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Metabolic and storage lung diseases: spectrum of imaging appearances.

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Metabolic and storage lung diseases: spectrum of imaging appearances.

Insights Imaging. 2013 Sep 27;

Authors: Renapurkar RD, Kanne JP

Abstract
Metabolic and storage diseases constitute a heterogeneous group of disorders that occur in the setting of altered biochemical homeostasis. Many of these disorders affect the lungs, either exclusively or as part of a systemic syndrome. For example, amyloidosis can be limited to the tracheobronchial tree or involve the kidneys, lungs and heart. The indolent course of some of these disorders and the non-specific clinical symptoms often result in a diagnostic challenge. Imaging, particularly high-resolution computed tomography (HRCT), is an invaluable asset in the diagnosis of these clinical conditions. Some metabolic and storage diseases have characteristic HRCT appearances, helping narrow the differential diagnosis. Correlation of the radiological and histopathological findings of this group of diseases has also helped improve understanding of these disorders. In addition, CT can offer guidance when tissue sampling is warranted and aid in histopathological diagnosis. This article describes the pertinent clinical features of the more common metabolic and storage diseases affecting the lungs, illustrates their respective HRCT findings and provides the relevant differential diagnosis.
TEACHING POINTS: • To recognise the various metabolic and storage lung diseases • To identify the characteristic imaging findings in various metabolic and storage lung diseases • To discuss the relevant differential diagnoses of each of these diseases.

PMID: 24078438 [PubMed - as supplied by publisher]

Childhood Interstitial Lung Diseases: An 18-year Retrospective Analysis.

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Childhood Interstitial Lung Diseases: An 18-year Retrospective Analysis.

Pediatrics. 2013 Oct;132(4):684-691

Authors: Soares JJ, Deutsch GH, Moore PE, Fazili MF, Austin ED, Brown RF, Sokolow AG, Hilmes MA, Young LR

Abstract
OBJECTIVE: Childhood interstitial lung diseases (ILD) occur in a variety of clinical contexts. Advances in the understanding of disease pathogenesis and use of standardized terminology have facilitated increased case ascertainment. However, as all studies have been performed at specialized referral centers, the applicability of these findings to general pulmonary practice has been uncertain. The objective of this study was to determine the historical occurrence of childhood ILD to provide information reflecting general pediatric pulmonary practice patterns.
METHODS: Childhood ILD cases seen at Vanderbilt Children's Hospital from 1994 to 2011 were retrospectively reviewed and classified according to the current pediatric diffuse lung disease histopathologic classification system.
RESULTS: A total of 93 cases were identified, of which 91.4% were classifiable. A total of 68.8% (64/93) of subjects underwent lung biopsy in their evaluations. The largest classification categories were disorders related to systemic disease processes (24.7%), disorders of the immunocompromised host (24.7%), and disorders more prevalent in infancy (22.6%). Eight cases of neuroendocrine cell hyperplasia of infancy (NEHI) were identified, including 5 that were previously unrecognized before this review.
CONCLUSIONS: Our findings demonstrate the general scope of childhood ILD and that these cases present within a variety of pediatric subspecialties. Retrospective review was valuable in recognizing more recently described forms of childhood ILD. As a significant portion of cases were classifiable based on clinical, genetic, and/or radiographic criteria, we urge greater consideration to noninvasive diagnostic approaches and suggest modification to the current childhood ILD classification scheme to accommodate the increasing number of cases diagnosed without lung biopsy.

PMID: 24081995 [PubMed - as supplied by publisher]

Vitamin D in asthma and future perspectives.

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Vitamin D in asthma and future perspectives.

Drug Des Devel Ther. 2013;7:1003-1013

Authors: Huang H, Porpodis K, Zarogoulidis P, Domvri K, Giouleka P, Papaiwannou A, Primikyri S, Mylonaki E, Spyratos D, Hohenforst-Schmidt W, Kioumis I, Zarogoulidis K

Abstract
Humans have the ability to synthesize vitamin D during the action of ultraviolet (UV) radiation upon the skin. Apart from the regulation of calcium and phosphate metabolism, another critical role for vitamin D in immunity and respiratory health has been revealed, since vitamin D receptors have also been found in other body cells. The term "vitamin D insufficiency" has been used to describe low levels of serum 25-hydroxyvitamin D that may be associated with a wide range of pulmonary diseases, including viral and bacterial respiratory infection, asthma, chronic obstructive pulmonary disease, and cancer. This review focuses on the controversial relationship between vitamin D and asthma. Also, it has been found that different gene polymorphisms of the vitamin D receptor have variable associations with asthma. Other studies investigated the vitamin D receptor signaling pathway in vitro or in experimental animal models and showed either a beneficial or a negative effect of vitamin D in asthma. Furthermore, a range of epidemiological studies has also suggested that vitamin D insufficiency is associated with low lung function. In the future, clinical trials in different asthmatic groups, such as infants, children of school age, and ethnic minorities are needed to establish the role of vitamin D supplementation to prevent and/or treat asthma.

PMID: 24082782 [PubMed - as supplied by publisher]

The role of macrophages in obstructive airways disease: Chronic obstructive pulmonary disease and asthma.

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The role of macrophages in obstructive airways disease: Chronic obstructive pulmonary disease and asthma.

Cytokine. 2013 Sep 28;

Authors: Pappas K, Papaioannou AI, Kostikas K, Tzanakis N

Abstract
Macrophages are a major cellular component of the innate immune system, and play an important role in the recognition of microbes, particulates, and immunogens and to the regulation of inflammatory responses. In the lung, macrophages react with soluble proteins that bind microbial products in order to remove pathogens and particles and to maintain the sterility of the airway tract. Chronic obstructive pulmonary disease and asthma are both obstructive airway diseases that involve chronic inflammation of the respiratory tract which contributes to disease progression. In the case of COPD, there is increasing evidence that lung macrophages orchestrate inflammation through the release of chemokines that attract neutrophils, monocytes and T cells and the release of several proteases. On the other hand, in asthma, it seems that alveolar macrophages are inappropriately activated and are implicated in the development and progression of the disease. In this review we summarize the current basic and clinical research studies which highlight the role of macrophages in asthma and COPD.

PMID: 24084332 [PubMed - as supplied by publisher]

Perinatal factors in neonatal and pediatric lung diseases.

Perinatal factors in neonatal and pediatric lung diseases.

Expert Rev Respir Med. 2013 Oct 3;

Authors: Britt RD, Faksh A, Vogel E, Martin RJ, Pabelick CM, Prakash Y

Abstract
Wheezing and asthma are significant clinical problems for infants and young children, particularly following premature birth. Recurrent wheezing in infants can progress to persistent asthma. As in adults, altered airway structure (remodeling) and function (increased bronchoconstriction) are also important in neonatal and pediatric airway diseases. Accumulating evidence suggests that airway disease in children is influenced by perinatal factors including perturbations in normal fetal lung development, postnatal interventions in the intensive care unit (ICU) and environmental and other insults in the neonatal period. Here, in addition to genetics, maternal health, environmental processes, innate immunity and impaired lung development/function can all influence pathogenesis of airway disease in children. We summarize current understanding of how prenatal and postnatal factors can contribute to development of airway diseases in neonates and children. Understanding these mechanisms will help identify and develop novel therapies for childhood airway diseases.

PMID: 24090092 [PubMed - as supplied by publisher]

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