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The CF Foundation’s Mission
Thomas G. Keens, M.D.Professor of Pediatrics, Physiology and Biophysics,
Keck School of Medicine of the University of Sothern !alifornia.
Division of Pediatric Plmonology and Sleep Medicine,
!hildren"s #ospital $os %ngeles.
&'mail(
)o !onflicts of *nterest to Disclose
Mechanisms of
Plmonary Disease
)+-B( %dvanced Pathophysiology.
%dvanced )rse Practice Program.
U!$% School of )rsing. cto/er +0, +-01.
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2espiratory
Physiology and
Pathophysiology
)+-B( %dvanced Pathophysiology.
%dvanced )rse Practice Program.
U!$% School of )rsing. cto/er +0, +-01.
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The maintenance
of normal arterial
o3ygen tension4Po+5, car/on
dio3ide tension
4Pco+5, and p#,6ithot e3cessive
cardiac or
plmonary 6ork.
)ormal $ng7nction
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• %de8ate
9entilation.
• %de8ate
Perfsion.
• Distri/tion of
9entilation.
• Diffsion of + and!+ across the
alveolar'capillary
mem/rane.!omroe, :.#., et al. The Lung. ;ear BookMedical P/lishers, !hicago, 0<=+.
%de8ate Gas &3change
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!omroe, :.#., et al. The Lung. ;ear BookMedical P/lishers, !hicago, 0<=+.
•)on'niform
distri/tion of
ventilation is
the mostimportant
case of gas
e3changea/normalities
in hman lng
disease.
%de8ate Gas &3change
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)etter, 7.#. Respiratory System. 9olme >, !*B%!ollection of Medical *llstrations. 0<><.
•%ir6ays(
•+ generations.
•-'0=( condcting
air6ays.•0>'+( respiratory
/ronchioles.
•%lveoli(
•--'1-- million
alveoli.
•Gas e3change
srface.
Strctre ofthe $ngs
7/17/2019 Pulmonary Pathophysiology
http://slidepdf.com/reader/full/pulmonary-pathophysiology-568c25a9a550f 9/146Berne, 2.M., and M.). $evy. Principles of Physiology. Mos/y, St. $ois, +---.
7/17/2019 Pulmonary Pathophysiology
http://slidepdf.com/reader/full/pulmonary-pathophysiology-568c25a9a550f 10/146Berne, 2.M., and M.). $evy. Principles of Physiology. Mos/y, St. $ois, +---.
7/17/2019 Pulmonary Pathophysiology
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• Site of most gas e3change.
• %dlts have --'1-- million
alveoli.• + and !+ e3change is /y
diffsion across the alveolar
capillary mem/rane.
• Thin mem/rane -.1'0.- m thick.
%lveoli
7/17/2019 Pulmonary Pathophysiology
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7/17/2019 Pulmonary Pathophysiology
http://slidepdf.com/reader/full/pulmonary-pathophysiology-568c25a9a550f 13/146Tisi, G.M. Pulmonary Physiology in Clinical Medicine. ?illiams and ?ilkins, Baltimore, 0<@-.
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%lveolar srface area of adlt lngs A >1 M+ A <- s8are
yards. 40.> C0=-thE the area of a college foot/all field5.
L
A
%lveolar Srface %rea
of %dlt $ngs
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!omroe, :.#., et al. The Lung. ;ear Book Medical P/lishers, !hicago, 0<=+.
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!ompensation for
2egional #ypo3ia
• ne can not correct regional
↓
Po+ /y↑
9F in another
lng region.
•Ths,↓
Po+ is the most
sensitive inde3 of the
presence of lng disease.
• ne can ↑Po+ /y ↑+
entering the hypo3ic region,
/y↑
7*o+.
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Tisi, G.M. Pulmonary Physiology in Clinical Medicine. ?illiams and ?ilkins, Baltimore, 0<@-.
7/17/2019 Pulmonary Pathophysiology
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Tisi, G.M. Pulmonary Physiology in Clinical Medicine. ?illiams and ?ilkins, Baltimore, 0<@-.
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Types of Respiratory Dead Space
!omroe, :.#., et al. The Lung. ;ear Book Medical P/lishers, !hicago, 0<=+.
Types of 2espiratory Dead Space
!ondcting %lveoli 6ith
%ir6ays )o Bloodflo6
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•Diffsion defect.
•#ypoventilation.
•Decreased 9F.
•2ight'to'$eft shnt.
!ases of #ypo3emia
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•#ypoventilation.
•Decreased 9F.
!ases of #ypercapnia
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%ir6ay/strction
Decreased!ompliance
Distri/tion of 9entilation
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•!ompliance.
•2esistance.
!ompliance A
9olme
Pressre
2esistance A
Pressre
7lo6
Plmonary Mechanics
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•Slope of the line is
compliance.
•The pressre'
volme crve of the
lng is not a line,
/t a loop.
•2eferred to as
hysteresis.Tisi, G.M. Pulmonary Physiology in Clinical Medicine.?illiams and ?ilkins, Baltimore, 0<@-.
Plmonary
!ompliance 4!$5
!$ A 9olme
Pressre
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Tisi, G.M. Pulmonary Physiology in Clinical Medicine. ?illiams and ?ilkins, Baltimore, 0<@-.
!$ = 9olme
Pressre
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!omroe, :.#., et al. The Lung. ;ear Book Medical P/lishers, !hicago, 0<=+.
2esistance A Pressre
7lo6
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&3trathoracic Upper %ir6ay /strction
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*ntra Plmonary %ir6ay /strction
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Tisi, G.M. Pulmonary Physiology in Clinical Medicine. ?illiams and ?ilkins, Baltimore, 0<@-.
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P % ti
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Sasaki, #., et al. J. Appl. Physiol., 4! @1@'@=<, 0<>@.
Pressre %cting
on %ir6ays
%i / t ti
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%ir6ay /strction
!herniack, 2.M., et al. Respiration in "ealth and #isease. ?.B. Sanders, Philadelphia, 0<>+.
# i fl ti
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#yperinflation
!herniack, 2.M., et al. Respiration in "ealth and #isease. ?.B. Sanders, Philadelphia, 0<>+.
%t l t i
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%telectasis
!herniack, 2.M., et al. Respiration in "ealth and #isease. ?.B. Sanders, Philadelphia, 0<>+.
%i / t ti
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• *ncreased air6ay resistance.• Bronchospasm.
• %ir6ay *nflammation.
• Mcos plgging.
• &3trinsic compression of the air6ay.
• 7oreign /ody in the air6ay.
• Decreased &lastic 2ecoil.
• &mphysema.
%ir6ay /strction
%i * fl ti
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!ases air6ayo/strction /y(
•Mcosal edema.
• Mcos plgging.
•*ncreased mcos
secretions.
• Bronchospasm.
!ommon in(
• %sthma.
• !hronic
/ronchitis.
• !ystic
7i/rosis.
%ir6ay *nflammation
%i / t ti
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!herniack, 2.M., et al. Respiration in "ealth and #isease. ?.B. Sanders, Philadelphia, 0<>+.
%ir6ay /strction
& h
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Tisi, G.M. Pulmonary Physiology in Clinical Medicine. ?illiams and ?ilkins, Baltimore, 0<@-.
&mphysema
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)etter, 7.#. Respiratory System. 9olme >, !*B% !ollection of Medical *llstrations. 0<><.
$ng
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2est &3ercise
$ng
Base
$ng
%pe3
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)+-B( %dvanced Pathophysiology
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)+-B( %dvanced Pathophysiology.
%dvanced )rse Practice Program.
U!$% School of )rsing. cto/er +0, +-01.
PlmonaryDisorders
P l Di d
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Plmonary Disorders
•Pnemonia.
•%spiration
pnemonia.
• *nflena pnemonia.
• T/erclosis.
•Plmonary fi/rosis.
•Bronchiolitis.
•%sthma.
•!hronic /ronchitis.
•&mphysema.
•Bronchiectasis.
•!ystic fi/rosis.
•Pnemothora3.
•Plmonary edema.
•Plmonaryem/ols.
P i
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• *nfection of the lng.• %lveoli are filled 6ith
infected material.
• 2e8ires treatment
of the infection 6ith
anti/iotics.
• Systemic
anti/iotics are
sally re8ired.
Pnemonia
Pne monia
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• *nfection may alsocase(
• Bronchospasm
re8iring/ronchodilators.
• ↑ plmonary
capillarypermea/ility
casing edema.
Pnemonia
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)ormal !hest H'ray
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Pnemonia
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Pnemonia
2adiographic Patterns
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2$$
2U$
2M$
$U$
$$$
2adiographic Patterns
of $o/ar *nfiltrates
Pnemonia !aveats
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•2ecrrent pnemonias are not normal.
•!onsider nderlying disorders 6hich might
predispose the patient to I+ pnemonias(
• !hronic $ng Disease.
• 2ecrrent aspiration.
• Untreated asthma.
• !ystic 7i/rosis.• %/normal mcociliary clearance, host
defenses, or immne deficiency,
• !ongenital malformations.
Pnemonia !aveats
%spiration
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• %spiration occrs
most commonly in
patients 6ith
s6allo6ingdysfnction.
• &lderly.
•)erologicallyimpaired.
• Seires.
• G&2D.
%spiration
Pnemonia
%spiration Pnemonia
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2M$ is the most common site.
%spiration Pnemonia
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0<0@ *nflena pandemic killed as
many as 1-,---,--- people 6orld'
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*nflena 9irs
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*nflena Pnemonia
*nflena
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• %nnal ot/reaksin 6inter.
• #igh fever, mscle
aches.
• Pnemonia is the
sal case of
death.• %ntiviral agents
only partly
effective.
*nflena
Pnemonia
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T/erclosis
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• +,---,--- deaths per
year 6orld6ide.
• ne'third of 6orld"s
poplation infected.
• I@,---,--- ne6cases diagnosed
each year.
• Mltiple drg
resistant TB
emerging as a
6orld6ide p/lic
health threat.
T/erclosis
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T/erclosis 4Gohn comple35
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4 5
T/erclosis 4Gohn comple35
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T/erclosis
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Miliary T/erclosis
T/erclosis( !aseating Granloma
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T/erclosis( !aseating Granloma
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T/erclosis !avitary $ng Disease
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T/erclosis !avitary $ng Disease
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T/erclin Skin Test
T/erclin Skin Test
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• Diagnoses TB infection.
•Positive +'0+ 6eeks afterinfection.
• !riteria for positive TST.
*ndration Poplation 42isk5
1 mm*mmnocompromised
!ontact 6ith TBpatient.
0- mm2isk of dissemination.*ncreased e3posre to
TB disease.
01 mm )o risk factors.%merican %cademy of Pediatrics.
The Red $oo%. +--=.
T/erclin Skin Test
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• Diagnoses TB infection.
•Positive +'0+ 6eeks afterinfection.
• !riteria for positive TST.
*ndration Poplation 42isk5
1 mm*mmnocompromised
!ontact 6ith TBpatient.
0- mm2isk of dissemination.*ncreased e3posre to
TB disease.
01 mm )o risk factors.%merican %cademy of Pediatrics.
The Red $oo%. +--=.
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*nterstitial Pnemonitis
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•*f ntreated, interstitial
pnemonitis progresses to
fi/rosis, destroying alveolar
tisse and /lood vessels ↓
lng volmes and↓
!$.
•Patients have shortness of
/reath, tachypnea, and↓
e3ercise tolerance.
•May reslt from many types
of lng inJry.
and 7i/rosis
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Digital !l//ing
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!ommonly seen in chronic lng diseases
associated 6ith hypo3ia andor inflammation.
Digital !l//ing
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Plmonary 7i/rosis
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Plmonary 7i/rosis
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)ormal !hest !T Scan
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Plmonary 7i/rosis
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Plmonary 7i/rosis
*nterstitial Pnemonitis
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• 2estrictive lng disease.• Treatment is that of the
nderlying case.
• #igh dose systemiccorticosteroids sppress
active inflammation.
• ther chronic medications(
• %athioprine.
• !yclophosphamide.
•#ydro3ychlora8ine.
and 7i/rosis
Bronchiolitis
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•%cte, life'threatening
disorder in infants.
•*nfectios
inflammation of
/ronchioles >1'-- indiameter.
•2espiratory syncytial
virs 42S95.•#ypo3ia, air6ay
o/strction, and
hyperinflation.
o c o t s
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Bronchiolitis
%sthma
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• Most common chronic lng
disease.
• 01 of U.S. children.
•!haracteried /y episodes of
air6ay narro6ing reslting from
/ronchospasm and
inflammation.
• *nhalation is the rote of choicefor the administration of
medications, as it is associated
6ith the least side effects.
%sthma
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&3pert Panel 2eport ( &uidelines for the #iagnosis and Management of Asthma, +-->.
*nflammation
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•%ir6ay mcosal inflammation
cases s6elling of the air6ay
lining and mcos secretion.
• 2elease of inflammatorymediators.
• !ytokines.
• $ekotrienes.
• Primary target to control asthma
symptoms.
*nflammation
)e/liers
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)e/liers
Metered Dose *nhalers
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!hronic Bronchitis
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•!hronic infection and
inflammation of the/ronchi and /ronchioles.
•)arro6ing of air6ays de
to /ronchospasm,inflammation, and
infection.
•Bronchiectasis( air6ayslose cartilaginos
spport collect infectios
materials.
)etter, 7.#. Respiratory System. 9olme >, !*B% !ollection of Medical *llstrations. 0<><.
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!hronic Bronchitis
4LBle Bloater5
Netter, F.H.
Respiratory
System. Volume
7, CIBA Collection
of Medical
Illustrations.
1979.
!hronic Bronchitis
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Treatment.• Bronchodilators to treat
/ronchospasm.
• *nhaled corticosteroids totreat inflammation.
•%nti/iotics to treat
infection.
• Spplemental + to treat
hypo3ia.
&mphysema
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• Destrction of alveolar
6alls.
• Decreased srface area
availa/le for gas
e3change.
• Decreased elastic
spport of air6ays.
• %ir6ay o/strction.
• Severe hyperinflation
often present.
)etter, 7.#. Respiratory System. 9olme >, !*B% !ollection of Medical *llstrations. 0<><.
&mphysema
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Tisi, G.M. Pulmonary Physiology in Clinical Medicine. ?illiams and ?ilkins, Baltimore, 0<@-.
Cest !"ra# of $m%#sema &'(in) (uffer*+
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)etter, 7.#. Respiratory System. 9olme >, !*B% !ollection of Medical *llstrations. 0<><.
%ir6ay /strction in
& h 4 P 5
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Tisi, G.M. Pulmonary Physiology in Clinical Medicine. ?illiams and ?ilkins, Baltimore, 0<@-.
&mphysema 4↓Pel5
&mphysema
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Treatment.• Bronchodilators to reverse
/ronchospasm.
• !orticosteroids forinflammation.
• %nti/iotics to treat infection.
• Diretics and digitalis to
treat right heart failre 4cor
plmonale5.
p y
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% 0+'year old /oy
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6as che6ing on his
pencil eraser tip.#e accidentally
s6allo6ed it.
#e coghed for
+'months.
Did he really
s'allo' the eraser
tipN
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2etained 7oreign Body%spiration
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• 7oreign /ody pts pressre on air6ay mcosa
compromises /loodflo6, and destroys air6ays.
• !hronic infection sets in, casing frther air6ay
destrction.
%spiration
Bronchiectasis
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• %/normal dilatation of
/ronchi.
• *nvolves destrction of
cartilaginos 6alls.
• Most commonly de to
chronic infection.
• !an /e focal or diffse.
• %l6ays de to an
nderlying lng disease.
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Bronchiectasis
Bronchiectasis
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• !ommon in chronic lng
diseases.•!ystic 7i/rosis.
•Primary ciliary dyskinesia.
•*mmne deficiencies.•T/erclosis.
• !hronic infection destroys
cartilaginos air6ays.• /strctive lng disease.
• 2isk of massive
hemoptysis.
L? th hild h
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L?oe the child 6ho
tastes salty from a kiss on the /ro6,
for he is he3ed,
and soon mst die. ''' Medieval German 7olk Saying
!ystic 7i/rosis
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• Most common inherited
serios e3ocrinedisorder in !acasians.
• %tosomal recessive.
• Mltiple systems
involved.
• Most serios clinicalpro/lem and most
common case of death
is !7 lng disease.
• Discovery of the
!ystic 7i/rosis
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!ystic 7i/rosis
gene in 0<@<.
• !ystic 7i/rosis
Transmem/rane
!ondctance
2eglator4!7T25 Protein.
• !hloride
channel ine3ocrine cells.
2ommens, :.M., et al.
Science, (4! 0-1<'0-@-, 0<@<.
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Normal CF
Cl"H-
Na
L
Na
S L
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H-
A S L
H-
H-H-
A S
Matsi, #., et al. Cell, )! 0--1'0-01, 0<<@.
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!7 Bronchiectasis
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!7 Bronchiectasis
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!7 Bronchiectasis
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!7
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!ystic 7i/rosis
!ystic 7i/rosis#ome Plmonary Therapy
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Median Srvival for !7 *mproves
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0<O- 0<1- 0<=- 0<>- 0<@- 0<<- +--- +-0- +-+--
0-
+-
-
O-
1-
%ge 4years5
Pnemothora3
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Pnemothora3 can arise from
rptred /llae or /le/s
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Pnemothora3
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Pnemothora3
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Pnemothora3 in an *nfant
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Tension Pnemothora3
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7lid &3change inPlmonary !apillaries
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•The alveolar'capillary
mem/rane is poros.
•7lid e3change canoccr across themem/rane.
•*n the lng, flidshold remain in thecapillary, not in thealveols.
•?hat forces keep flidin plmonarycapillariesN
Tisi, G.M. Pulmonary Physiology in Clinical
Medicine. ?illiams and ?ilkins, Baltimore, 0<@-.
7lid &3change inPlmonary !apillaries
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Tisi, G.M. Pulmonary Physiology in Clinical Medicine. ?illiams and ?ilkins, Baltimore, 0<@-.
!apillary %lveols
•↑ !apillary
hydrostaticpressre.
•↑ %lveolar
srface
tension.
•↑ !olloid
osmoticpressre.
•↑%lveolar
pressre.
7lid &3change inPlmonary !apillaries
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Tisi, G.M. Pulmonary Physiology in Clinical
Medicine. ?illiams and ?ilkins, Baltimore, 0<@-.
• ↑↑ capillary
hydrostaticpressre pshes
flid into alveoli,
casing plmonary
edema.
• ↑ capillary
hydrostatic
pressre pshesflid into interstitial
space, casing stiff
lngs.
Plmonary $ymphatics
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• 7lid is cleared
from the interstitialspace /y
lymphatics.
•Plmonary
lymphatics drain
throgh the hilm
to the thoracic dctin the sperior
vena cava.)etter, 7.#. Respiratory System. 9olme >, !*B%
!ollection of Medical *llstrations. 0<><.
#eart 7ailre andPlmonary &dema
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/isi, 0.M. Pulmonary Physiology in Clinical
Medicine. illiams and il)ins, Baltimore, 1923.
$eft #eart 7ailre.
• Blood /acks p from theleft ventricle into the
lngs.
• *ncreases capillary
pressre.
2ight #eart 7ailre.
• Blood /acks p from
the right ventricle into
systemic veins.
• ↓ Thoracic dct flo6.
• ↓ Plmonary flid
clearance
•!lot travels from venos
Plmonary &m/olism
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•!lot travels from venos
system to the lngs.
•!an case infarction of a
portion of lng.
•Massive P& can occlde
most or all of plmonaryotflo6 tract.
•!an case 2ight #eart
7ailre.
•!an case sdden death.
•2e8ires prompt diagnosis
and treatment.
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•Deep venos throm/osis in
Plmonary &m/olism
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•Deep venos throm/osis in
femoral veins is a common case.
•ther risk factors are
immo/iliation of legs for
prolonged periods.
•Signs and Symptoms(
•!ogh.
•!hest pain.
•Dyspnea.
•#ypo3ia.
•Treatment is anticoaglation.
%lthogh 6e have revie6ed a
$ng 2esponse to *nJry
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%lthogh 6e have revie6ed a
nm/er of specific lng
diseases, the lng has only a
fe6 6ays to respond to
inJry.
• *nflammation.
• %ir6ay o/strction,
remodeling, and
/ronchiectasis.
• 7i/rosis.
• $oss of alveoli.
• 2epair 4retrn to normal
?hy the 2espiratory System isthe most important organ system
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LTo kno6 even
one life has
/reathed easier
/ecase yohave lived ' that
is to have
scceeded.
2alph ?aldo &merson
0@-'0@@+
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