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Cystic fibrosis MRI

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https://www.readbyqxmd.com/read/28132845/use-of-hyperpolarized-helium-3-mri-to-assess-response-to-ivacaftor-treatment-in-patients-with-cystic-fibrosis
#1
Talissa A Altes, Mac Johnson, Meredith Fidler, Martyn Botfield, Nicholas J Tustison, Carlos Leiva-Salinas, Eduard E de Lange, Deborah Froh, John P Mugler
BACKGROUND: This pilot study evaluated the effect of short- and long-term ivacaftor treatment on hyperpolarized (3)He-magnetic resonance imaging (MRI)-defined ventilation defects in patients with cystic fibrosis aged ≥12years with a G551D-CFTR mutation. METHODS: Part A (single-blind) comprised 4weeks of ivacaftor treatment; Part B (open-label) comprised 48weeks of treatment. The primary outcome was change from baseline in total ventilation defect (TVD; total defect volume:total lung volume ratio)...
January 26, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28120553/what-makes-a-good-pediatric-transplant-lung-insights-from-in-vivo-lung-morphometry-with-hyperpolarized-3-he-magnetic-resonance-imaging
#2
Emily F Fishman, James D Quirk, Stuart C Sweet, Jason C Woods, David S Gierada, Mark S Conradi, Marilyn J Siegel, Dmitriy A Yablonskiy
Obtaining information on transplanted lung microstructure is an important part of the current care for monitoring transplant recipients. However, until now this information was only available from invasive lung biopsy. The objective of this study was to evaluate the use of an innovative non-invasive technique, in vivo lung morphometry with hyperpolarized ³He MRI-to characterize lung microstructure in the pediatric lung transplant population. This technique yields quantitative measurements of acinar airways' (alveolar ducts and sacs) parameters, such as acinar airway radii and alveolar depth...
January 24, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28116474/prenatal-magnetic-resonance-and-ultrasonographic-findings-in-small-bowel-obstruction-imaging-clues-and-postnatal-outcomes
#3
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/28040479/chest-imaging-in-cystic-fibrosis-studies-what-counts-and-can-be-counted
#4
REVIEW
Rhonda Szczesniak, Lidija Turkovic, Eleni-Rosalina Andrinopoulou, Harm A W M Tiddens
BACKGROUND: The dawn of precision medicine and CFTR modulators require more detailed assessment of lung structure in cystic fibrosis (CF) clinical studies. Various imaging markers have emerged and are measurable, but clarity is needed to identify what markers should count for clinical studies. High-resolution chest computed tomography (CT) scoring has yielded sensitive markers for the study of CF disease progression. Once completed, CT scores from ongoing randomized controlled trials can be used to examine relationships between imaging endpoints and therapeutic effectiveness...
December 28, 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28007460/mri-accelerating-progress-in-functional-assessment-of-cystic-fibrosis-lung-disease
#5
EDITORIAL
Mark O Wielpütz, Marcus A Mall
No abstract text is available yet for this article.
December 19, 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/27832324/secretin-stimulated-mri-assessment-of-exocrine-pancreatic-function-in-patients-with-cystic-fibrosis-and-healthy-controls
#6
Adnan Madzak, Trond Engjom, Gaute K Wathle, Søren Schou Olesen, Erling Tjora, Pål R Njølstad, Birger Norderud Lærum, Asbjørn Mohr Drewes, Georg Dimcevski, Jens Brøndum Frøkjær, Ingfrid S Haldorsen
PURPOSE: Secretin-stimulated magnetic resonance imaging (s-MRI) and pancreatic diffusion weighted imaging (DWI) are novel non-invasive imaging techniques for assessment of exocrine pancreatic insufficiency (EPI). The aim was to validate s-MRI assessed pancreatic secreted volume using novel semi-automatic quantification software, and to assess the ability of s-MRI with DWI to diagnose EPI in patients with cystic fibrosis (CF). METHODS: s-MRI and DWI was performed in 19 patients with CF (median age 21 years; range 16-56; eight men) and in 10 healthy controls (HC) (median age 46 years; range 20-65; four men)...
November 10, 2016: Abdominal Radiology
https://www.readbyqxmd.com/read/27776652/biliary-tract-enhancement-in-gadoxetic-acid-enhanced-mri-correlates-with-liver-function-biomarkers
#7
Yoshifumi Noda, Satoshi Goshima, Kimihiro Kajita, Hiroshi Kawada, Nobuyuki Kawai, Hiromi Koyasu, Masayuki Matsuo, Kyongtae T Bae
PURPOSE: To evaluate the association between gadoxetic-acid-enhanced magnetic resonance (MR) imaging measurements and laboratory and clinical biomarkers of liver function and fibrosis. MATERIALS AND METHODS: One hundred thirty nine consecutive patients with suspected liver disease or liver tumor underwent gadoxetic-acid-enhanced MR imaging. MR imaging measurements during the hepatobiliary phase included biliary tract structure-to-muscle signal intensity ratio (SIR)...
November 2016: European Journal of Radiology
https://www.readbyqxmd.com/read/27662104/early-detection-and-sensitive-monitoring-of-cf-lung-disease-prospects-of-improved-and-safer-imaging
#8
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/27575911/comparison-of-lung-clearance-index-and-magnetic-resonance-imaging-for-assessment-of-lung-disease-in-children-with-cystic-fibrosis
#9
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...
February 1, 2017: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/27551814/ultrashort-echo-time-magnetic-resonance-imaging-is-a-sensitive-method-for-the-evaluation-of-early-cystic-fibrosis-lung-disease
#10
David J Roach, Yannick Crémillieux, Robert J Fleck, Alan S Brody, Suraj D Serai, Rhonda D Szczesniak, Stephanie Kerlakian, John P Clancy, Jason C Woods
RATIONALE: Recent advancements that have been made in magnetic resonance imaging (MRI) improve our ability to assess pulmonary structure and function in patients with cystic fibrosis (CF). A nonionizing imaging modality that can be used as a serial monitoring tool throughout life can positively affect patient care and outcomes. OBJECTIVES: To compare an ultrashort echo-time MRI method with computed tomography (CT) as a biomarker of lung structure abnormalities in young children with early CF lung disease...
November 2016: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/27492388/feasibility-tolerability-and-safety-of-pediatric-hyperpolarized-129-xe-magnetic-resonance-imaging-in-healthy-volunteers-and-children-with-cystic-fibrosis
#11
Laura L Walkup, Robert P Thomen, Teckla G Akinyi, Erin Watters, Kai Ruppert, John P Clancy, Jason C Woods, Zackary I Cleveland
BACKGROUND: Hyperpolarized (129)Xe is a promising contrast agent for MRI of pediatric lung function, but its safety and tolerability in children have not been rigorously assessed. OBJECTIVE: To assess the feasibility, safety and tolerability of hyperpolarized (129)Xe gas as an inhaled contrast agent for pediatric pulmonary MRI in healthy control subjects and in children with cystic fibrosis. MATERIALS AND METHODS: Seventeen healthy control subjects (ages 6-15 years, 11 boys) and 11 children with cystic fibrosis (ages 8-16 years, 4 boys) underwent (129)Xe MRI, receiving up to three doses of (129)Xe gas prepared by either a commercially available or a homebuilt (129)Xe polarizer...
November 2016: Pediatric Radiology
https://www.readbyqxmd.com/read/27477942/hyperpolarized-129-xe-for-investigation-of-mild-cystic-fibrosis-lung-disease-in-pediatric-patients
#12
Robert P Thomen, Laura L Walkup, David J Roach, Zackary I Cleveland, John P Clancy, Jason C Woods
BACKGROUND: Cystic fibrosis (CF) is a genetic disease which carries high morbidity and mortality from lung-function decline. Monitoring disease progression and treatment response in young patients is desirable, but serial imaging via CT is often considered prohibitive, and detailed functional information cannot be obtained using conventional imaging techniques. Hyperpolarized (129)Xe magnetic resonance imaging (MRI) can depict and quantify regional ventilation, but has not been investigated in pediatrics...
July 28, 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/27445541/relevance-of-3d-cholangiography-and-transient-elastography-to-assess-cystic-fibrosis-associated-liver-disease
#13
C Lemaitre, S Dominique, E Billoud, M Eliezer, H Montialoux, M Quillard, G Riachi, E Koning, H Morisse-Pradier, G Savoye, C Savoye-Collet, O Goria
Background. Cystic fibrosis-associated liver disease (CFLD) is a major cause of death. The objective of our retrospective study was to describe the relevance of magnetic resonance imaging (MRI) and liver stiffness measurement (LSM) for CFLD evaluation. Methods. All cystic fibrosis adult patients evaluated by MRI and LSM were included. MR signs of portal hypertension (PHT), dysmorphia, or cholangitis were collected and LSM expressed in kPa and Metavir. Results. Of 25 patients, 52% had abnormal MRI. Median LSM was 5...
2016: Canadian Respiratory Journal: Journal of the Canadian Thoracic Society
https://www.readbyqxmd.com/read/27428024/hyperpolarized-gas-magnetic-resonance-lung-imaging-in-children-and-young-adults
#14
Lucia Flors, John P Mugler, Eduard E de Lange, Grady W Miller, Jaime F Mata, Nick Tustison, Iulian C Ruset, F William Hersman, Talissa A Altes
The assessment of early pulmonary disease and its severity can be difficult in young children, as procedures such as spirometry cannot be performed on them. Computed tomography provides detailed structural images of the pulmonary parenchyma, but its major drawback is that the patient is exposed to ionizing radiation. In this context, magnetic resonance imaging (MRI) is a promising technique for the evaluation of pediatric lung disease, especially when serial imaging is needed. Traditionally, MRI played a small role in evaluating the pulmonary parenchyma...
September 2016: Journal of Thoracic Imaging
https://www.readbyqxmd.com/read/27337056/characterizing-lung-disease-in-cystic-fibrosis-with-magnetic-resonance-imaging-and-airway-physiology
#15
Rebecca J Theilmann, Chantal Darquenne, Ann R Elliott, Barbara A Bailey, Douglas J Conrad
Translational investigations in cystic fibrosis (CF) have a need for improved quantitative and longitudinal measures of disease status. To establish a non-invasive quantitative MRI technique to monitor lung health in patients with CF and correlate MR metrics with airway physiology as measured by multiple breath washout (MBW). Data were collected in 12 CF patients and 12 healthy controls. Regional (central and peripheral lung) measures of fractional lung water density (FLD: air to 100% fluid) were acquired both at FRC and TLC on a 1...
2016: PloS One
https://www.readbyqxmd.com/read/27334896/rare-case-of-hepatic-gaucheroma-in-a-child-on-enzyme-replacement-therapy
#16
Sophy Korula, Penny Owens, Amanda Charlton, Kaustuv Bhattacharya
BACKGROUND: We present a 6 year old boy with type I Gaucher treated from 16 months with ERT, developing focal Gaucheroma in the liver at 3.5 years. CASE: The subject presented at 13 months of age with anaemia, thrombocytopenia and hepatosplenomegaly. Gaucher disease was confirmed by leucocyte enzyme assay. A homozygous change: c.1193G>A (p.Arg398Gln) in the GBA gene was identified. He had normal neurology with normal saccades. Imiglucerase was administered at 60 IU/kg/fortnight from 15 months as per Australian regulations with good clinical response...
June 23, 2016: JIMD Reports
https://www.readbyqxmd.com/read/27074425/imaging-of-cystic-fibrosis-lung-disease-and-clinical-interpretation
#17
M O Wielpütz, M Eichinger, J Biederer, S Wege, M Stahl, O Sommerburg, M A Mall, H U Kauczor, M Puderbach
UNLABELLED: Progressive lung disease in cystic fibrosis (CF) is the life-limiting factor of this autosomal recessive genetic disorder. Increasing implementation of CF newborn screening allows for a diagnosis even in pre-symptomatic stages. Improvements in therapy have led to a significant improvement in survival, the majority now being of adult age. Imaging provides detailed information on the regional distribution of CF lung disease, hence longitudinal imaging is recommended for disease monitoring in the clinical routine...
September 2016: RöFo: Fortschritte Auf Dem Gebiete der Röntgenstrahlen und der Nuklearmedizin
https://www.readbyqxmd.com/read/27069980/innovations-in-macroscopic-evaluation-of-pancreatic-specimens-and-radiologic-correlation
#18
Charikleia Triantopoulou, Kleo Papaparaskeva, Christos Agalianos, Christos Dervenis
The purpose of this study was to evaluate the feasibility of a novel dissection technique of surgical specimens in different cases of pancreatic tumors and provide a radiologic pathologic correlation. In our hospital, that is a referral center for pancreatic diseases, the macroscopic evaluation of the pancreatectomy specimens is performed by the pathologists using the axial slicing technique (instead of the traditional procedure with longitudinal opening of the main pancreatic and/or common bile duct and slicing along the plane defined by both ducts)...
2016: European Journal of Radiology Open
https://www.readbyqxmd.com/read/27049043/ct-abnormalities-bacteriology-and-symptoms-of-sinonasal-disease-in-children-with-cystic-fibrosis
#19
M C Berkhout, F Klerx-Melis, W J Fokkens, M Nuijsink, W M C van Aalderen, H G M Heijerman
BACKGROUND: Sinonasal pathology in adults with Cystic Fibrosis (CF) is common but the extent of CT-abnormalities and symptoms of sinonasal disease in children with CF and the age of onset are less frequently studied. METHODS: In this observational, cross-sectional study 58 children with CF from two CF centres were included. All subjects completed a questionnaire regarding sinonasal symptoms, underwent a CT scan of the paranasal sinuses, and in each subject a culture of the upper airways was performed...
November 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/26901001/imaging-of-cystic-fibrosis-and-pediatric-bronchiectasis
#20
REVIEW
Kevin P Murphy, Michael M Maher, Owen J O'Connor
1. CT is superior to pulmonary function tests and chest radiography for the assessment and monitoring of cystic fibrosis (CF)-related lung disease and, also, of pediatric bronchiectasis not caused by CF (hereafter referred to as non-CF bronchiectasis). 2. Low-dose CT protocols that impart radiation doses similar to those used in chest radiography are feasible for the surveillance of patients with bronchiectasis. 3. Chest radiography is still most commonly used as the first-line imaging examination of choice for the assessment of acute complications related to bronchiectasis...
March 2016: AJR. American Journal of Roentgenology
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