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Childhood interstitial lung

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https://www.readbyqxmd.com/read/29726829/childhood-sarcoidosis-presenting-as-recurrent-facial-palsy
#1
Gouri Rao Passi, Kriti Arora, Narendra Gokhale
BACKGROUND: Recurrent facial palsy in a patient merits investigation for underlying etiology. CASE CHARACTERISTICS: 8-year-old boy with erythematous itchy skin lesion and recurrent facial palsy. OBSERVATION: He had a past history of aseptic meningitis and nephrocalcinosis. Raised angiotensin converting enzyme levels, interstitial lung disease on CT chest, and non caseating granulomas on skin biopsy clinched the diagnosis of sarcoidosis. MESSAGE: Multisystem involvement and recurrent lower motor facial nerve palsy is a clinical clue for sarcoidosis...
April 15, 2018: Indian Pediatrics
https://www.readbyqxmd.com/read/29687227/high-resolution-ct-findings-of-pulmonary-interstitial-glycogenosis
#2
Jason P Weinman, Christina J White, Deborah R Liptzin, Robin R Deterding, Csaba Galambos, Lorna P Browne
BACKGROUND: Pulmonary interstitial glycogenosis is a form of childhood interstitial lung disease characterized by the histological finding of abundant glycogen-laden mesenchymal cells within the pulmonary interstitium. Patients present in the neonatal period with disproportionate respiratory distress. Often, pulmonary interstitial glycogenosis is accompanied by alveolar simplification complicating recognition and diagnosis. Despite the recognition of pulmonary interstitial glycogenosis as a distinct entity, only a few case reports describing imaging findings are found in the literature, with no published systematic review available...
April 23, 2018: Pediatric Radiology
https://www.readbyqxmd.com/read/29627169/side-effects-of-medications-used-to-treat-childhood-interstitial-lung-disease
#3
REVIEW
Oded Breuer, André Schultz
Interstitial lung disease in children (chILD) comprises a range of different rare diseases. There is limited evidence for the treatment of chILD and no randomised clinical trials of treatment have been undertaken. Most treatments are therefore prescribed off-label based on expert opinion. The off-label nature of prescription of drugs for chILD highlights the importance of a solid understanding of the side effects to facilitate risk-benefit assessment. The European Respiratory Society chILD guidelines recommend the use of systemic glucocorticosteroids, hydroxychloroquine and azithromycin...
March 17, 2018: Paediatric Respiratory Reviews
https://www.readbyqxmd.com/read/29580292/hermansky-pudlak-syndrome-type-2-manifests-with-fibrosing-lung-disease-early-in-childhood
#4
Meike Hengst, Lutz Naehrlich, Poornima Mahavadi, Joerg Grosse-Onnebrink, Suzanne Terheggen-Lagro, Lars Høsøien Skanke, Luise A Schuch, Frank Brasch, Andreas Guenther, Simone Reu, Julia Ley-Zaporozhan, Matthias Griese
BACKGROUND: Hermansky-Pudlak syndrome (HPS), a hereditary multisystem disorder with oculocutaneous albinism, may be caused by mutations in one of at least 10 separate genes. The HPS-2 subtype is distinguished by the presence of neutropenia and knowledge of its pulmonary phenotype in children is scarce. METHODS: Six children with genetically proven HPS-2 presented to the chILD-EU register between 2009 and 2017; the data were collected systematically and imaging studies were scored blinded...
March 27, 2018: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/29569581/genetic-basis-for-childhood-interstitial-lung-disease-among-japanese-infants-and-children
#5
Itaru Hayasaka, Kazutoshi Cho, Takuma Akimoto, Masahiko Ikeda, Yutaka Uzuki, Masafumi Yamada, Koh Nakata, Itsuko Furuta, Tadashi Ariga, Hisanori Minakami
BackgroundGenetic variants responsible for childhood interstitial lung disease (chILD) have not been studied extensively in Japanese patients.MethodsThe study population consisted of 62 Japanese chILD patients. Twenty-one and four patients had pulmonary hypertension resistant to treatment (PH) and hypothyroidism, respectively. Analyses of genetic variants were performed in all 62 patients for SFTPC and ABCA3, in all 21 PH patients for FOXF1, and in a limited number of patients for NKX2.1.ResultsCausative genetic variants for chILD were identified in 11 (18%) patients: SFTPC variants in six, NKX2...
February 2018: Pediatric Research
https://www.readbyqxmd.com/read/29569218/ct-features-of-diffuse-lung-disease-in-infancy
#6
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/29436403/chronic-interstitial-lung-disease-in-children
#7
REVIEW
Matthias Griese
Children's interstitial lung diseases (chILD) are increasingly recognised and contain many lung developmental and genetic disorders not yet identified in adult pneumology. Worldwide, several registers have been established. The Australasian Registry Network for Orphan Lung Disease (ARNOLD) has identified problems in estimating rare disease prevalence; focusing on chILD in immunocompetent patients, a period prevalence of 1.5 cases per million children and a mortality rate of 7% were determined. The chILD-EU register highlighted the workload to be covered per patient included and provided protocols for diagnosis and initial treatment, similar to the United States chILD network...
March 31, 2018: European Respiratory Review: An Official Journal of the European Respiratory Society
https://www.readbyqxmd.com/read/29430789/intratumoral-delivery-of-interferon%C3%AE-secreting-mesenchymal-stromal-cells-repolarizes-tumor-associated-macrophages-and-suppresses-neuroblastoma-proliferation-in-vivo
#8
Theresa Relation, Tai Yi, Adam J Guess, Krista La Perle, Satoru Otsuru, Suheyla Hasgur, Massimo Dominici, Christopher Breuer, Edwin M Horwitz
Neuroblastoma, the most common extracranial solid tumor in childhood, remains a therapeutic challenge. However, one promising patient treatment strategy is the delivery of anti-tumor therapeutic agents via mesenchymal stromal cell (MSC) therapy. MSCs have been safely used to treat genetic bone diseases such as osteogenesis imperfecta, cardiovascular diseases, autoimmune diseases, and cancer. The pro-inflammatory cytokine interferon-gamma (IFNγ) has been shown to decrease tumor proliferation by altering the tumor microenvironment (TME)...
February 12, 2018: Stem Cells
https://www.readbyqxmd.com/read/29423877/nhlrc2-variants-identified-in-patients-with-fibrosis-neurodegeneration-and-cerebral-angiomatosis-finca-characterisation-of-a-novel-cerebropulmonary-disease
#9
Johanna Uusimaa, Riitta Kaarteenaho, Teija Paakkola, Hannu Tuominen, Minna K Karjalainen, Javad Nadaf, Teppo Varilo, Meri Uusi-Mäkelä, Maria Suo-Palosaari, Ilkka Pietilä, Anniina E Hiltunen, Lloyd Ruddock, Heli Alanen, Ekaterina Biterova, Ilkka Miinalainen, Annamari Salminen, Raija Soininen, Aki Manninen, Raija Sormunen, Mika Kaakinen, Reetta Vuolteenaho, Riitta Herva, Päivi Vieira, Teija Dunder, Hannaleena Kokkonen, Jukka S Moilanen, Heikki Rantala, Lawrence M Nogee, Jacek Majewski, Mika Rämet, Mikko Hallman, Reetta Hinttala
A novel multi-organ disease that is fatal in early childhood was identified in three patients from two non-consanguineous families. These children were born asymptomatic but at the age of 2 months they manifested progressive multi-organ symptoms resembling no previously known disease. The main clinical features included progressive cerebropulmonary symptoms, malabsorption, progressive growth failure, recurrent infections, chronic haemolytic anaemia and transient liver dysfunction. In the affected children, neuropathology revealed increased angiomatosis-like leptomeningeal, cortical and superficial white matter vascularisation and congestion, vacuolar degeneration and myelin loss in white matter, as well as neuronal degeneration...
May 2018: Acta Neuropathologica
https://www.readbyqxmd.com/read/29333842/an-adolescent-with-idiopathic-pleuroparenchymal-fibroelastosis-case-report
#10
Emine Atag, Nilay Bas Ikizoglu, Yasemin Gokdemir, Ela Erdem Eralp, Gursu Kiyan, Dilek Yilmazbayhan, Bulent Karadag
Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare disorder recently included in rare idiopathic interstitial pneumonias according to the updated American Thoracic Society/European Respiratory Society classification. IPPFE is characterized by pleural and subpleural parenchymal fibrosis causing volume loss predominantly in the upper lung lobes. Age of onset is variable, IPPFE mainly occurs in third and fourth decades. We present a 16 year old patient with a 2-year history of exertional dyspnea, nonproductive cough and weight loss...
February 1, 2018: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/29019755/interstitial-lung-disease-in-children-made-easier%C3%A2-well-almost
#11
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/28956954/hiv-related-chronic-lung-disease-in-adolescents-are-we-prepared-for-the-future
#12
Pierre Goussard, Robert P Gie
Chronic lung diseases (CLD) are the most chronic disease occurring in adolescents living with human immunodeficiency virus (ALHIV). In ALHIV who received antiretroviral therapy (ART) late in childhood, bronchiectasis, bronchiolitis obliterans and interstitial pneumonitis are common. In adolescents who received ART early in life the spectrum of CLD has changed with asthma and chronic obstructive pulmonary disease being common. Areas covered: The aim of this paper was to review CLD in ALHIV. We conducted a literature review of electronic databases focusing on CLD that were common prior to the introduction of ART (1996-2004), the present situation where ART is widely available (2005 to 2016), and articles which aided us speculating on the impact of HIV-related CLD in adolescents transitioning to adult HIV-clinics...
December 2017: Expert Review of Respiratory Medicine
https://www.readbyqxmd.com/read/28898549/filamin-a-flna-mutation-a-newcomer-to-the-childhood-interstitial-lung-disease-child-classification
#13
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/28844075/hrct-findings-of-childhood-follicular-bronchiolitis
#14
Jason P Weinman, David A Manning, Deborah R Liptzin, Amanda J Krausert, Lorna P Browne
BACKGROUND: Follicular bronchiolitis is a lymphoproliferative form of interstitial lung disease (ILD) defined by the presence of peribronchial lymphoid follicles. Follicular bronchiolitis has been associated with viral infection, autoimmune disease and immunodeficiency. The most common clinical manifestation is respiratory distress in infancy followed by a prolonged course with gradual improvement. We found no reports of systematic review of high-resolution computed tomography (HRCT) findings in pediatric follicular bronchiolitis...
December 2017: Pediatric Radiology
https://www.readbyqxmd.com/read/28743279/childhood-interstitial-lung-diseases-in-immunocompetent-children-in-australia-and-new-zealand-a-decade-s-experience
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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