Reply to: “Assessment of liver phenotype in adults with severe alpha-1 antitrypsin deficiency (Pi*ZZ genotype)”

We appreciate the interest and comments by Dr. Kumpers1 et al. on our recent work characterizing the clinical and histological findings in patients with severe alpha-1 antitrypsin (AAT) deficiency.2 To that end, we reviewed how the proposed liver stiffness measurements (LSM) ≥7.1 kPa performed in our cohort. Using that cut-off, the prevalence of clinically significant fibrosis defined as stage ≥2 would be 26%, which is remarkably similar to the 23.6% reported by Hamesch et al.3 in a large study that did not include biopsies.
Source: Journal of Hepatology - Category: Gastroenterology Authors: Tags: Letter to the Editor Source Type: research

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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
This study aimed to evaluate the relations between complementary feeding, intestinal barrier function, and environmental enteropathy (EE) in infants. Data from 233 children were obtained from the Brazilian site of the MAL-ED cohort study. Habitual dietary intake from complementary feeding was estimated using seven 24 h dietary recalls, from 9 to 15 months of age. Intestinal barrier function was assessed using the lactulose/mannitol test (L/M) and EE was determined as a composite measure using fecal biomarkers concentrations - alpha-1-antitrypsin, myeloperoxidase (MPO) and neopterin (NEO) at 15 months of age. The nutrient a...
Source: The British Journal of Nutrition - Category: Nutrition Authors: Tags: Br J Nutr Source Type: research
AbstractPrimary intraosseous xanthomas of the jaws (PIXJ) are rare and predominantly affect the posterior mandible (86%) of normolipemic patients, with a mean age of 30  years and no gender predilection. Clinically, PIXJ exhibit indolent biologic behavior; curettage is considered treatment of choice. Only 36 PIXJ have been reported. Apoptosis-related hyaline globules (HGs), also known as “thanatosomes”, have not been previously reported in PIXJ. Cases diagnose d as xanthoma of bone were retrieved. Six cases fulfilling currently accepted criteria were identified and their clinicopathologic and immunohistoch...
Source: Head and Neck Pathology - Category: Pathology Source Type: research
Abstract Background: Although the role of electron microscopy is diminishing in several areas of adult pathology, it remains an essential tool for the study of pediatric liver biopsies.Methods: Clinical charts, histologic slides and EM materials of native liver biopsies from patients
Source: Ultrastructural Pathology - Category: Pathology Authors: Tags: Ultrastruct Pathol Source Type: research
AT.AR Abstract INTRODUCTION: Alpha-1 antitrypsin deficiency (AATD) is still underdiagnosed, despite the recommendation to determine AAT in patients with chronic obstructive pulmonary disease (COPD). OBJECTIVE: To estimate the prevalence of AATD in COPD patients adjusted according to the population of the COPD prevalence study in Argentina (EPOC.AR). MATERIAL AND METHODS: This was a multicenter prospective cross-sectional study of a population aged≥30 years of age diagnosed with COPD, involving AAT quantification in dry blood spot and subsequent genotyping in subjects with
Source: Archivos de Bronconeumologia - Category: Respiratory Medicine Authors: Tags: Arch Bronconeumol Source Type: research
Delgado BM PMID: 31864682 [PubMed - as supplied by publisher]
Source: Archivos de Bronconeumologia - Category: Respiratory Medicine Authors: Tags: Arch Bronconeumol Source Type: research
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