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alpha-1 antitrypsin

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https://www.readbyqxmd.com/read/29778740/alpha-1-antitrypsin-ameliorates-inflammation-and-neurodegeneration-in-the-diabetic-mouse-retina
#1
Gustavo Ortiz, Emiliano S Lopez, Juan P Salica, Constanza Potilinski, Mariano Fernández Acquier, Eduardo Chuluyan, Juan E Gallo
Diabetic retinopathy (DR) is the most common cause of blindness in the working age population. Early events of DR are accompanied by neurodegeneration of the inner retina resulting in ganglion cell loss. These findings together with reduced retinal thickness are observed within the first weeks of experimental DR. Besides, an inflammatory process is triggered in DR in which the innate immune response plays a relevant role. Alpha 1 antitrypsin (AAT), an inhibitor of serine proteases, has shown anti-inflammatory properties in several diseases...
May 17, 2018: Experimental Eye Research
https://www.readbyqxmd.com/read/29770228/acoustic-radiation-force-impulse-arfi-elastography-in-a-cohort-of-alpha-1-antitrypsin-deficient-individuals-and-healthy-volunteers
#2
Sandra Diaz, Behrouz Mostafavi, Hanan A Tanash, Eeva Piitulainen
Background: Acoustic radiation force impulse (ARFI) elastography has been used to assess liver stiffness non-invasively. However, its usefulness in alpha-1 antitripsin-deficient (AATD) individuals is unknown. Purpose: To assess if liver fibrosis is present in a cohort of AATD individuals using ARFI elastography. Material and Methods: Eighty-three participants aged 38-39 years, except for two who were aged 40 years, underwent ultrasound elastography of the liver with ARFI technique...
April 2018: Acta Radiologica Open
https://www.readbyqxmd.com/read/29769092/mineralization-of-alpha-1-antitrypsin-inclusion-bodies-in-mmalton-alpha-1-antitrypsin-deficiency
#3
Francesco Callea, Isabella Giovannoni, Paola Francalanci, Renata Boldrini, Gavino Faa, Daniela Medicina, Valerio Nobili, Valeer J Desmet, Kamal Ishak, Kuniaki Seyama, Emanuele Bellacchio
BACKGROUND: Alpha-1-antitrypsin (AAT) deficiency (AATD) of Z, Mmalton, Siiyama type is associated with liver storage of the mutant proteins and liver disease. The Z variant can be diagnosed on isoelectric focusing (IEF) while Mmalton and Siiyama may be missed or misdiagnosed with this technique. Therefore, molecular analysis is mandatory for their characterization. In particular, that holds true for the Mmalton variant as on IEF profile it resembles the wild M2 subtype. METHODS: This is a retrospective analysis involving review of medical records and of liver biopsy specimens from a series of Mmalton, Z and Siiyama Alpha-1-antitrypsin deficiency patients...
May 16, 2018: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/29768056/presentation-and-prognosis-of-liver-disease-in-alpha-1-antitrypsin-deficiency
#4
Sarah Townsend, Philip Newsome, Alice M Turner
No abstract text is available yet for this article.
May 16, 2018: Expert Review of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/29758408/abundant-plasma-protein-depletion-using-ammonium-sulfate-precipitation-and-protein-a-affinity-chromatography
#5
Lentel Pringels, Valérie Broeckx, Kurt Boonen, Bart Landuyt, Liliane Schoofs
Plasma is a highly valuable resource for biomarker research since it is easy obtainable and contains a high amount of information on patient health status. Although advancements in the field of proteomics enabled analysis of the plasma proteome, identification of low abundant proteins remains challenging due to high complexity and large dynamic range. In order to reduce the dynamic range of protein concentrations, a tandem depletion technique consisting of ammonium sulfate precipitation and Protein A affinity chromatography was developed...
May 1, 2018: Journal of Chromatography. B, Analytical Technologies in the Biomedical and Life Sciences
https://www.readbyqxmd.com/read/29729575/proteome-analysis-of-the-potential-serum-biomarkers-for-chronic-benzene-poisoning
#6
Zhimin Zhang, Peimao Li, Dafeng Lin, Dianpeng Wang, Yanfang Zhang
The aim of our study is to seek novel specific biomarkers which could provide clues to the mechanism of chronic benzene poisoning (CBP) and might also be used as specific markers for early detection and diagnosis. In this study, a comparative serological proteome analysis between normal controls and CBP patients at three different levels of poisoning were performed via a 2D-DIGE and MALDI-TOF-MS. As the result a total of 10 proteins were found significantly altered between the normal and the mild, moderate and severe poisoning...
April 27, 2018: Environmental Toxicology and Pharmacology
https://www.readbyqxmd.com/read/29707779/alpha-1-antitrypsin-deficiency-panniculitis-clinical-and-pathologic-characteristics-of-10-cases
#7
Emma F Johnson, Stanislav N Tolkachjov, Lawrence E Gibson
BACKGROUND: Alpha-1 antitrypsin deficiency (AATD) may be associated with liver and lung disease and rarely causes panniculitis. OBJECTIVE: We evaluated the clinicopathologic and laboratory findings of AATD panniculitis in 10 patients. METHODS: We conducted a retrospective review of all cases of AATD panniculitis at Mayo Clinic, Rochester, MN, from 1989 to 2016. RESULTS: Ten patients with AATD panniculitis were included...
April 29, 2018: International Journal of Dermatology
https://www.readbyqxmd.com/read/29696962/alpha1-antitrypsin-deficiency-what-s-new-after-european-respiratory-society-statement
#8
Filippo Patrucco, Ludovica Venezia, Francesco Gavelli, Rinaldo Pellicano, Paolo Solidoro
Alpha-1 antitrypsin deficiency (AATD) is a clinically under-recognized inherited disorder affecting the lungs and the liver. The most common manifestations are pulmonary emphysema, bronchiectasis and liver disease. The recent publication of the European Respiratory Society statement on diagnosis and treatment of pulmonary diseases has replaced the 2003 American Thoracic Society and European Respiratory Society one. New outcome parameters have been introduced and validated by observational and randomized clinical trials, and new information about efficacy and safety of augmentation therapy have been published...
April 24, 2018: Panminerva Medica
https://www.readbyqxmd.com/read/29693942/intraoperative-aortic-dissection-during-lung-transplantation-in-a-patient-with-alpha-1-antitrypsin-deficiency
#9
Kenichiro Tatsumi, Yusuke Hamai, Toshiyuki Mizota, Kazuhiko Fukuda
Alpha-1 antitrypsin deficiency (AATD) is an inherited disorder affecting the lung, liver, and rarely skin. The most frequent features of AATD consist of chronic lung disorders related to protease-antiprotease imbalance in the respiratory system, to which lung transplantation is frequently indicated. We describe a case of aortic dissection in a 55-year-old male who underwent left single lung transplantation for respiratory failure due to AATD-related emphysema. Extracorporeal membrane oxygenation (ECMO) was indicated during the procedure, and an arterial cannula was placed into the descending aorta and a venous cannula was placed into the right femoral vein...
May 2017: Masui. the Japanese Journal of Anesthesiology
https://www.readbyqxmd.com/read/29625713/are-there-differences-between-the-available-treatments-for-emphysema-associated-with-alpha-1-antitrypsin-deficiency
#10
Cristina Esquinas, Marc Miravitlles
No abstract text is available yet for this article.
April 3, 2018: Archivos de Bronconeumología
https://www.readbyqxmd.com/read/29619129/sickle-cell-anemia-patients-in-use-of-hydroxyurea-association-between-polymorphisms-in-genes-encoding-metabolizing-drug-enzymes-and-laboratory-parameters
#11
Sètondji Cocou Modeste Alexandre Yahouédéhou, Magda Oliveira Seixas Carvalho, Rodrigo Mota Oliveira, Rayra Pereira Santiago, Caroline Conceição da Guarda, Suellen Pinheiro Carvalho, Júnia Raquel Dutra Ferreira, Milena Magalhães Aleluia, Elisângela Vitória Adorno, Marilda de Souza Gonçalves
This study investigated associations between SNPs in genes encoding metabolizing drug enzymes and laboratory parameters in sickle cell anemia patients under hydroxyurea (SCA-HU+ ). We evaluated hematologic and biochemical parameters by electronic methods and SNPs by PCR-RFLP and multiplex PCR in 35 SCA-HU+ patients and 67 SCA-HU- patients. The HbS, total cholesterol, lactate dehydrogenase, aspartate aminotransferase, total bilirubin and fractions levels, and leukocyte, eosinophil, monocyte, and erythroblast counts were reduced in SCA-HU+ patients ( p < 0...
2018: Disease Markers
https://www.readbyqxmd.com/read/29618937/the-impact-of-alpha-1-antitrypsin-augmentation-therapy-on-neutrophil-driven-respiratory-disease-in-deficient-individuals
#12
REVIEW
Danielle M Dunlea, Laura T Fee, Thomas McEnery, Noel G McElvaney, Emer P Reeves
Alpha-1 antitrypsin (AAT) is the most abundant serine protease inhibitor circulating in the blood. AAT deficiency (AATD) is an autosomal codominant condition affecting an estimated 3.4 million individuals worldwide. The clinical disease associated with AATD can present in a number of ways including COPD, liver disease, panniculitis and antineutrophil cytoplasmic antibody vasculitis. AATD is the only proven genetic risk factor for the development of COPD, and deficient individuals who smoke are disposed to more aggressive disease...
2018: Journal of Inflammation Research
https://www.readbyqxmd.com/read/29616482/impact-of-a-health-management-program-on-healthcare-outcomes-among-patients-on-augmentation-therapy-for-alpha-1-antitrypsin-deficiency-an-insurance-claims-analysis
#13
Michael A Campos, Michael C Runken, Angela M Davis, Michael P Johnson, Glenda A Stone, Ami R Buikema
INTRODUCTION: Alpha 1-antitrypsin deficiency (AATD) is a genetic disorder which reduces serum alpha 1-antitrypsin (AAT or alpha1-proteinase inhibitor, A1PI) and increases the risk of chronic obstructive pulmonary disease (COPD). Management strategies include intravenous A1PI augmentation, and, in some cases, a health management program (Prolastin Direct® ; PD). OBJECTIVES: This study compared clinical and economic outcomes between patients with and without PD program participation...
April 2018: Advances in Therapy
https://www.readbyqxmd.com/read/29615836/long-term-evolution-of-lung-function-in-individuals-with-alpha-1-antitrypsin-deficiency-from-the-spanish-registry-redaat
#14
Cristina Esquinas, Sonia Serreri, Miriam Barrecheguren, Esther Rodriguez, Alexa Nuñez, Francisco Casas-Maldonado, Ignacio Blanco, Pietro Pirina, Beatriz Lara, Marc Miravitlles
Background: The clinical course of alpha-1 antitrypsin deficiency (AATD) is very heterogeneous. It is estimated that 60% of individuals with severe AATD (Pi*ZZ) develop emphysema. The main objective of this study was to describe the outcomes of long-term lung function in individuals with AATD-associated emphysema after at least 8 years of follow-up. Materials and methods: We performed a retrospective analysis of longitudinal follow-up data of AATD PiZZ patients from the Spanish registry (AATD Spanish Registry [REDAAT])...
2018: International Journal of Chronic Obstructive Pulmonary Disease
https://www.readbyqxmd.com/read/29607607/alpha-1-antitrypsin-in-cell-and-organ-transplantation
#15
Mel Berger, Mingyao Liu, Marc E Uknis, Maria Koulmanda
Limited availability of donor organs and risk of ischemia-reperfusion injury (IRI) seriously restrict organ transplantation. Therapeutics that can prevent or reduce IRI could potentially increase the number of transplants by increasing use of borderline organs and decreasing discards. Alpha-1 antitrypsin (AAT) is an acute phase reactant and serine protease inhibitor which limits inflammatory tissue damage. Purified plasma-derived AAT has been well-tolerated in more than 30 years of use to prevent emphysema in AAT deficient individuals...
April 1, 2018: American Journal of Transplantation
https://www.readbyqxmd.com/read/29603653/an-analytical-method-for-assessing-optimal-storage-conditions-of-gingival-crevicular-fluid-and-disclosing-a-peptide-biomarker-signature-of-gingivitis-by-maldi-tof-ms
#16
Mariaimmacolata Preianò, Giuseppina Maggisano, Maria Stella Murfuni, Chiara Villella, Corrado Pelaia, Tiziana Montalcini, Nicola Lombardo, Girolamo Pelaia, Rocco Savino, Rosa Terracciano
PURPOSE: Gingival crevicular fluid (GCF) is an important diagnostic source of biomarkers for both periodontitis and gingivitis. However, GCF peptide signature may change depending on factors such as handling and storage. Here we propose a standardized methodology for GCF analysis by MALDI-TOF/TOF-MS in order to distinguish a characteristic peptide signature of gingivitis. EXPERIMENTAL DESIGN: The best storage/handling conditions which may ensure the stability of the endogenous peptidome in GCF is determined and then MALDI-TOF MS comparative analysis is performed...
March 30, 2018: Proteomics. Clinical Applications
https://www.readbyqxmd.com/read/29597895/in-vivo-genome-editing-partially-restores-alpha1-antitrypsin-in-a-murine-model-of-aat-deficiency
#17
Chun-Qing Song, Dan Wang, Tingting Jiang, Kevin O'Connor, Qiushi Tang, Lingling Cai, Xiangrui Li, Zhiping Weng, Hao Yin, Guangping Gao, Christian Mueller, Terence R Flotte, Wen Xue
CRISPR genome editing holds promise in the treatment of genetic diseases that currently lack effective long-term therapies. Patients with Alpha-1 Antitrypsin (AAT) deficiency develop progressive lung disease due to the loss of AAT's antiprotease function and liver disease due to a toxic gain of function of the common mutant allele. However, it remains unknown whether CRISPR-mediated AAT correction in the liver, where AAT is primarily expressed, can correct either or both defects. Here we show that AAV delivery of CRISPR can effectively correct Z-AAT mutation in the liver of a transgenic mouse model...
March 29, 2018: Human Gene Therapy
https://www.readbyqxmd.com/read/29592975/fulminant-hepatic-failure-in-the-setting-of-progressive-anca-associated-vasculitis-associated-with-a-rare-alpha-1-antitrypsin-phenotype-piee
#18
Ronald Reilkoff, Laurel Stephenson
Abnormalities in alpha-1 antitrypsin (AAT) proteins are risk factors for human disease. While the most common is AAT deficiency, a genetic disorder associated with chronic obstructive pulmonary disease, additional disorders associated with AAT abnormalities are increasingly recognised. We describe a middle-aged woman who presented with fulminant hepatic and multiorgan failure. Evaluation revealed the patient to have a rare AAT phenotype PiEE. Her clinical presentation was consistent with antineutrophilic cytoplasmic antibody-associated vasculitis, and her history suggested features of panniculitis...
March 28, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29588497/targeted-in-vivo-knock-in-of-human-alpha-1-antitrypsin-cdna-using-adenoviral-delivery-of-crispr-cas9
#19
Calvin J Stephens, Elena Kashentseva, William Everett, Lyudmila Kaliberova, David T Curiel
Serum deficiency diseases such as alpha-1-antitrypsin deficiency are characterized by reduced function of serum proteins, caused by deleterious genetic mutations. These diseases are promising targets for genetic interventions. Gene therapies using viral vectors have been used to introduce correct copies of the disease-causing gene in preclinical and clinical studies. However, these studies highlighted that disease-alleviating gene expression is lost over time. Integration into a specific chromosomal site could provide lasting therapeutic expression to overcome this major limitation...
March 27, 2018: Gene Therapy
https://www.readbyqxmd.com/read/29573789/alpha-1-antitrypsin-inhibits-microglia-activation-and-facilitates-the-survival-of-ipsc-grafts-in-hypertension-mouse-model
#20
Sijing Yang, Bikun Xian, Kaijing Li, Ziming Luo, Yuchun Liu, Dongpeng Hu, Jian Ge
This study was conducted to investigate the use of Alpha 1-antitrypsin (AAT) to inhibit microglia activation in chronic hypertension model and provide a permissive environment for stem cell transplantation. Chronic ocular hypertension of C57BL/6 mice using magnetic microbead injection was induced 3 weeks prior to iPSCs transplantation. The ocular hypertension model was assessed histologically and intraocular pressure was measured. Survival of grafted cells and microglia activation were examined by flow cytometry and immunofluorescence in AAT and PBS treated hosts...
March 16, 2018: Cellular Immunology
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