Interstitial lung diseases in children

Nathan, Nadia; Berdah, Laura; Delestrain, Céline; Sileo, Chiara; Clément, Annick

doi:10.1016/j.lpm.2019.06.007

Cited by 36 publications

(33 citation statements)

References 285 publications

(190 reference statements)

Supporting

Mentioning

Contrasting

Order By: Relevance

“…In addition to the diseases listed in Table 1 , ABCA transporters have been described as key proteins in several other human disorders, including neonatal respiratory distress syndrome (ABCA3), 139 chronic interstitial lung disease (ABCA3), 140 cataract-microcornea syndrome (ABCA3), 141 hypertrichosis terminalis (ABCA5), 142 or Harlequin ichtyosis (ABCA12). 143…”

Section: Part I: Status Quomentioning

confidence: 99%

Strategies to gain novel Alzheimer’s disease diagnostics and therapeutics using modulators of ABCA transporters

Pahnke

Bascuñana

Brackhan

et al. 2021

Free Neuropathology

View full text Add to dashboard Cite

Adenosine-triphosphate-(ATP)-binding cassette (ABC) transport proteins are ubiquitously present membrane-bound efflux pumps that distribute endo- and xenobiotics across intra- and intercellular barriers. Discovered over 40 years ago, ABC transporters have been identified as key players in various human diseases, such as multidrug-resistant cancer and atherosclerosis, but also neurodegenerative diseases, such as Alzheimer’s disease (AD). Most prominent and well-studied are ABCB1, ABCC1, and ABCG2, not only due to their contribution to the multidrug resistance (MDR) phenotype in cancer, but also due to their contribution to AD. However, our understanding of other ABC transporters is limited, and most of the 49 human ABC transporters have been largely neglected as potential targets for novel small-molecule drugs. This is especially true for the ABCA subfamily, which contains several members known to play a role in AD initiation and progression. This review provides up-to-date information on the proposed functional background and pathological role of ABCA transporters in AD. We also provide an overview of small-molecules shown to interact with ABCA transporters as well as potential in silico , in vitro , and in vivo methodologies to gain novel templates for the development of innovative ABC transporter-targeting diagnostics and therapeutics.

show abstract

Section: Part I: Status Quomentioning

confidence: 99%

Strategies to gain novel Alzheimer’s disease diagnostics and therapeutics using modulators of ABCA transporters

Pahnke

Bascuñana

Brackhan

et al. 2021

Free Neuropathology

View full text Add to dashboard Cite

show abstract

“…Interstitial lung diseases are grouped as ILD specific to children aged <2 years and as ILD not specific to age. While surfactant protein deficiency, neuroendocrine cell hyperplasia of infancy, pulmonary interstitial glycogenesis, and developmental disorders are specific to infancy, ILD related to exposure/environment insults, ILD related to systemic and immune diseases, and ILD related to primary lung parenchyma dysfunctions constitute the non-age specific group (88). Anti-inflammatory and immunosuppressive agents are the cornerstones of pharmacological therapy, while oxygen and ventilatory support are required for patients with hypoxemia and respiratory failure (9).…”

Section: Interstitial Lung Diseasementioning

confidence: 99%

Non-invasive Ventilation for Children With Chronic Lung Disease

2020

View full text Add to dashboard Cite

Advances in medical care and supportive care options have contributed to the survival of children with complex disorders, including children with chronic lung disease. By delivering a positive pressure or a volume during the patient's inspiration, NIV is able to reverse nocturnal alveolar hypoventilation in patients who experience hypoventilation during sleep, such as patients with chronic lung disease. Bronchopulmonary dysplasia (BPD) is a common complication of prematurity, and despite significant advances in neonatal care over recent decades its incidence has not diminished. Most affected infants have mild disease and require a short period of oxygen supplementation or respiratory support. However, severely affected infants can become dependent on positive pressure support for a prolonged period. In case of established severe BPD, respiratory support with non-invasive or invasive positive pressure ventilation is required. Patients with cystic fibrosis (CF) and advanced lung disease develop hypoxaemia and hypercapnia during sleep and hypoventilation during sleep usually predates daytime hypercapnia. Hypoxaemia and hypercapnia indicates poor prognosis and prompts referral for lung transplantation. The prevention of respiratory failure during sleep in CF may prolong survival. Long-term oxygen therapy has not been shown to improve survival in people with CF. A Cochrane review on the use NIV in CF concluded that NIV in combination with oxygen therapy improves gas exchange during sleep to a greater extent than oxygen therapy alone in people with moderate to severe CF lung disease. Uncontrolled, non-randomized studies suggest survival benefit with NIV in addition to being an effective bridge to transplantation. Complications of NIV relate mainly to prolonged use of a face or nasal mask which can lead to skin trauma, and neurodevelopmental delay by acting as a physical barrier to social interaction. Another associated risk is pulmonary aspiration caused by vomiting whilst wearing a face mask. Adherence to NIV is one of the major barriers to treatment in children. This article will review the current evidence for indications, adverse effects and long term follow up including adherence to NIV in children with chronic lung disease.

show abstract

“…For alveolar epithelium these mutations may result in a cell toxicity, increased endoplasmic reticulum stress, inflammation and enhanced apoptosis. The distorted epithelial-mesenchymal transition under the influence of paracrine inflammatory autacoids causes later diffuse interstitial fibrosis of lungs [27].…”

Section: Aetiology: Genes and Environmental Factors Mosaic Interplaymentioning

confidence: 99%

Old wine in new wineskins. Hamman-Rich syndrome has much in common with severe COVID-19: Do they share any link of pathogenesis or depend on the similar aetiological factor(s)?

Ivashkevich

Korovin

Petyaeva

et al. 2020

Vestnik SPbSU. Medicine

View full text Add to dashboard Cite

For the first time the acute interstitial pneumonitis as a main cause of adult idiopathic respiratory distress syndrome was described in 1933–35 by the American medical scholars Louis Virgil Hamman and Arnold Rice Rich as “acute fibrosing alveolitis” later given the eponymous name Hamman-Rich syndrome. This disease targets the interstitium of the lungs and characterized by fulminant onset, acute respiratory disorder with possibility of respiratory failure, being clinically similar to respiratory distress syndrome with the pathomorphologically revealed diffuse alveolar damage. Hamman-Rich syndrome is characterized by rapid progression from symptoms of common cold such as cough, fever, chills and dyspnea to severe respiratory failure. The syndrome affects apparently healthy individuals and is not associated with any anamnestic chronic lung diseases, smoking habits, neither with patients’ age or sex. The aetiology and pathogenesis of the Hamman-Rich syndrome are not clear yet. The familiar genetic predisposition is of definite meaning, especially related to mutations of surfactant proteins and their metabolism. The possible mechanistic links of this fulminant lung disease involve: NK-cells, auto-aggressive immune activation and certain types of cytokines during their excessive systemic action, so-called cytokine storm (e.g. interleukin-18 and interleukin-2), which play the leading part in the patho-genesis of acute lung cell injury resulting in diffuse non-remitting pneumofibrosis. But all these clinical and pathogenetic features are also common for severe COVID-19. Computed tomography is used for instrumental diagnosis of Hamman-Rich syndrome and also gives the lung images quite similar to that of COVID-19, including ground glass opacity symptoms. The authors hypothesize that the same link(s) of pathogenesis underlie the severe course of COVID-19 and Hamman-Rich syndrome. It is possible that cases of Hamman-Rich syndrome were due to the circulation in populations of a less virulent, unrecognized coronavirus, which interacts with the immune system of genetically susceptible individuals in a way similar to SARS-CoV2. For sure, the identification of the characteristics of the genome of patients with Hamman-Rich syndrome will shed light on the genetic mechanisms that predispose to the severe course of COVID-19 and vice versa — the experience of severe COVID-19 treatment can be applied to the therapy of Hamman-Rich syndrome as well.

show abstract

Interstitial lung diseases in children

Cited by 36 publications

References 285 publications

Strategies to gain novel Alzheimer’s disease diagnostics and therapeutics using modulators of ABCA transporters

Strategies to gain novel Alzheimer’s disease diagnostics and therapeutics using modulators of ABCA transporters

Non-invasive Ventilation for Children With Chronic Lung Disease

Old wine in new wineskins. Hamman-Rich syndrome has much in common with severe COVID-19: Do they share any link of pathogenesis or depend on the similar aetiological factor(s)?

Contact Info

Product

Resources

About