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Cystic fibrosis in infants

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https://www.readbyqxmd.com/read/27889352/oxidized-glutathione-and-uric-acid-as-biomarkers-of-early-cystic-fibrosis-lung-disease
#1
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
#2
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
#3
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
#4
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
#5
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...
October 25, 2016: 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
#6
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 years of age from the Canadian healthcare payer's perspective. Methods In 2014, a Markov cohort model of CF disease and infant RSV infections in the Canadian setting was developed based on literature data...
October 21, 2016: Human Vaccines & Immunotherapeutics
https://www.readbyqxmd.com/read/27768016/distal-intestinal-obstruction-syndrome-a-rare-complication-of-cystic-fibrosis-in-infants
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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
https://www.readbyqxmd.com/read/27510673/severe-liver-dysfunction-in-an-infant-with-cystic-fibrosis-masquerading-as-metabolic-liver-disease
#15
K P Srikanth, Inusha Panigrahi, Babu Ram Thapa, Kim Vaiphei
We present a rare presentation of cystic fibrosis with neonatal cholestasis. Histological features of mucoviscidosis were present in liver involving the biliary tract, intestinal mucosa, pancreas, and lung. Besides, there was a rare association with autosomal dominant type of polycystic renal disease.
July 2016: Indian Journal of Pathology & Microbiology
https://www.readbyqxmd.com/read/27496146/early-severe-anemia-as-the-first-sign-of-cystic-fibrosis
#16
Tugba Sismanlar, Ayşe Tana Aslan, Mehmet Köse, Sevgi Pekcan, Fatih Süheyl Ezgü, Işıl İrem Budakoğlu, İdil Yenicesu
UNLABELLED: Severe anemia is reported to occur rarely in patients with cystic fibrosis (CF). This study aimed to determine the factors associated with early severe anemia in infants with CF. This study included 231 infants with CF from 3 pediatric CF centers ten year period that were retrospectively reviewed in terms of severe anemia as the first sign of CF. Factors that could affect anemia, such as age, pancreatic insufficiency, mutations, vitamin A and E, and albumin level were evaluated...
September 2016: European Journal of Pediatrics
https://www.readbyqxmd.com/read/27493195/novel-methodology-to-perform-sulfur-hexafluoride-sf6-based-multiple-breath-washin-and-washout-in-infants-using-current-commercially-available-equipment
#17
Per Magnus Gustafsson, Paul David Robinson, Anders Lindblad, Daniel Oberli
Multiple breath inert gas washout (MBW) is ideally suited for early detection and monitoring of serious lung disease, such as cystic fibrosis, in infants and young children. Validated commercial options for the MBW technique are limited, and suitability of Nitrogen (N2) based MBW is of concern given the detrimental effect of exposure to pure O2 on infant breathing pattern. We propose novel methodology using commercially available N2 MBW equipment to facilitate 4% sulfur hexafluoride (SF6) multiple breath inert gas washin and washout suitable for the infant age range...
August 4, 2016: Journal of Applied Physiology
https://www.readbyqxmd.com/read/27485696/parent-experience-with-false-positive-newborn-screening-results-for-cystic-fibrosis
#18
Robin Z Hayeems, Fiona A Miller, Carolyn J Barg, Yvonne Bombard, Elizabeth Kerr, Karen Tam, June C Carroll, Beth K Potter, Pranesh Chakraborty, Christine Davies, Jennifer Milburn, Sarah Patton, Jessica P Bytautas, Louise Taylor, April Price, Tanja Gonska, Katherine Keenan, Felix Ratjen, Astrid Guttmann
BACKGROUND: The risk of psychosocial harm in families of infants with false-positive (FP) newborn bloodspot screening (NBS) results for cystic fibrosis (CF) is a longstanding concern. Whether well designed retrieval and confirmatory testing systems can mitigate risks remains unknown. METHODS: Using a mixed-methods cohort design, we obtained prospective self-report data from mothers of infants with FP CF NBS results 2 to 3 months after confirmatory testing at Ontario's largest follow-up center, and from a randomly selected control sample of mothers of screen negative infants from the same region...
September 2016: Pediatrics
https://www.readbyqxmd.com/read/27469178/cystic-fibrosis-diagnosis-and-newborn-screening
#19
REVIEW
Margaret Rosenfeld, Marci K Sontag, Clement L Ren
The diagnosis of cystic fibrosis (CF) has evolved over the past decade as newborn screening has become universal in the United States and elsewhere. The heterogeneity of phenotypes associated with CF transmembrane conductance regulator (CFTR) dysfunction and mutations in the CFTR gene has become clearer, ranging from classic pancreatic-insufficient CF to manifestations in only 1 organ system to indeterminate diagnoses identified by newborn screening. The tools available for diagnosis have also expanded. This article reviews the newest diagnostic criteria for CF, newborn screening, prenatal screening and diagnosis, and indeterminate diagnoses in newborn-screened infants and symptomatic adults...
August 2016: Pediatric Clinics of North America
https://www.readbyqxmd.com/read/27447840/abnormal-glucose-tolerance-in-infants-and-young-children-with-cystic-fibrosis
#20
Yaling Yi, Andrew W Norris, Kai Wang, Xingshen Sun, Aliye Uc, Antoinette Moran, John F Engelhardt, Katie Larson Ode
RATIONALE: In cystic fibrosis, abnormal glucose tolerance is associated with decreased lung function and worsened outcomes. Translational evidence indicates that abnormal glucose tolerance may begin in early life. OBJECTIVES: To determine whether very young children with cystic fibrosis have increased abnormal glucose tolerance prevalence compared with control subjects. The secondary objective was to compare area under the curve for glucose and insulin in children with cystic fibrosis with control subjects...
October 15, 2016: American Journal of Respiratory and Critical Care Medicine
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