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Objectives: Aim of this study was determining the relationship between burden of care and fear of disease progression in mothers of children with cystic fibrosis. Methodology: 142 mothers of children with cystic fibrosis (CF) participated in this correlational study. Data were collected with sequential method. The researchers employed the self-report Zarit burden of care and fear of disease progression questionnaire in parents of children with CF. Mothers answered the questioners separately. Data were analyzed with statistical analysis of variance and independent t-test. Results: According to the results, the mean maternal burden of care was 34.12 ± 69.77, and the mean fear of disease progression was 32.12 ± 92.11. The burden of care had the highest regression effect on fear of disease progression in mothers of children with CF (β = 0.896). With an increase of one unit of standard deviation in the burden of care, an increase of 0.896 units occurred in fear of disease progression in parents of children with CF. Conclusion: Mothers of children with CF experience a moderate burden of care and fear of disease progression. An increase in the burden of care and psychological stress increases the fear of disease progression in these mothers.

There is no specific test in the definitive diagnostic approach to Allergic bronchopulmonary aspergillosis (ABPA) especially in the background of cystic fibrosis, but comprehensive and simultaneous clinical, radiological and serological examination will be the basis of ABPA diagnosis. The increasing in attenuation of bronchoceles in imaging has recently been proposed as a valuable diagnostic criterion.The present study aimed to assess bronchocele attenuation in pulmonary CT scan of patients with complicated cystic fibrosis for diagnosis of ABPA.This cross-sectional study was performed on 74 consecutive patients aged 3-18 years suffering cystic fibrosis presented with exacerbation of pulmonary symptoms and were suspected of having ABPA. All were examined by 16 Slice CT Scan and the density of bronchoceles above 5 mm in diameter were measured in Hounsfield unit. The total serum IgE titer, skin prick test for aspergillus and anti-aspergillus IgG and IgE level were obtained for all subjects and both cutoff values of IgE level (500 IU/mL and1000 IU/mL) were considered as the criteria for ABPA diagnosis.Considering IgE level of greater than 500 IU/mL and 1000 IU/mL as the diagnostic criteria, 24.3% and 10.8% had evidence of ABPA, respectively. Considering the two pointed diagnostic IgE ranges and based on the analysis of the area under the ROC curve, bronchocele attenuation could effectively predict the presence of ABPA with the best cutoff values of 37.25 (with a sensitivity of 70.6% and a specificity of 66.7%) and 40.00 (with a sensitivity of 85.7% and a specificity of 65.1%), respectively.The presence of bronchocele and an increase in its attenuation on CT scan will be diagnostic for the occurrence of ABPA.

Cystic fibrosis (CF) is one of the most common autosomal recessive illnesses. Inflammatory responses, a lot of mucus in the respiratory system, and pulmonary and respiratory disorders are just a few of the issues these patients experience. Numerous research has been carried out to investigate cytokines in patients, but to the best of our knowledge, Iran has not provided any information on cytokine changes of CF patients. The purpose of our research was to investigate pro-inflammatory and anti-inflammatory cytokines in CF patients and analyzed the correlation of cytokines with each other’s and with FEV1and BMI in different microbial groups. In this study, 42 CF patients and 10 healthy people were examined to measure the levels of IL-10, IL-4, IFN-γ, IL-17, TGF-β, and IL-8 in serum and PBMC samples by ELISA and Real-time PCR methods. In the following, the patient's sputum samples were cultured in specific media and the relationship between cytokines and the microbiota was investigated. The results showed that patients had higher levels of IL-10, IL-4, and IFN-γ and lower levels of IL-17. There was a correlation between IL-10, IL-4, and IFN-γ in patients and by increasing IL-4, IL-10 and IFN-γ were increased. Patients withP. aeruginosahad higher levels of IL-10, IL-4, and IFN-γ, but there was no change in these markers in patients with other bacteria such asS. aureus, Acinetobacter, Citrobacter, Candida, and Klebsiella. TGF-β and IL-17 levels were reduced in patients withP. aeruginosaand other bacteria, respectively. However, in patients with microorganisms other thanP. aeruginosa, FEV1 had a positive relationship with IL-8, and with an increase in FEV1, the level of IL-8 increased. This contrasts with the investigation of the relationship between cytokines and FEV and BMI, which did not show any relationship between the factors in patients withP. aeruginosa. Our study's findings revealed that CF patient's cytokines differed from those of the control group. As a result, it is possible to recommend using the studied cytokines as markers for disease modulation.

Cytokines play a crucial role in the immune system's regulation by mediating protective responses to infections. anti-inflammatory and pro-inflammatory cytokines are in equilibrium. Therefore, any alteration in cytokine production or cytokine receptor expression might result in pathological illnesses and health issues. Cystic fibrosis (CF) is a genetic disease caused by mutations in the CF transmembrane regulator (CFTR) gene. Lung infection in these patients is related to chronic bacterial airway infection and inflammation, which is triggered by some inflammatory cytokines. Our goal was to compare the cytokine patterns in CF patient's serum and PBMCs caused by microbial pathogens that colonized their airways to controls.ELISA and Real-time PCR were used to determine the levels of IL-10, IFN-γ, IL-4, TGF-β, IL-8, and IL-17 in serum and PBMC cells. Blood parameters in both patients and healthy people were studied.An increase in IL-10, IFN-γ, IL-4 (p-v = 0.03, 0.024 and 0.003) levels and a decrease in IL-17 (p-v = 0.004) was found in Pseudomonas aeruginosa positive patients. There were no different in TGF-β and IL-8 (p-value = 0.778 and 0.903) in this patients. IL-10, IFN-γ, and IL-4 (p-value = 0.023, 0.001 and 0.002) levels were high in Staphylococcus aureus positive patients and TGF-β, IL-17, and IL-8 (p-value = 0.085, 0.167 and 0.362) were not significantly different in the patient and control groups. IFN-γ and IL-4 levels were higher in patients without infection who had normal microbiota (p-v = 0.002 and 0.024). In patients with P. aeruginosa, WBC and platelets increased, and MCH and MCV decreased. Patients with normal microbiota had less MCV.According to our research, patients with P. aeruginosa, S. aureus, and normal microbiota are exposed to cytokine alterations and changes in blood factors. The link between the CF patient's airway microbiota and the kind of generated cytokines might lead to the modulation of inflammatory cytokines alone or in combination with antibiotics, reducing disease-causing effects while avoiding drug resistance.

Chronic bacterial infections of the airways are present in most patients with cystic fibrosis (CF). Although most pathogens are acquired from the environment, there is great evidence of patient-to-patient transmission. Therefore, evaluating the genetic variation of strains isolated from CF patients is recommended for the purpose of examining hospital infection.The aim of this study was to determine the antibiotic susceptibility pattern and genotyping of Staphylococcus aureus and Pseudomonas aeruginosa strains isolated from sputum samples of children with CF referred to a single pediatric CF center in Tehran, Iran.In this cross-sectional study, the antimicrobial susceptibility profiles of strains isolated from patients with CF during 1 year were determined. Pseudomonas aeruginosa and S. aureus isolates were genotyped using the random amplified polymorphic DNA polymerase chain reaction method and were analyzed using GelCompar II software.Of 534 patients with CF, 384 had negative sputum cultures (72%), and 94 strains of P. aeruginosa (18%) and 53 strains of S. aureus (10%) were isolated. The mean age of the patients was 8.22 ± 5.7 years (range, 2 months to 18 years). The P. aeruginosa strains showed high sensitivity to ceftazidime (96%), piperacillin/tazobactam (96%), and imipenem (94%). All strains of S. aureus were susceptible to vancomycin, and 13% of the strains were methicillin-resistant S. aureus. High resistance to penicillin (92%) and erythromycin (88.5%) were reported. The results of P. aeruginosa genotyping revealed that there were six major clusters in this hospital. Also, based on the analysis of genotyping results, S. aureus strains were obtained from five clusters, most of which were located in cluster B1 (34 isolates, 64%).The results of this study show the possibility of strains being transferred from one part of the hospital to another (especially from the respiratory ward to other areas). Hence, high attention should be paid to the basic methods of preventing infection.

Background Inborn errors of immunity (IEI) and autoantibodies to type I interferons (IFNs) underlie critical COVID-19 pneumonia in at least 15% of the patients, while the causes of multisystem inflammatory syndrome in children (MIS-C) remain elusive. Objectives To detect causal genetic variants in very rare cases with concomitant critical COVID-19 pneumonia and MIS-C. Methods Whole exome sequencing was performed, and the impact of candidate gene variants was investigated. Plasma levels of cytokines, specific antibodies against the virus, and autoantibodies against type I IFNs were also measured. Results We report a 3-year-old child who died on day 56 of SARS-CoV-2 infection with an unusual clinical presentation, combining both critical COVID-19 pneumonia and MIS-C. We identified a large, homozygous loss-of-function deletion in IFNAR1, underlying autosomal recessive IFNAR1 deficiency. Conclusions Our findings confirm that impaired type I IFN immunity can underlie critical COVID-19 pneumonia, while suggesting that it can also unexpectedly underlie concomitant MIS-C. Our report further raises the possibility that inherited or acquired dysregulation of type I IFN immunity might contribute to MIS-C in other patients.

Background: Most severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2)-infected individuals are asymptomatic or only exhibit mild disease. In about 10% of cases, the infection leads to hypoxemic pneumonia, although it is much more rare in children. Objective: We evaluated 31 young patients aged 0.5 to 19 years who had preexisting inborn errors of immunity (IEI) but lacked a molecular diagnosis and were later diagnosed with coronavirus disease 2019 (COVID-19) complications. Methods: Genetic evaluation by whole-exome sequencing was performed in all patients. SARS-CoV-2-specific antibodies, autoantibodies against type I IFN (IFN-I), and inflammatory factors in plasma were measured. We also reviewed COVID-19 disease severity/outcome in reported IEI patients. Results: A potential genetic cause of the IEI was identified in 28 patients (90.3%), including mutations that may affect IFN signaling, T- and B-cell function, the inflammasome, and the complement system. From tested patients 65.5% had detectable virus-specific antibodies, and 6.8% had autoantibodies neutralizing IFN-I. Five patients (16.1%) fulfilled the diagnostic criteria of multisystem inflammatory syndrome in children. Eleven patients (35.4%) died of COVID-19 complications. All together, at least 381 IEI children with COVID-19 have been reported in the literature to date. Although many patients with asymptomatic or mild disease may not have been reported, severe presentation of COVID-19 was observed in 23.6% of the published cases, and the mortality rate was 8.7%. Conclusions: Young patients with preexisting IEI may have higher mortality than children without IEI when infected with SARS-CoV-2. Elucidating the genetic basis of IEI patients with severe/critical COVID-19 may help to develop better strategies for prevention and treatment of severe COVID-19 disease and complications in pediatric patients.

Cough is part of the daily life of patients with Cystic fibrosis (CF) and its most common symptom. This study explored the experiences of adolescents with CF in Iran during the COVID-19 pandemic in relation to their cough.In this qualitative study, we conducted 32 semi-structured interviews with 21 adolescents with CF. We analyzed the data thematically.We identified three main themes among adolescents with CF in relation to coughing: 1. Cough is a permanent companion; 2. Coughing raises fear of double stigma; 3. Patients' individualized coping strategies to deal with coughing. Participants complained that cough interrupted daily tasks and sleep, drew unwanted attention in public places, and elicited questions about whether they were COVID-19 patients or substance users-both highly stigmatized identities.Although coughing is a protective mechanism for CF patients, frequent coughing often causes major challenges, particularly during the COVID pandemic, when people were acutely sensitive and aware about coughing. During the COVID-19 pandemic, in addition to taking care of themselves and managing the disease, CF patients therefore had to also overcome issues related to social stigma and isolation.Healthcare workers play an important role in increasing public awareness about CF and its symptoms, including cough. During the pandemic, healthcare workers can help reduce the stigma of coughing through public education. Healthcare workers can actively communicate with patients to identify severe and ineffective cases of cough due to exacerbation of the disease and refer them to a specialist.

Hyper Immunoglobulin M (HIgM) syndrome is a heterogeneous group of primary immunodeficiency disorders, characterized by recurrent infections and associated with decreased serum IgG and IgA, but normal or increased IgM. The aim of the present study was to evaluate respiratory manifestations in patients with HIgM syndrome. A total number of 62 patients, including 46 males and 16 females were included in the present study. To investigate the respiratory complications among HIgM patients, we evaluated the clinical hospital records, immunologic and molecular diagnostic assays, pulmonary function tests (PFT), and high-resolution computed tomography (HRCT) scans. Pneumonia was the most common respiratory manifestation (n = 35, 56.4%), followed by otitis media (45.1%), sinusitis (33.8%), and bronchiectasis (14.5%). 52.1% of the patients had abnormal PFT results, with a predominant restrictive pattern of changes. HRCT scans demonstrated abnormal findings in 85.7% of patients with found mutations. Ten cases had hilar lymphadenopathy and para-hilar infiltrates in their HRCT findings. Genetic diagnosis was confirmed in 29 HIgM patients (72.4% CD40 ligand (CD40L) and 24.1% activation-induced cytidine deaminase (AICDA/AID) deficiencies). Majority of patients with CD40L (71.4%) and AID (57.1%) deficiencies had missense mutations. Pneumonia and abnormal high-resolution computed tomography (HRCT) findings were more frequent among patients with CD40L mutation. Respiratory failure constituted the major cause of mortality (37.5%) with majority of cases occurring in CD40L-deficient patients (50%). Respiratory complications are common in patients with HIgM syndrome. A proper awareness of respiratory manifestations in patients with HIgM may result in improved management, reduced morbidity and mortality, and an improvement in the quality of life of the patients.

Most adult Cystic Fibrosis (CF) patients frequently suffer from Pseudomonas aeruginosa (PA) infection, which is strongly associated with inflammation, lung destruction, and increased mortality. Diagnosis of PA infection in the primary stage is essential to initiate the treatment and reduce the risk of chronic infection. Sputum culture is the gold standard ‎for infection detection, but it is time-consuming. The objective of this study was to suggest and examine a method to decide about PA infection status in CF patients based only on their respiratory sound. Respiratory sounds were recorded from 36 CF patients. Some features which were generated from Tunable Q-factor wavelet transform(TQWT) components, were investigated. The features were fed into Support Vector Machine and also Ensemble classifier. The proposed method achieved an accuracy of 90.3% in identifying PA infection in CF patients. Furthermore, the probability of categorizing respiratory sounds as PA CF decreased significantly after the treatment of PA infection(P-value

The 6-min walk test (6MWT) is a sub-maximal exercise test and has been widely used for evaluating of exercise capacity of patients with cystic fibrosis (CF) in recent years. Few studies have examined the relationship between 6MWT and parameters used to assess the severity of the disease in children with CF. In this study, we have examined this relationship to find out if 6MWT can be a marker of the severity of cystic fibrosis. A cross-sectional study was done to analyze the correlations among spirometry parameters, body mass index (BMI), chest tomography (CT), and 6MWT. CF patients, aged 7–14 years, were involved. Seventy-six patients, 32F/44M, mean age 10.49 ± 3.18 years, were studied. The mean distance in 6MWT was 447 ± 84.1. The following correlations versus distance were found: FEV1 (r = 0.255, p = 0.026), FVC(r = 0.285, p = 0.013), FEF25–75% (r = 0.546, p

To investigate the correlation between severity of lung disease determined by chest computed tomography (CT) and 6-min walk test (6MWT) with health-related quality of life (HRQoL) score in cystic fibrosis (CF) patients. This cross-sectional study evaluated 76 CF patients referred to CF Clinic, aged 7–14 y. Subjects were asked to complete Pediatric quality of life (PedsQL4.0) forms, during their outpatient visits to determine their HRQoL score. Patients’ lung disease severity was quantified by Bhalla score determined by the child’s chest CT and their 6MWT. These three variables were then analyzed to determine whether there is correlation between HRQoL with severity of lung disease. The mean distance of patients 6MWT score was 447.4 ± 81.4 m. There was a positive correlation between distance and HRQoL score in total, social, school and emotional function (p

Background: Cystic fibrosis (CF) is an autosomal recessive disease characterized by respiratory, gastrointestinal, and reproductive tract problems. Patients with CF often suffer from chronic pulmonary infections due to the Pseudomonas aeruginosa, which frequently ends in pulmonary exacerbations. With their anti-inflammatory, anti-virulence, and anti-biofilm properties, macrolides such as azithromycin (AZM) play a beneficial role in the treatment of CF. Objectives: We evaluated the ability of biofilm formation among P. aeruginosa isolates from CF patients and compared the anti-bacterial and anti-biofilm effect of the AZM on them. Methods: Minimum inhibitory concentration (MIC) and minimum bactericidal concentration (MBC) of AZM against 21 P. aeruginosa isolates from Iranian CF patients were determined. Then, we investigated the ability of biofilm formation by P. aeruginosa and also anti-biofilm effect of AZM on biofilm producing P. aeruginosa isolates. Results: The results showed a high level of AZM MIC (≤ 512 µg/mL) and MBC (≤ 2048 µg/mL) against P. aeruginosa isolates. Most isolates (70%) were biofilm producers, 20% being strong producers. The AZM anti-biofilm effect showed that sub-MIC concentration (≤ 64 µg/mL) could inhibit biofilm production. Conclusions: P. aeruginosa isolated from CF patients showed a high level of MIC and MBC for AZM that indicated a weak anti-bacterial effect of AZM. However, AZM inhibited biofilm formation in low doses; this might lead to dangerous chronic pulmonary infection by P. aeruginosa.

The importance of the host inflammatory response, as a central pathological feature of cystic fibrosis, is well recognized. Additionally, hyperglycemia can induce an immune response and consecutively may exacerbate symptoms of this disease. Hence, adherence to a low glycemic index diet, through normalizing blood glucose levels, may reduce inflammation in patients with this disease. This study aimed to compare effects of a low glycemic index/high-fat, high-calorie diet and routine high-fat, high-calorie diet on inflammatory biomarkers in patients with cystic fibrosis. In this randomized clinical trial, 44 children and adolescents with cystic fibrosis were randomly assigned to receive for three months either a high-fat, high-calorie diet (n = 22) or a low glycemic index/high-fat, high-calorie diet (n = 22) with similar calorie and macronutrients composition to the control diet. Patients in first arm were allowed to use all sources of carbohydrates with different glycemic indices, whereas those in another arm consumed carbohydrates from low glycemic index sources. Serum levels of the pro-inflammatory cytokines IL-6, IL-17A, and IFNγ, and the anti-inflammatory cytokine IL-10 were measured at baseline and after the end of the trial. There were significant differences between groups for IL-6 (P = 0.02) and IL-17 (P = 0.01), in favor of the low glycemic diet, but no between-group differences were detected in IL-10 and IFN-γ. Although serum levels of IL-17 were reduced in both the groups as compared with the baseline values, this reduction was only significant in the group assigned to the low glycemic diet (P= 0.007), In addition, IL-6 serum levels decreased and those of IL-10 increased significantly as compared with the baseline values in the low glycemic diet (P= 0.01). It seems that adherence to a low glycemic index/high-fat, high-calorie diet for three months can improve some inflammatory biomarkers in children and adolescents with cystic fibrosis compared with the high-fat, high-calorie diet.

Background: Flexible fiberoptic bronchoscopy (FFB) is known as an important diagnostic and therapeutic modality in the evaluation of respiratory disorders in pediatric population. Objectives: The study aims to highlight common indications and risk of complications associated with bronchoscopy in our population. Methods: This retrospective evaluation was performed in all patients that underwent flexible bronchoscopy at Children's Medical Center (affiliated to Tehran University of Medical Sciences) between April 2011 and September 2016. Results: Of 800 bronchoscopies, 574 (71.7%) were performed for diagnostic and 226 (28.2%) for therapeutic purposes. Major indications included radiographic abnormalities (30%), a foreign body or suspected foreign body (28.5% of all FFBs) and stridor or wheezing (25% of all FBB). The incidence of major complications associated with FFBs was 0.87%. The most frequent complication was pneumothorax, followed by lung hemorrhage, and respiratory failure. Conclusions: Our findings support early intervention and utilization of bronchoscopy in the pediatric population with variable respiratory complaints.

Background: The decrease in physical activities following increased usage of computer and digital games has led to serious health consequences in children. This study investigates the prevalence of obesity, cellphone and computer usage and physical activity levels and their relationship with spirometry indicators in Iranian children. Materials and Methods: This is a cross-sectional study during 2013 to 2014 on high-school students in Isfahan, Iran. Sample size determined 1,690 students and sampling performed from 10 girls and 10 boy's high schools, based on multi-staged cluster randomized scheme. A research-made questionnaire was developed to complete by students interview and also contact with teachers and parents and measuring height and weight by researchers. If the asthma was probable (based symptoms and examined by physician) the spirometry was performed. Results: Overall 1,622 high-school students with the average age of 12.9 (+ 1.1) years were recruited. Prevalence of obesity and overweight were significantly higher in boys (P

A new framework for model-based lung tissue segmentation in three-dimensional thoracic CT images is proposed. In the first stage, a parametric model for lung segmenting surface is created using shape representation based on level sets method. This model is constituted by the sum of a mean distance function and a number of weighted eigenshapes. Consequently, unlike the other model-based segmentation methods, there is no need to specify any marker point in this model. In the second stage, the segmenting surface is varied so as to be matched with the binarized input image. For this purpose, a region-based energy function is minimized with respect to the parameters including the weights of eigenshapes and coefficients of a three-dimensional similarity transform. Finally, the resulted segmenting surface is post-processed in order to improve its fitness with the lung borders of the input image. The experimental results demonstrated the outperformance of the proposed framework over its model-based counterparts in model matching stage. Moreover, it performed slightly better in terms of final segmentation results.

[Background] Congenital agammaglobulinemia is the first primary immunodeficiency disorder characterized by a defect in B lymphocyte development and subsequently decreased immunoglobulin levels. These patients are prone to suffer from recurrent infections mostly involving the respiratory tract. In this study, we aimed to describe in detail respiratory tract complications as the most prominent clinical feature among agammaglobulinemic patients., [Methods] A total number of 115 patients were included. Demographic, clinical, and genetic data were collected from the patients’ medical records. Among the available patients, pulmonary function tests (PFTs) and/or high‐resolution computed tomography (HRCT) were performed., [Results] Respiratory tract complications (85.2%) especially pneumonia (62.6%) were the most prominent clinical features in our cohort. Among patients with abnormal PFT results (N = 19), a mixed respiratory pattern was observed in 36.8%. HRCT was carried out in 29 patients; Bhalla scoring‐based evaluation of these patients indicated excellent (44.8%), followed by good (34.5%) and mild (20.7%) results. Bronchiectasis was found in 13 patients undergoing HRCT (44.8%). We found significant inverse correlations between the Bhalla score and incidence rate of pneumonia, as well as the presence of bronchiectasis. Patients with abnormal PFT results had statistically significant higher bronchiectasis frequency and lower Bhalla scores compared to those with normal results. Forty‐one patients were deceased, and here, respiratory failure was the most common cause of death (45.5%)., [Conclusion] High prevalence of respiratory tract infections among agammaglobulinemic patients and subsequent progression to permanent lung damage highlights the importance of implementing respiratory evaluation as part of routine follow‐up program of agammaglobulinemic patients. Physicians should be aware of this and regularly monitor the respiratory function of these patients to allow for timely diagnosis and treatment initiation aiming to improve patients’ prognosis and quality of life., This work was supported by a grant (37023‐154‐04‐96) from Tehran University of Medical Science.

Background: Oral azithromycin is recommended as anti-inflammatory therapy for patients with cystic fibrosis (CF) 6 years of age and older to improve lung function and reduce exacerbations. Objectives: We evaluated the short-term efficacy and safety of nebulized azithromycin as anti-inflammatory therapy in children with CF chronically infected with Pseudomonas aeruginosa. Methods: In a randomized prospective study, children aged 8 - 18 years with clinically stable CF and FEV1 25% - 75% predicted with no recent use of oral, intravenous or inhaled antipseudomonal antibiotics and azithromycin were randomized to receive either nebulized azithromycin (70 mg daily) or oral azithromycin 3 times per week for 28 days. Primary endpoint was changes in pulmonary function (FEV1). Secondary outcomes included changes in Pseudomonas aeruginosa (PA) colonization characteristics (count, phenotype), quality of life and weight. Results: A total of 280 patients were screened, of whom 60 were found eligible. Forty five patients (25 in the nebulized group) and 20 in the oral group) completed the study. After 28 days of treatment, nebulized azithromycin was significantly associated with improvements in FEV1 % predicted, quality of life measure and weight. Also PA count was significantly decreased. Importantly, improvements of FEV1 % predicted, quality of life, weight and the decline in PA count were statistically significantly greater with nebulized azithromycin compared with oral azithromycin. No significant difference was observed in PA phenotype in either treatment arms. Conclusions: Nebulized azithromycin is associated with a significant improvement in lung function, PA count, quality of life and weight in clinically stable CF children with chronic PA infection. The improvements observed were greater compared with oral azithromycin. Nebulized azithromycin could be suggested as a new therapeutic strategy for this life-limiting disease. Further clinical trials with novel nebulizer formulation of azithromycin and large number of participants are needed to further assess the efficacy, safety and sustained effect of this new therapeutic approach in children with CF.

Background: Inflammation plays an important role in pathogenesis of cystic fibrosis, and diet is considered as an effective factor in controlling inflammation. Low glycemic index diet seems to be effective for improving glycemic control and reducing the inflammation and lipid profile. Hence, this study was planned to compare the effects of a low glycemic index/high fat, high calorie diet with the high fat, high calorie diet on glycemic status, lipid profile, and inflammatory markers in patients with cystic fibrosis. Methods: A total of 60 children and adolescence with cystic fibrosis will be randomized to receive a high fat, high calorie diet (n=30) or low glycemic index/high fat, high calorie diet (n=30) with similar calorie and macronutrients composition, for three months. Patients in high fat, high calorie diet arm will be able to use all sources of carbohydrates with different glycaemic indices; while those in another arm will be received their carbohydrate from low glycemic index sources. Before and after the intervention, serum levels of lipid profile (triglyceride, total cholesterol, HDL cholesterol, LDL cholesterol), insulin, glucose, HbA1c, the homeostasis model assessment-estimated insulin resistance index, and some inflammatory markers (IL-6, IL-10, IL-17A, and IFNγ) will be measured. Discussion: To our knowledge, this study will be the first to examine the effects of a low glycemic index/high fat, high calorie diet compared to the high fat, high calorie diet in cystic fibrosis patients. The findings of this study might provide evidence to improve the nutritional status and immune system as well as to prevent the disease complications in these patients.

Low glycemic index diets seem to be potentially effective to improve glycemic control and reduce lipid profiles. Hence, this study aimed to evaluate the effect of a low glycemic index/high fat, high-calorie diet on glycemic status and lipid profiles of patients with cystic fibrosis.In this randomized clinical trial, 44 children and adolescents with cystic fibrosis were randomized to receive for three months either a high fat, high-calorie diet (n = 22) or a low glycemic index/high fat, high-calorie diet (n = 22) with similar calorie and macronutrients composition. Patients in high fat, high-calorie diet arm were allowed to use all sources of carbohydrates with different glycaemic indices; whereas those in another arm consumed carbohydrates from low glycemic index sources. Serum levels of lipid profiles (triglyceride, total cholesterol, HDL cholesterol, LDL cholesterol), insulin, fasting blood glucose, and glycated hemoglobin were measured at baseline and after the intervention.Between-group differences were significant only for fasting blood glucose (P 0.001). However, fasting blood glucose (P = 0.003) and glycated hemoglobin (P = 0.002) significantly decreased after the intervention in the low glycemic index group, while in another group a significant increase in fasting blood glucose (P = 0.038) and triglyceride (P = 0.004) was found. No significant within-group differences were observed in other variables in both groups.It seems that adherence to a low glycemic index/high fat, high-calorie diet can improve glycemic indices in children and adolescents with cystic fibrosis compared to the high fat, high-calorie diet.IRCT2017102325267N5.

Introduction: Lobar atelectasis is one of the common problems in patients with cystic fibrosis and can be a cause of respiratory symptoms in these patients even in the absence of bacterial infection. Case Presentation: Here we introduce a 9-year-old boy with cystic fibrosis who had many admissions because of respiratory distress due to lung collapse mainly caused by plastic bronchitis. Conclusions: In a number of patients with cystic fibrosis, lobar atelectasis is seen which is resistant to medical treatments. Early fiberoptic bronchoscopy as a measure for mechanical removal of the blocking agent and sometimes direct injection of drugs like dornase alfa into the bronchus is a successful modality in the treatment of lobar collapses in chronic pulmonary diseases.

Background: Cystic fibrosis is a complex, multi-system impairment with poly pharmacy which affects both children and their parents. Adherence to treatment is essential to optimize CF disease management. Aims and Objectives: The aim of the present study was to evaluate the medication adherence and indeed identify the factors affecting adherence in this population. Methods: An observational prospective study was conducted to recruited CF outpatient at the CF clinic of Children’s Medical Center in Iran. Children with CF and their parents were consecutively recruited. Baseline data, medical history, clinical outcome data, were collected via interview and review of medical notes. The Morisky Medication Adherence scale (MMAS) was used to measure adherence. Results: Three hundred patients with CF (age 1-25 years, mean 7.6 years, 58.7% males) and parent pairs were recruited. The mean score of MMAS in pulmonary, antibiotics, gastrointestinal and supplement medications was 5.1, 4.6, 4.1 and 3.3 respectively. According to the categorization of Morisky Scale, 58% of patients who received inhaler medications and 65% of patients who received antibiotics had a low adherence (lower than 6 scores). High adherence was associated with child age, parental higher educational degree and their beliefs, duration of illness and frequency of healthcare visit ((P=0.001). Conclusion: The results indicated that the non-adherence was very common in patients with CF. Our results suggest the need for educational interventions and planned patient monitoring program in patients with CF to optimize management and health outcomes.

Objective Respiratory disease in Cystic fibrosis (CF) patients is one of the main causes of the reduction in pulmonary function and death. The primary goals of CF treatment include maintaining or improving pulmonary function and reducing the rate of pulmonary function decline. Therefore, the severity of lung disease should be monitored in CF patients. The objective of this study is to examine multichannel lung sound analysis in detecting the severity of lung disease in CF patients. Methods 209 multichannel lung sound samples were recorded from thirty seven CF patients using a thirty channel acquisition system. Then, expiration to inspiration lung sound power ratio features in different frequency bands (E/I F) were extracted from large airway, upper airway and peripheral airway channels. These features were compared between the groups with different severity levels of the lung disease using Support Vector Machine, Artificial Neural Network, Decision tree and Naive Baysian classifiers by ‘leave-one-sample-out’ method. Results It was shown that features of upper airways and peripheral airways were more effective in discriminating normal from mild (91.1%) and moderate from severe (92.8%) respiratory sound samples, respectively. The best result for discriminating between all groups of severity was related to neural network classifier which performs 89.05% average accuracy. Also, ‘leave-one-subject-out’ method confirmed the results. Conclusion The proposed multichannel lung sound analysis method was successful in discriminating different severity levels of CF lung disease. Moreover, analysis of different lung region signals in consecutive levels of lung disease was consistent with regional damage of lung in CF.

BackgroundBronchiectasis is defined by permanent and abnormal widening of the bronchi. Although this process occurs in the context of chronic airway infection and inflammation, since there is no accurate estimation of the etiology of the disease. This study aimed to determine the most important cause of bronchiectasis in Tehran, Iran.Materials and MethodsIn this retrospective cohort study we used the information of 91 patients admitted to two subspecialty lung hospitals in Tehran-Iran, where a wide range of bronchiectasis patients from around the country referred during 2013to 2014 period. Patients referring with the manifestation of chronic productive cough who had not responded to conventional treatment with the evidences of bronchiectasis on high resolution computed tomography were included. Data were analyzed using SPSS-16.0.ResultsThe etiology of bronchiectasis was diagnosed in 73 of 91 patients (80.2%), the most important of which included cystic fibrosis, post infectious, and primary ciliary dyskinesia (PCD). The most common causes of bronchiectasis in the children group (Age ≤ 18 years), were cystic fibrosis (57.1%), allergic bronchopulmonary aspergillusis (14.3%) and PCD (9.5%), respectively. In the adults group (Age >18 years), the most common causes were post infectious (22.6%), PCD (15.7%) and cystic fibrosis (14.3%), respectively.ConclusionMain causes of bronchiectasis in this study were not significantly different from other studies. Special attention should be paid to the probable causes of bronchiectasis in order to effectively execute on-time diagnosis, proper treatment and management of complications.

Introduction: Primary ciliary dyskinesia [PCD] is generally considered as a rare autosomal recessive disorder. Previous studies reported various prevalence of PCD among patients with bronchiectasis. Material and methods: Six PCD patients who were diagnosed during the investigation of 40 patients with bronchiectasis were enrolled in this study. Ultra structural studies for both epithelium and cilia were performed, and the deformities in detailed electron microscopic images confirmed the diagnosis of PCD. Results: Four patients experienced the first symptoms shortly after the birth, 1 by the age of 1 and 1 by the age of 4 years. Except of 1 case that was diagnosed 2 months after the onset of disease, diagnosis delay was longer than 5 years in all patients. Consanguineous marriage was observed in the parents of all patients. Upper respiratory tract infections were documented for all patients. Conclusions: PCD should be considered as a probable underlying disorder in patients with bronchiectasis. Past medical history of otitis media and history of similar clinical findings in family members should raise suspicion toward PCD.

[Background] Common variable immunodeficiency (CVID) is a heterogeneous group of disorders, characterized by recurrent upper and lower respiratory tract infections and some noninfectious clinical complications., [Objective] To provide a detailed evaluation of respiratory presentations and complications in a cohort of Iranian patients with CVID., [Methods] A retrospective cohort study was conducted on 245 CVID patients who were recorded in the Iranian primary immunodeficiency disorders registry network. Respiratory manifestations were evaluated by reviewing clinical hospital records, immunologic findings, pulmonary function tests (PFT), and high-resolution computed tomography (HRCT) scans., [Results] Most of the patients (n = 208, 85.2%) had experienced at least 1 episode of acute respiratory manifestation, and pneumonia was observed in 31.6 % (n = 77) of cases as a first disease manifestation. During the follow-up, pneumonia, sinusitis, and otitis media were documented in 166 (68.6%), 125 (51.2%), and 103 (42.6%) cases, respectively. Abnormal PFT measurements were documented in 53.8% of patients. Among these patients, 21.5% showed restrictive changes, whereas 18.4% of patients showed an obstructive pattern. Bronchiectasis was the most frequent radiological finding, confirmed in 27.2% of patients. Patients with bronchiectasis were older at the time of immunodeficiency diagnosis (P < .001) and had longer diagnosis delay (P < .001) when compared with patients without bronchiectasis., [Conclusion] This study highlights the importance of monitoring the respiratory tract system even in asymptomatic patients. Pulmonary function tests and CT scans are the most commonly used techniques aiming to identify these patients early, aiming to reduce the rate of long-term respiratory complications., Funding Sources: This research was supported by the Tehran University of Medical Sciences, Tehran, Iran (grant no. 35741).

Airway and vessel characterization of bronchiectasis patterns in lung high-resolution computed tomography (HRCT) images of cystic fibrosis (CF) patients is very important to compute the score of disease severity. We propose a hybrid and evolutionary optimized threshold and model-based method for characterization of airway and vessel in lung HRCT images of CF patients. First, the initial model of airway and vessel is obtained using the enhanced threshold-based method. Then, the model is fitted to the actual image by optimizing its parameters using particle swarm optimization (PSO) evolutionary algorithm. The experimental results demonstrated the outperformance of the proposed method over its counterpart in R-squared, mean and variance of error, and run time. Moreover, the proposed method outperformed its counterpart for airway inner diameter/vessel diameter (AID/VD) and airway wall thickness/vessel diameter (AWT/VD) biomarkers in R-squared and slope of regression analysis.

Home mechanical ventilation is a promising option for children requiring long-term mechanical-assisted ventilation, while data on cost-effectiveness of this approach is limited.To investigate the cost-effectiveness of home mechanical ventilation in children requiring long-term mechanical-assisted ventilation.A retrospective cohort was conducted on 67 children (32 girls, 47.7%) requiring mechanical-assisted ventilation. Underlying diseases of children were congenital airway malformations in 24, cystic fibrosis in 4, severe laryngomalacia in 16, poly neuropathy syndrome in 6, mitochondrial myopathy in 5, hypoxic ischemic encephalopathy in 6, and cerebral palsy in 2. Children were admitted in pediatric intensive care units (ICU) for 2 weeks. After discharge, they were on home mechanical ventilation and were followed for 1 year. Data on daily costs of admission at ICU, rehospitalizations, weaning, educational performance and muscle strength were gathered.Mean age of children at time of initiation of mechanical-assisted ventilation was 5.8 years (ranged from 2 months to 15 years). Mean number of re-hospitalizations was 3.4_4.9 times with mean duration of 9.44_2.53 days. Of children on mechanical ventilation, 1 attended school, 2 were weaned, and 21 experienced improvement in muscle strength. No fatal or serious complications were observed while children were on home mechanical ventilation. Mean costs of daily ICU admission was 912_1028 $, while the mean daily cost of home mechanical ventilation was 60.86_4.95 $ (p0.001).Home mechanical ventilation is more cost-effective compared to ICU admission for only mechanical-assisted ventilation.p/p.

BACKGROUND: Some evidence suggests that childhood croup could be associated with increased incidence of adulthood bronchial reactivity, but its significance is uncertain. The aim of the present study was to evaluate the long-term outcome of early life croup. PATIENTS AND METHODS: This case–control study was conducted in 2010–2012 in Isfahan, Iran. The case group consisted of 164 adolescents with a history of severe croup in early life and an equal number of healthy controls without any history of croup or other chronic or recurrent respiratory diseases. The two groups were compared according to pulmonary function tests and bronchial reactivity (exercise challenge test). Statistical analyses were performed using the SPSS software package, version 20 (SPSS Inc., Chicago, IL, USA). P < 0.05 was considered significant. RESULTS: Baseline spirometric values (forced expiratory volume in 1st s (FEV1), forced volume capacity (FVC), FEV1/FVC, and forced expiratory flow at 25%–75% (FEF25–75) were similar in case and control groups. A reduction in FEV1 and FEF25–75 after exercise challenge test was seen in 9% and 12.8% of patients, respectively, whereas this was reduced in only 4.2% and 6.1% of the controls (P = 0.034 and P = 0.021, respectively). CONCLUSION: Our findings suggest that childhood croup might be a predisposing factor for bronchial hyperreactivity in adulthood. Longitudinal studies are necessary to confirm the clinical significance of these findings.

Primary immunodeficiency disorders (PIDs) are caused by 1 or more defects of the immune system. Patients are more likely to experience recurrent and/or severe infections and tend to develop a wide range of complications. Respiratory diseases are the main and initial manifestation in most cases and the most common complication. Pulmonary complications cause significant morbidity and mortality in patients with PIDs. Early diagnosis and appropriate treatment can prevent or at least slow the development of respiratory complications. Since the spectrum of pulmonary complications in PIDs is broad, we divided pulmonary complications into upper respiratory complications (eg, sinusitis, otitis media, and laryngeal angioedema) and lower respiratory complications (eg, pneumonia, bronchitis, bronchiectasis, interstitial lung diseases, organizing pneumonia, pulmonary adenopathies and malignancies, hyperreactive airway diseases, pulmonary dysgenesis, and adverse reactions to treatment). This review covers the main respiratory manifestations in patients with PIDs.