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https://www.readbyqxmd.com/read/29766677/sleeping-child-neuroendocrine-cell-hyperplasia-of-infancy-and-polysomnography
#1
Deborah R Liptzin, Stephen M M Hawkins, Brandie D Wagner, Robin R Deterding
OBJECTIVES: Neuroendocrine cell hyperplasia of infancy (NEHI) is a children's interstitial and diffuse lung disease of unknown etiology that presents in infancy with characteristic findings of tachypnea, retractions, crackles, and hypoxemia. At the present, the mainstay of treatment is oxygen supplementation to normalize oxygen saturations and decrease work of breathing. There are characteristic pulmonary function, radiographic, and histologic findings, but polysomnography (PSG) data has not been reported...
May 15, 2018: Pediatric Pulmonology
https://www.readbyqxmd.com/read/29620595/pulmonary-function-test-abnormalities-in-children-with-inflammatory-bowel-disease-is-it-common
#2
Doaa Mohamed El Amrousy, Samir Hassan, Heba El-Ashry, Mohamed Yousef, Ragia Sharshar
OBJECTIVE: The aim of the study was to evaluate the frequency and type of pulmonary dysfunction in newly diagnosed children with inflammatory bowel disease (IBD) and the correlation between pulmonary function tests (PFTs) and IBD activity. METHODS: It is an observational case-control study. One hundred newly diagnosed children with IBD were enrolled as the patient group, which was subdivided into 52 with Crohn disease (CD) and 48 with ulcerative colitis (UC). Fifty healthy children matched for age, sex, height, and body mass index (BMI) served as the control group...
April 3, 2018: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29614564/-the-classification-of-congenital-respiratory-diseases-in-children
#3
(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/29569218/ct-features-of-diffuse-lung-disease-in-infancy
#4
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/29516392/diffuse-lung-disease-associated-with-neurofibromatosis-type-1-can-also-affect-children
#5
Paolo Spinnato, Giancarlo Facchini, Alberto Bazzocchi, Ugo Albisinni
No abstract text is available yet for this article.
April 2018: World Journal of Pediatrics: WJP
https://www.readbyqxmd.com/read/29198536/infants-with-atypical-presentations-of-alveolar-capillary-dysplasia-with-misalignment-of-the-pulmonary-veins-who-underwent-bilateral-lung-transplantation
#6
Christopher T Towe, Frances V White, R Mark Grady, Stuart C Sweet, Pirooz Eghtesady, Daniel J Wegner, Partha Sen, Przemyslaw Szafranski, Pawel Stankiewicz, Aaron Hamvas, F Sessions Cole, Jennifer A Wambach
OBJECTIVE: To describe disease course, histopathology, and outcomes for infants with atypical presentations of alveolar capillary dysplasia with misalignment of the pulmonary veins (ACDMPV) who underwent bilateral lung transplantation. STUDY DESIGN: We reviewed clinical history, diagnostic studies, explant histology, genetic sequence results, and post-transplant course for 6 infants with atypical ACDMPV who underwent bilateral lung transplantation at St. Louis Children's Hospital...
March 2018: Journal of Pediatrics
https://www.readbyqxmd.com/read/29141316/-a-clinical-analysis-of-micafungin-treatment-of-pulmonary-invasive-fungal-infection-in-pediatric-patients-with-acute-leukemia-or-post-hematopoietic-stem-cells-transplantation
#7
K Huang, K Y Qiu, L L Deng, J P Fang, Y Li, H X Guo, D H Zhou
Objective: To investigate the efficacy and safety of micafungin (MCF) for pulmonary invasive fungal disease (PIFD) in pediatric patients with acute leukemia or post hematopoietic stem cells transplantation. Method: Twenty-five neutropenic PIFD children with acute leukemia or post hematopoietic stem cells transplantation in Sun Yat-sen Memorial Hospital of Sun Yat-sen University were selected from January 2012 to June 2015, including 12 males and 13 females, age range 2-15 (average 6.2±2.0) years. There were 12 cases of acute leukemia (AL) after chemotherapy, 4 cases of acute leukemia (AL) after allogeneic hematopoietic stem cell transplantation (allo-HSCT) and 9 cases of β-thalassemia major after allo-HSCT...
November 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/29141314/-a-novel-compound-heterozygous-mutation-in-abca3-gene-in-a-child-with-diffuse-parenchymal-lung-disease
#8
Y M Bao, X L Liu, X L Liu, J H Chen, Y J Zheng
Objective: To summarize the clinical characteristics of the diffuse parenchymal lung diseases in a child caused by a novel compound heterozygous ABCA3 mutation and explore the association between the phenotype and ABCA3 mutation. Method: The clinical material of a patient diagnosed with diffuse parenchymal lung disease with ABCA3 mutation in December 2016 in Shenzhen Children's Hospital was analyzed. The information about ABCA3 gene mutation updated before April, 2017 was searched and collected from the gene databases (including 1000Genomes, HGMD, EXAC) and the literatures (including Wanfang Chinese database and Pubmed)...
November 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/29019755/interstitial-lung-disease-in-children-made-easier%C3%A2-well-almost
#9
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
#10
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
#11
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/28831595/juvenile-systemic-sclerosis-experience-from-a-tertiary-care-center-from-india
#12
Narendra Kumar Bagri, Dinesh Raj, Jasmeet Kaur, Harish Punia, Isha Saini, Rakesh Lodha, S K Kabra
Juvenile systemic sclerosis (JSSc) is a rare disorder with paucity of information on its treatment and longterm  outcome. Herein, we are sharing our experience with this rare entity. Case records of children, diagnosed to have systemic sclerosis attending Pediatric Rheumatology Clinic at All India Institute of Medical Sciences, New Delhi from January 1998 to June 2016 were reviewed. The demographic, clinical, laboratory, treatment and outcome details were recorded. Disease outcome was classified arbitrarily as controlled, partly controlled or non-responsive/progressive based on: (A) ability to perform activities of daily life (ADL) and (B) presence or absence of musculoskeletal symptoms, skin changes (ulceration/progressive digital pitting/gangrene), and visceral organ involvement (dyspahgia, cardiopulmonary symptoms)...
October 2017: Rheumatology International
https://www.readbyqxmd.com/read/28828139/an-unusual-radiologic-manifestation-of-hypersensitivity-pneumonia
#13
Seema Toso, Meryle Laurent, Isabelle Ruchonnet-Metrailler, Jean-Claude Pache, Sylviane Hanquinet
Hypersensitivity pneumonia is clinically suspected and can be characterized on computed tomography by its pattern of diffuse lung disease, in children, as in adults. However, identifying the diagnosis is not always as simple. We report an organizing pneumonia pattern of hypersensitivity pneumonia that can be seen in adult patients, but has not been reported in the pediatric population.
September 2017: Radiology Case Reports
https://www.readbyqxmd.com/read/28744781/lung-function-in-pregnancy-in-langerhans-cell-histiocytosis
#14
Elżbieta Radzikowska, Elżbieta Wiatr, Monika Franczuk, Iwona Bestry, Kazimierz Roszkowski-Śliż
Pulmonary Langerhans cell histiocytosis (LCH) is a rare disease, affecting usually young people. The course of the disease is variable. In some pulmonary LCH patients a severe lung destruction and progression in spite of chemotherapy is observed, but in others just a cessation of smoking induces a regression of the disease. In the present study we seek to determine the influence of pregnancy on pulmonary function in LCH patients, an unchartered area of research. We addressed the issue by investigating eight pregnant women out of the 45 women hospitalized with the diagnosis of pulmonary LCH in the period from 2000 to 2015...
2018: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28719106/altered-pulmonary-gas-transfer-capacity-and-capillary-blood-volume-in-pediatric-crohn-s-disease
#15
Marie Verstraete, Marie-Luce Choukroun, Valerie Siao-Him Fa, Michael Fayon, Laurent Rebouissoux, Raphael Enaud, Thierry Lamireau
OBJECTIVES: To describe diffusing capacity for carbon monoxide (DLCO) and its components, that is, membrane diffusing capacity (DmCO) and pulmonary capillary blood volume (Vc) in children with Crohn's disease (CD), and to investigate the correlation between these parameters and disease activity. WORKING HYPOTHESIS: The most common lung function abnormalities are a reduced pulmonary DLCO and small airways disorders which are in many instances, clinically silent. No valid explanations have been proposed regarding the modifications in gas transfer capacity in active CD...
August 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28665296/comparison-of-feasibility-and-estimates-of-central-and-peripheral-nitric-oxide-parameters-by-different-mathematical-models
#16
COMPARATIVE STUDY
Tuomas Karvonen, Hannu Kankaanranta, Seppo Saarelainen, Eeva Moilanen, Lauri Lehtimäki
INTRODUCTION: Assessment of the central and peripheral nitric oxide (NO) dynamics of the lung provides information on the severity and anatomical site of pulmonary inflammation. Several mathematical methods for calculating alveolar and bronchial NO parameters have been introduced. Our aim was to compare these methods. METHODS: The study included 69 healthy adults, 66 healthy children, 73 asbestos-exposed subjects and 72 subjects with chronic obstructive pulmonary disease (COPD)...
September 13, 2017: Journal of Breath Research
https://www.readbyqxmd.com/read/28655531/results-of-the-third-aieop-cooperative-protocol-on-wilms-tumor-tw2003-and-related-considerations
#17
Filippo Spreafico, Davide Biasoni, Salvatore Lo Vullo, Lorenza Gandola, Paolo D'Angelo, Monica Terenziani, Maurizio Bianchi, Massimo Provenzi, Paolo Indolfi, Andrea Pession, Marilina Nantron, Andrea Di Cataldo, Carlo Morosi, Daniela Perotti, Serena Catania, Franca Fossati Bellani, Paola Collini
PURPOSE: TW2003, the third Italian prospective study on Wilms tumor, aimed to improve survival in patients with stage III-IV tumors, de-escalate therapy for stage I-II nonanaplastic tumors, refine the risk stratification of therapy, and develop a national infrastructure for biobanking and central pathology review. MATERIALS AND METHODS: TW2003 recruited children 18 years old or younger with primary intrarenal tumors. Local physicians chose nephrectomy with or without preoperative chemotherapy as the initial treatment based on the risk of unsafe and/or incomplete immediate surgery...
November 2017: Journal of Urology
https://www.readbyqxmd.com/read/28649564/primary-ciliary-dyskinesia-an-update-on-clinical-aspects-genetics-diagnosis-and-future-treatment-strategies
#18
REVIEW
Virginia Mirra, Claudius Werner, Francesca Santamaria
Primary ciliary dyskinesia (PCD) is an orphan disease (MIM 244400), autosomal recessive inherited, characterized by motile ciliary dysfunction. The estimated prevalence of PCD is 1:10,000 to 1:20,000 live-born children, but true prevalence could be even higher. PCD is characterized by chronic upper and lower respiratory tract disease, infertility/ectopic pregnancy, and situs anomalies, that occur in ≈50% of PCD patients (Kartagener syndrome), and these may be associated with congenital heart abnormalities...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28592015/-clinical-manifestations-of-three-cases-of-surfactant-protein-c-p-v39l-mutation
#19
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/28544706/exhaled-nitric-oxide-pulmonary-function-and-disease-activity-in-children-with-systemic-lupus-erythematosus
#20
Jitladda Deerojanawong, Pitchaya Leartphichalak, Ankanee Chanakul, Suchada Sritippayawan, Rujipat Samransamruajkit
AIM: To determine the association among fractional exhaled nitric oxide (FENO), pulmonary function, and disease activity in children with systemic lupus erythematosus (SLE). METHODS: Children aged 7-18 years, diagnosed with SLE under the criteria of the American Rheumatism Association (revised 2012), were enrolled. All eligible participants had disease activity, FENO, and pulmonary function evaluated and re-evaluated at 6-month follow-up. RESULTS: Twenty-four children (95...
October 2017: Pediatric Pulmonology
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