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Interstitial lung disease child

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
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
Jennifer A Wambach, Ping Yang, Daniel J Wegner, Hillary B Heins, Lyudmila N Kaliberova, Sergey A Kaliberov, David T Curiel, Frances V White, Aaron Hamvas, Brian P Hackett, F Sessions Cole
RATIONALE: Mutations in the ATP-binding cassette transporter A3 gene (ABCA3) result in severe neonatal respiratory distress syndrome (RDS) and childhood interstitial lung disease (chILD). As most ABCA3 mutations are rare or private, determination of mutation pathogenicity is often based on results from in silico prediction tools, identification in unrelated diseased individuals, statistical association studies, or expert opinion. Functional biologic studies of ABCA3 mutations are needed to confirm mutation pathogenicity and inform clinical decision making...
July 2, 2016: American Journal of Respiratory Cell and Molecular Biology
Melissa Kaori Silva Litao, Don Hayes, Saurabh Chiwane, Lawrence M Nogee, Geoffrey Kurland, Lokesh Guglani
Mutations of the Surfactant Protein C (SPC) gene (SFTPC) have been associated with childhood interstitial lung disease (chILD) with variable age of onset, severity of lung disease, and outcomes. We report a novel mutation in SFTPC [c.435G->A, p.(Gln145)] that was associated with onset of symptoms in early infancy, progressive respiratory failure with need for prolonged mechanical ventilatory support, and eventual lung transplant at 1 year of age. While the mutation was not predicted to alter the amino acid sequence of the SP-C precursor protein, analysis of SP-C transcripts demonstrated skipping of exon 4...
June 30, 2016: Pediatric Pulmonology
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
Erica J Hines, Mark Walsh, Jane E Armes, Tonia Douglas, Jasneek Chawla
Childhood Interstitial lung disease (chILD) is an umbrella term used to define a broad range of rare, diffuse pulmonary disorders with altered interstitial structure that leads to abnormal gas exchange. Presentation of chILD in infancy can be difficult to differentiate from other common causes of diffuse lung disease. This article aimed at paediatricians provides an overview of interstitial lung disease presenting in infancy and includes key clinical features, a suggested approach to investigation and a summary of management...
April 2016: Journal of Paediatrics and Child Health
Won Kyoung Jhang, Seong Jong Park, Eun Lee, Song I Yang, Soo Jong Hong, Ju-Hee Seo, Hyung-Young Kim, Jeong-Jun Park, Tae-Jin Yun, Hyeong Ryul Kim, Yong-Hee Kim, Dong Kwan Kim, Seung-Il Park, Sang-Oh Lee, Sang-Bum Hong, Tae-Sun Shim, In-Cheol Choi, Jinho Yu
From 2006 to 2011, an outbreak of a particular type of childhood interstitial lung disease occurred in Korea. The condition was intractable and progressed to severe respiratory failure, with a high mortality rate. Moreover, in several familial cases, the disease affected young women and children simultaneously. Epidemiologic, animal, and post-interventional studies identified the cause as inhalation of humidifier disinfectants. Here, we report a 4-year-old girl who suffered from severe progressive respiratory failure...
May 2016: Journal of Korean Medical Science
Chun-Ting Chen, Yu-Chen Tseng, Chih-Wei Yang, Hsuan-Hwai Lin, Peng-Jen Chen, Tien-Yu Huang, Yu-Lueng Shih, Wei-Kuo Chang, Tsai-Yuan Hsieh, Heng-Cheng Chu
The incidence of Sjögren syndrome (SS) in primary biliary cirrhosis (PBC) patients is high. The influence of SS on the clinical outcomes of PBC patients, however, remains unclear. Our study retrospectively collected data on PBC-only patients and PBC patients with concomitant SS (PBC-SS) to compare the clinical differences of long-term outcomes between them.A total of 183 patients were diagnosed with PBC from January 1999 to December 2014 at our hospital. Of these, the authors excluded patients with diabetes, hypertension, advanced liver cirrhosis at initial diagnosis of PBC (Child-Turcotte-Pugh classification score of ≥7) and other liver diseases (ie, alcoholic liver disease, alpha-antitrypsin deficiency, viral hepatitis, and primary sclerosing cholangitis), and autoimmune diseases such as systemic lupus erythematosus and rheumatoid arthritis...
January 2016: Medicine (Baltimore)
David Drummond, Caroline Thumerelle, Philippe Reix, Michael Fayon, Ralph Epaud, Annick Clement, Malika Mahloul, Delphine Habouria, Christophe Delacourt, Alice Hadchouel
INTRODUCTION: There is a lack of evidence concerning the effectiveness of immunoprophylaxis with palivizumab in children with childhood interstitial lung disease (chILD). In this retrospective study, we evaluated the effectiveness of palivizumab for decreasing the rate of RSV-related hospitalizations in children under the age of 24 months with chILD treated with corticosteroids. METHODS: A retrospective national study was conducted in France. Patients born between 2007 and 2013, diagnosed with chILD and on corticosteroid treatment were identified through the French online database for pediatric interstitial lung disease (Respirare(®) )...
July 2016: Pediatric Pulmonology
Patrizia Dello Russo, Alessandra Franzoni, Federica Baldan, Cinzia Puppin, Giovanna De Maglio, Carla Pittini, Luigi Cattarossi, Stefano Pizzolitto, Giuseppe Damante
BACKGROUND: Pulmonary capillary hemangiomatosis (PCH) is an uncommon pulmonary disorder, with variable clinical features depending on which lung structure is affected, and it is usually linked to pulmonary arterial hypertension. Congenital PCH has been very rarely described and, so far, the only causative gene identified is EIF2AK4, which encodes for a translation initiation factor. However, not all PCH cases might carry a mutation in this gene. CASE PRESENTATION: We report the clinical and cytogenetic characterization of a patient (male, newborn, first child of healthy non-consanguineous parents) died after three days of life with severe neonatal pulmonary hypertension, due to diffuse capillary hemangiomatosis diagnosed post mortem...
2015: BMC Medical Genetics
Matthias Griese
No abstract text is available yet for this article.
October 2015: Annals of the American Thoracic Society
Matthias Griese, Elke Lorenz, Meike Hengst, Andrea Schams, Traudl Wesselak, Daniela Rauch, Thomas Wittmann, Valerie Kirchberger, Amparo Escribano, Thomas Schaible, Winfried Baden, Johannes Schulze, Heiko Krude, Charalampos Aslanidis, Nicolaus Schwerk, Matthias Kappler, Dominik Hartl, Peter Lohse, Ralf Zarbock
BACKGROUND: Children's interstitial lung diseases (chILD) comprise a broad spectrum of diseases. Besides the genetically defined surfactant dysfunction disorders, most entities pathologically involve the alveolar surfactant region, possibly affecting the surfactant proteins SP-B and SP-C. Therefore, our objective was to determine the value of quantitation of SP-B and SP-C levels in bronchoalveolar lavage fluid (BALF) for the diagnosis of chILD. METHODS: Levels of SP-B and SP-C in BALF from 302 children with chILD and in controls were quantified using western blotting...
January 2016: Pediatric Research
Leland L Fan, Megan K Dishop, Csaba Galambos, Frederic B Askin, Frances V White, Claire Langston, Deborah R Liptzin, Miranda E Kroehl, Gail H Deutsch, Lisa R Young, Geoffrey Kurland, James Hagood, Sharon Dell, Bruce C Trapnell, Robin R Deterding
RATIONALE: Children's Interstitial and Diffuse Lung Disease (chILD) is a heterogeneous group of disorders that is challenging to categorize. In previous study, a classification scheme was successfully applied to children 0 to 2 years of age who underwent lung biopsies for chILD. This classification scheme has not been evaluated in children 2 to 18 years of age. OBJECTIVES: This multicenter interdisciplinary study sought to describe the spectrum of biopsy-proven chILD in North America and to apply a previously reported classification scheme in children 2 to 18 years of age...
October 2015: Annals of the American Thoracic Society
Mahesh Babu Ramamurthy, Daniel Y T Goh, Michael Teik Chung Lim
The concept of Childhood Interstitial Lung Disease (ChILD) is relatively young. There has been tremendous progress in this field in the last decade. The key advance has been the recognition of interstitial lung diseases that are often distinct and occur mainly in infants. Diagnosis is challenging because the incidence is low and no single center in the world has enough cases to promote experience and clinical skills. This has led to formation of international groups of people interested in the field and the "Children's interstitial and diffuse lung disease research network" (ChILDRN) is one such group which contributed to the progress of this field...
October 2015: Indian Journal of Pediatrics
Surafel Mulugeta, Shin-Ichi Nureki, Michael F Beers
Dating back nearly 35 years ago to the Witschi hypothesis, epithelial cell dysfunction and abnormal wound healing have reemerged as central concepts in the pathophysiology of idiopathic pulmonary fibrosis (IPF) in adults and in interstitial lung disease in children. Alveolar type 2 (AT2) cells represent a metabolically active compartment in the distal air spaces responsible for pulmonary surfactant biosynthesis and function as a progenitor population required for maintenance of alveolar integrity. Rare mutations in surfactant system components have provided new clues to understanding broader questions regarding the role of AT2 cell dysfunction in the pathophysiology of fibrotic lung diseases...
September 15, 2015: American Journal of Physiology. Lung Cellular and Molecular Physiology
Andrew Bush, Steve Cunningham, Jacques de Blic, Angelo Barbato, Annick Clement, Ralph Epaud, Meike Hengst, Nural Kiper, Andrew G Nicholson, Martin Wetzke, Deborah Snijders, Nicolaus Schwerk, Matthias Griese
Interstitial lung disease in children (chILD) is rare, and most centres will only see a few cases/year. There are numerous possible underlying diagnoses, with specific and non-specific treatment possibilities. The chILD-EU collaboration has brought together centres from across Europe to advance understanding of these considerations, and as part of this process, has created standard operating procedures and protocols for the investigation of chILD. Where established consensus documents exist already, for example, for the performance of bronchoalveolar lavage and processing of lung biopsies, these have been adopted...
November 2015: Thorax
Justine Munoz, Michel Rodière, Nadia Jeremiah, Frédéric Rieux-Laucat, Anthony Oojageer, Gillian I Rice, Flore Rozenberg, Yanick J Crow, Didier Bessis
IMPORTANCE: The type I interferonopathies comprise a recently recognized group of mendelian diseases characterized by an upregulation of type I interferon signaling. These monogenic phenotypes include classic Aicardi-Goutières syndrome and syndromic forms of systemic lupus erythematosus, including familial chilblain lupus and spondyloenchondrodysplasia. Dermatologic features provide a major diagnostic clue to this disease grouping, as exemplified by the recently described stimulator of interferon genes-associated vasculopathy with onset in infancy (SAVI) caused by gain-of-function mutations in TMEM173...
August 2015: JAMA Dermatology
Hee Mang Yoon, Eun Lee, Jin Seong Lee, Kyung-Hyun Do, Ah Young Jung, Chong Hyun Yoon, Seon-Ok Kim, Se-Jin Jang, Soo-Jong Hong, Young Ah Cho
OBJECTIVES: To report radiologic findings with histopathologic correlations of humidifier disinfectant-associated children's interstitial lung disease (HD-chILD) and to compare computed tomography (CT) findings between survivors and non-survivors. METHODS: Forty-seven children with HD-chILD (27.4 ± 12.4 months old) were categorized as survivors (n = 25) and non-survivors (n = 22). The patterns, distributions, and chronological changes in lung lesions at follow-up CT were investigated...
January 2016: European Radiology
Neil J Hime, Yvonne Zurynski, Dominic Fitzgerald, Hiran Selvadurai, Amy Phu, Marie Deverell, Elizabeth J Elliott, Adam Jaffe
OBJECTIVES: Childhood interstitial lung disease (chILD) is a group of rare chronic and complex disorders of variable pathology. There has been no systematic review of published chILD research. This study aimed to describe chILD classification systems, epidemiology, morbidity, treatments, outcomes, and the impact of chILD on families and the burden on health services. METHODS: A systematic literature search for original studies on chILD was undertaken in the major biomedical databases to the end of December 2013...
December 2015: Pediatric Pulmonology
Carlee Gilbert, Andrew Bush, Steve Cunningham
AIMS: To present experiences of UK families of children diagnosed with ILD, to inform clinical practice and service development. METHODS: Thirty seven such families completed an anonymous web-based survey between February and March 2014. RESULTS: Median time from first symptoms to diagnosis was 25 weeks. Most reported that they were happy with the overall management of their child. Areas highlighted for development included improved communication especially the need for written information; psychological support (91% reported anxiety)...
December 2015: Pediatric Pulmonology
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