Alpha-1 Antitrypsin Deficiency (Symptoms, Treatment, LIfe Expectancy)

Title: Alpha-1 Antitrypsin Deficiency (Symptoms, Treatment, LIfe Expectancy)Category: Diseases and ConditionsCreated: 12/31/1997 12:00:00 AMLast Editorial Review: 6/19/2017 12:00:00 AM
Source: MedicineNet Lungs General - Category: Respiratory Medicine Source Type: news

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The majority of potential donor lungs are not being used. The primary reason for underutilization is the concern for primary graft dysfunction (PGD). There is currently no clinically available therapy for PGD. Alpha 1 antitrypsin (A1AT) is a serine protease inhibitor with anti-inflammatory and cytoprotective properties. We and others have shown benefits of A1AT in small and large animal lung transplant studies. Before performing clinical trials, evidence of therapeutic efficacy in human lungs would be valuable.
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Tags: (154) Source Type: research
Bocchino Alpha-1-antitrypsin deficiency (AATd) is a hereditary disease, mainly characterized by early onset and the lower lobes’ predominant emphysema. Bronchiectasis is characterized by dilatation of the bronchial wall and a clinical syndrome whose features are a cough, sputum production and frequent respiratory exacerbations. In the literature, there are many papers concerning these two clinical entities, but there is still a lot of debate about a possible association between them, in particular about the frequency of their association and causal links. The aim of this short communication is to show the li...
Source: International Journal of Environmental Research and Public Health - Category: Environmental Health Authors: Tags: Communication Source Type: research
This study aimed to investigate prognostic biomarkers of HGSOC through proteomic analysis. We conducted label-free liquid chromatography-mass spectrometry using chemotherapy-naïve, fresh-frozen primary HGSOC specimens, and compared the results between a favorable prognosis group (progression-free survival (PFS) ≥ 18 months, n = 6) and a poor prognosis group (PFS < 18 months, n = 6). Among 658 differentially expressed proteins, 288 proteins were upregulated in the favorable prognosis group and 370 proteins were upregulated in the poor prognosis group. Using hierarchical clustering, we selected &al...
Source: Cancers - Category: Cancer & Oncology Authors: Tags: Article Source Type: research
CONCLUSIONS: The prevalence of irritable bowel syndrome in adolescents was high, with bullying, female gender, age 14-16 years, constipation and diarrhea, and dietary consumption of soft drinks, coffee, and tea as risk factors. A significant association with intestinal inflammation was found. PMID: 32156535 [PubMed - as supplied by publisher]
Source: Jornal de Pediatria - Category: Pediatrics Authors: Tags: J Pediatr (Rio J) Source Type: research
The gene encoding alpha-1 antitrypsin (SERPINA1) is the strongest genetic risk locus for Chronic obstructive pulmonary disease (COPD) susceptibility. While the deficiency alleles PI types S and Z increase the risk for COPD in ZZ homozygotes and ZS compound heterozygotes as compared with wild-type allele homozygotes (MM), contradictory data exists regarding MZ heterozygotes. Ortega et al (Am J Respir Crit Care Med. 2019 Oct 29. https://doi.org/10.1164/rccm.201904-0769OC) performed deep next-generation sequencing and a comprehensive genetic study of this locus in a large cohort of current and former heavy smokers complemente...
Source: Journal of Allergy and Clinical Immunology - Category: Allergy & Immunology Authors: Tags: News beyond our pages Source Type: research
Authors: Zhang Y, Ying X, Zhao Q, Ma J, Zhang D, He C, Han S Abstract Background: Hepatocellular carcinoma (HCC) is a malignant tumor associated with a poor prognosis. Serum biomarkers of HCC have the potential to improve the diagnosis, provide a means to monitor the tumors, and predict their malignancy. Proteins that are expressed differentially between HCC patients and normal controls have the potential to be biomarkers. Method: Serum samples from 10 confirmed HCC patients and 10 controls were collected. The differentially expressed proteins in the serum were identified using an isobaric tags for relative and...
Source: Disease Markers - Category: Laboratory Medicine Tags: Dis Markers Source Type: research
In conclusion, proteomic analysis was able to reveal differences in protein expression between migraine sufferers and non-headache women; as in other neurological diseases characterized by neuroinflammation, the serum proteome of migraine women presents an abundance of proteins indicative of cellular damage, oxidative stress and inflammation. This relevant inflammatory status, if confirmed in larger series, could represent a target for menstrual-related migraine treatment.Graphical abstract
Source: Journal of Pharmaceutical and Biomedical Analysis - Category: Drugs & Pharmacology Source Type: research
CONCLUSION: AAT inhibits formaldehyde-induced apoptosis of HPMCs via a caspase-mediated pathway. These data support a potential use for AAT as a therapeutic agent for the inhibition of peritoneal cell apoptosis during peritoneal dialysis. PMID: 32063193 [PubMed - as supplied by publisher]
Source: Peritoneal Dialysis International - Category: Urology & Nephrology Tags: Perit Dial Int Source Type: research
α1-antitrypsin deficiency (AATD) is a hereditary disorder associated with a risk of developing liver disease and pulmonary emphysema, and other chronic respiratory disorders (mainly asthma and bronchiectasis); Z variant is the commonest deficient variant of AAT. Determining AAT concentration in serum or plasma and identifying allelic variants by phenotyping or genotyping are fundamental in the diagnosis of AATD. Initial evaluation and annual follow-up measurement of lung function, including post-bronchodilator forced expiratory volume in 1 s and gas transfer inform on disease progression. Lung densitometry is th...
Source: European Respiratory Review - Category: Respiratory Medicine Authors: Tags: COPD and smoking, Genetics Review Source Type: research
CONCLUSIONS: Beside the role in the first diagnostic step of liver injuries, the utility of liver transaminases is also maintained during the follow-up of liver diseases and in their prognostic assessment. PMID: 31994373 [PubMed - as supplied by publisher]
Source: Minerva Gastroenterologica e Dietologica - Category: Gastroenterology Tags: Minerva Gastroenterol Dietol Source Type: research
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