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cystic fibrosis children

Joseph L Kuti, Rebecca S Pettit, Natalie Neu, Jeffrey J Cies, Craig Lapin, Marianne S Muhlebach, Kimberly J Novak, Sean T Nguyen, Lisa Saiman, David P Nicolau
Meropenem exposures from 15 children (8-17 years old) with cystic fibrosis (CF) acute pulmonary exacerbation were analyzed to define the pharmacodynamic threshold required for a positive response. The primary endpoint was the relative increase in forced expiratory volume in 1 s (↑FEV1 ) between pre- and posttreatment. Meropenem pharmacodynamic indices (fT > MIC, fAUC/MIC, fCmin /MIC) over the first 24 h were estimated for each participant based on their individual parameter estimates and the isolated pathogen with the highest meropenem MIC...
February 24, 2018: Diagnostic Microbiology and Infectious Disease
Catherine Giguere-Rich, Amy Mathew, Elizabeth Reid, Kimberly Autore, Margaret F Guill
This clinical observation describes the enteral nutrition (EN) management of 2 toddlers at high nutrition risk due to cystic fibrosis (CF), exocrine pancreatic insufficiency, and comorbid medical conditions. The first case report describes a boy with severe malabsorption after intestinal resection. The second case report reviews a boy with CF and neuroblastoma. When pancreatic enzyme replacement therapy with EN was not effective or appropriate, use of an in-line digestive cartridge was initiated. While using the digestive cartridge, both children showed improvements in their anthropometric measures...
April 2018: Nutrition in Clinical Practice
Hannah T Gibson, Catherine M McDonald, Jennifer Willahan Derrick, Dennis L Eggett, Sarah Gunnell Bellini
BACKGROUND: Body mass index (BMI) is used to determine nutrition status in children with cystic fibrosis (CF); however, lean body mass (LBM) is more strongly associated with pulmonary function. Handgrip strength (HGS) measures muscle function and is reflective of LBM. The aims of this study were to assess relationships among HGS, nutrition status, and pulmonary function; changes in HGS posthospitalization; and any relationship between HGS and nutrient intake. METHODS: Twenty-three children with CF aged 6-18 years participated...
April 2018: Nutrition in Clinical Practice
Fiona Moola
While cross-respiratory infection guidelines reduce the risk of infection, this may have deleterious impacts on the socialization skills of young people living with cystic fibrosis (CF). The purpose of this study was to explore the experience of social isolation among young Canadians with CF, as well as how they navigate these complex contamination restrictions. The thematic analytic qualitative research tradition was adopted and social theories of risk provided theoretical grounding. The youth described a pressing sense of social isolation in their everyday lives...
January 1, 2018: Journal of Child Health Care: for Professionals Working with Children in the Hospital and Community
Cibelle Andrade Lima, Armèle Dornelas de Andrade, Shirley Lima Campos, Daniella Cunha Brandão, Ianny Pereira Mourato, Murilo Carlos Amorim de Britto
Cystic Fibrosis (CF) is a multisystem disorder. The involvement of the respiratory system is frequent and culminates in dyspnea and exercise intolerance. Functional capacity is an important diagnostic tool, because it reflects the cardiorespiratory status, quality of life and prognosis. This systematic review aims to assess the reproducibility and validity of the six minute walk test (6MWT) to reflect the functional capacity of children and adolescents with cystic fibrosis, and also the correlation between 6MWT and lung function...
April 2018: Respiratory Medicine
Ozlem Satırer, Ayse Mete Yesil, Nagehan Emiralioglu, Gökcen Dilsa Tugcu, Ebru Yalcın, Deniz Dogru, Nural Kiper, Ugur Ozcelik
INTRODUCTION: Non-cystic fibrosis(CF) bronchiectasis has been recognized in children for the past 200 years. Early childhood pneumonia and underlying conditions such as immunodeficiency, primary ciliary dyskinesia(PCD), and congenital lung pathology should be considered in the etiology. The aim of our study was to describe the clinical characteristics, laboratory, and radiological findings of a large population of patients with non-CF bronchiectasis at a tertiary center. METHODS: We analyzed the clinical findings of 187 patients diagnosed with non-CF bronchiectasis over a period of 10 years (January 2005-December 2015) at the Hacettepe University Faculty of Medicine Department of Pediatric Pulmonology...
April 2018: Respiratory Medicine
Angela Gentile, Maria Florencia Lucion, Maria Del Valle Juarez, Ana Clara Martinez, Viviana Romanin, Julia Bakir, Mariana Viegas, Alicia Mistchenko
BACKGROUND: Influenza is an important cause of acute lower respiratory tract infection (aLRTI), hospitalization, and mortality in children. This study aimed to describe the clinical and epidemiologic patterns and infection factors associated with influenza, and compare case features of influenza A and B. METHODS: In a prospective, cross-sectional study, patients admitted for aLRTI, between 2000 and 2015, were tested for respiratory syncytial virus, adenovirus, influenza, or parainfluenza, and confirmed by fluorescent antibody (FA) or real-time polymerase chain reaction (RT-PCR) assay of nasopharyngeal aspirates...
2018: PloS One
A Grangeia, S Alves, L Gonçalves, I Gregório, A C Santos, H Barros, A Barros, F Carvalho, C Moura
In Portugal, the spectrum of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene variants is not known. The main objective of this work was to determine the type and frequency of CFTR variants in a sample from northern Portugal by the complete analysis of the CFTR coding sequencing performed in 512 Portuguese children. A total of 30 different CFTR sequence variants, already reported as cystic fibrosis (CF) or CFTR related disorders variants, were detected. Ninety-two children (18.0%; 95%CI: 14.7-21...
January 2018: Pulmonology
M Scalbert-Dujardin, A Boldron, E Leroy, J Bazin, E Froment-Leclercq
INTRODUCTION: The main aim of this study is to evaluate the effectiveness of preventive actions regarding influenza in the studied populations. The secondary objective is to analyze and understand the mechanisms which bring about a behavioural change regarding influenza vaccination. METHODS: The interventional and prospective study was undertaken in the form of an anonymous questionnaire about influenza vaccination coverage and about the reasons for vaccinating or not vaccinating...
March 24, 2018: Revue des Maladies Respiratoires
Sally Spencer, Lambert M Felix, Stephen J Milan, Rebecca Normansell, Pieter C Goeminne, James D Chalmers, Tim Donovan
BACKGROUND: Bronchiectasis is a chronic inflammatory disease characterised by a recurrent cycle of respiratory bacterial infections associated with cough, sputum production and impaired quality of life. Antibiotics are the main therapeutic option for managing bronchiectasis exacerbations. Evidence suggests that inhaled antibiotics may be associated with more effective eradication of infective organisms and a lower risk of developing antibiotic resistance when compared with orally administered antibiotics...
March 27, 2018: Cochrane Database of Systematic Reviews
Stephen J McWilliam, Daniel J Antoine, Andrea L Jorgensen, Rosalind L Smyth, Munir Pirmohamed
Aminoglycosides are commonly used for the treatment of pulmonary exacerbations in patients with cystic fibrosis (CF). However, they are potentially nephrotoxic. This prospective observational cohort study aimed to investigate the potential validity of two urinary renal biomarkers, Kidney Injury Molecule-1 (KIM-1) and Neutrophil Gelatinase-associated Lipocalin (NGAL), in identifying aminoglycoside-induced nephrotoxicity in children with CF. Children and young adults up to 20 years of age with a confirmed diagnosis of CF were recruited from ten United Kingdom hospitals...
March 23, 2018: Scientific Reports
Patricia Leutz-Schmidt, Mirjam Stahl, Olaf Sommerburg, Monika Eichinger, Michael U Puderbach, Jens-Peter Schenk, Abdulsattar Alrajab, Simon M F Triphan, Hans-Ulrich Kauczor, Marcus A Mall, Mark O Wielpütz
OBJECTIVES: To determine if morphological non-contrast enhanced magnetic resonance imaging (MRI) of the lung is sensitive to detect mosaic signal intensity in infants and preschool children with cystic fibrosis (CF). MATERIALS AND METHODS: 50 infant and preschool CF patients (mean age 3.5 ± 1.4y, range 0-6y) routinely underwent morphological (T2-weighted turbo-spin echo sequence with half-Fourier acquisition, HASTE) and contrast-enhanced 4D perfusion MRI (gradient echo sequence with parallel imaging and echo sharing, TWIST)...
April 2018: European Journal of Radiology
Peter A Muller, Jennifer L Mueller, Michelle Mellenthin, Rashmi Murthy, Michael Capps, Brandie Wagner, Melody Alsaker, Robin Deterding, Scott D Sagel, Jordana Hoppe
Lung function monitoring by spiromety plays a critical role in the clinical care of pediatric cystic fibrosis (CF) patients, but many young children are unable to perform spirometry, and the outputs are often normal even in the presence of lung disease. Measures derived from electrical impedance tomography (EIT) images were studied for their utility as potential surrogates for spirometry in CF patients and to assess response to intravenous antibiotic treatment for acute pulmonary exacerbations (PEx) in a subset of patients...
March 22, 2018: Physiological Measurement
Paola Mosconi, Cinzia Colombo, Anna Roberto, Giulia Candiani, Maria Teresa Greco, Roberto Satolli, Carlo Castellani
Background: Health technology assessment and ethical issues have to be dealt with in deciding on national carrier screening for cystic fibrosis (CF)-the most frequent severe autosomal recessive disease in Caucasian populations and several stakeholders need to be involved. A citizens' jury is one way to ask citizens to deliberate on controversial topics in the interests of a society. The aims of this project were to gather opinions about CF carrier screening through citizens' jury deliberations and to match them with the findings of a large online consultation survey open to the general population, people with CF and families and health professionals...
March 19, 2018: European Journal of Public Health
J Manji, A Thamboo, M Tacey, C Garnis, N K Chadha
BACKGROUND: Sinonasal disease is a common feature of cystic fibrosis (CF) and can cause significant morbidity in these patients. Our objective was to determine if CF individuals with concomitant nasal polyposis (NP) express a unique profile of inflammation and if so, whether these inflammatory cytokine mediators have predictive value in identifying these individuals for prompt management by an Otolaryngologist. METHODOLOGY: Nasal lavage samples and clinical outcomes of disease severity were obtained from thirty-eight pediatric CF individuals...
March 21, 2018: Rhinology
K Schneider
The radiographic technique of pediatric chest X‑rays is substantially different from that in adults. In nearly all cases ap/pa X‑rays are sufficient and lateral radiographs are rarely needed. In the first years of life the thymus may overshadow the heart, the great vessels and the lung hila. The most important anatomical structures essential for diagnosing pathological findings of the lungs and mediastinum are the trachea with the bifurcation and the main bronchi with the adjacent great vessels. For the assessment of distended lungs and intrathoracic consolidations, fundamental knowledge of the anatomy in childhood and malformations which can involve the airways, the lungs, the heart, as well as systemic and pulmonary vessels are indispensable...
March 19, 2018: Der Radiologe
Ruchita Dixit, Sowmya Nettem, Simerjit S Madan, Htoo Htoo Kyaw Soe, Adinegara Bl Abas, Leah D Vance, Patrick J Stover
BACKGROUND: Sickle cell disease (SCD) is a group of disorders that affects haemoglobin, which causes distorted sickle- or crescent-shaped red blood cells. It is characterized by anaemia, increased susceptibility to infections and episodes of pain. The disease is acquired by inheriting abnormal genes from both parents, the combination giving rise to different forms of the disease. Due to increased erythropoiesis in people with SCD, it is hypothesized that they are at an increased risk for folate deficiency...
March 16, 2018: Cochrane Database of Systematic Reviews
Carol Kelly, James D Chalmers, Iain Crossingham, Nicola Relph, Lambert M Felix, David J Evans, Stephen J Milan, Sally Spencer
BACKGROUND: Bronchiectasis is a chronic respiratory disease characterised by abnormal and irreversible dilatation and distortion of the smaller airways. Bacterial colonisation of the damaged airways leads to chronic cough and sputum production, often with breathlessness and further structural damage to the airways. Long-term macrolide antibiotic therapy may suppress bacterial infection and reduce inflammation, leading to fewer exacerbations, fewer symptoms, improved lung function, and improved quality of life...
March 15, 2018: Cochrane Database of Systematic Reviews
Norita Hussein, Stephen F Weng, Joe Kai, Jos Kleijnen, Nadeem Qureshi
BACKGROUND: Globally, about five per cent of children are born with congenital or genetic disorders. The most common autosomal recessive conditions are thalassaemia, sickle cell disease, cystic fibrosis and Tay-Sachs disease, with higher carrier rates in specific patient populations. Identifying and counselling couples at genetic risk of the conditions before pregnancy enables them to make fully informed reproductive decisions, with some of these choices not being available if genetic counselling is only offered in an antenatal setting...
March 14, 2018: Cochrane Database of Systematic Reviews
Julia Gray Farber, Mary G Prieur, Christine Roach, Rosemary Shay, Michelle Walter, Drucy Borowitz, Elisabeth P Dellon
Background Despite the chronic, progressive, and life-threatening nature of cystic fibrosis (CF), there are no guidelines for when and how to communicate prognosis to children with CF. METHODS: Semi-structured interviews with young adults with CF, parents of young adults with CF, and multidisciplinary CF health care providers assessed recall of and practices for communicating about prognosis. Recommendations for improvements were also solicited. RESULTS: Young adults with CF recalled learning that life expectancy is limited by CF between the ages of 8 and 16 years, and that CF is a progressive disease between the ages of 7 and 19 years...
March 12, 2018: Pediatric Pulmonology
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