Airway Disorders Flashcards
What is the most common cause of severe chronic lung disease in young adults?
Cystic Fibrosis (CF)
What is the hallmark lab finding in all CF patients?
Sweat Cl >60 on TWO occasions
What is the typical CF pt look like (general appearance)?
Young patient who looks malnourished or who has a low BMI
What is pathophysiology behind CF?
Its a genetic disorder that causes mucous buildup and tissue damage in the lungs due to abnormalities in a membrane chloride channel.
Where do you hear the crackles in a CF patient? apical or base?
apical crackles
Is there a cure for CF?
NO, you just basically treat symptoms and REFER to CF center!
What are some symptoms that CF patients present with?
Chronic PRODUCTIVE cough Dyspnea Wheezing low exercise tolerance Hemoptysis Chronic rhinosinusitis sx steatorrhea, diarrhea, abdominal pain
What disease is caused by abnormal dilation and destruction of the bronchial walls?
Bronchiectasis
Bronchiectasis favors M or W?
Women!
What are some causes of Bronchiectasis?
CF!!, Obstruction, Infection
Where do you hear the crackles in a patient with Bronchiectasis?
Bronchiectasis = BASE
Patients with bronchiectasis have similar symptoms to those with CF, what is a unique sx of bronchiectasis?
FOUL smelling sputum
What imaging modality best diagnoses Bronchiectasis?
HIGH RES CT!
When you see imaging descriptions such as “tram tracks”, “ring shadow” or “gloved finger” you should think of what disease as part of your differential?
Bronchiectasis
How do you treat Bronchiectasis?
EMPIRIC ABX- Amoxicillin
Inhaled bronchodilators - to help them breath due to mucous accumulation
HYDRATE
VACCINE
Stridor is best heard on expiration or inspiration?
Inspiration, best over upper chest and back
in severe bronchiectasis, what PE finding is always present?
Clubbing
What is is the most common organism recovered from non-cystic fibrosis patients with bronchiectasis?
H. Flu –> this is why you need the vaccine in the management
T/F. Cystic fibrosis patients have an increased risk of malignancies of the GI tract, osteopenia, and arthropathies?
true
What are non-pulmonary disorders that are commonly seen in patients presenting with undiagnosed CF?
Pancreatitis and infertility
Nearly all men with CF have congenital bilateral absence of what?
vas deferens with azospermia (remember infertility!)
What kind of pattern is seen on PFTs with CF?
mixed obstructive and restrictive pattern
What is the only definitive treatment for advanced CF?
lung transplant
Beside the seasonal flu shot which other vaccine is recommended in CF patients?
pneumococcal.
Death from cystic fibrosis is a result of:
pulmonary complications (pneumonia, pneumothorax, hemoptysis), or terminal respiratory failure and cor pulmonale.
T/F. CF have a smaller incidence of atypical mycobacterial colonization.
False. Yearly screening with sputum acid-fast bacilli cultures is advised
2nd highest death rate of CF, behind caucasians?
American Indians
