Review
Respiratory System
Wicharn Boonjindasup, Anne B. Chang, Julie M. Marchant, J. Yoon Irons, Margaret S. McElrea
Summary: The study found that at least ten attempts (with the best two within <10% variability) are required to achieve the best and reliable MIP/MEP values in children with CF. The number of attempts required was inconsistent between test days, with some children requiring fewer attempts on the second day while others needed more.
Article
Medicine, General & Internal
Monica Bennett, Andreia Filipa Nogueira, Maria Manuel Flores, Teresa Reis Silva
Summary: This case describes an adolescent with cystic fibrosis, diagnosed through detailed investigations and genetic studies. The patient had typical CF symptoms, but the presence of specific gene mutations led to atypical clinical manifestations.
Article
Pediatrics
Hulya Nilgun Gurses, Hikmet Ucgun, Melih Zeren, Hilal Denizoglu Kulli, Erkan Cakir
Summary: This study compared the efficacy of comprehensive respiratory physiotherapy (CRP) in children with cystic fibrosis (CF) and non-cystic fibrosis bronchiectasis (NCFB). Both groups showed significant improvements in pulmonary function, exercise capacity, and respiratory and peripheral muscle strength after 8 weeks of CRP. The NCFB group had further improvements in maximum expiratory pressure, exercise capacity, and peripheral muscle strength compared to the CF group.
EUROPEAN JOURNAL OF PEDIATRICS
(2022)
Review
Pediatrics
Linjie Zhang, Seiko Nomiyama, Renata Marcos Bedran, Cristina Goncalves Alvim, Jose Dirceu Ribeiro, Paulo Camargos
Summary: This study conducted a systematic review and meta-analysis to evaluate the diagnostic accuracy of sweat conductivity (SC) for cystic fibrosis (CF) diagnosis. The results showed that SC demonstrated excellent diagnostic performance and can be considered as an alternative to the sweat test for CF diagnosis.
ARCHIVES OF DISEASE IN CHILDHOOD
(2023)
Review
Critical Care Medicine
Christina M. Mingora, Patrick A. Flume
Summary: Cystic fibrosis is an autosomal recessive genetic condition with significant manifestations in the respiratory system. Despite advancements in treatment, pulmonary complications like pneumothorax and respiratory failure still occur. Palliative care and CF transmembrane conductance regulator therapies play evolving roles in managing the care of CF patients.
Review
Cell Biology
Sophie Gohy, Alexandra Moeremans, Charles Pilette, Amandine Collin
Summary: The respiratory epithelium serves as the first line of defense against inhaled pathogens in cystic fibrosis patients, with mucosal alterations and local immunity linked to immunoglobulin A playing crucial roles in inflammation and bacterial overgrowth within the airways.
Article
Health Care Sciences & Services
Mihaela Dediu, Ioana Mihaiela Ciuca, Monica Steluta Marc, Estera Boeriu, Liviu Laurentiu Pop
Summary: This cross-sectional study aimed to identify major factors influencing pulmonary function in CF patients, with results showing significant impact of BMI and age on lung function. The research suggests that better nutritional status and infection prophylaxis should be emphasized in the comprehensive clinical management strategy for pediatric CF patients in Romania.
JOURNAL OF MULTIDISCIPLINARY HEALTHCARE
(2021)
Review
Pharmacology & Pharmacy
Xiaoxuan Han, Danni Li, Felisa Reyes-Ortega, Elena K. Schneider-Futschik
Summary: Pulmonary drug delivery is widely used for the treatment of acute and chronic respiratory diseases. Targeted lung delivery is particularly important for diseases like cystic fibrosis. However, formulating dry powder for pulmonary delivery is challenging due to aerodynamic restrictions and lung tolerance. This review provides an overview of the respiratory tract structure in cystic fibrosis patients, discusses the advantages of targeted lung delivery, and examines the factors affecting clinical efficacy.
Article
Respiratory System
David P. Nichols, Pradeep K. Singh, Arthur Baines, Lindsay J. Caverly, James F. Chmiel, Ronald L. GIbson, Jorge Lascano, Sarah J. Morgan, George Retsch-Bogart, Lisa Saiman, Hossein Sadeghi, Joanne L. Billings, Sonya L. Heltshe, Shannon Kirby, Ada Kong, Jerry A. Nick, Nicole Mayer-Hamblett
Summary: The study aimed to test the effects of combining azithromycin with inhaled tobramycin on clinical and microbiological outcomes in patients with cystic fibrosis and P. aeruginosa airway infection. The results showed that participants randomized to placebo with inhaled tobramycin did not experience significantly greater improvements in lung function or other clinical outcomes compared with those randomized to azithromycin with tobramycin, despite having greater reduction in P. aeruginosa density.
Article
Physiology
Fanny Gabrysz-Forget, Anne-Catherine Maynard-Paquette, Aileen Kharat, Francois Tremblay, Maite Silviet-Carricart, Annick Lavoie, Martin Girard, Bruno-Pierre Dube
Summary: In patients with cystic fibrosis, diaphragm ultrasound can serve as a reliable and non-invasive marker of disease severity, providing information on respiratory and extra-pulmonary manifestations of the disease and clinical outcomes. Further research is needed to explore its potential as a longitudinal marker of disease progression, response to interventions, or therapeutic target.
FRONTIERS IN PHYSIOLOGY
(2022)
Article
Radiology, Nuclear Medicine & Medical Imaging
Layla Diab-Caceres, Rosa Maria Giron-Moreno, Elena Garcia-Castillo, Maria Teresa Pastor-Sanz, Casilda Olveira, Marta Maria Garcia-Clemente, Rosa Nieto-Royo, Concha Prados-Sanchez, Paloma Caballero-Sanchez, Maria Jose Olivera-Serrano, Alicia Padilla-Galo, Encarnacion Nava-Tomas, Amparo Esteban-Peris, Maria Fernandez-Velilla, Maribel Torres, Rosa Mar Gomez-Punter, Julio Ancochea
Summary: The study found that the modified Bhalla score can predict future PEx in CF patients and is well correlated with pulmonary function test results. A statistical model based on the overall Bhalla score was constructed to predict the number of PEx.
EUROPEAN RADIOLOGY
(2021)
Article
Immunology
Robert E. Sealy, Sherri L. Surman, Peter Vogel, Julia L. Hurwitz
Summary: Cystic fibrosis is a rare genetic disease that often leads to lung complications. Vitamin A supplementation may help reduce virus-induced lung pathology for CF patients.
FRONTIERS IN IMMUNOLOGY
(2021)
Article
Immunology
Shivanthan Shanthikumar, Sarath C. Ranganathan, Richard Saffery, Melanie R. Neeland
Summary: By using high parameter flow cytometry and inflammatory cytokine profiling, the study investigated the immune response in preschool children with CF and healthy controls, revealing pulmonary infiltration of CD66b(+) granulocytes and elevated levels of inflammatory cytokines in CF children. Moreover, the proportions of systemic neutrophils in CF children were positively correlated with age, while CD4 T cells and B cells showed an inverse association with age. This work emphasizes the importance of multiplex immune profiling and advanced analytical pipelines in understanding lung disease mechanisms in childhood.
FRONTIERS IN IMMUNOLOGY
(2021)
Article
Pediatrics
Renata Marcos Bedran, Cristina Goncalves Alvim, Olivia Goncalves Sader, Jose Vicente Alves Junior, Fernando Henrique Pereira, Daniela Magalhaes Nolasco, Linjie Zhang, Paulo Camargos
Summary: Sweat conductivity showed excellent accuracy in diagnosing cystic fibrosis among newborns and very young infants.
ARCHIVES OF DISEASE IN CHILDHOOD
(2023)
Article
Pediatrics
Pelin Asfuroglu, Tugba Sismanlar Eyuboglu, Ayse Tana Aslan, Tugba Ramasli Gursoy, Nagehan Emiralioglu, Ebru Yalcin, Nural Kiper, Velat Sen, Hadice Selimoglu Sen, Derya Ufuk Altintas, Dilek Ozcan, Ayse Ayzit Kilinc, Haluk Cokugras, Azer Kilic Baskan, Hakan Yazan, Ufuk Erenberk, Guzide Dogan, Gokcen Unal, Asli Imran Yilmaz, Ozlem Keskin, Elif Arik, Ercan Kucukosmanoglu, Ilim Irmak, Ebru Damadoglu, Gokcen Kartal Ozturk, Figen Gulen, Abdurrahman Erdem Basaran, Aysen Bingol, Sukru Cekic, Nihat Sapan, Gonca Kilic, Koray Harmanci, Mehmet Kose, Ali Ozdemir, Gokcen Dilsa Tugcu, Sanem Eryilmaz Polat, Melih Hangul, Gizem Ozcan, Zeynep Gokce Gayretli Aydin, Hasan Yuksel, Erdem Topal, Sebnem Ozdogan, Gonul Caltepe, Ayse Suleyman, Demet Can, Pervin Korkmaz Ekren, Cem Murat Bal, Mehmet Kilic, Guzin Cinel, Nazan Cobanoglu, Sevgi Pekcan, Erkan Cakir, Ugur Ozcelik, Deniz Dogru
Summary: This study evaluated the impact of a newly established CF registry system in Turkey on the follow-up and care of CF patients. The results showed that the registry system led to higher z-scores of height and BMI, increased usage of medications for lung function improvement, and lower incidence of PBS. This suggests that the registry system has positively influenced the care of CF patients.
PEDIATRIC PULMONOLOGY
(2022)
Article
Allergy
Claudia Silva Schindel, Daniele Schiwe, Joao Paulo Heinzmann-Filho, Mailise Fatima Gheller, Natalia Evangelista Campos, Paulo Marcio Pitrez, Marcio Vinicius Fagundes Donadio
Summary: This study evaluated the exercise capacity in children and adolescents with STRA and found that approximately 30% of patients had impaired exercise capacity. The results indicate that lung function and exercise-induced bronchoconstriction are important factors influencing aerobic fitness.
Article
Rehabilitation
Pitiguara de Freitas Coelho, Roberta Ribeiro Batista Barbosa, Rodrigo dos Santos Lugao, Fernanda Mayrink Goncalves Liberato, Pamela Reis Vidal, Roberta de Cassia Nunes Cruz Melotti, Marcio Vinicius Fagundes Donadio
Summary: Autonomic nervous system balance is altered in patients with cystic fibrosis (CF). Heart rate variability (HRV) is correlated with exercise capacity and levels of daily physical activity in CF patients.
HONG KONG PHYSIOTHERAPY JOURNAL
(2022)
Article
Developmental Biology
Carolina Luft, Luis Eduardo Wearick-Silva, Mariana Severo Costa, Leonardo Pedrazza, Gessica Luana Antunes, Rodrigo Grassi-Oliveira, Jarbas Rodrigues Oliveira, Marcio Vinicius Fagundes Donadio
Summary: The study revealed that stress during pregnancy can impact maternal behavior and inflammatory response in the olfactory bulb of mice. Stressed females showed decreased maternal index and NF-kappa B1 gene expression, while increased expression of interleukin-1 beta was observed.
INTERNATIONAL JOURNAL OF DEVELOPMENTAL NEUROSCIENCE
(2022)
Review
Sport Sciences
Agustin Jesus Estevez-Gonzalez, Margarita Perez-Ruiz, Fernando Cobo-Vicente, Marcio Vinicius Fagundes Donadio, Eneko Larumbe-Zabala
Summary: This study analyzed the effects of physical training programs on heart rate variability in children and adolescents with chronic diseases. The results showed that exercise can moderately increase time-domain variables and have a moderate effect on some frequency-domain variables.
INTERNATIONAL JOURNAL OF SPORTS MEDICINE
(2022)
Article
Biochemistry & Molecular Biology
Gabriela Viegas Haute, Carolina Luft, Leonardo Pedrazza, Marcio Vinicius Fagundes Donadio, Jarbas Rodrigues de Oliveira
Summary: The study showed that OG can act as an inhibitor of ROS release, NETs formation and modulate LPS effect on neutrophil apoptosis. It also inhibited PBMCs proliferation. However, there was no observed increase in the survival rate of septic animals.
MOLECULAR BIOLOGY REPORTS
(2022)
Article
Pediatrics
Rodrigo dos Santos Lugao, Roberta Ribeiro Batista Barbosa, Pitiguara de Freitas Coelho, Fernanda Mayrink Goncalves Liberato, Pamela Reis Vidal, Roberta Barcellos Couto Olimpio de Carvalho, Roberta de Cassia Nunes Cruz Melotti, Marcio Vinicius Fagundes Donadio
Summary: This study assesses the association between sleep disorders and heart rate variability in children and adolescents with cystic fibrosis. The results show a weak correlation between sleep disorders and HRV parameters, particularly in individuals with reduced pulmonary function. Additionally, a high prevalence of autonomic nervous system disorders, nocturnal hypoxemia, and obstructive sleep apnea syndrome was found.
REVISTA PAULISTA DE PEDIATRIA
(2022)
Article
Cardiac & Cardiovascular Systems
M. S. Walker, F. S. Tarasiuk, A. S. Gustavo, M. S. Oliveira, M. V. F. Donadio, A. M. P. Feoli
Summary: This research aimed to evaluate the effects of three different interventions on the consumption of ultra-processed foods in adults with MetS. The findings indicate that these interventions were able to significantly reduce the amount of ultra-processed food consumed, which can have a positive impact on the prevention and treatment of MetS.
NUTRITION METABOLISM AND CARDIOVASCULAR DISEASES
(2022)
Article
Orthopedics
Debora Schmidt, Taila Cristina Piva, Sheila Suzana Glaeser, Daniele Martins Piekala, Paula Pinheiro Berto, Gilberto Friedman, Graciele Sbruzzi
Summary: The study aimed to identify the occurrence and factors associated with ICU-acquired weakness in COVID-19 patients. It found that patients with ICUAW had lower muscle strength and mobility, and factors independently associated with ICUAW included bed rest time and use of corticosteroids.
Article
Pharmacology & Pharmacy
Matheus Scherer Bastos, Rafaela Mallmann Saalfeld, Bruna Pasqualotto Costa, Krist Helen Antunes, Denizar Melo, Marcio Vinicius Fagundes Donadio, Eliane Romanato Santarem, Jarbas Rodrigues de Oliveira
Summary: Coumaric acid has the potential to be a therapeutic agent for liver fibrosis. It can reduce cell proliferation, inhibit the expression of cell cycle proteins, and revert activated liver cells to a quiescent state.
NAUNYN-SCHMIEDEBERGS ARCHIVES OF PHARMACOLOGY
(2023)
Correction
Pharmacology & Pharmacy
Matheus Scherer Bastos, Rafaela Mallmann Saalfeld, Bruna Pasqualotto Costa, Krist Helen Antunes, Denizar Melo, Marcio Vinicius Fagundes Donadio, Eliane Romanato Santarem, Jarbas Rodrigues de Oliveira
NAUNYN-SCHMIEDEBERGS ARCHIVES OF PHARMACOLOGY
(2023)
Review
Pediatrics
Daniele Schiwe, Fernanda Maria Vendrusculo, Nicolas Acosta Becker, Marcio Vinicius Fagundes Donadio
Summary: This study aimed to investigate the impact of asthma on heart rate variability in children and adolescents. A systematic review of observational studies and clinical trials found that children and adolescents with asthma have lower heart rate variability and impaired sympathetic modulation.
PEDIATRIC PULMONOLOGY
(2023)
Article
Pediatrics
Marcio Vinicius Fagundes Donadio, Marta Amor Barbosa, Fernanda Maria Vendrusculo, Tamara Iturriaga Ramirez, Elena Santana-Sosa, Veronica Sanz-Santiago, Margarita Perez-Ruiz
Summary: Exercise intolerance is common in children and adolescents with cystic fibrosis and asthma. This study aimed to evaluate the association between exercise capacity and lung function, ventilatory limitation, and ventilatory efficiency in these patients. The results showed differences in ventilatory limitation mechanisms between the two groups.
PEDIATRIC PULMONOLOGY
(2023)
Article
Nutrition & Dietetics
Lisiane Marcal Perez, Eduardo Mundstock, Marina Azambuja Amaral, Fernanda Maria Vendrusculo, Wilson Canon-Montanez, Rita Mattiello
Summary: This study found that family income is associated with body composition in children and adolescents. Lower family income is associated with lower fat-free mass, fat percentage, and skeletal muscle mass.
REVISTA DE NUTRICAO-BRAZILIAN JOURNAL OF NUTRITION
(2022)