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Diffuse parenchymal lung disease

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https://www.readbyqxmd.com/read/28433201/humoral-primary-immunodeficiency-diseases-clinical-overview-and-chest-high-resolution-computed-tomography-hrct-features-in-the-adult-population
#1
REVIEW
L Cereser, R Girometti, P d'Angelo, M De Carli, A De Pellegrin, C Zuiani
Humoral primary immunodeficiency diseases (hPIDs) are a heterogeneous group of hereditary disorders resulting in abnormal susceptibility to infections of the sinopulmonary tract. Some of these conditions (e.g., common variable immunodeficiency disorders [CVID]) imply a number of non-infectious thoracic complications such as non-infectious airway disorders, diffuse lung parenchymal diseases, and neoplasms. Chest high-resolution computed tomography (HRCT) is a key imaging tool to characterise and quantify the extent of underlying thoracic involvement, as well as to direct and monitor treatment...
April 19, 2017: Clinical Radiology
https://www.readbyqxmd.com/read/28427553/familial-pulmonary-fibrosis-clinical-and-radiological-characteristics-and-progression-analysis-in-different-high-resolution-ct-patterns
#2
David Bennett, Maria Antonietta Mazzei, Nevada Cioffi Squitieri, Elena Bargagli, Rosa Metella Refini, Antonella Fossi, Luca Volterrani, Paola Rottoli
BACKGROUND: Familial pulmonary fibrosis (FPF) is defined as an idiopathic diffuse parenchymal lung disease affecting two or more members of the same primary biological family. The aim of the present study was to contribute to the clinical, functional and radiological characterisation of FPF with particular regards to disease progression and survival. METHODS: Baseline clinical, functional and radiological data of a FPF population (n = 46 patients) were retrospectively collected and analysed according to the 2011 IPF guidelines HRCT classification...
May 2017: Respiratory Medicine
https://www.readbyqxmd.com/read/28399377/transbronchial-lung-cryobiopsy-and-video-assisted-thoracoscopic-lung-biopsy-in-the-diagnosis-of-diffuse-parenchymal-lung-disease-a-meta-analysis-of-diagnostic-test-accuracy
#3
Imran H Iftikhar, Lana Alghothani, Alejandro Sardi, David Berkowitz, Ali I Musani
BACKGROUND: Transbronchial lung cryobiopsy (TBLC) is increasingly being used for the assessment of diffuse pulmonary lung diseases (DPLD). Several studies have shown larger biopsy samples and higher yields compared to conventional transbronchial biopsies. However, the higher risk of bleeding and other complications has raised concerns for widespread use of this modality. OBJECTIVE: To study the diagnostic accuracy and safety profile of TBLC and compare with video assisted thoracoscopic (VATS), by reviewing available evidence from the literature...
April 11, 2017: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/28393574/transbronchial-biopsies-safely-diagnose-amyloid-lung-disease
#4
Praveen Govender, Colleen M Keyes, Elizabeth A Hankinson, Carl J O'Hara, Vaishali Sanchorawala, John L Berk
BACKGROUND: Autopsy identifies lung involvement in 58-92% of patients with the most prevalent forms of systemic amyloidoses. In the absence of lung biopsies, amyloid lung disease often goes unrecognized. Report of a death following transbronchial biopsies in a patient with systemic amyloidosis cautioned against the procedure in this patient cohort. We reviewed our experience with transbronchial biopsies in patients with amyloidosis to determine the safety and utility of bronchoscopic lung biopsies...
April 10, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28388260/acute-exacerbation-and-decline-in-forced-vital-capacity-are-associated-with-increased-mortality-in-idiopathic-pulmonary-fibrosis
#5
Miya O Paterniti, Youwei Bi, Dinko Rekić, Yaning Wang, Banu A Karimi-Shah, Badrul A Chowdhury
RATIONALE: Exploration of forced vital capacity (FVC) as it relates to mortality in idiopathic pulmonary fibrosis (IPF), a chronic, progressive, and ultimately fatal parenchymal lung disease, is important both clinically and to the current drug development paradigm. We evaluated the association between FVC decline and mortality in the largest, well-characterized placebo cohort to date. Additionally, we sought to explore the risk of death from acute exacerbations and further validate previously identified baseline predictors of mortality...
April 7, 2017: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/28377567/chronic-granulomatous-disease-presenting-as-aspergillus-fumigatus-pneumonia-in-a-previously-healthy-young-woman
#6
David Williams, Dipen Kadaria, Amik Sodhi, Roy Fox, Glenn Williams, Stephen Threlkeld
BACKGROUND Chronic Granulomatous Disease (CGD) is a rare immunodeficiency disease caused by a genetic defect in the NADPH (nicotinamide adenine dinucleotide phosphate) oxidase enzyme, resulting in increased susceptibility to bacterial and fungal infections. The inheritance can be X-linked or autosomal recessive. Patients usually present with repeated infections early in life. We present an unusual case of a 23-year-old patient diagnosed with CGD. CASE REPORT A 23-year-old white woman with no previous history of recurrent infections presented with complaints of fever, shortness of breath, and diffuse myalgia...
April 5, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28266906/pirfenidone-for-the-treatment-of-idiopathic-pulmonary-fibrosis
#7
Peter M George, Athol U Wells
Idiopathic pulmonary fibrosis (IPF) is a diffuse parenchymal lung disease with no cure. Up until recently, no treatment had been proven to alter its natural history as judged by rate of lung function decline. In 2014 however, the emergence of two novel anti-fibrotic agents, Pirfenidone and Nintedanib revolutionized the management of this condition. Both have demonstrated the ability to deliver a major reduction in the rate of chronic IPF progression. Areas Covered: This review article focuses on Pirfenidone - a pyridone derivative initially designed as an analgesic and anti-pyretic agent...
May 2017: Expert Review of Clinical Pharmacology
https://www.readbyqxmd.com/read/28245447/transbronchial-lung-cryobiopsy-in-diffuse-parenchymal-lung-disease-comparison-between-biopsy-from-1-segment-and-biopsy-from-2-segments-diagnostic-yield-and-complications
#8
Claudia Ravaglia, Athol U Wells, Sara Tomassetti, Alessandra Dubini, Alberto Cavazza, Sara Piciucchi, Nicola Sverzellati, Carlo Gurioli, Christian Gurioli, Ulrich Costabel, Paola Tantalocco, Jay H Ryu, Marco Chilosi, Venerino Poletti
BACKGROUND: Transbronchial lung cryobiopsy is an innovative method of obtaining samples from the parenchyma of patients with diffuse parenchymal lung diseases. However, the technique is not yet standardized, and uncertainty exists about the optimal protocol, including the number of samples, the biopsy size, and the choice of the biopsy site. OBJECTIVES: To compare the diagnostic yield and complications of cryobiopsy with different strategies adopted to sample lung tissue (number of samples, biopsy site, and sample size)...
2017: Respiration; International Review of Thoracic Diseases
https://www.readbyqxmd.com/read/28182861/diffuse-pulmonary-ossification-in-fibrosing-interstitial-lung-diseases-prevalence-and-associations
#9
Ryoko Egashira, Joseph Jacob, Maria A Kokosi, Anne-Laure Brun, Alexandra Rice, Andrew G Nicholson, Athol U Wells, David M Hansell
Purpose To investigate the prevalence of diffuse pulmonary ossification (DPO) in patients with fibrosing interstitial lung disease (ILD) and determine whether there are differences among the types of ILDs. Materials and Methods Institutional review board approval was given and patient consent was not required for this study. The study population comprised 892 consecutive patients with fibrosing ILD, including 456 patients with idiopathic pulmonary fibrosis (IPF) (men, 366; women, 90; median age, 72 years [range, 38-93 years]), 244 with nonspecific interstitial pneumonia (men, 79; women, 165; median age, 60...
February 9, 2017: Radiology
https://www.readbyqxmd.com/read/28130610/automated-computer-based-ct-stratification-as-a-predictor-of-outcome-in-hypersensitivity-pneumonitis
#10
Joseph Jacob, B J Bartholmai, S Rajagopalan, R Karwoski, S M Mak, W Mok, G Della Casa, K Sugino, S L F Walsh, A U Wells, D M Hansell
BACKGROUND: Hypersensitivity pneumonitis (HP) has a variable clinical course. Modelling of quantitative CALIPER-derived CT data can identify distinct disease phenotypes. Mortality prediction using CALIPER analysis was compared to the interstitial lung disease gender, age, physiology (ILD-GAP) outcome model. METHODS: CALIPER CT analysis of parenchymal patterns in 98 consecutive HP patients was compared to visual CT scoring by two radiologists. Functional indices including forced vital capacity (FVC) and diffusion capacity for carbon monoxide (DLco) in univariate and multivariate Cox mortality models...
January 27, 2017: European Radiology
https://www.readbyqxmd.com/read/28076684/radiographic-differentiation-of-advanced-fibrocystic-lung-diseases
#11
Masanori Akira
The concept of end-stage lung disease suggests a final common pathway for most diffuse parenchymal lung diseases. In accordance with this concept, end-stage disease is characterized radiographically and pathologically by the presence of extensive honeycombing. However, sequential computed tomographic (CT) scans obtained from patients with chronic diffuse lung disease evolve over time to show various advanced lung disease patterns other than honeycombing. In addition, several radiographically distinct honeycomb patterns, including microcystic, macrocystic, mixed, and combined emphysema and honeycombing, differentiate one advanced lung disease from another...
March 2017: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/28050417/diffuse-parenchymal-lung-diseases-with-clinicoradiological-discordance-role-of-transbronchial-lung-biopsy-as-a-diagnostic-tool-an-observational-study
#12
K P Suraj, Kiran Vishnu Narayan, Jyothi Edakalavan, Neethu Kesava Kumar
INTRODUCTION: The diagnosis of Diffuse Parenchymal Lung Disease (DPLD) requires a multidisciplinary approach with reconciliation of clinicoradiological and histopathological data. But when the preliminary clinicoradiological profile fails to suggest a diagnosis, an adequate lung biopsy specimen with meticulous histological examination and a multidisciplinary approach usually yields results. There is also a high chance of sampling error due to patchy and heterogeneous involvement of the disease process and due to the small volume of tissue taken...
November 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28034695/aberrant-lung-remodeling-in-a-mouse-model-of-surfactant-dysregulation-induced-by-modulation-of-the-abca3-gene
#13
Michael F Beers, Lars Knudsen, Yaniv Tomer, Julian Maronn, Ming Zhao, Matthias Ochs, Surafel Mulugeta
The lipid transporter, ATP binding cassette class A3 (ABCA3), plays a critical role in the biogenesis of alveolar type 2 (AT2) cell lamellar bodies (LBs). A relatively large number of mutations in the ABCA3 gene have been identified in association with diffuse parenchymal lung disease (DPLD), the most common of which is a missense mutation (valine substitution for lysine at residue 292 (ABCA3(E292V))) that leads to functional impairment of the transporter in vitro. The consequences of ABCA3(E292)(V) gene expression in vivo are unknown...
March 2017: Annals of Anatomy, Anatomischer Anzeiger: Official Organ of the Anatomische Gesellschaft
https://www.readbyqxmd.com/read/28025703/the-biology-of-the-abca3-lipid-transporter-in-lung-health-and-disease
#14
REVIEW
Michael F Beers, Surafel Mulugeta
The lipid transporter, ATP-binding cassette class A3 (ABCA3), is a highly conserved multi-membrane-spanning protein that plays a critical role in the regulation of pulmonary surfactant homeostasis. Mutations in ABCA3 have been increasingly recognized as one of the causes of inherited pulmonary diseases. These monogenic disorders produce familial lung abnormalities with pathological presentations ranging from neonatal surfactant-deficiency-induced respiratory failure to childhood or adult diffuse parenchymal lung diseases for which specific treatment modalities remain limited...
March 2017: Cell and Tissue Research
https://www.readbyqxmd.com/read/27922241/an-unusual-case-of-neuroblastoma-a-17-year-old-adolescent-presented-with-bilateral-diffuse-lung-metastasis-at-initial-diagnosis
#15
Çağlar Ödek, Tanıl Kendirli, Esra Pekpak, Ayhan Yaman, Başak Adaklı-Aksoy, Handan Dinçaslan, Nurdan Taçyıldız, Emel Ünal, Erdal İnce, Gülsan Yavuz
Neuroblastoma (NB) is the most frequently diagnosed neoplasm during infancy and its incidence declines within the first 3-5 years of life. It can be rarely diagnosed in adolescents and young adults. Adolescents have advanced stage of disease, higher frequency of uncommon metastatic sites such as lungs, and worse outcomes. Herein, we describe an unusual case of NB in a 17-year-old adolescent presented with lung metastasis at diagnosis. The patient was diagnosed with stage IV NB. Thorax high-resolution computed tomography (HRCT) scan revealed irregular septal thickening with ground glass opacity consistent with pulmonary parenchymal metastases...
2016: Turkish Journal of Pediatrics
https://www.readbyqxmd.com/read/27920654/a-rare-cause-of-pulmonary-nodules
#16
Michael Tsuyoshi Chew, Eric Chak, Karen Matsukuma
Crohn's disease is a chronic, idiopathic autoimmune disorder that primarily targets the gastrointestinal (GI) system. It is characterized by transmural inflammation of the GI tract that can occur anywhere from the mouth to the anus. Not infrequently, the disease may also have extraintestinal manifestations (EIMs) that can affect almost any organ system. It is estimated that EIMs affect up to 36% of patients with Crohn's disease, but the incidence and prevalence of pulmonary involvement are variable in the literature and may be as low as 0...
September 2016: Case Reports in Gastroenterology
https://www.readbyqxmd.com/read/27884593/idiopathic-interstitial-pneumonia-associated-pulmonary-hypertension-a-target-for-therapy
#17
Steven D Nathan, Jürgen Behr, Vincent Cottin, Harold R Collard, Marius M Hoeper, Fernando J Martinez, Athol U Wells
Advances in the treatment of idiopathic interstitial pneumonia (IIP) represent an urgent, unmet medical need for patients with this category of diffuse parenchymal lung disease. IIPs involve varying combinations of fibrosis and inflammation of unknown cause and may be associated with pulmonary hypertension (PH). When it occurs, PH is associated with higher oxygen needs, greater functional impairment, and increased mortality. However, whether or when PH is a maladaptive versus adaptive phenomenon remains to be determined...
November 5, 2016: Respiratory Medicine
https://www.readbyqxmd.com/read/27876024/evaluation-of-computer-based-computer-tomography-stratification-against-outcome-models-in-connective-tissue-disease-related-interstitial-lung-disease-a-patient-outcome-study
#18
Joseph Jacob, Brian J Bartholmai, Srinivasan Rajagopalan, Anne Laure Brun, Ryoko Egashira, Ronald Karwoski, Maria Kokosi, Athol U Wells, David M Hansell
BACKGROUND: To evaluate computer-based computer tomography (CT) analysis (CALIPER) against visual CT scoring and pulmonary function tests (PFTs) when predicting mortality in patients with connective tissue disease-related interstitial lung disease (CTD-ILD). To identify outcome differences between distinct CTD-ILD groups derived following automated stratification of CALIPER variables. METHODS: A total of 203 consecutive patients with assorted CTD-ILDs had CT parenchymal patterns evaluated by CALIPER and visual CT scoring: honeycombing, reticular pattern, ground glass opacities, pulmonary vessel volume, emphysema, and traction bronchiectasis...
November 23, 2016: BMC Medicine
https://www.readbyqxmd.com/read/27853529/current-concepts-and-dilemmas-in-idiopathic-interstitial-pneumonias
#19
REVIEW
Jay H Ryu, Teng Moua, Natalya Azadeh, Misbah Baqir, Eunhee S Yi
Idiopathic interstitial pneumonias comprise approximately one-third of interstitial lung diseases (also called diffuse parenchymal infiltrative lung diseases). The classification of idiopathic interstitial pneumonias has undergone several revisions since the initial description of 40 years ago, and the most recent version was published in 2013. Although some aspects have been clarified, this group of heterogeneous disorders continues to be a source of confusion and misunderstanding in clinical applications...
2016: F1000Research
https://www.readbyqxmd.com/read/27833411/the-impact-of-iterative-reconstruction-in-low-dose-computed-tomography-on-the-evaluation-of-diffuse-interstitial-lung-disease
#20
Hyun-Ju Lim, Myung Jin Chung, Kyung Eun Shin, Hye Sun Hwang, Kyung Soo Lee
OBJECTIVE: To evaluate the impact of iterative reconstruction (IR) on the assessment of diffuse interstitial lung disease (DILD) using CT. MATERIALS AND METHODS: An American College of Radiology (ACR) phantom (module 4 to assess spatial resolution) was scanned with 10-100 effective mAs at 120 kVp. The images were reconstructed using filtered back projection (FBP), adaptive statistical iterative reconstruction (ASIR), with blending ratios of 0%, 30%, 70% and 100%, and model-based iterative reconstruction (MBIR), and their spatial resolution was objectively assessed by the line pair structure method...
November 2016: Korean Journal of Radiology: Official Journal of the Korean Radiological Society
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