Monograph

The ERS Monograph is the quarterly book series from the ERS.

Each Monograph covers a specific area of respiratory medicine, providing in-depth reviews that give clinicians at all levels a concise, comprehensive guide to symptoms, diagnosis and treatment.


a1-Antitrypsin Deficiency

European Respiratory Society Monograph, Vol. 85. 2019
ISBN (electronic): 978-1-84984-109-2
DOI: 10.1183/2312508X.erm8519

This Monograph offers a comprehensive and up-to-date overview of AATD. It covers basic biology, genetics, laboratory diagnostics and the major organ manifestations; describes the clinical presentation of AATD in both adults and children; and features chapters on genetic counselling, patient views and future therapies. The content has been tailored to meet the needs of the physician, who takes care of lung and liver patients in daily practice, and the general practitioner, who is responsible for the medical guidance of these patients.


Preface
John R. Hurst
Eur Respir Monogr 2019; 85: v
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Guest Editors

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Introduction
Pavel Strnad, Mark Brantly, Robert Bals
Eur Respir Monogr 2019; 85: ix-xi
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List of abbreviations

Eur Respir Monogr 2019; 85: xii
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Patient view
Marion Wilkens, Terry Hannah
Eur Respir Monogr 2019; 85: 1-5
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Serpinopathies
David A. Lomas, James A. Irving, Bibek Gooptu
Eur Respir Monogr 2019; 85: 6-26
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Genetics and epidemiology of AATD
Brian D. Hobbs, Edwin K. Silverman, Michael H. Cho
Eur Respir Monogr 2019; 85: 27-38
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Laboratory diagnosis of AATD
Ilaria Ferrarotti, Stefania Ottaviani
Eur Respir Monogr 2019; 85: 39-51
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Mechanisms of lung disease in AATD
Cesare Saltini, Karina Krotova
Eur Respir Monogr 2019; 85: 52-63
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Clinical approach to the diagnosis and assessment of AATD
Miriam Barrecheguren, Robert Bals, Marc Miravitlles
Eur Respir Monogr 2019; 85: 64-77
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Treatment of lung disease in patients with AATD
Paul Ellis, Asger Dirksen, Alice M. Turner
Eur Respir Monogr 2019; 85: 78-92
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Mechanisms of liver disease in AATD
Pasquale Piccolo, Sergio Attanasio, Nicola Brunetti-Pierri
Eur Respir Monogr 2019; 85: 93-104
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Clinical approach to paediatric liver disease
Jeffrey H. Teckman, Dhiren Patel
Eur Respir Monogr 2019; 85: 105-113
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Clinical approach to liver disease in adults with AATD
Pavel Strnad, Carolin V. Schneider, Virginia Clark
Eur Respir Monogr 2019; 85: 114-126
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Rare manifestations of AATD
Alessandro N. Franciosi, Tomás P. Carroll, Noel G. McElvaney
Eur Respir Monogr 2019; 85: 127-142
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Genetic counselling
Joanna Chorostowska-Wynimko, Aleksandra Jezela-Stanek
Eur Respir Monogr 2019; 85: 143-149
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Registries: roles, objectives and registry data study outcomes
Iris G.M. Schouten, Jan Stolk, Jan Fuge, Marise J. Kasteleyn, Sabina Janciauskiene
Eur Respir Monogr 2019; 85: 150-158
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Future directions: diagnostic approaches and therapy with AAT
Sabina Janciauskiene, Tobias Welte
Eur Respir Monogr 2019; 85: 159-178
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Case 1
Jeffrey H. Teckman, Dhiren Patel
Eur Respir Monogr 2019; 85: 179-183
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Case 2
Robert Bals
Eur Respir Monogr 2019; 85: 184-186
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Case 3
Ali Ataya
Eur Respir Monogr 2019; 85: 187-191
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Case 4
Carolin V. Schneider, Pavel Strnad
Eur Respir Monogr 2019; 85: 192-195
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