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Children's interstitial lung disease

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https://www.readbyqxmd.com/read/29239833/diagnostic-utility-of-3t-lung-mri-in-children-with-interstitial-lung-disease-a-prospective-pilot-study
#1
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...
November 24, 2017: Academic Radiology
https://www.readbyqxmd.com/read/29165615/human-herpesvirus-8-in-perinatally-hiv-infected-children-with-interstitial-lung-disease
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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/28672090/efficacy-of-mycophenolate-on-lung-disease-and-autoimmunity-in-children-with-immunodeficiency
#9
Giorgia Bucciol, Angelamaria Petrone, Maria Caterina Putti
The autoimmune manifestations of primary immunodeficiencies, such as autoimmune lymphoproliferative syndrome (ALPS) and common variable immunodeficiency (CVID), often constitute a great therapeutic challenge and have a significant impact on patients' morbidity and mortality. The most common autoimmune presentations are autoimmune cytopenias, but organ-related autoimmunity is also frequently observed. From a pulmonology perspective, granulomatous/lymphocytic interstitial lung disease (GLILD) is a severe immunological complication which significantly worsens the clinical outcome of these patients and for which there are currently few guidelines or protocols for treatment...
October 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28642621/variants-of-the-abca3-gene-might-contribute-to-susceptibility-to-interstitial-lung-diseases-in-the-chinese-population
#10
Wei Zhou, Yi Zhuang, Jiapeng Sun, Xiaofen Wang, Qingya Zhao, Lizhi Xu, Yaping Wang
ATP-binding cassette A3 (ABCA3) is a phospholipid carrier that is mainly expressed in the alveolar epithelium. Biallelic mutations of ABCA3 has been associated with fatal respiratory distress syndrome and interstitial lung disease (ILD) in children. However, whether variations in ABCA3 have a role in the development of adult ILD, including idiopathic pulmonary fibrosis (IPF), remains to be addressed. In this study, we screened for germline variants of ABCA3 by exons-sequencing in 30 patients with sporadic IPF and in 30 matched healthy controls...
June 22, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28592015/-clinical-manifestations-of-three-cases-of-surfactant-protein-c-p-v39l-mutation
#11
J H Chen, D Y Zhao, S H An, Y J Zheng, H P Wang, H L Ma
Objective: To investigate the clinical manifestations of surfactant protein C gene (SFTPC) exon-2 c. 115G>G/T (p.V39L). Method: Patients were screened for the entire coding sequence of SFTPC. Three cases from three children's hospital with mutation in p. V39L were reported. Result: All the three cases were females. The age of onset ranged from 2 months to 7 years. Two cases had recurrent lower respiratory tract infection and failed to thrive. One had chronic anoxia and clubbing fingers. Chest computed tomography (CT) showed diffused ground glass pattern, localized emphysema and intralobular septal thickening...
June 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/28512724/congenital-pulmonary-alveolar-proteinosis-from-birth-to-ten-years-of-age
#12
Sandra Alavuk Kundović, Ljiljana Popović
Pulmonary alveolar proteinosis is a rare lung disease in which lipoproteinaceous material accumulates within the alveoli, interfering with gas exchange. The disease is classified into congenital, secondary, and acquired. The congenital form includes inborn errors of surfactant metabolism, lysinuric protein intolerance and mutations in the components of granulocyte-macrophage colony-stimulating factor receptor. The main symptoms are non-specific. The radiologic appearance of pulmonary alveolar proteinosis is bilateral, symmetric and perihilar airspace consolidation...
September 2017: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/28410589/lung-involvement-in-childhood-onset-granulomatosis-with-polyangiitis
#13
REVIEW
Giovanni Filocamo, Sofia Torreggiani, Carlo Agostoni, Susanna Esposito
Granulomatosis with polyangiitis is an ANCA-associated systemic vasculitis with a low incidence in the pediatric population. Lung involvement is a common manifestation in children affected by granulomatosis with polyangiitis, both at disease's onset and during flares. Its severity is variable, ranging from asymptomatic pulmonary lesions to dramatic life-threatening clinical presentations such as diffuse alveolar haemorrhage. Several radiologic findings have been described, but the most frequent abnormalities detected are nodular lesions and fixed infiltrates...
April 14, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28363760/interstitial-lung-disease-in-newborns
#14
REVIEW
Lawrence M Nogee
The term 'interstitial lung disease' (ILD) refers to a group of disorders involving both the airspaces and tissue compartments of the lung, and these disorders are more accurately termed diffuse lung diseases. Although rare, they are associated with significant morbidity and mortality, with the prognosis depending upon the specific diagnosis. The major categories of ILD in children that present in the neonatal period include developmental disorders, growth disorders, surfactant dysfunction disorders, and specific conditions of unknown etiology unique to infancy...
August 2017: Seminars in Fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/28349192/-interstitial-processes-of-the-lungs-in-childhood
#15
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/28331982/autoantibodies-in-children-with-juvenile-dermatomyositis-a-single-centre-experience-from-north-west-india
#16
Altaf Hussain, Amit Rawat, Ankur Kumar Jindal, Anju Gupta, Surjit Singh
The objective of this study is to determine autoantibody profile in children with juvenile dermatomyositis (JDM). Children who were diagnosed with JDM (either recently diagnosed during the study period or follow-up patients) were included in the study. Autoantibodies were detected with commercially available Immunodot kit. Thirty patients were included in the study. Nine out of thirty patients (30%) were positive for one of the 12 autoantibodies tested. Anti-SRP antibody was most common antibody detected in 3 patients followed by anti-MDA-5 antibody in 2 patients; while anti-Jo1 antibody, anti-TIF1-γ antibody, anti-Mi-2 antibody, and anti-PM-Scl antibody were positive in 1 patient each...
May 2017: Rheumatology International
https://www.readbyqxmd.com/read/28289906/pediatric-chest-ct-at-chest-radiograph-doses-when-is-the-ultralow-dose-chest-ct-clinically-appropriate
#17
Javier E Villanueva-Meyer, David M Naeger, Jesse L Courtier, Michael D Hope, Jack W Lambert, John D MacKenzie, Andrew S Phelps
PURPOSE: Computed tomography (CT) use in emergency departments represents a significant contribution to pediatric patients' exposure to ionizing radiation. Here, we evaluate whether ultralow-dose chest CT can be diagnostically adequate for other diagnoses and whether model-based iterative reconstruction (MBIR) can improve diagnostic adequacy compared to adaptive statistical iterative reconstruction (ASIR) at ultralow doses. METHODS: Twenty children underwent chest CTs: 10 standard-dose reconstructed with ASIR and 10 ultralow-dose reconstructed with ASIR and MBIR...
August 2017: Emergency Radiology
https://www.readbyqxmd.com/read/28275537/a-histologically-proven-case-of-lymphocytic-interstitial-pneumonia-in-a-hiv-infected-adult-with-an-undetectable-viral-load
#18
Sarah Assaf, Pujan Patel, David Stoeckel
Lymphocytic interstitial pneumonia (LIP) is on the spectrum of lymphoproliferative diseases that can affect the lungs. Although common in human immunodeficiency virus (HIV) infected children, it is rarely reported in adults. A 51-year-old HIV infected female patient presented with worsening dyspnea over five months. She had radiological findings of bilateral lung nodular infiltrates. Her CD4 count was 835 cells/uL and her HIV viral load was undetectable. Bronchoalveolar lavage did not yield any infectious pathogen...
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/28045846/lung-ultrasound-as-first-line-examination-for-the-diagnosis-of-community-acquired-pneumonia-in-children
#19
Contantinia Boursiani, Maria Tsolia, Chrysoula Koumanidou, Aikaterini Malagari, Marina Vakaki, Georgios Karapostolakis, Argyro Mazioti, Efthymia Alexopoulou
OBJECTIVES: The diagnosis of pediatric community-acquired pneumonia (CAP) is based on clinical criteria. Even though chest x-ray (CXR) is only recommended in severe cases, it is often requested from physicians in mild cases, thus increasing radiation exposure. Lung ultrasound (LUS) is not included in the diagnostic workup. The objective of this study was to evaluate the diagnostic performance of LUS against CXR. METHODS: Children who presented to the emergency department with clinical signs suggesting CAP and had already been evaluated with a CXR were included in the study...
January 2017: Pediatric Emergency Care
https://www.readbyqxmd.com/read/27983684/lung-involvement-in-children-with-hereditary-autoinflammatory-disorders
#20
REVIEW
Giusyda Tarantino, Susanna Esposito, Laura Andreozzi, Benedetta Bracci, Francesca D'Errico, Donato Rigante
Short-lived systemic inflammatory reactions arising from disrupted rules in the innate immune system are the operating platforms of hereditary autoinflammatory disorders (HAIDs). Multiple organs may be involved and aseptic inflammation leading to disease-specific phenotypes defines most HAIDs. Lungs are infrequently involved in children with HAIDs: the most common pulmonary manifestation is pleuritis in familial Mediterranean fever (FMF) and tumor necrosis factor receptor-associated periodic syndrome (TRAPS), respectively caused by mutations in the MEFV and TNFRSF1A genes, while interstitial lung disease can be observed in STING-associated vasculopathy with onset in infancy (SAVI), caused by mutations in the TMEM173 gene...
December 15, 2016: International Journal of Molecular Sciences
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