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Background High-resolution computed tomography (HRCT) is the gold standard for the evaluation of cystic fibrosis (CF) lung disease; however, lung ultrasound (LUS) is being increasingly used for the assessment of lung in these patients due to its lower cost, availability, and lack of irradiation. We aimed to determine the diagnostic performance of LUS for the evaluation of CF pulmonary exacerbation. Methods This cross-sectional study included patients with CF pulmonary exacerbation admitted to Masih Daneshvari Hospital, Tehran, Iran, from March 21, 2020 to March 20, 2021. Age, gender, and body mass index (BMI) of the patients were recorded. All patients underwent chest X-ray (CXR), HRCT, and LUS on admission. Pleural thickening, atelectasis, air bronchogram, B-line, and consolidation were noted in LUS and then compared with the corresponding findings in CXR and HRCT. Taking HRCT findings as reference, sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and diagnostic accuracy (DA) of LUS and CXR for the detection of each pulmonary abnormality were determined. Results Of the 30 patients included in this study, with a mean age of 19.62 ± 5.53 years, 14 (46.7%) were male. Of the 15 patients aged 2–20 years, BMI was below the 5th percentile in 10 (66.7%), within the 5–10 percentiles in 1 (6.7%), 10–25 percentiles in 3 (20%), and 25-50 percentiles in 1 (6.7%). The mean BMI for 15 patients > 20 years was 18.03 ± 2.53 kg/m2. LUS had better diagnostic performance compared to CXR for the detection of air bronchogram, consolidation, and pleural thickening (area under the receiver operating characteristic curve [AUROC]: 0.966 vs. 0.483, 0.900 vs. 0.575, and 0.656 vs. 0.531, respectively). Also, LUS was 100% and 96.7% specific for the diagnosis of pleural effusion and atelectasis, respectively. Conclusions LUS appears to be superior to CXR and comparable with HRCT for the evaluation of CF pulmonary exacerbation, especially in terms of air bronchogram and consolidation detection. LUS can be used to lengthen the HRCT evaluation intervals in this regard or utilized along with HRCT for better evaluation of CF pulmonary exacerbation.

Background Allergic bronchopulmonary aspergillosis (ABPA) has been reported in various degrees among patients with persistent allergic asthma (PAA). Currently, there is no gold standard approach for diagnosis of ABPA. Objectives In the current study, we aimed the evaluation of three different mainly used algorithms as Rosenberg & Patterson (A), ISHAM Working Group (B) and Greenberger (C) for diagnosis of ABPA in 200 patients with underlying PAA. Methods All patients were evaluated using Aspergillus skin prick test (SPTAf), Aspergillus-specific IgE (sIgEAf) and IgG (sIgGAf), total IgE (tIgE), pulmonary function tests, radiological findings and peripheral blood eosinophil count. The prevalence rate of ABPA in PAA patients was estimated by three diagnostic criteria. We used Latent Class Analysis for the evaluation of different diagnostic parameters in different applied ABPA diagnostic algorithms. Results Aspergillus sensitisation was observed in 30 (15.0%) patients. According to algorithms A, B and C, nine (4.5%), six (3.0%) and 11 (5.5%) of patients were diagnosed with ABPA, respectively. The sensitivity and specificity of criteria B and C were (55.6% and 99.5%) and (100.0% and 98.9%) respectively. sIgEAf and sIgGAf showed the high significant sensitivity. The performance of algorithm A, in terms of sensitivity and specificity, was somewhat better than algorithm B. Conclusion Our study demonstrated that the sensitivity of different diagnostic algorithms could change the prevalence rate of ABPA. We also found that all of three criteria resulted an adequate specificity for ABPA diagnosis. A consensus patterns combining elements of all three criteria may warrant a better diagnostic algorithm.

Background: The provision of facilities to achieve better living conditions for patients with cystic fibrosis (CF) is of particular importance. Smoking is one of the known factors that aggravate CF. However, the effects of exposure to secondhand smoke (consumed by parents) if not consumed by the individual him/herself are not very evident. Objectives: This study aimed to evaluate the quality of life in patients with CF based on a history of smoking in parents. Methods: This cross-sectional analytical study was conducted on 100 patients with CF referred to the National Research Institute of Tuberculosis and Lung Diseases in Tehran, Iran, who were admitted in 2019 - 2021, using convenience sampling. After measuring height and weight, body mass index (BMI) was calculated, and the relationship between these factors (including a family history of smoking, pulmonary function tests, and BMI) with the quality of life of these patients was assessed. The data collection tool was a questionnaire. Data analysis was performed using SPSS software (version 20). The independent t-test and Pearson correlation test were used in this study, with a significance level of 0.05. Results: There was no statistically significant difference in the quality of life of the patients in the two groups whose parents were smokers and nonsmokers; however, physical and social functions and disease severity were lower in the smoking family group than in the other group (P > 0.05). In this study, there was no significant relationship between the quality of life of patients with CF in physical, emotional, and social functions and disease severity with a family history of smoking (P > 0.05); nevertheless, the quality of life was lower in patients whose fathers were smokers. In the present study, there was no significant relationship between patients’ quality of life in the smoking family group with the number of daily consumption of tobacco (cigarettes) in those around and the duration of family smoking (year). Conclusions: The findings of this study showed that smoking reduces the quality of life of patients with CF. Therefore, the need for the attention of parents and relatives of patients with CF to increase patients’ quality of life is seriously felt. There was no statistically significant relationship between smoking in patients with CF and reduced quality of life in this study; however, it is suggested to perform this study on a larger sample and in different cities in patients with CF and compare the results.

Background: Patients with chronic diseases and their caregivers are at risk for mental disorders, such as depression and anxiety. Objectives: This study aimed to investigate the prevalence of depression and anxiety symptoms among cystic fibrosis (CF) patients and their parent caregivers in Iran. Methods: This descriptive-analytical cross-sectional study was carried out on 96 participants, including 60 patients and 36 parents. The Hospital Anxiety and Depression Scale was used to measure the depression and anxiety in this study. The research site was the Pediatric Respiratory Diseases Research Center of the National Research Institute of Tuberculosis and Lung Diseases in Tehran, Iran. SPSS software (version 22) was used to examine and analyze the raw data. Results: The prevalence of anxiety in patients with a mean incidence of 12.17% was higher than that in parents with a mean incidence of 11.81%. Moreover, the prevalence of depression in patients and parents (10.58%) was almost similar. In the patients’ group, there was a direct and significant relationship between the duration of diagnosis with depression and anxiety; nevertheless, there was an inverse and significant relationship between the patient’s age and anxiety. In the parents’ group, there was a significant inverse association between age and depression, as well as between the duration of diagnosis and anxiety. However, there was no significant relationship between anxiety and depression, neither in the parents’ group nor in the patients’ group. Conclusions: The high prevalence of depression and anxiety symptoms among CF patients and their parents indicates the poor mental state of these individuals, which requires planning and implementation of psychological interventions for this group.

Introduction: Immunodeficient children are at a high risk of disseminated Bacillus Calmette-Guérin [BCG] infection. We assessed the literature on clinical manifestations of BCGosis in children with specific primary immunodeficiencies. Material and methods: We conducted a systematic review of clinical practice articles by searching Medline, PubMed, Embase, Scopus, Web of Science and Google Scholar from their inception to date. Results: Thirty-seven articles were included regarding BCG vaccination and its dissemination in children with primary immunodeficiencies. Articles on dissemination after intravesicular BCG were excluded from the study. Conclusions: Since disseminated BCG vaccination may be the first manifestation of a primary immunodeficiency disease, a comprehensive search for immunological defects in children developing these problems after BCG vaccination seems rational.

Background Cystic fibrosis (CF) is the most prevalent lethal chronic genetic and multivariate disease, widespread in Iranian population. The aim of this study was to evaluate the condition of patients with CF from various dimensions in the last decades in Iran. Materials and Methods In this cross-sectional study the data were collected from disease registrydatabase of CF patients in Pediatric Respiratory Disease Research Center in Masih Daneshvari, Tehran, Iran. The patients were divided into female and male.The data were collected based on province, birthplace province, marital status, and degree of education, parental consanguinity ratio, age, and age of onset of symptoms, age of onset of diagnosis and body mass index (BMI). Results Majority of patients (n=169, 59.3%) were male, born in Tehran province (n=81, 28.4%), and live in Tehran province (n=102, 35.8%). Majority of parents (61.1%, n=174) had consanguineous relation and the rest (n=94, 33%) did not have any consanguineous relation. Results show that mean of age, age of diagnosis, mean for expression of first symptoms and mean of time interval between the onset of symptoms and the diagnosis of the disease were 14.52, 2.51, 6.57, and 4.07 years, respectively. Results showed that high percentage of patients (n=191, 67%) had lower BMI (25). Correlation coefficient also showed positive relation between start age of symptoms and diagnosis age of disease. Conclusion In Iran, CF is a multisystem disease and age for its expression is different. Since mean for time interval between the onset of symptoms and the diagnosis of the disease

Cystic fibrosis (CF) is a common progressive genetic disorder among children which involves lungs, kidneys, intestine and liver. Apart from the significance of genetic factors, various environmental factors particularly bone mineral density are directly associated with CF. Remarkably, bone disease is appeared as a routine and common trait in long term CF survivors which implies that environmental parameters including calcium and vitamin D intake as well as drug inducement are the most important risk factors causing low bone mineral density. Conspicuously, absolute and notable treatment of cystic fibrosis associated to bone disorder must involve investigating the risk factors including the reduced intake of certain vitamins and minerals due to pancreatic inadequacy, modified hormone production, severe and chronic lung infection with increased ranges of bone function cytokines for a weak bone health situation. In this review, focus is on these considerable factors alongside the genetic factors in cystic fibrosis.

Background: Cystic Fibrosis (CF) is a hereditary autosomal recessive genetic disorder that can affect many organs including the lungs and the digestive system. We aimed to assess the effects of coenzyme Q10 level on clinical parameters in cystic fibrosis patients. Materials and Methods This cross-sectional study was performed on 40 patients (13 females and 19 males) with cystic fibrosis who were admitted in Masih Daneshvari Hospital in Tehran- Iran, in 2017. A researcher-made questionnaire was distributed among them, and then the height, weight and arm circumference of the patients were measured and also their serum Q10 levels were evaluated. Then, the pulmonary function was evaluated using a 6-minute respiration test and a spirometry test. Finally, their total scores were calculated based on Shwachman score. Results: The age range of participants was from 6 to 27 years old. Anthropometric indices such as weight (p=0.02, r=-0.408), age (p=0.016, r=-0.422), height (p=0.002, r=-0.520), birth weight (p=0.0113, R=-0.286), and height at birth (p=0.037, r=-0.37) had a significant negative relationship with coenzyme Q10. There was relationship between anthropometric indices and spirometry tests, including the association of FEV1 (act) with weight (p=0.00), and arm circumference (p=0.00) which were determined. Conclusion: According to the results, there was no direct relationship between muscle mass and Q10. Besides, the mean age of Q10 was greater than that of in age group of 9-16 years old, which can be considered for nutrition or childhood reserves for coenzyme Q10 production, which is decreased due to increasing age and lack of intake or loss of body power for the production of coenzyme Q10.

Background and aims Respiratory infections are expressed very soon in the life in humoral immunodeficiencies and often lead to chronic irreversible complications such as bronchiectasis and chronic airflow limitation. This study was conducted to evaluate the pulmonary complications of predominantly antibody immunodeficiencies to show the benefits of timely diagnosis and appropriate therapy. Patients and methods The information of 48 patients involved with a type of predominantly antibody immunodeficiencies, including sex, type of primary immunodeficiency, age at the onset of symptoms, age at diagnosis, recurrent infections, respiratory symptoms, and pulmonary radiological and functional abnormalities were recorded and analyzed. Results In 48 patients evaluated, the mean age at diagnosis was 25.63 years. The mean diagnostic delay was estimated to be 13.62 years. The most recurring clinical manifestations, sinusitis (69.6%), otitis (43.5%), and recurrent pneumonia were the cause of frequent admissions in 68.8% of these patients. Bronchiectasis was frequently found (58.3%) in these patients mostly involving the middle and lower lobes (48.8% and 41.5%, respectively). Conclusions Respiratory complications, infectious or non-infectious, determine the prognosis of the disease in patients with predominantly antibody immunodeficiencies. Timely diagnosis and appropriate management may improve life expectancy and the quality of life in these patients.

Background and Purpose: Aspergillus fumigatus as a ubiquitous fungus can be found in the respiratory tract of the asthmatic and healthy people. The inhalation of Aspergillus spores leads to an immune response in individuals with asthma and results in the aggravation of the clinical symptoms. The present study aimed to investigate the prevalence of specific immunoglobulin E and G (IgE and IgG) against A.fumigatus in asthmatic patients.Materials and Methods: This study was conducted on 200 consecutive patients with moderate to severe asthma referring to Masih Daneshvari hospital Tehran, Iran, from January 2016 to February 2018. Skin prick test (SPT) was performed in all subjects with Aspergillus allergens. Moreover, all patients underwent specific IgE testing for Aspergillus using Hycor method. Enzyme immune assay was applied to measure total IgE and Aspergillus-specific IgG.Results: According to the results, the mean age of the patients was 45.8 years (age range: 18-78 years). The mean levels of total IgE and Aspergillus specific IgE in asthmatic patients were obtained as 316.3 (range: 6-1300 IU/ml) and 1.5 (range: 0.1-61.3 IU/ml), respectively. Out of 200 patients, 27 (13.5%), 65 (32.5%), 22 (11.0%), and 86 (43.0%) cases had positive Aspergillus SPT, total IgE of > 417 IU/ml, Aspergillus-specific IgE, and IgG, respectively. The level of these variables in patients with severe asthma were 16 (16.5%), 36 (37.1%), 15 (15.5%), and 46 (47.4%), respectively.Conclusion: As the findings indicated, reactivity to Aspergillus is a remarkable phenomenon in asthmatic patients. It is also emphasised that the climatic condition may affect the positive rate of hypersensitivity to Aspergillus.

Background: The aim of the present study was to investigate the added value of F-18 fludeoxyglucose (FDG) positron-emission tomography (PET)/computed tomography (CT) compared with conventional imaging modalities for the evaluation of locoregional and distant sites of recurrence in breast cancer patients. Methods: From May 2013 to September 2016, 109 patients with suspected recurrent breast cancer who underwent conventional imaging and F-18 FDG PET/CT with an interval of 6 weeks were consecutively enrolled (mean age: 52.66 years; range: 29?79). Histopathologic results and clinical follow up based on the gold-standard imaging modality or serial imaging were considered as the reference for verification of F-18 FDG PET/CT findings. Results: Of 109 patients, 81 were found to have at least one site of recurrence (74.31%). Local recurrence was correctly identified in 32/32 patients following PET/CT, which was higher than that on conventional imaging (20/32, 62.5%). PET/CT detected 27 additional nodal metastases compared with conventional imaging (59 vs. 32, 45.76%), most frequently in the hilar/mediastinal region (n=27), followed by the supraclavicular lymph nodes (n=20, 62.5%), internal mammary lymph nodes (n=6, 18.77%), and axillary basin (n=6, 18.77%). Additional sites of distant metastasis were identified in 41 patients (37.61%) following F-18 FDG PET/CT imaging, 48.78% of which were localized in the skeletal system (n=20), 21.95% in the liver (n=9), 12.19% in the lungs (n=5), 12.19% in the brain (n=5), and 4.87% in the adrenal glands (n=2). Conclusion: F-18 FDG PET/CT serves as a useful supplement to conventional imaging techniques by identifying additional sites of disease recurrence in patients with breast cancer, which may change the preferred treatment strategy, particularly in regions that are not routinely evaluated by conventional imaging.

Background: Cystic Fibrosis (CF) is the most fatal genetic disorder among white populations. It is a multi-system disease with various symptoms, which causes many different complications. miRNAs are a class of endogenous small non-coding RNAs of 19-22 nt that regulate mRNAs post-transcriptionally. Different types of miRNAs, including miR-155, are expressed in CF lung epithelial cells. The elevated expression level of miR-155 contributes to the pro-inflammatory expression of IL-8 in CF lung epithelial cells. Therefore, miR-155 may play an important role in the activation of IL-8 in CF. Objectives: The present study aimed to investigate the relationship between miR-155 expression level and the clinical manifestations of CF patients. Methods: The participants of this cross-sectional study included 30 CF patients (according to sweat test and < F508 in the genetic study) with age ranging from 5 to 27 years (M = 17, F = 13) and 30 healthy individuals with age ranging from 8 to 28 (M = 17, F = 13). Using RNA purification Kit, microRNAs, we extracted microRNAs from serum samples, and cDNA was synthesized via the cDNA synthesis kit. Then, the levels of miR-155 were measured by real-time PCR, and the expression levels were compared in different groups of CF patients according to the Shwachman-Kulczycki scoring system. Results: The expression level of miR-155 was elevated in CF patients compared to healthy controls (Fold change: 1.41, P value = 0.056). Interestingly, the elevated level of miR-155 in severe and moderate patients (according to Shwachman-Kulczycki score) showed a significant difference compared to the other patients, where age and sex were not an influential factor. Conclusions: Serum expression levels of miR-155 are different in CF patients, along with the severity of the disease. By suppressing the expression of miR-155, more investigations might lead to the development of new treatment strategies for CF.

Pulmonary Alveolar Proteinosis (PAP) is an uncommon pulmonary disease characterized by the accumulation of surfactant composed of proteins and lipids due to disruption of surfactant clearance by alveolar macrophages. The current standard treatment is lung lavage. There are no specific criteria for lavage, but in case of observing these signs it is recommended to perform lavage for the patient: progressive respiratory failure, no labored breathing at rest, and drop in oxygen level during activity (5%).In this study, patients with PAP admitted to Pediatric ward of Masih Daneshvari Hospital were studied. The required data were collected including the patient's demographic data, clinical signs and radiographic data, the number of admissions, the age of diagnosis, detection and treatment methods, number of lavage, current condition of the patient, and in case of death, the cause of death.In this study, 17 patients with PAP who were admitted during the past 15 years were examined; among which 7 patients were boys (41.2%) and 10 were girls (58.8%). The mean age of population was 11.79±7.21 years. Transbronchial Lung Biopsy (TBLB) (47.1%) and open lung biopsy (52.9%) were used for diagnosis of patients. Lung lavage was used to treat patients, 15 of whom were treated by this method. Five of the patients died because of their serious conditions.Therapy method in the present study was lavage for both lungs, and it was performed for all patients except for two patients due to their anatomical complications. This method is still considered as the gold standard for PAP. Considering the findings from previous studies and the present study, it seems that Whole Lung Lavage (WLL) was fruitful for patients who had the indication for using this therapy and it played a significant role in improving the prognosis of patients. Besides, it is recommended to do follow-up regularly in order to have more therapeutic efficacy and increased patient longevity.

Background: Two major complications of Aspergillus sensitization in patients with asthma, including severe asthma with fungal sensitization (SAFS) and asthma associated with fungal sensitization (AAFS), have been recently described. Objectives: In the present study, we aimed to evaluate the prevalence of SAFS and AAFS in Iranian patients with asthma. Methods: Two hundred consecutive outpatients aged ≥ 18 years with moderate to severe allergic asthma, referred to a pulmonary subspecialty hospital (Tehran, Iran) for 25 months, were included in the study. Skin prick test (SPT), total IgE (tIgE), and specific IgE (sIgEAf) and IgG against Aspergillus fumigatus (sIgGAf) were determined for all subjects. Comprehensive criteria were applied for the diagnosis of SAFS and AAFS. Results: Of 200 included patients, 103 (51.5%) and 97 (48.5%) were with moderate and severe asthma, respectively. Of these patients, 111 (55.5%) were female. The mean (range) of age was 45.8 (18 - 78) years. Of 200 patients, 27 (13.5%), 22 (11.0%), 114 (57.0%), and 131 (65.5%) were positive for Aspergillus SPT, sIgEAf, sIgGAf, and tIgE, respectively. The overall prevalence of SAFS in patients with severe asthma and AASF in patients with moderate asthma were 7.2% (7/97) and 3.9% (4/103), respectively. Conclusions: According to the findings, the prevalence of SAFS and AAFS in Iranian patients with severe and moderate allergic asthma was lower than the previously published global data. This low-prevalence reported rate may be due to the fact that we applied strict criteria in the present study.

Introduction Asthma exacerbations may occur due to a variety of triggers including respiratory viruses. The aim of this study was to determine the role of particular viral infections in asthma exacerbations in children. Materials and methods The study was performed at Dr. Daneshvari Hospital Pediatric Emergency Department, Shahid Beheshti University of Medical Sciences, Tehran, Iran between 2014 and 2015. A nasopharyngeal aspirate or swab was obtained from each patient during admission. All samples were maintained at 4 °C until submission to the virology laboratory and were tested for respiratory viruses by nucleic acid testing. Results A total of 60 patients with asthma exacerbations were recruited for this study. Of the 60 samples collected from the patients with acute asthma exacerbations, rhinovirus was detected in 12 patients (20%), respiratory syncytial virus in 5 (8%), adenovirus in 5 (8%), and influenza virus in 1 (1.6%). Respiratory pathogens were not detected in 37 (61%) samples. All the samples investigated showed single viral infection. Conclusions To conclude, the most common viruses detected were rhinovirus followed by respiratory syncytial virus (RSV) and adenovirus. RSV was more commonly associated with more severe attacks. Both the study design (e.g., time of sampling, age of the patients, etc.) and also the method used for viral detection influence the frequency of detection of the respiratory viruses.

Background: The present systematic review and meta-analysis is designed in order to assess the association between passive and active smoking and lipid profile of children and adolescents. Materials and Methods: An extensive search was done in databases of Medline, EMBASE, Web of Science, Scopus and CINAHL until October 2017. Two independent researchers screened articles and in the next step, full texts of probably relevant articles were read and summarized. At the end, results of mentioned studies were pooled and a standardized mean difference (SMD) with 95% confidence interval (95% CI) was reported. Results: Data from 17 studies (containing 41619 children and adolescents; age group between 4 and 18 years old; 51.72% boys) were entered. Comparing serum level of high density lipoprotein (HDL) in two groups of exposed and non-exposed to cigarette smoking showed that active exposure (SMD= -0.40, 95% CI: -0.59 to -0.21) and passive exposure to cigarette smoke (SMD= -0.18, 95% CI: -0.30 to -0.06) decreases the serum level of mentioned lipoprotein. Additionally, active exposure to cigarette smoke (SMD=0.16, 95% CI: 0.06 to 0.27) causes a modest increase in serum level of triglyceride. However, cigarette smoke exposure does not have any effect on the level of total cholesterol and low density lipoprotein (LDL). Conclusion: The present meta-analysis showed that exposure to cigarette smoke leads to a significant decrease in the level of HDL and triglyceride but, it does not have any effect of the level of total cholesterol and LDL in children and adolescents.

Background: Smoking is a known predisposing factor to exacerbations in CF patients. But the effects of second-hand tobacco smoking are not yet clear. Hence, this study determined the clinical and spirometric presentations and urinary cotinine levels among cystic fibrosis patients over seven years of age in relation to their parent’s smoking history. Materials and Methods: In this cross-sectional comparative study, 58 consecutive cystic fibrosis patients older than seven years of age were enrolled. These patients were divided into two equal groups: those with second-hand tobacco smoking and those without. Pulmonary function tests and hospital admission rates were compared across the groups. Results: The mean hospital admission times were 5.1±2.4 in the group with passive smoking, and 2.6±1.3 times in the group without (P

Purpose: The role of computed tomography (CT) scan, as a promising prognostic imaging modality in cystic fibrosis(CF), has been widely investigated, focusing on parenchymal abnormalities. The aim of the present study was to evaluate the diagnostic performance of thoracic vascular parameters on CT to detect pulmonary hypertension (PH). Material and methods: CF patients who contemporaneously underwent CT and echocardiography were retrospectively enrolled. Baseline characteristics in addition to pulmonary artery diameter (PAD) and pulmonary to aortic (PA/A) ratio were compared between cohorts with and without PH, based on the results of echocardiography separately in paediatric patients (< 18) and adults (≥ 18). Results: Of a total 119 CF patients, 39 (32.8%) had PH (paediatric: 23/78, 29.5%, adult: 16/41, 39%). In paediatric CF patients, mean age, HCo3, PCo2, and pulmonary artery diameter (PAD) were significantly higher in the PH group compared to the non-PH group. Mean pulmo however, only PAD remained as the independent predictor of PH based on multivariate analysis (overall: 22.86 mm [±3.86] vs. 18.43 mm [±4.72], p = 0.005, paediatric patients: 22.63 mm [±4.4] vs. 17.10 mm [±4.64], p = 0.03). Using a cut off of 19.25 mm, the diagnostic performance of PAD to detect PH was found to be as follows: sensitivity = 82%, specificity = 70%, and accuracy = 73.1%. No significant difference was demonstrated in PAD between PH and non-PH groups in adults with CF (23.19 [±3.60] vs. 21.34 [±3.49], p = 0.7). Conclusions: In CF patients, PAD revealed an age-dependent performance to detect PH. PAD can be applied to predict pulmonary hypertension in paediatric CF patients and may be recommended to be routinely measured on follow-up chest CT scan in childhood CF.

Background: Failure to maintain bone mass density is a major complication in patients with cystic fibrosis (CF). This study was conducted to evaluate the prevalence of low bone mineral density (BMD) and also identifying associated risk factors in CF patients. Materials and Methods: Present study conducted on 59 CF patients aged 5-35 years referred to respiratory clinic of Masih Daneshvari Hospital, Tehran-Iran. BMD was measured using dual energy X-ray absorptiometry (DXA) scan. Patients were divided in two groups: cases aged 5-18 years as group A and cases over 18 years as group B. Anthropometric variables, corticosteroid usage, pulmonary function test, serum calcium, phosphate and 25-OH vitamin D were assessed and correlation of them with BMD was investigated. Results: Low BMD (Z score < -2 standard deviation) was found in 72.8% (44) of patients. There was a positive correlation between malnutrition, Forced Expiratory Volume 1 (FEV1) and BMD (r=59 and 0.47, P

Cystic fibrosis (CF) has been generally well defined throughout the world although its prevalence is very difficult to ascertain for a number of reasons, including the fact that the medical/scientific literature and patient registries vary in quality in different countries. In the present review literature (1938–2017), we found out the higher frequency of registered CF patients in the European Union. This could be due to strong clinical awareness and greater health facilities. In contrast, we found a very irregular report from Asian and African countries. In these countries, the national CF registration system is mostly lacking or it is individualized-based research. In this situation, the estimation of various risk factors such as ethnicity and/or races is remained to be resolved. Overall, this review outlines the urgent need for revitalization of national and global CF registration, worldwide.

Background: Exhaled nitric oxide (FeNO) in cystic fibrosis (CF) patients is reduced when compared with healthy people, and it has now been found that FeNO has a relative association with airway clearance index. Studies have shown the role of some infections in changing the FeNO level; however, the role of respiratory infections in FeNO has not yet been thoroughly studied. Objectives: The objective of this study was to investigate the possibility of FeNO usage to monitor the infections in CF patients. Methods: This cross-sectional case-control study was conducted on CF patients between the age of five to 18 with positive sputum culture, through simple non-random census method. FeNO levels were measured in 30 healthy children and 30 CF children with positive sputum culture. CF patients were treated by antibiotic therapy for two weeks; in the case offending negative sputum culture, the FeNO level was re-measured. FeNO levels were re-evaluated in 13 patients after four weeks. Results: There was no statistical difference between both groups in terms of age and weight. The level of FeNO in CF patients was significantly lower than in healthy children (22.1 ± 10.1 versus 30.0 ± 11.0 in the control group and P = 0.003). In 27 children, two weeks after administration of antibiotic therapy, sputum culture was negative. The mean of FeNO in these patients was 16.4 ± 5.5 at the time of the negative sputum culture, which was significantly lower than FeNO before starting the treatment. (P: 0.003). The mean FeNO was 13.0 ± 7.41 in 13 patients who were re-measured four weeks after starting the treatment. There was no significant difference between FeNO level two weeks after treatment and four weeks after starting treatment (P: 0.292). Patients with pseudomona sputum culture were not significantly different from those with non-pseudomona sputum culture in terms of primary FeNO and FeNO changes after the treatment (P value: 0.084 and 0.094, respectively). Conclusions: However, in our study, FeNO was decreased after administration of antibiotic treatment in CF patients, but according to the sample size and conflicting or similar results in other studies, currently, FeNO levels cannot be used as a way of monitoring the treatment of infection in CF patients.

Introduction and objectives Exposure to fungi in patients with asthma leads to the release of various fungal antigens, which can increase the severity of asthma. Regarding this, the present study was conducted to evaluate the relationship between the colonisation of Aspergillus species and spirometry results in allergic asthma. Materials and methods Two hundred sixteen patients with mild to severe asthma and 30 healthy controls were included. All participants underwent pulmonary function tests. Furthermore, sputum samples were collected from each subject. Each sputum sample was subjected to direct microscopic examination and fungal culture. All cultured Aspergillus colonies were identified at species level by molecular methods. Finally, all available data from sputum culture and spirometry test were analysed. Results Out of 216 sputum samples, 145 (67.1%) were positive for fungal growth. Furthermore, out of 264 grown fungal colonies, 137 (51.9%) were Aspergillus species. Among the Aspergillus isolates, A. flavus (29.2%) was the most prevalent species, followed by A. fumigatus (27.7%). The mean forced expiratory volume in one second (FEV1) in the mild, moderate and severe asthmatic patients with a positive sputum culture for fungi were obtained as 90.0 ± 11.1, 71.1 ± 15.9 and 54.9 ± 16.4, respectively. In general, Aspergillus species colonisation had no statistically significant effect on spirometry results of study patients. Conclusion Our results showed that there is no difference in the FEV1 and forced vital capacity between Aspergillus positive and negative patients in any asthma severity group.

ObjectiveSinusitis is a common complaint in children with cystic fibrosis. However, the actual prevalence of chronic rhinosinusitis and its effect on the quality of life of children have not been well considered. Therefore, the objective of this study was to determine the effect of sinonasal quality of life in children with cystic fibrosis.Materials and methodsThis study was a diagnostic study performed on 80 children with cystic fibrosis ranging from 2 to 20 years old, who were referred to the cystic fibrosis clinic of Masih Daneshvari Hospital from 2017–2018. The questionnaires used in this study were chronic rhinosinusitis screening questionnaire based on the European task force and the evaluation of the sinonasal quality of life was based on the SN-5 survey.ResultsOf the 80 patients with fibrosis from 2 to 20 years old who were recruited in the study, 41 patients were female (51.3%) and 39 were male (48.8%). In 61 cases (76.3%), there was no chronic rhinosinusitis and 19 cases (23.8%) had chronic rhinosinusitis. The mean SN-5 score in 19 patients with chronic rhinosinusitis was 3.4105 and the mean score of patients without rhinosinusitis was 1.8426, with a P-value of 0.000. The mean SN-5 score was significant between the two groups. In patients with nasal congestion, there was a significant difference in quality of life factors such as sinus infection, nasal obstruction, and allergy symptoms (PConclusionIn children with cystic fibrosis, the quality of life of sinonasal has a significant relationship with absence of chronic rhinosinusitis. This study showed that children with chronic renosinusitis have significantly lower quality of sinonasal life than children with chronic rhinosinusitis. The results demonstrated that quality of life scores in sinus infections, nasal obstruction, and allergy symptoms were significantly higher in patients with chronic rhinosinusitis than in those without chronic rhinosinusitis. The findings of this study are important for improving children’s health related quality of life, as it leads to promoting communication between the patient and the health care provider, identifying overlooked problems, monitoring the progress of the disease and the burden of treatment, and promoting interventions in the daily life of patients.

Background: Cystic Fibrosis (CF) is a life-threatening recessive genetic disorder resulting from mutations in the gene encoding the fibrosis transmembrane conductance regulator protein (CFTR). The CF clinical phenotype shows wide variation ranging from severe disease in early childhood in those homozygous for the p.Phe508del mutation to absence of the vas deferens in otherwise healthy men homozygous for the p.Arg117His mutation. Materials and Methods: DNA was extracted from whole blood from 62 patients with CF. The CFTR mutation was determined by Allele-Specific PCR assay. The spearman and linear regression analysis were used to obtain the correlation between phenotype and genotype relationship. Results: Out of total 62 patients, 35 (56.4%) were male. The mean age of the patients was 15.56 ± 6.65 years. Mutations in CFTR were detected in 64.5% of the patients. The commonest mutations were p.Phe508del (33.9%), p.Arg117His; [5T] (5.64%), p.Arg117His; [7T] (4.03%) and p.Trp1282X (5.64%). Mutations p.Ile507del (4%), p.Gly542X (4%), p.Asn1303Lys (2.42%), c.489+1G>T (1.6%), p.Gly551Asp (1.6%) and c.1585-1G>A (1.6%) were also detected. Most mutations were detected in west and south of Iran, while p.Phe508del mutation was dominant mutation (75%) in east and southeast of Iran. The study showed either an association between this mutation with severity of disease and sex or an association between p.Arg117His mutations and age at diagnosis. Conclusion: The geographic distribution of gene mutation in Iranian cystic fibrosis patients was very heterogenic. In spite of the study that showed a correlation between p.Phe508del and severity of disease, to find any correlation between genotype and phenotype a broad and multi-centered study is recommended.

Summary Objective.There are different diagnostic criteria for the diagnosis of Allergic bronchopulmonary aspergillosis (ABPA) in CF patients. In this present study we evaluated the prevalence of ABPA in Iranian CF patients by two more usual diagnostic criteria as ISHAM working criteria (A) and CF Foundation Consensus Conference criteria (B). Methods.Eighty-six CF patients were included in the study. All CF patients underwent for Aspergillus skin prick test (AST), Aspergillus-specific IgE (sIgEAf) and Aspergillus-specific IgG (sIgGAf), total IgE. The ABPA prevalence was estimated by two diagnostic criteria, (A) and (B) and compared. Results. The frequency of positive AST, total IgE, sIgEAf and sIgGAf were 47 (54.6%), 9 (10.5%), 42 (48.8%) and 67 (77.9%), respectively. The obtained rate of ABPA prevalence (10.5%) was identical in two diagnostic criteria A and B (kappa value of 1.000). Conclusions.The applied diagnostic criteria had no significant effect on the reported rate of ABPA prevalence.

Background: Respiratory failure secondary to recurrent respiratory infections is the most common cause of death in Cystic fibrosis (CF). Objectives: To assess the efficacy of noninvasive positive pressure ventilation (NIPPV) on respiratory function, use of accessory respiratory muscles, ease of physiotherapy for respiratory clearance, quality of sleep and nutritional status in CF patients with respiratory failure. Methods: All CF patients admitted to the pediatric department of Masih Daneshvari hospital, Tehran from March 2015 to January 2016 were studied. The inclusion criteria were clinical evidence of respiratory distress and signs of respiratory acidosis. The exclusion criteria were nausea and vomiting, not tolerating NIPPV, need for intubation, pneumothorax, presence of giant bullous emphysema or decreased level of consciousness. Baseline spirometry and venous blood gas (VBG) was obtained before and after NIPPV. A simple questionnaire was filled out for the nutritional status, sleep quality, ease of physiotherapy for airway clearance and the volume of secretions after NIPPV compared to baseline. Paired samples t-test and Wilcoxon Signed Rank test in SPSS version 16 were used for the before and after comparison of numerical and ordinal variables, respectively. Results: Out of a total of 53 CF hospitalized patients 17 met the inclusion criteria. There were 10 (58.8%) males and 7 (41.1%) females with a mean age of 11 ± 4.57 (10 - 25) years, mean weight of 31.85 ± 10.11 (13 - 48) kg and mean BMI of 14.16 ± 2.71 (5.9 - 18). The mean Saturation of peripheral oxygen (SPO2), respiratory rate (RR) and mean partial pressure of CO2 (PCO2) of patients before the intervention were 75 ± 13.25% (52 - 90%), 32 ± 11.17 (22 - 55) mmHg and 55.20 ± 17.62 (30.3 - 108), mmHg respectively. A significant difference was noted after the intervention in SPO2, venous PCO2 and RR compared to baseline (P < 0.05). The change in sleep quality (P = 0.001), nutritional status (P = 0.001) and ease of physiotherapy for respiratory clearance (P = 0.008) after NIPPV was statistically significant while the change in use of accessory respiratory muscles (P = 0.785) and the volume of secretions (P = 0.1) was not significant. Conclusions: NIPPV in CF patients with respiratory failure can improve the respiratory function, sleep quality and nutritional status of patients. Also, NIPPV enhances airway clearance during respiratory physiotherapy.

Background: The effect of various environmental and intrinsic stimulators on the development of brown adipose tissue (BAT) has been widely investigated by PET-based researches. However, evidence regarding the influencing factors on the level of BAT metabolic activity is scarce. Objectives: The aim of the present study was to evaluate the frequency of cancer-related characteristics in addition to anthropometrics and demographics in BAT-bearing cancer patients at 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) scan and any correlation between the level of BAT metabolic activity and the influencing factors. Patients and Methods: Reports from a total of 3762 F-18 FDG PET/CT scans were retrospectively reviewed to identify BAT-bearing cancer population. Demographic, anthropometric and cancer related characteristics were recorded. Maximum standardized uptake values (SUVmax) was measured separately for each anatomical region. Descriptive quantitative variables were expressed as either frequency or mean. Independent T test, Mann-Withney U test, Pearson correlation coefficients, one-way analysis of variance and linear regression test (IBM SPSS version 23) were used as appropriate (P value