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‫الجلسة‬ ‫ميثاق‬
Illustrate Tracheobronchial division of
right lung
 Illustrate Tracheobronchial division of
Left lung
UPPER
UPPER
Middle
Lower
Lower
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
History
Laennec in 1819
after Postmortem examination of
Lung of infant who died following
whooping cough
Laennec???
BRONCHIECTASIS
Bronchion=Windpipe
Ektasis=Stretching out
Q : Define BRONCHIECTASIS?
BRONCHIECTASIS
Abnormal permanent dilatation
of one or more bronchi
with chronic airway inflammation
associated
chronic sputum production
recurrent chest infection
& airway obstruction
BRONCHIECTASIS
Q: List Four causes of Decline
Prevalence
Unknown, DECLINE?
1. Introduction of antibiotics
2. Treatment of childhood infection
3. Effective Vaccination Programs
4. Decline of prevalence of Pulmonary TB.
More? Advance of HRCT ( diagnosis of subclinical)
Q: Enumerate five etiological
causes of bronchiectasis
Q:Describe pathogenesis of two
etiologies
aetiologies
•
Raghu G et al. AJRCCM 2011;183:788824.
Images purchased from iStockPhoto. PRC-2184
Immunological
Immunodeficiency
Congenital & acquired
Hyper immune
Developmental Defect :
Structural :
✓ Agenesis
✓ sequestrated segment
✓ Trachiobronchomegally
Alpha one antitrypsin
Biochemical
✓ Primary
✓ Secondary
infection
Inflammations
✓ Autoimmune
✓ Aspiration
✓ inhalation
Genetic
structural:
primary cillary
dyskinasia
Etiology
Pathogenic mechanism
Bronchites
Peumonia
Tuberculosis
Primary infective insult
Common varied
Selective or Functional IG↓
Secondry
hypogammaglobulenemia
HIV
Immunedeficiency Syndrom
Congenital & aquired
Allergic Bronchopulmonary
myocosis
Hyperimmune Response
Intraluminal ( Tumer- FB) 0r
extraluminal(
lymphadenopathy)
Infection secondary to
bronchial obstruction
IBD-SLE-RA- HP- inhalation
Aspiration Toxic gases &
gastric content
Agenesis
sequestrated segment –
Trachiobronchomegally
Alpha one antitrypsin
Inflammations &
autoimmune diseases
Developmental Defect :
Structural
Biochemical
Genitic :CF
Genitic : primary cillary
dyskinasia
Impairment of the mucus
clearance
Etiology
Pathogenic mechanism
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 .
1-What is your provisional diagnosis?
2- List three investigation To achieve the
diagnosis
3- outline the treatment of previous
condition
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:
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
Kartagener’s syndrome:
•Chronic sinusitis
•Absent Frontal sinus
•Otitis media
• bronchiectasis
•Situs inversus or isolated dextro cardia
•Male infertility (immotile spermatozoa
•Female infertility (immotile Fimberia)
Describe Clinical presentation
and diagnosis of Cystic
fibrosis.
Cystic Fibrosis
Cystic fibrosis (CF) is inherited
genetic disorders that present
as multi system Disease.
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
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
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
Diagnosis
Diagnosis
*Sweat test
*Nasal potential difference
*Tests of pancreatic function
*Genetic tests
*Screening
*Immunoreactive trypsin
* DNA analyses
*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
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
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
Describe pathogenesis of bronchiectasis.
Atelectasis theory
Pressure of
secretions theory
Traction theory
-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. -
Site:
Left side >Rt side
- Lower lobe
- linguala
- middle lobe
–Upper lobe
Classification
*Follicular
*Saccular
*Atelectatic
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
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
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
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.
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
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
Infective exacerbation
Haemoptysis
Sinusities
Brain Abscess
Amylodosis
Disseminated Asperegillosis
Systemic Hypersensitivity vacuities
Operative Complication
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
Mild : ttt of excerbation of bronchitis
( H.influenza & Strept.P)
Moderate&Sever :
Psudompnous & Staph aureous
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
Learning moment
Bronchectasis DISEASE MFM faculty 2017 .pdf

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Bronchectasis DISEASE MFM faculty 2017 .pdf

  • 1.
  • 2.
  • 4.
  • 5.
  • 6. Illustrate Tracheobronchial division of right lung  Illustrate Tracheobronchial division of Left lung
  • 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
  • 9. History Laennec in 1819 after Postmortem examination of Lung of infant who died following whooping cough Laennec???
  • 11. BRONCHIECTASIS Abnormal permanent dilatation of one or more bronchi with chronic airway inflammation associated chronic sputum production recurrent chest infection & airway obstruction
  • 12. BRONCHIECTASIS Q: List Four causes of Decline Prevalence
  • 13. Unknown, DECLINE? 1. Introduction of antibiotics 2. Treatment of childhood infection 3. Effective Vaccination Programs 4. Decline of prevalence of Pulmonary TB. More? Advance of HRCT ( diagnosis of subclinical)
  • 14. Q: Enumerate five etiological causes of bronchiectasis Q:Describe pathogenesis of two etiologies
  • 15. aetiologies • Raghu G et al. AJRCCM 2011;183:788824. Images purchased from iStockPhoto. PRC-2184 Immunological Immunodeficiency Congenital & acquired Hyper immune Developmental Defect : Structural : ✓ Agenesis ✓ sequestrated segment ✓ Trachiobronchomegally Alpha one antitrypsin Biochemical ✓ Primary ✓ Secondary infection Inflammations ✓ Autoimmune ✓ Aspiration ✓ inhalation Genetic structural: primary cillary dyskinasia
  • 16. Etiology Pathogenic mechanism Bronchites Peumonia Tuberculosis Primary infective insult Common varied Selective or Functional IG↓ Secondry hypogammaglobulenemia HIV Immunedeficiency Syndrom Congenital & aquired Allergic Bronchopulmonary myocosis Hyperimmune Response Intraluminal ( Tumer- FB) 0r extraluminal( lymphadenopathy) Infection secondary to bronchial obstruction
  • 17. IBD-SLE-RA- HP- inhalation Aspiration Toxic gases & gastric content Agenesis sequestrated segment – Trachiobronchomegally Alpha one antitrypsin Inflammations & autoimmune diseases Developmental Defect : Structural Biochemical Genitic :CF Genitic : primary cillary dyskinasia Impairment of the mucus clearance Etiology Pathogenic mechanism
  • 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
  • 22. Kartagener’s syndrome: •Chronic sinusitis •Absent Frontal sinus •Otitis media • bronchiectasis •Situs inversus or isolated dextro cardia •Male infertility (immotile spermatozoa •Female infertility (immotile Fimberia)
  • 23.
  • 24. Describe Clinical presentation and diagnosis of Cystic fibrosis.
  • 25. Cystic Fibrosis Cystic fibrosis (CF) is inherited genetic disorders that present as multi system Disease.
  • 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
  • 29. Diagnosis Diagnosis *Sweat test *Nasal potential difference *Tests of pancreatic function *Genetic tests *Screening *Immunoreactive trypsin * DNA analyses
  • 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
  • 33. Describe pathogenesis of bronchiectasis.
  • 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
  • 44.
  • 45. Infective exacerbation Haemoptysis Sinusities Brain Abscess Amylodosis Disseminated Asperegillosis Systemic Hypersensitivity vacuities Operative Complication
  • 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