Respiratory Cases

Penny is brought in by her parents for her 18 month check up.

This is your first time seeing Penny as they recently relocated to your town from the state of
Nevada. They brought prior records with them as you are reviewing the records you note the
following:
Penny is an 18 month old caucasian female. She was born at 39 weeks, normal, vaginal
delivery, 6 lbs., 2 oz, no complications.
Penny has been hospitalized with pneumonia 3 times since birth and has been treated multiple
times with antibiotics for frequent sinusitis/ear infections.

On Pennys physical exam you note the following:

Her height and weight are in the 5th percentile, which is consistent with her prior records.
She has a wet, productive cough, no fever.
Mild clubbing of her fingers

Based on your review of records and Pennys clinical presentation

What concerns do you have about her pulmonary history?
Recurrent lung infection in 18 months of life with hospitalization
Frequent Sinusitis
Frequent ear infection

What pulmonary disease are you concerned about?

- Cystic fibrosis
What further testing/information might you want to obtain?
- genetic testing
- Sweat test
- X-ray of chest

Outline the pathophysiology of CF and explain why the sweat test is the diagnostic
standard.
- In cystic fibrosis, there is a defect in the cystic fibrosis transmembrane receptor regulator
(CFTR) gene. This gene regulated the secretion of chloride ion and hence, there is reduction in
cl- secretion. The dehydration that ensue due to increased sodium and water reabsorption
makes the mucus in the airway thickened, and unable to perform its protective functions. The
individual are consequently exposed to recurrent lung infections. The decreased chloride
secretion occur round the body. The sweat test is used to test for the level of chloride in the
sweat, in which case, it is expected to be reduced in CF
 Patient presents with week long hx of increasing SOB
He is having some pain with inspiration on the Left.

Physical exam reveals decreased BS on the LEFT and dullness to percussion.

Chest X-ray reveals: an area of opacity in the left hemithorax with possible meniscus sign
depending on the magnitude of effusion

Pleural tap is performed. ID the TYPE of effusion and possible CAUSES based on the fluid
obtained during the tap:

1. Watery fluid
a. Type:Transudative PE
b. Cause: CCF or Chronic renal failure
2. Fluid with high concentration of plasma proteins and WBCs
a. Type:Exudative PE
b. Cause: pneumonia , bronchogenic carcinoma
3. Milky fluid containing lymph and fat droplets
a. Type: chylothorax
b. Cause: Rupture of cisterna chyli due to trauma
4. Frank blood
a. Type: Hemothorax
b. Cause: Aortic dissection, Aortic rupture from RTA
5. Purulent fluid, cx growth + for Staph aureus
a. Type: Empyema thoracis
b. Cause: Pulomonary TB

CO2 and O2
Mr. Jones is a 65 year old gentleman admitted with severe COPD exacerbation secondary to
viral URI. His ABG on RA: pH 7.30, pO2 74, pCO2 50; He is transferred to your unit on 100%
NRB mask.

Mr. Smith is a 65 year old gentleman admitted with ARDS and is on mechanical ventilator
support with FiO2 1.0

Both patients are currently receiving 100% O2

What are your concerns regarding high levels of O2 support for these 2 patients?
- Oxygen is a vasoconstrictor, and there may be reduced perfusion of peripheral
tissues
- Increased carbondioxide retention
- Oxidant injury via formation of reactive oxygen species, which leads to cellular
injury and death
- Seizures have also been reported in hyperbaric oxygen therapy

Outline the physiologic effects of high O2 support on each patient.

- Hyperbaric O2 improves breathlessness in COPD during exercise. Several
mechanisms have been reported for achieving this. Decrease in minute ventilation,
decrease in dynamic hyperinflation, reduction in the hypoxic pulmonary vasoconstriction,
reduction in the pulmonary vascular resistance and eventually increase in cardiac.
Improvement in breathlessness could also result from improvement in the ventilatory
muscle function
- High pressure O2 administration in ARDS reduces minute ventilation and work of
breathing in such patients mostly via reduction in respiratory rate with compensatory
increase in tidal volume. This may prevent muscle fatigue in ARDS. Eventually Mr Smith
could be put off mechanical ventilator
Case Study #3

An obese 67 year old gentleman presents to your clinic with C/O fatigue, chest discomfort,
dyspnea on exertion. He has a 20+ year hx of obstructive sleep apnea (OSA), but does not
routinely wear his home bipap machine because it makes him feel like hes suffocating.

He has no prior cardiac history or significant family hx or cardiac disease

PE reveals +JVD and peripheral edema

Labs: negative cardiac enzymes

PFTs: normal FEV and FEV1

ECHOCARDIOGRAM reveals right ventricular hypertrophy and Pulmonary artery pressure of

approximately 55 mmHg

CXR reveals:
enlarged Right and Main Pulmonary arteries and right heart border (dilated right atrium)

What is your suspected diagnosis?

Right heart failure secondary to pulmonary hypertension ( Cor pulmonale)

Outline the pathophysiology of this diagnosis

- The Rt heart failure seen in Corpulmonale results from increase in pulmonary
vascular resistance. Increase in the pulmonary hypertension can result from anumber of
mechanisms- pulmomary vasoconstriction due to alveolar hypoxia, anatomic changes in
the pulmonary vascular bed due to parenchymal or alveolar lung disease, increased
blood viscosity due to blood disorders( Sickle cell, polycythemia) , or idiopathic primary
pulmonary hypertension

and
 Explain HOW this pulmonary disorder is causing cardiac abnormalities
- The Rt ventricle is a thin-walled chamber that responds more to changes in
volume than pressure. Due to increased afterload in pulmonary hypertension, the Rt
ventricle increase systolic pressure to maintain the pressure gradient, and this eventually
leads to Rt ventricular hypertrophy, increase in Rt ventricular end-diastolic pressure and
Rt ventricular collapse over time. The resulting Rt vent stroke volume due to increase
afterload leads to reduction in Lt ventricular end-diastolic volume, and consequently
reduced CO; The result is reduction in the rt coronary circulation because the rt coronary
artery branches off the aorta.

Case Study #4

The patients flow volume loop is the orange figure below

(Picture is in the book pg. 435)

Use the PFT values and the shape of the flow-volume loop to answer the following questions:
1. Based on the PFT data, what TYPE of lung disease can you say this patient has?
- Restrictive lung disease

2. What are some causes of this type of lung disease?

- Interstitial pulmonary fibrosis, ALS ,kyphoscoliosis, pleural effusion

Case Study #5

Pulmonary Function Tests reveal the following:

FCV: normal
FEV1: reduced
FEV1/FVC ratio: reduced

The patients flow volume loop is marked with the orange arrow below

(picture is in the book pg. 436)

 Use the PFT values and the shape of the flow volume loop to answer the following questions:
1. Based on the PFT data, what TYPE of lung disease can you say this patient has?
- Obstructive airway disease

2. What are some causes of this type of lung disease?

- COPD, asthma