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Interstitial lung disease child

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https://www.readbyqxmd.com/read/29056600/international-management-platform-for-children-s-interstitial-lung-disease-child-eu
#1
Matthias Griese, Elias Seidl, Meike Hengst, Simone Reu, Hans Rock, Gisela Anthony, Nural Kiper, Nagehan Emiralioğlu, Deborah Snijders, Lutz Goldbeck, Reiner Leidl, Julia Ley-Zaporozhan, Ingrid Krüger-Stollfuss, Birgit Kammer, Traudl Wesselak, Claudia Eismann, Andrea Schams, Doerthe Neuner, Morag MacLean, Andrew G Nicholson, McCann Lauren, Annick Clement, Ralph Epaud, Jacques de Blic, Michael Ashworth, Paul Aurora, Alistair Calder, Martin Wetzke, Matthias Kappler, Steve Cunningham, Nicolaus Schwerk, Andy Bush
BACKGROUND: Children's interstitial lung diseases (chILD) cover many rare entities, frequently not diagnosed or studied in detail. There is a great need for specialised advice and for internationally agreed subclassification of entities collected in a register.Our objective was to implement an international management platform with independent multidisciplinary review of cases at presentation for long-term follow-up and to test if this would allow for more accurate diagnosis. Also, quality and reproducibility of a diagnostic subclassification system were assessed using a collection of 25 complex chILD cases...
October 22, 2017: Thorax
https://www.readbyqxmd.com/read/29019755/interstitial-lung-disease-in-children-made-easier%C3%A2-well-almost
#2
Thomas R Semple, Michael T Ashworth, Catherine M Owens
Interstitial lung disease (ILD) in pediatric patients is different from that in adults, with a vast array of pathologic conditions unique to childhood, varied modes of presentation, and a different range of radiologic appearances. Although rare, childhood ILD (chILD) is associated with significant morbidity and mortality, most notably in conditions of disordered surfactant function, with respiratory failure in 100% of neonates with surfactant protein B dysfunction and 100% mortality without lung transplantation...
October 2017: Radiographics: a Review Publication of the Radiological Society of North America, Inc
https://www.readbyqxmd.com/read/28990899/cytomegalovirus-pneumonia-in-an-immunosuppressed-child-mimicking-interstitial-lung-disease
#3
Esra Karakuş, Güzin Cinel, Saliha Kanık Yüksek, Belgin Gülhan
No abstract text is available yet for this article.
June 2017: Tüberküloz Ve Toraks
https://www.readbyqxmd.com/read/28946688/role-of-high-resolution-chest-computed-tomography-in-a-child-with-persistent-tachypnoea-and-intercostal-retractions-a-case-report-of-neuroendocrine-cell-hyperplasia
#4
Mara Lelii, Maria Francesca Patria, Raffaella Pinzani, Rossana Tenconi, Alessandro Mori, Nicola Bonelli, Nicola Principi, Susanna Esposito
Background: Chronic interstitial lung diseases in children (chILD) are a heterogeneous group of disorders that can represent a clinical challenge for pediatric pneumologists. Among them, neuroendocrine cell hyperplasia of infancy (NEHI) is a diffuse lung disease prevalent in the first years of life that spontaneously improves over time. The clinical presentation of NEHI is indistinguishable from other interstitial lung diseases, so a correct and non-invasive diagnosis by chest computed tomography (CT) without lung biopsy might not be simple...
September 25, 2017: International Journal of Environmental Research and Public Health
https://www.readbyqxmd.com/read/28898549/filamin-a-flna-mutation-a-newcomer-to-the-childhood-interstitial-lung-disease-child-classification
#5
Susan C Shelmerdine, Thomas Semple, Colin Wallis, Paul Aurora, Shahin Moledina, Michael T Ashworth, Catherine M Owens
AIM: Interstitial lung disease (ILD) in infants represents a rare and heterogenous group of disorders, distinct from those occurring in adults. In recent years a new entity within this category is being recognized, namely filamin A (FLNA) mutation related lung disease. Our aims are to describe the clinical and radiological course of patients with this disease entity to aid clinicians in the prognostic counseling and management of similar patients they may encounter. METHOD: A retrospective case note review was conducted of all patients treated at our institution (a specialist tertiary referral childrens' center) for genetically confirmed FLNA mutation related lung disease...
October 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28743279/childhood-interstitial-lung-diseases-in-immunocompetent-children-in-australia-and-new-zealand-a-decade-s-experience
#6
Vishal Saddi, Sean Beggs, Bruce Bennetts, Joanne Harrison, Neil Hime, Nitin Kapur, Jill Lipsett, Lawrence M Nogee, Amy Phu, Sadasivam Suresh, André Schultz, Hiran Selvadurai, Stephanie Sherrard, Roxanne Strachan, Julian Vyas, Yvonne Zurynski, Adam Jaffé
BACKGROUND: Childhood interstitial lung disease (chILD) represents a rare heterogeneous group of respiratory disorders. In the absence of randomized controlled clinical trials, global collaborations have utilized case series with an aim to standardising approaches to diagnosis and management. Australasian data are lacking. The aim of this study was to calculate prevalence and report the experience of chILD in Australasia over a decade. METHODS: Paediatric pulmonologists in Australia and New Zealand involved in the care of patients aged 0-18 years with chILD completed a questionnaire on demographics, clinical features and outcomes, over a 10 year period...
July 25, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28561249/interstitial-lung-disease-in-women-of-child-bearing-age
#7
Ruben Mylvaganam, Sakshi Dua, Catherine Nelson-Piercy, Aditi Mathur
No abstract text is available yet for this article.
April 2017: Seminars in Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/28349192/-interstitial-processes-of-the-lungs-in-childhood
#8
H Popper
Interstitial processes in the lungs of children can be due to several underlying diseases. Knowledge of the child's age is important as genetic aberrations play a major role in diseases in the first 2 years, whereas immunological diseases are more common starting in kindergarden age. In general lung diseases are rare in children, which makes the diagnostics difficult and results in a delayed diagnosis. In addition, pediatric pulmonologists are often very reluctant to perform lung biopsies due to a lack of a specialized pathologist...
July 2017: Der Pathologe
https://www.readbyqxmd.com/read/28272068/rare-disease-heralded-by-pulmonary-manifestations-avoiding-pitfalls-of-an-asthma-label
#9
S Bajaj, M Muranjan, S Karande, D Prabhat
Pulmonary manifestations are seldom recognized as symptoms of storage disorders. The report describes the diagnostic journey in a 30-month-old male infant, born of a third-degree consanguineous marriage referred to our institute as severe persistent asthma. History revealed that the child had progressively worsening breathlessness and persistent dry cough not associated with fever but accompanied by weight loss. On physical examination, there was growth failure, respiratory distress, clubbing, hepatosplenomegaly, and occasional rhonchi...
April 2017: Journal of Postgraduate Medicine
https://www.readbyqxmd.com/read/27903688/management-of-children-with-interstitial-lung-diseases-the-difficult-issue-of-acute-exacerbations
#10
EDITORIAL
Annick Clement, Jacques de Blic, Ralph Epaud, Laurie Galeron, Nadia Nathan, Alice Hadchouel, Angelo Barbato, Deborah Snijders, Nural Kiper, Steve Cunningham, Matthias Griese, Andrew Bush, Nicolaus Schwerk
No abstract text is available yet for this article.
December 2016: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/27774750/pulmonary-hypertension-in-childhood-interstitial-lung-disease-a-systematic-review-of-the-literature
#11
REVIEW
Susan Bromley, David Vizcaya
Childhood interstitial lung disease (chILD) comprises a wide heterogeneous group of rare parenchymal lung disorders associated with substantial morbidity and mortality. Pulmonary hypertension is a common comorbidity in adults with interstitial lung disease (ILD) and associated with poor survival. We aimed to systematically review the literature regarding the occurrence of pulmonary hypertension (PH) in chILD, its effect on prognosis and healthcare use, and its treatment in clinical practice. Searches of PubMed and EMBASE databases (up to February 2016), and American Thoracic Society conference abstracts (2009-2015) were conducted using relevant keywords...
May 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/27686729/molecular-mechanisms-underlying-fibrosis-and-elastin-destruction-in-childhood-interstitial-lung-diseases
#12
Enas A Hamed, Mostafa M El-Saied, Khaled Saad, Hazem Abu-Zeid Yousef, Amany O Mohamed, Dina Sabry
OBJECTIVE: This study aimed to evaluate fibrosis and elastin destruction in childhood interstitial lung disease (chILD) patients. METHODS: Sixty patients and twenty healthy children were recruited. On admission, evaluation of chILD severity was made using Fan chILD score. Participants provided urine and blood samples. Plasma levels of transforming growth factor (TGF)-β1, connective tissue growth factor (CCN2), soluble factor related apoptosis (sFas) and long non-coding RNAs and urinary levels of desmosine/urinary creatinine (UDes/UCr) were measured...
December 2016: Pathophysiology: the Official Journal of the International Society for Pathophysiology
https://www.readbyqxmd.com/read/27629751/neuroendocrine-cell-hyperplasia-of-infancy-an-unusual-cause-of-hypoxemia-in-children
#13
Silvia Caimmi, Amelia Licari, Davide Caimmi, Anna Rispoli, Eugenio Baraldi, Fiorella Calabrese, Gian Luigi Marseglia
BACKGROUND: Childhood interstitial lung disease (chILD) is a heterogeneous group of rare disorders characterized by abnormal imaging findings, impaired gas exchange; and is associated with substantial morbidity and mortality. Neuroendocrine cell hyperplasia (NEHI) is a unique sub-group, which is more prevalent in infants and children younger than 2 years of age, and typically manifests with chronic tachypnea, retractions, hypoxemia and failure to thrive. NEHI insidiously appears in the first year of life, subtly masquerading as one of the more common lung diseases of childhood...
September 15, 2016: Italian Journal of Pediatrics
https://www.readbyqxmd.com/read/27374344/functional-characterization-of-atp-binding-cassette-transporter-a3-mutations-from-infants-with-respiratory-distress-syndrome
#14
Jennifer A Wambach, Ping Yang, Daniel J Wegner, Hillary B Heins, Lyudmila N Kaliberova, Sergey A Kaliberov, David T Curiel, Frances V White, Aaron Hamvas, Brian P Hackett, F Sessions Cole
Mutations in the ATP-binding cassette transporter A3 gene (ABCA3) result in severe neonatal respiratory distress syndrome and childhood interstitial lung disease. As most ABCA3 mutations are rare or private, determination of mutation pathogenicity is often based on results from in silico prediction tools, identification in unrelated diseased individuals, statistical association studies, or expert opinion. Functional biologic studies of ABCA3 mutations are needed to confirm mutation pathogenicity and inform clinical decision making...
November 2016: American Journal of Respiratory Cell and Molecular Biology
https://www.readbyqxmd.com/read/27362365/a-novel-surfactant-protein-c-gene-mutation-associated-with-progressive-respiratory-failure-in-infancy
#15
REVIEW
Melissa Kaori Silva Litao, Don Hayes, Saurabh Chiwane, Lawrence M Nogee, Geoffrey Kurland, Lokesh Guglani
Mutations of the Surfactant Protein C (SPC) gene (SFTPC) have been associated with childhood interstitial lung disease (chILD) with variable age of onset, severity of lung disease, and outcomes. We report a novel mutation in SFTPC [c.435G->A, p.(Gln145)] that was associated with onset of symptoms in early infancy, progressive respiratory failure with need for prolonged mechanical ventilatory support, and eventual lung transplant at 1 year of age. While the mutation was not predicted to alter the amino acid sequence of the SP-C precursor protein, analysis of SP-C transcripts demonstrated skipping of exon 4...
January 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/27245831/interstitial-lung-disease-in-children-younger-than-2-years
#16
REVIEW
Paolo Spagnolo, Andrew Bush
Childhood interstitial lung disease (chILD) represents a highly heterogeneous group of rare disorders associated with substantial morbidity and mortality. Although our understanding of chILD remains limited, important advances have recently been made, the most important being probably the appreciation that disorders that present in early life are distinct from those occurring in older children and adults, albeit with some overlap. chILD manifests with diffuse pulmonary infiltrates and nonspecific respiratory signs and symptoms, making exclusion of common conditions presenting in a similar fashion an essential preliminary step...
June 2016: Pediatrics
https://www.readbyqxmd.com/read/27145498/interstitial-lung-disease-in-infancy-a-general-approach
#17
REVIEW
Erica J Hines, Mark Walsh, Jane E Armes, Tonia Douglas, Jasneek Chawla
Childhood Interstitial lung disease (chILD) is an umbrella term used to define a broad range of rare, diffuse pulmonary disorders with altered interstitial structure that leads to abnormal gas exchange. Presentation of chILD in infancy can be difficult to differentiate from other common causes of diffuse lung disease. This article aimed at paediatricians provides an overview of interstitial lung disease presenting in infancy and includes key clinical features, a suggested approach to investigation and a summary of management...
April 2016: Journal of Paediatrics and Child Health
https://www.readbyqxmd.com/read/27134508/the-first-successful-heart-lung-transplant-in-a-korean-child-with-humidifier-disinfectant-associated-interstitial-lung-disease
#18
Won Kyoung Jhang, Seong Jong Park, Eun Lee, Song I Yang, Soo Jong Hong, Ju-Hee Seo, Hyung-Young Kim, Jeong-Jun Park, Tae-Jin Yun, Hyeong Ryul Kim, Yong-Hee Kim, Dong Kwan Kim, Seung-Il Park, Sang-Oh Lee, Sang-Bum Hong, Tae-Sun Shim, In-Cheol Choi, Jinho Yu
From 2006 to 2011, an outbreak of a particular type of childhood interstitial lung disease occurred in Korea. The condition was intractable and progressed to severe respiratory failure, with a high mortality rate. Moreover, in several familial cases, the disease affected young women and children simultaneously. Epidemiologic, animal, and post-interventional studies identified the cause as inhalation of humidifier disinfectants. Here, we report a 4-year-old girl who suffered from severe progressive respiratory failure...
May 2016: Journal of Korean Medical Science
https://www.readbyqxmd.com/read/26765478/increased-risks-of-spontaneous-bacterial-peritonitis-and-interstitial-lung-disease-in-primary-biliary-cirrhosis-patients-with-concomitant-sj%C3%A3-gren-syndrome
#19
Chun-Ting Chen, Yu-Chen Tseng, Chih-Wei Yang, Hsuan-Hwai Lin, Peng-Jen Chen, Tien-Yu Huang, Yu-Lueng Shih, Wei-Kuo Chang, Tsai-Yuan Hsieh, Heng-Cheng Chu
The incidence of Sjögren syndrome (SS) in primary biliary cirrhosis (PBC) patients is high. The influence of SS on the clinical outcomes of PBC patients, however, remains unclear. Our study retrospectively collected data on PBC-only patients and PBC patients with concomitant SS (PBC-SS) to compare the clinical differences of long-term outcomes between them.A total of 183 patients were diagnosed with PBC from January 1999 to December 2014 at our hospital. Of these, the authors excluded patients with diabetes, hypertension, advanced liver cirrhosis at initial diagnosis of PBC (Child-Turcotte-Pugh classification score of ≥7) and other liver diseases (ie, alcoholic liver disease, alpha-antitrypsin deficiency, viral hepatitis, and primary sclerosing cholangitis), and autoimmune diseases such as systemic lupus erythematosus and rheumatoid arthritis...
January 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/26636747/effectiveness-of-palivizumab-in-children-with-childhood-interstitial-lung-disease-the-french-experience
#20
David Drummond, Caroline Thumerelle, Philippe Reix, Michael Fayon, Ralph Epaud, Annick Clement, Malika Mahloul, Delphine Habouria, Christophe Delacourt, Alice Hadchouel
INTRODUCTION: There is a lack of evidence concerning the effectiveness of immunoprophylaxis with palivizumab in children with childhood interstitial lung disease (chILD). In this retrospective study, we evaluated the effectiveness of palivizumab for decreasing the rate of RSV-related hospitalizations in children under the age of 24 months with chILD treated with corticosteroids. METHODS: A retrospective national study was conducted in France. Patients born between 2007 and 2013, diagnosed with chILD and on corticosteroid treatment were identified through the French online database for pediatric interstitial lung disease (Respirare(®) )...
July 2016: Pediatric Pulmonology
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