TANAFFOS
Year:2018 | Volume:17 | Issue:2|Papers Count:13

Cystic Fibrosis (CF) is the most common lethal autosomal recessive disease that affects many organs including, lung, pancreas and liver. Cystic fibrosis is a monogenic disease and occurs in the white Caucasians. Massive neutrophil granulocyte influx in the airways is one of the characteristics of CF. Extracellular Vesicles (EVs), microvesicles, and exosomes are vesicles released from cells into extracellular space of the body and are able to influence other cells by different methods. They have an important role in the intracellular communication by transferring information between donor and recipients cells. Granulocytes are known as the main source of microparticles in the CF patients. Microparticles derived from neutrophils are associated with the extensive neutrophil influx into airways and aggregation at the epithelial surface of the CF patient’ s respiratory tract. Exosomes are found in almost all body fluids, such as urine, sputum, Bronchoalveolar Lavage (BAL), milk, Cerebrospinal Fluid (CSF), plasma and sputum. Examination of exosomes derived from CF patients may be helpful in the characterization of pathogenesis of disease in detail. In this mini review, we have summarized the role of microparticles and exosomes in pathogenesis of CF and finally discussed the feasibility of this particle in treatment approaches.

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: Cardiovascular disease is one of the most common disorders associated with chronic obstructive pulmonary disease (COPD). There are few studies on the effects of physical exercises, especially aerobic exercises, on serum levels of apolipoprotein A1 and apolipoprotein B in patients with COPD. The current study aimed at determining the effect of aerobic exercises on serum levels of apolipoprotein A1 and B and apo-A1/apo-B ratio. Materials and Methods: In the current randomized, controlled, clinical trial, with a pretest posttest control group design, 22 males with COPD were randomly assigned to the aerobic exercise and control groups. The aerobic exercise program was performed within two months based on three 30-40-minute sessions per week. Serum levels were measured and evaluated before and after aerobic exercises. Data were analyzed using the paired samples t test. Results: In the aerobic exercise group, the mean of Apo A1 and Apo B after the intervention (169. 36± 5. 42 and 93. 63± 5. 24 mg/dL, respectively) was significantly higher than that of before the intervention (146± 6. 09 and 83. 27± 4. 44 mg/dL, respectively) (P-value=0. 001). However, apoA1/Apo B ratio did not significantly change after the intervention compared with that of before the intervention (1. 85± 0. 10 vs. 1. 80± 0. 13) (P >0. 05). There was no significant change in the mean Apo A1 and Apo B levels and Apo A1/Apo B ratio after the intervention in the control group. Conclusion: Regular aerobic physical exercises are effective in increasing the serum level of Apo A1 in patients with COPD. Due to the proven protective role of Apo A1 in patients with COPD, this biomarker can improve respiratory efficacy in such patients.

Background: The aim of the present study was threefold: to assess the association between baseline FEF25-75 and Airway Hyper-responsiveness (AHR), to specify whether a decrease in FEF25-75 may reflect severe hyper-responsiveness, and finally to confirm a FEF 25-75 cut-off value. Materials and Methods: In a cross sectional study in Imam Khomeini Hospital, Ahvaz, patients suffering from respiratory symptoms due the 2013 autumn rainfall with normal FEV1 and FEV1/FVC were evaluated by methacholine challenge test. Those with PD20<1000, 10002000 μ g were classified as severe, moderate and mild AHR, respectively. Data were analyzed using Chi-square, Independent t-test, One-way ANOVA and Receiver Operating Characteristic (ROC) curve. Results: Among the 234 patients, mean baseline FEF25-75 was 84. 2± 22. 7% for 54 patients having a negative bronchial provocation test result and 70. 9± 19. 2% for 179 patients with a positive bronchial provocation test result (P < 0. 0001). No change was observed in the median PD20 among patients with a higher baseline FEF25-75. ROC analysis showed that FEF25-75 could potentially be a predictor of AHR, but it could not confirm the cut-off value of FEF25-75 for these patients. Conclusion: When asthma begins, AHR could be predicted by impaired FEF25-75 with normal FEV1 and FEV1/FVC. However, we could not determine a cut-off value, and no association was found between a greater impairment of FEF25-75 and a more severe AHR.

Background: Inhaled corticosteroids have been successfully used to improve lung function. Budesonide nebulizer suspension and Budesonide inhalation suspension are two inhaled corticosteroids used in treating chronic obstructive pulmonary disorder (COPD). We compared the efficacy of Budesonide nebulizer suspension and Budesonide inhalation suspension in the treatment of patients with acute exacerbations of COPD, to prioritize the two treatments. Materials and Methods: In our study, 90 patients were recruited and divided randomly into two groups: Budesonide nebulizer suspension (Pulmicort Nebulizer) and Budesonide inhalation suspension (Pulmicort Turbuhaler) groups. Demographic characteristics, patient clinical information, and paraclinical data including arterial blood gases (ABG) and O2 Saturation were recorded both at the beginning of hospitalization and on the seventh day of treatment. The collected data were analyzed through independent sample t-test, paired sample t-test, chi-square test, and linear regression using SPSS version 20. Results: Our findings revealed that there were no significant differences in O2SAT or ABG between the two groups at baseline or after seven days of treatment (P > 0. 05). After seven days of treatment, mean O2SAT and arterial blood gases had increased significantly in each group (P < 0. 001). Conclusion: This study found no significant difference between the two groups with respect to the method of treatment. Therefore, Turbuhaler can be used at home by patients so, it leads to elimination of costs and hospital stays.

Background: Asthma is a common long term inflammatory disease during adolescent. Absence of school education and reduction of mental and social mindedness are among the most common problems found in adolescents with asthma. Therefore, the present study was aimed to examine the effect of Roy adaptation model on controlling maladaptation behaviors in adolescents with asthma. Materials and Methods: This study is a semi-experimental research that was conducted with the participation of all adolescents with asthma referred to the Asthma and Allergy Clinic of Shariati Hospital. Random sampling was used for a total of 64 adolescents to have two groups of intervention and control. Data collection was through a questionnaire based on the Roy’ s Adaptation Model. Over six weeks, adolescents were trained in six two-hour sessions. Data were analyzed by descriptive and analytical statistics consisting of Mann-Whitney, ANOVA, paired t-test and independent t-test. Results: The mean age of adolescents with asthma in the intervention and control groups was 15. 8± 3. 5 and 14. 8± 3. 5 years, respectively. Also, the mean score of maladaptation behaviors in four physiological, self-concept, role-function and interdependence modes had a significant difference in intervention group before and after training (p<0. 001), but there was no significant difference in all modes before and after intervention in control group (p>0. 05). Conclusion: Given the effect of Roy adaptation model on the different aspects of maladaptation behaviors in adolescents with asthma, it is recommended to use this model as a healthcare intervention for controlling maladaptation behaviors in adolescents with other chronic disease.

Background: Ventilator-Associated Pneumonia (VAP) is an important cause of morbidity and mortality in patients admitted to Intensive Care Unit (ICU). The current study conducted to estimate VAP incidence, attributable mortality and case fatality rate, cost, so related factors can affect the outcome in patients. Materials and Methods: In this descriptive longitudinal study, demographic, clinical and para-clinical data were collected and attributable mortality and case fatality rate was estimated. Multivariable analysis was done to predict the possible risk factors on the outcome of VAP patients. Also, patients' survival curve was plotted based on their length of ICU stay. Finally, the additional cost due to VAP in ICU was estimated. Results: Totally, 8% ICU admissions were affected by VAP and 4% expired during the ICU stay. Further, the attributable mortality rate of VAP was high as compared with standard mortality rate. The most case fatality rate was for Acinetobacter sp. (n=17 60. 7%). In multivariable logistic regression analysis, age greater than 40 years, more than 96 hours mechanical ventilation and uncontrolled diabetes mellitus were predictor factors of higher mortality. Inverse association between survival time and ICU length of stay was reported. Finally, the additional cost of VAP was estimated of about 700 US$ per patients. Conclusion: According to the results, strategies to prevent mortality by reducing the duration of ventilation and ICU length of stay should be performed. Also, mandatory fees for the family and the healthcare system should be planned.

Background: Despite various applications of tele-ICU, there are still many questions about its costs and advantages in ICU. Some of its advantages are accelerating consultations and bringing physicians’ satisfaction from tele-consultation outcomes. The aim of this study is to discuss these advantages. Materials and Methods: Initially a telemedicine network was implemented and in the case of having no related specialist, the physicians used telemedicine network to perform specialized tele-consultation to thoracic surgery ICU patients. ICU patient’ s documents during a year before tele-consultation were studied and delay time in consultation was recorded and compared between the two phases. Finally, the physicians’ satisfaction with tele-consultation was evaluated. Results: Fifty-eight tele-consultations in various medical fields were carried out, of which 27 were neurology cases. From the time of receiving a consultation request to its performance, the mean time was 1. 3 days in tele-consultation. Tele-consultations were given 2. 5 times faster than face to face method. In evaluation of physicians’ satisfaction, 82. 75% of them were fully satisfied from tele-consultation, 12. 06% were partly satisfied and 5. 17% were not satisfied. Conclusion: Since the length of hospitalization in ICU is crucial due to heavy costs of treatment, high risk of contamination and limited beds, performing timely consultation is a key factor in reducing hospitalization period. Tele-consultation in thoracic surgery ICU not only accelerates patient care, but also results in higher physician satisfaction.

Background: One of the main challenges of surgical treatment in Obstructive Sleep Apnea (OSA) is identifying the correct site of upper airway obstruction in an individual patient. Drug-Induced Sleep Endoscopy (DISE) in sedated patients with obstructive sleep apnea is the technique of choice for revealing anatomic and dynamic collapsible areas. Materials and Methods: In a prospective cross-sectional study adult patients with OSA documented by polysomnography were evaluated by sleep endoscopy. DISE had been done by an otolaryngologist in the setting of operating room during infusion of propofol and after the start of snoring. Endoscopic findings were recorded and evaluated from the aspect of obstruction level, severity, and multiplicity. Results: Twenty OSA patients (60% men) with mean± SD age of 38. 9± 9. 26 years and mean Body Mass Index (BMI) of 26. 57 kg/m2 were included in the study. OSA was severe in 11(55%) and moderate in 5(25%) subjects. Unilevel airway collapse was observed as retropalatal in 4(20%) and retrolingual in 3(15%) subjects. Multilevel collapse had been observed in the other 13(65%) patients. Most patients (65%) had multilevel obstruction especially those with BMI>30 (p<0. 05). With increasing BMI, obstruction changed from unilevel to multilevel. None of the subjects showed complications with propofol or endoscopy procedure. Conclusion: Our study showed DISE is safe, easy to perform, and informative in OSA patients. In particular, we observed a significant association between obesity and multilevel upper airway collapse.

Background: We report a case series of two patients in the intensive care unit with massive pulmonary embolism and obstructive shock who had resolution of shock after repeated administration of alteplase. Case Summaries: Both patients were initially dosed 10 mg of alteplase followed by infusion of 90 mg over 2 hours, but remained in obstructive shock requiring significant inotropic and vasopressor support. Both patients were deemed poor candidates for embolectomy. The first patient received repeated doses reaching an accumulative dose of 200 mg alteplase over 15 hours. The second patient received an accumulative dose of 250 mg alteplase over 36 hours. Both patients had resolution of shock within 24 hours of repeated administration of alteplase, but also experienced significant drops in hemoglobin, which were supported with transfusions. They were transferred out of the intensive care unit after resolution of obstructive shock and hemorrhage. The first patient died one week after transfer from the intensive care unit due to invasive candidiasis and septic shock. The second patient was weaned from the ventilator and discharged home. Conclusion: Patients with obstructive shock secondary to massive pulmonary embolism despite a one-time dose of alteplase and poor candidacy for embolectomy may benefit from repeated doses of alteplase. Due to the short half-life, repeated administration of thrombolytic may be appropriate for younger patients without absolute contraindications to thrombolysis, but future studies are needed to identify the optimal patient population.

Intensive care unit-acquired weakness is characterised by severe impairment of muscle function that often arises after prolonged mechanical ventilation, difficult weaning, and severe sepsis. Elastic band exercises constitute an inexpensive and simple technique that is quite appealing for implementation in a “ protected environment” such as the intensive care unit; however, elastic band application in the intensive care unit and in critical patients has not yet been described. A 72-year-old male was referred to the respiratory intensive care unit for hypoxemic respiratory failure due to acute respiratory distress syndrome. Upper limb active exercises were performed using an elastic band exploring three main movement rays: abduction, forward flexion, and external rotation. At discharge, major improvements were observed for upper limb activities. The patient was also able to maintain a sitting position at the edge of the bed starting from day 27. We found that an elastic band exercise program in a critical ill patient recovering from intensive care unit-acquired weakness was a suitable, safe, viable, and inexpensive therapeutic option to preserve residual upper limb motor activities and improve trunk control.

Most authors believe that the optimal treatment for pulmonary hydatid cyst is surgery. Albendazole has been used as a prophylactic measure for reducing recurrence rate but there are some controversies about this strategy. Some researchers have described the increased risk of spontaneous rupture of cysts following albendazole treatment. In this case report, we present a case of spontaneous rupture of pulmonary hydatid cyst with fatal outcome that may be the adverse cause of albendazole.

Chronic Eosinophilic Pneumonia (CEP) is an idiopathic disorder characterized by an abnormal marked accumulation of eosinophils in the interstitial and alveolar spaces of the lung. CEP is typically suspected in a patient with progressive dyspnoea over one to four months and a chest radiograph showing bilateral peripheral or pleural-based opacities. Dominant extrapulmonary manifestations in CEP are rare. We report a 44-year-old Iranian woman with progressive dyspnea, peripheral chest opacity, and cardiac involvement. A diagnosis of CPE was considered base on clinical and para clinical criteria.