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Children interstitial lung disease

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https://www.readbyqxmd.com/read/28592015/-clinical-manifestations-of-three-cases-of-surfactant-protein-c-p-v39l-mutation
#1
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
#2
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...
May 17, 2017: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/28410589/lung-involvement-in-childhood-onset-granulomatosis-with-polyangiitis
#3
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
#4
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...
March 28, 2017: Seminars in Fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/28349192/-interstitial-processes-of-the-lungs-in-childhood
#5
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...
March 27, 2017: Der Pathologe
https://www.readbyqxmd.com/read/28331982/autoantibodies-in-children-with-juvenile-dermatomyositis-a-single-centre-experience-from-north-west-india
#6
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
#7
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...
March 14, 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
#8
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
#9
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
#10
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
https://www.readbyqxmd.com/read/27906425/the-correlation-between-children-s-status-asthmatics-and-interstitial-lung-disease
#11
X-M Han, Y-Y Cheng, Y-F Gong, M-M Jiang
OBJECTIVE: Investigating the correlation between children's status asthmatics and interstitial lung disease (ILD). PATIENTS AND METHODS: We continuously selected 20 cases of children's status asthmatics combined with ILD (group A), 20 cases of pure status asthmatics (group B), 20 cases of pure ILD (group C) and 20 cases of healthy children (group D). We measured Th1/Th2 by flow cytometry as well as the level of expression of hs-CRP, IL-7 cytokines (TSLP), monocyte chemoattractant protein-1 (MCP-1) and anti-Jo-1 antibody by ELISA method...
November 2016: European Review for Medical and Pharmacological Sciences
https://www.readbyqxmd.com/read/27903688/management-of-children-with-interstitial-lung-diseases-the-difficult-issue-of-acute-exacerbations
#12
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/27753690/bronchiectasis-and-other-chronic-lung-diseases-in-adolescents-living-with-hiv
#13
Engi F Attia, Robert F Miller, Rashida A Ferrand
PURPOSE OF REVIEW: The incidence of pulmonary infections has declined dramatically with improved access to antiretroviral therapy (ART) and cotrimoxazole prophylaxis, but chronic lung disease (CLD) is an increasingly recognized but poorly understood complication in adolescents with perinatally acquired HIV. RECENT FINDINGS: There is a high prevalence of chronic respiratory symptoms, abnormal spirometry and chest radiographic abnormalities among HIV-infected adolescents in sub-Saharan Africa, wherein 90% of the world's HIV-infected children live...
February 2017: Current Opinion in Infectious Diseases
https://www.readbyqxmd.com/read/27737017/identification-of-novel-rosavirus-species-that-infects-diverse-rodent-species-and-causes-multisystemic-dissemination-in-mouse-model
#14
Susanna K P Lau, Patrick C Y Woo, Kenneth S M Li, Hao-Ji Zhang, Rachel Y Y Fan, Anna J X Zhang, Brandon C C Chan, Carol S F Lam, Cyril C Y Yip, Ming-Chi Yuen, Kwok-Hung Chan, Zhi-Wei Chen, Kwok-Yung Yuen
While novel picornaviruses are being discovered in rodents, their host range and pathogenicity are largely unknown. We identified two novel picornaviruses, rosavirus B from the street rat, Norway rat, and rosavirus C from five different wild rat species (chestnut spiny rat, greater bandicoot rat, Indochinese forest rat, roof rat and Coxing's white-bellied rat) in China. Analysis of 13 complete genome sequences showed that "Rosavirus B" and "Rosavirus C" represent two potentially novel picornavirus species infecting different rodents...
October 2016: PLoS Pathogens
https://www.readbyqxmd.com/read/27686729/molecular-mechanisms-underlying-fibrosis-and-elastin-destruction-in-childhood-interstitial-lung-diseases
#15
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...
September 21, 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
#16
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/27613991/severe-pulmonary-fibrosis-as-the-first-manifestation-of-interferonopathy-tmem173-mutation
#17
Cécile Picard, Guillaume Thouvenin, Caroline Kannengiesser, Jean-Christophe Dubus, Nadia Jeremiah, Frédéric Rieux-Laucat, Bruno Crestani, Alexandre Belot, Françoise Thivolet-Béjui, Véronique Secq, Christelle Ménard, Martine Reynaud-Gaubert, Philippe Reix
We report three cases of pulmonary disease suggesting fibrosis in two familial and one sporadic case. Pulmonary symptoms were associated with various clinical features of systemic inflammation and vasculitis involving the skin, and appeared at different ages. A strong interferon signature was found in all three cases. Disease was not responsive to corticosteroids, and lung transplantation was considered for all three subjects at an early age. One of them underwent double-lung transplantation, but she immediately experienced a primary graft dysfunction and died soon after...
September 2016: Chest
https://www.readbyqxmd.com/read/27554814/efficacy-of-the-janus-kinase-1-2-inhibitor-ruxolitinib-in-the-treatment-of-vasculopathy-associated-with-tmem173-activating-mutations-in-3-children
#18
Marie-Louise Frémond, Mathieu Paul Rodero, Nadia Jeremiah, Alexandre Belot, Eric Jeziorski, Darragh Duffy, Didier Bessis, Guilhem Cros, Gillian I Rice, Bruno Charbit, Anne Hulin, Nihel Khoudour, Consuelo Modesto Caballero, Christine Bodemer, Monique Fabre, Laureline Berteloot, Muriel Le Bourgeois, Philippe Reix, Thierry Walzer, Despina Moshous, Stéphane Blanche, Alain Fischer, Brigitte Bader-Meunier, Fréderic Rieux-Laucat, Yanick Joseph Crow, Bénédicte Neven
No abstract text is available yet for this article.
December 2016: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/27455022/-pulmonary-surfactant-homeostasis-associated-genetic-abnormalities-and-lung-diseases
#19
REVIEW
Xiaojing Jiang, Xiuzhu Sun, Weihua Du, Haisheng Hao, Xueming Zhao, Dong Wang, Huabin Zhu, Yan Liu
Pulmonary surfactant (PS) is synthesized and secreted by alveolar epithelial type II (AEII) cells, which is a complex compound formed by proteins and lipids. Surfactant participates in a range of physiological processes such as reducing the surface tension, keeping the balance of alveolar fluid, maintaining normal alveolar morphology and conducting host defense. Genetic disorders of the surfactant homeostasis genes may result in lack of surfactant or cytotoxicity, and lead to multiple lung diseases in neonates, children and adults, including neonatal respiratory distress syndrome, interstitial pneumonia, pulmonary alveolar proteinosis, and pulmonary fibrosis...
August 2016: Zhonghua Yi Xue Yi Chuan Xue za Zhi, Zhonghua Yixue Yichuanxue Zazhi, Chinese Journal of Medical Genetics
https://www.readbyqxmd.com/read/27363601/burden-of-respiratory-tract-infections-at-post-mortem-in-zambian-children
#20
Matthew Bates, Aaron Shibemba, Victor Mudenda, Charles Chimoga, John Tembo, Mwila Kabwe, Moses Chilufya, Michael Hoelscher, Markus Maeurer, Sylvester Sinyangwe, Peter Mwaba, Nathan Kapata, Alimuddin Zumla
BACKGROUND: Autopsy studies are the gold standard for determining cause-of-death and can inform on improved diagnostic strategies and algorithms to improve patient care. We conducted a cross-sectional observational autopsy study to describe the burden of respiratory tract infections in inpatient children who died at the University Teaching Hospital in Lusaka, Zambia. METHODS: Gross pathology was recorded and lung tissue was analysed by histopathology and molecular diagnostics...
July 1, 2016: BMC Medicine
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