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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:: Cystic fibrosis (CF) is an autosomal recessive disease characterized by a progressive and recurrent lung infection. Objective:: This study aimed to evaluate the levels of pro-inflammatory cytokines and their correlations with lung function in CF patients. Methods:: A matched case-control study was conducted among 18 CF (children and adults) and 18 control patients (age and gender-matched) who were admitted to Masih Daneshvari Hospital (Tehran, Iran). The controls had no obvious inflammatory lung disease. The samples of bronchoalveolar lavage (BAL) fluid, serum, and sputum of participants were collected to determine concentrations of inflammatory cytokines such as interleukins (IL-8, IL-1β) and tumour necrosis factor- alpha (TNF-α) using enzyme-linked immunosorbent assay (ELISA). Spirometry was applied and functional pulmonary indices [forced vital capacity (FVC) and forced expiratory volume in 1 second (FEV1)] were assessed. Results:: The mean age of CF patients was 15.43 ± 5.970 years (range 4-24). The FVC in a majority of the CF patients (66.7%) was below 60% and only 33.3% of the patients exhibited normal or mild respiratory dysfunction. There were significant differences between FVC and FEV1 measurements before and after bronchoscopy. In addition, IL-8 levels in all three samples (serum, sputum, and BAL) of CF patients and levels of IL-1β and TNF-α in BAL and sputum samples of CF patients were significantly higher than the control group (p Conclusion:: Increased IL-8 and TNF-α levels seemed to be associated with signs of clinical deterioration and might be useful as diagnostic markers.

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.

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: 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.

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 Finding a valid diagnosis is mostly a prolonged process. Current advances in the sector of artificial intelligence have led to the appearance of expert systems that enrich the experiences and capabilities of doctors for making decisions for their patients. Objective The objective of this research was developing a fuzzy expert system for diagnosing Cystic Fibrosis (CF). Methods Defining the risk factors and then, designing the fuzzy expert system for diagnosis of CF were carried out in this cross-sectional study. To evaluate the performance of the proposed system, a dataset that corresponded to 70 patients with respiratory disease who were serially admitted to the CF Clinic in the Pediatric Respiratory Diseases Center, Masih Daneshvari Hospital in Tehran, Iran during August 2016 to January 2017 was considered. Whole procedures of system construction were implemented in a MATLAB environment. Results Results showed that the suggested system can be used as a strong diagnostic tool with 93.02% precision, 89.29% specificity, 95.24% sensitivity and 92.86% accuracy for diagnosing CF. There was also a good relationship between the user and the system through the appealing user interface. Conclusion The system is equipped with information, knowledge, and expertise from certified specialists; hence, as a training tool it can be useful for new physicians. It is worth mentioning that the accomplishment of this project depends on advocacy of decision making in CF diagnosis. Nevertheless, it is expected that the system will reduce the number of false positives and false negatives in unusual cases.

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.

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.

In this present study, for the first time, we evaluated the cystic fibrosis (CF) patients for the Scedosporium species and their antifungal susceptibility against eight antifungal agents. During one-year period, 90 Sputum samples were collected from Iranian CF patients. All samples were evaluated by direct microscopic examination, culture onto four different media including Malt extract agar, Inhibitory mold agar, Brain Heart Infusion and Scedo-Select III. The mold isolated fungi were identified by PCR-Sequencing of ITS and β-tubulin genes. In-vitro antifungal susceptibility was performed according to the Clinical & Laboratory Standards Institute (CLSI) M38-A2 guidelines. Out of 90 CF patients, 47 (52.2%) were male. The age of the patients ranged from 1 to 34 years (median of 15.84 ± 7.41 years). Overall, 3 (3.3%) cases were positive for Scedosporium spp. of which two isolates were characterized as Scedosporium boydii and one isolate as S. ellipsoideum. Among Aspergillus genus, A. flavus (29.4%) was the most prevalent species followed by A. tubingensis (24.7%), A. niger (17.0%) and A. fumigatus (14.5%). The minimum effective concentration ranges of micafungin, anidulafungin, and caspofungin were 0.008–0.031 μg/mL, 0.0625–0.25 μg/mL, and 0.0625–0.25 μg/mL, respectively. All isolates of Scedosporium species showed high minimum inhibitory concentration to the triazoles tested, except voriconazole. Our results showed that A. flavus and Scedosporium species are the most prevalent molds isolated from CF patient populations in Iran. Our findings have also showed that Scedo-Select III can be used as a reliable culture media for isolation of Scedosporium spp. in clinical samples.

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.

A 12-year-old boy with cystic fibrosis (CF) and a history of glucocorticoid-dependent allergic bronchopulmonary aspergillosis (ABPA) was referred to our hospital. The ABPA was diagnosed when he was 8 years old and he had been treated with several course of oral glucocorticoids for recurrent exacerbations. He was readmitted when aged 12 with a history of worsening shortness of breath and chest tightness. A recurrence of ABPA was diagnosed based on eosinophilia and elevation of Aspergillusspecific IgE and IgG, and total IgE. Thoracic high-resolution computed tomography (HRCT) showed central bronchiectasis with parenchymal infiltrates. The treatment started with itraconazole and oral corticosteroid. After 2 months of treatment, he was re-admitted to the hospital due to a progressive worsening of respiratory symptoms. Chest HRCT revealed the a sub segmental atelectasis in the left lung. Microscopic examination of sputum and BAL samples demonstrated septate hyphae consistent with Aspergillus species. Sputum and BAL culture yielded Aspergillus ochraceus and Aspergillus terreus, which were both sensitive to itraconazole and voriconazole. The treatment was switched to voriconazole and the patient showed significant clinical, serological and mycological improvement after three months. This case shows that voriconazole may be used as an alternative for treatment of ABPA due to Aspergillus terreus.

Background: Due to the predisposing conditions in patients with cystic fibrosis (CF) caused by defective mucociliary clearance facilitating colonization and invasion with Candida species has dramatically increased. Traditional methods for identifying problems are imminent and time-consuming. Therefore, molecular techniques utilizing amplification of target DNA provide quick and precise methods for the diagnosis and identification of Candida species. Therefore, the aim of the current study was to identify the most medically common isolated Candida species from the air way of CF patients by PCR-RFLP and amplification of HWP1 gene. Materials and Methods: A total of 42 CF patients presenting symptoms who referred to pediatric respiratory diseases research center were screened for the presence of Candida spp. The isolates initially were phenotypically identified and confirmed by molecular approaches based on restriction fragment length polymorphism (PCR-RFLP) for the discrimination of C. albicans of non-albicans and the amplification of HWP1 gene for the discrimination of C. albicans from C. dubliniensis and C. africana was conducted. Results: The results show that C. albicans was the most frequently isolated species (83.8%) followed by non-albicans included C. parapsilosis (7.1%), C. glabrata (3.2%), and C. tropicalis (3.2%). The restriction patterns of each Candida species were perfectly specific. Since MspI could not discriminate between the three morphological related species, C. albicans, C. dubliniensis and C. africana, we used PCR amplification of HWP1 gene, which (7.1%) species from C. albicans identified as C. dubliniensis, however C. africana strains were not found. Discussion: The present study found that C. albicans as predominant species wereisolated from the CF patients. It could be concluded that molecular diagnostic methods are reliable and would be useful for the identification of medically important Candida species in clinical samples. Therefore, considerable attention has been paid to the prevention and treatment of microbial growth, which has resulted in the improvement of patient management.

Identification of modifier genes influencing phenotype of cystic fibrosis (CF) patients has become a challenge in CF pathophysiology, prognostic estimations and development of new therapeutic strategies. The aim of this study was to explore the association between four genetic polymorphisms of three modifier genes with CF, by comparing their alleles, genotypes and haplotype frequencies in patients and controls. In this favor, two regulatory polymorphic loci in TNFα promoter (−857C/T, rs1799724 and -238A/G, rs361525) and two functional polymorphic loci in TNFR1 (+36A/G, rs767455) and TNFR2 (+587T/G, rs 1061622 ) were genotyped in 70 patients and 79 controls, using PCR-RFLP. Clinical pulmonary data were also recorded from all studied patients. Results indicated that an association was observed between both T allele and CT/TT genotypes of TNFα (P = 0.0005, OR = 7.06, 95% CI = 3.71–13.45) with CF under dominant model of inheritance. GG genotype of TNFR2 +587 (P = 0.0005, OR = 4.92, 95%CI = 2.34–10.34) was significantly associated with CF using recessive model. Consistently, more severe pulmonary disorder was found for patients carrying either T dominant allele of TNFα −857 or GG genotype of TNFR2 +587 polymorphic sites. Despite an association of A-T and G-T haplotypes with CF, no significant association was found between these haplotypes and clinical parameters of CF. Overall, TNFα −857 T allele and GG genotype of TNFR2 +587 were more frequent in CF patients compared to healthy controls and hence, they showed an association with CF and severe pulmonary phenotype in Iranian patients.

Introduction: Cystic fibrosis is a chronic disease with multiple organ involvement and chiefly results in chronic respiratory infections, pancreatic insufficiency and associated complications. The age at diagnosis, clinical presentation, rate of disease progression and prognosis is variable among patients. This study is designed to evaluate the behavior of disease to provide epidemiologic data for early recognition and proper management. Material and methods: The study was designed as an active surveillance of 192 patients diagnosed with cystic fibrosis in a tertiary lung disease centre between 2008 and 2015. The diagnosis of cystic fibrosis was established in all patients accordingly to conventional criteria, including two positive sweat chloride tests and clinical signs and symptoms. Demographic, clinical and laboratory data were obtained from these patients in each hospitalization and also every follow-up visit and carefully evaluated for complications of this chronic disease. Results: The majority of patients showed positive culture for Pseudomonas aeroginosa. Bronchiectasis was the most prevalent finding in chest CT scan. 44.3% of patients had been treated for allergic bronchopulmonary aspergillosis and all had sinus disease. Increased pulmonary artery pressure was observed in 40% of patients with cystic fibrosis. 33 patients died which consisted 17.1% of all the patients.The mean age of mortaliy was 18.15 year. Conclusions: The clinical outcome of cystic fibrosis is variable in different countries which may reflect environmental influences and the role of early diagnosis on long term outcomes. However, the role of early diagnosis in long-term outcomes of the disease can not be ignored.

Background: Cystic fibrosis is a complex progressive disease which assessing its progression and severity is essential. For this purpose there are scoring systems available to evaluate the disease severity. Objectives: The aim of the present study was to determine the clinical status of CF patients using shwachman score system in the pediatric pulmonary ward of Masih Daneshvari Hospital. Patients and Methods: A cross-sectional study was conducted to evaluate the clinical status with shwachman score system. 23 patients ranging from 5 to23 years were enrolled in this study. All data was extracted objectively from Shwachman-Kulczycki scoring system. Results: The overall mean Shwachman-Kulczycki score was 53.48 ± 13.8. Total scores of < 40 (severe), 41-55, 56-70, and 71-85 were detected in 1.7%, 39.1%, 30.4% and 8.7% of patients respectively. None of the patients were categorized in the excellent range. We found no statistically significant correlation between mortality rate and clinical score (P = 0.136). However, the results showed a statistically significant correlation between mortality rate and Shwachman score, (P = 0.02). Conclusions: Shwachman-Kulczycki score is an easily applied scoring system which can reflect the clinical status of patients objectively. However, concurrent use of other evaluating tools such as pulmonary function test (PFT) and Computed tomography scoring systems provide a more robust monitoring and a reliable evaluation tool.

Article type: Original Article Background: Cystic fibrosis (CF) is a genetic disease with an autosomal recessive pattern of inheritance. CF caused by a mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene and characterized by impaired transport of chloride ions across the cell membrane. Staphylococcus aureus, Pseudomonas aeruginosa, and Burkholderia cepacia have been identified in the cultures of respiratory secretions of CF patients, and infections of these microorganisms are associated with high rates of morbidity and mortality. In patients with CF, severe inflammation of the airway can cause advanced bronchiectasis, which may result in respiratory failure and death. Objectives: This study aimed at evaluating the clinical findings of laboratory tests, bacterial colonization, and drug resistance in children and young adults with CF who hospitalized at the Pediatric Pulmonary Department of Masih Daneshvari Hospital, Tehran, Iran.

Assessing the prognosis of cystic fibrosis (CF) and evaluating the effect of indicators of mortality is very important in predicting the life expectancy of the CF patients.Determining the effect of seven variables including sex, Forced Expiratory Volume in one second (FEV1), Body Mass Index (BMI), bacteriology, hemoglobin (Hb), pulmonary arterial pressure (PAP) and the number of previous admissions on the survival of 27 patients admitted in Pediatric Pulmonary Ward of Masih Daneshvari Hospital in 2007-2009.27 CF patients were enrolled in a retrospective cross-sectional study. Patients data were collected during 2 years of study. Data of patients who died and those who remained alive were compared by independent samples t-tests and Chi-square.Twenty seven CF patients (11 female, 10 male) with age range of 5-19 years and mean age of 13.11 +/- 4.69 were studied. There was no difference in age, sex, FEV1, BMI, Hb between the deceased and alive group (p0.05). Mean PAP for expired patients and alive patients was 40 +/- 15.1 and 68 +/- 11.5 respectively. The number of admissions during last 6 months was dominant in those patients who died. 50% of the alive patients were colonized with Pseudomonas. This is compared to deceased patients which 100% were colonized with Pseudomonas. There was a strong correlation between death and number of previous admissions, PAP and Pseudomonas infection (p0.05).Pseudomonas infection, number of previous admissions and the severity of pulmonary hypertension has shown to be the major predictors of mortality in our study.