Characterization of the Serum a1-Antitripsine Level in Primary Spontaneous Pneumotorax Patients

2018 ◽  
Vol 69 (9) ◽  
pp. 2591-2593
Author(s):  
Cristina Grigorescu ◽  
Liviu Ciprian Gavril ◽  
Laura Gavril ◽  
Tiberiu Lunguleac ◽  
Bogdan Mihnea Ciuntu ◽  
...  
Keyword(s):  
Serum Level ◽  
Pulmonary Emphysema ◽  
Spontaneous Pneumothorax ◽  
Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin ◽  
Determining Factor

Diagnosis of primary or idiopathic spontaneous pneumothorax is one of exclusion, and in fact defines an entity that may have a difficult or impossible cause to be highlighted by current means, we consider it appropriate to study these etiopathogenic aspects. There is a definite association between alpha-1 antitrypsin deficiency and pulmonary emphysema and indirect spontaneous pneumothorax secondary to an emphysematous pulmonary lesion. Dose of alpha-1 antitrypsin is an immunoturbinimetric method for in vitro determination of alpha-1 antitrypsin in human serum and plasma. This product is calibrated to be used for the Daytona RX analyzer. The serum level of alpha-1-antitrypsin is not a determining factor in the postoperative evolution characterized by the interval until air loss disappears, but certainly exerts some influence, the exact level of which remains to be determined.

scholarly journals Kraujo krešumo sistemos pokyčiai sergant paveldimąja plaučių emfizema

2005 ◽  
Vol 3 (1) ◽  
pp. 0-0
Author(s):  
Vytis Bajoriūnas ◽  
Romaldas Rubikas ◽  
Paulius Gradauskas ◽  
Diana Samiatina ◽  
Algirdas Vilčinskas ◽  
...  
Keyword(s):  
Pulmonary Emphysema ◽  
Spontaneous Pneumothorax ◽  
Blood Clotting ◽  
Pleural Cavity ◽  
University Hospital ◽  
The Family ◽  
Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin ◽  
Medical University

Vytis Bajoriūnas, Romaldas Rubikas, Paulius Gradauskas, Diana Samiatina, Algirdas Vilčinskas, Leonas JasulaitisKauno medicinos universitetoTorakalinės chirurgijos klinika,Eivenių g. 2, LT-50009 KaunasEl paštas: [email protected], [email protected] Įvadas Pateikiami penkių gydytų pacientų ligos istorijų duomenys, aprašoma kraujo krešumo sistemos patologija sergant paveldima plaučių emfizema. Ligoniai ir metodai 2000–2003 metais Torakalinės chirurgijos klinikoje gydyti penki vienos giminės nariai, sirgę spontaninio pneumotorakso komplikuota plaučių emfizema. Įgimta plaučių emfizema šeimoje sirgo ir šiuo metu serga 12 žmonių. Visi ligoniai buvo operuoti. Atliktos torakotomijos, rezekuota pažeista plaučio dalis, atlikta dalinė arba visiškoji pleurektomija, drenuota pleuros ertmė. Rezultatai Pooperaciniu laikotarpiu visiems ligoniams padidėjo kraujavimas į pleuros ertmę. Netekto kraujo tūris buvo papildomas hemotransfuzija, šviežiai šaldytos plazmos infuzijomis. Dėl kraujavimo į pleuros ertmę ar susidariusio hemotorakso buvo atliktos trys retorakotomijos. Pakartotinių operacijų metu buvo rastas įvairaus dydžio hemotoraksas (600–1100 ml), difuzinis kraujavimas iš krūtinės sienos be aiškaus vieno kraujavimo židinio. Visi pacientai pasveiko. Išvada Turimi duomenys yra būdingi įgimtam alfa-1 antitripsino sintezės defektui. Reikšminiai žodžiai: plaučių emfizema, spontaninis pneumotoraksas, pooperacinės komplikacijos, antitrombinas III Malfunction of blood clotting associated with hereditary pulmonary emphysema Vytis Bajoriūnas, Romaldas Rubikas, Paulius Gradauskas, Diana Samiatina, Algirdas Vilčinskas, Leonas JasulaitisClinic of Thoracic Surgery,Kaunas University of Medicine,Eivenių str. 2, LT-50009 Kaunas, LithuaniaE-mail: [email protected], [email protected] Background / objective There were five members of one family suffering from pulmonary emphysema complicated with spontaneous pneumothorax, treated at Kaunas Medical University Hospital since 2000 till 2003. The data from the case histories of all the patients are presented and the possible reasons for blood clotting dysfunction are discussed. Patients and methods Twelve members of the family have been or still are suffering from congenital pulmonary emphysema. Five members of the family were operated on. The surgey involved thoracotomy, resection of the damaged section of a lung, partial or total pleurectomy, pleural cavity drainage. Results All the patients underwent surgery, and in all cases the postoperative intrapleural bleeding was uncommonly intensive. In three cases rethoracotomies were performed. All patients survived. Conclusion The presented data characterise congenital alpha-1-antitrypsin deficiency. Keywords: pulmonary emphysema, spontaneous pneumothorax, post-operative complications, antithrombin

scholarly journals Alpha-1 antitrypsin deficiency as a common treatable mechanism in chronic respiratory disorders and for conditions different from pulmonary emphysema? A commentary on the new European Respiratory Society statement

2018 ◽  
Vol 13 ◽  
Author(s):  
Stefano Aliberti ◽  
Andrea Gramegna ◽  
Marco Confalonieri ◽  
Angelo Corsico ◽  
Luca Richeldi ◽  
...  
Keyword(s):  
Lung Disease ◽  
Pulmonary Emphysema ◽  
Future Research ◽  
Common Mechanism ◽  
Main Body ◽  
New Paradigm ◽  
European Respiratory Society ◽  
Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin

Background: The European Respiratory Society recently published an important statement reviewing available evidence on diagnosis and treatment of lung disease associated to alpha-1 antitrypsin deficiency (AATD). Several issues on this topic still remain unresolved and subject of interpretation according to different standard procedures and healthcare systems worldwide. The purpose of this commentary is to offer a critical contribution to most of these controversial issues in light of an Italian perspective for the management of this disease. Main body: The clinical spectrum of AATD lung disease might include different manifestations and the traditional paradigm of a younger emphysematous patient has been revealing insufficient. Targeting with appropriate testing only COPD patients might be considered a limited approach leading to underestimation of the real prevalence of the disease. Several reports have suggested the association between AATD and other chronic respiratory conditions, as asthma and bronchiectasis. A deeper evaluation of clinical, radiological, microbiological and functional variables is, therefore, needed in order to investigate different phenotypes in AATD patients. In addition, a new line of translational research in AATD might focus on the development of personalized therapeutic regimens taking into account the patient clinical profile and needs. Conclusions: Over the past years, AATD has been interpreted as a common mechanism of inflammatory disequilibrium and tissue damage across different conditions. Future research is gradually pointing toward this new paradigm by expanding the evidence of the role of AAT as a potent immunomodulatory and anti-inflammatory drug in conditions different from pulmonary emphysema.

scholarly journals In Vitro Investigations on Optimizing and Nebulization of IVT-mRNA Formulations for Potential Pulmonary-Based Alpha-1-Antitrypsin Deficiency Treatment

Pharmaceutics ◽  
2021 ◽  
Vol 13 (8) ◽  
pp. 1281
Author(s):  
Shan Guan ◽  
Max Darmstädter ◽  
Chuanfei Xu ◽  
Joseph Rosenecker
Keyword(s):  
Epithelial Cells ◽  
Transfection Efficiency ◽  
Genetic Diseases ◽  
Airway Epithelial Cells ◽  
Airway Epithelial ◽  
Significant Toxicity ◽  
Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin

In vitro-transcribed (IVT) mRNA has come into focus in recent years as a potential therapeutic approach for the treatment of genetic diseases. The nebulized formulations of IVT-mRNA-encoding alpha-1-antitrypsin (A1AT-mRNA) would be a highly acceptable and tolerable remedy for the protein replacement therapy for alpha-1-antitrypsin deficiency in the future. Here we show that lipoplexes containing A1AT-mRNA prepared in optimum conditions could successfully transfect human bronchial epithelial cells without significant toxicity. A reduction in transfection efficiency was observed for aerosolized lipoplexes that can be partially overcome by increasing the initial number of components. A1AT produced from cells transfected by nebulized A1AT-mRNA lipoplexes is functional and could successfully inhibit the enzyme activity of trypsin as well as elastase. Our data indicate that aerosolization of A1AT-mRNA therapy constitutes a potentially powerful means to transfect airway epithelial cells with the purpose of producing functional A1AT, while bringing along the unique advantages of IVT-mRNA.

Does the disease state influence the responsiveness of human airways studied in vitro?

1996 ◽  
Vol 80 (6) ◽  
pp. 2211-2216 ◽  
Author(s):  
C. L. Armour ◽  
K. O. McKay ◽  
P. R. Johnson ◽  
A. R. Glanville ◽  
J. L. Black
Keyword(s):  
Electrical Field Stimulation ◽  
Airway Disease ◽  
Human Airway ◽  
Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin ◽  
Human Airways ◽  
Deficiency Group ◽  
Comprehensive Study

Human airway tissue has been used in vitro to study mechanisms of airway disease. However, there has never been a comprehensive study that has looked at the influence of disease on the subsequent in vitro responsiveness of human airways. In this study, we obtained airway tissue from patients who were undergoing resection of the lung for carcinoma. We then compared the airway responsiveness in these tissues and in tissues from patients who had undergone lung transplantation for alpha-1-antitrypsin deficiency, emphysema, or cystic fibrosis with the responsiveness in tissues obtained from donor lungs, i.e., nondiseased. When the relationships between concentration and response were compared, we found that for histamine, electrical field stimulation, levcromakalim, and isoproterenol similar responses could be expected in tissues obtained from all the sources studied. This was not true for acetylcholine in that there were significantly lower responses in tissues from patients with alpha-1-antitrypsin deficiency (P = 0.02; n = 9) or from patients having a lung resected for carcinoma (P = 0.01; n = 6) compared with that of the nondiseased group (n = 6). Similarly, for carbachol, the responses were significantly lower in the alpha-1-antitrypsin deficiency group (P = 0.001; n = 10) and in specimens resected for carcinoma (P = 0.001; n = 6) than in the nondiseased group (n = 9). We conclude that, apart from acetylcholine and carbachol, contractile and relaxant agonists give similar responses when used in human airway tissues from various sources. Our results highlight the importance of stating the source of tissue when human airways are to be studied.

Spontaneous Pneumothorax in Flight as First Manifestation of Alpha-1 Antitrypsin Deficiency

2008 ◽  
Vol 79 (7) ◽  
pp. 704-706 ◽  
Author(s):  
Yi-Chang Lin ◽  
Wen-Kuan Chiu ◽  
Hung Chang ◽  
Yeung-Leung Cheng ◽  
Jen-Chin Chen
Keyword(s):  
Spontaneous Pneumothorax ◽  
Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin

scholarly journals STUDY ON A CASE WITH PULMONARY EMPHYSEMA COMBINED WITH ALPHA 1-ANTITRYPSIN DEFICIENCY AND IT'S FAMILY

1978 ◽  
Vol 67 (3) ◽  
pp. 295-300
Author(s):  
Osamu KITADA ◽  
Minoru SUGITA ◽  
Hideaki KIKUCHI ◽  
Zenji HORAI ◽  
Shigeko INOKUMA
Keyword(s):  
Pulmonary Emphysema ◽  
Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin

scholarly journals A Novel Small Molecule Inhibits Intrahepatocellular Accumulation of Z-Variant Alpha 1-Antitrypsin In Vitro and In Vivo

Cells ◽  
2019 ◽  
Vol 8 (12) ◽  
pp. 1586 ◽  
Author(s):  
Xiaojuan Zhang ◽  
Kien Pham ◽  
Danmeng Li ◽  
Ryan J. Schutte ◽  
David Hernandez Gonzalo ◽  
...  
Keyword(s):  
Liver Disease ◽  
Cell Model ◽  
Curative Therapy ◽  
Animal Models Of Disease ◽  
Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin ◽  
Models Of Disease

Alpha 1-antitrypsin deficiency (AATD) is the most common genetic cause of liver disease in children and is associated with early-onset chronic liver disease in adults. AATD associated liver injury is caused by hepatotoxic retention of polymerized mutant alpha 1-antitrypsin molecules within the endoplasmic reticulum. Currently, there is no curative therapy for AATD. In this study, we selected small molecules with the potential to bind mutant alpha 1-antitrypsin (Z-variant) to inhibit its accumulation in hepatocytes. We used molecular docking to select candidate compounds that were validated in cell and animal models of disease. A crystal structure of polymerized alpha 1-antitrypsin molecule was used as the basis for docking 139,735 compounds. Effects of the top scoring compounds were investigated in a cell model that stably expresses Z-variant alpha 1-antitrypsin and in PiZ mice expressing Z-variant human alpha 1-antitrypsin (Z-hAAT), encoded by SERPINA1*E342K. 4′,′5-(Methylenedioxy)-2-nitrocinnamic acid was predicted to bind cleaved alpha 1-antitrypsin at the polymerization interface, and observed to co-localize with Z-hAAT, increase Z-hAAT degradation, inhibit intracellular accumulation of Z-hAAT, and alleviate liver fibrosis.

Sign in / Sign up

Export Citation Format

Share Document