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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.