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Antitrypsin

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https://www.readbyqxmd.com/read/27926963/-how-carl-bertil-laurell-and-sten-eriksson-detected-the-alpha-1-antitrypsin-deficiency-50-years-ago-and-what-then-came-a-somewhat-headstrong-and-personal-retrospection
#1
https://www.readbyqxmd.com/read/27917957/refined-purification-strategy-for-reliable-proteomic-profiling-of-hdl2-3-impact-on-proteomic-complexity
#2
Michael Holzer, Sabine Kern, Ruth Birner-Grünberger, Sanja Curcic, Akos Heinemann, Gunther Marsche
Proteomics have extended the list of high-density lipoprotein (HDL) associated proteins to about 90. One of the major issues of global protein characterization is establishing specificity of association as opposed to contamination, a fact which has never been addressed for isolated HDL. We have developed a refined purification strategy to isolate HDL by density, followed by purification by size to generate "highly purified" fractions of HDL2/3, which allow the reliable quantification of the HDL proteome for biomarker discovery...
December 5, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27916926/ulcerative-colitis-and-crohn-s-disease-are-associated-with-decreased-serum-selenium-concentrations-and-increased-cardiovascular-risk
#3
Teresa Castro Aguilar-Tablada, Miguel Navarro-Alarcón, Javier Quesada Granados, Cristina Samaniego Sánchez, José Ángel Rufián-Henares, Flor Nogueras-Lopez
The incidence of inflammatory bowel disease (IBD) and associated oxidative stress is increasing. The antioxidant mineral selenium (Se) was measured in serum samples from 106 IBD patients (53 with ulcerative colitis (UC) and 53 with Crohn's disease (CD)) and from 30 healthy controls. Serum Se concentrations were significantly lower in UC and CD patients than in healthy controls (p < 0.001) and significantly lower in CD patients than in UC patients (p = 0.006). Se concentrations in patients were significantly influenced by sex, body mass index (BMI), the inflammatory biomarker α-1-antitrypsin, surgery, medical treatment, the severity, extent, and form of the disease and the length of time since onset (p < 0...
December 1, 2016: Nutrients
https://www.readbyqxmd.com/read/27916480/long-term-efficacy-and-safety-of-%C3%AE-1-proteinase-inhibitor-treatment-for-emphysema-caused-by-severe-%C3%AE-1-antitrypsin-deficiency-an-open-label-extension-trial-rapid-ole
#4
Noel G McElvaney, Jonathan Burdon, Mark Holmes, Allan Glanville, Peter A B Wark, Philip J Thompson, Paul Hernandez, Jan Chlumsky, Helmut Teschler, Joachim H Ficker, Niels Seersholm, Alan Altraja, Riitta Mäkitaro, Joanna Chorostowska-Wynimko, Marek Sanak, Paul I Stoicescu, Eeva Piitulainen, Oliver Vit, Marion Wencker, Michael A Tortorici, Michael Fries, Jonathan M Edelman, Kenneth R Chapman
BACKGROUND: Purified α1 proteinase inhibitor (A1PI) slowed emphysema progression in patients with severe α1 antitrypsin deficiency in a randomised controlled trial (RAPID-RCT), which was followed by an open-label extension trial (RAPID-OLE). The aim was to investigate the prolonged treatment effect of A1PI on the progression of emphysema as assessed by the loss of lung density in relation to RAPID-RCT. METHODS: Patients who had received either A1PI treatment (Zemaira or Respreeza; early-start group) or placebo (delayed-start group) in the RAPID-RCT trial were included in this 2-year open-label extension trial (RAPID-OLE)...
December 1, 2016: Lancet Respiratory Medicine
https://www.readbyqxmd.com/read/27916479/compelling-evidence-for-the-efficacy-of-%C3%AE-1-antitrypsin-augmentation-treatment-for-%C3%AE-1-antitrypsin-deficiency
#5
Ronald G Crystal
No abstract text is available yet for this article.
December 1, 2016: Lancet Respiratory Medicine
https://www.readbyqxmd.com/read/27898561/alpha-1-antitrypsin-deficiency-pathophysiologic-features-and-the-importance-of-screening
#6
Kelly McCosh
Alpha-1 antitrypsin deficiency is an inherited disorder that may lead to early development of emphysema and also can cause serious hepatic disease. Because the condition is underrecognized, diagnosis may be delayed. This article aims to increase awareness about alpha-1 antitrypsin deficiency and screening guidelines that can help primary care providers identify patients early for better outcomes.
December 2016: JAAPA: Official Journal of the American Academy of Physician Assistants
https://www.readbyqxmd.com/read/27879179/-two-sisters-with-lung-emphysema
#7
I Piscaer, F M E Franssen, N H T Ten Hacken, E F M Wouters, R Janssen
BACKGROUND: α1-antitrypsin is an antiprotease that is mainly produced in the liver; it plays a crucial role in the protection of lung parenchyma against the destructive effects of proteases. Mutations in the α1-antitrypsin gene can cause α1-antitrypsin deficiency. Individuals homozygous for the Z-genotype have drastically lowered serum α1-antitrypsine concentrations and often develop lung emphysema at an early age. CASE DESCRIPTION: A 38-year-old woman and her 43-year-old sister both developed lung emphysema at an early age; this could be attributed to severe α1-antitrypsin deficiency...
2016: Nederlands Tijdschrift Voor Geneeskunde
https://www.readbyqxmd.com/read/27877030/application-of-a-diagnostic-algorithm-for-the-rare-deficient-variant-mmalton-of-alpha-1-antitrypsin-deficiency-a-new-approach
#8
Irene Belmonte, Miriam Barrecheguren, Rosa M López-Martínez, Cristina Esquinas, Esther Rodríguez, Marc Miravitlles, Francisco Rodríguez-Frías
BACKGROUND AND OBJECTIVES: Alpha-1-antitrypsin deficiency (AATD) is associated with a high risk for the development of early-onset emphysema and liver disease. A large majority of subjects with severe AATD carry the ZZ genotype, which can be easily detected. Another rare pathologic variant, the Mmalton allele, causes a deficiency similar to that of the Z variant, but it is not easily recognizable and its detection seems to be underestimated. Therefore, we have included a rapid allele-specific genotyping assay for the detection of the Mmalton variant in the diagnostic algorithm of AATD used in our laboratory...
2016: International Journal of Chronic Obstructive Pulmonary Disease
https://www.readbyqxmd.com/read/27871846/the-oxidant-antioxidant-equilibrium-activities-of-selected-lysosomal-enzymes-and-activity-of-acute-phase-protein-in-peripheral-blood-of-18-year-old-football-players-after-aerobic-cycle-ergometer-test-combined-with-ice-water-immersion-or-recovery-at-room-temperature
#9
Paweł Sutkowy, Alina Woźniak, Tomasz Boraczyński, Michał Boraczyński, Celestyna Mila-Kierzenkowska
The goal of the study was to evaluate the effect of an aerobic exercise bout followed by ice-water immersion or recovery at room temperature on the redox state, activities of selected lysosomal enzymes and activity of α1-antitrypsin (AAT) in the blood of healthy sportsmen. Eleven amateur football players aged 18 were randomly assigned to two similar 30-min aerobic cycle ergometer tests followed by a recovery at room temperature (20 °C; Experiment 1) or ice-water immersion (3 °C, 5 min; Experiment 2). Peripheral blood was collected three times during both study experiments: before (baseline), as well as 20 and 40 min after the recovery or immersion...
November 18, 2016: Cryobiology
https://www.readbyqxmd.com/read/27867571/role-of-oxidative-stress-and-outcome-of-various-surgical-approaches-among-patients-with-bullous-lung-disease-candidate-for-surgical-interference
#10
Ahmed Farouk, Mohammed H Hassan, Mohammed Alaa Nady, Mohammed Farouk Abdel Hafez
BACKGROUND: Bullous lung disease is characterized by formation of blebs, bullae and emphysema. We investigate the role of oxidative stress in the pathogenesis of bullous lung disease and compare between conventional thoracotomy versus video assisted thoracoscopic approach in surgical management of such patients. METHODS: This study was a prospective case control study and it was carried out on 21 patients (16 males and 5 females) with bullous lung disease selected as candidate for surgical interference...
October 2016: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/27862254/hk2-proximal-tubule-epithelial-cells-synthesize-and-secrete-plasma-proteins-predominantly-through-the-apical-surface
#11
Ke-Wei Zhao, Elsa J Brochmann Murray, Samuel S Murray
Renal proximal tubule epithelial cells (PTECs) are known to reabsorb salts and small plasma proteins filtered through Bowman's capsule. Following acute kidney injury, PTECs assume some characteristics of hepatocytes in producing various plasma proteins. We now demonstrate that even at a resting state, a PTEC cell line, HK2 expresses mRNAs for and synthesizes and secretes plasma proteins in a complex with complement C3, an α2 -macroglobulin family chaperone, including albumin, transferrin, α1 -antitrypsin, α1 -antichymotrypsin, α2 -HS-glycoprotein, ceruloplasmin, haptoglobin, C1-inhibitor, secreted phosphoprotein-24, and insulin-like growth factor-1...
November 9, 2016: Journal of Cellular Biochemistry
https://www.readbyqxmd.com/read/27861298/treatment-with-%C3%AE-1-antitrypsin-for-steroid-refractory-acute-intestinal-graft-versus-host-disease-a-report-of-2-cases
#12
Romana Raphaela Gerner, Clemens Feistritzer, Alexander Rupert Moschen, Brigitte Kircher, Patrizia Moser, Herbert Tilg, David Nachbaur
No abstract text is available yet for this article.
December 2016: Transplantation
https://www.readbyqxmd.com/read/27857848/the-effect-of-%C3%AE-1-antitrypsin-deficiency-combined-with-increased-bacterial-loads-on-chronic-obstructive-pulmonary-disease-pharmacotherapy-a-prospective-parallel-controlled-pilot-study
#13
Marwa G Hennawy, Noha M Elhosseiny, Hussein Sultan, Wael Abdelfattah, Yousry Akl, Nirmeen A Sabry, Ahmed S Attia
Chronic obstructive pulmonary disease (COPD) is caused by α1-antitrypsin deficiency (AATD) genetic susceptibility and exacerbated by infection. The current pilot study aimed at studying the combined effect of AATD and bacterial loads on the efficacy of COPD conventional pharmacotherapy. Fifty-nine subjects (29 controls and 30 COPD patients) were tested for genetic AATD and respiratory function. The bacterial loads were determined to the patients' group who were then given a long acting beta-agonist and corticosteroid inhaler for 6 months...
November 2016: Journal of Advanced Research
https://www.readbyqxmd.com/read/27855621/alpha-1-antitrypsin-deficiency-current-perspective-from-genetics-to-diagnosis-and-therapeutic-approaches
#14
Simona Santangelo, Simone Scarlata, Luana M Poeta, Adam J Bialas, Gregorino Paone, Raffaele Antonelli Incalzi
Alpha-1 antitrypsin (A1AT) is a 52-kDa, acute phase glycoprotein encoded by the protease inhibitor (PI) locus, located on the long arm of chromosome 14 (14q31-32.3). Its structure is composed of a total of 7 exons, 4 coding (II, III, IV, and V) and 3 non-coding (Ia, Ib, and Ic). A1AT is produced primarily by hepatocytes and acts as a serine protease inhibitor with anti-protease and immunoregulatory activities. The main target of A1AT is neutrophil elastase (NE), an enzyme released during a neutrophil-mediated inflammatory process...
November 18, 2016: Current Medicinal Chemistry
https://www.readbyqxmd.com/read/27855487/transfer-of-therapeutic-genes-into-fetal-rhesus-monkeys-using-recombinant-adeno-associated-type-i-viral-vectors
#15
Thomas J Conlon, Cathryn S Mah, Christina A R Pacak, Mary B Rucker Henninger, Kirsten E Erger, Marda L Jorgensen, Charles C Lee, Alice F Tarantal, Barry J Byrne
Neuromuscular disorders such as Pompe disease (glycogen storage disease, type II), result in early and potentially irreversible cellular damage with a very limited opportunity for intervention in the newborn period. Pompe disease is due to deficiency in acid α-glucosidase (GAA) leading to lysosomal accumulation of glycogen in all cell types, abnormal myofibrillogenesis, respiratory insufficiency, neurological deficits, and reduced contractile function in striated muscle. Previous studies have shown that fetal delivery of recombinant adeno-associated virus (rAAV) encoding GAA to the peritoneal cavity of Gaa-/- mice resulted in high-level transduction of the diaphragm...
November 17, 2016: Human Gene Therapy. Clinical Development
https://www.readbyqxmd.com/read/27827549/advances-in-identifying-urine-serum-biomarkers-in-alpha-1-antitrypsin-deficiency-for-more-personalized-future-treatment-strategies
#16
Ilaria Ferrarotti, Angelo Guido Corsico, Jan Stolk, Stefania Ottaviani, Marco Fumagalli, Sabina Janciauskiene, Paolo Iadarola
Alpha1-antitrypsin deficiency (AATD) is a genetic disorder characterized by reduced serum levels of alpha1-antitrypsin (AAT) and increased risk for developing both early-onset lung emphysema and chronic liver disease. Laboratory diagnosis of AATD is not just a matter of degree, although the AAT serum level is the most important determinant for risk of lung damage. While being a single-gene disease, the clinical phenotype of AATD is heterogeneous. The current standard of care for patients affected by AATD-associated pulmonary emphysema is replacement therapy with weekly i...
November 9, 2016: COPD
https://www.readbyqxmd.com/read/27824593/the-prevalence-of-diagnosed-%C3%AE-1-antitrypsin-deficiency-and-its-comorbidities-results-from-a-large-population-based-database
#17
Timm Greulich, Christoph Nell, David Hohmann, Marco Grebe, Sabina Janciauskiene, Andreas Rembert Koczulla, Claus Franz Vogelmeier
α1-Antitrypsin deficiency (AATD) is a genetically determined disorder that is associated with different clinical manifestations. We aimed to assess the prevalence of diagnosed AATD and its comorbidities using a large healthcare database.In this retrospective longitudinal observational study, we analysed data from 4 million insurants. Using International Classification of Diseases revision 10 (ICD-10) codes, we assessed the prevalence, comorbidities and healthcare utilisation of AATD patients (E88.0 repeatedly coded) relative to non-AATD patients with chronic obstructive pulmonary disease (COPD), emphysema or asthma...
October 20, 2016: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/27821710/exploration-of-%C3%AE-1-antitrypsin-treatment-protocol-for-islet-transplantation-dosing-plan-and-route-of-administration
#18
Boris M Baranovski, Eyal Ozeri, Galit Shahaf, David E Ochayon, Ronen Schuster, Nofar Bahar, Noa Kalay, Pablo Cal, Mark I Mizrahi, Omer Nisim, Pnina Strauss, Eran Schenker, Eli C Lewis
Life-long weekly infusions of human α1-antitrypsin (hAAT) are currently administered as augmentation therapy for patients with genetic AAT deficiency (AATD). Several recent clinical trials attempt to extend hAAT therapy to conditions outside AATD, including type 1 diabetes. Since the endpoint for AATD is primarily the reduction of risk for pulmonary emphysema, the present study explores hAAT dose protocols and routes of administration in attempt to optimize hAAT therapy for islet-related injury. Islet-grafted mice were treated with hAAT (Glassia™; i...
November 7, 2016: Journal of Pharmacology and Experimental Therapeutics
https://www.readbyqxmd.com/read/27819491/alpha1-antitrypsin-deficiency-increased-knowledge-and-diagnostic-testing-after-viewing-short-instructional-video
#19
Joanna L Nolte, Ali Ataya, Hunter Merrill, Mikala Childs, Mark Brantly
Many individuals with Alpha-1 Antitrypsin Deficiency (AATD) are unaware of their diagnosis. In the absence of an AATD diagnosis, irreversible damage continues, and incorrect care is provided. Research demonstrates low levels of knowledge about AATD among health care providers. To address this ongoing issue, a short educational video was developed for health care providers with the goal of increasing knowledge and testing for AATD. A five-question test on the video material was developed. Invitations to participate in the study were sent via email to providers at both public teaching hospitals and private practices across the country...
November 7, 2016: COPD
https://www.readbyqxmd.com/read/27818805/hepatocellular-carcinoma-with-prominent-intracytoplasmic-inclusions-a-report-of-two-cases
#20
Adeline R Chelliah, Jasim M Radhi
Hepatocellular carcinoma (HCC) is the commonest primary malignant neoplasm of the liver in most countries with a notoriously poor prognosis. Variation in global incidence is well-recognized and the occurrence of HCC is linked to several established environmental, dietary, and lifestyle factors. HCC demonstrates morphological heterogeneity both within the same tumor and from patient to patient. Differing architectural patterns and cytological variants may be seen. Inclusion bodies are believed to represent organized structures of proteins which contribute to their pathogenesis and share several constituents like chaperones, p62, ubiquitin, and Valosin containing protein...
2016: Case Reports in Hepatology
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