Faculty Opinions recommendation of Activation of complement component 3 is associated with airways disease and pulmonary emphysema in alpha-1 antitrypsin deficiency.

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.

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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

Multidisciplinary Respiratory Medicine ◽

10.4081/mrm.2018.200 ◽

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.

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

Nihon Naika Gakkai Zasshi ◽

10.2169/naika.67.295 ◽

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

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Kraujo krešumo sistemos pokyčiai sergant paveldimąja plaučių emfizema

Lietuvos chirurgija ◽

10.15388/lietchirur.2005.1.2330 ◽

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

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