Chapter 12: The Lungs (Part 2) Flashcards
What is Kartagener Syndrome?
Defective cilia
What is the genetic component of Kartagener syndrome?
autosomal recessive, mutated dynein
What respiratory syndrome has poor mucociliary clearance?
Kartagener Syndrome
What are the locations of poor mucociliary clearance in Kartagener syndrome?
Respiratory tract, sinuses, and eustachian tubes
Poor mucoiliary clearance in _______ _______ can cause female sterility.
fallopian tubes
Poor mucoiliary clearance in ____ ___ _______ can cause male sterility
flagella of sperm
Kartagener syndrome can lead to what three respiratory diseases?
Bronchiectasis, chronic sinusitis and situs inversus
What is a group of disorders that has decreased compliance of the lung?
Chronic Interstitial Lung Disease
What are some of the clinical features seen on a lung in Chronic Interstitial Lung Disease?
“ground glass shadows
“honeycomb lung”
diffuse/bilateral & patchy fibrosis
What are the two main causes of Chronic Interstitial Lung Disease?
1) Hypoxia/Dyspnea
2) Pulmonary HTN
What is the prognosis for Chronic Interstitial Lung Disease?
Progressive, can lead to respiratory failure and if combined with pulmonary HTN can lead to cor pulmonale
What are the major symptoms of Idiopathic Pulmonary Fibrosis?
Insidious onset of dyspnea, cyanosis
Nonproductive cough, velcro-like crackles
What is unique about the diagnosis of Idiopathic Pulmonary Fibrosis?
It is a diagnosis of exclusion
What does the biopsy reveal in Idiopathic Pulmonary Fibrosis?
Usual Interstitial Pneumonia
What is the hypothesis behind idiopathic pulmonary fibrosis?
repeated alveolar endothelial activation; faulty repair causes excess fibrosis
Who is at a greater risk for developing idiopathic pulmonary fibrosis?
Males, MC >60 years old
What is the prognosis for idiopathic pulmonary Fibrosis?
poor, progressive, avg. survival is <3 years
What type of Fibrosing Chronic Interstitial Lung Disease has a non-neoplastic lung reaction to the inhalation of particulates?
pneumoconioses