"A consequence is the perpetuation of a vicious cycle with TGF-β promoting epithelial cell apoptosis, which in turn increases the local production of TGF-β . ET-1 is also considered to be an important actor, based on the current knowledge of its numerous functions including fibroblast and smooth muscle cell mitogen, and stimulant of collagen synthesis [23,24]. Recent studies showed that ET-1 is produced by AEC, and could induce alveolar EMT via stimulation of endogenous TGF-β production. "
[Show abstract][Hide abstract] ABSTRACT: Interstitial lung disease (ILD) in infants and children comprises a large spectrum of rare respiratory disorders that are mostly chronic and associated with high morbidity and mortality. These disorders are characterized by inflammatory and fibrotic changes that affect alveolar walls. Typical features of ILD include dyspnea, diffuse infiltrates on chest radiographs, and abnormal pulmonary function tests with restrictive ventilatory defect and/or impaired gas exchange. Many pathological situations can impair gas exchange and, therefore, may contribute to progressive lung damage and ILD. Consequently, diagnosis approach needs to be structured with a clinical evaluation requiring a careful history paying attention to exposures and systemic diseases. Several classifications for ILD have been proposed but none is entirely satisfactory especially in children. The present article reviews current concepts of pathophysiological mechanisms, etiology and diagnostic approaches, as well as therapeutic strategies. The following diagnostic grouping is used to discuss the various causes of pediatric ILD: 1) exposure-related ILD; 2) systemic disease-associated ILD; 3) alveolar structure disorder-associated ILD; and 4) ILD specific to infancy. Therapeutic options include mainly anti-inflammatory, immunosuppressive, and/or anti-fibrotic drugs. The outcome is highly variable with a mortality rate around 15%. An overall favorable response to corticosteroid therapy is observed in around 50% of cases, often associated with sequelae such as limited exercise tolerance or the need for long-term oxygen therapy.
[Show abstract][Hide abstract] ABSTRACT: da pulmoner hipertansiyonun (PH) göreceli yüksek prevalansini (%30-40) açiklayabilecek ortak pa- tomekanizmalara sahiptir. PH özellikle, İAH hastalarinda egzersiz sinirlamasi ve kötü prognoza kat- kida bulunmaktadir. Spesifik klinik semptomlarin olmamasi çoğunlukla taninin geç konulmasina yol açmaktadir. Klinik karar ve difüzyon kapasitesindeki orantisiz düşüş yani sira, şiddetli hipok- semi veya egzersiz oksijen desatürasyonu, ekokardiyografi ve B-tipi natriüretik peptid (BNP) ve N- terminal pro-hormon BNP gibi biyo-belirteçler PH'un belirlenmesinde faydali araçlardir. Ancak ta- niyi doğrulamak için halen sağ kalp kateterizasyonu gereklidir. İAH hastalarinda PH'un yönetimi, altta yatan hastalik sürecinin tedavisi ile uzun süreli oksijen tedavisini içermektedir. Etkilenmiş hastalar, uygun olduğunda gecikmeksizin akciğer transplantasyon sirasina alinmalidir. Ancak yaş ve komorbiditeler nedeniyle İAH hastalarinin sadece küçük bir kismi akciğer transplantasyonuna uygun olacaktir. Birçok İAH'da tatmin edici tedavilerin yokluğu durumunda, etkilenen hastalarda PH'un klinik yükü de göz önüne alindiğinda, PAH için onaylanmiş olan spesifik vazomodülatör te- daviler İAH hastalari için umut verici olabilir. Sonuç olarak, İAH hastalarinda spesifik PH tedavisi- nin etkinliğini değerlendirmek için uygun olarak tasarlanmiş klinik çalişmalara acil ihtiyaç vardir.
[Show abstract][Hide abstract] ABSTRACT: In the lungs, parenchymal and vascular remodelling share pathomechanisms that may explain the relatively high prevalence (30-40%) of pulmonary hypertension (PH) in interstitial lung disease (ILD) patients. Notably, PH significantly contributes to exercise limitation and dismal prognosis of ILD patients. The absence of specific clinical symptoms commonly leads to delayed diagnosis. Besides clinical judgment and out-of-proportion reduction in diffusing capacity, severe hypoxaemia or exercise oxygen desaturation, echocardiography and biomarkers such as B-type natriuretic peptide (BNP) and N-terminal pro-hormone BNP are potentially helpful tools in identifying PH. However, right heart catheterisation is still necessary to confirm the diagnosis. Management of PH in ILD comprises treatment of the underlying disease process and long-term oxygen therapy. Affected patients should be listed for lung transplantation without delay, when appropriate. However, due to age and comorbidities only a minority of ILD patients will be eligible for lung transplantation. In the absence of satisfactory therapies for many ILDs, and considering the clinical burden of PH in affected patients, specific vasomodulatory therapies presently approved for PAH may be promising options for ILD patients. Consequently, there is an urgent need for adequately designed clinical trials to assess the effectiveness of specific PH therapy in the context of ILDs.
European Respiratory Journal 07/2008; 31(6):1357-67. DOI:10.1183/09031936.00171307 · 7.13 Impact Factor
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