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Objectives: Assuming a causal relationship between depression and the progression of CF, the aim of our study was to show that elevated symptoms of depression could be a predictor of worse lung function. Methods: 22 CF patients were recruited form our adult CF centre (13 women, 9 men, mean age 24, 18–33 years). The patients completed Patient Health Questionnaire-9 (PHQ 9) and the Generalized Anxiety Disorder 7- item scale (GAD 7). Body mass indeX (BMI) and pulmonary function testing were measured. Results: Level of anxiety and depression showed no significant association with decreased lung function. This can be explained by the fact that none of the subjects had serious anxiety and/or depression and prospective study should bed one. Conclusion: The interaction between depression and disease progression could be linked to the lack of hope, optimism and perceived self-efficacy in depressed patients, consequently inhibiting their fighting spirit against the disease and necessary adherence to treatment. Thus, depressed patients may fail to respond to standard medical treatment due to the nature of their mental health problems, and may experience a greater loss of lung function than necessary. This effect may then reinforce the negative thoughts related to their own prognosis, triggering a vicious psychosomatic cycle. In contrast, patients who are not depressed and already have severely restricted lung function might be more able to make use of resources for their daily treatment routines. This, in turn, might result in stable or even slightly improved outcomes.

Objectives: Our aim was to determine genetic, demographic and clinical characteristics of our adult cystic fibrosis (CF) patients during a five year follow up as well as trends of inpatient stays and prevalence of CF associated complications. Methods: In our adult CF centre, which is the first of its kind in Croatia, we performed a cross sectional study collecting and reviewing retrospectively medical records of CF patients from 2013 to 2017. Results: A total of 25 patients were included. 11 (44%) were male and 14 (56%) were female, median age 25 years (18 ± 33). The median age of diagnosis was 2 years (0–21). Homozygous F508del mutation was observed in 18 patients (72%). Transfer from pediatric to adult CF health care occurs at a median age of 22 years. The BMI was 21.21 kg/m2 and has not declined during a five year follow up whereas median percentage of FEV1 was 46% (16 ± 104) with a predicted average annual FEV1 decline of 1.3%. All patients had pancreatic insufficiency and bronchiectasis. 6 patients (24%) had CF related diabetes, 1 patient (4%) had CF related liver disease, 9 patients (36%) rhinosinusitis and 9 patients (36%) CF related bone disease. Chronic Pseudomonas aeruginosa infection is present in 18 patients (72%), chronic Staphylococcus aureus in 12 (48%) and 9 patients (36%) had both. Other pathogens included Stenotrophomonas Maltophilia, Burkholderia cepacia and Aspergillus fumigatus. From 2013 until 2017 there were 183 hospitalizations, average 7.32 per year with pulmonary exacerbation being the most common reason. In observed period, 4 patients (16%) received a lung transplant, while 4 patients (16%) died, 2 due to complication after lung transplantation. Conclusion: Knowing the importance of longitudinal studies, we have presented first such data on national level regarding adult CF population in Croatia. These efforts would ultimately be used to establish national registry of CF helping to improve outcomes in people with CF especially adult patients.

Objectives: Lung transplantation is a complex, high-risk, potentially lifesaving therapy for the patient with the end-stage of cystic fibrosis (CF). The decision to pursue transplantation involves comparing the likelihood of survival with and without transplantation as well as assessing the effect of wait-listing and transplantation on the patient’s quality of life. The aim of this study was to examine is there a difference in psychological status between CF patients who have not been transplanted and CF lung transplant recipients. Methods: Two patient groups, adult CF patients (N = 22) and adult CF lung transplant recipients (N = 6), participated in our study. The patients completed Patient Health Questionnaire-9 (PHQ 9) and the Generalized Anxiety Disorder 7-item scale (GAD 7). These data were compared to health status data, including pulmonary function testing and nutritional status measures. Results: In adult CF group, 52% of patients had mild to moderate anxiety and 95% mild to moderate depression. In adult CF lung transplant recipients group, 17% of subjects had minimal anxiety and 83% had minimal depression. Conclusion: In this study we find significant difference in the level of anxiety and depression between CF patients who have not been transplanted and CF lung transplant recipients. Lung transplantation in CF patient significantly contributed to the improvement of the psychological condition and quality of life.

Objectives: Living with chronic illness such as cystic fibrosis (CF) is accompanied by medical and psychological difficulties. To identify and treat depression and anxiety in CF, the CF Foundation and the European CF Society invited a panel of experts, including physicians, psychologists, psychiatrists, nurses, social workers, a pharmacist, parents and an individual with CF, to develop consensus recommendations for clinical care. The aim of this study was to assess anxiety and depression by using validated mental health survey data. Methods: CF patients were recruited from adult CF centre in Zagreb, Croatia from October 2016 to December 2017. 22 patients (13 female, 9 male, mean age 24, 18–33 years) completed the Patient Health Questionnaire (PHQ-9) and the Generalized Anxiety Disorder 7-item scale (GAD-7). Results: 82% and 59% of our patients had a normal anxiety and depression score (0–4), 41% of patients have symptoms of anxiety, and 18% of the patients have symptoms of depression (score between 5 and 9). No one had any serious anxiety and/or depression present. Conclusion: Anxiety and depression are serious complication of chronic illnesses such as cystic fibrosis. Our results highlight the need for improved early identification and management strategies for psychological illnesses in adult CF patients. Research efforts are needed to identify effective strategies for delivering anxiety and depression treatment in CF patients and to assess their risks and benefits. Furthermore, strategies that help patients maintain or even improve lung function may be an interesting topic for further studies to learn more about resources and successful coping strategies.

U posljednja dva desetljeća znatno je porasla upotreba neinvazivne mehaničke ventilacije (NIV) kao učinkovite metode zbrinjavanja akutne i kronične respiracijske insufi cijencije. U bolesnika s kroničnom respiracijskom insufi cijencijom NIV se može primijeniti u kućnim uvjetima. Najčešće bolesti koje dovode do razvoja kronične respiracijske insufi cijencije jesu: kronična opstruktivna plućna bolest, sindrom hipoventilacije u pretilih, restriktivne plućne bolesti i neuromuskularne bolesti. Primjena NIV-a poboljšava vrijednosti dišnih plinova, simptome, kvalitetu života i produžuje životni vijek bolesnika s kroničnom respiracijskom insufi cijencijom. Danas se NIV uglavnom provodi primjenom pozitivnog tlaka zraka na dišne putove bolesnika, a dvorazinska tlačna potpora najčešći je način ventilacije. Izbor adekvatnog sučelja osnova je uspješne primjene NIV-a. Kućni NIV može se indicirati i započeti u specijaliziranim centrima. Liječnik je odgovoran za postavljanje indikacija, izbor ventilatora, načina i parametara ventilacije. Ciljevi ovih smjernica jesu: pružanje informacija o tehničkim aspektima kućnog NIV-a kliničarima i određivanje indikacija, kontraindikacija te preporu čenih postavka ventilacije za svaku skupinu bolesti

First Experience with Osimertinib in Patients with Newly Developed T790M Mutation Previously Treated with EGFR – TKIs in Croatia

Osimertinib u liječenju pacijenata s T790M mutacijom.

Chronic obstructive pulmonary disease (COPD) is a leading cause of mortality and morbidity worldwide, with high and growing prevalence. Its underdiagnosis and hence under-treatment is a general feature across all countries. This is particularly true for the mild or early stages of the disease, when symptoms do not yet interfere with daily living activities and both patients and doctors are likely to underestimate the presence of the disease. A diagnosis of COPD requires spirometry in subjects with a history of exposure to known risk factors and symptoms. Postbronchodilator forced expiratory volume in 1 second (FEV1)/forced vital capacity

Metastatic cancer mimics interstitial lung disease.

Background: Erlotinib is epidermal growth factor receptor (EGFR) tyrosine-kinase inhibitor which showed efficacy and tolerability in patients with advanced non- small cell lung cancer (NSCLC), especially in group of patients which harbor activating mutations in EGFR. Aim: To determine safety and efficacy of erlotinib in patients with advanced (stage IIIB and IV) squamous NSCLC. Methods: Patients with advanced squamous NSCLC who had failed prior chemotherapy were treated with oral erlotinib 150 mg daily until disease progression or unacceptable toxicity. Data was analyzed retrospectively. Results: A total of 122 patients (107 men and 15 women, mean age 62±8 years) with advanced squamous NSCLC were enrolled in the study from 2006 to 2012 in 14 centers in throughout Croatia. More than 50% of patients were active smokers. Most of the patients were in performance status ECOG 1 and 2 (91%). Vast majority of patients was treated with erlotinib in third line setting. After cycle 2, 10% of patients had partial response (PR), and 45% of patients had stable diseases. In total, 55% of patients had disease control after cycle 2. Progression free survival (PFS) was 3.7 months in overall population. Statistically significant differences in PFS were recorded according to response to treatment ; patients with PR after two cycles had PFS of 6.2 months comparing with patients with progressive disease of 2.0 months (P

Uvod: Eisenmengerov sindrom je najrazvijeniji oblik plućne arterijske hipertenzije uzrokovane kongenitalnim srčanim greškama. Tri su skupine lijekova do sada odobrene za liječenje endotelne disfunkcije plućnog krvožilja u ovih bolesnika: prostanoidi, antagonisti endotelinskih receptora i inhibitori fosfodiseteraze-5. Metode: Retrospektivna analiza podataka iz Registra plućne hipertenzije Klinike za plućne bolesti Kliničkog bolničkog centra Zagreb. Od ukupno 33 pacijenta sa korigiranom ili nekorigiranom srčanom greškom u Registru koji prati pacijente od 2000. do 2014.god. njih 18 je liječeno specifičnim vazodilatatorima. Analizirani su demografski podatci te varijable koje utječu na procjenu funkcionalnog statusa pacijenta (NYHA status, šestominutni test hoda i saturacija arterijske krvi kisikom) prije početka liječenja i na posljednjoj kontroli. Rezultati: Od rujna 2000. do rujna 2014. god. liječeno je 18 pacijenata s verificiranim Eisenmengerovim sindromom. Svi pacijenti stariji su od 18 godina i 13/18 (72 %) su ženskog spola. Prosječna dob u vrijeme početka liječenja specifičnim vazodilatatorima je 34, 3 godine, a trajanje istog u prosjeku je 4, 4 godine. Pacijenti su liječeni bosentanom (11 pacijenata), sildenafilom (12 pacijenata), iloprostom (6 pacijenata) te kombinacijama navedenih, a jedan pacijent liječen je ambrisentanom. U ovih 18 pacijenata uključeno je 10 pacijenata sa VSD-om (od kojih jedan korigirani), četiri s ASD-om (1 korigirani), jedan pacijent s transpozicijom velikih krvnih žila, dva pacijenta s arteriovenskim kanalom i Downovim sindromom te jedan pacijent s aortopulmonalnim prozorom. Funkcijski status pacijenata značajno se poboljšao nakon uključivanja lijeka (NYHA 2, 2±0, 5 vs. NYHA 2, 8±0, 7, P=0.032). Saturacija arterijske krvi prije i nakon uvođenja lijekova nije se značajno mijenjala (satO2 87±8 vs.87±9%, P=0.941), kao ni nalaz šestminutnog testa hoda (hodna pruga 323±93 vs. 353±69 m, P=0.532). Zaključak: Liječenje plućne hipertenzije specifičnim vazodilatorima u skupini naših pacijenata ukazuje na dugoročni benefit s obzirom na poboljšanje funkcijskog statusa pacijenata. Nije uočen pad saturacije kisikom u ispitivanoj populaciji bolesnika.

Background: Lung cancer is the most common tumor in men and the second most common tumor in women according to the latest data available from the Croatian National Cancer Registry. Approximately 20% of all lung cancers are categorized as small cell lung cancer (SCLC). Topotecan is recommended as second-line chemotherapy in treatment of SCLC. Topotecan can be administrated orally with the same effectiveness as parenteral application. Methods: The aim of this study was to determine toxicity of oral topotecan and to establish the frequency of drug related admissions. Results: The study included 48 patients, 13 woman and 35 men with SCLC, ranging from 45 to 72 years, with the mean age being of 60.5 years, that referred to University Hospital Center Zagreb, from January 2012 to June 2014. Included patients had ECOG performance status of 0 or 1. Topotecan was administrated every 21 day, at the dose of 2.3 mg/m2 /day, during 5 days. Average number of courses received was 2.7. Of all included patients 18 of them (37.5%) were admitted to hospital because of adverse events related to topotecan administration. The majority of patient hospitalizations (11 patients, 22.9%) was due to febrile neutropenia. Other reasons for hospitalization were severe diarrhea in 4 patients (8.4%), pneumonia in one patient (2.1%), severe pancytopenia in one patient (2.1%) and severe electrolyte imbalance in one patient (2.1%). Anemia that needed transfusion occurred in 16 patients (33.3%), with grade 3 or 4 anemia occurring in 13 of them (27.1%). Grade 3 or 4 thrombocytpenia occurred in six patients (12.6%) and all of them were treated with transfusions. Sixteen patients (33.3%) received granulocyte colony stimulating factor for treatment of neutropenia. Grade 3 or 4 neutropenia occurred in 14 patients (29.1%). Of other, non-hematologic adverse effects the most serious was grade 3 or 4 diarrhea that occurred in 4 patients (8.4%). Conclusions: Although the route of administration makes oral topotecan a more convenient treatment method, our results show high rates of myelotoxicity during therapy, what is in concordance with the majority of previous studies.

Toxicity of oral topotecan in second-line chemotherapy of SCLC.

Extracporporeal carbon dioxide removal using the pumpless extracorporeal lung assist device in patient with pulmonary exacerbation of cystic fibrosis.

A case series of 6 patients with partial anomalous pulmonary venous connection (PAPVC).

Cerebral sarcoidosis - an additional cause of OSA in a patient with metabolic and endocrinological polymorbidity?

Lung transplantation in patient with pulmonary alveolar microlithiasis - a case report.