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Cystic fibrosis in infants

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https://www.readbyqxmd.com/read/28811149/three-center-feasibility-of-lung-clearance-index-in-infants-and-preschool-children-with-cystic-fibrosis-and-other-lung-diseases
#1
Mirjam Stahl, Simon Y Graeber, Cornelia Joachim, Sandra Barth, Isabell Ricklefs, Gesa Diekmann, Matthias V Kopp, Lutz Naehrlich, Marcus A Mall
BACKGROUND: Lung clearance index (LCI) detects early ventilation inhomogeneity and has been suggested as sensitive endpoint in multicenter intervention trials in infants and preschoolers with cystic fibrosis (CF). However, the feasibility of multicenter LCI in this age group has not been determined. We, therefore, investigated the feasibility of LCI in infants and preschoolers with and without CF in a three-center setting. METHODS: Following central training, standardized SF6-MBW measurements were performed in 73 sedated children (10 controls, 49 with CF and 14 with other lung diseases), mean age 2...
August 12, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28745925/clinical-care-of-children-with-primary-ciliary-dyskinesia
#2
Jane S Lucas, Mikkel Christian Alanin, Samuel Collins, Amanda Harris, Helle Krogh Johansen, Kim G Nielsen, Jean Francois Papon, Phil Robinson, Woolf T Walker
Primary ciliary dyskinesia (PCD) is a rare heterogeneous disorder, usually inherited as an autosomal recessive condition but X-linked inheritance is also described. Abnormal ciliary function in childhood leads to neonatal respiratory distress in term infants, persistent wet cough, bronchiectasis, chronic rhinosinusitis, and hearing impairment; approximately 50% of patients have situs inversus. There is a paucity of evidence for treating PCD, hence consensus guidelines are predominantly influenced by knowledge from cystic fibrosis (CF)...
July 26, 2017: Expert Review of Respiratory Medicine
https://www.readbyqxmd.com/read/28716479/lower-exhaled-nitric-oxide-in-infants-with-cystic-fibrosis-compared-to-healthy-controls
#3
Insa Korten, Margot Liechti, Florian Singer, Gaudenz Hafen, Isabelle Rochat, Pinelopi Anagnostopoulou, Dominik Müller-Suter, Jakob Usemann, Alexander Moeller, Urs Frey, Philipp Latzin, Carmen Casaulta
Exhaled nitric oxide (FENO) is a well-known, non-invasive airway biomarker. In patients with Cystic Fibrosis (CF) FENO is decreased. To understand if reduced FENO is primary related to Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) dysfunction or an epiphenomenon of chronic inflammation, we measured FENO in 34 infants with CF prior to clinical symptoms and in 68 healthy controls. FENO was lower in CF compared to controls (p=0.0006) and the effect was more pronounced in CF infants without residual CFTR function (p<0...
July 14, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28712180/causes-of-chronic-non-infectious-diarrhoea-in-infants-less-than-6-months-of-age-rarely-recognized-entities
#4
Iqra Mushtaq, Huma Ashraf Cheema, Hassan Suleman Malik, Nadia Waheed, Muhammad Almas Hashmi, Hassan Suleman Malik
BACKGROUND: Non-infectious causes of chronic diarrhoea are important and easily missed. The study was done with the objectives to identify different causes of chronic non-infectious diarrhoea in infants less than 6 months of age. METHODS: All patients less than 6 months of age presenting for the first time to a Paediatric Gastroenterology tertiary care centre with a history of chronic diarrhoea and negative stool cultures were enrolled over a period of 8 months...
January 2017: Journal of Ayub Medical College, Abbottabad: JAMC
https://www.readbyqxmd.com/read/28708417/association-of-antibiotics-airway-microbiome-and-inflammation-in-infants-with-cystic-fibrosis
#5
Jessica E Pittman, Kristine M Wylie, Kathryn Akers, Gregory A Storch, Joseph Hatch, Jane Quante, Katherine B Frayman, Nadeene Clarke, Miriam Davis, Stephen M Stick, Graham L Hall, Gregory Montgomery, Sarath Ranganathan, Stephanie D Davis, Thomas W Ferkol
RATIONALE: The underlying defect in the cystic fibrosis (CF) airway leads to defective mucociliary clearance and impaired bacterial killing, resulting in endobronchial infection and inflammation that contributes to progressive lung disease. Little is known about the respiratory microbiota in the early CF airway and its relationship to inflammation. OBJECTIVES: To examine the bacterial microbiota and inflammatory profiles in bronchoalveolar lavage fluid and oropharyngeal secretions in infants with CF...
July 14, 2017: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/28653300/defining-the-incidence-and-associated-morbidity-and-mortality-of-severe-respiratory-syncytial-virus-infection-among-children-with-chronic-diseases
#6
REVIEW
Paolo Manzoni, Josep Figueras-Aloy, Eric A F Simões, Paul A Checchia, Brigitte Fauroux, Louis Bont, Bosco Paes, Xavier Carbonell-Estrany
INTRODUCTION: REGAL (RSV Evidence-a Geographical Archive of the Literature) has provided a comprehensive review of the published evidence in the field of respiratory syncytial virus (RSV) in Western countries over the last 20 years. This review covers the risk and burden of RSV infection in children with underlying medical conditions or chronic diseases (excluding prematurity and congenital heart disease). METHODS: A systematic review of publications between January 1, 1995 and December 31, 2015 across PubMed, Embase, The Cochrane Library, and Clinicaltrials...
June 26, 2017: Infectious Diseases and Therapy
https://www.readbyqxmd.com/read/28649564/primary-ciliary-dyskinesia-an-update-on-clinical-aspects-genetics-diagnosis-and-future-treatment-strategies
#7
REVIEW
Virginia Mirra, Claudius Werner, Francesca Santamaria
Primary ciliary dyskinesia (PCD) is an orphan disease (MIM 244400), autosomal recessive inherited, characterized by motile ciliary dysfunction. The estimated prevalence of PCD is 1:10,000 to 1:20,000 live-born children, but true prevalence could be even higher. PCD is characterized by chronic upper and lower respiratory tract disease, infertility/ectopic pregnancy, and situs anomalies, that occur in ≈50% of PCD patients (Kartagener syndrome), and these may be associated with congenital heart abnormalities...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28646623/pregnancy-outcome-in-women-with-cystic-fibrosis-related-diabetes
#8
Quitterie Reynaud, Stéphanie Poupon-Bourdy, Muriel Rabilloud, Lina Al Mufti, Christine Rousset Jablonski, Lydie Lemonnier, Raphaële Nove-Josserand, Sandrine Touzet, Isabelle Durieu
With increasing life expectancy more women with cystic fibrosis and diabetes mellitus become pregnant. We investigated how pre-gestational diabetes (cystic fibrosis related diabetes) influenced pregnancy outcome and the clinical status of these women MATERIAL AND METHODS: We analyzed all pregnancies reported to the French cystic fibrosis registry between 2001 and 2012, and compared forced expiratory volume (FEV1 ) and body mass index before and after pregnancy in women with and without pre-gestational diabetes having a first delivery RESULTS: A total 249 women delivered 314 infants...
June 24, 2017: Acta Obstetricia et Gynecologica Scandinavica
https://www.readbyqxmd.com/read/28508992/palivizumab-prophylaxis-in-infants-with-cystic-fibrosis-does-not-delay-first-isolation-of-pseudomonas-aeruginosa-or-staphylococcus-aureus
#9
Clélia Buchs, Marie-Laure Dalphin, Stéphane Sanchez, Marie Perceval, Laurianne Coutier, Catherine Mainguy, Behrouz Kassaï-Koupaï, Philippe Reix
Respiratory syncytial virus (RSV) infections may worsen cystic fibrosis (CF) lung disease and favor Pseudomonas aeruginosa (Pa) or Staphylococcus aureus (Sa) acquisition, which is of particular importance in the youngest patients. We aimed to determine the effectiveness of PVZ on microbiological outcomes in young children with CF. We conducted a retrospective case-control study to compare these outcomes in children who systematically received PVZ (PVZ+; n = 40) or not (PVZ-; n = 140). One case was matched with at least three same-gender controls born the same year and month...
May 16, 2017: European Journal of Pediatrics
https://www.readbyqxmd.com/read/28481640/multiple-breath-washout-outcomes-are-sensitive-to-inflammation-and-infection-in-children-with-cystic-fibrosis
#10
Kathryn A Ramsey, Rachel E Foong, Jasmine Grdosic, Alana Harper, Billy Skoric, Charles Clem, Miriam Davis, Lidija Turkovic, Stephen M Stick, Stephanie D Davis, Sarath C Ranganathan, Graham L Hall
RATIONALE: The lung clearance index is a measure of ventilation distribution from the multiple breath washout technique. The lung clearance index is increased in the presence of lower respiratory tract inflammation and infection in infants with cystic fibrosis, however the associations during the preschool years are unknown. OBJECTIVES: We assessed the ability of the lung clearance index to detect the presence and extent of lower respiratory tract inflammation and infection in preschool children with cystic fibrosis...
May 8, 2017: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/28454512/optimization-of-the-french-cystic-fibrosis-newborn-screening-programme-by-a-centralized-tracking-process
#11
Anne Munck, Dominique Delmas, Marie-Pierre Audrézet, Lydie Lemonnier, David Cheillan, Michel Roussey
Objectives To evaluate the French cystic fibrosis newborn screening algorithm, based on data tracked by a centralized monitoring process, from 2002 to 2014. The programme aimed to attain European Standards in terms of positive predictive value, sensitivity, the ratio of screen positive patients diagnosed with cystic fibrosis to infants who screen positive but with inconclusive diagnosis (CFSPID), and time to diagnosis. Methods Retrospective analysis of programme performance, compliance with the algorithm, and changes in screening strategy...
January 1, 2017: Journal of Medical Screening
https://www.readbyqxmd.com/read/28437538/effects-of-diagnosis-by-newborn-screening-for-cystic-fibrosis-on-weight-and-length-in-the-first-year-of-life
#12
MULTICENTER STUDY
Daniel H Leung, Sonya L Heltshe, Drucy Borowitz, Daniel Gelfond, Margaret Kloster, James E Heubi, Michael Stalvey, Bonnie W Ramsey
Importance: Since the implementation of universal newborn screening (NBS) for cystic fibrosis (CF), the timing and magnitude of growth deficiency or its association with correlates of disease among infants with CF who underwent NBS has not been well described. Objective: To examine incremental weight gain, linear growth, and clinical features in the first year of life among infants with CF who underwent NBS. Design, Setting, and Participants: The Baby Observational and Nutrition Study (BONUS), a multicenter, longitudinal, observational cohort study, was conducted during regular CF clinic visits in the first 12 months of life at 28 US Cystic Fibrosis Foundation-accredited Care Centers from January 7, 2012, through May 31, 2015...
June 1, 2017: JAMA Pediatrics
https://www.readbyqxmd.com/read/28423192/systematic-review-of-the-safety-and-efficacy-of-palivizumab-among-infants-and-young-children-with-cystic-fibrosis
#13
Kok Pim Kua, Shaun Wen Huey Lee
BACKGROUND: Respiratory syncytial virus (RSV) is a common pathogen in infants with cystic fibrosis (CF). The use of palivizumab prophylaxis for RSV infection as the standard of care for infants with CF remains controversial. OBJECTIVE: To evaluate the efficacy of palivizumab in reducing the incidence of RSV hospitalization in children with CF who are younger than 2 years. METHODS: Four electronic databases (PubMed, Embase, CINAHL, and CENTRAL) were searched from inception until January 31, 2017, for clinical studies investigating the use of palivizumab in infants with CF aged less than 2 years...
April 19, 2017: Pharmacotherapy
https://www.readbyqxmd.com/read/28420572/nutritional-management-of-cystic-fibrosis-an-update-for-the-21st-century
#14
REVIEW
Sarah Collins
Nutritional management is an essential part of multidisciplinary care for infants, children and adults with cystic fibrosis (CF). In 2016 two updated nutritional consensus guidelines were published [1,2]. This review will explore some of the key points in the nutritional management of people with CF in the 21st Century.
March 14, 2017: Paediatric Respiratory Reviews
https://www.readbyqxmd.com/read/28417451/prophylactic-anti-staphylococcal-antibiotics-for-cystic-fibrosis
#15
REVIEW
Alan R Smyth, Margaret Rosenfeld
BACKGROUND: Staphylococcus aureus causes pulmonary infection in young children with cystic fibrosis. Prophylactic antibiotics are prescribed hoping to prevent such infection and lung damage. Antibiotics have adverse effects and long-term use might lead to infection with Pseudomonas aeruginosa. This is an update of a previously published review. OBJECTIVES: To assess continuous oral antibiotic prophylaxis to prevent the acquisition of Staphylococcus aureus versus no prophylaxis in people with cystic fibrosis, we tested these hypotheses...
April 18, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28356374/concordance-between-upper-and-lower-airway-microbiota-in-infants-with-cystic-fibrosis
#16
Sabine M P J Prevaes, Wouter A A de Steenhuijsen Piters, Karin M de Winter-de Groot, Hettie M Janssens, Gerdien A Tramper-Stranders, Mei Ling J N Chu, Harm A Tiddens, Mireille van Westreenen, Cornelis K van der Ent, Elisabeth A M Sanders, Debby Bogaert
Nasopharyngeal and oropharyngeal samples are commonly used to direct therapy for lower respiratory tract infections in non-expectorating infants with cystic fibrosis (CF).We aimed to investigate the concordance between the bacterial community compositions of 25 sets of nasopharyngeal, oropharyngeal and bronchoalveolar lavage (BAL) samples from 17 infants with CF aged ∼5 months (n=13) and ∼12 months (n=12) using conventional culturing and 16S-rRNA sequencing.Clustering analyses demonstrated that BAL microbiota profiles were in general characterised by a mixture of oral and nasopharyngeal bacteria, including commensals like Streptococcus, Neisseria, Veillonella and Rothia spp...
March 2017: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/28334845/infantile-tremor-syndrome-and-subdural-hemorrhage-in-an-infant-with-cystic-fibrosis
#17
Anirban Mandal, Mayank Priyadarshi, Kanaram Jat, Sushil Kumar Kabra
Cystic fibrosis (CF), an autosomal recessive disease with multi-system involvement, may present with bleeding in infancy owing to vitamin K malabsorption. Infantile tremor syndrome (ITS) is an obscure condition associated with vitamin B12 and other micronutrient deficiencies, described predominantly in Indian subcontinent. We describe an infant presenting with ITS and chronic subdural hemorrhage. He was subsequently diagnosed to have CF. The ITS and subdural hemorrhage is rarely reported in children with CF...
February 23, 2017: Journal of Tropical Pediatrics
https://www.readbyqxmd.com/read/28279431/psychosocial-response-to-uncertain-newborn-screening-results-for-cystic-fibrosis
#18
Robin Z Hayeems, Fiona A Miller, Carolyn J Barg, Yvonne Bombard, June C Carroll, Karen Tam, Elizabeth Kerr, Pranesh Chakraborty, Beth K Potter, Sarah Patton, Jessica P Bytautas, Louise Taylor, Christine Davies, Jennifer Milburn, April Price, Tanja Gonska, Katherine Keenan, Felix Ratjen, Astrid Guttmann
OBJECTIVE: To explore the psychosocial implications of diagnostic uncertainty that result from inconclusive results generated by newborn bloodspot screening (NBS) for cystic fibrosis (CF). STUDY DESIGN: Using a mixed methods prospective cohort study of children who received NBS for CF, we compared psychosocial outcomes of parents whose children who received persistently inconclusive results with those whose children received true positive or screen-negative results...
May 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/28258861/-management-of-infants-whose-diagnosis-is-inconclusive-at-neonatal-screening-for-cystic-fibrosis
#19
I Sermet-Gaudelus, J Brouard, M-P Audrézet, L Couderc Kohen, L Weiss, N Wizla, S Vrielynck, K LLerena, M Le Bourgeois, E Deneuville, N Remus, T Nguyen-Khoa, C Raynal, M Roussey, E Girodon
Neonatal screening for cystic fibrosis (CF) may detect infants with elevated immunoreactive trypsinogen (IRT) levels but with inconclusive sweat tests and/or DNA results. This includes cases associating (1) either the presence of at most one CF-causing mutation and sweat chloride values between 30 and 59mmol/L or (2) two CFTR mutations with at least one of unknown pathogenicity and a sweat chloride below 60mmol/L. This encompasses different clinical situations whose progression cannot be predicted. These cases require redoing the sweat test at 12 months and if possible at 6 and 24 months of life...
March 1, 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/28129812/cystic-fibrosis-transmembrane-conductance-regulator-related-metabolic-syndrome-and-cystic-fibrosis-screen-positive-inconclusive-diagnosis
#20
Clement L Ren, Drucy S Borowitz, Tanja Gonska, Michelle S Howenstine, Hara Levy, John Massie, Carlos Milla, Anne Munck, Kevin W Southern
OBJECTIVE: An unintended consequence of cystic fibrosis (CF) newborn screening (NBS) is the identification of infants with a positive NBS test but inconclusive diagnostic testing. These infants are classified as CF transmembrane conductance regulator-related metabolic syndrome (CRMS) in the US and CF screen positive, inconclusive diagnosis (CFSPID) in other countries. Diagnostic and management decisions of these infants are challenges for CF healthcare professionals and stressful situations for families...
February 2017: Journal of Pediatrics
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