Ann Thorac Med
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Idiopathic pulmonary fibrosis (IPF) is rare and can be challenging to diagnose. Limited data is available from the Middle Eastern region, especially Saudi Arabia. ⋯ In Saudi Arabia, IPF patients tended to be slightly older and the disease progression was somewhat slower than reported IPF cohorts in other populations. They had frequent hospital admissions and a long hospital length of stay. The influence of genetics and co-morbid diseases on the incidence and outcome of IPF should be explored further.
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Pulmonary hypertension (PH) is relatively uncommon in children. Pulmonary arterial hypertension (PAH) in pediatric comprises a wide spectrum of diseases, from a transient neonatal condition to a progressive disease associated with morbidity and mortality. ⋯ Despite better understanding of PH and the availability of new medications during recent decades; the diagnosis, investigation and choice of therapy remain a challenge in children, as evidence-based recommendations depend mainly on adult studies. In this review, we provide a detailed discussion about the distinctive features of PAH in pediatric, mainly emphacysing on classification and diagnostic algorithm.
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Pulmonary hypertension (PH) is a phenotype characterized by functional and structural changes in the pulmonary vasculature, leading to increased vascular resistance.[12] The World Health Organization has classified PH into five different types: arterial, venous, hypoxic, thromboembolic or miscellaneous; details are available in the main guidelines. Group I of this classification, designated as pulmonary arterial hypertension (PAH), will remain the main focus here. The pathophysiology involves signaling, endothelial dysfunction, activation of fibroblasts and smooth muscle cells, interaction between cells within the vascular wall, and the circulating cells; as a consequence plexiform lesions are formed, which is common to both idiopathic and heritable PAH but are also seen in other forms of PAH.[234] As the pathology of PAH in the lung is well known, this article focuses on the genetic aspects associated with the disease and is a gist of several available articles in literature.
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This prospective study was conducted to evaluate the value of sonographic B-lines (previously called "comet tail artifacts"), which are long, vertical, well-defined, hyperechoic, dynamic lines originating from the pleural line in assessment of interstitial lung diseases (ILD) and compare them with the findings of chest high-resolution computed tomography (HRCT) and pulmonary function tests (PFTs). ⋯ B-lines that are lung Ultrasound signs seem to be useful in the assessment of ILD.