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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.

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 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.

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 Hospitalized pediatric patients are at an increased risk of experiencing potential drug-drug interactions (pDDIs) due to polypharmacy and the unlicensed and off-label administration of drugs. The aim of this study is to characterize clinically significant pDDIs in pediatric patients hospitalized in a tertiary respiratory center. Methods A retrospective analysis of medications prescribed to pediatric patients admitted to the pediatric ward (PW) and pediatric intensive care unit (PICU) of a respiratory referral center was carried out over a six-month period. The pDDIs were identified using the Lexi-Interact database and considered as clinically relevant according to the severity rating as defined in the database. Frequency, drug classes, mechanisms, clinical managements, and risk factors were recorded for these potential interactions. Results Eight hundred and forty-five pDDIs were identified from the analysis of 176 prescriptions. Of the total pDDIs, 10.2% in PW and 14.6% in PICU were classified as clinically significant. Anti-infective agents and central nervous system drugs were the main drug classes involved in clinically significant pDDIs as object and/or precipitant drugs. A higher number of medications [odds ratio (OR): 4.8; 95% confidence interval (CI): 2.0–11.4; p < 0.001] and the existence of a nonrespiratory disease, which led to a respiratory disorder (OR: 3.8; 95% CI: 1.40–10.4; p < 0.05), were the main risk factors associated with an increased incidence of pDDIs. Conclusions A high and similar risk of pDDIs exists in pediatric patients with respiratory disorders hospitalized in PW and PICU. The patients prescribed a higher number of medications and presenting respiratory symptoms induced by a nonrespiratory disease require extra care and monitoring. Pediatricians should be educated about clinically significant DDIs for highly prescribed medications in their settings in order to take preventive measures and safeguard patient safety.

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.

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.

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: Empyema in pediatric, if not treated adequately, would be an aggressive disease with many complications. Complete treatment of empyema includes broad antibiotics administration during the first week of the disease. However, in some cases, organized fibrinous tissue prevents antibiotic penetration and surgical intervention is required. Objectives: The aim of this study was to compare the duration of admission, duration of intravenous antibiotic therapy, time to response to treatment, and outcomes of medical and surgical interventions. Patients and Methods: In a retrospective, observational study, 60 patients with empyema were included; 35 were treated medically (medical group) and 25 surgically (surgical group). Results: Age and sex were not significantly different between the two groups. Only one of the patients in the medical group and one in the surgical group had immune deficiency. Coughing was presented in 25 patients in medical group and in 17 patients in surgical group as a symptom. Hospitalization days of were significantly lower in the medical group (12.4 ± 5.5) compared with the surgical group (20.4 ± 5.2) (P = 0.041). Duration of intravenous antibiotic therapy was not significantly lower in the medical group compared with the surgical group (P = 0.085). Time to response to treatment (from the admission day to the clinical improvement) was significantly lower in the medical group compared with the surgical one (P = 0.024). Mortality was 0% in both groups. Conclusions: If medical treatment gets started early in the course of empyema, prognosis would be excellent. Besides, there was no significant difference in short- and long-term outcomes between medical or surgical interventions.

Background: Congenital lung malformation (CLM) is a rare condition with high incidence of mortality and morbidity. CLM is often discovered incidentally on routine prenatal ultrasound or other postnatal imaging tests. Depending on the size of lesion and degree of functional impairment, prognosis is different. Today, fetal lung abnormalities are increasingly detected prenatally by ultrasound imaging. Objectives: This study aimed to evaluate the CLM cases in pediatric pulmonary ward of Masih Daneshvari Hospital during an 8-year period from 2002 to 2010. Materials and Methods: In a retrospective study, 21 patients (12 females, nine males), diagnosed with CLM, were enrolled. The mean age of participants was 7.3 years (ranging from 1 month to 16 years). All patients were histopathologically diagnosed with CLM and their demographic data including age, sex, and clinical signs and symptoms were recorded. To confirm the diagnoses and rule out the differentials, patients underwent bronchoscopy, angiography and other proper diagnostic procedures. Afterwards, they were divided into several subgroups based on the malformation and type of involvement, including agenesia, hypoplasia, aplasia, bronchogenic cyst, and sequestration. Results: We reported two agenesis, five hypoplasia, six aplasia, two bronchogenic cyst, and six sequestration cases. All patients were symptomatic at the time of diagnostic workup. The most common clinical signs and symptoms were respiratory distress and recurrent pulmonary infections. Conclusions: Considering the wide range of clinical manifestations from no clinical symptom to severe respiratory distress at birth, imaging including chest X-ray, CT scan with contrast and MRI are of great values for early diagnosis of CLM. Early diagnosis could provide a more appropriate timing of evaluation and treatment options, lower mortality and morbidity, and better prognosis.