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Respiratory system
a. Introduction, Respiratory Function Test
b. Clubbing.
c. Nose,Larynx,Pharynx,Sudden Infant death.
d. Pulmonary edema.
e. Congital Lung Lesion.
f. Pulmonary hemorrhagic hemoptysis
g. Atelectasis
h. Pulmonary hemosiderosis
i. Pulmonary embolism
j. Aspiration $.
k. Inhalation Hypersesitivity Pneumonia.
l. Esinophilic Pneumonia(Loffler $)
m. Causes of recurrent Pneumonia.
n. Pleurisy pleural effusion empyema.
o. Pneumothorax,tension pneumothorax, Pneumomediastinum.
p. Bronchopulmonry dysplasia.
q. Interstitial lung disease
r. Bronchiectasis
s. Pulmonary abscess
t. Cystic fibrosis,primary ciliary dyskinesia.
u. Chest wall anomalies.
v. Neuromuscular disease of chest
w. Pulmonary hypertension
x. Vascular ring pulmonary A-V fistula

Prepared by Dr. Amr Malash

RESPIRATORY SYSTEM
(VT): Tidal volume: Amount of air move in and out each breath.
(VC): Viral capacity: Amount of air move in and out after
maximum insp. And max expiration.

(IC): Insp. Capacity: Amount of air inspired by max inspiration.


(ERV). Exp. reserve volume: Amount of air expired by max
expiration.

(RV). Residual volume: Amount of air remaining in lung after


max. expiration.

(FRC): Functional residual capacity: Amount of air left in lung


after tidal expiration.

(TIC): Total lung capacity: Amount of air left in lung after max
respiration
N.B.: Functional residual capacity:
During insp. Alveolar PO2 (PAO2) as fresh atmosphere gas enter lung.
During exp. alveolar PCO2 (PACO2) as Capillary Blood put CO2 to
alveoli and take O2..
FRC act as buffer minimize shanges in PAO2 &PACO2.

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FRC in interstitial and thoroacic wall disease lead to hypoxia as it lead


to sharp decline in PAO2 during expiration as limited volume for gas
exchange.
This can be ameliorated by PEEP and inspiratory time during
mechanical ventilation
FRC in obstructive lung disease: Incomplete expiration lead to
tidal resp. effort work of breathing and inside of barotraumas with
mechanical ventilation.

Respiratory
Function Test
Insp. capacity
VC
TLC TV(Vt)

FRC

Tidal volume
(TV)

Exp. reserve volume

(RV) residual volume

N.B.: Hypoxic respiratory failure:

FiO2 < 60 mmHg with FiO2>60%.


Hypercarbic respiratory failure:
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PCO2 50 mmHg.
N.B. Clubbing:
1. Chronic hypoxia with chronic lung disease
2. Cardiac: cyanotic heart disease. Cong. Heart failure, bacterial
endocarditis.
3. GIT: Crohns disease, ulcerative colitis, liver cirrhosis, intistinal
lymphoma
4. Other: Hypothyroidism, fabry, reaynaund disease
5. Unilat. Clubbing: vac. Disease: Subclavian A. aneurysm, Brachial
AV fistula.

Window angle

To detect clubbing
1. In N. if we put 2 finger opposite each
other window appear in clubbing this
window disappear
2. Phalangeal depth ratio

finger

Normal
With disappear this window

IPD >DPD
Normal:
InterPhalangeal depth
distal phalangeal depth.
In Clubbung: reverse
appear: IPD DPD

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Disappear of window
NB.
1. Fluoroscopy: useful in evaluation of strider.
2. Barium swallow: TOF, vascular ring, GE reflux.
In patient with Rec. pneumonia, Strider, persistent wheezes of
unknown origin.
Milk Scan for GE Reflux.
3. CT with contact, CT, MRI of chest: Has replaced bronchography
also pulm. Atriography and lung scan
NB. Sudden infant death $:
Cardiac, CNS (AV malformation), Metabolic disease.
Immunodef. Can be the causes of rec. sudden infant death also
Pulm. Hypertention & flaccid child is a cause.
Investigation: Echo, ECG for long QT interval , Metabolic screen,
CT.
Pacifier, supine position sleep, No soft bed or comfort, no
smoking, no sharing bed all effective.
4. Res. Function test: Only to D.D. restrictive from obstructive
lung disease
N.B. Choanal atresia: Can be with CHARGE $
(Cloboma, Heart, Atresic choana, Retarted Growth or CNS,
Genital anomalies (hypogonadism), Ear anomalies (deafness)
Unilateral: Can be asymptomatic for long period unilat.
Discharge.
Bilateral: Cry relieve cyanosis
CT best diagnostic

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N.B. Midline nasal mass: Dermoid, gliomus, encephalocele,


CT must be done in this case.
N.B.: Apply Vaseline in winter to nose can epistaxis.
N.B.: nasal polyps: Most common with cystic fibrosis may be
with allergic rhinitis and chronic sinusitis.

Zink given as oral lozenges: is effective in


treatment of common cold $ and inhibit essential
enzymes for rhinovirus.
N.B.:

Echinacea herbal medicine not effective in common cold.

N.B. Ethmoid sinus pneumatized at birth but


maxillary sinus pneumatised at 4 year of age&
frontal at 7 years.

Retropharyngeal &lat. Pharyngeal


abscess
N.B.

mostly in boys <4 years, CT is best diagnostic or


Soft tissue neck film: air fluid level in retropharyngeal space Or
width.
Best treatment: Unasyn + 3rd G cephalosporin all I.V. to cover
aerobic and also anaerobic (sulbactam in unasyn).
If not surgical drainage.
Uncommon complication is Lemeirre disease: Thrombophlebitis of
Int. jugal & metastatic abscess in lung.

N.B. Peritonsillar abscess:


antibiotics
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Surgical drainage + I.V

N.B. Unilat vocal cord paralysis : aspiration chocking,


cough,
Weak cry.
Vocal cord paralysis can happened after cardiac surgery usually
resolve will 6-12 months.

N.B.: Hypoxic respiratory failure:

FiO2 < 60 mmHg with FiO2>60%.


Hypercarbic respiratory failure:
PCO2 50 mmHg.

PULMONARY EDEMA
Cardiogenic

Non-cardiogenic
(ARDS)
Acute resp. distress syndr.

Heart size:
Greater than N
Edema distribution:Central or even
Pleural effusion:
present
Air bronchogram :not present
Auscultation: Gallop rhysm
Generalised edema:usually present

Normal
Patchy or peripheral
Not usually
usually present
Not present
No

Causes of non-cardiogenic pulm. Edema:


Bacterial or viral pneumonia, sepsis,
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Pulmonary venoocclussive disease, drawning, aspiration


pneumonia,
Re-expansion pulm. Edema, pancreatitis, severe hypoalbuminemia
C/P: Interstitial and alveolar edema lead to atelectasis &
surfactant production, tidal volume, wheezes, crepitation,
respiratory distress.
Diag: 1. Chest X-ray
2. Brain natriuretic peptide Test (BNP): D.D: Cardiac for noncardiac causes of pulm. Edema(measure BNP hormone level in
blood).
BNP 500 pg/mL suggest Cardiac cause.
BNP < 100 suggest Pulmonary cause.
Treatment
1. O2 esp PEEP or CPAP compliance improve surfactant
function.
2. Diuretics esp. frusemide.
3. Morphine helpful as vasodilator & sedating.
4. Mech. Ventilation.
5. Inotrope for cardiac cause.
6. bronchodilator + treatment of cause.

N.B.:

Congenital lobar emphysema:

Usually left upper lobe but can be in upper or middle


CT: to detect lobe & herniation
MRI: to demonestrate any vascular lesion which can cause
extraluminant obstruction.
According to Symptoms, If $ severe
surgical excision but
bronchoscopy must be done.
Prepared by Dr. Amr Malash

1st if asymptomatic conservative treatment


N.B.: Bullous emphysema:
Bullous emphysema: bleb or cysts ( pneumatocelce) can appear during
birth or secondary to pneumonia or TB.
May be singel or multilocular may be with air fluid level and should
be D.D. from pulmonary abscess.
Treatment: Conservative except with major $ aspiration or excision.

N.B.: 1 antitrypsin def.:


Major liver disease in neonate & children emphysema in adult.
Rarely pulm. Disease in child.
Tretment: enzyme replacement therapy.

Brochiolitis Obliterance:
serious condition complicate viral bronchiolitis
CT : patchy area of translucency and bronchiectasis.
The only definitive diagnosis is lung biopsy.
Treatment: Steroid treatment esp.in Bronchiolitis obliterance organizing
pneumonia (BOOP) but relapse can occure.So better treatment with
steroid for 1 year to prevent relpse.

N.B.: Pulmonary alveolar microlithiasis: Rare calcium


phosphorus Lithiasis in lung.
Diagnosis: CT, lung biopsy
Treatment: Steroid treatment .

N.B: Pulmonary sequestration:

Lung tissue not connect to


main bronchus and receive Art supply from Systemic circulation.
CT, MRI diagnose it.
Treatment: Surgical
N.B: Bronchogenic cyst:
Congenital condition rarely diagnosed at birth.
fever, chest pain and may be dysphagia
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CT, MRI diag., treatment surgical excision.

N.B.: Lung hernia:


Congenital in 20% esp. in cervical area.
Conservative treatment.

N.B.: Congenital pulm. Lymphangiectasia:


Due to venous obst with cardiac condition
Or part of Generalised lymphangiectasia
Diag.: Lung biopsy

PULMONARY HEMORRHAGE & HEMOPTYSIS

Focal
Bronchitis or bronchietasis
TB
Trauma Tumor
Pulm. A.V. malformation
Pulm. Embolism

Diffuse
Idiopathic infency
Cow milk hypereson. (Heiner $)
Good Pasteur $
Collagen vascular
Henoch scheten
Wegner granuloma

Treatment:
1. Volume resuscitation & blood transfusion.
2. Mechanical ventilation.
3. Corticosteroid treatment.
4. If not stopped bronchoscopy or embolization or lobectomy
5. Treatment of cause

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ATELECTASIS
Indication of Bronchoscopy:
1. Foreign body.
2. Massive both lung collapse
3. Isolated area of atelectasis
persist several weeks.
4. Lobar atelectasis persist
for 7 days after supp.
Treatment.

Causes:
1.
2.
3.
4.
5.

Viral infection esp. resp. sync. Virus.


Mucous plug, asthma pneumonia.
Post-operative.
Foreign body.
Pneumothorax or effusion.
90% of upper lobe,
63% Right upper lobe
Asthmatic patient right middle lobe.
F.B. acc. To site of obst. of F.B.

In neonate esp ET tube: ET tube Moving to right bronchus lead to


obst & inflammation to bronchitis right upper lobe collapse
Treatment:
1. Suction and treatment of cause, chest physiotherapy.
Prepared by Dr. Amr Malash

2. Bronchodilator + steroid treatment in asthmatic ( right middle lobe


collapse).
3. CPAP & suction after introduced of normal saline
4. Recombination human DNase (rh DNase) used in cystic fibrosis
can be used to thick secretion.
5. Bronchoscopic examination immediate in FB., Bilateral atelectasis
Or 7 days lober alelectasis and no response to normal treatment.

Pulmonary hemosiderosis:
Must be diffuse alveolar hemorrhage & chronic course

Triad of:
1. Iron def. anemia :Microcytic hypochromic with iron and
TIB capacity with retics & bilirubin
2. Chronic Hemoptysis .
3. Alveolar infiltration on chest X-ray & CT.
May be primary as:
1. Good Pasteur: hematuria, hemoptysis (anti-basent memb. Ab).
2. Cows Milk allergy.
Or Secondary: Autoimmune disease, cardiac cause,
henoch. Schonlein Purpra.
C/P: Hemoptysis+ anemia + evidence of alveolar Infiltration.
Investigation: For autoimmune disease, good poster
Treatment: Steroid treatment 2-5 mg/kg/day
Then taper & then alternative day therapy.
N.B.: Good posteur disease is the commonest causes of pulm.
Hemosiderosis.

PULMONARY EMBOLISM
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Causes:

Neonate with centeral venous catheter for TPN.


Thrombotic disease, Protein C, S.
Surgical: major surgery or orthopedic or neurosurgery.
C/P:
Cyanosis, dyspnea, chest pain diaphoresis
+ pre-disposing factor (crepitation may be heard).
Diag.:
Chest X-ray often normal.
CT with contrast is valuable 90%.
Pulm. Angiography gold standard diagnosed.
Treatment: Mechanical ventilation, Inotropic agent,
Heparine to keep PTT: 1.5-2 times control.

ASPIRATION SYNDROME
Gastric content: After anesthesia, gastroenteritis.
Hypoxia, hemorrhagic pnemonitis, pulm. Edema
Treatment: O2 therapy esp. mechanical ventilation,
Bronchodilator steroid therapy
Antibiotic prophylactic: should cover anerobic bacterial &
pseudomonas in hosp. patient.
Resolution usually 2-3 weeks,prolonged lung damage can appear.

Hydracarbon: Severe pneumonitis, pulm. Edema can appear 30


minutes up to 6-8 hours after ingestion.
X-ray findings appear 8 hours after ingestion.
Corticosteroid may help fibrosis.

Chronic recurrent aspiration:


1. Gastroesophageal reflux
2. Oropharyngeal in coordination most commonest.
(CP patient & neuromuscular disease patients)

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Diag: barium swallow: Barium swallow with video fluoroscopy


must specific.
Milk Scan: very specific.
Treatment: treatment of GE reflux.
Gastrostomy, Tracheostomy.

Hypersensitivity pneumonia:
Secondary to inhation: farmer lung pigeon breeder lung,
Humidifier lung (contaminates humidifier)
C/P: Interstitial lung disease, +ve history of exposure
BA lavage: Lymphocytosis
Treatment: Prednisone treatment 1-2 mg./kg/day may be up to 6
months.

Esinophilic lung disease: (Loffler syndrome)


Secondary to parasitic infestation esp. ascaris, toxocar, Ancylostema
Secondary to drug induced.
C/P: Like interstetial lung disease + Peripheral esinophilia
Diag.: Lung biopsy
Treatment: Steroid treatment.

Causes of rec. pneumonia:


1. Cystic fibrosis ( immotile cilia $, Kartagner $).
2. Sickle cell disease.
3. Immunodeficiency ( granuloyste or IgE).
4. TOF or GE reflux.
5. Sequestration lobe, lober emphysema.
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6. Bronchiectasis.

Pleurisy, pleural effusion, emphysema:


Inflammation of pleura often accompanied with effusion.

Pleural effusion
Transudate

Exudate

Complicated empyema

clear

cloudy

purulent

Cell count: <1000

>1000

Cell type:

lymphocyte

polymorph.

Gram stain:

Negative

Colour:

Protein( > 3gram): NO


Prepared by Dr. Amr Malash

Usually +ve
Common

>5000
Polymorph.
80% positive
Common

LDH :

<200

>200

PH :

7.4

7.2-7.4

> 1000
<7.2
Chest tube placement required

Diagnosis: CT, ULTRASOUND help to D.D


Treatment of empyema: antibiotics, chest tube, fibrinolytic as
streptokinase
Selective video assist thoracoscopy (VATS) may be helpful.

Cardio surgery consultation in empyema is imp


.
Antibiotics 2-3 weeks I.V then oral to complete total 4-6 weeks
antibiotics.

Dry (plastic) pleurisy:


Bacterialor viral pulmonary Infection or TB.
Pain with deep breathing cough straining .
May refer to shoulder or back which lead to grunting and guarding of
respiration.
Diag.: Only CT or ultrasound (small amount of exudates).
Treatment of cause + Analgesics non-steroidal anti-inflammation is
effective

Serofibrinous (Serosanginous) Pleurisy:


Exudates protein > 3gm, LDH> 200 PH 7.2-7.4

N.B.: PH <7.2 suggest empyema


PH <7.2 + lymphocyte TB
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LDH >1000 + purulen empyema


Pleural fluid glucose <50 empyema

N.B.: Rapid removal of 1 liter of pleural fluid reexpansion pulmonary edema.


Treatment: of cause + aspiration (draining) usually more comfortable &
fasten therapy.
N.B.: Imp. To D.D. exudates from empyema as empyema chest
tube.
If fasten re-accumulation or suspect empysema chest tube must
be done.
Purulen pleurisy or empyema: complicate 5-10% of bacterial pneumonia
staph, strept, gram ve, TB.
I.V. antibiotic for 3-4 weeks usually slow important & take time with
slow response to antibiotics + chest tube drainage immediately.
If patient remain febrile & dyspnea > 72 hours of chest tube + I.V.
antibiotics
VATS (video assist thoracoscopic surgery).
Instillation fibrnolyte (Streptokinase) in pleural space but anaphylaxis,
hemorrhage manifestation can happened.

Hydrothorax:

Cardiac, Hepatic, Renal


Usually bilateral but in cardiac may be right side more or limited to right
side.
Diag.: Specific gravity <1015, few cell low protein & LDH low.

Hemothorax:
1.
2.
3.
4.
5.

Traumatic
TB. Empyema
Intrathoracic neoplasm or metastasis
Cong. Lung sequestration, A.V. malformation.
Patent ductus arteriosis blood disease

NB. Hemo-pneumothorax: Pleural hemorrhage with pneumothorax


Prepared by Dr. Amr Malash

Treatment: Chest tube + transfusion may be indicated.

Chylothorax:

Escaped chyle from thoracic duct pleura


1. Traumatic cardiosurgery
2. Primary or metastatic malignancy esp. lymphoma.
3. New born venus pressure during delivery thoracic duct
rupture
Usually on right side, no pain as chyle not irritant.
Milky colour but in Neonate not yet fed clear.
Triglyceride level in pleural fluid >110.
Ratio pleural fluid triglyceride to serum triglycride > 1.
Cell : primary lymphocyte with fat, protein.
Loss of T lymphocyte in chyle immunodeficiency &
hypogammaglobulinemia infection
Chest tube enteral feeding with low fat or MCT
If no resolution in 2 weeks TPN if no ligation thoracis duct .
Or pleuro peritoneal shunt, If surgical fail parenteral octreotide.

Pneumothorax:
May be spontaneous or follow staph pneumonia, asthmatic bronchitis
Spontaneous: Usually in teenage & young adult
Secondary: Staph pneumonia, asthmatic. Cyst fibrosis
Diag: Chest X-ray especially in Expiration views: accentuate contrast
bet. lung marking & pnuemothorax (exp. film)
Tension pneumothorax mean : shift of mediastinum.
However,in RDS bilat pneumothorax without shift , so better diagnosis
on basis of evidence of circulatory compromise or hearing (hiss) on
chest tube.

Prepared by Dr. Amr Malash

D.D. for small diaphragmatic Hernia: Barium study or ultrasound.


Treament:
Small <5% in asthmatic or spontaneous : resolve spontaneously.
If >5% or tension pneumothorax chest tube for 48-72 hours.

Pneumomediastinum:

Most common cause: Asthmatic


bronchitis in neonate only pneumo-mediastinum can cause circulatory
compromise otherwise resolve spontaneously.

N.B.: Broncho-pulmonary dysplasia:


Infant require O2 > 35 weeks,
Treatment:
1. Inhaled B2 stimuli or steroid
2. Diuretic therapy
3. Anti-reflux treatment for GE reflux is very important.
4. Adequate caloric intake 120-160 Kcal/Kg/day.

Interstitial lung disease:


1.
2.
3.
4.

Adenovirus, influenza, chlamydia, mycoplasma.


Aspiration pneumonitis.
Allergic hypersensitivity pneumonitis.
Lymphocyte interstitial pneumonitis most common interstitial
lung disease with immunodeficiency or autoimmune disease.
5. Esinophilic interstitial pneumonitis.
6. Surfactant B or C protein def.
7. Pulmonary hemosiderosis
Diagnosis:
1. Chest X-ray may be normal & does not correlate with extend of
disease.
2. CT chest is best diagnostic for inf. Lung disease
Prepared by Dr. Amr Malash

3. Lung biopsy is best diagnostic


Pulmonary broncho-alve. Lavage (BAL) may be helpful
Treatment:
1. Zithromax,clarithromycin for chlamydia
2. Prednisone 1-2 mg. /Kg/day for 6-8 weeks with tapery according
to clinical response
3. If parasitic infestation treatment treat it as ascaris (esinophilia in
CBC)
4. Bronchodilator
5. Hydroxychloroquine (used to treatment malaria) is effective
treatment
6. I.V. immunoglobulin

N.B.: Pulmonary alveolar protienosis:


1. Fulminant caurse appear in neonate like respiratory distress
syndrome picture due to def. of Protien C, B of surfactant usually
in full term infant(Fatal course).
2. Gradual caurse: Like interstitial pneumonia.
Diag: Lung biopsy
Treatment: SC or inhalation GM CSF (Granulocytic macrophage
colony stim. Factor)
Some success in neonates.
So RDS picture in full term infant should be put in D.D. with prot.B
or C of surfactant.

Bronchiectasis:
Irreversible abnormal dilation of bronchial tree.
Cystic fibrosis is the commonest cause. Other ciliary dyskinesia,
pertussis infection, T.B. immunodeficiency right middle lobe $ by ext.
compression by lymph nodes.
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C/P: Cough, copious, purulen sputum,


Hemoptysis, frequent.
Clubbing.
Anorexia, loss of weight.
Diag: CT chest, scanning of lung
Treatment: Antibial 4-8 weeks + physiotherapy
Prophylactic treatment with macrotide or nebulizer antibiotics.
Surgery for local bronchiectasis.

Pulmonary abscess:
Aspiration of infected material or FB is commenest cause (posttonsillectomy or adenoidectomy),
Immune deficiency, pneumonia, blood porn infection.
Diag: CT chest is most sensitive
D.D: Pneumatocele from abscess:
Abscess thick wall with low density center progress to air fluid level.
Pneumatocele: Thin wall with or without fluid level.

Treatment: Antibiotic treatment 2-3 weeks I.V. then oral antibitocis to


cover total 4-6 weeks.
Vancomycin + Metronidazol or clindamycin (to cover anerobic) +
cephalosporin or aminoglycoside to cover gram-ve bacteria.
If worse cond. Within 7-10 days surgical drainage.
N.B.: 30% of viral pneumonia complicate with bacterial infection.

Cystic fibrosis:
$: Respiratory, meconium ileus, meconium plug $ in infancy,
maldigestion & steatorrhea (pancreatic), liver dysfunction.

Prepared by Dr. Amr Malash

: Sweat chloride test, Serum trypsingen & fat in stool for


pancreatic function
Treatment: Human recombinant DNase (2.5 mg) aerosol inhalation is
very effective mucolytic with better lung function
N.B.: Zithromax reduce virulence property pseudomonas can be
given weekly as prophylactic.
Only oral quinolene is effective against pseudomonas.
N.B.: Aerosolized tobramycin 300 mg. twice daily is effective against
resistance pseudomonas, also gentamycin (both less side effect than I.V)
N.B.: Endoscopy and lavage, antibiotic as gentamycin or Tobramycin
can be instilled directly drug lavage.
N.B. Atelectasis: Antibiotic, physiotherapy if no improvement in 5-7
days bronchoscopic examination required.
N.B.: In case of hemoptysis physiotherapy stop 24 hours.
N.B. Pneumothorax: Chest tube removed on 3rd day.
N.B. Pancreatic enzyme: Microspheres
N.B. Meconium ileus: Gastrographin enema diag. & therapeutic if not
surgery.

Primary clilary dyskinesia: (Immotile cilia $):


50% have kartagner$, situs inversus, chronic sinusitis + recurrent otitis
media, Air way disease end in bronchiectasis.
Diag.: Scraping or brushing nasal mucosa & examine directly by light
or elect. Microscope.
Gold stander diagnosis : Nasal or bronchial biopsy and ex. cilia
Symptomatic treatment.

Prepared by Dr. Amr Malash

Pectus evacuation:

Funnel chest:((

Congenital or with Marfan $, Ehler danlos $


Lab: ECG to exclude Wolff parkinsonian,
Echo mitral valve prolapsed may be present.
C/P: Exercise intolerance, rec. resp. infection

Pctus carinatum (Pigeon chest):

male > 4 times than

females
exercise intolerance, recurrent chest infection, recurrent asthmatic
bronchitis

Sternal cleft:

Failure fusion of sterum, surgery required early in

life.

Scoliosis:

Curve >10 degree defined as scoliosis esp. with cobb


technique( radiologically angle of scoliosis).

Neuromuscular disease:
Butulism, Guillian barri, polio, myasthenia, spinal muscle dystrophy.
Rec. infection lead to lung fibrosis and further accelerate loss of function
of lung parenchyma lead to hypoxia
Pulmonary hypertension corpulmonale.
NB.: Change in voice and speech, use of ala-nasi, use of acc. Muscle at
rest is serious manifestation.
Prepared by Dr. Amr Malash

NB.: 1. Influenza vaccine , pneumococcal vaccine must given


3. Theophyllin, corticosteroid, carnitine Supp. Unknown result.
Evaluation of neuro-muscle disease patient:
If vital capacity and max respiratory Pressure >60% :
evaluate
pulm. Function test every 6 months.
If vital capacity and max res. Pressure <60% :
evaluate pulm.
Function test (PFT) of every 3 months
+ nutritional consult, regular resp. therapy, percussion device, chest
physiotherapy + Annular influenza vaccine.

Pulmonary hypertension
Primary pulmonary
disease
Hypertension
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pulmonary vascular
Eisenmenger $

Exercise intolerance, chest pain


Syncope, headache, peripheral
cyanosis.
*If foramen oral patent right to left
shunt
Sign& $ of right side heart failure
Prominent pulm. A in chest X-ray.
Prominent pulm. Vascular bed in
chest X-ray.
Echo heart to D.D.
Primary pulm. from Eisenmeger $
Treatment:
1. Calcium Ch. Blocker (nifedipine)
2. Viagra 0.5-1 mg/Kg/day
Max. 20 mg. /3 times /day.
In Neonates: 0.1 mg/kg/day.
3. Contra nitre oxide treatment.

VSD, ASD, patent ductus.


Symptom developed in 2nd or
3rd decade of life.
NB. In trisomy 21 symptom
may appear early in life.
Cent. Cyanosis
Polycythemia
Clubbing
Brain, abscess, stroke,
hemoptysis from right to
left shunt.
Treatment:
is avoidance not respond to
pulm. Dilator.

NB. Table 434.1 Page 1938 is very important to care of post-operative


cong. Heart disease and complication

N.B. Managing cyanotic heart disease:


1.
2.
3.
4.

Avoid hypoxia
Avoid hypoglycemia & treatment
Avoid hypothermia
Proper treatment of iron deficiency anemia

Prepared by Dr. Amr Malash

5. Prostagindin E1 0.01-0.2 ug/Kg/min


6. Cyanotic spell propranolol : 0.5-1 mg./kg/6 hours orally or
0.01:0.1 mg/Kg/dose I.V.

N.B.: Sudden death: (not sudden infant death):


Usually: Valve aortic stenosis, coronary A. Abn., cardiomyopathy,
cardiac arrhythmia.
Every patient with syncope or chest pain should be evaluated well.

Vascular rings: Cong. Abnormalities of aortic arch & its


major branches result in formation of vascular rings around trachea,
esophagus most common:Double aortic arch, anomalies inominate A.,
Aberrant Right subclaviant A.
Symptom : Strider , resp. distress, chronic wheezes,
Extension of neck relieve noisy respiration.
Swallowing dysfunction
Reflux apnea.
Diagnosis: Barium swallow of esophagus
Echocardiography
CT, MRI.
Treatment: Surgery.
*

Pulmonary AV fistula : Osler-weber Rendu $

Usually bet Pulm. A & vein.


Usually with other Angioma (hemorrhagic telangiectasia) of nose,
GIT, liver
Symptom: Dyspnea, cyanosis, polycythemia, continuous murmur,
recurrent epistaxis, GIT bleeding (Other angioma 50%). CNS
manifestation from paradoxical emboli or cerebral abscess.
Diag: Chest X-ray: Multiple opacities, MRI, CT.
Treatment: Surgery.

Prepared by Dr. Amr Malash

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