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Childhood interstitial lung

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https://www.readbyqxmd.com/read/28743279/childhood-interstitial-lung-diseases-in-immunocompetent-children-in-australia-and-new-zealand-a-decade-s-experience
#1
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/28546377/respiratory-care-considerations-in-the-childhood-cancer-patient
#2
Lama Elbahlawan, K Jason Rains, Dennis C Stokes
This article reviews the common pulmonary complications seen in the pediatric oncology population and our approach to diagnosis, management, and therapy considerations in this specialized population, including patients receiving chemotherapy, radiation, and hematopoietic stem cell transplantation. Although infections cause the most significant complications in this population, non-infectious complications, including acute lung injury from chemotherapy or radiation, idiopathic interstitial pneumonia, diffuse alveolar hemorrhage, bronchiolitis obliterans, and cryptogenic organizing pneumonia, also occur commonly...
June 2017: Respiratory Care
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/28349192/-interstitial-processes-of-the-lungs-in-childhood
#4
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/28228103/disease-manifestations-and-burden-of-illness-in-patients-with-acid-sphingomyelinase-deficiency-asmd
#5
REVIEW
Margaret M McGovern, Ruzan Avetisyan, Bernd-Jan Sanson, Olivier Lidove
Acid sphingomyelinase deficiency (ASMD), a rare lysosomal storage disease, is an autosomal recessive genetic disorder caused by different SMPD1 mutations. Historically, ASMD has been classified as Niemann-Pick disease (NPD) types A (NPD A) and B (NPD B). NPD A is associated with a uniformly devastating disease course, with rapidly progressing psychomotor degeneration, leading to death typically by the age of 3 years, most often from respiratory failure. In contrast, the clinical phenotype and life expectancy of patients with NPD B may vary widely...
February 23, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28148924/mutations-in-methionyl-trna-synthetase-gene-in-a-chinese-family-with-interstitial-lung-and-liver-disease-postnatal-growth-failure-and-anemia
#6
Yu Sun, Guorui Hu, Jihang Luo, Di Fang, Yongguo Yu, Xiang Wang, Jing Chen, Wenjuan Qiu
Methionyl-tRNA synthetase (MARS) catalyzes the ligation of methionine to tRNA. Heterozygous MARS mutations have been reported to cause Charcot-Marie-Tooth disease, axonal, type 2U (CMT2U). Homozygous or compound heterozygous mutations in MARS gene would cause interstitial lung and liver disease (ILLD), a severe disease onset in infancy or early childhood. Here we report a Chinese ILLD family with two affected boys diagnosed by exome sequencing. They carry novel compound heterozygous MARS mutations (p.Asp145Asn and p...
June 2017: Journal of Human Genetics
https://www.readbyqxmd.com/read/28029320/pulmonary-infiltrates-in-a-patient-with-advanced-melanoma
#7
REVIEW
Charles A Powell
The Oncology Grand Rounds series is designed to place original reports published in the Journal into clinical context. A case presentation is followed by a description of diagnostic and management challenges, a review of the relevant literature, and a summary of the authors' suggested management approaches. The goal of this series is to help readers better understand how to apply the results of key studies, including those published in Journal of Clinical Oncology, to patients seen in their own clinical practice...
March 2017: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/27774750/pulmonary-hypertension-in-childhood-interstitial-lung-disease-a-systematic-review-of-the-literature
#8
REVIEW
Susan Bromley, David Vizcaya
Childhood interstitial lung disease (chILD) comprises a wide heterogeneous group of rare parenchymal lung disorders associated with substantial morbidity and mortality. Pulmonary hypertension is a common comorbidity in adults with interstitial lung disease (ILD) and associated with poor survival. We aimed to systematically review the literature regarding the occurrence of pulmonary hypertension (PH) in chILD, its effect on prognosis and healthcare use, and its treatment in clinical practice. Searches of PubMed and EMBASE databases (up to February 2016), and American Thoracic Society conference abstracts (2009-2015) were conducted using relevant keywords...
May 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/27719976/current-update-on-interstitial-lung-disease-of-infancy-new-classification-system-diagnostic-evaluation-imaging-algorithms-imaging-findings-and-prognosis
#9
REVIEW
Paul G Thacker, Sara O Vargas, Martha P Fishman, Alicia M Casey, Edward Y Lee
Childhood interstitial lung disease represents a rare and heterogeneous group of diseases that can result in significant morbidity and mortality, some leading to death during infancy. CT is the imaging test of choice. Although many CT findings are nonspecific and a definitive diagnosis usually cannot be reached by CT alone, the interpreting radiologist is instrumental in defining disease extent and refining the diagnosis. Chest CTs are of key importance in guiding site selection for lung biopsy and for following disease progression and response to treatment...
November 2016: Radiologic Clinics of North America
https://www.readbyqxmd.com/read/27717396/viable-phenotype-of-ilneb-syndrome-without-nephrotic-impairment-in-siblings-heterozygous-for-unreported-integrin-alpha3-mutations
#10
Elisa Adele Colombo, Luigina Spaccini, Ludovica Volpi, Gloria Negri, Davide Cittaro, Dejan Lazarevic, Salvatore Zirpoli, Andrea Farolfi, Cristina Gervasini, Maria Vittoria Cubellis, Lidia Larizza
BACKGROUND: Integrin α3 (ITGA3) gene mutations are associated with Interstitial Lung disease, Nephrotic syndrome and Epidermolysis bullosa (ILNEB syndrome). To date only six patients are reported: all carried homozygous ITGA3 mutations and presented a dramatically severe phenotype leading to death before age 2 years, from multi-organ failure due to interstitial lung disease and congenital nephrotic syndrome. The involvement of skin and cutaneous adnexa was variable with sparse hair and nail dysplasia combined or not to skin lesions ranging from skin fragility to epidermolysis bullosa-like blistering...
October 7, 2016: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/27686729/molecular-mechanisms-underlying-fibrosis-and-elastin-destruction-in-childhood-interstitial-lung-diseases
#11
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
#12
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/27554814/efficacy-of-the-janus-kinase-1-2-inhibitor-ruxolitinib-in-the-treatment-of-vasculopathy-associated-with-tmem173-activating-mutations-in-3-children
#13
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/27417306/genetic-disorders-of-surfactant-protein-dysfunction-when-to-consider-and-how-to-investigate
#14
REVIEW
Atul Gupta, Sean Lee Zheng
Genetic mutations affecting proteins required for normal surfactant protein function are a rare cause of respiratory disease. The genes identified that cause respiratory disease are surfactant protein B, surfactant protein C, ATP binding cassette number A3 and thyroid transcription factor-1. Surfactant protein dysfunction syndromes are highly variable in their onset and presentation, and are dependent on the genes involved and environmental factors. This heterogeneous group of conditions can be associated with significant morbidity and mortality...
January 2017: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/27374344/functional-characterization-of-atp-binding-cassette-transporter-a3-mutations-from-infants-with-respiratory-distress-syndrome
#15
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
Mutations in the ATP-binding cassette transporter A3 gene (ABCA3) result in severe neonatal respiratory distress syndrome and childhood interstitial lung disease. 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...
November 2016: American Journal of Respiratory Cell and Molecular Biology
https://www.readbyqxmd.com/read/27362365/a-novel-surfactant-protein-c-gene-mutation-associated-with-progressive-respiratory-failure-in-infancy
#16
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
https://www.readbyqxmd.com/read/27245831/interstitial-lung-disease-in-children-younger-than-2-years
#17
REVIEW
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
https://www.readbyqxmd.com/read/27197936/pulmonary-interstitial-glycogenosis-in-a-patient-with-trisomy-21
#18
A K Morrison, M Patel, S L Johnson, R LeGallo, W G Teague, B Vergales
Pulmonary interstitial glycogenosis is an interstitial lung disease of childhood that has been increasingly reported over the past decade. Here, we present a case of pulmonary interstitial glycogenosis associated with trisomy 21, pulmonary arterial hypertension, and congenital heart disease in a 34 week premature infant.
May 17, 2016: Journal of Neonatal-perinatal Medicine
https://www.readbyqxmd.com/read/27145498/interstitial-lung-disease-in-infancy-a-general-approach
#19
REVIEW
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
https://www.readbyqxmd.com/read/27134508/the-first-successful-heart-lung-transplant-in-a-korean-child-with-humidifier-disinfectant-associated-interstitial-lung-disease
#20
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
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