Encyclopedia of respiratory medicine

2401 Pages·2019·41.376 MB·English

by  Laurent G., Shapiro S. (eds.)

INTRODUCTION
R espiratorydiseasesrepresentoneofthelargesthealthproblemswordwide.Diseasessuchasasthmaand
the smokingrelated diseases are alreadycommonand increasingsowe urgentlyneedbetterapproaches
totreatorcurethesediseases.Atthesametime,newrespiratorydiseasessuchasthoseassociatedwithviruses
threaten pandemics that challenge our national health systems.
Withthesecontinuedchallengesfornewtreatmentwithbetterpatientcare,clinicalandrespiratoryresearchers
have sought better approaches to all aspects of patient care from improved diagnoses to superior therapies.
This has lead to an explosion of new research with an increasingly better understanding of how to diagnose
diseases and then develop new therapies. Thus, for example ever improving technologies for imaging lung
disease have lead to increasingly better diagnoses, although challenges remain as we seek to further improve
resolution. At the same time, the revolution in molecular biology, culminating with the publication of the
completehumangenome,hasleadtohopesforfindingmoreprecisecluestodiseasesusceptibilitypathogenesis
in genetic analysis. This is leading to new concepts in pharmacogenomics as we start to use new drugs,
including those used for lung cancers, being directed at mutations associated with disease.
ThisisthefirstEncyclopaediaofRespiratoryMedicine.Itisourhopethatitiscomprehensiveandcaptures
the key aspects of current patient care, as well as the exciting developments in respiratory science that we all
believe will eventually lead to better patient care in the twenty first century.
This encyclopaediaiscomprehensive inscopeandprovidesclinicianandresearcherwithasnapshotofthe
current state of knowledge in respiratory medicine. All entries have adhered to a structured layout, starting
withanabstractcrystallizingthekeyfactsandfinishingwithreadinglistsforthosewhowanttodelvefurther
intothesubject.Inaddition,mostentrieshaveacolourdiagramdesignedtohelpunderstandingandprovidea
valuable aid for undergraduate and post-graduate teaching.
Theseareexcitingtimesforrespiratorymedicine.Wehopethisencyclopediawillbecomeavalubletoolfor
clinicians and researches at all stages of their careers from those beginning their carreers to those established
but wanting to update themselves on the new developments.
Finally,wewouldliketothankourAdvisoryEditorialBoardwhohelpedsomuchinshapingthecontentsof
this works, as well as the authors who wrote the articles and faced the challenge of condensing areas of
respiratory medicine, often the subject of entire textbooks, into a short article of 4000 words or less.
GEOFFREY J. LAURENT
STEVEN D. SHAPIRO
FOREWORD
Animalslivebytwoprincipalthings,foodandbreath.Ofthese,byfarthemostimportantistherespiration,forifitisstopped,theman
willnotendurelong,butimmediatelydies.–AretaeustheCappocian(150–200AD)
O f course, not all medical specialists would agree with this statement, and those who disagree would be
quick to posit that it is the failure of ‘‘their’’ particular organ that tends to cause immediate death.
However,thatisnottheissue.Thepointofthisquotationistoillustratethattheproperfunctioningofthelung
has been a subject of great interest for centuries. The Greek physician Aretaeus devoted many of his
observations to diabetes, but his manuscript ‘‘On the Causes and Indications of Acute and Chronic Diseases’’
also discussed lung diseases, such as pneumonia. Since his time, great numbers of physicians from all conti-
nents and cultures have contributed to our knowledge of respiratory diseases.
While acknowledging our rich history of discoveries about pulmonary and respiratory medicine— dis-
coveries that were made by men and women whose names symbolize the great journey of this specialty—one
must concede that the field experiencedanextraordinarygrowthspurt beginninginthe 1940s.Knowledgeof
respiratory physiology, which developed very fast during World War II, created a tidal wave of interest that
continued for years afterward. The ability to measure and understand respiratory physiology and its alter-
ations became a diagnostic tool, and it opened the door to therapeutic or respiratory support procedures.
But, then, in the 1950s and 1960s cell biology and subcellular research entered the scene. The potential of
molecular biology and genetics was quickly recognized, and respiratory medicine appreciated that a better un-
derstanding of normal and disordered biological respiratory processes hinged on use of these new approaches.
Lung and respiratory researchers, impelled in part by the ever-increasing public health burdens of respiratory
diseases, seized the opportunity. The stage was set for progress to occur. The architects of this ‘‘revolution’’ in
respiratory medicine are well known; it is our good fortune that many have contributed to these four volumes.
Four volumes! y Encyclopedia! y Indeed, these four volumes truly constitute an encyclopedia of pulmo-
nary biology and respiratory medicine!
Respiratorymedicineisstillgrowing.Becauseitissuchadynamicandexcitingfield,newinvestigatorswill
almost surely want to be part of it. However, to do so they will need to know about the established state of
knowledge that will be the basis of their work. New investigators in the science of respiratory medicine,
whetherinterestedinfundamentalresearchorclinicalresearchorapplication,willfindideasandinspirationin
these volumes. All of the tools of the trade are assembled therein.
Asnoted,respiratorymedicinehasbeenaprogressiveandexpandingfieldbut,asisthecasewithmanyfields
of medicine, the transfer of what we know to the general practice of medicine has been slow and limited.
Translation, as it is called, is an emerging discipline in need of assistance; fortunately, the breadth of the
knowledge presented in these volumes provides tools to facilitate this translation process.
Thisfour-volumeencyclopediais,atonce,bothatributetothecenturiesofpioneeringinvestigationsinthe
fieldofrespiratorymedicineandafoundationforevengreateraccomplishmentsinthefuture.Thepresentation
ofallthisknowledgeintheseexcellentandcomprehensivevolumescanonlyservetostimulatefurtherworkof
equal or surpassing significance. The editors and the authors are to be commended for their contributions to
this singular effort. Because of their work, respiratory science and medicine will advance faster and patients
worldwide will be the beneficiaries.
Claude Lenfant, MD
Gaithersburg, Maryland
Notes on the Subject Index
To save space in the index, the following abbreviations have been used:
ALI acute lung injury
ARDS acute respiratory distress syndrome
BAL bronchoalveolar lavage
BPD bronchopulmonary dysplasia
CAP community-acquired pneumonia
CFTR cystic fibrosis transporter regulation
COP cryptogenic organizing pneumonia
COPD chronic obstructive pulmonary disease
CWP coal workers’ pneumoconiosis
G-CSF granulocyte colony-stimulating factor
GERD gastroesophageal reflux disease
GM-CSF granulocyte-macrophage colony-stimulating factor
HUVS hypocomplementemic urticarial vasculitis syndrome
IL interleukin
IPF idiopathic pulmonary fibrosis
IPH idiopathic pulmonary hemosiderosis
MCP monocyte chemoattractant protein
M-CSF macrophage colony-stimulating factor
MIP macrophage inflammatory protein
MMP matrix metalloproteinase
NSCLC non-small cell lung carcinoma
PPAR peroxisome proliferator-activated receptor
SCLC small-cell lung carcinoma
SP surfactant protein
TGF transforming growth factor
TIMP tissue inhibitor of metalloproteinases
TNF tumor necrosis factor
VEGF vascular endothelial growth factor
Editorial Advisory Board
Kenneth B. Adler, North Carolina State University, Raleigh, NC, USA
Peter J. Barnes, Imperial College London, UK
Paul Borm, Zuyd University, Heerlen, The Netherlands
Arnold R. Brody, Tulane Medical School, New Orleans, LA, USA
Rachel C. Chambers, University College London, UK
Augustine M. K. Choi, University of Pittsburgh, PA, USA
Jack A. Elias, Yale University School of Medicine, New Haven, CT, USA
Patricia W. Finn, University of California San Diego, La Jolla, CA, USA
Stephen T. Holgate, University of Southampton, Southampton, UK
Steven Idell, The University of Texas Health Center at Tyler, TX, USA
Sebastian L. Johnston, National Heart and Lung Institute, Imperial college London, UK
Talmadge E. King, Jr, University of California, San Francisco, CA, USA
Stella Kourembanas, Children’s Hospital Boston, Harvard Medical School, Boston, MA, USA
Y. C. Gary Lee, University College London, UK
Richard Marshall, University College London, UK
Sadis Matalon, University of Alabama, Birmingham, AL, USA
Joel Moss, National Institutes of Health, Bethesda, MD, USA
William C. Parks, University of Washington, Seattle, WA, USA
Charles G. Plopper, University of California, Davis, CA, USA
Bruce W. S. Robinson, The University of Western Australia, Nedlands, Australia
Neil Schluger, Columbia University College of Physicians and Surgeons, New York, NY, USA
Edwin K. Silverman, Brigham and Women’s Hospital Boston, MA, USA
Eric S. Silverman, Brigham and Women’s Hospital, Boston, MA, USA
Peter Sly, Institute for Child Health Research, West Perth, Australia
Kingman Strohl, Case Western Reserve University, Cleveland, OH, USA
Teresa D. Tetley, Imperial College London, UK
John B. West, University of California, San Diego, CA, USA
Editors
Geoffrey J Laurent, Royal Free and University College Medical School, London, UK
Steven D Shapiro, Brigham and Woman’s Hospital, Boston, USA
Dedication
To my family, Lal, Guy, David and Gabrielle (GJL).
MycontributiontothisworkwouldnothavebeenpossiblewithouttheloveandsupportfrommywifeNicole
and my daughters Calli, Tess, Skylar, and Ellery. I also thank my mentors and trainees for my continual
education and theDivision ofPulmonary andCriticalCare Medicine atBrigham and Women’s Hospital who
took care of our patients allowing me the time to undertake this project (SDS)
Permission Acknowledgments
The following material is reproduced with kind permission of Lippincott Williams and Wilkins
Figure 4 and 8 of ARTERIAL BLOOD GASES
Table 1 of ARTERIES AND VEINS
Figure 2 and 3 of BREATHING | Breathing in the Newborn
Figure 2a, 2b, 3, 4 and 5 of DRUG-INDUCED PULMONARY DISEASE
Table 1, 2 and 3 of DRUG-INDUCED PULMONARY DISEASE
Figure 2 of ENVIRONMENTAL POLLUTANTS | Diesel exhaust particles
Figure 4 of EXERCISE PHYSIOLOGY
Figure 2 of FLUID BALANCE IN THE LUNG
Figure 2 of GASTROESOPHAGEAL REFLUX
Figure 2 of GENE REGULATION
Figure 2 of HIGH ALTITUDE, PHYSIOLOGYAND DISEASES
Figure 2 and 3 of IDIOPATHIC PULMONARY HEMOSIDEROSIS
Table 1 of IDIOPATHIC PULMONARY HEMOSIDEROSIS
Figure 1, 2 and 3 of OXYGEN-HEMOGLOBIN DISSOCIATION CURVE
Figure 10a, 10b and 11a of SYSTEMIC DISEASE | Eosinophilic Lung Diseases
http://www.lww.com
The following material is reproduced with kind permission of Nature Publishing Group
Figure 2 of COAGULATION CASCADE | iuPA, tPA, uPAR
Figure 1 of COAGULATION CASCADE | Tissue Factor
Figure 1a of MATRIX METALLOPROTEINASES
Figure 1 of MYOFIBROBLASTS
2
Figure 1 of VESICULAR TRAFFICKING
http://www.nature.com/nature and http://www.nature.com/reviews
The following material is reproduced with kind permission of Taylor & Francis Ltd
Figure 2 of AUTOANTIBODIES
Table 1 of BASAL CELLS
Figure 1 of NEUROPHYSIOLOGY | Neuroendocrine Cells
Table 1 of NEUROPHYSIOLOGY | Neuroendocrine Cells
Figure 1 and 2 of SURFACANT | Overview
Tables 1, 2, 3 and 4 of SURFACANT | Overview
http://www.tandf.co.uk/journals
A
ACETYLCHOLINE
J Zaagsmaand HMeurs,Universityof Groningen, vagosympathetic trunk; when applied to a second,
Groningen, TheNetherlands unstimulated heart, the perfusate slowed its rate,
&2006ElsevierLtd.Allrightsreserved. resembling the effect of vagus stimulation. In
1926 Loewi provided evidence for identification of
Vagusstoff as acetylcholine. Acetylcholine is the
Abstract
neurotransmitter of all sympathetic and parasympa-
Intheairways,acetylcholineisaneurotransmitterinparasym- thetic autonomic ganglia and of the postganglionic
patheticgangliaandinpostganglionicparasympatheticnerves, parasympatheticnerves.Intheairways,theparasym-
aswellasanonneuralparacrinemediatorinvariouscellsinthe
patheticgangliaarelocatednearorwithintheairway
airwaywall.Ganglionictransmissionbyacetylcholineismedi-
wall. Ganglionic transmission mediated by acetyl-
atedbynicotinicreceptors,whichareligand-gatedinchannels,
whereaspostganglionictransmissionisthroughG-protein-cou- cholineisthroughnicotinicreceptorswhichbelongto
pled muscarinic receptors. Of the five mammalian muscarinic the family of ligand-gated ion channels. Postgan-
receptor subtypes, mainly M1, M2, and M3 receptors are in- glionic transmission by acetylcholine, released from
volvedinairwayfunctions.G -coupledM receptorsfacilitate
q 1 parasympathetic nerve terminals, is through mu-
ganglionic transmission mediated by nicotinic receptors and
scarinic receptors of which five different subtypes
modulatesurfactantproduction andfluid resorption in theal-
veoli. Prejunctional G -coupled M receptors in parasympa- have been identified, all being G-protein-coupled
i/o 2
theticnerveterminalsattenuateacetylcholinereleaseuponnerve receptors. During periods of airway inflammation
stimulation.M2receptorsarealsoabundantlypresentinairway vagal release of acetylcholine may be increased by
smoothmuscle;however,themajorfunctionofthesepostjunct-
various mechanisms. Hence, both in asthma and
ionalM receptorsisunknown.PostjunctionalG -coupledM
2 q 3 (particularly) in chronic obstructive pulmonary dis-
receptorsmediateairwaysmoothmusclecontractionandmucus
secretion.DysfunctionoftheprejunctionalM autoreceptorin- ease (COPD) blockade of postjunctional muscarinic
2
ducedbyallergicairwayinflammationhasbeenimpliedinex- receptors is the key to reversing airway obstructions.
aggeratedvagalreflexactivityandairwayhyperresponsiveness
in asthma. Inflammation-induced increased M receptor stim-
3
ulation may be involved in airway remodeling in chronic
Synthesis, Storage, and Release
asthma. Possible mechanisms include potentiation of growth
factor-inducedproliferationofairwaysmoothmusclecellsand Acetylcholine is synthesized from choline and acetyl-
inductionofacontractilephenotypeofthesecells.Exaggerated
coenzyme A (acetyl-CoA) in the cytoplasm of the
M receptor stimulation may also cause reduced responsive-
3
ness to b -adrenoceptor agonists by transductional cross-talk nerve terminal through the enzyme choline acetyl-
2
between phosphoinositide metabolism and adenylyl cyclase, transferase (ChAT). Choline is taken up by the nerve
whichinvolvesproteinkinaseC-induceduncouplingoftheb2- terminal from the extracellular fluid through a so-
adrenoceptorfromtheeffectorsystem.Muscarinicreceptoran- dium-dependent carrier; this transport is the rate-
tagonistshavebeenshowntobeeffectiveinairwaydiseaseslike
limitingprocessinacetylcholinesynthesis.Acetyl-CoA
asthmaand,especially,chronicobstructivepulmonarydisease.
is synthesized in mitochondria which are abundantly
Of these, tiotropium bromide is particularly useful, due to its
longdurationofactionaswellasitskineticselectivityforthe present in the nerve endings. Most of the synthesized
M3receptor. acetylcholine is actively transported from the cytosol
into synaptic vesicles by a specific transporter; this
vesicular (‘quantal’) package of acetylcholine reaches
Introduction
up to 50000molecules per vesicle.
Acetylcholineisaneurotransmitterinthecentraland Release of acetylcholine is initiated by influx of
peripheralnervoussystemwhereitplaysamajorrole Ca2þ ionsthroughvoltage-operatedN-orP-typecal-
in the afferent neurons of both the autonomic cium channels. The increased intracellular Ca2þ ions
and somatic (voluntary) branches. As a chemical bind to a vesicle-associated protein (synaptotagmin)
transmitter, it has been identified as ‘Vagusstoff’ in which favors association of a second vesicle protein
1921 by Otto Loewi showing its release from (synaptobrevin) with one or more proteins in the
an isolated frog heart following stimulation of the plasma membrane of the nerve terminal. Following