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Pediatric Pulmonology

Deborah R Liptzin, Christopher D Baker, Jeffrey R Darst, Jason P Weinman, Megan K Dishop, Csaba Galambos, John T Brinton, Robin R Deterding
OBJECTIVES: We sought to describe the phenotype for patients with P.I.G. including presentation, evaluation, cardiac co-morbidities, high resolution computed tomography findings, and outcomes. METHODS: With institutional review board approval, we performed a retrospective review of patients with biopsy-proven P.I.G. Biopsies, high resolution chest computed tomography, and cardiac evaluations were reviewed and characterized by experts in each field. RESULTS: Sixty-two percent of the patients were male...
July 18, 2018: Pediatric Pulmonology
Eline Lauwers, Kris Ides, Kim Van Hoorenbeeck, Stijn Verhulst
BACKGROUND: Intrapulmonary percussive ventilation (IPV) is frequently used in clinical practice to enhance sputum evacuation and lung recruitment. However, the evidence in different respiratory pathologies, especially in children, is still lacking. This systematic review aims to enlist the effectiveness of IPV as an airway clearance technique in pediatric patients. DATA SOURCES: A systematic literature search was performed in PubMed, Web of Science, and the Cochrane Library databases...
July 18, 2018: Pediatric Pulmonology
Andrea J Thompson, Emma L Sidebotham, Philip A J Chetcuti, David C G Crabbe
AIM: To report the natural history of a cohort of children with prenatally diagnosed congenital lung malformations (CLM) which we set out to manage expectantly. METHODS: Retrospective review of children born between 1995 and 2013 with a CLM identified on prenatal ultrasound. Prenatal ultrasound data were analyzed along with patient medical records, radiology, and pathology. RESULTS: One hundred fifty-nine newborns with a fetal lung lesion were identified during the study period...
July 15, 2018: Pediatric Pulmonology
Mehmet Buyuktiryaki, Hayriye G Kanmaz, Nilufer Okur, Handan Bezirganoglu, Buse Bekmez, Gulsum Kadioglu Simsek, Fuat E Canpolat, Suna S Oguz, Cuneyt Tayman
BACKGROUND AND OBJECTIVES: It is believed, that sustained lung inflation (SLI) at birth in preterm infants reduces the need for mechanical ventilation (MV) and improves respiratory outcomes. The aim of this study was to compare need for MV in preterm infants at high risk for respiratory distress syndrome (RDS) after prophylactic SLI via short binasal prongs at birth combined with early nasal continuous positive airway pressure (nCPAP) versus nCPAP alone. METHODS: Medical records of infants born at 260/7 to 296/7 weeks gestation through 2015 and 2017 were retrospectively assessed...
July 12, 2018: Pediatric Pulmonology
Abigail Strang, Lauren Ryan, Tariq Rahman, Sona Balasubramanian, Jobayer Hossain, Robert Heinle, Thomas H Shaffer
BACKGROUND: Pulmonary function testing (PFT) is essential for the clinical assessment of respiratory problems. Respiratory inductance plethysmography (RIP) is a non-invasive method of PFT requiring minimal patient cooperation. RIP measures the volumetric change in the ribcage and abdomen, from which work of breathing (WOB) indices are derived. WOB indices include: phase angle (Ф), percent ribcage (RC%), respiratory rate (RR), and labored breathing index (LBI). Heart rate (HR) is collected separately...
July 12, 2018: Pediatric Pulmonology
Roberto Bottino, Federica Pontiggia, Cinzia Ricci, Alessandro Gambacorta, Angela Paladini, Vladimiras Chijenas, Arunas Liubsys, Jurate Navikiene, Ausrine Pliauckiene, Domenica Mercadante, Mariarosa Colnaghi, Milena Tana, Chiara Tirone, Alessandra Lio, Claudia Aurilia, Roberta Pastorino, Velia Purcaro, Gianfranco Maffei, Pio Liberatore, Chiara Consigli, Cristina Haass, Gianluca Lista, Massimo Agosti, Fabio Mosca, Giovanni Vento
OBJECTIVE: To compare short-term application of nasal high-frequency oscillatory ventilation (nHFOV) with nasal continuous positive airway pressure (nCPAP). WORKING HYPOTHESIS: nHFOV improves CO2 removal with respect to nCPAP in preterm infants needing noninvasive respiratory support and persistent oxygen supply after the first 72 h of life. STUDY DESIGN: Multicenter non-blinded prospective randomized crossover study. PATIENT SELECTION: Thirty premature infants from eight tertiary neonatal intensive care units, of mean ± SD 26...
July 12, 2018: Pediatric Pulmonology
Ahsan Bashir, Augusto A Litonjua
No abstract text is available yet for this article.
July 12, 2018: Pediatric Pulmonology
Erica A Roesch, David P Nichols, James F Chmiel
Inflammation plays a critical role in cystic fibrosis (CF) lung pathology and disease progression making it an active area of research and important therapeutic target. In this review, we explore the most recent research on the major contributors to the exuberant inflammatory response seen in CF as well as potential therapeutics to combat this response. Absence of functional cystic fibrosis transmembrane conductance regulator (CFTR) alters anion transport across CF airway epithelial cells and ultimately results in dehydration of the airway surface liquid...
July 12, 2018: Pediatric Pulmonology
Tuğba Şişmanlar Eyüboğlu, Ayşe Tana Aslan, Alper Ceylan, Azime Şebnem Soysal, Işıl İrem Budakoğlu, Tansu Ulukavak Çiftçi, Oğuz Köktürk
BACKGROUND: Primary ciliary dyskinesia (PCD) patients have higher incidence of sleep disordered breathing which lead neurocognitive impairments such as attention-deficit/hyperactivity disorder (ADHD). It may effect academic performance of children and may cause impairment in emotional relationships. This study aim to evaluate hyperactivity and attention deficiency in PCD patients and investigate the relationship between sleep and hyperactivity and attention deficiency in PCD patients...
July 10, 2018: Pediatric Pulmonology
Michael C Tracy, Richard B Moss
Fungal infection in cystic fibrosis (CF) is a recognized challenge, with many areas requiring further investigation. Consensus definitions exist for allergic bronchopulmonary aspergillus in CF, but the full scope of clinically relevant non-allergic fungal disease in CF-asymptomatic colonization, transient or chronic infection localized to endobronchial mucus plugs or airway tissue, and invasive disease-is yet to be clearly defined. Recent advances in mycological culture and non-culture identification have expanded the list of both potential pathogens and community commensals in the lower respiratory tract...
July 10, 2018: Pediatric Pulmonology
Cecilia Hagman, Lars J Björklund, Gunnel Hellgren, Ellen Tufvesson, Ingrid Hansen-Pupp
BACKGROUND: Club cell secretory protein (CC16) probably has a role in protecting the lung from inflammation. AIM: To evaluate if low levels of CC16 in gastric fluid at birth, reflecting low levels of CC16 in the lung, would be associated with lung inflammation and respiratory morbidity. METHODS: A study of 64 infants with mean gestational age 26.1 weeks. CC16 was analyzed in gastric fluid at birth. CC16, pro-inflammatory cytokines, and MMP-9 were analyzed in tracheal aspirate within 24 h from birth...
July 10, 2018: Pediatric Pulmonology
Federica Trucco, Michelle Chatwin, Thomas Semple, Mark Rosenthal, Andrew Bush, Hui-Leng Tan
STUDY OBJECTIVES: Obstructive sleep apnoea (OSAS) in children with Down syndrome (DS) is now well recognized, but other forms of sleep disordered breathing (SDB) in this population are less well described. Anecdotally, respiratory support for SDB treatment in this population is not easily tolerated. We aimed to characterize the types of SDB in children with DS referred to a tertiary respiratory center and to assess the effectiveness and adherence to respiratory support. METHODS: Retrospective study of DS patients <18 years old under follow-up at a tertiary respiratory center...
July 10, 2018: Pediatric Pulmonology
Mikhail Kazachkov, Bianca C Kapoor, Patrick W Malecha, Benjamin G Wu, Yonghua Li, Jeremiah Levine, Jessica Erkman, Kathryn Fitzgerald, Libia Moy, Leopoldo N Segal
In pediatric patients with chronic cough, respiratory culture techniques commonly yield negative results. Studies using culture-independent methods have found a high relative abundance of oral microbes in the lower airways, suggesting that the topographical continuity, and dynamics of the intraluminal contents of the aerodigestive system likely influence the lower airway microbiota. We hypothesize that in subjects with chronic cough, clinical diagnosis will correlate with distinct microbial signatures detected using culture-independent methods...
July 8, 2018: Pediatric Pulmonology
Katy Newbegin, Katie Pilkington, Shivanthan Shanthikumar, Sarath Ranganathan
BACKGROUND: In cystic fibrosis (CF), irreversible lung disease arises in early life, and is often asymptomatic and unrecognised. Chest computed tomography (CT) scans have been used to detect asymptomatic lung disease in research; however, the clinical utility of chest CT is unknown. This study aimed to determine the effect of surveillance CT in early life on the clinical management of patients with CF. WORKING HYPOTHESIS: Surveillance CT in early life changes the management of patients with CF...
July 8, 2018: Pediatric Pulmonology
Emily F Muther, Deepika Polineni, Greg S Sawicki
Individuals living with cystic fibrosis (CF), and their families, have experienced significant improvements in treatment and related research that have enhanced outcomes and survival. Despite such advancement, the burden of living with CF still exists. Many psychosocial stressors and risk factors are associated with the impact of CF. The identification and treatment of such risk factors are discussed throughout this review, with an emphasis on strategies to address psychosocial risk and the importance of promoting resiliency in those touched by CF...
July 6, 2018: Pediatric Pulmonology
Michael S Schechter
Cystic Fibrosis (CF) lung disease is characterized by intermittent acute episodes of worsening signs, symptoms, and pulmonary function; these so-called pulmonary exacerbations (PEx) appear to be important drivers of long-term declines in lung function, quality of life, and life expectancy. Surveillance for development of PEx and their treatment is a fundamental component of chronic CF management, and the merits of novel CF therapies are often judged based on their ability to reduce the frequency of PEx. Given the central role that they play, it is surprising how poorly PEx are understood, how thin is the evidence base for their treatment and how often they are left unrecognized and untreated in clinical practice...
July 6, 2018: Pediatric Pulmonology
Tae-Jung Sung, Sook M Hwang, Min Y Kim, Shin G Park, Ka Y Choi
BACKGROUND: To assess the relationship between HRCT abnormalities and the clinical severity of "new" bronchopulmonary dysplasia (BPD) and respiratory assistance in <1500 g preterm infants. METHODS: HRCT was performed at the time of discharge in 109 preterm infants with BPD who were born between 2008 and 2014. BPD severity was defined as mild, moderate, or severe according to the NIH diagnostic criteria. Total HRCT scores for each infant were obtained from the sum of the number of BPD lobes assessed by two radiologists: a hyperaeration score (Hs) composed of decreased attenuation, mosaic attenuation, and bulla/bleb, and a parenchyma score (Ps) composed of linear lesions, consolidation, bronchial wall thickening, and bronchiectasis...
July 6, 2018: Pediatric Pulmonology
Jun Luo, Suzanne Shepard, Kathleen Nilan, Audrey Wood, Heather M Monk, Erik A Jensen, Ann T Harrington, Kathryn Maschhoff, Haresh Kirpalani, Zhichun Feng, Huayan Zhang
OBJECTIVES: To examine growth, sedation needs, and participation in developmental activities before and after tracheostomy among infants with severe bronchopulmonary dysplasia. METHODS: Retrospective analysis of infants born at <32 weeks' gestation or birth weights <1500 g with severe BPD who underwent tracheostomy placement between January 1, 2010 and December 31, 2016 in a quaternary referral newborn and infant intensive care unit. Changes in growth parameters and frequency/type of participation in physical therapy sessions performed during the 4-weeks before tracheostomy and 4-weeks after the first tracheostomy tube change were compared...
July 3, 2018: Pediatric Pulmonology
Nicole Beydon, Thu Thuy Nguyen, Francis Amsallem, André Denjean, Grazia Fenu, Paul Seddon, France Mentré, Corinne Alberti, Enrico Lombardi
AIM: Using a non-invasive lung function technique (interrupter resistance, Rint), we aimed to determine whether a dose-response to salbutamol could be detected in wheezy preschool children and if so, which dose of salbutamol should be administered to routinely evaluate bronchial reversibility. METHOD: Wheezy children (3 to <7 years) were enrolled in a prospective multicenter study. Rint was measured at baseline, and after random assignment to a first dose (100 or 200 μg) and a second dose (cumulative dose: 400, 600, or 800 μg) of salbutamol...
July 3, 2018: Pediatric Pulmonology
Daniel Trachsel, Jürg Hammer
BACKGROUND: Laryngeal clefts (LC) are uncommon but important causes of stridor in infants. Direct laryngoscopy is the recommended method for the detection of LC because LC may be missed by flexible endoscopy. As laryngomalacia by far outnumbers any other cause of stridor in this age group flexible bronchoscopy is usually the preferred method for the evaluation of significant infantile stridor. The aim is to illustrate how the application of CPAP assists the detection of LC during flexible endoscopy...
July 3, 2018: Pediatric Pulmonology
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