Revealing the molecular signaling pathways of mucus stasis in cystic fibrosis

Mucus obstruction is a hallmark of cystic fibrosis (CF) airway disease, leading to chronic infection, dysregulated inflammation, and progressive lung disease. As mucus hyperexpression is a key component in the initiation and perpetuation of airway obstruction, the triggers underlying mucin release must be identified and understood. In this issue of the JCI, Chen et al. sought to delineate the mechanisms that allow IL-1α/IL-1β to perpetuate the mucoinflammatory environment characteristic of the CF airway. The authors demonstrated that IL-1α and IL-1β stimulated non-CF human bronchial epithelial (HBE) cells to upregulate and secrete both MUC5B and MUC5AC in a dose-dependent manner, an effect that was neutralized by the inhibition of the IL-1α/IL-1β receptor (IL-1R1). Further experiments using mouse models and excised lung tissue identified contributors that drive a vicious feedback cycle of hyperconcentrated mucus secretions and persistent inflammation in the CF airway, factors that are likely at the nidus of progressive lung disease.
Source: Journal of Clinical Investigation - Category: Biomedical Science Authors: Source Type: research

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Source: Jornal Brasileiro de Pneumologia - Category: Respiratory Medicine Source Type: research
AbstractObjectiveThe Phenotype Risk Score (PheRS) is a method to detect Mendelian disease patterns using phenotypes from the electronic health record (EHR). We compared the performance of different approaches mapping EHR phenotypes to Mendelian disease features.Materials and MethodsPheRS utilizes Mendelian diseases descriptions annotated with Human Phenotype Ontology (HPO) terms. In previous work, we presented a map linking phecodes (based on International Classification of Diseases [ICD]-Ninth Revision) to HPO terms. For this study, we integrated ICD-Tenth Revision codes and lab data. We also created a new map between HPO...
Source: Journal of the American Medical Informatics Association - Category: Information Technology Source Type: research
H Kulkarni, S Kansra, S KarandeJournal of Postgraduate Medicine 2019 65(4):193-196
Source: Journal of Postgraduate Medicine - Category: Internal Medicine Authors: Source Type: research
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Improved lung function, increased BMI, reduced need for IV antibiotics seen after one year of therapy
Source: Pulmonary Medicine News - Doctors Lounge - Category: Respiratory Medicine Tags: Family Medicine, Infections, Internal Medicine, Pediatrics, Pharmacy, Pulmonology, Journal, Source Type: news
Authors: Lee L, Smith-Whitley K, Banks S, Puckrein G Abstract Sickle cell disease (SCD) is an inherited blood disorder most common among African American and Hispanic American persons. The disease can cause substantial, long-term, and costly health problems, including infections, stroke, and kidney failure, many of which can reduce life expectancy. Disparities in receiving health care among African Americans and other racial/ethnic minority groups in the United States are well known and directly related to poor outcomes associated with SCD. As an orphan disease-one that affects
Source: Public Health Reports - Category: International Medicine & Public Health Tags: Public Health Rep Source Type: research
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Source: - Pharma News - Category: Pharmaceuticals Source Type: news
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Source: Revista Paulista de Pediatria - Category: Pediatrics Source Type: research
Conclusions: The frequency of HS in patients with CF is high and it is not related to malnutrition, according to the parameters of BMI, TSF and MAMC. The values of MAC and MAMC indicated a greater reserve of muscle mass in patients with HS.RESUMO Objetivo: Determinar a preval ência de esteatose hepática (EH) em crianças e adolescentes com fibrose cística (FC) e associá-la com o estado nutricional. Métodos: Estudo transversal com crianças e adolescentes com diagnóstico de FC. Foram aferidos o peso e a altura para o cálculo do índice de massa corpóre...
Source: Revista Paulista de Pediatria - Category: Pediatrics Source Type: research
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