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A recent survey conducted in Egypt aimed to assess the knowledge and accessibility of resources related to cystic fibrosis (CF) among physicians in the country. The survey found that awareness of CF and screening in Egypt is low, resulting in delayed diagnoses and worse clinical outcomes. The study also highlighted the need for educational initiatives to improve CF knowledge among physicians, particularly those working in the Ministry of Health. Additionally, the survey emphasized the importance of establishing multidisciplinary teams and implementing quality improvement initiatives to enhance CF care in Egypt. [Extracted from the article]

Systemic lupus erythematosus (SLE) is a common autoimmune disease marked by the formation of apoptotic debris and the presence of autoantibodies that target nuclear components. At this moment, the actual cause of SLE is uncertain. Genetic variables have been well proven to have a significant role in the propensity of SLE. This study aimed to investigate the effect of (ZNF76) rs (10947540) and (SCUBE) rs (1888822) gene polymorphism in patients with systemic lupus erythematosus. A case control study has been carried out at Medical Biochemistry & Molecular biology and Rheumatology unit of Internal Medicine Departments, Faculty of Medicine, Menoufia University, Egypt, for 1-year duration between 1 June 2022 and 1 June 2023. Sixty patients were females (75%) and twenty patients were males (25%). Their ages ranged from 19 to 53 years. Their disease durations ranged from 7 months to 20 years. The findings indicated that the TC genotype of the ZNF76 rs10947540 gene increases the risk of SLE by 2.274-fold, while the dominant TC + CC increases the risk by 2.472-fold, and the C allele increases the risk by 2.115-fold. Additionally, the results showed that the TT genotype of the SCUBE3 rs1888822 gene increases the risk of SLE by 3.702-fold, the dominant GT + TT increases the risk by 2.304-fold, and the T allele increases the risk by 2.089-fold, while the GT genotype increases the risk by 1.918-fold. The study revealed significant associations between the genotypes of these polymorphisms and certain clinical parameters in SLE patients. These findings highlight the potential genetic contributions to SLE susceptibility and its clinical manifestations, providing valuable insights for future research and potential personalized approaches to the management of this complex autoimmune disease. [ABSTRACT FROM AUTHOR]

Background: Different respiratory sampling methods exist to identify lower airway pathogens in patients with cystic fibrosis (CF). Bronchoalveolar lavage (BAL) is considered the "gold standard.". Because BAL is invasive and cannot be repeated frequently, identification of lower respiratory tract pathogens in CF patients is challenging. Other sampling techniques are sputum, cough, and nasal swabs. The aim of the work: To differentiate between the microbiological results of bronchoalveolar lavage (BAL), sputum and cough swabs in CF children and to evaluate their accuracy in detecting lower respiratory tract organisms. Patients and Methods: Microbiological results detected by these different sampling methods were compared in all enrolled CF patients (n=31). Specificity, sensitivity, positive (PPV), and negative (NPV) predictive values were calculated. Results: Regarding microbiological results, Concordance was found between BAL and sputum as regards Pseudomonas spp., Klebsiella spp. and Enterobacter spp., except for Staph aureus (P 0.039). overall low sensitivity (7.1%) and low accuracy (16.1%) of cough swabs to detect lower airway pathogens [Pseudomonas spp., Staphylococcus aureus spp., Klebsiella spp. and Enterobacter spp.] in children with CF followed by sputum analysis as it showed sensitivity (85.7%) and accuracy (80.6%) while BAL showed highest sensitivity (86.2%) and highest accuracy (82.9%). Conclusion: Our findings suggested that sputum samples showed a good bacteriologic concordance with BAL in detecting lower airway pathogens, While Cough swabs showed the lowest accuracy in detecting the presence of CF pathogens in clinically stable CF children. However, BAL should be performed in symptomatic patients who could not expectorate and had a negative cough swab and sputum sample. [ABSTRACT FROM AUTHOR]

Background: Ang II inhibits fibrinolysis and promotes the production of thrombin. The elevation in Ang II levels is substantially linked to lung injury and viral load in cases with severe Corona Virus Infectious Disease 2019 (COVID-19). Aim of the work: This study aimed to determine the level of serum Ang II as a diagnostic biomarker of COVID-19 severity and a prognostic biomarker for the outcome in confirmed COVID-19 children. Patients and Methods: This case-control study was carried out on 45 positive COVID19 Real-time polymerase chain reaction (RT-PCR) children as the cases and 45 healthy children as the controls. Clinical examination, laboratory assays, and imaging studies were done on the patients to determine COVID–19 infection severity. Serum Ang-II was evaluated utilizing Enzyme-Linked Immunosorbent Assay (ELISA) for both groups as well as linked to patients' radiological, laboratory, and clinical parameters. Results: The levels of serum Ang II were substantially elevated in the cases compared to controls (p-value= 0.001) and in the non-survivor COVID-19 group than in the survivor group (p-value = 0.0001). The median serum Ang II level in COVID-19 cases was 100 interquartile range (IQR):88-137) ng/L and 20 (IQR:15-25) ng/L in the controls respectively. There were statistically significant positive correlations between serum Ang II as well as clinical parameters and different grades of clinical severity classifications, laboratory parameters and radiological parameters according to COVID-19 Reporting and Data System (Co-RAD) score of Computed tomography (CT) chest of the patient group. Conclusion: We concluded that early measurement of Ang II serum levels in confirmed COVID-19 children might be a helpful diagnostic and prognostic biomarker to identify high-risk patients for highly severe disease progression. [ABSTRACT FROM AUTHOR]

Background: A central feature of most cases of bronchiectasis is airway inflammation. The elevated neutrophil count has been strongly correlated with impaired lung function, bronchiectasis severity, and disease duration. There is a failure of resolution of inflammation in bronchiectasis, leading to irreversible damage and dilatation of the bronchial airways with loss of mucociliary function. Lipoxins were the first agents to be identified as anti-inflammatory endogenous lipid mediators involved in the resolution of inflammation. Aim of the Work: This study aimed to measure Lipoxin A4 level in bronchoalveolar lavage (BAL) of children with Cystic Fibrosis bronchiectasis (CF) and Non-CF bronchiectasis as a prognostic marker for clinical and radiological severity in these children as regard Bronchiectasis severity score. Methods: A cross-sectional study was conducted at Children's Hospital, Ain Shams University from December 2020 to December 2021, on children following up at the Pediatric Pulmonology Clinic. The study included 15 patients with cystic fibrosis (CF) bronchiectasis, 15 patients with non-CF bronchiectasis, and 30 control cases, without breathing difficulty or signs of RDS. Human Lipoxin A4 was measured in BAL by ELISA. Results: There was a significant difference between patients and controls as regards Lipoxin A4 level (p = 0.002),being higher in control group, but no significant difference in its level between CF and non-CF bronchiectasischildren(p = 0.663). Conclusion: Severe bronchiectasis is associated with decreased airway Lipoxin A4 levels as lowest level of lipoxinA4 was found in severe cases by bronchiectasis severity score. [ABSTRACT FROM AUTHOR]

Background: Cystic fibrosis (CF) is an exhausting and time-consuming disease that can interfere or impair typical daily life activities and social interactions. Aim and Objectives: Evaluation of psychological aspects and Quality of life in cystic fibrosis patients. Subjects and Methods: This was a cross sectional study, conducted at the Paediatric Chest Clinic, Children's Hospital, Ain Shams University on 31 patients with verified diagnosis of CF compared to 31 healthy children. Psychological assessment was done using Children depression Inventory (CDI) Scale, Spence Children's Anxiety Scale, and The Pediatric Quality of Life (PedsQLTM) Scales. Result: CF patients experienced statistically significant higher levels of depression and anxiety than their healthy peers (p < 0.001; p = 0.001, respectively). There were no significant differences in PedsQLTM Scales between the CF patients and the healthy controls (p > 0.05). Conclusion: This study is significantly important for revealing that psychiatric symptoms are prevalent among children with CF, which implies that annual symptom screening is justified. [ABSTRACT FROM AUTHOR]

Background: Coronavirus disease 2019 (COVID-19) is a newly discovered RNA betacoronavirus infection. It has been caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Angiotensin II (Ang-II) is a mitochondrial toxin that under normal circumstances is rapidly removed by angiotensin converting enzyme 2 (ACE-2). SARS-CoV-2 uses the receptor ACE-2 for host cell entry and viral replication. ACE-2 proteins are occupied and downregulated by SARS-CoV-2. ACE-2 downregulation has been directly linked to toxic over accumulation of Ang-II. Accumulating Ang-II triggers many severe and fatal conditions such as acute respiratory distress syndrome (ARDS). Aim of the Work: To assess the serum level of Ang-II and correlate it with the severity of COVID-19 infection in pediatric patients. Patients and Methods: This case control study was conducted on 45 positive COVID-19 RT-PCR children who were compared to age and sex matched 45 healthy controls. Clinical examination, laboratory assays and imaging studies were done for the patients group to assess the severity of COVID-19 infection. Serum Ang-II was measured by ELISA for both groups and corre-lated with clinical (heart rate, blood pressure, respiratory rate and oxygen saturation), laboratory (total leucocytic count, lymphocytes, neutrophils, serum ferritin, ESR and CRP) and radiological (chest x-ray and CORAD score of CT chest) parameters of the patients. Results: The median "IQR" of serum Ang-II was significantly higher in the COVID-19 patients than in the controls (100 (88137) ng/L) versus 20 (15-25) ng/L) (p-value= 0.001). There were statistically significant positive correlations between serum Ang-II levels and different grades of clinical severity classification (rs = 0.92, p-value=0.001) and CORAD score of CT chest. On the other hand, no statistically significant correlations were found between serum levels of Ang-II and total leucocytic count, lymphocytes, neutrophils, ESR and CRP. Conclusion: Serum Ang-II could be used as a biomarker in predicting the severity of COVID-19 infection in pediatrics. [ABSTRACT FROM AUTHOR]

OBJECTIVE To assess associations between current use of sodium–glucose cotransporter 2 inhibitors (SGLT2is), glucagon-like peptide 1 receptor agonists (GLP-1RAs), and their combination and risk for major adverse cardiac and cerebrovascular events (MACCE) and heart failure (HF) in people with type 2 diabetes. RESEARCH DESIGN AND METHODS In three nested case-control studies involving patients with type 2 diabetes in England and Wales (primary care data from the Clinical Practice Research Datalink and Secure Anonymised Information Linkage Databank with linkage to hospital and mortality records), we matched each patient experiencing an event with up to 20 control subjects. Adjusted odds ratios (ORs) for MACCE and HF among patients receiving SGLT2i or GLP-1RA regimens versus other combinations were estimated using conditional logistic regression and pooled using random-effectsmeta-analysis. RESULTS Among 336,334 people with type 2 diabetes and without cardiovascular disease, 18,531 (5.5%) experienced a MACCE. In a cohort of 411,206 with type 2 diabetes and without HF, 17,451 (4.2%) experienced an HF event. Compared with other combination regimens, the adjusted pooled OR and 95% CI for MACCE associated with SGLT2i regimens was 0.82 (0.73, 0.92), with GLP-1RA regimens 0.93 (0.81, 1.06), and with the SGLT2i/GLP-1RA combination 0.70 (0.50, 0.98). Corresponding data for HF were SGLT2i 0.49 (0.42, 0.58), GLP-1RA 0.82 (0.71, 0.95), and SGLT2i/GLP-1RA combination 0.43 (0.28, 0.64). CONCLUSIONS SGLT2i and SGLT2i/GLP-1RA combination regimens may be beneficial in primary prevention of MACCE and HF and GLP-1RA for HF. These data call for primary prevention trials using these agents and their combination. [ABSTRACT FROM AUTHOR]

Background: Streptococcus pneumoniae (S. pneumoniae) is a main pathogen causing acute infectious exacerbations in chronic lung diseases (CLD) children. Determining the local antibiotic susceptibility pattern of colonizing strains in these patients is crucial for empirical therapy. Moreover, identifying prevalent types is important to evaluate the effectiveness of available vaccines. This study aimed to detect antibiotic susceptibility and searching for some capsular gene types among S. pneumoniae isolates colonizing respiratory tract in CLD children. Methods: Bronchoalveolar lavage (BAL) samples were collected from 51 CLD children undergoing bronchoscopy in Ain Shams University Pediatric Hospital. All identified S. pneumoniae isolates were tested for antibiotic susceptibility. Two-steps sequential multiplex PCR technique was performed to detect capsular gene types: [3/ 22f/(22A) / 19A / 6A/6B /4 / 14/ 12F/(12A) / 9V/(9A). Results: From 51 BAL samples, 32 (62.75%) pneumococcal strains were isolated. Most of the isolates had capsular gene type 6A/6B (65.6%). Capsular gene type 14 was detected in 25% of isolates. In 9.4% of strains, capsular type could not be identified. All isolates were sensitive to vancomycin. The lowest resistance rate was to levofloxacin (6.3%) and linezolid (9.4%), while the highest rates were to clindamycin (71.9%) and erythromycin (68.8%). Conclusion: Streptococcus pneumoniae colonizing CLD children showed high resistance to clindamycin and erythromycin thus highlighting the importance of antimicrobial stewardship programs in all levels of healthcare. Capsular gene type 6A/6B was the commonest colonizing type suggesting that CLD children can benefit from the currently available PCV13 vaccine. [ABSTRACT FROM AUTHOR]

Primary ciliary dyskinesia (PCD) is a rare genetic disorder of motile cilia dysfunction generally inherited as an autosomal recessive disease. Genetic testing is increasingly considered an early step in the PCD diagnostic workflow. We used targeted panel next‐generation sequencing (NGS) for genetic screening of 33 Egyptian families with clinically highly suspected PCD. All variants prioritized were Sanger confirmed in the affected individuals and correctly segregated within the family. Targeted NGS yielded a high diagnostic output (70%) with biallelic mutations identified in known PCD genes. Mutations were identified in 13 genes overall, with CCDC40 and CCDC39 the most frequently mutated genes among Egyptian patients. Most identified mutations were predicted null effect variants (79%) and not reported before (85%). This study reveals that the genetic landscape of PCD among Egyptians is highly heterogeneous, indicating that a targeted NGS approach covering multiple genes will provide a superior diagnostic yield compared to Sanger sequencing for genetic diagnosis. The high diagnostic output achieved here highlights the potential of placing genetic testing early within the diagnostic workflow for PCD, in particular in developing countries where other diagnostic tests can be less available. [ABSTRACT FROM AUTHOR]