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Children's interstitial lung disease

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https://www.readbyqxmd.com/read/29627169/side-effects-of-medications-used-to-treat-childhood-interstitial-lung-disease
#1
REVIEW
Oded Breuer, André Schultz
Interstitial lung disease in children (chILD) comprises a range of different rare diseases. There is limited evidence for the treatment of chILD and no randomised clinical trials of treatment have been undertaken. Most treatments are therefore prescribed off-label based on expert opinion. The off-label nature of prescription of drugs for chILD highlights the importance of a solid understanding of the side effects to facilitate risk-benefit assessment. The European Respiratory Society chILD guidelines recommend the use of systemic glucocorticosteroids, hydroxychloroquine and azithromycin...
March 17, 2018: Paediatric Respiratory Reviews
https://www.readbyqxmd.com/read/29614564/-the-classification-of-congenital-respiratory-diseases-in-children
#2
(no author information available yet)
No abstract text is available yet for this article.
April 2, 2018: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/29580292/hermansky-pudlak-syndrome-type-2-manifests-with-fibrosing-lung-disease-early-in-childhood
#3
Meike Hengst, Lutz Naehrlich, Poornima Mahavadi, Joerg Grosse-Onnebrink, Suzanne Terheggen-Lagro, Lars Høsøien Skanke, Luise A Schuch, Frank Brasch, Andreas Guenther, Simone Reu, Julia Ley-Zaporozhan, Matthias Griese
BACKGROUND: Hermansky-Pudlak syndrome (HPS), a hereditary multisystem disorder with oculocutaneous albinism, may be caused by mutations in one of at least 10 separate genes. The HPS-2 subtype is distinguished by the presence of neutropenia and knowledge of its pulmonary phenotype in children is scarce. METHODS: Six children with genetically proven HPS-2 presented to the chILD-EU register between 2009 and 2017; the data were collected systematically and imaging studies were scored blinded...
March 27, 2018: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/29578653/diagnostic-accuracy-and-therapeutic-relevance-of-thoracoscopic-lung-biopsies-in-children
#4
Caroline Fortmann, Nicolaus Schwerk, Martin Wetzke, Nagoud Schukfeh, Benno M Ure, Jens Dingemann
INTRODUCTION: Histopathological assessment of lung biopsies does play an important diagnostic role in children's interstitial lung disease (ChILD). Thoracoscopic lung biopsy has been shown to be safe and effective. The aim of this study was to evaluate the diagnostic accuracy of thoracoscopic lung biopsies in children with ChILD. Furthermore, therapeutic relevance of the procedure, operative details, and perioperative complications of our series were investigated. METHODS: We retrospectively reviewed all consecutive thoracoscopic lung biopsies taken from children with suspected ChILD in our institution over an 11-year period...
March 26, 2018: Pediatric Pulmonology
https://www.readbyqxmd.com/read/29569581/genetic-basis-for-childhood-interstitial-lung-disease-among-japanese-infants-and-children
#5
Itaru Hayasaka, Kazutoshi Cho, Takuma Akimoto, Masahiko Ikeda, Yutaka Uzuki, Masafumi Yamada, Koh Nakata, Itsuko Furuta, Tadashi Ariga, Hisanori Minakami
BackgroundGenetic variants responsible for childhood interstitial lung disease (chILD) have not been studied extensively in Japanese patients.MethodsThe study population consisted of 62 Japanese chILD patients. Twenty-one and four patients had pulmonary hypertension resistant to treatment (PH) and hypothyroidism, respectively. Analyses of genetic variants were performed in all 62 patients for SFTPC and ABCA3, in all 21 PH patients for FOXF1, and in a limited number of patients for NKX2.1.ResultsCausative genetic variants for chILD were identified in 11 (18%) patients: SFTPC variants in six, NKX2...
February 2018: Pediatric Research
https://www.readbyqxmd.com/read/29569218/ct-features-of-diffuse-lung-disease-in-infancy
#6
Paolo Toma, Aurelio Secinaro, Oliviero Sacco, Davide Curione, Renato Cutrera, Nicola Ullmann, Claudio Granata
Diffuse lung disease in infancy includes a wide range of very rare and peculiar pulmonary conditions usually not seen in older children, in whom diffuse lung disease has much greater overlap with adult disorders. The acronym chILD (childhood Interstitial Lung Disease) commonly defines these disorders, although air spaces, airways, alveolar epithelium, vasculature, pleura, and pleural spaces can also be involved, besides the pulmonary interstitium. chILD can be caused by diffuse developmental disorders, alveolar growth abnormalities, surfactant dysfunction disorders, and other specific conditions of poorly understood etiology...
March 22, 2018: La Radiologia Medica
https://www.readbyqxmd.com/read/29517585/genetic-causes-and-clinical-management-of-pediatric-interstitial-lung-diseases
#7
Nadia Nathan, Keren Borensztajn, Annick Clement
PURPOSE OF REVIEW: Interstitial lung disease (ILD) in children (chILD) is an umbrella term for a heterogeneous group of rare respiratory disorders that are mostly chronic and associated with high morbidity and mortality. The pathogenesis of the various chILD is complex and implicates genetic contributors. The purpose of this review is to provide updated information on the molecular defects associated with the development of chILD. RECENT FINDINGS: Currently, the main mutations are identified in the surfactant genes SFTPA1, SFTPA2, SFTPB, SFTPC, ABCA3, and NKX2-1...
May 2018: Current Opinion in Pulmonary Medicine
https://www.readbyqxmd.com/read/29505158/abca3-missense-mutations-causing-surfactant-dysfunction-disorders-have-distinct-cellular-phenotypes
#8
Ulrike Schindlbeck, Thomas Wittmann, Stefanie Höppner, Susanna Kinting, Gerhard Liebisch, Jan Hegermann, Matthias Griese
Mutations in the ATP-binding cassette subfamily A member 3 (ABCA3) gene are the most common monogenetic cause of surfactant dysfunction disorders in newborns and interstitial lung diseases in children and young adults. Although the effect of mutations resulting in truncated or incomplete proteins can be predicted, the consequences of missense variants cannot be as easily. Our aim was to investigate the intracellular handling and disturbance of the cellular surfactant system in a stable cell model with several different clinically relevant ABCA3 missense mutations...
March 5, 2018: Human Mutation
https://www.readbyqxmd.com/read/29436403/chronic-interstitial-lung-disease-in-children
#9
REVIEW
Matthias Griese
Children's interstitial lung diseases (chILD) are increasingly recognised and contain many lung developmental and genetic disorders not yet identified in adult pneumology. Worldwide, several registers have been established. The Australasian Registry Network for Orphan Lung Disease (ARNOLD) has identified problems in estimating rare disease prevalence; focusing on chILD in immunocompetent patients, a period prevalence of 1.5 cases per million children and a mortality rate of 7% were determined. The chILD-EU register highlighted the workload to be covered per patient included and provided protocols for diagnosis and initial treatment, similar to the United States chILD network...
March 31, 2018: European Respiratory Review: An Official Journal of the European Respiratory Society
https://www.readbyqxmd.com/read/29423877/nhlrc2-variants-identified-in-patients-with-fibrosis-neurodegeneration-and-cerebral-angiomatosis-finca-characterisation-of-a-novel-cerebropulmonary-disease
#10
Johanna Uusimaa, Riitta Kaarteenaho, Teija Paakkola, Hannu Tuominen, Minna K Karjalainen, Javad Nadaf, Teppo Varilo, Meri Uusi-Mäkelä, Maria Suo-Palosaari, Ilkka Pietilä, Anniina E Hiltunen, Lloyd Ruddock, Heli Alanen, Ekaterina Biterova, Ilkka Miinalainen, Annamari Salminen, Raija Soininen, Aki Manninen, Raija Sormunen, Mika Kaakinen, Reetta Vuolteenaho, Riitta Herva, Päivi Vieira, Teija Dunder, Hannaleena Kokkonen, Jukka S Moilanen, Heikki Rantala, Lawrence M Nogee, Jacek Majewski, Mika Rämet, Mikko Hallman, Reetta Hinttala
A novel multi-organ disease that is fatal in early childhood was identified in three patients from two non-consanguineous families. These children were born asymptomatic but at the age of 2 months they manifested progressive multi-organ symptoms resembling no previously known disease. The main clinical features included progressive cerebropulmonary symptoms, malabsorption, progressive growth failure, recurrent infections, chronic haemolytic anaemia and transient liver dysfunction. In the affected children, neuropathology revealed increased angiomatosis-like leptomeningeal, cortical and superficial white matter vascularisation and congestion, vacuolar degeneration and myelin loss in white matter, as well as neuronal degeneration...
February 8, 2018: Acta Neuropathologica
https://www.readbyqxmd.com/read/29325094/functional-rescue-of-misfolding-abca3-mutations-by-small-molecular-correctors
#11
Susanna Kinting, Stefanie Höppner, Ulrike Schindlbeck, Maria E Forstner, Jacqueline Harfst, Thomas Wittmann, Matthias Griese
Adenosine triphosphate (ATP)-binding cassette subfamily A member 3 (ABCA3), a phospholipid transporter in lung lamellar bodies (LBs), is essential for the assembly of pulmonary surfactant and LB biogenesis. Mutations in the ABCA3 gene are an important genetic cause for respiratory distress syndrome in neonates and interstitial lung disease in children and adults, for which there is currently no cure. The aim of this study was to prove that disease causing misfolding ABCA3 mutations can be corrected in vitro and to investigate available options for correction...
March 15, 2018: Human Molecular Genetics
https://www.readbyqxmd.com/read/29258484/lung-ultrasonography-to-diagnose-community-acquired-pneumonia-in-children
#12
Nicola Principi, Andrea Esposito, Caterina Giannitto, Susanna Esposito
BACKGROUND: Early diagnosis of community-acquired pneumonia (CAP) is essential to reduce the total burden of this disease. Traditionally, chest radiography (CR) is used to identify true CAP. However, CR is not a perfect diagnostic test for CAP. The use of lung ultrasonography (LUS) has been suggested as an alternative to overcome the problems associated with CR and increase the feasibility and accuracy of CAP diagnosis. LUS has largely been used for the diagnosis of several lung problems, including CAP, in adult patients with satisfactory results...
December 19, 2017: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/29239833/diagnostic-utility-of-3t-lung-mri-in-children-with-interstitial-lung-disease-a-prospective-pilot-study
#13
Kushaljit Singh Sodhi, Madhurima Sharma, Edward Y Lee, Akshay Kumar Saxena, Joseph L Mathew, Meenu Singh, Niranjan Khandelwal
RATIONALE AND OBJECTIVES: The objective of this study was to assess the diagnostic utility of 3-tesla (3T) magnetic resonance imaging (MRI) of lungs in the detection of interstitial lung disease (ILD) in pediatric patients. MATERIALS AND METHODS: Twelve children (mean: 8.5 years, range: 4-12 years) with ILD were consecutively enrolled in this prospective study. HRCT and 3T lung MRI were performed in all patients within 2 days of each other. The sensitivity, the specificity, the positive predictive value, and the negative predictive value of detecting lung abnormalities related to ILD with 3T lung MRI were calculated, with high-resolution computed tomography (HRCT) as a standard of reference...
March 2018: Academic Radiology
https://www.readbyqxmd.com/read/29165615/human-herpesvirus-8-in-perinatally-hiv-infected-children-with-interstitial-lung-disease
#14
Marcelo Comerlato Scotta, Alexandre Todorovic Fabro, Paula Renata Lima Machado, Simone Gusmão Ramos, Maria Célia Cervi, Benedito Antônio Lopes da Fonseca, Fabrizio Motta, Bento Vidal de Moura Negrini
Introduction: Human herpesvirus 8 (HHV-8) is associated with the pathogenesis of Kaposi Sarcoma and interstitial pneumonitis in adults. This study aims to evaluate association between HHV-8 and interstitial lung disease in HIV-infected children. Methods: HIV-infected children with interstitial pneumonitis underwent lung biopsies in a tertiary hospital and were investigated for HHV-8, Epstein-Barr virus (EBV) and cytomegalovirus (CMV) using polymerase chain reaction (PCR) and immunohistochemistry in lung tissue...
November 20, 2017: Journal of Tropical Pediatrics
https://www.readbyqxmd.com/read/29056600/international-management-platform-for-children-s-interstitial-lung-disease-child-eu
#15
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...
March 2018: Thorax
https://www.readbyqxmd.com/read/29019755/interstitial-lung-disease-in-children-made-easier%C3%A2-well-almost
#16
REVIEW
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/28946688/role-of-high-resolution-chest-computed-tomography-in-a-child-with-persistent-tachypnoea-and-intercostal-retractions-a-case-report-of-neuroendocrine-cell-hyperplasia
#17
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
#18
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/28844075/hrct-findings-of-childhood-follicular-bronchiolitis
#19
Jason P Weinman, David A Manning, Deborah R Liptzin, Amanda J Krausert, Lorna P Browne
BACKGROUND: Follicular bronchiolitis is a lymphoproliferative form of interstitial lung disease (ILD) defined by the presence of peribronchial lymphoid follicles. Follicular bronchiolitis has been associated with viral infection, autoimmune disease and immunodeficiency. The most common clinical manifestation is respiratory distress in infancy followed by a prolonged course with gradual improvement. We found no reports of systematic review of high-resolution computed tomography (HRCT) findings in pediatric follicular bronchiolitis...
December 2017: Pediatric Radiology
https://www.readbyqxmd.com/read/28743279/childhood-interstitial-lung-diseases-in-immunocompetent-children-in-australia-and-new-zealand-a-decade-s-experience
#20
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
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