8. By the end of this session the student able to :
1- Identify the definition ,Prevalence, and etiology
2- illustrate the Pathogenesis ( mechanism )
,Pathology &
Pathphysiology
3-Diagnose the bronchiectasis via Clinical Picture and
investigation
4- demonstrate the optimal Treatment
5- differentiate Complication
18. A 33 year old man , employer, non smoker married 5
years ago with no offespring ,
with history of recurrent upper and lower respiratory
tract infectioin
presented with Productive cough and expectoration of
larg amount of mucopurulent greenish sputum
associated with
recurrent attackes of blood tinged sputum and SOB
2weeks ago
Also suffering of fever , anoroxia and headach
Admitted to chest department ultrasound was routinely
done before and revealed dextrocardia
His spouse thought medical advice regarding primary
infertility
Semen analysis was requested from her investigation .
19. 1-What is your provisional diagnosis?
2- List three investigation To achieve the
diagnosis
3- outline the treatment of previous
condition
20. Examination of cilia (or sperm tail) under the E/M
revealed : nine pairs of microtubules that run
longitudinally are arranged circumferentially around
the cilia
The dynein arms are ATPase radial projections, two
arise from each of nine pairs of microtubules
(i) absence of dynein arms or radial spokes
(ii) presence of spatial transposition of microtubules
(iii) presence of random ciliary orientation in which
the cilia are
anchored in disorganized fashion at the cell surface .
(iv) absence of spoke heads or central sheaths
Immotile cilia Syndrome:
21. Immotile cilia Syndrome :
Diagnosis:
Abnormal saccharin test, time is recorded for saccharin
to be tasted in the mouth after a 0.5-mm particle has been
deposited on the inferior turbinate in the nose. This
usually takes about 30 min but times of up to 1h may be
normal.
running a bronchoscopic cytology brush along the mucosal
surface of the inferior turbinate
video microscopy to measure ciliary beat frequency
Electron microscopy of sperm tails and respiratory cilia
may produce diagnostic
26. Cystic Fibrosis
Autosomal recessive disease resulting
from mutation of Cystic fibrosis trans-
membrane conductance regulator
(CFTR) Gene located on chromosome
SEVEN
Activity of a chloride channel known as
the cystic fibrosis transmembrane
conductance regulator (CFTR) is
reduced
27. Cystic Fibrosis
CF is characterized by:
Respiratory air ways -----Chronic bacterial
infection -----Bronchiectasis &
bronchiolectasis
Pancreatic exocrine gland: insufficiency
Sweet gland: abnormal function
Intestinal dysfunction
Urogenital dysfunction
28. Clinical Picture
Childhood presentations
Meconium ileus
Prolonged neonatal jaundice
Failure to thrive
Recurrent infections
Nasal polyps and sinusitis are rare in the
infant but increasingly common in
preschool and school-age children.
Neonatal screening
30. *Classic triad :
•pulmonary disease
•pancreatic insufficiency (Pancreatic amylase
& lipase)
•raised sweat sodium concentration of
70mmol/L or more,
*genetic typing
*>90% of men are azoospermic as a result of
congenital bilateral absence of the vas
deferens,
Diagnosis
31. Tracheobronchomegaly (Mounier-Kuhn syndrome)
*the cartilagenous rings of the trachea and its divisions
as far as the segmental bronchi are enlarged, producing
marked dilatation of the trachea and central bronchi
*trachea and main bronchi to dilate during inspiration and to
collapse on expiration.
*associated with chronic lower respiratory tract infection and
bronchiectasis and tends to be
*Diagnosed radiologically ( CT chest ) in the fourth or fifth
decades
32. Williams–Campbell syndrome
(bronchomalacia)
•rare congenital syndrome
•characterized by defective or completely absent
bronchial wall cartilage Producing a mechanical
abnormality bronchiectasis .
• The defect may extend from the 4th the 8th
generations of bronchi
•cough begin from infancy
•CT shows a remarkable ballooning expansion of the
proximal bronchi during inspiration, collapse
during expiration,
• these demonstrated bronchographically
35. -Dilatation & Peribronchial thickening of
Medium sized bronchi from 4-9.
Lined with columinar or squamous epthelium with
cillary destruction.
Cuttoff the small sized bronchi from their parent.
-Inflammation----- Atrophy----- muscle &elastic tissue
destruction replaced with fibrosis.
–Vascular atrophy –opening of anastomosis systemic
to pulmonary shunting. -
36.
37. Site:
Left side >Rt side
- Lower lobe
- linguala
- middle lobe
–Upper lobe
Classification
*Follicular
*Saccular
*Atelectatic
38. Pulmonary Function Test :
•
Obstructive lung disease (
FEV1/FVC<70& FEV1<80% of predicted )
•
Air way Trapping ( increase TLC & RV ,
RV /TLC)
•
DLCO normal then Decreased
ABG:
•
Normal
•
Hypoxemia with normal – CO2
39. Cough
* most common
*productive with mucopurluent sputum
*triggered by postural change, such as lying
down at night, rising in the morning or
stooping down for some task.
Hemoptasis
Dysnea is late symptoms
Toxic symptoms and URT infection
40. General :
Toxic manifestation , Clubbing , Cyanosis If
(RF) , LL-oedema & Congested nick vein if
( Cor -pulmonal )
Local :
Mild cases no abnormality
Coarse mid-inspiratory crackles and are not
shifted by coughing
Signs of collapse and fibrosis may
bepresentg
Occationally rhonchi
41. Routine :
CBC, LFT, KFT, ESR
Sputum : Gram Stain , C/S for aerobic and
anaerobic organism , Zn For AFB , culture
for TB
Serological test for Atypical organism
Other Tests :
Blood culture
Bronchoscope (protective specimens brush
or BAL) for C/S & ZN for AFB.
42. RADIOLOGY
-Cxray unreliable <50% sensitivity.
- Bronchography &HRCT gold stander .
-End on :ring shadow-honey comb- cystic lung
(0.5-2 cm ring with air fluid level .
-Slid on :paralleled -tramline line –solid tebular
opacity (tooth path) gloved finger shadow.
-Volume lose: fibrosis & atelectasis
-Pulmonary hypertension (cardiomegaly proximal artery
dilatation
-Kartagener Syndrome : dextrocardia or situs inverses totals
43. RADIOLOGY
-B. Dilatation- B. thickening = diameter of
adjacent BV – Signet ring appearance.
Lack of B .tapering.
-Increase Transradiancy due to airway
trapping.
Morphological type :
1- Cystic 2- cylindrical 3- varicose 4- saccular
46. Treatment
Medical : Relieve & control symptoms
Surgical : eliminate the disease
Arm of Tretment :
1- control infection & inflammation via antibiotics .
2- Removal of secreation via postural drainage &
bronchodilators .
When? Decision to treate.
Which? Choice of Antibiotics
How? Dose route duration
47.
48. Mild : ttt of excerbation of bronchitis
( H.influenza & Strept.P)
Moderate&Sever :
Psudompnous & Staph aureous
49. Pathogen-Specific Therapy
Nafcillin or
oxacillin
Staphylococcus aureus,
methicillin-susceptible
S. aureus,
methicillin-resistant
Vancomycin
or linezolid
P. aeruginosa .IV macrolide or
•fluoroquinolone &
• aminoglycoside IV,
• orquinolone
• and antipseudomonal
B-lactam