil-kolina medu hrvatskim anesteziolozima--je li taj lijek zaista odbacen?

The aim was to establish the prevalence of succinylcholine use among Croatian anesthesiologists in adult elective and emergency surgery, as well as in pediatric surgery, regarding gender, position, working place, and working experience of physicians. The anesthesiologists were expected to express their personal opinions regarding the drug, as well as experienced side effects in their own clinical practice. A total of 125 anesthesiologists (out of 590 in Croatia) from both university and county hospitals in Croatia anonymously filled out the questionnaire regarding the use of succinylcholine (Appendix 1). The questionnaire was structured to assess the use of succinylcholine in adult elective and emergency surgery, and in pediatric anesthesia, to obtain the reasons for the preference or rejection of succinylcholine, and information about observed side effects. The differences in use regarding gender, position, working place, and working experience were tested using chi-squared test and Fisher's exact test. p < 0.05 was considered significant. Vast majority (approximately 70%) of anesthesiologists in Croatia still use succinylcholine. The percentages of anesthesiologists that never use succinylcholine in adult elective, adult emergency and pediatric surgery were 20%, 6%, and 31%, respectively. There were no significant differences in the use of succinylcholine regarding position, working place, and working experience, but male anesthesiologists used it less frequently in pediatric anesthesia compared with their female colleagues (chi2 = 5.08; p = 0.02). Forty-two per cent never experienced a complication from the drug use. The most frequently reported side effects were bradycardias (67%) and myalgias (54%), followed by prolonged blockade (33%), and allergy (33%). Asystole was reported by 10% of the respondents. In conclusion, succinylcholine is still widely used by anesthesiologists in Croatia. The majority of surveyed physicians were aware of its possible dangerous adverse effects, but still use it in certain situations. Therefore, indications and contraindications for its use deserve expert consensus guidelines based on the available scientific evidence.

Postoperative morbidity and histopathologic characteristics of tonsillar tissue following coblation tonsillectomy in children: a prospective randomized single-blind study.

The aim of this prospective randomized single blind study was to determine the depth of thermal damage to tonsillar tissue due to coblation, and to compare it with thermal damage to tonsillar tissue following conventional tonsillectomy; to correlate the depth of thermal damage to tonsillar tissue with the parameters of postoperative morbidity, to compare intraoperative blood loss, postoperative pain severity, time to resuming normal physical activity, and incidence of postoperative bleeding between two groups of tonsillectomized children aged up to 16 years. 72 children aged 3-16 years scheduled for tonsillectomy randomly assigned into two groups submitted either to conventional tonsillectomy with bipolar diathermy coagulation or to coblation tonsillectomy, with a 14-day follow up. Statistically significant differences were observed in the depth of thermal damage to tonsillar tissue (p < 0.001), intraoperative blood loss (p < 0.004), in postoperative pain severity (p < 0.05) and in time to resuming normal physical activity between the two groups (p < 0.001). There was no case of reactionary or secondary bleeding in either group. In this paper for the first time we have correlated postoperative morbidity and thermal tissue damage: less thermal damage is associated with less postoperative morbidity.

[Surgery as a treatment modality for sleep disordered breathing]. / Kirursko lijecenje poremecaja disanja tijekom spavanja.

The main characteristic of all types of sleep disordered breathing (SDB) is partial or complete obstruction in the upper airway. Patency of the pharyngeal airway is maintained by two opposing forces: negative intraluminal pressure and the activity of the upper airway musculature. Different anatomical abnormalities can disrupt this delicate balance with resultant compromise of the upper airway. Thus, the main goal of the medical and surgical therapy is to alleviate this obstruction and increase airway patency. If multilevel obstruction is noted, multilevel-surgery is needed. It can be done as one-stage or multi-stage procedure which depends on the current patient state and surgical skill. Technical developments and their implementation in everyday surgical practice made some of the surgical methods for SDB treatment more convinient for the patient. Surgery can be performed in ambulatory facility and the procedures are repeatable with no adverse effects which significantly decreases the postoperative morbidity. Almost all surgical techniques for SDB treatment have good results in properly selected patients. That is why the key for the successful SDB treatment is a proper multidisciplinary diagnostic algorithm and a team work.

[Adenotonsillectomy as a treatment modality for sleep disordered breathing in children: case report]. / Adenotonzilektomija u lijecenju poremecaja disanja tijekom spavanja u djecjoj dobi: prikaz bolesnika.

Children with sleep disordered breathing (SDB) can present with a range of symptoms, from simple snoring to obstructive sleep apnea (OSA) with secondary growth impairment, neurocognitive defects and sometimes cardiovascular sequelae that rearely lead to death. The most common cause of SDB in children is adenoid and tonsillar hypertrophy. The most common treatment for children with SDB not caused by malformations or illness is surgery, in the first place--adenotonsillectomy. We are presenting the case of the 5-year-old boy with OSA in whom the recent diagnostic and therapeutic approach was performed, along with treatment outcome following the surgery and after the 6-month follow-up.

[Sleep disordered breathing]. / Poremecaji disanja tijekom spavanja.

Sleep apnea is a breathing disorder characterized by cessation of breathing during sleep, oxygen desaturation and awakenings during night. There are several types of breathing disorders during sleep. Obstructive sleep apnea (OSA) is also characterized by snoring and excessive daytime sleepiness. Central sleep apnea (CSA) is less common and characterized by reduced respiratory drive from the central nervous system. Upper airway resistance syndrome (UARS) is characterized by excessive daytime sleepiness, absence ofapneas, hypopneas and lack of significant oxygen desaturation. The consequences of the abnormal breathing during sleep include excessive daytime sleepiness, development of arterial hypertension, ischemic cardiac disease, neurocognitive dysfunction, glaucomic optico-neuropathy, metabolic dysfunction. The early diagnosis requires detailed anamnestic data, standardized questionnaires for detection of sleep disordered breathing and whole-night polysomnography in the sleep laboratory. Obstructive sleep apnea can be treated with continuous positive airway pressure (CPAP), oral appliances, and surgery (e.g., uvulopalatopharyngoplasty, UPPP). Early diagnosis of OSA enables early treatment, improvement of its symptoms and eventually reduces development of co-morbidities.

Progression of optic neuritis to multiple sclerosis in the County of Split-Dalmatia, Croatia.

The aim of this study was to determine the incidence of monosymptomatic optic neuritis (MON) and progression of MON to multiple sclerosis (MS) from the Mediterranean region of southern Europe in the County of Split-Dalmatia, Croatia during the 11 years period from 1991 to 2001. This study was made retrospectively on the 87 cases (59 female, aged 25.9 +/- 11.3 and 28 male aged 29.9 +/- 9.2) of MON, which were treated at the Department of Ophthalmology and Department of Neurology, Split, University Hospital, from January 1991 to December 2001. In each case the diagnosis was confirmed by a chart review and cases were ascribed to the data of admittance at hospital. The annual incidence of MON was 1.9 per 100,000 (95% CI, 0.4-3.5). The incidence among males was 1.2 (95% CI, 0-2.9) cases / 100,000 per year and 2.5 (95% CI, 0.1-4.9) among females. A significant seasonal variations in the incidence of MON was not found (chi2 = 6.81, p = 0.08). MS developed in 20 of 87 patients (22.9%) and median time was 25 (SE 8) months, (95% CI, 9-41) after the MON onset. After two years 12.6% of patients with MON developed MS, 20.6% after 5 years and 22.9% after 10 years. MS was slightly but not significantly more frequent in women than in men (chi2 = 0.72, p = 0.3). In conclusion, the progression of MON to MS in the County of Split-Dalmatia, Croatia was at a relatively moderate frequency.

Prevalence and risk factors for developing oral allergy syndrome in adult patients with seasonal allergic rhinitis.

The aim of this study was to assess the prevalence of oral allergy syndrome (OAS) in patients with seasonal allergic rhinitis (SAR) and the possible risk factors for its development. This cross-sectional study was conducted in primary care offices in the Split-Dalmatia County during the period from March 1 to September 30, 2012. Data sources were medical history with confirmation of SAR (positive skin-prick test to seasonal inhalant allergens: grass, tree and weed pollens), anthropometric patient data (age, sex, weight and height), and a questionnaire in which patients evaluated their nasal and ocular symptoms, comorbidities and lifestyle. The χ2-test, Pearson χ2-test, Spearman's rho correlation coefficient and Kolmogorov-Smirnov test were used on statistical analysis. The prevalence of OAS was 45.7%. The risk factors for OAS development were diabetes (p < 0.001), severity of nasal symptoms (p < 0.05) and severity of ocular symptoms (p < 0.001). In conclusion, the prevalence of OAS in the Split-Dalmatia County is very high. The risks factors for OAS in patients with SAR are diabetes and severity of nasal and ocular symptoms.

[Gas gangrene and hyperbaric oxygenation--a lesson forgotten or never learned?]. / Plinska gangrena i hiperbaricna oksigenacija--zaboravljena ili nikad naucena lekcija?

Gas gangrene develops in devitalized hypoxic tissue. It spreads rapidly under strong influence of enzymes produced by the causing bacteria and often results in fatal outcome. It is of utmost importance to stop toxin production as soon as possible, which is most effectively achieved by early application of hyperbaric oxygenation (HBO2), as the first measure in a "trident" (HBO2, antibiotics, surgical measures). The paper reviews the most important data from microbiology, pathophysiology and epidemiology of gas gangrene, current clinical practice, scientific basis for application of HBO2 in the treatment of this disease, and data from the archives of the Naval Medical Institute of the Croatian Navy in Split (NMI). At the NMI, in the period from 1982 to 2000, HBO2 was administered in the treatment of 21 patients with gas gangrene, of average age 41.6 +/- 16.3 years. The average treatment consisted of 6.8 +/- 3.8 sessions. 13 (76%) patients were cured.

Is the systemic inflammatory reaction to surgery responsible for post-operative pain after tonsillectomy, and is it "technique-related"?

AIMS: Investigate the influence of operative technique on post-operative morbidity and the systemic inflammatory response after tonsillectomy. In addition, our aim was to compare the systemic inflammatory reaction, post-operative pain severity, and required time before the resumption of normal physical activity between two groups of tonsillectomized children and to correlate characteristics of the systemic inflammatory reaction to post-operative morbidity. PARTICIPANTS AND METHODS: This prospective, randomized, and single-blind study included 100 children between the ages of 3-16 years and who were scheduled for a tonsillectomy at our department for chronic tonsillitis and/or respiratory obstruction. The children were randomly assigned into one of two groups: either a conventional tonsillectomy with bipolar diathermy coagulation or a radiofrequency tonsillectomy procedure; both groups had a 14-day follow-up. We investigated the severity and duration of postoperative pain (based on the use of analgesics during the postoperative period), the postoperative day that they resumed normal physical activity, and the rate of postoperative hemorrhage. In order to assess the systemic inflammatory response, serum C-reactive protein (CRP) levels were measured before the surgery and seven days after the procedure. RESULTS: After the surgery CRP levels increased to a statistically significant level (t = -4.7; p < 0.001) in conventional tonsillectomy group. There was a statistically significant correlation between increased blood CRP levels after the surgery and the level of post-operative analgesic consumption, which was based on an increased number of analgesic applications (r = 0.28; p < 0.01) and a greater number of days in which analgesics were consumed (r = 0.26; p < 0.01). There was also a correlation between increased blood CRP levels and a longer required time to resume normal physical activities (r = 0.30; p < 0.01). CONCLUSION: Post-operative morbidity after tonsillectomy appears to depend on the systemic inflammatory response to surgery. This response is "technique-related," wherein a less-aggressive surgical technique produces a weaker post-operative inflammatory response and less post-operative morbidity.

Hyperbaric oxygen in the treatment of sudden hearing loss.

Treatment of idiopathic sudden hearing loss (SHL) is still a great problem for the physicians, due to the still unexplained etiopathogenesis of the disease. This retrospective study includes 115 patients with idiopathic SHL and compares two therapy regimens. Of the total number of patients, 51 had been primarily treated by hyperbaric oxygen (HBO) at 2.8 bar. The remaining 64 patients had been treated by pentoxifylline infusions in increasing and decreasing doses. Hearing thresholds were ascertained at the frequencies of 250, 500, 1,000, 2,000 and 4,000 Hz using pure-tone audiometry. The results after treatment showed a statistically significant improvement of hearing by 46.35 (SD +/-18.58) dB hearing level (HL) in the group of patients treated with HBO, compared with an improvement of hearing by 21.48 dB HL (SD +/-13.5) in the group of patients treated with pentoxifylline infusions (p < 0.001). Furthermore, hearing recovered to physiological values in 24 of 51 (47.1%) HBO-treated patients, compared to only 4 of 64 (6.2%) of the pentoxifylline-treated patients. Furthermore, after pentoxifylline treatment only 8/64 (12.5%) patients showed partial recovery, whereas after HBO therapy 21/51 (41.2%) patients improved up to a mild persistent hearing loss. This indicates strong evidence that HBO therapy is more effective in the treatment of idiopathic SHL than conventional rheological treatment. However, more prospective clinical trials should confirm the efficiency of HBO therapy and lead to its wider application.

Primary mucosal melanoma of the eustachian tube.

Melanomas of the upper airway tract constitute around 1% of the total melanomas of the head and neck. They are usually more advanced in depth of invasion or size at the time of diagnosis than melanomas of the skin. The case of a 74-year-old Caucasian female with primary malignant melanoma of the left eustachian tube and bilateral neck metastasis is presented. The procedure from the diagnosis of neck metastasis to the location of the primary melanoma is described. The management of the malignant melanoma neck metastasis should also involve endoscopic examination of the upper aerodigestive tract, since the primary lesion can occur there.