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Alpha-1 antitrypsin deficiency

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Search Period All yearsLast 12 monthsLast 5 years Topic All topicsGeneralNutrition and dietAlternative treatment methods Type of research Any type of researchHighest Level of Evidence Only (35)Meta-analysis (8)Treatment guideline (5)Phase 3 study (8)Randomized trial (8)Systematic review (6)Clinical Trial (169)Review article (176)Early study (phase 1 or 2) (18)Case report (114)Laboratory or animal study (26)Commentary or editorial (90)Retracted publication (1) Only studies currently recruiting participants (28)

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Publications (1028)

• Description of individual patientsiThe story of one or a couple of patients. Informative, but you cannot infer from it whether something works in general. Azygos Vein Central Venous Access in a Patient with Thoracic Central Venous Obstruction. (2022/03/01) ♡
• Sensitive and specific measurement of alpha(1)-antitrypsin activity with an elastase complex formation immunosorbent assay (ECFISA). (2022/02/05) ♡
• Commentary or editorialiAn expert's opinion or commentary, not new research. Alpha-1 antitrypsin deficiency-associated panniculitis: A potentially challenging diagnosis but an important one for patients. (2022/02/01) ♡
• Prevalence of Alpha-1 Antitrypsin Deficiency, Self-Reported Behavior Change, and Health Care Engagement Among Direct-to-Consumer Recipients of a Personalized Genetic Risk Report. (2022/02/01) ♡
• Hepatobiliary phenotypes of adults with alpha-1 antitrypsin deficiency. (2022/02/01) ♡
• Production and characterization of mono-PEGylated alpha-1 antitrypsin for augmentation therapy. (2022/01/25) ♡
• Alpha-1 antitrypsin deficiency is significantly associated with atopy in asthmatic patients. (2022/01/01) ♡
• Matrix metalloproteinase-13 is fully activated by neutrophil elastase and inactivates its serpin inhibitor, alpha-1 antitrypsin: Implications for osteoarthritis. (2022/01/01) ♡
• Treatment guidelineiAn official agreement between doctors about how this disease should be treated. This is not a single study but the conclusion of an entire medical field. Non-invasive diagnosis and follow-up of rare genetic liver diseases. (2022/01/01) ♡
• α(1) -Antitrypsin Z allele and risk of venous thromboembolism in the general population. (2022/01/01) ♡
• Clinical trialiResearch in patients, without randomization between groups. Useful, but less certain than a randomized trial. Neurodevelopmental Outcomes in Children With Inherited Liver Disease and Native Liver. (2022/01/01) ♡
• Distribution of the Clinical Manifestations of Alpha 1 Antitrypsin Deficiency in Respiratory Outpatients from an Area of Northern Italy. (2022/01/01) ♡
• Commentary or editorialiAn expert's opinion or commentary, not new research. Response to "AAT deficiency: PI*Mpalermo and PI*Siiyama, current diagnosis and future risk". (2022/01/01) ♡
• Commentary or editorialiAn expert's opinion or commentary, not new research. Alpha-1 antitrypsin deficiency: PI*M(palermo) and PI*S(iiyama) , current damage and future risk. (2022/01/01) ♡
• Proteolysis and Deficiency of α1-Proteinase Inhibitor in SARS-CoV-2 Infection. (2022/01/01) ♡
• Lung Function and Health Status in Individuals with Severe Alpha-1-Antitrypsin Deficiency at the Age of 42. (2021/12/23) ♡
• HRD1-mediated METTL14 degradation regulates m(6)A mRNA modification to suppress ER proteotoxic liver disease. (2021/12/16) ♡
• C3d Elicits Neutrophil Degranulation and Decreases Endothelial Cell Migration, with Implications for Patients with Alpha-1 Antitrypsin Deficiency. (2021/12/16) ♡
• The Mechanism of Mitochondrial Injury in Alpha-1 Antitrypsin Deficiency Mediated Liver Disease. (2021/12/09) ♡
• Laboratory or animal researchiNo research in humans yet. Promising in a test tube or in mice unfortunately means nothing for patients. In vitro and in vivo modulation of NADPH oxidase activity and reactive oxygen species production in human neutrophils by α(1)-antitrypsin. (2021/12/06) ♡
• Clinical trialiResearch in patients, without randomization between groups. Useful, but less certain than a randomized trial. Transcriptomics of bronchoalveolar lavage cells identifies new molecular endotypes of sarcoidosis. (2021/12/02) ♡
• Relationship between alpha-1 antitrypsin deficiency and obstructive sleep apnea. (2021/12/01) ♡
• Description of individual patientsiThe story of one or a couple of patients. Informative, but you cannot infer from it whether something works in general. [MDR tuberculosis, Alpha-1-anti-trypsin Deficiency, Cough in a Geriatric Nurse]. (2021/12/01) ♡
• Relationship between Depression and Anxiety, Health Status and Lung Function in Patients with Alpha-1 Antitrypsin Deficiency. (2021/12/01) ♡
• Review articleiA summary of what is known about a topic, written by experts. Not compiled according to fixed search rules, so the selection of studies may be biased. Exosomes in chronic respiratory diseases. (2021/12/01) ♡
• Chronic Type A Aortic Dissection Repair in a Double Lung Transplant Recipient. (2021/12/01) ♡
• Review articleiA summary of what is known about a topic, written by experts. Not compiled according to fixed search rules, so the selection of studies may be biased. Abnormal liver enzymes: A review for clinicians. (2021/11/27) ♡
• Review articleiA summary of what is known about a topic, written by experts. Not compiled according to fixed search rules, so the selection of studies may be biased. The emerging role of proteases in α(1)-antitrypsin deficiency and beyond. (2021/11/22) ♡
• Review articleiA summary of what is known about a topic, written by experts. Not compiled according to fixed search rules, so the selection of studies may be biased. Protein Misfolding and Aggregation: The Relatedness between Parkinson's Disease and Hepatic Endoplasmic Reticulum Storage Disorders. (2021/11/18) ♡
• Alternative poly-adenylation modulates α1-antitrypsin expression in chronic obstructive pulmonary disease. (2021/11/16) ♡
• Nascent chains can form co-translational folding intermediates that promote post-translational folding outcomes in a disease-causing protein. (2021/11/08) ♡
• Adenine base editing reduces misfolded protein accumulation and toxicity in alpha-1 antitrypsin deficient patient iPSC-hepatocytes. (2021/11/03) ♡
• Investigating the Link between Alpha-1 Antitrypsin Deficiency and Abdominal Aortic Aneurysms. (2021/11/01) ♡
• Review articleiA summary of what is known about a topic, written by experts. Not compiled according to fixed search rules, so the selection of studies may be biased. Alpha 1 Antitrypsin Therapy in Patients with Alpha 1 Antitrypsin Deficiency: Perspectives from a Registry Study and Practical Considerations for Self-Administration During the COVID-19 Pandemic. (2021/11/01) ♡
• The need for continuous quality assessment for providing optimal comprehensive care for patients with alpha-1 antitrypsin deficiency. (2021/11/01) ♡
• Review articleiA summary of what is known about a topic, written by experts. Not compiled according to fixed search rules, so the selection of studies may be biased. Current trends in candidate selection, contraindications, and indications for lung transplantation. (2021/11/01) ♡
• IntraIndividual Variability in Serum Alpha-1 Antitrypsin Levels. (2021/10/28) ♡
• Description of individual patientsiThe story of one or a couple of patients. Informative, but you cannot infer from it whether something works in general. Hypertransaminasemia and liver fibrosis associated with haptoglobin retention and anhaptoglobinemia in a paediatric patient. (2021/10/01) ♡
• Description of individual patientsiThe story of one or a couple of patients. Informative, but you cannot infer from it whether something works in general. SERPINA1, generalized pustular psoriasis, and adult-onset immunodeficiency. (2021/10/01) ♡
• Description of individual patientsiThe story of one or a couple of patients. Informative, but you cannot infer from it whether something works in general. A 70-Year-Old Man With Cough and Recurrent Respiratory Infections. (2021/10/01) ♡
• Description of individual patientsiThe story of one or a couple of patients. Informative, but you cannot infer from it whether something works in general. First presentation of portal hypertension complicated by hepatopulmonary syndrome. (2021/09/20) ♡
• Review articleiA summary of what is known about a topic, written by experts. Not compiled according to fixed search rules, so the selection of studies may be biased. Estimated Prevalence and Number of PiMZ Genotypes of Alpha-1 Antitrypsin in Seventy-Four Countries Worldwide. (2021/09/17) ♡
• Association between circulating alpha-1 antitrypsin polymers and lung and liver disease. (2021/09/15) ♡
• Alpha-1 Antitrypsin Z Variant (AAT PI*Z) as a Risk Factor for Intrahepatic Cholestasis of Pregnancy. (2021/09/07) ♡
• A Particular SORL1 Micro-haplotype May Prevent Severe Liver Disease in a French Cohort of Alpha 1-Antitrypsin-deficient Children. (2021/09/01) ♡
• Description of individual patientsiThe story of one or a couple of patients. Informative, but you cannot infer from it whether something works in general. Is the diagnostic rate for the common subtypes of A1AT deficiency consistent across two Canadian Provinces? (2021/09/01) ♡
• Insights into the potential role of alpha1-antitrypsin in COVID-19 patients: Mechanisms, current update, and future perspectives. (2021/09/01) ♡
• Review articleiA summary of what is known about a topic, written by experts. Not compiled according to fixed search rules, so the selection of studies may be biased. Living with the enemy: from protein-misfolding pathologies we know, to those we want to know. (2021/09/01) ♡
• Review articleiA summary of what is known about a topic, written by experts. Not compiled according to fixed search rules, so the selection of studies may be biased. Pulmonary transplantation of alpha-1 antitrypsin (AAT)-transgenic macrophages provides a source of functional human AAT in vivo. (2021/09/01) ♡
• Quantitative (18)F-fluorodeoxyglucose positron emission tomography/computed tomography to assess pulmonary inflammation in COPD. (2021/08/31) ♡

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