Alpha1-antitrypsin deficiency: An updated review.

Alpha1-antitrypsin deficiency (AATD) is a rare autosomal recessive disease associated with the homozygous Z variant of the SERPINA1 gene. Clinical expression of AATD, reported 60 years ago associate a severe deficiency, pulmonary emphysema and/or liver fibrosis. Pulmonary emphysema is due to the severe alpha1-antitrypsin deficiency of the ZZ homozygous status and is favored by smoking.

Embolization of Recurrent Pulmonary Arteriovenous Malformations by Ethylene Vinyl Alcohol Copolymer (Onyx) in Hereditary Hemorrhagic Telangiectasia: Safety and Efficacy.

Objectives: To evaluate short- and long-term safety and efficacy of embolization with Onyx for recurrent pulmonary arteriovenous malformations (PAVMs) in hereditary hemorrhagic telangiectasia (HHT).

Methods: In total, 45 consecutive patients (51% women, mean (SD) age 53 (18) years) with HHT referred to a reference center for treatment of recurrent PAVM were retrospectively included from April 2014 to July 2021. Inclusion criteria included evidence of PAVM recurrence on CT or angiography, embolization using Onyx and a minimal 1-year-follow-up CT or angiography.

Effect of contrast material injection protocol on first-pass myocardial perfusion assessed by dual-energy dual-layer computed tomography.

Background: Dual-energy dual-layer computed tomography (CT) scanners can provide useful tools, such as iodine maps and virtual monochromatic images (VMI), for the evaluation of myocardial perfusion defects. Data about the influence of acquisition protocols and normal values are still lacking.

Methods: Clinically indicated coronary CT-angiographies performed between January-October 2018 in a single university hospital with dual-energy dual-layer CT (DE-DLCT) and different injection protocols were retrospectively evaluated.

Progressive fibrosing interstitial lung disease: a clinical cohort (the PROGRESS study).

In patients with chronic fibrosing interstitial lung disease (ILD), a progressive fibrosing phenotype (PF-ILD) may develop, but information on the frequency and characteristics of this population outside clinical trials is lacking.We assessed the characteristics and outcomes of patients with PF-ILD other than idiopathic pulmonary fibrosis (IPF) in a real-world, single-centre clinical cohort. The files of all consecutive adult patients with fibrosing ILD (2010-2017) were examined retrospectively for pre-defined criteria of ≥10% fibrosis on high-resolution computed tomography and progressive disease during overlapping windows of 2 years.