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Cystic fibrosis in infants

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https://www.readbyqxmd.com/read/28508992/palivizumab-prophylaxis-in-infants-with-cystic-fibrosis-does-not-delay-first-isolation-of-pseudomonas-aeruginosa-or-staphylococcus-aureus
#1
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
#2
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
#3
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
#4
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...
April 24, 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
#5
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 of age. 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
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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
https://www.readbyqxmd.com/read/28116474/prenatal-magnetic-resonance-and-ultrasonographic-findings-in-small-bowel-obstruction-imaging-clues-and-postnatal-outcomes
#13
Eva I Rubio, Anna R Blask, Andrea T Badillo, Dorothy I Bulas
BACKGROUND: Prenatal small-bowel obstruction can result from single or multiple atresias, and it can be an isolated abnormality or part of a syndrome. It is sometimes the first manifestation of cystic fibrosis. Accurate prediction of the level of obstruction and length of bowel affected can be difficult, presenting a challenge for counseling families and planning perinatal management. OBJECTIVE: To review the prenatal US and MRI findings of small-bowel obstruction and to assess whether fetal MRI adds information that could improve prenatal counseling and perinatal management...
January 23, 2017: Pediatric Radiology
https://www.readbyqxmd.com/read/28079575/cystic-biliary-atresia-and-choledochal-cysts-are-distinct-histopathologic-entities
#14
Inna N Lobeck, Rachel Sheridan, Mark Lovell, Phylicia Dupree, Greg M Tiao, Kevin E Bove
Cystic biliary atresia (CBA), a rare cystic expansion of atretic extrahepatic bile ducts in young infants, overlaps in age at presentation and imaging features with early choledochal cysts (CC). Treatment and prognosis differ; histologic differences are unsettled. We compared 10 patients with CBA, 1975 to 2015, to an age-similar cohort of 13 infants, and to older patients who had surgery for CC. Operative details, imaging, and clinical courses were correlated to pathologic specimens. Immunostains for smooth muscle actin and myosin heavy chain were used to evaluate cyst walls and atretic segments...
March 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28005688/respiratory-syncytial-virus-infection-associated-hospitalization-rates-in-infants-and-children-with-cystic-fibrosis
#15
Jakob Metz, Ernst Eber, Bernhard Resch
BACKGROUND: Infections with respiratory syncytial virus (RSV) are the leading cause for hospital admissions in infants and young children. The incidence of RSV-related hospitalizations in patients with cystic fibrosis (CF) is unclear. To date, no effective treatment for RSV infections is available. Thus, prophylaxis with the monoclonal antibody palivizumab is an important option. METHODS: In a retrospective, single-center study at the Department of Pediatrics and Adolescent Medicine of the Medical University Graz, Austria, we analyzed all CF patients born between 1995 and 2012, who were admitted for respiratory problems between 1995 and 2014...
June 2017: Pediatric Infectious Disease Journal
https://www.readbyqxmd.com/read/27934649/safety-and-effectiveness-of-arginine-in-adults
#16
REVIEW
Catherine J McNeal, Cynthia J Meininger, Deepika Reddy, Colin D Wilborn, Guoyao Wu
l-Arginine (Arg) appears to have a beneficial effect on the regulation of nutrient metabolism to enhance lean tissue deposition and on insulin resistance in humans. The observed safe level for oral administration of Arg is ∼20 g/d, but higher levels have been tested in short-term studies without serious adverse effects; however, more data are needed in both animal models and humans to fully evaluate safety as well as efficacy. The primary objective of this review is to summarize the current knowledge of the safety, pharmacokinetics, and effectiveness of oral Arg in adults...
December 2016: Journal of Nutrition
https://www.readbyqxmd.com/read/27930727/airway-microbiota-in-bronchoalveolar-lavage-fluid-from-clinically-well-infants-with-cystic-fibrosis
#17
Theresa A Laguna, Brandie D Wagner, Cynthia B Williams, Mark J Stevens, Charles E Robertson, Cole W Welchlin, Catherine E Moen, Edith T Zemanick, Jonathan K Harris
BACKGROUND: Upper airway cultures guide the identification and treatment of lung pathogens in infants with cystic fibrosis (CF); however, this may not fully reflect the spectrum of bacteria present in the lower airway. Our objectives were to characterize the airway microbiota using bronchoalveolar lavage fluid (BALF) from asymptomatic CF infants during the first year of life and to investigate the relationship between BALF microbiota, standard culture and clinical characteristics. METHODS: BALF, nasopharyngeal (NP) culture and infant pulmonary function testing data were collected at 6 months and one year of age during periods of clinical stability from infants diagnosed with CF by newborn screening...
2016: PloS One
https://www.readbyqxmd.com/read/27927050/the-use-of-multiple-breath-washout-for-assessing-cystic-fibrosis-in-infants
#18
Gwyneth Davies, Paul Aurora
Lung Clearance Index, measured using the multiple breath washout (MBW) technique, may be a useful test in infants with Cystic Fibrosis (CF). However, the requirement for specialised equipment and a number of important technical and methodological considerations relevant to testing in infants have complicated matters, and to date prevented its widespread translatability in this age group. Areas covered: We review the current status of infant MBW testing in CF, focusing on Lung Clearance Index. This includes a review of recent developments in the field relevant to testing methodology in the infant population, use in evaluating lung disease in CF in infancy, and the associated challenges which remain...
January 2017: Expert Review of Respiratory Medicine
https://www.readbyqxmd.com/read/27911585/early-lung-disease-in-infants-and-pre-school-children-with-cystic-fibrosis-what-have-we-learnt-and-what-should-we-do-about-it
#19
Sarath C Ranganathan, Graham L Hall, Peter D Sly, Stephen M Stick, Tonia A Douglas
The past decade has seen significant advances in understanding of the pathogenesis and progression of lung disease in cystic fibrosis. Pulmonary inflammation, infection and structural lung damage manifest very early in life and is prevalent among preschool children and infants, often in the absence of symptoms or signs. Early childhood represents a pivotal period amenable to intervention strategies that could delay or prevent the onset of lung damage and alter the longer term clinical trajectory for individuals with CF...
December 2, 2016: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/27889352/oxidized-glutathione-and-uric-acid-as-biomarkers-of-early-cystic-fibrosis-lung-disease
#20
Nina Dickerhof, Rufus Turner, Irada Khalilova, Emmanuelle Fantino, Peter D Sly, Anthony J Kettle
BACKGROUND: In cystic fibrosis (CF) there is an urgent need for earlier diagnosis of pulmonary infections and inflammation using blood- and urine-based biomarkers. METHODS: Using mass spectrometry, oxidation products of glutathione and uric acid were measured in matched samples of bronchoalveolar lavage (BAL), serum and urine from 36 infants and children with CF, and related to markers of neutrophilic inflammation and infection in BAL. RESULTS: Oxidation products of glutathione (glutathione sulfonamide, GSA) and uric acid (allantoin), were elevated in BAL of children with pulmonary infections with Pseudomonas aeruginosa (PsA) compared to those without (p<0...
March 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
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