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Cystic fibrosis in infants

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https://www.readbyqxmd.com/read/28079575/cystic-biliary-atresia-and-choledochal-cysts-are-distinct-histopathologic-entities
#1
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...
January 10, 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
#2
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 Paediatrics 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...
December 21, 2016: Pediatric Infectious Disease Journal
https://www.readbyqxmd.com/read/27934649/safety-and-effectiveness-of-arginine-in-adults
#3
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
#4
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
#5
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
#6
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
#7
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...
November 24, 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/27888005/a-simplified-semi-quantitative-structural-lung-disease-computed-tomography-outcome-during-quiet-breathing-in-infants-with-cystic-fibrosis
#8
Magali Saguintaah, Yann Cabon, Rémi Gauthier, Cécile Duboibaudry, Laure Couderc, Muriel Le Bourgeois, Philippe Reix, Raphael Chiron, Ikram Taleb Arrada, Nicolas Molinari, Stefan Matecki
Chest tomography (CT) using the controlled ventilation technique (CTCV) is a sensitive method to detect features of lung cystic fibrosis (CF) disease in infants with CF. However, this technique needs sedation and is not easily applied for the clinician who may need, in the follow-up, to evaluate more precisely lung disease in infants with CF. Thus, our study aims to evaluate if CT assessment of lung disease, without the need of sedation, during quiet breathing, using a semi-quantitative scoring system, is reproducible and may discriminate infants with CF from control infants at an early stage of the lung disease...
November 22, 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/27856730/multidrug-and-carbapenem-resistant-pseudomonas-aeruginosa-in-children-united-states-1999-2012
#9
Latania K Logan, Sumanth Gandra, Siddhartha Mandal, Eili Y Klein, Jordan Levinson, Robert A Weinstein, Ramanan Laxminarayan
BACKGROUND: Pseudomonas aeruginosa is a common cause of healthcare-associated infection. Multidrug-resistant (MDR) (>3 classes) and carbapenem-resistant (CR) P aeruginosa are significant threats globally. We used a large reference-laboratory database to study the epidemiology of P aeruginosa in children in the United States. METHODS: Antimicrobial susceptibility data from the Surveillance Network were used to phenotypically identify MDR and CR P aeruginosa isolates in children aged 1 to 17 years between January 1999 and July 2012...
November 16, 2016: Journal of the Pediatric Infectious Diseases Society
https://www.readbyqxmd.com/read/27806795/-infantile-cholestasis-caused-by-cftr-mutation-case-report-and-literature-review
#10
L Li, N L Wang, J Y Gong, J S Wang
Objective: To study the clinical presentation, biochemical features and genetic analysis of an infant with cholestasis related to the CFTR mutations. Method: The clinical presentation, laboratory investigations and management of a case with infantile cholestasis caused by CFTR mutations were summarized and the relevant literature was reviewed. Result: (1) The patient was a 5 months old boy with cholestasis which developed in neonatal period with delayed meconium exclusion.The laparoscopic exploration was performed to exclude biliary atresia because of acholic stool when he was two months old...
November 2, 2016: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/27793338/cystic-fibrosis-is-associated-with-adverse-neonatal-outcomes-in-washington-state-1996-2013
#11
Kathleen J Ramos, Coralynn S Sack, Kristina H Mitchell, Christopher H Goss, Jacqueline R Starr
OBJECTIVE: To determine whether cystic fibrosis (CF) is associated with adverse neonatal outcomes in a recent birth cohort in the US. STUDY DESIGN: A retrospective matched cohort study of infants born in Washington State from 1996 to 2013 was identified through birth certificate data and linked to statewide hospital discharge data. Infants with CF were identified by hospitalization (through age 5 years) in which a CF-specific International Classification of Diseases, Ninth Revision code was recorded...
January 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/27768505/the-cost-effectiveness-of-palivizumab-in-infants-with-cystic-fibrosis-in-the-canadian-setting-a-decision-analysis-model
#12
Ashleigh A McGirr, Kevin L Schwartz, Upton Allen, Melinda Solomon, Beate Sander
BACKGROUND: Children with cystic fibrosis (CF) are at higher risk of severe respiratory syncytial virus (RSV) infection, which can lead to a decline in lung function. A monoclonal antibody, palivizumab (PMB), effectively prevents RSV hospitalizations; however, the high cost of PMB, approximately C$10,000 per patient per RSV season, limits its widespread use. We assess the cost-effectiveness of PMB prophylaxis in CF children less than 2 y of age from the Canadian healthcare payer's perspective...
October 21, 2016: Human Vaccines & Immunotherapeutics
https://www.readbyqxmd.com/read/27768016/distal-intestinal-obstruction-syndrome-a-rare-complication-of-cystic-fibrosis-in-infants
#13
Dana T Anton-Pǎduraru, Amalia M Bontea, Ioana Cernescu, Smaranda Diaconescu
No abstract text is available yet for this article.
December 2016: Minerva Pediatrica
https://www.readbyqxmd.com/read/27662104/early-detection-and-sensitive-monitoring-of-cf-lung-disease-prospects-of-improved-and-safer-imaging
#14
REVIEW
Marcus A Mall, Mirjam Stahl, Simon Y Graeber, Olaf Sommerburg, Hans-Ulrich Kauczor, Mark O Wielpütz
Recent imaging studies using chest computed tomography (CT) in presymptomatic infants and young children with cystic fibrosis (CF) diagnosed by newborn screening presented compelling evidence of early onset and progression of structural lung damage in CF. These data argue persuasively that non-invasive outcome measures for early detection and sensitive monitoring of lung disease applicable in the clinical setting will be instrumental for further improvement of clinical care and the development of early intervention therapies that have the potential to prevent irreversible lung damage...
October 2016: Pediatric Pulmonology
https://www.readbyqxmd.com/read/27662103/novel-insights-into-the-diagnostic-and-therapeutic-challenges-of-the-cftr-metabolic-syndrome-cf-screen-positive-indeterminate-diagnosis
#15
Evans Machogu, Clement L Ren
The growth of cystic fibrosis newborn screening (CF NBS) has led to an increased number of infants with a positive NBS test but inconclusive CF diagnostic testing. In the USA this condition is called CFTR related metabolic syndrome (CRMS), while in Europe the term CF screen positive, inconclusive diagnosis (CFSPID) is used. Recent advances in CF genetics and epidemiologic studies of CRMS/CFSPID have provided new insights into the prevalence and outcomes associated with this condition. Pediatr Pulmonol. 2016;51:S45-S48...
October 2016: Pediatric Pulmonology
https://www.readbyqxmd.com/read/27608173/a-secondary-benefit-the-reproductive-impact-of-carrier-results-from-newborn-screening-for-cystic-fibrosis
#16
Yvonne Bombard, Fiona A Miller, Carolyn J Barg, Sarah J Patton, June C Carroll, Pranesh Chakraborty, Beth K Potter, Karen Tam, Louise Taylor, Elizabeth Kerr, Christine Davies, Jennifer Milburn, Felix Ratjen, Astrid Guttmann, Robin Z Hayeems
PURPOSE: Newborn screening (NBS) for cystic fibrosis (CF) can identify carriers, which is considered a benefit that enables reproductive planning. We examined the reproductive impact of carrier result disclosure of NBS for CF. METHODS: We surveyed mothers of carrier infants after NBS (Time 1) and 1 year later (Time 2) to ascertain intended and reported communication of their infants' carrier results to relatives, carrier testing for themselves/other children, and reproductive decisions...
September 8, 2016: Genetics in Medicine: Official Journal of the American College of Medical Genetics
https://www.readbyqxmd.com/read/27583671/cystic-fibrosis-screen-positive-inconclusive-diagnosis
#17
Jürg Barben, Kevin W Southern
PURPOSE OF REVIEW: A challenging sequelae of newborn bloodspot screening (NBS) for cystic fibrosis (CF) has been the identification of infants with an unclear diagnosis after a positive NBS result, which leads to uncertainty for healthcare professionals and families. This review describes the classification, frequency, clinical outcome and early management of these infants. RECENT FINDINGS: In the US, infants with an inconclusive diagnosis after NBS are labelled 'CF transmembrane conductance regulator (CFTR)-related metabolic syndrome' (CRMS), and in Europe 'CF screen positive, inconclusive diagnosis' (CFSPID)...
November 2016: Current Opinion in Pulmonary Medicine
https://www.readbyqxmd.com/read/27575911/comparison-of-lung-clearance-index-and-magnetic-resonance-imaging-for-assessment-of-lung-disease-in-children-with-cystic-fibrosis
#18
Mirjam Stahl, Mark O Wielpütz, Simon Y Graeber, Cornelia Joachim, Olaf Sommerburg, Hans-Ulrich Kauczor, Michael Puderbach, Monika Eichinger, Marcus A Mall
RATIONALE: Early onset and progression of lung disease in children with cystic fibrosis (CF) indicates that sensitive noninvasive outcome measures are needed for diagnostic monitoring and early intervention clinical trials. The lung clearance index (LCI) and chest magnetic resonance imaging (MRI) were shown to detect early lung disease in CF, however, the relationship between the two measures remains unknown. OBJECTIVES: To correlate the LCI with abnormalities detected by MRI and compare the sensitivity of the two techniques to detect responses to therapy for pulmonary exacerbations in children with CF...
August 30, 2016: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/27574881/combined-multichannel-intraluminal-impedance-and-ph-measurement-in-detecting-gastroesophageal-reflux-disease-in-children
#19
Mark Safe, Jemma Cho, Usha Krishnan
OBJECTIVE: The aim of the study was to evaluate and compare multichannel intraluminal impedance-pH (MII-pH) monitoring with standard investigations including pH testing for detecting gastroesophageal reflux disease (GERD) in children. METHODS: A retrospective review of all MII-pH studies performed between July 2007 and March 2013 at Sydney Children's Hospital. Results from MII-pH testing, esophagogastroduodenoscopy (EGD), barium meal and pepsin assay, symptoms, underlying comorbidities, age, and medication usage were evaluated...
November 2016: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/27556254/risk-stratification-model-to-detect-early-pulmonary-disease-in-infants-with-cystic-fibrosis-diagnosed-by-newborn-screening
#20
Lacrecia J Britton, Gabriela R Oates, Robert A Oster, Staci T Self, Robert B Troxler, Wynton C Hoover, Hector H Gutierrez, William T Harris
OBJECTIVE: The clinical benefit of newborn screening (NBS) for cystic fibrosis (CF) has been primarily nutritional, with less overt respiratory impact. Identification of risk factors for infant CF lung disease could facilitate targeted interventions to improve pulmonary outcomes. METHODS: This retrospective study evaluated socioeconomic information, clinical data, and results from routine infant pulmonary function testing (iPFT) of infants diagnosed with CF through NBS (N = 43) at a single CF center over a 4-year period (2008-2012)...
November 2016: Pediatric Pulmonology
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