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Children diffuse lung disease

Joanna S Cohen, Naomi Hughes, Sonny Tat, James M Chamberlain, Stephen J Teach, Keith Boniface
OBJECTIVES: Recent literature suggests that bedside lung ultrasound may have a role in the evaluation of infants with bronchiolitis. B lines, which are multiple and diffuse vertical artifacts spreading from the lung pleural interface to the edge of the ultrasound screen, have been associated with thickened interlobular septa, extravascular lung water, and diffuse parenchymal disease. The aims of this study were (1) to describe the prevalence of B lines in children younger than 24 months presenting to the emergency department with wheezing, (2) to determine the interrater reliability of lung ultrasound findings in this setting, and (3) to determine the association of B lines with atopy and other clinical findings...
September 23, 2016: Pediatric Emergency Care
Babak Pourakbari, Shima Mahmoudi, Amir Hossein Jafari, Abbas Bahador, Sepideh Keshavarz Valian, Reihaneh Hosseinpour Sadeghi, Setareh Mamishi
INTRODUCTION: Diffuse lung diseases (DLD) in children involve a group of heterogeneous, rare disorders. In spite of the low diagnostic yield in pediatric DLD, bronchoalveolar lavage (BAL) can be used to diagnose specific disorders. There are few studies about microbial and cellular profiles of BAL samples in these patients. This study was conducted to evaluate the clinical, cytological and microbiological evaluation of BAL in children with DLD. METHODS: The clinical, cytological and microbiological profiles of BAL samples of all patients with DLD who underwent the fiberoptic bronchoscopy (FOB) at Children's Medical Center, an Iranian referral pediatrics Hospital during a year were evaluated...
September 22, 2016: Microbial Pathogenesis
Paolo Tomà, Laura Lancella, Laura Menchini, Roberta Lombardi, Aurelio Secinaro, Alberto Villani
The incidence of tuberculosis is increasing in the developed world and children in particular represent a high-risk group for developing the disease. The aim of this review is to analyse the spectrum of radiological signs as reported in the recent literature, in light of our series over a 15-year period, to pinpoint the most common radiological patterns in a developed country and to determine the role played by the different chest imaging techniques in diagnosis improvement. Lung TB was present in 217 out of 255 patients (85 %): 146 patients were under 5 years of age (76 under 2 years) and 71 over 5 years (41 over 10 years)...
September 20, 2016: La Radiologia Medica
Carlo Caffarelli, Francesca Santamaria, Dora Di Mauro, Carla Mastrorilli, Virginia Mirra, Sergio Bernasconi
This review focuses key advances in different pediatric fields that were published in Italian Journal of Pediatrics and in international journals in 2015. Weaning studies continue to show promise for preventing food allergy. New diagnostic tools are available for identifying the allergic origin of allergic-like symptoms. Advances have been reported in obesity, short stature and autoimmune endocrine disorders. New molecules are offered to reduce weight gain and insulin-resistance in obese children. Regional investigations may provide suggestions for preventing short stature...
August 27, 2016: Italian Journal of Pediatrics
Laurie L Carr, Jeffrey A Kern, Gail H Deutsch
Although incidental reactive pulmonary neuroendocrine cell hyperplasia (PNECH) is seen on biopsy specimens in adults with chronic lung disease, disorders characterized by marked PNECH are rare. Primary hyperplasia of neuroendocrine cells in the lung and obstructive lung disease related to remodeling or physiologic constriction of small airways define diffuse idiopathic neuroendocrine cell hyperplasia (DIPNECH) in the adult and neuroendocrine cell hyperplasia of infancy (NEHI) in children. DIPENCH and NEHI share a similar physiology, typical imaging appearance, and increased neuroendocrine cells on biopsy...
September 2016: Clinics in Chest Medicine
Izabela Grabska-Kobylecka, Dariusz Nowak, Anna Wlodarczyk, Piotr Bialasiewicz
Henoch-Schonlein purpura (HSP) is a generalized form of IgA-mediated vasculitis that usually spares pulmonary circulation. Nevertheless, it is conceivable that subclinical changes at the HSP onset may lead to lung impairment in the long term. Therefore, we decided to follow a group of HSP patients for 4 years to monitor changes in pulmonary function. A group of 11 children and adolescents diagnosed with HSP without apparent pulmonary involvement was subjected to pulmonary function tests (PFTs), i.e., spirometry, body plethysmography, and diffusing capacity for CO (DLCO); these tests were repeated after 48 months...
November 2016: Clinical Rheumatology
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
Sylvia Nyilas, Florian Singer, Nitin Kumar, Sophie Yammine, Delphine Meier-Girard, Cordula Koerner-Rettberg, Carmen Casaulta, Urs Frey, Philipp Latzin
Inert tracer gas washout (IGW) measurements detect increased ventilation inhomogeneity (VI) in chronic lung diseases. Their suitability for different diseases, such as cystic fibrosis (CF) and primary ciliary dyskinesia (PCD), has already been shown. However, it is still unclear if physiological phenotypes based on different IGW variables can be defined independently of underlying disease. Eighty school-age children, 20 with CF, 20 with PCD, 20 former preterm children, and 20 healthy children, performed nitrogen multiple-breath washout, double-tracer gas (DTG) single-breath washout, and spirometry...
July 1, 2016: Journal of Applied Physiology
Manuela Dicembrino, Dora Haag, Mariana Álvarez, Agustina Díaz Cazaux, Claudio Castaños
Pulmonary vascular disease in children is multifactorial and heterogeneous. While it shares some features with pulmonary hypertension in adults, there are differences in the associated comorbidities and conditions, the coexistence of genetic or developmental diseases. Interstitial lung diseases may be responsible for this entity. One is alveolar capillary dysplasia with misalignment of pulmonary veins, a rare pathology but with a mortality rate of 100%, characterized by a failure in the formation of lung tissue that eventually results in impaired gas diffusion...
June 1, 2016: Archivos Argentinos de Pediatría
Danveer Bhadu, Puneet Kumar, Kiran Preet Malhotra, Aarti Sharma, Meha Sharma, Durgesh Srivastava
Microscopic polyangiitis is a small vessel vasculitis, associated with myeloperoxidaseantineutrophil cytoplasmic antibody. It rarely occurs in children. Central nervous system involvement in pediatric microscopic polyangiitis is not a well known entity with perhaps only five cases till date. We hereby present a 14-year-old girl with arthralgia, seizure, leukocytoclastic vasculitis, interstitial lung disease secondary to recurrent pulmonary hemorrhage, pauci-immune glomerulonephritis and high titers of MPO-ANCA, hence diagnostic of microscopic polyangiitis...
February 18, 2016: Acta Reumatológica Portuguesa
Sophie Yammine, Sylvia Nyilas, Carmen Casaulta, Susanne Schibli, Philipp Latzin, Christiane Sokollik
BACKGROUND: Extraintestinal manifestations are common among patients with inflammatory bowel disease (IBD), whereas pulmonary involvement is considered rare. However, chronic lung diseases begin with subclinical changes of the small airways and often originate in childhood. Pulmonary involvement, particularly of the small airways, can be assessed using novel inert gas washout tests. METHODS: In this prospective, single-center study, 30 children and adolescents (mean age, 14 years; SD, ±2...
August 2016: Inflammatory Bowel Diseases
Megan K Dishop
The differential diagnosis of diffuse lung disease in children differs considerably from adults, and analysis of pediatric lung biopsies may prove challenging for pathologists with more extensive exposure to adult lung biopsies. Biopsy diagnosis of pediatric lung disease continues to evolve as new pathologic entities are recognized and new genetic determinants of disease are discovered. This article describes the clinical characteristics, pathologic features, and differential diagnosis of challenging and recently described entities in pediatric lung disease...
September 2010: Surgical Pathology Clinics
Eveliina Ronkainen, Teija Dunder, Tuula Kaukola, Riitta Marttila, Mikko Hallman
OBJECTIVE: Children born preterm have lower lung function compared with term-born children. Intrauterine growth restriction (IUGR) may predispose individuals to chronic obstructive pulmonary disease. The purpose of this observational study was to investigate the role of IUGR as predictor of lung function at school age in children born very preterm. We further studied the difference in lung function between term-born and preterm-born children with distinct morbidities. DESIGN: Preterm-born children and age-matched and sex-matched term-born comparison groups (88 of each) were studied at the mean age of 11 years...
September 2016: Archives of Disease in Childhood. Fetal and Neonatal Edition
Manju L Prasad, Monika Vyas, Matthew J Horne, Renu K Virk, Raffaella Morotti, Zongzhi Liu, Giovanni Tallini, Marina N Nikiforova, Emily R Christison-Lagay, Robert Udelsman, Catherine A Dinauer, Yuri E Nikiforov
BACKGROUND: An increase in thyroid cancers, predominantly papillary thyroid carcinoma (PTC), has been recently reported in children. METHODS: The histopathology of 28 consecutive PTCs from the northeast United States was reviewed. None of the patients (ages 6-18 years; 20 females, 8 males) had significant exposure to radiation. Nucleic acid from tumors was tested for genetic abnormalities (n = 27). Negative results were reevaluated by targeted next-generation sequencing...
April 1, 2016: Cancer
Yagmur Sisman, Frederik Buchvald, Anne Katrine Blyme, Jann Mortensen, Kim Gjerum Nielsen
BACKGROUND: Hypersensitivity pneumonitis (HP) is an immune-mediated diffuse lung disease. Significant improvement in lung function and diffusing capacity after treatment was previously demonstrated, while long-term data focusing specifically on peripheral airway impairment and peak oxygen uptake (fitness) are lacking. Hence, the aim of this study was to conduct a comprehensive study to determine the stability of pulmonary function and fitness in patients previously diagnosed with HP. METHODS: We performed a cross-sectional follow-up study with inclusion of longitudinal data if available in patients previously diagnosed with biopsy and high-resolution computed tomography-verified HP during childhood...
August 2016: Pediatric Pulmonology
Silvia Montella, Timothy J Vece, Claire Langston, Paola Carrera, Lawrence M Nogee, Aaron Hamvas, Angelo Manna, Mariarosaria Cervasio, Mara Cervasio, Francesca Santamaria
BACKGROUND: Surfactant metabolism disorders may result in diffuse lung disease in children. CASE PRESENTATION: We report a 3-years-old boy with dry cough, progressive hypoxemia, dyspnea and bilateral ground glass opacities at chest high-resolution computed tomography (HRCT) who had no variants in genes encoding surfactant proteins or transcription factors. Lung histology strongly suggested an abnormality of surfactant protein. A 7-month course of pulse intravenous high-dose methylprednisolone plus oral hydroxychloroquine and azithromycin led to gradual weaning from oxygen and oral steroids, and to improvement of cough and dyspnea...
2015: Italian Journal of Pediatrics
Alan Lunt, Emily McGhee, Polly Robinson, David Rees, Susan Height, Anne Greenough
Lung function abnormalities occur in children with sickle cell disease (SCD) and may be associated with elevated pulmonary blood volume. To investigate that association, we determined whether blood transfusion in SCD children acutely increased pulmonary capillary blood volume (PCBV) and increased respiratory system resistance (Rrs5). Measurements of Rrs5 and spirometry were made before and after blood transfusion in 18 children, median age 14.2 (6.6-18.5) years. Diffusing capacity for carbon monoxide and nitric oxide were assessed to calculate the PCBV...
February 1, 2016: Respiratory Physiology & Neurobiology
Thomas Semple, Catherine M Owens
Diffuse interstitial lung disease in children differs markedly from interstitial lung disease in adults and is a distinct entity. The childhood interstitial lung disease (ChILD) classification, devised in 2010 separates conditions into those occurring in infancy, and those not specific to infants, the later group containing many conditions related to systemic diseases (including connective tissue diseases and depositional/storage disorders), and conditions occurring in immunocompromised children. In this article, we briefly review normal lung growth and development...
May 2016: La Radiologia Medica
Timothy J Vece, Lisa R Young
Diffuse lung diseases in children, also called children's interstitial lung disease, are a diverse group of rare disorders that cause disturbances of gas exchange in the lungs. Although individually rare, there are many different forms of diffuse lung disease in children, and collectively these disorders are associated with significant morbidity and mortality, as well as health-care resource utilization. Over the past several years, there have been many significant advances in the field, including genetic discoveries and the development of clinical practice guidelines...
March 2016: Chest
Robin R Deterding
Children's interstitial and diffuse lung disease (chILD) is a term that encompasses a large and diverse group of rare pediatric diseases and disorders. Significant progress has been made over the last 2 decades in classification, clinical care, research, and organizational structure to enhance the care of children with these high-morbidity and -mortality diseases. New diseases have been defined clinically and genetically, classification systems developed and applied, organizations formed such as the chILD Research Network (chILDRN) and chILD Foundation, and basic and translational science expanded to focus on chILD diseases...
October 2015: Annals of the American Thoracic Society
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