scholarly journals Diagnostic Barriers in Children with Immunodeficiencies in Central Asia: A Case-Based Discussion

2021 ◽  
Vol 13 (3) ◽  
pp. 483-489
Author(s):  
Zhanna Dauyey ◽  
Dimitri Poddighe
Keyword(s):  
Cystic Fibrosis ◽  
Central Asia ◽  
Low Socioeconomic Status ◽  
English Language ◽  
Respiratory Infections ◽  
Clinical History ◽  
Medical Problem ◽  
Medical Attention ◽  
Clinical Issue ◽  
Recurrent Respiratory Infections

Common variable immunodeficiency (CVID) is a primary immune deficit (PID) mainly characterized by hypogammaglobulinemia. In addition to increased susceptibility to infections and several immune-mediated manifestations, patients with CVID frequently develop bronchiectasis because of recurrent respiratory infections. This occurrence could be more likely if the diagnosis of CVID is delayed, as it often happens in less resourced clinical settings. A 15-year-old female patient was admitted to a tertiary hospital in Kazakhstan for consultation regarding a previous and established diagnosis of bronchiectasis. The clinical history was characterized by recurrent respiratory infections for several years, in addition to the development of a mixed restrictive-obstructive respiratory syndrome. Therefore, she underwent chest computerized tomography, which confirmed the presence of multiple and bilateral bronchiectasis. The clinical discussion on this patient highlighted that serum immunoglobulins were never measured previously and, thus, their assessment was strongly recommended. Based on that, a diagnosis of CVID was finally achieved, and the patient started the appropriate immunoglobulin replacement therapy. To our knowledge, this report is the first English-language publication on CVID and bronchiectasis from Central Asia. Bronchiectasis is currently an important medical problem in developing countries and populations with low socioeconomic status, where the diagnosis of the underlying cystic fibrosis and non-cystic fibrosis comorbidities can be delayed and more difficult than in countries with more accessible health care systems and facilities. This case report emphasized this important clinical issue in Central Asia and should raise the medical attention and awareness of this health problem, in order to improve the diagnostic timing and rate.

scholarly journals Treatment of Pulmonary Disease of Cystic Fibrosis: A Comprehensive Review

Antibiotics ◽  
2021 ◽  
Vol 10 (5) ◽  
pp. 486
Author(s):  
Rosa María Girón Moreno ◽  
Marta García-Clemente ◽  
Layla Diab-Cáceres ◽  
Adrián Martínez-Vergara ◽  
Miguel Ángel Martínez-García ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Genetic Disease ◽  
Drug Response ◽  
Respiratory Infections ◽  
Regulatory Protein ◽  
Anion Channel ◽  
Transmembrane Conductance ◽  
Recurrent Respiratory Infections ◽  
Altered Protein ◽  
Genetic Complexity

Cystic fibrosis (CF) is a genetic disease that causes absence or dysfunction of a protein named transmembrane conductance regulatory protein (CFTR) that works as an anion channel. As a result, the secretions of the organs where CFTR is expressed are very viscous, so their functionality is altered. The main cause of morbidity is due to the involvement of the respiratory system as a result of recurrent respiratory infections by different pathogens. In recent decades, survival has been increasing, rising by around age 50. This is due to the monitoring of patients in multidisciplinary units, early diagnosis with neonatal screening, and advances in treatments. In this chapter, we will approach the different therapies used in CF for the treatment of symptoms, obstruction, inflammation, and infection. Moreover, we will discuss specific and personalized treatments to correct the defective gene and repair the altered protein CFTR. The obstacle for personalized CF treatment is to predict the drug response of patients due to genetic complexity and heterogeneity of uncommon mutations.

scholarly journals Frequency of Cystic Fibrosis in Children with Recurrent Respiratory Infections by Sweat Chloride Testing: A Hospital-Based Cross-sectional Study

2020 ◽  
Vol 24 (4) ◽  
pp. 302-305
Author(s):  
Nahdia Zaman ◽  
Anam Zafar ◽  
Maryam Amjad ◽  
Shahzadi Sumbal Ghazi ◽  
Uzma Abid ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Respiratory Infections ◽  
Cross Sectional Study ◽  
P Value ◽  
Sectional Study ◽  
Cross Sectional ◽  
Sweat Chloride ◽  
Recurrent Respiratory Infections ◽  
Ethical Approval ◽  
Tract Infections

Objective: This study was designed to find out the frequency of cystic fibrosis in children with recurrent respiratory infections by performing a sweat chloride test.Material and Methods: This cross-sectional study was carried out from 1st September 2015 to 28th February 2016 in the department of Pediatric Medicine at the Children’s Hospital and the Institute of Child Health, Lahore after ethical approval. A total of 300 cases of recurrent respiratory tract infections were included. The sweat chloride test was done by using pilocarpine-induced iontophoresis and measuring chloride levels on the forearm or thigh by sweat analyzer at the time of induction. Cystic fibrosis was labeled if sweat chloride level ranges above 60 mEq/L. Data was collected on Performa and SPSS version 23 was used to analyze the data. Results: Mean age of patients was 6.24 ± 2.7 years with 52.7% males and 47.3% females. Most patients (86.3%) have more than 5 episodes of respiratory infection in a year. Cystic fibrosis was diagnosed in 19 (6.3%) patients. There was no relationship between the frequency of respiratory infections in a year and cystic fibrosis (p-value 0.78).Conclusion: This hospital-based study showed quite a high incidence of cystic fibrosis in our population. So facilities including neonatal screening along with sweat chloride testing and genetic analysis should be increased.

scholarly journals Bronchial hygiene technique in patients with cystic fibrosis

2021 ◽  
Author(s):  
Thainá Gomes Vieira Carvalho ◽  
Joyce Mothé de Souza ◽  
Elisa Haddad Pessanha Rangel ◽  
Caio Gomes Muniz ◽  
Julia Maria Maia de Azevedo ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Lung Function ◽  
Respiratory Infections ◽  
Walk Test ◽  
Review Of The Literature ◽  
Peripheral Airways ◽  
Randomized Controlled ◽  
Recurrent Respiratory Infections ◽  
Consequent Improvement ◽  
Tracheal Aspiration

Cystic Fibrosis is characterized by excess pulmonary secretions that cause recurrent respiratory infections, with consequent deterioration of gas exchange. Bronchial hygiene techniques aim to mobilize secretions from the peripheral airways so that they can be eliminated by coughing or tracheal aspiration. To identify the effects of different bronchial hygiene techniques on improving lung function in patients with Cystic Fibrosis. Through a systematic review of the literature, randomized controlled trials (RCTs) published between 2007 and 2021 were selected, according to the highest score in the PEDro score. The search involved the PEDro and PubMed databases, using the following keywords: bronchial hygiene. Six ECR`s were included. One study performedthe techniques during anesthesia and observed increased resistance and reduced compliance. Regarding FEV1, 3 RCTs with hospitalized patients showed improvement in lung function, regardless of the technique used. In outpatients, there was no improvement. Regarding secretion weight, the cough machine produced more secretion than autogenous drainage, as well as a drop in saturation after the 2-min walk test, and increased FEV1. Bronchial hygiene techniques in patients with cystic fibrosis have been shown to be effective in removing mucus, with consequent improvement in lung function and aerobic fitness.

Persistent Allergic Minimum Phlogosis as Mechanistic Link Between Recurrent Respiratory Infections and Atopy: A Single Center Pilot Study

2017 ◽  
Vol 13 (1) ◽  
Author(s):  
Caterina Cuppari ◽  
Sara Manti ◽  
Annamaria Salpietro ◽  
Simona Valenti ◽  
Dominique De Vivo ◽  
...  
Keyword(s):  
Pilot Study ◽  
Respiratory Infections ◽  
Single Center ◽  
Recurrent Respiratory Infections

scholarly journals Pseudomonas aeruginosa PA14 Enhances the Efficacy of Norfloxacin against Staphylococcus aureus Newman Biofilms

2020 ◽  
Vol 202 (18) ◽  
Author(s):  
Giulia Orazi ◽  
Fabrice Jean-Pierre ◽  
George A. O’Toole
Keyword(s):  
Cystic Fibrosis ◽  
Staphylococcus Aureus ◽  
Pseudomonas Aeruginosa ◽  
Antimicrobial Agents ◽  
Respiratory Infections ◽  
Multiple Infection ◽  
Bacterial Biofilms ◽  
Interspecies Interactions ◽  
Chronic Infections ◽  
Content Type

ABSTRACT The thick mucus within the airways of individuals with cystic fibrosis (CF) promotes frequent respiratory infections that are often polymicrobial. Pseudomonas aeruginosa and Staphylococcus aureus are two of the most prevalent pathogens that cause CF pulmonary infections, and both are among the most common etiologic agents of chronic wound infections. Furthermore, the ability of P. aeruginosa and S. aureus to form biofilms promotes the establishment of chronic infections that are often difficult to eradicate using antimicrobial agents. In this study, we found that multiple LasR-regulated exoproducts of P. aeruginosa, including 2-heptyl-4-hydroxyquinoline N-oxide (HQNO), siderophores, phenazines, and rhamnolipids, likely contribute to the ability of P. aeruginosa PA14 to shift S. aureus Newman norfloxacin susceptibility profiles. Here, we observe that exposure to P. aeruginosa exoproducts leads to an increase in intracellular norfloxacin accumulation by S. aureus. We previously showed that P. aeruginosa supernatant dissipates the S. aureus membrane potential, and furthermore, depletion of the S. aureus proton motive force recapitulates the effect of the P. aeruginosa PA14 supernatant on shifting norfloxacin sensitivity profiles of biofilm-grown S. aureus Newman. From these results, we hypothesize that exposure to P. aeruginosa PA14 exoproducts leads to increased uptake of the drug and/or an impaired ability of S. aureus Newman to efflux norfloxacin. Surprisingly, the effect observed here of P. aeruginosa PA14 exoproducts on S. aureus Newman susceptibility to norfloxacin seemed to be specific to these strains and this antibiotic. Our results illustrate that microbially derived products can alter the ability of antimicrobial agents to kill bacterial biofilms. IMPORTANCE Pseudomonas aeruginosa and Staphylococcus aureus are frequently coisolated from multiple infection sites, including the lungs of individuals with cystic fibrosis (CF) and nonhealing diabetic foot ulcers. Coinfection with P. aeruginosa and S. aureus has been shown to produce worse outcomes compared to infection with either organism alone. Furthermore, the ability of these pathogens to form biofilms enables them to cause persistent infection and withstand antimicrobial therapy. In this study, we found that P. aeruginosa-secreted products dramatically increase the ability of the antibiotic norfloxacin to kill S. aureus biofilms. Understanding how interspecies interactions alter the antibiotic susceptibility of bacterial biofilms may inform treatment decisions and inspire the development of new therapeutic strategies.

scholarly journals The Multifaceted Roles of MicroRNAs in Cystic Fibrosis

Diagnostics ◽  
2020 ◽  
Vol 10 (12) ◽  
pp. 1102
Author(s):  
Fatima Domenica Elisa De Palma ◽  
Valeria Raia ◽  
Guido Kroemer ◽  
Maria Chiara Maiuri
Keyword(s):  
Cystic Fibrosis ◽  
Respiratory Infections ◽  
Current Knowledge ◽  
Pancreatic Insufficiency ◽  
Clinical Manifestations ◽  
Predictive Biomarkers ◽  
Loss Of Function ◽  
Gene Encoding ◽  
Multisystemic Disease

Cystic fibrosis (CF) is a lifelong disorder affecting 1 in 3500 live births worldwide. It is a monogenetic autosomal recessive disease caused by loss-of-function mutations in the gene encoding the chloride channel cystic fibrosis transmembrane conductance regulator (CFTR), the impairment of which leads to ionic disequilibria in exocrine organs. This translates into a chronic multisystemic disease characterized by airway obstruction, respiratory infections, and pancreatic insufficiency as well as hepatobiliary and gastrointestinal dysfunction. Molecular characterization of the mutational heterogeneity of CFTR (affected by more than 2000 variants) improved the understanding and management of CF. However, these CFTR variants are linked to different clinical manifestations and phenotypes, and they affect response to treatments. Expanding evidence suggests that multisystemic disease affects CF pathology via impairing either CFTR or proteins regulated by CFTR. Thus, altering the expression of miRNAs in vivo could constitute an appealing strategy for developing new CF therapies. In this review, we will first describe the pathophysiology and clinical management of CF. Then, we will summarize the current knowledge on altered miRNAs in CF patients, with a focus on the miRNAs involved in the deregulation of CFTR and in the modulation of inflammation. We will highlight recent findings on the potential utility of measuring circulating miRNAs in CF as diagnostic, prognostic, and predictive biomarkers. Finally, we will provide an overview on potential miRNA-based therapeutic approaches.

Good's syndrome : A case with recurrent respiratory infections

2013 ◽  
Vol 24 ◽  
pp. e143
Author(s):  
S. Ersan ◽  
G. Ersan ◽  
C. Arslan ◽  
S. Atalay ◽  
S. Kose
Keyword(s):  
Respiratory Infections ◽  
Good’S Syndrome ◽  
Recurrent Respiratory Infections

scholarly journals Vx-809/Vx-770 treatment reduces inflammatory response to Pseudomonas aeruginosa in primary differentiated cystic fibrosis bronchial epithelial cells

2018 ◽  
Vol 314 (4) ◽  
pp. L635-L641 ◽  
Author(s):  
Manon Ruffin ◽  
Lucie Roussel ◽  
Émilie Maillé ◽  
Simon Rousseau ◽  
Emmanuelle Brochiero
Keyword(s):  
Cystic Fibrosis ◽  
Pseudomonas Aeruginosa ◽  
Inflammatory Response ◽  
Respiratory Infections ◽  
Bronchial Epithelial Cell ◽  
Transmembrane Conductance Regulator ◽  
Transmembrane Conductance ◽  
Bronchial Epithelial ◽  
Primary Bronchial Epithelial Cell ◽  
Beneficial Impact

Cystic fibrosis patients exhibit chronic Pseudomonas aeruginosa respiratory infections and sustained proinflammatory state favoring lung tissue damage and remodeling, ultimately leading to respiratory failure. Loss of cystic fibrosis transmembrane conductance regulator (CFTR) function is associated with MAPK hyperactivation and increased cytokines expression, such as interleukin-8 [chemoattractant chemokine (C-X-C motif) ligand 8 (CXCL8)]. Recently, new therapeutic strategies directly targeting the basic CFTR defect have been developed, and ORKAMBI (Vx-809/Vx-770 combination) is the only Food and Drug Administration-approved treatment for CF patients homozygous for the F508del mutation. Here we aimed to determine the effect of the Vx-809/Vx-770 combination on the induction of the inflammatory response by fully differentiated primary bronchial epithelial cell cultures from CF patients carrying F508del mutations, following exposure to P. aeruginosa exoproducts. Our data unveiled that CFTR functional rescue with Vx-809/Vx-770 drastically reduces CXCL8 (as well as CXCL1 and CXCL2) transcripts and p38 MAPK phosphorylation in response to P. aeruginosa exposure through a CFTR-dependent mechanism. These results suggest that ORKAMBI has anti-inflammatory properties that could decrease lung inflammation and contribute to the observed beneficial impact of this treatment in CF patients.

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