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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.

Djeca koja boluju od recidivirajućih abdominalnih bolova vrlo su raznolika skupina bolesnika. Organski, psihogeni i funkcionalni poremećaji preklapaju se u većoj ili manjoj mjeri otežavajući dijagnozu i liječenje. Doprinos Rimskih kriterija je u definiranju simptoma koji pomažu pri dijagnostici funkcionalnih poremećaja i na taj način smanjuju broj nepotrebnih pretraga. Svrha ovog postupnika je pomoći liječnicima u dijagnostici i liječenju djece s recidivirajućim abdomnalnim bolovima.

Izražaj perforina u citotoksičkim T-limfocitima dojenčadi s bronhiolitisom uzrokovanim respiracijskim sincicijskim virusom*

Respiratory syncytial virus (RSV) is the leading cause of serious lower respiratory tract illness in infants and young children. Approximately 80% of childhood bronchiolitis cases and 50% of infant pneumonias are attributable to RSV infection. RSV fusion (F) protein, responsible for the merging of RSV infected with adjacent uninfected epithelial cells, also stimulates cytokine production in monocytes through CD14 and Toll-like receptor 4 (TLR4). Another surface glycoprotein, protein G, functionally mimics CX3C chemokine fractalkine, regulator of cytotoxic effector lymphocytes' tissue migration. It has been shown in mice that RSV G glycoprotein modifies cytokine and chemokine expression, sensitizes for pulmonary eosinophilia and alters pulmonary leukocyte trafficking. In our present study we analyzed the expression of perforin, IFN-?, IL-4 and fractalkine receptor CX3CR1 on cytotoxic T lymphocytes (n=11), and TLR4 expression on monocytes. The samples were obtained from peripheral blood of acutely ill infants with bronchiolitis (aged from 2 weeks to 5 months) and 4-6 weeks after the resolution of the disease. The observed effects of RSV infection on the above listed cytokines and receptor expression will be discussed.

Experiments of Nature, a term which denotes inborn errors of metabolism and/or somatic cell (e.g. lymphocytes) dysfunction, have long been a driving force in scientific achievements of contemporary medicine. To illustrate this we are presenting two children with such a disorder: a boy of 20 months, with disseminated BCG infection, and a 7 year old girl who, besides chronic mucocutaneous candidiasis, suffered from a persistent BCG infection, localized primarily at the site of vaccination. DTH reaction (PPD 3 TU, 10 TU) was negative in both patients, despite findings of acid-fast bacille in the granulomatous tissue of the skin, lymph node tissue, and bronchial aspirate. Immunologic studies in the boy showed normal to borderline lymphocyte count (30% in differential, 3600 cells per microliter) and CD3 count (47%, 1704 cells per microliter), low CD4 count (40%, 680 cells per microliter), and CD8 count (16%, 272 cells per microliter), normal CD4/CD8 ratio (2.5), and normal mitotic activity (PHA 93586 Cpm), normal leukocyte number (12100 cells per microliter), and NBT test (30% positive), normal serum immunoglobulin levels (IgG 8.51, IgA 0.70 and IgM 1.48 g/L), and normal issoagglutinin titer (anti-B positive 1:256). Findings in the girl, which have been obtained, were similar to the respective findings in the boy. As cases of SCID, CGD, CD4 deficiency and IFN-gamma receptor deficiency have been described in some children with disseminated BCG infection it could be said that, despite the most recent achievements in mollecular biological techniques, Experiments of Nature still point the way, just as AE Garrod and RA Good wrote long time ago.