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cystic fibrosis children

Maiara Brusco de Freitas, Emilia Addison Machado Moreira, Camila Tomio, Yara Maria Franco Moreno, Felipe Perozzo Daltoe, Eliana Barbosa, Norberto Ludwig Neto, Vittoria Buccigrossi, Alfredo Guarino
The aim of the present study was to evaluate the effect of cystic fibrosis and antibiotic therapy on intestinal microbiota composition and intestinal inflammation in children and adolescents. A cross-sectional controlled study was conducted with 36 children and adolescents: 19 in the cystic fibrosis group (CFG) and 17 in the control group (CG) matched for age and sex. The CFG was subdivided based on the use of antibiotic therapy (CFAB group) and non-use of antibiotic therapy (CFnAB group). The following data were evaluated: colonization, antibiotic therapy, mutation, breastfeeding, use of infant formula, type of delivery, introduction of solid foods, body mass index, fecal calprotectin and intestinal microbiota composition (fluorescence in situ hybridization)...
2018: PloS One
Maisam Abu-El-Haija, Aliye Uc, Steven L Werlin, Alvin Jay Freeman, Miglena Georgieva, Danijela Jojkić-Pavkov, Daina Kalnins, Brigitte Kochavi, Bart G P Koot, Stephanie Van Biervliet, Jaroslaw Walkowiak, Michael Wilschanski, Veronique D Morinville
OBJECTIVES: Wide variations exist in how physicians manage the nutritional aspects of children affected by acute pancreatitis (AP), acute recurrent pancreatitis (ARP), and chronic (CP) pancreatitis. Better consensus for optimal management is needed. METHODS: This consensus statement on nutrition in pediatric pancreatic diseases was developed through a joint ESPGHAN-NASPGHAN working group that performed an evidence-based search of the literature on nutrition in AP, ARP, and CP with a focus on pediatrics...
July 2018: Journal of Pediatric Gastroenterology and Nutrition
Gisela Hovold, Victoria Palmcrantz, Fredrik Kahn, Arne Egesten, Lisa I Påhlman
BACKGROUND: Cystic fibrosis (CF) is associated with bacterial pulmonary infections and neutrophil-dominated inflammation in the airways. The aim of this study was to evaluate the neutrophil-derived protein Heparin-binding protein (HBP) as a potential sputum marker of airway inflammation and bacterial load. METHODS: Nineteen CF patients, aged 6-18 years, were prospectively followed for 6 months with sputum sampling at every visit to the CF clinic. A total of 41 sputum samples were collected...
June 20, 2018: BMC Pulmonary Medicine
Jordana E Hoppe, Brandie D Wagner, Frank J Accurso, Edith T Zemanick, Scott D Sagel
BACKGROUND: Pulmonary exacerbations (PEx) in children with cystic fibrosis (CF) are frequently treated in the outpatient setting with oral antibiotics. However, little is known about the characteristics of PEx managed on an outpatient basis and the effectiveness of oral antibiotic therapy. We sought to prospectively evaluate clinical and laboratory changes associated with oral antibiotic treatment for PEx. METHODS: Children with CF between 8 and 18 years of age prescribed two weeks of oral antibiotics for a PEx were eligible to enroll...
June 16, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
Peter König, Zarah Ner, James D Acton, Bin Ge, John Hewett
Introduction- FEV1 is considered the gold standard spirometric measure for the assessment and management of cystic fibrosis lung disease. Recent evidence suggests that tests at lower lung volumes may be more sensitive. Objectives- To assess how many other spirometric tests are abnormal in the presence of a normal FEV1 (≥80%) and which spirometric tests are most sensitive in detecting airway obstruction. Methods -This was a retrospective analysis of 3,169 spirometry tests on 184 patients with cystic fibrosis aged 6 - 57 years...
June 19, 2018: Clinical Respiratory Journal
James A Hammond, Gary J Connett
Lumacaftor/ivacaftor is a precision medicine targeting the defective cystic fibrosis transmembrane regulator (CFTR) protein in cystic fibrosis (CF) patients homozygous for Phe508del genotype. Whilst there is evidence for efficacy in children aged 6-11 years who are stable with good lung function, there are little data about the use of this medication for children with acute deterioration in this age group. We describe the use of this drug to treat a child with an unusually severe exacerbation of CF lung disease and review the potential of lumacaftor/ivacaftor as a rescue therapy in the paediatric CF population...
May 19, 2018: Paediatric Respiratory Reviews
Zihang Lu, Rachel E Foong, Krzysztof Kowalik, Theo J Moraes, Ayanna Boyce, Aimee Dubeau, Susan Balkovec, Per Magnus Gustafsson, Allan B Becker, Piush J Mandhane, Stuart E Turvey, Wendy Lou, Felix Ratjen, Malcolm Sears, Padmaja Subbarao
BACKGROUND: The care of infants with recurrent wheezing relies largely on clinical assessment. The lung clearance index (LCI), a measure of ventilation inhomogeneity, is a sensitive marker of early airway disease in children with cystic fibrosis, but its utility has not been explored in infants with recurrent wheezing. OBJECTIVE: To assess ventilation inhomogeneity using LCI among infants with a history of recurrent wheezing compared with healthy controls. METHODS: This is a case-control study, including 37 infants with recurrent wheezing recruited from outpatient clinics, and 113 healthy infants from a longitudinal birth cohort, the Canadian Healthy Infant Longitudinal Development study...
June 15, 2018: Thorax
Jennifer Adjemian, Kenneth N Olivier, D Rebecca Prevots
RATIONALE: Pulmonary nontuberculous mycobacterial (NTM) disease represents a significant threat to cystic fibrosis (CF) patients, with an estimated annual prevalence of 12%. Prior studies report an increasing annual NTM prevalence in the general population, though similar trends in persons with CF have not been assessed. OBJECTIVES: This study aimed to identify the prevalence, geographic patterns, temporal trends and risk factors for NTM positivity by mycobacterial species among persons with CF throughout the United States...
June 13, 2018: Annals of the American Thoracic Society
Jessica Green, Francis J Gilchrist, Will Carroll
BACKGROUND: Cystic fibrosis (CF) is the most common, life-limiting, genetically inherited disease. It affects multiple organs, particularly the respiratory system. However, gastrointestinal problems such as constipation and distal intestinal obstruction syndrome (DIOS) are also important and well-recognised complications in CF. They share similar symptoms e.g. bloating, abdominal pain, but are distinct conditions. Constipation occurs when there is gradual faecal impaction of the colon, but DIOS occurs when there is an accumulation of faeces and sticky mucus, forming a mass in the distal part of the small intestine...
June 12, 2018: Cochrane Database of Systematic Reviews
Krishna Mohan Gulla, Arvind Balaji, Aparna Mukherjee, Kana Ram Jat, Jhuma Sankar, Rakesh Lodha, Sushil K Kabra
Objective: To study the clinical impact of respiratory viral infection in children with cystic fibrosis (CF). Design: Retrospective cohort study. Setting: Tertiary care referral centre for CF in India. Participants/patients: Children with CF attending a pediatric chest clinic. Methods: Case records of the children with CF who had a pulmonary exacerbation with documented acute respiratory viral infection between October 2013 and December 2014 (Group I) and an equal number of controls (Group II) with pulmonary exacerbation in absence of acute respiratory viral infection were reviewed...
June 9, 2018: Journal of Tropical Pediatrics
Sara Ebrahimi, Gholamreza Khademi, Seyed Ali Jafari, Nona Zaboli Nejad, Abdolreza Norouzy, Bahareh Imani
Very-early-onset inflammatory bowel disease (VEO-IBD) has a distinct phenotype and should be considered a specific entity. VEO-IBD presents with very severe clinical pictures and is frequently known by an indeterminate colitis whose clinical remission is unmanageable. This study examines the case of a neonate with VEO-IBD, not responding to medical and surgical treatment. A 7-day-old Iranian female neonate presented with severe bloody diarrhea, poor feeding, abdominal distention, and dehydration suggesting severe proctocolitis due to an allergy to the protein in cow's milk...
May 2018: Iranian Journal of Medical Sciences
Nicole Mayer-Hamblett, George Retsch-Bogart, Margaret Kloster, Frank Accurso, Margaret Rosenfeld, Gary Albers, Philip Black, Perry Brown, AnneMarie Cairns, Stephanie D Davis, Gavin R Graff, Gwendolyn S Kerby, David Orenstein, Rachael Buckingham, Bonnie W Ramsey
RATIONALE: New isolation of Pseudomonas aeruginosa (Pa) is generally treated with inhaled antipseudomonal antibiotics such as tobramycin inhalation solution (TIS). A therapeutic approach complementing traditional antimicrobial therapy by reducing the risk of pulmonary exacerbation (PEx) and inflammation may ultimately prolong time to Pa recurrence. OBJECTIVES: To test the hypothesis that the addition of azithromycin to TIS in children with CF and early Pa decreases the risk of PEx and prolongs time to Pa recurrence...
June 11, 2018: American Journal of Respiratory and Critical Care Medicine
Margaret Rosenfeld, Claire E Wainwright, Mark Higgins, Linda T Wang, Charlotte McKee, Daniel Campbell, Simon Tian, Jennifer Schneider, Steve Cunningham, Jane C Davies
BACKGROUND: Ivacaftor is generally safe and effective in patients aged 2 years and older who have cystic fibrosis and specific CFTR mutations. We assessed its use in children aged 12 to <24 months. METHODS: The ARRIVAL study is a phase 3, single-arm, two-part, multicentre study. Eligible children were aged 12 to <24 months at enrolment and had a confirmed diagnosis of cystic fibrosis and a CFTR gating mutation on at least one allele and could participate in one or both parts of the study...
June 6, 2018: Lancet Respiratory Medicine
Samuel T Montgomery, A Susanne Dittrich, Luke W Garratt, Lidija Turkovic, Dario L Frey, Stephen M Stick, Marcus A Mall, Anthony Kicic
BACKGROUND: Little is known about the role of interleukin (IL)-1 in the pathogenesis of cystic fibrosis (CF) lung disease. This study investigated the relationship between IL-1 signalling, neutrophilic inflammation and structural lung changes in children with CF. METHODS: Bronchoalveolar lavage fluid (BALf) from 102 children with CF were used to determine IL-1α, IL-1β, IL-8 levels and neutrophil elastase (NE) activity, which were then correlated to structural lung changes observed on chest computed tomography (CT) scans...
June 5, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
Sahana Shankar, Rishi Bolia, Alexandra Hodgson, Jonathan R Bishop, Helen M Evans, Mark R Oliver
We report the first two pediatric patients with CF who underwent successful combined liver-pancreas transplantation in Australia and New Zealand for CF liver disease and CF-related diabetes mellitus.
June 6, 2018: Pediatric Transplantation
Brian R Branchford, Shannon L Carpenter
Venous thromboembolism (VTE), comprising deep vein thrombosis (DVT), and pulmonary embolism (PE), is becoming increasingly recognized as a cause of morbidity and mortality in pediatrics, particularly among hospitalized children. Furthermore, evidence is accumulating that suggests the inflammatory response may be a cause, as well as consequence, of VTE, but current anticoagulation treatment regimens are not designed to inhibit inflammation. In fact, many established clinical VTE risk factors such as surgery, obesity, cystic fibrosis, sepsis, systemic infection, cancer, inflammatory bowel disease, and lupus likely modulate thrombosis through inflammatory mediators...
2018: Frontiers in Pediatrics
Astrid M Ring, Frederik F Buchvald, Mathias G Holgersen, Kent Green, Kim G Nielsen
BACKGROUND: Peak oxygen uptake (VO2peak ) is associated with morbidity and mortality in health and disease, and provides important information of global physical health not achieved from standard pulmonary function tests. Primary ciliary dyskinesia (PCD) and cystic fibrosis (CF) are genetically determined diseases involving different basic defects, but both showing impaired mucociliary clearance leading to chronic infections and pulmonary destruction early in life. PCD is generally considered a milder disease than CF and it is hypothesized that children with CF would have consistently lower VO2peak and pulmonary function than children with PCD...
June 2018: Respiratory Medicine
Mark O Wielpütz, Oyunbileg von Stackelberg, Mirjam Stahl, Bertram J Jobst, Monika Eichinger, Michael U Puderbach, Lutz Nährlich, Sandra Barth, Christian Schneider, Matthias V Kopp, Isabell Ricklefs, Michael Buchholz, Burkhard Tümmler, Christian Dopfer, Jens Vogel-Claussen, Hans-Ulrich Kauczor, Marcus A Mall
BACKGROUND: A recent single-centre study demonstrated that MRI is sensitive to detect early abnormalities in the lung and response to therapy in infants and preschool children with cystic fibrosis (CF) supporting MRI as an outcome measure of early CF lung disease. However, the feasibility of multicentre standardisation remains unknown. OBJECTIVE: To determine the feasibility of multicentre standardisation of chest MRI in infants and preschool children with CF. METHODS: A standardised chest 1...
May 24, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
Jun Ting Chau, Karen Peebles, Yvonne Belessis, Adam Jaffe, Michael Doumit
BACKGROUND: Oropharyngeal suction and oropharyngeal swab are two methods of obtaining airway samples with similar diagnostic accuracy in children with cystic fibrosis (CF). The primary aim was comparing distress between suctioning and swabbing. A secondary aim was establishing the reliability of the Groningen Distress Rating Scale (GDRS). METHODS: Randomised oropharyngeal suction or swab occurred over two visits. Two physiotherapists and the child's parent rated distress using the GDRS...
May 25, 2018: Archives of Disease in Childhood
Nadine Pellen, Laëtitia Guéganton, Dominique Pougheon Bertrand, Gilles Rault
BACKGROUND: The French Cystic Fibrosis Registry takes a census of the population of patients and records their annual data transmitted by Cystic Fibrosis Centers (CFCs). Quality of patient data has been a focus in the past years, with the implementation of automated controls before data integration. The objective was to assess, at the 14 CFCs trained in the quality improvement named Hospital Program to Improve Outcomes and Expertise in Cystic Fibrosis (PHARE-M), the quality of the 2012 and 2013 data transmitted to the French Registry with respect to the rules established to obtain forced expiratory volume in 1 second (FEV1%) and anthropometric data...
February 8, 2018: Orphanet Journal of Rare Diseases
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