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Children's interstitial lung disease

Engi F Attia, Robert F Miller, Rashida A Ferrand
PURPOSE OF REVIEW: The incidence of pulmonary infections has declined dramatically with improved access to antiretroviral therapy (ART) and cotrimoxazole prophylaxis, but chronic lung disease (CLD) is an increasingly recognized but poorly understood complication in adolescents with perinatally acquired HIV. RECENT FINDINGS: There is a high prevalence of chronic respiratory symptoms, abnormal spirometry and chest radiographic abnormalities among HIV-infected adolescents in sub-Saharan Africa, wherein 90% of the world's HIV-infected children live...
October 7, 2016: Current Opinion in Infectious Diseases
Susanna K P Lau, Patrick C Y Woo, Kenneth S M Li, Hao-Ji Zhang, Rachel Y Y Fan, Anna J X Zhang, Brandon C C Chan, Carol S F Lam, Cyril C Y Yip, Ming-Chi Yuen, Kwok-Hung Chan, Zhi-Wei Chen, Kwok-Yung Yuen
While novel picornaviruses are being discovered in rodents, their host range and pathogenicity are largely unknown. We identified two novel picornaviruses, rosavirus B from the street rat, Norway rat, and rosavirus C from five different wild rat species (chestnut spiny rat, greater bandicoot rat, Indochinese forest rat, roof rat and Coxing's white-bellied rat) in China. Analysis of 13 complete genome sequences showed that "Rosavirus B" and "Rosavirus C" represent two potentially novel picornavirus species infecting different rodents...
October 2016: PLoS Pathogens
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
Cécile Picard, Guillaume Thouvenin, Caroline Kannengiesser, Jean-Christophe Dubus, Nadia Jeremiah, Frédéric Rieux-Laucat, Bruno Crestani, Alexandre Belot, Françoise Thivolet-Béjui, Véronique Secq, Christelle Ménard, Martine Reynaud-Gaubert, Philippe Reix
We report three cases of pulmonary disease suggesting fibrosis in two familial and one sporadic case. Pulmonary symptoms were associated with various clinical features of systemic inflammation and vasculitis involving the skin, and appeared at different ages. A strong interferon signature was found in all three cases. Disease was not responsive to corticosteroids, and lung transplantation was considered for all three subjects at an early age. One of them underwent double-lung transplantation, but she immediately experienced a primary graft dysfunction and died soon after...
September 2016: Chest
Xiaojing Jiang, Xiuzhu Sun, Weihua Du, Haisheng Hao, Xueming Zhao, Dong Wang, Huabin Zhu, Yan Liu
Pulmonary surfactant (PS) is synthesized and secreted by alveolar epithelial type II (AEII) cells, which is a complex compound formed by proteins and lipids. Surfactant participates in a range of physiological processes such as reducing the surface tension, keeping the balance of alveolar fluid, maintaining normal alveolar morphology and conducting host defense. Genetic disorders of the surfactant homeostasis genes may result in lack of surfactant or cytotoxicity, and lead to multiple lung diseases in neonates, children and adults, including neonatal respiratory distress syndrome, interstitial pneumonia, pulmonary alveolar proteinosis, and pulmonary fibrosis...
August 2016: Zhonghua Yi Xue Yi Chuan Xue za Zhi, Zhonghua Yixue Yichuanxue Zazhi, Chinese Journal of Medical Genetics
Sabrina Frixel, Amelie S Lotz-Havla, Sunčana Kern, Eva Kaltenborn, Thomas Wittmann, Søren W Gersting, Ania C Muntau, Ralf Zarbock, Matthias Griese
ABCA3 is a surfactant lipid transporter in the limiting membrane of lamellar bodies in alveolar type II cells. Mutations in the ATP-binding cassette, sub-family A (ABC1), member 3 (ABCA3) gene cause respiratory distress syndrome in newborns, and chronic interstitial lung disease in children and adults. ABCA3 belongs to the class of full ABC transporters, which are supposed to be functional in their monomeric forms. Although other family members e.g., ABCA1 and ABCC7 have been shown to function as oligomers, the oligomerization state of ABCA3 is unknown...
August 2016: International Journal of Molecular Medicine
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
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
J R Liu, W B Shen, Z Wen, R An, C J Zhou, S Y Zhao
OBJECTIVE: To analyze the clinical characteristics and diagnosis of 2 cases with diffuse pulmonary lymphatic disease. METHOD: Clinical manifestations of the children were retrospectively analyzed. Two patients were both from Beijing Children Hospital in 2013 and 2014.Diffuse pulmonary lymphatic disease was diagnosed by pathology of pleura in case 1 and by lymphoscintigraphy in case 2. RESULT: The first patient was a male aged 10-year-5-month who presented with a history of pleural effusion for 2 years...
May 2016: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
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
M A Dziekiewicz, K Karolewska-Bochenek, Ł Dembiński, A Gawronska, K Krenke, J Lange, M Banasiuk, E Kuchar, M Kulus, P Albrecht, A Banaszkiewicz
Gastroesophageal reflux disease is common in adult patients with interstitial lung disease. However, no data currently exist regarding the prevalence and characteristics of the disease in pediatric patients with interstitial lung disease. The aim of the present study was to prospectively assess the incidence of gastroesophageal reflux disease and characterize its features in children with interstitial lung disease. Gastroesophageal reflux disease was established based on 24 h pH-impedance monitoring (MII-pH)...
2016: Advances in Experimental Medicine and Biology
M Fuger, M-P Clair, N El Ayoun Ibrahim, S L'Excellent, L Nizery, C O'Neill, L Tabone, O Truffinet, C Yakovleff, J de Blic
Chronic interstitial lung disease (ILD) in children is a heterogeneous group of rare lung disorders characterized by an inflammatory process of the alveolar wall and the pulmonary interstitium that induces gas exchange disorders. The diagnostic approach to an ILD involves three essential steps: recognizing the ILD, appreciating the impact, and identifying the cause. The spectrum of clinical findings depends to a large extent on age. In the newborn, the beginning is often abrupt (neonatal respiratory distress), whereas there is a more gradual onset in infants (failure to thrive, tachypnea, indrawing of the respiratory muscles)...
May 2016: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
M Patricia George, Vikas Singh, Mark T Gladwin
The goal of this review is to describe evolving epidemiology of noninfectious, nonneoplastic pulmonary complications of HIV infection, including HIV-associated pulmonary arterial hypertension (HIV-PAH) and interstitial lung disease (ILD). The development of antiretroviral therapy has rendered HIV a chronic illness in treated patients, and the landscape of HIV-associated medical conditions continues to evolve. Although there has been a shift away from AIDS-defining infectious diseases and malignancies, HIV-PAH continues to affect survival adversely when compared with HIV-infected patients without PAH...
April 2016: Seminars in Respiratory and Critical Care Medicine
Cord Sunderkötter, Alexander Nast, Margitta Worm, Reinhard Dengler, Thomas Dörner, Horst Ganter, Reinhard Hohlfeld, Arthur Melms, Nico Melzer, Kai Rösler, Jens Schmidt, Michael Sinnreich, Maggi C Walter, Julia Wanschitz, Heinz Wiendl
The present guidelines on dermatomyositis (DM) represent an excerpt from the interdisciplinary S2k guidelines on myositis syndromes of the German Society of Neurology (available at The cardinal symptom of myositis in DM is symmetrical proximal muscle weakness. Elevated creatine kinase, CRP or ESR as well as electromyography and muscle biopsy also provide important diagnostic clues. Pharyngeal, respiratory, cardiac, and neck muscles may also be affected. Given that approximately 30% of patients also develop interstitial lung disease, pulmonary function tests should be part of the diagnostic workup...
March 2016: Journal der Deutschen Dermatologischen Gesellschaft, Journal of the German Society of Dermatology: JDDG
Thomas Wittmann, Sabrina Frixel, Stefanie Höppner, Ulrike Schindlbeck, Andrea Schams, Matthias V Kappler, Jan Hegermann, Christoph Wrede, Gerhard Liebisch, Anne Vierzig, Angela Zacharasiewicz, Matthias Kopp, Christian F Poets, Winfried Baden, Dominik Hartl, Anton H Van Kaam, Peter Lohse, Charalampos Aslanidis, Ralf Zarbock, Matthias Griese
RATIONALE: The ABCA3 gene encodes a lipid transporter in type II pneumocytes critical for survival and normal respiratory function. The frequent ABCA3 variant R288K increases the risk for neonatal respiratory distress syndrome among term and late preterm neonates, but its role in children's interstitial lung disease has not been studied in detail. OBJECTIVES: In a retrospective cohort study of 228 children with interstitial lung disease related to the alveolar surfactant system, the frequency of R288K was assessed and the phenotype of patients carrying a single R288K variant further characterized by clinical course, lung histology, computed tomography, and bronchoalveolar lavage phosphatidylcholine PC 32:0...
February 26, 2016: Molecular Medicine
Teresa Salerno, Donatella Peca, Laura Menchini, Alessandra Schiavino, Renata Boldrini, Fulvio Esposito, Olivier Danhaive, Renato Cutrera
BACKGROUND: Monoallelic mutations of the Surfactant Protein C gene (SFTPC) are associated with Interstitial Lung Disease in children. I73T is the most common mutation, accounting for 30 % of all cases reported. CASE PRESENTATION: We describe three patients carrying the same I73T SPC mutation with very different phenotypes, clinical course (ranging from mild respiratory symptoms to death for respiratory failure) and outcome. CONCLUSIONS: The disease mechanisms associated with SP-C mutations suggest that the combination of individual genetic background and environmental factors contribute largely to the wide variability of clinical expression...
2016: Italian Journal of Pediatrics
Biman Saikia, Sudhir Gupta
Common variable immunodeficiency (CVID) is the most common primary immunodeficiency of young adolescents and adults which also affects the children. The disease remains largely under-diagnosed in India and Southeast Asian countries. Although in majority of cases it is sporadic, disease may be inherited in a autosomal recessive pattern and rarely, in autosomal dominant pattern. Patients, in addition to frequent sino-pulmonary infections, are also susceptible to various autoimmune diseases and malignancy, predominantly lymphoma and leukemia...
April 2016: Indian Journal of Pediatrics
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
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