A national registry for childhood interstitial and diffuse lung diseases in the United States.

Author(s):  
Lisa Young ◽  
Rebekah Nevel ◽  
Alicia Casey ◽  
Martha Fishman ◽  
Sebastian Welsh ◽  
...  
2021 ◽  
pp. 028418512199579
Author(s):  
Simon S Martin ◽  
Delina Kolaneci ◽  
Julian L Wichmann ◽  
Lukas Lenga ◽  
Doris Leithner ◽  
...  

Background High-resolution computed tomography (HRCT) is essential in narrowing the possible differential diagnoses of diffuse and interstitial lung diseases. Purpose To investigate the value of a novel computer-based decision support system (CDSS) for facilitating diagnosis of diffuse lung diseases at HRCT. Material and Methods A CDSS was developed that includes about 100 different illustrations of the most common HRCT signs and patterns and describes the corresponding pathologies in detail. The logical set-up of the software facilitates a structured evaluation. By selecting one or more CT patterns, the program generates a ranked list of the most likely differential diagnoses. Three independent and blinded radiology residents initially evaluated 40 cases with different lung diseases alone; after at least 12 weeks, observers re-evaluated all cases using the CDSS. Results In 40 patients, a total of 113 HRCT patterns were evaluated. The percentage of correctly classified patterns was higher with CDSS (96.8%) compared to assessment without CDSS (90.3%; P < 0.01). Moreover, the percentage of correct diagnosis (81.7% vs. 64.2%) and differential diagnoses (89.2% vs. 38.3%) were superior with CDSS compared to evaluation without CDSS (both P < 0.01). Conclusion Addition of a CDSS using a structured approach providing explanations of typical HRCT patterns and graphical illustrations significantly improved the performance of trainees in characterizing and correctly identifying diffuse lung diseases.


2021 ◽  
pp. 00880-2020
Author(s):  
Salma G. Abdelhady ◽  
Eman M. Fouda ◽  
Malak A. Shaheen ◽  
Faten A. Ghazal ◽  
Ahmed M. Mostafa ◽  
...  

BackgroundChildhood interstitial and diffuse lung diseases (chILD) encompass a broad spectrum of rare pulmonary disorders. In most developing middle eastern countries, chILD is still underdiagnosed. Objective: To describe and investigate patients diagnosed with chILD in a tertiary university hospital in Egypt.MethodsWe analyzed data of consecutive subjects (<18 years) referred for further evaluation at the Children's Hospital, Ain Shams University. Diagnosis of chILD was made in accordance with the chILD-EU criteria. The following information was obtained: demographic data, clinical characteristics, chest CT findings, laboratory studies, spirometry, BAL and histopathology findings.Results22 subjects were enrolled over 24 months. Median age at diagnosis was 7 years (range 3.5–14 years). The most common manifestations were dyspnea (100%), cough (90.9%), clubbing (95.5%) and tachypnea (90.9%). Systematic evaluation led to the following diagnoses: hypersensitivity pneumonitis (n=3), idiopathic interstitial pneumonias (n=4), chILD related to chronic granulomatous disease (n=3), chILD related to small airway disease (n=3), postinfectious chILD (n=2), Langerhans cell histiocytosis (n=2), Idiopathic pulmonary hemosiderosis (n=2), granulomatous lymphocytic interstitial lung disease (n=1), systemic sclerosis (n=1), familial interstitial lung disease (n=1). Among the subjects who completed the diagnostic evaluation (n=19), treatment was changed in 13 (68.4%) subjects.ConclusionSystematic evaluation and multidisciplinary peer review of chILD patients at our tertiary hospital led to changes in management in 68% of the patients. This study also highlights the need for an Egyptian chILD network with genetic testing, as well as the value of collaborating with international groups in improving health care for children with chILD.


1989 ◽  
Vol 19 (3) ◽  
pp. 281-291 ◽  
Author(s):  
P. G. GIBSON ◽  
B. W. S. ROBINSON ◽  
G. McLENNAN ◽  
D. H. BRYANT ◽  
S. N. BREIT

2008 ◽  
pp. 695-705
Author(s):  
Eric J. Olson ◽  
Jeffrey L. Myers ◽  
Jay H. Ryu

2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Yupeng Li ◽  
Gangao Wu ◽  
Yu Shang ◽  
Yue Qi ◽  
Xue Wang ◽  
...  

Abstract Background Interstitial lung diseases (ILDs), a diverse group of diffuse lung diseases, mainly affect the lung parenchyma. The low-throughput ‘omics’ technologies (genomics, transcriptomics, proteomics) and relative drug information have begun to reshaped our understanding of ILDs, whereas, these data are scattered among massive references and are difficult to be fully exploited. Therefore, we manually mined and summarized these data at a database (ILDGDB, http://ildgdb.org/) and will continue to update it in the future. Main body The current version of ILDGDB incorporates 2018 entries representing 20 ILDs and over 600 genes obtained from over 3000 articles in four species. Each entry contains detailed information, including species, disease type, detailed description of gene (e.g. official symbol of gene), and the original reference etc. ILDGDB is free, and provides a user-friendly web page. Users can easily search for genes of interest, view their expression pattern and detailed information, manage genes sets and submit novel ILDs-gene association. Conclusion The main principle behind ILDGDB’s design is to provide an exploratory platform, with minimum filtering and interpretation, while making the presentation of the data very accessible, which will provide great help for researchers to decipher gene mechanisms and improve the prevention, diagnosis and therapy of ILDs.


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