Anatomic Alterations 

Bronchiectais 

• Chronic dilation and distortion of bronchial airways
• excessive production of often foul-smelling sputum
• bronchospasm 
• hyperinflation of alveoli (air trapping)
• atelectasis 
• consolidation and parenchymal fibrosis 
• hemmorrhage secondary to brocnhial arterial erosion

1. Cylindrical (Tubular)

2. Varicose (Fusiform)

3. Cystic (Saccular)

Acquired Bronchiectasis 

(Recurrent Pulmonary Infection)

• pneumonia 
• tuberculosis
• fungal infections 

In Children: 

• measles
• chickenpox 
• whooping cough 
• influenza

Acquired Bronchiectasis 

(Bronchial Obstruction) 

• aspiration of a foreign body
• mucous plugs
• tumors
• enlarged hilar lymph nodes

These conditions impair the mucociliary clearance mechanism  

• Recurrent Pulmonary Infection
• Bronchial Obstruction
• Inhalation and Aspiration

• Cystic Fibrosis 
• Kartagener's Syndrome 
• Systemic Disorders 

Congenital Bronchiectasis

(Cystic Fibrosis)

Kartagener's Syndrome is described as a triad disorder consisting of bronchiectasis, dectrocardia and rhinosinusitis. These patients have defective cilia lining throught the respiratory tract, lower and upper sinuses, Eustachian tubes, middle ears and fallopian tubes. 

-Accounts for as much as 20% of all congenital bronchiectasis

Congenital Bronchiectasis

(Systemic Disorders)

Clinical Findings 

Vital Signs & Physical Examination

Bronchiectasis 

• tachypnea 
• increased HR and BP
• use of accessory muscles 
• pursed-lip breathing 
• barrel chest 
• cyanosis 
• digital clubbing
• Peripheral Edema and Vein Distention   

Cough, Sputum and Hemoptysis 

 Bronchiectasis 

Chest Assesment Findings 

Bronchiectasis

In a Obstructive Bronchiectasis:

• Decreased Tactile Fremitus and vocal fremitus 
• Hyperresonant percussion note 
• Diminished breath sounds 
• Wheezing 
• Rhochi and wheezing 

In a Restrictive Bronchiectasis:

• Increased tactile and vocal fremitus 
• Bronchial breath sounds 
• Crackles
• Whispered pectoriloquy
• Dull percussion note  

PFT Test Findings for Moderate to Sever Bronchiectasis 

(When Primarily Obstructive) 

FVC decreased 

FEV1 decreased 

FEV1/FVC Ratio decreased 

FEF 25%-75% decreased 

FEF 50% decreased 

FEF200-1200 decreased 

PEFR decreased 

MVV decreasd 

Lung Volume and Capacity Findings 

in Moderate to Severe Bronhiectasis 

When Primarily Obstructive

Tidal Volume - Normal or Increased 

Inspiratory Reserve Volume - Normal or Decreased 

Expiratory Reserve Volume - Normal or Decreased 

Risidual Volume - Increased 

Vital Capacity - Decreased 

Inspiratory Capacity - Normal of Decreased

Fuctional Risidual Capacity - Increased 

Total Lung Capacity - Normal or Increased 

RV/TLC Ratio - Normal or Increased 

PFT Test Findings in Moderate to Severe Bronchiectasis 

When Primarily Restrictive

FVC - decreased 

FEVt - normal or decreased 

FEV1/FVC ratio - normal or increased

FEF25%-75% - normal or decreased 

FEF50% - normal or decreased 

FEF200-1200 - normal or decreased 

PEFR - normal or decreased 

MVV - normal or decreased 

Lung Volume and Capacity Findings 
Moderate to Severe Bronchiectasis

When Primarily Restrictive  

Tidal Volume - normal or decreased 

Inspiratory Reserve Volume - decreased 

Expiratory Reserve Volume - decreased 

Risidual Volume - decreased 

Vital Capacity - decreased 

Inspiratory Capacity - decreased 

Functional Risidual Capacity - decreased 

Total Lung Capacity - decreased 

RV/TLC ratio - normal 

ABG 

Mild to Moderate Stage 

Bronchiectasis

pH - increased 

PaCO2 - decreased 

HCO3 - decreased (slightly) 

PaO2 - decreased 

Acute Alveolar Hyperventilation with Hypoxemia 

(Acute Respiratory Alkalosis) 

ABG 

Severe Stage 

Bronchiectasis

pH - normal 

PaCO2 - increased 

HCO3 - increased (significantly)

PaO2 - decreased 

Chronic Ventilatory Failure with Hypoxemia 

Compensated Respiratory Acidosis 

• Streptococcus pneumoniae 
• Haemophilus influenzae 
• Psudomonas aeruginosa 
• Anaerobic organisms 

Chest Radiograph 

Obstructive in Nature 

• Translucent (Dark) in nature 
• Depressed or flattened diaphragms 
• Long and narrow heart 
• Enlarged heart 
• Areas of consolidation and/or atelectasis may or may not be seen 

Chest Radiograph 

(Primarily Restrictive in Nature) 

Bronchiectasis

• Atelectasis and consolidation 
• Infiltrates (suggesting pneumonia) 
• Increased opacity 

• excessive production and accumulation of thick, tenacious mucus in the TBT
• partial bronchial obstruction (mucous plugging) 
• hyperinflation of the alveoli 
• total bronchial obstrucition (mucous plugging) 
• atelectasis 

• Sometimes called the Sweat Chlorine Test
• Gold standard diagnostic test for CF
• During procedure a small amount of colorless, odorless sweat-producing chemical called pilocarpine is applied to the patient's arm or leg. An electrode is is attatched to the chemically prepared area and a mild electric current is applied to stimulate sweat production. 

• Children - greater than 60 
• Adults - greater thatn 80

Immunoreactive Trypsiogen Test 

IRT 

• ordered for (1) babies who are not creating enough sweat to perform a sweat test, or (2) infants with meconium ileus. 
• A blood sample is analyzed twice for a specific protien called tyrpsinogen - patients with CF have an elevated IRT

• Measures the amount of fat in the infant's stool and percentage of dietary fat taht is not absorbed by the body
• Under normal conditions the fat absorption is less than 7g of fat per 24 hours. 
• Elevated stool fecal fat value suggest CF.

• Measures the impaired transport of sodium and chloride across the epithelial cells lining the airways of the CF patient
• The NPD can be measured with a surface electrode over the nasal epithelial cells lining the inferior turbinate 
• An increased (more negative) NPD suggest CF. 

• With a sample of the patients blood a DNA test can be performed to analyze for the presence of CFTR gene mutations.

• Amniocentsis (2nd or 3rd trimester) - the amniotic fluid is obtained and tested to determine if both of the CFTR genes from the fetus are normal. 
• Chorionic villus biopsy (1st trimister) - with the aid of an ultrasound examination, a thin tube is inserted into the uterus to obtain a piece of the placenta 

PFT Test Findings 

Moderate to Severe CF

Forced Expiratory Flow Rate Findings 

Tidal Volume - Normal or Increased 

IRV - Normal or Decreased 

ERV - Normal or Decreased 

RV - Increased 

VC - Decreased 

IC - Normal or Decreased 

FRC - Increased 

TLC - Normal or Increased 

RV/TLC Ratio - Normal or Increased

ABG 

Mild to Moderate CF

pH - Increased 

PaCO2 - Decreased 

HCO3 - Decreased (Slightly) 

PaO2 - Decreased 

ABG 

Severe Stage of CF 

pH - Normal 

PaCO2 - Increased 

HCO3 - Increased 

PaO2 - Decreased 

• tachypnea 
• increased HR and BP 
• use of accessory muscles 
• pursed-lip breathing
• barrell chest 
• cyanosis 
• digital clubbing 
• distended neck veins 
• pitting edema 
• enlarged and tender liver 

Chest Assessment Findings 

Cystic Fibrosis 

• decreased or increased tactile and vocal fremitus
• hyperressonant percussion note 
• diminshed breath sounds 
• diminished heart sounds 
• bronchial breath sounds (over atelectasis) 
• crackles, rhonci and wheezing 

• Staphylococcus aureus 
• Haemophilus influenzae 
• Psuedomonas aeruginosa 
• Stenotrphomonas maltophilia 
• Burkholderia cepacia 
• Burkholderia pickettii 
• Burkholderia gladioli 

• Translucent (dark) lung fields
• Depressed or flattened diaphragms 
• Right ventricular enlargement
• Areas of atelectasis and fibrosis 
• Bronchiectasis 
• Pneumothorax 
• Abscess formation