Review
Medicine, General & Internal
Connie Yang, Mark Montgomery
Summary: The use of dornase alfa in cystic fibrosis patients may improve lung function and decrease pulmonary exacerbations in trials lasting from one month to two years. Adverse events such as voice alteration and rash appear to be the most frequently reported in randomized controlled trials compared to other treatments. However, there is not enough evidence to determine if dornase alfa is superior to other hyperosmolar agents in improving lung function.
COCHRANE DATABASE OF SYSTEMATIC REVIEWS
(2021)
Article
Microbiology
Samantha L. Durfey, Sudhakar Pipavath, Anna Li, Anh T. Vo, Anina Ratjen, Suzanne Carter, Sarah J. Morgan, Matthew C. Radey, Brenda Grogan, Stephen J. Salipante, Michael J. Welsh, David A. Stoltz, Christopher H. Goss, Edward F. McKone, Pradeep K. Singh
Summary: Studies show that chronic CF infections may persist despite improvements induced by CFTR modulators and aggressive antibiotic treatment. Combining CFTR modulators with intensive antibiotics may be most effective for patients with higher CFTR activity. Challenges remain in improving the health of people with CF.
Article
Microbiology
Erin M. Anderson, Neethu Shaji Saji, Alexander C. Anderson, Dyanne Brewer, Anthony J. Clarke, Cezar M. Khursigara
Summary: This study identified changes in the peptidoglycan composition of two epidemic strains of Pseudomonas aeruginosa when cultured under conditions resembling cystic fibrosis lung infections. The findings showed variations in key components related to virulence and antibiotic resistance, indicating strain-specific differences.
Article
Infectious Diseases
Margherita Degasperi, Riccardo Sgarra, Mario Mardirossian, Sabrina Pacor, Massimo Maschio, Marco Scocchi
Summary: This study developed a prodrug to extend the peptide chain of an antimicrobial peptide and added a sequence that can activate the peptide at the site of infection/inflammation, enabling a slow and targeted release. The prodrug showed good antimicrobial activity and lower cytotoxicity in the presence of elastase.
Article
Pediatrics
Dilber Ademhan Tural, Ebru Yalcin, Nagehan Emiralioglu, Beste Ozsezen, Birce Sunman, Halime Nayir Buyuksahin, Ismail Guzelkas, Deniz Dogru, Ugur Ozcelik, Nural Kiper
Summary: This study showed that long-term treatment with DPI mannitol and dornase alfa can lead to greater improvements in FEV1, FVC, FEV1/FVC, and FEF25-75 z-scores compared to treatment with dornase alfa alone in children with CF.
PEDIATRIC PULMONOLOGY
(2022)
Article
Immunology
Katrin Anne Becker, Rabea Verhaegh, Hedda-Luise Verhasselt, Simone Keitsch, Matthias Soddemann, Barbara Wilker, Gregory C. Wilson, Jan Buer, Syed A. Ahmad, Michael J. Edwards, Erich Gulbins
Summary: Genetic overexpression of acid ceramidase in cystic fibrosis mice increases immunity and protects against pulmonary infections with P. aeruginosa. Sphingosine has been shown to effectively kill pathogenic bacteria. Normalizing sphingosine levels in airway epithelial cells can prevent infections in cystic fibrosis patients.
INFECTION AND IMMUNITY
(2021)
Article
Microbiology
Madeline Mei, Preston Pheng, Detriana Kurzeja-Edwards, Stephen P. Diggle
Summary: Chronic, highly antibiotic-resistant infections in cystic fibrosis lungs contribute to increasing morbidity and mortality. Pseudomonas aeruginosa, a common CF pathogen, exhibits resistance to multiple antibiotics, contributing to antimicrobial resistance. This study found that the diversity of P. aeruginosa variants within CF lung infections leads to varying susceptibility to R-pyocins, and suggests that LPS packing density may play a significant role in R-pyocin susceptibility among CF variants.
MICROBIOLOGY SPECTRUM
(2023)
Article
Biochemistry & Molecular Biology
Michele Iafisco, Francesca Carella, Lorenzo Degli Esposti, Alessio Adamiano, Daniele Catalucci, Jessica Modica, Alessandra Bragonzi, Alberto Vitali, Riccardo Torelli, Maurizio Sanguinetti, Francesca Bugli
Summary: The use of inhalable calcium phosphate nanoparticles functionalized with colistin shows promising potential for improving the efficacy of cystic fibrosis treatment by enhancing drug delivery to bacteria in the airways.
JOURNAL OF INORGANIC BIOCHEMISTRY
(2022)
Article
Immunology
Kelly Kweng, Andrea Benedetti, Yvonne Yau, Valerie Waters, Dao Nguyen
Summary: This study found that PA isolates from new-onset CF infections exhibit strain-specific susceptibility to neutrophil antibacterial functions, and PA isolates resistant to neutrophil phagocytosis are independent risk factors for failed tobramycin eradication. Experimental results on neutrophil phagocytosis and intracellular killing showed that isolates from persistent infections were more resistant compared to those from eradicated infections.
JOURNAL OF INFECTIOUS DISEASES
(2022)
Article
Infectious Diseases
Katie A. Parsels, Keri A. Mastro, Jeffrey M. Steele, Stephen J. Thomas, Wesley D. Kufel
Summary: Cefiderocol is a novel siderophore cephalosporin that has shown activity against clinically relevant MDR Gram-negative bacteria. FDA-approved indications include complicated urinary tract infections and hospital-acquired bacterial pneumonia. Clinical trials have demonstrated non-inferiority to standard treatments, but higher all-cause mortality was observed in certain cases.
JOURNAL OF ANTIMICROBIAL CHEMOTHERAPY
(2021)
Article
Respiratory System
Luca Cavinato, Francesco R. Luly, Valentina Pastore, Daniele Chiappetta, Gloria Sangiorgi, Eva Ferrara, Pia Baiocchi, Giuseppe Mandarello, Giuseppe Cimino, Paola Del Porto, Fiorentina Ascenzioni
Summary: Cystic fibrosis (CF) is characterized by chronic bacterial lung infection and inflammation due to mutations in CF transmembrane conductance regulator (CFTR). Monocytes and monocyte-derived macrophages in CF have been shown to have defective phagocytosis and antimicrobial activity against relevant pathogens. This study evaluated the effect of CFTR triple modulator therapy on CF monocyte activity against Pseudomonas aeruginosa. The results showed that CF monocytes exhibited improved phagocytic and microbicidal activity, reduced oxidative burst, and decreased inflammatory response after ETI therapy.
EUROPEAN RESPIRATORY JOURNAL
(2023)
Article
Microbiology
Lucile Hubert, Thomas E. Barton, Hollie J. Leighton, Brogan Richards
Summary: A workshop was held to discuss preclinical testing of antimicrobials for cystic fibrosis (CF). The workshop aimed to address the communication gap between patients, clinicians, and researchers and identify priorities for developing CF therapeutics. Key points discussed included the disconnect within the CF community and the challenges of translating laboratory data to successful clinical outcomes. New models such as organ-on-a-chip technology and niche environment mimicking media are being developed to tackle these issues.
Article
Biochemistry & Molecular Biology
Tomasz Bogiel, Dagmara Depka, Mateusz Rzepka, Agnieszka Mikucka
Summary: This study assessed the susceptibility and virulence factor gene frequency of clinical P. aeruginosa strains causing bloodstream infections. The results showed that these strains were resistant to multiple antibiotics and had diverse compositions of virulence factor genes.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Review
Pediatrics
Vito Terlizzi, Chiara Castellani, Giovanni Taccetti, Beatrice Ferrari
Summary: Cystic fibrosis (CF) is a common inherited disease, mostly affecting Caucasian populations in Europe and the United States. Treatment of CF lung disease, a major complication of the condition, focuses on improving sputum clearance. Dornase alfa, a drug that reduces mucous viscosity, has shown positive effects on lung clearance index, a measure of ventilation inhomogeneity.
ITALIAN JOURNAL OF PEDIATRICS
(2022)
Article
Multidisciplinary Sciences
Weijun Xiu, Lili Ren, Huayu Xiao, Yue Zhang, Dou Wang, Kaili Yang, Siyu Wang, Lihui Yuwen, Xiao Li, Heng Dong, Qiang Li, Yongbin Mou, Yuqian Zhang, Zhaowei Yin, Bin Liang, Yu Gao, Lianhui Wang
Summary: An ultrasound-responsive catalytic micro-bubble is developed to combat chronic lung infections caused by Pseudomonas aeruginosa biofilms. These microbubbles physically disrupt the biofilm structure and enhance the penetration of nanoparticles and antibiotics. The nanoparticles chemically degrade the biofilm matrix and kill bacteria, while also activating the immune response. This strategy efficiently treats chronic lung infections by combining physical/chemical/antibiotic biofilm elimination and immune activation.
Article
Medicine, Research & Experimental
Max M. Yang, Nara S. Higano, Chamindu C. Gunatilaka, Erik B. Hysinger, Raouf S. Amin, Jason C. Woods, Alister J. Bates
Summary: The study found that the severity of SGS and axial position can affect the increase in work of breathing. WOB rapidly increases with %AO greater than 70%. Changes in axial position can also increase WOB, with an increase approximately equivalent to a 10% increase in %AO.
Article
Medicine, Research & Experimental
Deep B. Gandhi, Andrew Rice, Chamindu C. Gunatilaka, Nara S. Higano, Robert J. Fleck, Alessandro de Alarcon, Catherine K. Hart, I-Chun Kuo, Raouf S. Amin, Jason C. Woods, Erik B. Hysinger, Alister J. Bates
Summary: The study successfully detected SGS and treatment response in the rabbit model using UTE-MRI, with good correlation to ETT-sizing. Balloon dilation increased CSA at SGS but did not reach baseline values. SGS did not alter dynamic motion for the trachea in this rabbit model, but tracheas were significantly eccentric post-balloon dilation.
Article
Cardiac & Cardiovascular Systems
Keren Armoni Domany, Guixia Huang, Md Monir Hossain, Christine L. Schuler, Virend K. Somers, Stephen R. Daniels, Raouf Amin
Summary: This study investigated the changes in cardiac structure and function in children with OSA following adenotonsillectomy. The findings suggest that adenotonsillectomy significantly improves diastolic function in OSA children, while left ventricular remodeling remains unchanged despite OSA improvement.
AMERICAN JOURNAL OF CARDIOLOGY
(2021)
Article
Pediatrics
Adrienne P. Borschuk, Sara E. Williams, Jessica M. McClure, Megan Kendall, Lisa Mack, Michelle Coleman, Neepa Gurbani, Dan Benscoter, Raouf Amin, Lori J. Stark
Summary: This study described a novel behavioral health program and examined its impact on family caregiver engagement and psychological distress on a pediatric inpatient chronic ventilator unit. The results showed that the behavioral health program was associated with increased caregiver participation in rounds, while parents who completed a course of psychotherapy within the program demonstrated reduced time to training completion, higher staff-rated caregiver engagement, and significantly reduced levels of caregiver distress.
PEDIATRIC PULMONOLOGY
(2021)
Editorial Material
Critical Care Medicine
Raouf Amin
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
(2022)
Article
Clinical Neurology
Phoebe K. Yu, Jerilynn Radcliffe, H. Gerry Taylor, Raouf S. Amin, Cristina M. Baldassari, Thomas Boswick, Ronald D. Chervin, Lisa M. Elden, Susan L. Furth, Susan L. Garetz, Alisha George, Stacey L. Ishman, Erin M. Kirkham, Christopher Liu, Ron B. Mitchell, S. Kamal Naqvi, Carol L. Rosen, Kristie R. Ross, Jay Shah, Ignacio E. Tapia, Lisa R. Young, David A. Zopf, Rui Wang, Susan Redline
Summary: The relationship between the severity of obstructive sleep apnea and neurobehavioral morbidity is unclear. This study compares the neurobehavioral symptoms of mild sleep-disordered breathing and obstructive sleep apnea, finding that children with mild sleep-disordered breathing have more abnormal executive function scores and higher rates of inattention and hyperactivity compared to children with obstructive sleep apnea.
Article
Chemistry, Multidisciplinary
Dena Z. Khater, R. S. Amin, Mohamed Mahmoud, K. M. El-Khatib
Summary: In this study, mixed transition-metal oxides (MTMOs) were developed and tested as electrocatalysts for the oxygen reduction reaction (ORR) in neutral media. The results showed that MTMOs anchored on an activated carbon support exhibited higher ORR electrocatalytic activity compared to their counterparts anchored on a graphene support, making them a cost-effective alternative to expensive platinum in energy conversion and electrocatalysis.
Article
Chemistry, Physical
Dena Z. Khater, R. S. Amin, Amani E. Fetohi, K. M. El-Khatib, Mohamed Mahmoud
Summary: In this study, manganese oxide-silver nanocomposites anchored on graphitic mesoporous carbon were prepared as cathodic catalysts to improve the ORR kinetics and inhibit biofilm growth on the cathode surface of air-cathode microbial fuel cells fed with sewage. The MnOx-Ag/GMC nanocomposites showed excellent ORR performance and high antibacterial activity, leading to higher maximum power density and lower closed circuit potential decay in MFCs compared to Pt/C cathode-based MFCs.
SUSTAINABLE ENERGY & FUELS
(2022)
Correction
Chemistry, Physical
Dena Z. Khater, R. S. Amin, Amani E. Fetohi, K. M. El-Khatib, Mohamed Mahmoud
SUSTAINABLE ENERGY & FUELS
(2022)
Article
Otorhinolaryngology
Phoebe Kuo Yu, Kaitlyn Cook, Jiayan Liu, Raouf S. Amin, Craig Derkay, Lisa M. Elden, Susan L. Garetz, Alisha S. George, Sally Ibrahim, Stacey L. Ishman, Erin M. Kirkham, S. Kamal Naqvi, Jerilynn Radcliffe, Kristie R. Ross, Gopi B. Shah, Ignacio E. Tapia, H. Gerry Taylor, David A. Zopf, Susan Redline, Cristina M. Baldassari
Summary: The correlation between caregiver- and child-reported quality of life (QOL) in children with mild sleep-disordered breathing (SDB) was assessed. The study found a weak correlation between caregiver and child report, especially for young children. Higher child age and health literacy were associated with closer agreement between caregiver and child report.
OTOLARYNGOLOGY-HEAD AND NECK SURGERY
(2023)
Article
Biotechnology & Applied Microbiology
Enas M. Ghulam, Jane C. Khoury, Roman Jandarov, Raouf S. Amin, Eleni-Rosalina Andrinopoulou, Rhonda D. Szczesniak
Summary: This study proposes a Bayesian joint model with extended random effects structure for simultaneous inference on treatment effects over time and drop-out patterns in pediatric obstructive sleep apnea. The model includes flexible splines to characterize circadian variation in blood pressure sequences and shows discrepancies in estimating daytime blood pressure evolution compared to its conventional two-stage counterpart.
BIOMED RESEARCH INTERNATIONAL
(2022)
Meeting Abstract
Critical Care Medicine
Q. Xiao, K. McConnell, D. A. Ignatiuk, C. C. Gunatilaka, D. B. Gandhi, A. Schuh, R. S. Amin, A. Bates
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
(2021)
Meeting Abstract
Critical Care Medicine
N. J. Stewart, Q. Xiao, M. Willmering, C. C. Gunatilaka, R. P. Thomen, A. Schuh, G. Krishnamoorthy, H. Wang, R. S. Amin, C. L. Dumoulin, J. C. Woods, A. J. Bates
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
(2021)
Meeting Abstract
Critical Care Medicine
J. G. Morris, G. Mylavarapu, I. Y. Liu, R. S. Amin, R. J. Fleck
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
(2021)
Meeting Abstract
Critical Care Medicine
J. Gomez-Arroyo, M. Stephens, N. Gupta, F. X. McCormack, J. Elwing, R. S. Amin, G. Mylavarapu
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
(2021)
Article
Pediatrics
Tarun Aurora, Audrey Cole, Parul Rai, Paul Lavoie, Carrie Mcivor, Lisa M. Klesges, Guolian Kang, Janaka S. S. Liyanage, Heather M. Brandt, Jane S. Hankins
Summary: This study evaluated the effectiveness of a vaccine strategy bundle in increasing HPV vaccine initiation and completion rates in a specialty clinic setting. By implementing the bundle, which included staff education, provider incentives, offering vaccines in clinics, and verifying vaccine completion, the clinic successfully improved HPV vaccine initiation and completion rates among sickle cell disease patients.
JOURNAL OF PEDIATRICS
(2024)
Article
Pediatrics
Nienke M. Halbmeijer, Wes Onland, Jeroen Dudink, Filip Cools, Anne Debeer, Anton H. van Kaam, Manon J. N. L. Benders, Niek E. van der Aa
Summary: In ventilated infants born preterm, high dose systemic hydrocortisone initiated between 7 and 14 days after birth did not have a significant impact on brain development.
JOURNAL OF PEDIATRICS
(2024)
Article
Pediatrics
Katherine Melton, Jianfang Liu, Hossein Sadeghi, Maureen George, Arlene Smaldone
Summary: This study aims to identify predictors of change in lung function and body weight during health care transition in cystic fibrosis (CF) patients. The study findings highlight the importance of CF RISE program engagement and reducing gaps in care for improving the transition of adolescents and young adults with CF.
JOURNAL OF PEDIATRICS
(2024)
Article
Pediatrics
Laura A. Duckworth, Kimberly A. Sutton, Nurmohammad Shaikh, Jinli Wang, Carla Hall-Moore, Lori R. Holtz, Phillip I. Tarr, Ronald C. Rubenstein
Summary: The study tested the usefulness of various biomarkers as indicators of gut dysfunction in cystic fibrosis (CF) and investigated the repeatability of these measures in individuals over short periods and their correlation with clinical outcomes. The results showed that elevated levels of fLcn2 in individuals with CF may predict worsened pulmonary function.
JOURNAL OF PEDIATRICS
(2024)
Article
Pediatrics
Lindsey Haack, Nikkan Das, Arvind Hoskoppal, Mark Debrunner, Tarek Alsaied, Gaurav Arora
Summary: RAE on ECG has a low positive predictive value for RAE on echocardiogram in previously healthy young patients. The highest yield for RAE on echocardiogram was observed in patients who were <1 year of age, had RAE in the anterior precordial leads, or displayed right ventricular hypertrophy on ECG.
JOURNAL OF PEDIATRICS
(2024)
Article
Pediatrics
Michael A. Padula, Khatija Naing, Tara L. Wenger, Irfan Ahmad, Carl H. Coghill, K. Taylor Wild, S. Alex Rottgers, Cory M. Resnick, Jeffrey Goldstein, Zarmina Ehsan, Donna Watkins, Nicole Deptula, Kuan-Chi Lai, Janet Lioy, Semsa Gogcu, Christopher M. Cielo
Summary: This study describes the spectrum of disease and burden of care in infants with congenital micrognathia. The results show that these infants commonly require surgical intervention and tube feedings, and disparities based on race and among centers were identified.
JOURNAL OF PEDIATRICS
(2024)
Article
Pediatrics
Michael P. Fundora, Manvitha Kalicheti, Guantao Zhao, Kevin O. Maher, Nicoleta Serban
Summary: This study investigated the variation of outpatient opioid prescribing in postoperative pediatric cardiac patients across the US. The results showed that there were significant differences in opioid prescribing by race, ethnicity, sex, and region.
JOURNAL OF PEDIATRICS
(2024)
Article
Pediatrics
Jia Guo, Brooklyn J. Fraser, Leigh Blizzard, Michael D. Schmidt, Terence Dwyer, Alison J. Venn, Costan G. Magnussen
Summary: There is a correlation between childhood and adulthood cardiorespiratory fitness.
JOURNAL OF PEDIATRICS
(2024)
Article
Pediatrics
Nianzhou Xiao, Michelle Starr, Adrienne Stolfi, Gilad Hamdani, Shireen Hashmat, Stefan G. Kiessling, Christina Sethna, Mahmoud Kallash, Robyn Matloff, Robert Woroniecki, Keia Sanderson, Ikuyo Yamaguchi, Stephen D. Cha, Michael G. Semanik, Rahul Chanchlani, Joseph T. Flynn, Mark Mitsnefes
Summary: This multicenter study reports that most infants diagnosed with idiopathic hypertension in the NICU will discontinue antihypertensive treatment within 2 years of discharge. Antenatal steroid treatment is associated with a decreased likelihood of needing antihypertensive therapy for more than 1 year.
JOURNAL OF PEDIATRICS
(2024)
Editorial Material
Pediatrics
Kanwaljit Singh, John Concato, Jonathan M. Davis
JOURNAL OF PEDIATRICS
(2024)
Article
Pediatrics
Yaxing Meng, Harri Niinikoski, Suvi P. Rovio, Brooklyn J. Fraser, Feitong Wu, Antti Jula, Tapani Ronnemaa, Jorma S. A. Viikari, Olli T. Raitakari, Katja Pahkala, Costan G. Magnussen
Summary: This 26-year study shows a correlation between early-life non-HDL-C levels and future levels. Early dietary counseling can reduce the risk of high pediatric non-HDL-C, emphasizing the importance of early interventions in preventing cardiovascular risks.
JOURNAL OF PEDIATRICS
(2024)
Article
Pediatrics
Kelsey A. B. Gastineau, Rebecca Bell, Allison Hanes, Sandra Mckay, Eric Sigel, Filoteia Popescu, Evan C. Sommer, Shari Barkin
Summary: This study aimed to assess the self-reported counseling outcomes for a firearm safe storage counseling training program provided by the American Academy of Pediatrics. The results demonstrated significant improvement in counseling self-efficacy and frequency one month after the training.
JOURNAL OF PEDIATRICS
(2024)
Article
Pediatrics
Kathryn E. K. Berlin, William Scott, Sara Dawson, David Brousseau, Joanne M. Lagatta
Summary: This prospective cohort study aimed to investigate the impact of bronchopulmonary dysplasia (BPD) on the health-related quality of life (HRQL) of infants from NICU hospitalization to one year post-discharge. The study found that lower HRQL during NICU stay was associated with earlier gestational age, postnatal corticosteroid usage, outborn status, and gastrostomy tube placement. Lower HRQL at 3 and 12 months post-discharge was associated with readmissions, home oxygen use, parent-reported difficulty breathing, lower developmental scores, and not playing with other children. Most parents reported similar or improved HRQL after discharge, but parents of infants with respiratory symptoms experienced less improvement. Efforts to improve parent HRQL should focus on respiratory symptoms and social isolation.
JOURNAL OF PEDIATRICS
(2024)
Article
Pediatrics
Garett J. Griffith, Alan P. Wang, Robert I. Liem, Michael R. Carr, Tyler Corson, Kendra Ward
Summary: This study developed reference values for cardiorespiratory fitness in children aged 6-18 years without underlying heart disease, measured by peak oxygen uptake and treadmill time. Fitness levels increased with age in males but not females. Males generally exhibited higher fitness levels compared to females in the same age groups.
JOURNAL OF PEDIATRICS
(2024)
Article
Pediatrics
David S. Liu, Patricia Miller, Anna Rothenberg, Carley Vuillermin, Peter M. Waters, Andrea S. Bauer
Summary: This study aims to determine if children with elbow flexion contracture (EFC) caused by brachial plexus birth injury (BPBI) are more likely to develop shoulder contracture and undergo surgical treatment. A retrospective review was conducted on children under 2 years old with BPBI who presented to a single children's hospital. The results showed that patients with EFC had reduced shoulder range of motion and higher odds of shoulder contracture and surgical treatment. Prompt referral to a BPBI specialty clinic is recommended for evaluation and potential surgery.
JOURNAL OF PEDIATRICS
(2024)