CASE PRESENTATION

MILTON
201001014

Name: wu yiyun
Age:38
Sex: male
Occupation: Businessman (sells
aluminum products)

CC: Cough for the past 7 months

 HOP: A 38 years old male showed up to

the outpatient department one month
ago , complaining of intermittent cough
for the past seven months without any
chills or fever. He also noted that the
cough was accompanied with a lot of
green thick sputum in the morning. He
was also having dyspnea after a bout of
heavy work.
The patient denied any chest discomfort,
hemoptysis, weight loss, night sweats. He
has good appetite and sufficient sleep.

 During the early onset of disease the

patient went to a local doctor and
was given some cough suppressant
drugs, however, he could not
remember the names of those drugs.
After using them for several days,
the cough was relieved. However,
after a few days it appeared again as
a mild cough. One month ago, the
cough intensified and he came to our
hospital for treatment.

PE:
Social
history:

- T=37.5C
20
years
smoking ( 1 pack a day)
- Pulse=
95 of
beats/min
- HR= 95 beats/min
20
years of drinking beer (2-3 glasses
- RR= 20 beats/min
/day)
- BP= 118/90 mmHg

Diet:nodes
seafood
Lymph
= non (mainly)
palpable
Trachea = in normal position
Cyanosis = no
Clubbing = no
Jvp = normal
Breath sound = normal
Paleness = no
Abdomen = soft and non palpable, no pain, no ascites
Neurological sign = normal

TESTS
WHAT TESTS WOULD YOU RECOMMEND?
CBC
Pas stain
Acid fast stain
CXR
PFT
bronchioalveolar lavage
ABG
Biopsy
Tumor marker test
CT

Lab test: CBC

CRP1=21
WBC=5.9
NE=0.642
LYMP=0.249
MON0= 0.090
EO= 0.016
BASO= 0.003
GGT=130 U/L

ABG:
MARKER:
T=36.8C
FIO2= 21
17.88
PH= 7.38
PCO2= 45.3
PO2=41.7
HCO3=26.5
STHCO3= 25.3
BE= 1.5
SBE= 1.9
CTO2 =5.7
TCO2= 24.2
SO2 =76.7
AADO2= 53.7

TUMOR
CEA=

 What conditions can cause elevated

CEA?
Benign and malignant cancers
Cancers of the colon
Thyroid and ovarian cancers
Inflammatory bowel disorder
Liver cirrhosis
Chemotherapy and radiation therapy
Smokers

 CT

 CT

 CT

 CT

 What is the diagnosis?

 What are the differential diagnosis?

ARDS
Bacterial pneumonia
Acute interstitial pneumonia
Pulmonary edema
TB
Sarcoidosis
Bronchioalveolar carcinoma
Diffuse interstitial fibrosis

 Bacterial pneumonia ?
by: S. pneumonia

 What is ARDS?

 It is a severe life threatening medical

condition characterized by
inflammation in the lungs.
Pathology:
-diffuse damage to the alveolarcapillary interface leads to leakage of
protein-rich fluid causes edema and
formation of hyaline membranes in
alveoli.
CF: Hypoxemia, cyanosis

 What is cyanosis?

APPROACH TO CYANOSIS

Definition
Bluish discolouration of skin or
mucous membrane caused by
excess amounts of reduced
hemoglobin or abnormal
hemoglobin
4gm of reduced Hb in capillaries
required for cyanosis to be
apparent

Mechanism
caused by absolute increase in reduced Hb,higher
the Hb greater tendancy towards cyanosis

 In severe anemia , greater systemic

arterial desaturation required for
cyanosis to be evident
In polycythemia even lesser systemic
arterial oxygen saturation may result
in clinical cyanosis
If fetal Hb is high, tissue hypoxia may
occur even if cyanosis is
mild( arterial PaO2 low)

central

peripheral

CAUSE

ARTERIAL BLOOD
DESATURATION OR
ABNORMAL Hb

CUTANEOUS
VASOCONSTRICTION
DUE TO LOWCO

CONDITIONS

Seen in R-L shunt,

exposure to cold air
impaired pulmonary
or water and
function, abnormal Hb abnormally greater
extraction ofO2 from
normally saturated
blood

SITES

conjunctiva,palate,ton limited to
gue,inner side of
ears,nose,cheeks
lips& cheeks
outer side of lips
hands feet&digits
certainly central if
associated with
clubbing and
polycythemia,
probably central if it
deepens on effort

clubbing is absent

 ABG
What do you think the ABG would be in
this case?

ABG analysis & Acid-Base

Disorders

Outline
1.
2.
3.
4.

Discuss simple steps in analyzing ABGs

Calculate the anion gap
Calculate the delta gap
Differentials for specific acid-base disorders

Steps for ABG analysis

1.
2.
3.
4.
5.
6.
7.

What is the pH? Acidemia or Alkalemia?

What is the primary disorder present?
Is there appropriate compensation?
Is the compensation acute or chronic?
Is there an anion gap?
If there is a AG check the delta gap?
What is the differential for the clinical processes?

Normal Values
Variable
pH

Normal
Range
7.35 - 7.45

pCO2

35-45

Bicarbonate

22-26

Anion gap

10-14

Albumin

Step 1:
Look at the pH: is the blood acidemic or alkalemic?
EXAMPLE :
65yo M with CKD presenting with nausea,
diarrhea and acute respiratory distress

ABG :ABG 7.23/17/235 on 50% VM

BMP Na 123/ Cl 97/ HCO3 7/BUN 119/ Cr
5.1

ACIDMEIA OR ALKALEMIA ????

EXAMPLE ONE
ABG 7.23/17/235 on 50% VM
BMP Na 123/ Cl 97/ HCO3 7/BUN
119/ Cr 5.1
Answer PH = 7.23 , HCO3 7
Acidemia

Step 2: What is the primary

disorder?
What disorder is
present?

pH

pCO2 or HCO3

Respiratory Acidosis

pH low

pCO2 high

Metabolic Acidosis

pH low

HCO3 low

Respiratory Alkalosis

pH high

pCO2 low

Metabolic Alkalosis

pH high

HCO3 high

EXAMPLE
ABG 7.23/17/235 on 50% VM
BMP Na 123/ Cl 97/ HCO3 7/BUN 119/ Cr 5.
PH is low , CO2 is Low
PH and PCO2 are going in same directions then its
most likely primary metabolic will check to see if
there is a mixed disoder.

Step 3-4: Is there appropriate

compensation? Is it chronic or
Respiratory Acidosis
acute?

Acute: for every 10 increase in pCO2 -> HCO3 increases by 1

and there is a decrease of 0.08 in pH MEMORIZE
Chronic: for every 10 increase in pCO2 -> HCO3 increases by 4
and there is a decrease of 0.03 in pH

Respiratory Alkalosis

Acute: for every 10 decrease in pCO2 -> HCO3 decreases by 2

and there is a increase of 0.08 in PH MEMORIZE
Chronic: for every 10 decrease in pCO2 -> HCO3 decreases by 5
and there is a increase of 0.03 in PH

Step 3-4: Is there appropriate

compensation? Is it acute or
Metabolic Acidosis
chronic
?
Winters formula: pCO2 = 1.5[HCO3] + 8 2 MEMORIZE
If serum pCO2 > expected pCO2 -> additional respiratory
acidosis
Metabolic Alkalosis
For every 10 increase in HCO3 -> pCO2 increases by 6

EXAMPLE
ABG 7.23/17/235 on 50% VM
BMP Na 123/ Cl 97/ HCO3 7/BUN 119/ Cr 5.

Winters formula : 17= 1.5 (7) +8 = 18.5

So correct compensation so there is only
one disorder Primary metabolic

Step 5: Calculate the anion

gap
AG = Na Cl HCO3 (normal 12 2)
AG corrected = AG + 2.5[4 albumin]
If there is an anion Gap then calculate the
Delta/delta gap (step 6). Only need to calculate
delta gap (excess anion gap) when there is an anion
gap to determine additional hidden metabolic
disorders (nongap metabolic acidosis or metabolic
alkalosis)
If there is no anion gap then start analyzing for
non-anion acidosis

EXAMPLE
Calculate Anion gap
ABG 7.23/17/235 on 50% VM
BMP Na 123/ Cl 97/ HCO3 7/BUN 119/ Cr 5/
Albumin 4.

AG = Na Cl HCO3 (normal 12 2)
123 97 7 = 19

No need to correct for albumin as it is

4

Step 6: Calculate the different

needed
formulas
Delta gap = (actual AG 12) + HCO3
Adjusted HCO3 should be 24 (+_ 6) {18-30}
If delta gap > 30 -> additional metabolic alkalosis
If delta gap < 18 -> additional non-gap metabolic
acidosis
If delta gap 18 30 -> no additional metabolic
disorders

EXAMPLE : Delta Gap

ABG 7.23/17/235 on 50% VM
BMP Na 123/ Cl 97/ HCO3 7/BUN 119/ Cr 5/
Albumin 4.

Delta gap = (actual AG 12) +

HCO3
(19-12) +7 = 14
Delta gap < 18 -> additional non-gap
metabolic acidosis
So Metabolic acidosis anion and non
anion gap

Metobolic acidosis: Anion gap

acidosis

EXAMPLE: WHY ANION GAP?

65yo M with CKD presenting with nausea, diarrhea
and acute respiratory distress

ABG :ABG 7.23/17/235 on 50% VM

BMP Na 123/ Cl 97/ HCO3 7/BUN 119/ Cr
5.1

So for our patient for anion gap

portion its due to BUN of 119 UREMIA
But would still check lactic acid

Nongap metabolic acidosis

For non-gap metabolic acidosis, calculate the urine anion gap
UAG = UNA + UK UCL
If UAG>0: renal problem
If UAG<0: nonrenal problem (most commonly GI)

Causes of nongap metabolic acidosis - DURHAM

Diarrhea, ileostomy, colostomy, enteric fistulas
Ureteral diversions or pancreatic fistulas
RTA type I or IV, early renal failure
Hyperailmentation, hydrochloric acid administration
Acetazolamide, Addisons
Miscellaneous post-hypocapnia, toulene, sevelamer, cholestyramine ingestion

EXAMPLE : NON ANION GAP

ACIDOSIS
65yo M with CKD presenting with nausea,
diarrhea and acute respiratory distress

ABG :ABG 7.23/17/235 on 50% VM

BMP Na 123/ Cl 97/ HCO3 7/BUN 119/ Cr
5.1

Most likely due to the diarrhea

Metabolic alkalosis
Calculate the urinary chloride to differentiate saline
responsive vs saline resistant
Must be off diuretics in order to interpret urine chloride
Saline responsive
UCL<10

Saline-resistant UCL >10

Vomiting

If hypertensive: Cushings, Conns, RAS,

renal failure with alkali administartion

NG suction

If not hypertensive: severe hypokalemia,

hypomagnesemia, Bartters, Gittelmans,
licorice ingestion
Exogenous corticosteroid administration

Over-diuresis
Post-hypercapnia

Respiratory Alkalosis
Causes of Respiratory Alkalosis
Anxiety, pain, fever
Hypoxia, CHF
Lung disease with or without hypoxia pulmonary embolus, reactive
airway, pneumonia
CNS diseases
Drug use salicylates, catecholamines, progesterone
Pregnancy
Sepsis, hypotension
Hepatic encephalopathy, liver failure
Mechanical ventilation
Hypothyroidism
High altitude

Respiratory Acidosis
Causes of respiratory acidosis
CNS depression sedatives, narcotics, CVA
Neuromuscular disorders acute or chronic
Acute airway obstruction foreign body, tumor, reactive airway
Severe pneumonia, pulmonary edema, pleural effusion
Chest cavity problems hemothorax, pneumothorax, flail chest
Chronic lung disease obstructive or restrictive
Central hypoventilation, OSA

Steps for ABG analysis

1.
2.
3.
4.
5.
6.
7.

What is the pH? Acidemic or Alkalemic?

What is the primary disorder present?
Is there appropriate compensation?
Is the compensation acute or chronic?
Is there an anion gap?
If there is a AG, what is the delta gap?
What is the differential for the clinical processes?

 ABG
What do you think the ABG would be in
this case?

 PULMONARY FUNCTION TEST (PFT)

Pulmonary Physiology and Lung Function

Tests

Lung Function Testing (PFTs)

Spirometry (FV loop spirometry, dynamic
lung volumes)
Reversibility testing
Bronchial challenge testing
Static lung volumes (TLC, FRC)
Gas Transfer (DLCO, TLCO Transfer factor, KCO, )
Maximal respiratory pressures (Mouth
pressures)
CPEST (Cardiopulmonary exercise test)

General principles
Technical factors
operator
patient
within test repeatability

confounding factors

Quality control between test

repeatability
Appropriate reference values

Spirometry
Measures the ability to move air
rapidly
Depends on nervous system, musc skel,
skin + connective tissue, lungs, airways,
inhaled gas

How is the test done

Apparatus
true spirometers - volume & time
pneumotach, vane & hotwire anenometers
- flow

Method
Full inspiration, forced maximal expiration
Minimum 3 technically acceptable
attempts
within 5% repeatability FEV1 and FVC
Slow Vital Capacity may also be checked

Data generated
Volume time curve (spirogram)
FEV1, FVC, Ratio

Flow volume loop

Peak flow
FVC
FEF 25-75%
MEF 75, 50, and 25
Inspiratory flow data

NORMAL

Spirometry interpretation
Obstructive v. Restrictive
Mid flow obstruction
Shape of the FV loop
Obstruction v. restriction
Fixed large airway obstruction
Variable airway obstruction
Extrathoracic
Intrathoracic

Airflow obstruction
Mild on left
Severe on right

Stage I:

Classification of COPD
Severity
by
Spirometry
Mild
FEV /FVC < 0.70
1

FEV1 > 80% predicted

Stage II: Moderate

FEV1/FVC < 0.70

50% < FEV1 < 80% predicted

Stage III: Severe

FEV1/FVC < 0.70

30% < FEV1 < 50% predicted

Stage IV: Very Severe

FEV1/FVC < 0.70

FEV1 < 30% predicted or
FEV1 < 50% predicted plus

COPD
ID: CSM4166
Weight(kg): 79.0
PB: 753
SpirometryRef
FVC
4.86
FEV1
3.38
FEV1/FVC
70.0
FEF25-75%
3.11
9.02
PEF
Lung Volumes
TLC
RV
RV/TLC
FRC PL
ERV
VC
Resistance
Raw
sRaw
Diffusion
DLCO
DLCO /VA
VA

Date: 10/03/04
Height(cm): 184
Temp: 23
Pre
Pre Post
Meas % Ref
4.48 92
(1.61) (48)
(36.0)
(0.35) (11)
5.43 60

Gender: Male
BMI: 23.33
Post
Meas

Post
% Ref

Age: 62

% Chg

Comments:The patient could not fully expire during to FVC or SVC, therefore the results for both
vital capacities may be underestimated. See attached FV loops

Pulmonary restriction

Large Airway obstruction

Tracheal
Fixed obstruction expiratory and
inspiratory limitation
Variable obstruction
Inspiratory limitation indicates extrathoracic
obstruction
Expiratory limitation indictaes intrathoracic
obstruction

Variable extrathoracic

Large airway obstruction

Fixed

ID: CLV3379
Weight(kg): 82.0
PB: 765
Spirometry
Ref
FVC
4.93
FEV1
3.94
FEV1/FVC
79
FEF25-75%
4.31
PEF
8.95
Lung Volumes
TLC
RV
RV/TLC
FRC PL
ERV
VC
Resistance
Raw
sRaw
Diffusion
DLCO
DLCO /VA
VA

Fixed Extrathoracic Obstruction PFT

Date: 22/03/01
Height(cm): 171
Temp: 25
Pre
Meas
5.48
3.45
(63)
3.26
(3.83)

Pre Post
% Ref
111
88

Gender : Male
BMI: 28.04

Post
Meas

Age: 30

Post
% Ref

% Chg

76
(43)

Comments: All tests were done well with good patient effort and technique and results were
acceptable and reproducible.
Interpretation: Spirometry suggests obstructive pattern but flow loop consistent with fixed
extrathoracic obstruction. Lung volumes and gas transfer preserved, making bleomycin lung
disease unlikely. Does this subject have tracheal narrowing or an enlargedthyroid?.

Interpretation
Definition of significant response
FEV1 inc. by 15% AND 200ml
FEV1 or FVC inc. by 12% AND 200ml

What does reversibility mean?

Reversible airflow obstruction
Asthma
COPD with reversibility
COPD + asthma

ID: AKC1991
Weight(kg): 96.0
PB: 745 Temp:
Pre
Spirometry
Ref
FVC
5.71
FEV1
4.27
FEV1/FVC 74.0
FEF25-75% 4.19
PEF
10.27
Lung Volumes
TLC
RV
RV/TLC
FRC PL
ERV
VC
Resistance
Raw
sRaw
Diffusion
DLCO
DLCO /VA
VA

Bronchodilator Response PFT

Date: 21/06/04
Height(cm): 189
21
Pre
Meas
6.05
3.74
62.0
(1.99)
10.19

Post Post
% Ref
106 6.31
88
4.27
68
(47) 2.66
99
9.4

Gender: Male
BMI: 26.87
Post
Meas
110
100

% Ref
4
14

63
91

33
-8

Age: 40

% Chg

Comments: Acceptable and repeatable results obtained. Ventolin (2.5gm) was administered for
bronchodilator testing.
Interpretation:

Bronchodilator Response PFT

ID: AQA1519
Date: 16/08/04
Weight(kg): 47.0
Height(cm): 153
PB: 734 Temp: 22
Spirometry
Ref
FVC
2.54
FEV1
1.83
FEV1/FVC 73.0
FEF25-75%
2.26
PEF
5.15
Lung Volumes
TLC
RV
RV/TLC
FRC PL
ERV
VC
Resistance
Raw
sRaw
Diffusion
DLCO
DLCO /VA
VA

Pre
Meas
1.83
(0.76)
(41.0)
(0.25)
2.36

Pre Post
% Ref
72
2.66
(41) 1.17
(44.0)
(11) (0.35)
46
3.64

Gender Female :
BMI: 20.08
Post
Meas
105
64

Post
% Ref
45
54

(15.0)
71

41
54

Age: 63

% Chg

Comments: Acceptable and repeatable results obtained. 2.5 mg of Ventolin was administered for
2 mins for bronchodilator testing.
Interpretation: Clinical details provided: COPD, ex smoker ? reversibility. Spirometric lung
volumes are indicative of a very significant degree of airflow limitation with more profound flow
limitation at mid and low lung volumes. There is however a clinically important bronchodilator

Bronchial challenge testing Often used for asthma diagnosis

How?
Off inhalers
Check spirometry
Inhale a bronchoprovocator (histamine,
methacholine, saline) at inc. concentrations
measure spirometry after each inhalation

N.B. exercise as a bronchoprovocator

Bronchial challenge testing Data

PD20 = Provocative Dose required to
produce a 20% drop in FEV1
Histamine + if <4micromol

PC20 = Provocative Concentration

required to produce a 20% drop in FEV1
Histamine + if <8mg/ml

PC20/PD20 also used for Methacholine

Hypertonic saline

ID: NDZ2751
Weight(kg): 81.5
PB: 7510
Histamine
Post
Response Meas
FVC
6.48
FEV1
4.82
FEV1/FVC 74
FEF25-75%
3.86
PEF
9.88

Histamine Dose Response

Date: 16/12/03
Height(cm): 182
Temp: 23

Gender: Male
BMI: 24.60

Age : 31

Pre

Post

%
-3
-6

%
-2
-8

%
-4
-14

%
-14
-29

%
-3
2

-9
-11

-16
-15

Meas
6.30
5.02
80
4.62
10.03

-31
-19

-45
-31

Comments: Acceptable and repeatable results obtained. Histamine baseline Spirometry trials
5,6,7. Histamine test was positive with PD20 =0.419. Ventolin (2.5 mg) was administered to
release bronchoconstriction caused during bronchoprovocation challenge.
Interpretation: Baseline spirometry, static lung volumes and transfer factor are within normal
limits. Bronchial challenge testing shows bronchoconstriction/airways hypersensitivity which is
consistent with asthma in the appropriate clinical context.

20
1

Bronchial challenge interpretation

Threshold for positive may vary
centre to centre
Indicates Bronchial
hyperresponsiveness
Negative test virtually excludes
asthma
False positives post-infection

Static lung volumes

Why?
Measure residual volume (and therefore TLC)

How?
Measure the FRC
Plethysmography or Gas dilution
Plethysmography (bodybox) preferred
measures poorly ventilated airspaces
2 types - volume-displacement & volumeconstant

Lung volumes

Lung volumes interpretation

True restriction - reduced TLC
Hyperinflation - high TLC
Gas trapping - High RV, RV/TLC ratio

Neuromuscular disease - TLC,

preserved or raised RV

Transfer factor

Gas exchange by the lung depends on:

1. Ventilation of the airways and some air
spaces by bulk flow of gas;
2. Mixing and diffusion of gases in the alveolar
ducts, air sacs and alveoli;
3. Transfer of gases across the gaseous to liquid
interface of the alveolar membrane;
4. Mixing and diffusion in the lung parenchyma
and alveolar capillary plasma;
5. Chemical reaction with constituents of blood;
6. Circulation of blood between the pulmonary
and systemic vascular beds.

Transfer factor How?

Inhale to TLC a gas mix containing
known concentrations of CO & He
Hold breath 10 sec
Exhale
Discard dead space
Collect alveolar gas

Use He dilution to calculate VA &

starting Alveolar CO

DLCO Data generated

Then DLCO is calculated from the
difference between starting CO conc.,
and CO conc. after 10 sec in contact
with alveoli
Expressed in ml/mmHg/min
VA = TLC by single breath helium
dilution
DLCO/VA = transfer coefficient (KCO)

DLCO - interpretation
DLCO by:
Pulmonary vascular diseases
Conditions affecting alveoli
Cardiac diseases
Anaemia
Pregnancy
Recent smoking

DLCO - interpretation
DLCO by
Polycythaemia
Pulmonary haemorrhage
L to R shunt
Exercise

KCO (DLCO/VA)
Corrected for volume. Theoretical
function of the individual alveolus (??)

COPD PFT
ID: BDD9943
Weight(kg): 65.0
PB: 754
SpirometryRef
Chg
FVC
4.2
FEV1
3.1
FEV1/FVC
73.0
FEF25-75%
3.1
PEF
7.8
Lung Volumes
TLC
5.8
RV
2.0
RV/TLC
36.0
FRC PL
3.4
ERV
1.4
VC
4.2
Resistance
Raw
1.4
sRaw
4.6
Diffusion
DLCO
20.6
DLCO /VA
4.0
VA
6.3

Date: 23/06/04
Height(cm): 168
Temp: 21
Pre
Pre Post
Meas % Ref
(2.0)
(.8)
(37.0)
(.3)
(2.6)

(48.0)
(25.0)

(9.3)
(7.0)
(75.0)
(7.1)
(.3)
(2.4)

(162.0)
(346.0)

7.0
56.6

518.0
1243.0

14.7
3.1
(4.8)

71.0
78.0
(75.0)

Gender: Male
Race:
Post
Meas

Post
% Ref

Age: 55
BMI: 23.03

(10.0)
(33.0)

(211.0)
(19.0)
(57.0)

Comments: The patient could not fully expire during forced and slow expiration, therefore the
results were not quite accurate, even though they were repeatable.
Interpretation: Stable lung function.

Other patterns
Obesity
Restrictive Spirometry and TLC, very reduced
FRC, reduced RV. DLCO only reduced in very
gross obesity

Heart Failure
Obstructive in Acute, Restrictive in Chronic with
decreased gas transfer

Neuromuscular
Decreased FVC, lower when supine, decreased
TLC, preserved RV, preserved DLCO

PAP(pulmonary alveolar proteinosis)

It is a rare lung disease characterized by
abnormal intra-alveolar accumulation of
surfactant like lipoprotein aceous material.
CAUSES:
what do you think the causes are?
Causes are divided into three board catagories:Idiopathic (90%)
Secondary (5-10%)
-hematological malignancy
-toxic inhalation lung disease
(silicoproteinosis,tia-tanium oxide)
Immune deficiency
congenital

 Pathophysiology:
Deposition of pas (+ve)
lipoproteinaceous material in alveoli as
a result of impaired turnover of
surfactant. Granulocyte-macrophage
stimulating factor (GM-CSF) has been
implicated in the pathogenesis.
Complications:
Superimposed infection (Nocradia
asferiodes sp.)
Pulmonary fibrosis

Epidemiology
The disease is more common in males and
in tobacco smokers.
In a recent epidemiologic study from Japan,
Autoimmune PAP has an incidence and
prevalence higher than previously reported
and is not strongly linked to smoking,
occupational exposure, or other illnesses.
Endogenous lipoid pneumonia and nonspecific interstitial pneumonitis has been
seen prior to the development of PAP in a
child.

Clinical features:
What about the clinical features?
1/3 of the patients are asymptomatic
Dyspnea
Cough
Low grade fever
Weight loss
Fatigue
Most of the time extrapulmonary
symptoms are present in children (such
as diarrhea, vomiting, failure to thrive)

 PE:
Crackles
Clubbing
Cyanosis

How to diagnose:

CXR
PFT
Surgical/endoscopic biopsy of the lung
GOLD STANDARD: Broncho-alveolar lavage
and transbronchial lung biopsy

 Microscopically, the distal air spaces

are filled with a granular, eosinophilic
material that is positive with the PAS
stain and the PAS diastase stain. The
main histomorphologic differential
diagnosis is pulmonary edema, which
does not have dense bodies.

Intermediate magnification micrograph of pulmonary alveolar

proteinosis. H&E stain.

PAS STAIN

Treatment
there is no drug cure at the moment
Whole lung lavage
What is whole lung lavage?
the sterile fluid (NS) instilled into the
lung and then removed with the
surfactant material
- Double lung transplant ( curative)

Special thanks to my pulmonology teacher ( Dr. Xu Hui)