Search

Introduction: Idiopathic pulmonary fibrosis (IPF) is a fatal progressive fibrosing lung disease. A decreased 6-min walk distance (6MWD) and exercise-induced oxygen desaturation measured during the 6-min walk test (6MWT), are known predictors of mortality in patients with IPF. However, the use of antifibrotic drugs showed a survival benefit in IPF. Therefore, this study aimed to evaluate to what extend 6MWT-derived attributes are associated with two-year survival when antifibrotic drugs were introduced as part of standard IPF-care., Methods: This real-world data-study included patients with IPF with a 6MWT between 2015 and 2020, and used composite outcome: mortality or lung transplantation within 2 years of follow-up. Data were collected systematically, including demographics, pulmonary function tests, comorbidities, medications and 6MWT-derived attributes. The prediction attributes of 6MWT were studied with a Cox Proportional-Hazards model and Kaplan-Meier survival curves. The best discriminating attribute to predict mortality was added to the prediction model Gender-Age-Physiology (GAP)., Results: In 216 patients, 2-year transplant-free survival cut-off points were identified for the 6MWD (≥413 m), 6MWD %predicted (≥83 %), SpO 2 -nadir (≥86 %) and distance-saturation-product (≥374 m%), with the best discriminative value for SpO 2 -nadir (area under the curve: 0.761). 2-Year survival percentage of patients with SpO 2 -nadir below or above threshold (86 %) was 37.1 % and 80.0 %, respectively. Exercise-induced oxygen desaturation added to the GAP model showed an improvement in its predictive power., Conclusion: Patients with IPF who have an exercise-induced oxygen desaturation have worse prognosis. Addition of SpO 2 -nadir to the GAP model seems promising for use in clinical care of IPF patients., Competing Interests: Declaration of competing interest The author declares on behalf of all authors that they have no relevant or material financial interests related to the research described in this article., (Copyright © 2024 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Background and objective: Patients with pulmonary fibrosis (PF) have a clear exercise intolerance. The 4-meter-gait-speed (4MGS) test and the 5-repetitions-sit-to-stand (5STS) test are easy, inexpensive and reliable measures of functional performance. Both tests have been validated in healthy adults and patients with chronic obstructive pulmonary disease. 4MGS test and 5STS test have not been studied in patients with PF. Methods: In this cross-sectional clinimetric validation study 51 PF patients conducted in random order the 4MGS test, 5STS test and the 6-min walk test (6MWT) on a single day. Additionally, body weight, height, lean body mass, health-related quality of life, disease severity, handgrip strength, dyspnoea and leg fatigue were assessed. The setting was a tertiary referral center for Interstitial Lung Diseases. Results: Patients had a diagnosis of idiopathic pulmonary fibrosis (IPF, 37%), PF other than IPF (47%), or unclassified (16%). Patients walked 453±111m in six minutes. Moreover, it took the patients 2.0±0.5s to walk 4 m, and 12.0±3.8s for the 5STS test. The 4MGS test (r = 0.77; p<0.01) and the 5STS test (r = -0.41; p<0.01) correlated significantly with the distance walked in 6MWT. Indeed, 4MGS combined with handgrip strength and Medical Research Council dyspnoea grade could explain 75% of the variance in 6MWD. Conclusions: 4-meter-gait-speed and 5-repetitions sit-to-stand are significantly and independently correlated with the 6-minute walk distance in patients with pulmonary fibrosis. Indeed, 4-meter-gait-speed test may serve as a simple initial field test to assess exercise performance in patients with pulmonary fibrosis. (Sarcoidosis Vasc Diffuse Lung Dis 2018; 35: 317-326) ., (Copyright: © 2018 SARCOIDOSIS VASCULITIS AND DIFFUSE LUNG DISEASES.)

This review discusses the predictive value of animal models in assessment of possible risk from excess vitamin A consumption during pregnancy and the human evidence concerning risk of congenital malformations from excess vitamin A in the diet, consumed either as a constituent of normal foods or in the form of dietary vitamin supplements. Other sources of exposure to vitamin A include medicines (dermal and oral preparations) and cosmetics, but these are not further considered here. Conservative estimates of the likely safe intake of vitamin A during pregnancy are available, based on results from scientific research to date. However, current uncertainties are such that further research is needed to more clearly define intakes which may be on the borderline between those which are beneficial and those which may pose a risk to the developing embryo and fetus.