Sixty-five patients with severe alpha 1-antitrypsin (AAT) deficiency (phenotype PiZ) were followed with spirometry at regular intervals of one year and a median observation period of four years. The annual decline in pulmonary function was adjusted for sex, age and height by division with the predicted normal pulmonary function. The median decline in FEV1 was 1.9% predicted/year. The rate of decline was independent of age and pulmonary function, except for patients with FEV1 below 25% of predicted normal. There was a tendency towards a slower median decline in FEV1 in ex-smokers (1.7% predicted/year) compared to smokers (3.8% predicted/year) and never-smokers (3.7% predicted/year), however, this difference was not significant (p greater than 0.01). At the time of diagnosis smokers and ex-smokers had a lower FEV1 (44 and 38% predicted) than never-smokers (85% predicted) (p less than 0.02), and smokers and ex-smokers were generally younger (median age 44 and 42 years, respectively) than never smokers (median age 55 years) (p greater than 0.1). Our data indicate that smokers as well as nonsmokers with severe AAT deficiency are at risk of developing pulmonary emphysema. The disease seems to appear later in nonsmokers, though once initiated it progresses at the same rate.
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http://dx.doi.org/10.1007/BF02718180 | DOI Listing |
Cardiovasc Hematol Disord Drug Targets
January 2025
Division of Pulmonary and Critical Care Medicine, Texas Tech University Health Sciences Center, El Paso, TX, USA.
Background: Pulmonary embolism (PE) is a frequent cause of death. Acute PE may be treated either with full anticoagulation (AC) alone or thrombolytic therapy with systemic tissue-- type-plasminogen-activator (tPA) based on risk assessment. Currently, AC is the standard of care for most patients with intermediate-high-risk PE, with low-dose tPA emerging as an effective alternative.
View Article and Find Full Text PDFCureus
December 2024
Department of Critical Care Medicine, St. Luke's International Hospital, Tokyo, JPN.
Systemic capillary leak syndrome (SCLS) is a rare and life-threatening disorder characterized by acute hypotension, hypoalbuminemia, and hemoconcentration, which often results in severe respiratory complications, such as pulmonary edema. SCLS can be triggered by infections, including COVID-19, and is associated with a high mortality rate. Here, we report a case of COVID-19-associated SCLS in a 68-year-old man.
View Article and Find Full Text PDFInt J Nanomedicine
January 2025
School of Pharmacy, Jiangxi Medical College, Nanchang University, Nanchang, 330006, People's Republic of China.
Purpose: To improve the oral absorption of relugolix (RLGL), which has low oral bioavailability due to its low solubility and being a substrate of P-glycoprotein (P-gp). A solid self-microemulsifying drug delivery system of relugolix (RLGL-S-SMEDDS) was prepared and evaluated in vitro and in vivo.
Methods: The composition of the solid self-microemulsifying drug delivery system (S-SMEDDS) was selected by solubility study and pseudo-ternary phase diagram, and further optimized by Design-Expert optimization design.
JACC Case Rep
January 2025
Department of Cardiology, Málaga Regional University Hospital, Málaga, Spain.
Pickering syndrome, characterized by recurrent episodes of flash pulmonary edema (FPE) and renal impairment, is associated with renal artery stenosis (RAS). This case highlights its manifestation and management in an older adult patient. An 86-year-old woman with hypertension, chronic kidney disease, and a single functioning kidney presented with recurrent FPE episodes.
View Article and Find Full Text PDFJACC Adv
February 2025
Faculty of Medicine, Hong Kong University, Hong Kong, China.
Background: Whether medium-term increased water intake alone, or in combination with co-adjuvant nonexercise interventions aimed to expand blood volume (BV), improve the human cardiovascular phenotype and cardiorespiratory fitness remains unexplored.
Objectives: The purpose of this study was to determine the medium-term impact of increased (+40%) fluid (water) intake (IFI) or IFI plus head-up sleep (IFI + HUS) on BV and the cardiovascular phenotype in healthy individuals.
Methods: Healthy adults (n = 35, age 42 ± 18 years, 51% female) matched by sex, age, body composition, physical activity, and cardiorespiratory fitness were randomly allocated to IFI or IFI + HUS for 3 months.
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