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Lecture 1

Pulmonary I - Introduction, Normal & Vascular Diseases

The entire respiratory tract with the exception of true cords, epiglottis, and aryepiglottic folds are lined by what types of cells? Pseudostratified columnar epithelium. Most are ciliated and some produce mucous (goblet cells). Basal cells also exist, which are thought to be precursor for cells of the lung
What are Kulchitsky cells found in the lungs? Neuroendocrine cells that contain a variety of hormonally active polypeptides and vasoactive amines
Type of cell of the lung that makes up only 40% of the cells in the alveoli, but covers 95% of the alveolar surface. Mainly responsible for alveolar gas exchange. Type I pneumocytes
Type of cell of the lung that makes up 60% of the cells in the alveoli. These cells produce surfactant and also important for response in lung injury. Type II pneumocytes
Type of cell of the lung that are non-ciliated secretory cells Clara cells
What is the dual supply of the lung productive against? Ischemic injury
Describe the primary function of the pulmonary blood supply Primary function is gas exchange. Low pressure system consisting of arterioles, capillaries, venules, and veins.
Describe the primary function of bronchial arteries Originates off the aorta and follows the bronchial tree. Anastomoses distally with pulmonary circuit. Serves a nutritive function.
What are the mechanical and cellular defense mechanisms of the lung against environmental particles/insults (1) Nasal clearance (2) Tracheobraonchial clearance (3) Alveolar clearance
When does the surfactant production begin during embryonic development? Week 20-22
At what age are majority of alveoli develop? Most alveoli develop after birth through 2 years.
Signs of Acute/Active Pulmonary Injury (1) Inflammatory cells (2) Edema (3) Hyaline membranes (4) Fibroblastic changes (5) Myxoid fibrosis (6) Presence of regenerating cells
Signs of Chronic Pulmonary Injury (1) Mature collagen (2) Hyperplastic smooth muscle (3) Metaplasia (4) Honeycomb changes grossly
Why are certain upper respiratory diseases much more significant in children than adults? The diameter of airways in children are smaller than in adult, leading to a higher likelihood of functional obstruction of the airway
Describe the mechanism of atelectasis (1) bronchial obstruction (2) external compression of lung parenchyma (3) intrinsic inability to expand the lung
A condition characterized as compressive accumulation of air in the pleural cavity resulting in collapse of the lung. Most commonly occurs spontaneously, but also occurs due to injury or disease. Pneumothorax
Describe the mechanism of pulmonary edema (1) Increase in hydrostatic pressure within capillaries (2) Decrease in oncotic pressure in capillaries (3) Increased permeability of capillary wall secondary to injury/inflammation
Most common origin of pulmonary emboli Venous thrombosis of lower extremities occurring in the setting of venous stasis
Describe the pathophysiology of sudden death by massive pulmonary emboli. The obstruction of the pulmonary artery leading to a (1) marked decrease in left ventricular filling  (2) acute decrease in cardiac output  (3) hypotension  (4) hypoperfusion to heart and brain
What are the chances of developing a second pulmonary emboli without anticoagulation? 30%
What are the clinical symptoms of pulmonary emboli? (1) Dyspnea (80%) (2) Tachypnea (88%) (3) Pleuritic chest pain (62-80%) (4) Cyanosis
What percentage of pulmonary emboli results in infarction? 15-20%
What percentage of pulmonary emboli results in infarction in the setting of congestive heart failure? 90%
Etiology of cavitating pulmonary infarction Infectious embolus
What are the clinical symptoms of pulmonary infarction? (1) Dyspnea (2) Hemoptysis (3) Pleuritic chest pain (4) Cough (5) Fever
What is the most common cause of pulmonary hypertension? Pulmonary venous congestion
What is the prognosis of Primary pulmonary hypertension? Poor prognosis: death within 2-3 years of diagnosis in >50% of patients
What is the median age of Primary veno-occlusive disease? Median age: 15 years
What are the symptoms of pulmonary hypertension? (1) Fatigue (2) Dyspnea on exertion/rest (3) recurrent syncope (4) chest pain (4) cor pulmonale (5) sudden death
What are the histological findings in Grade I-III pulmonary hypertension? (1) muscular hypertrophy (2) Fibrosis
What are the histological findings in Grade IV-VI pulmonary hypertension? (1) plexiform lesions (2) arterial dilatation (3) Fibrinoid necrosis
A vasculitis characterized by (1) Necrotizing granulomatous lesion (2) necrotizing vasculitis Wegener’s Granulomatosis
Most common lesion of the kidney in Wegener’s Granulomatosis Necrotizing glomerulonephritis
Organs classically involved in Wegener’s Granulomatosis (1) Upper respiratory tract (nose, nasal sinus, nasopharynx) (2) lungs (3) kidneys
Classic symptoms of Wegener’s Granulomatosis (1) Nasal bleeding and/or purulent discharge (2) Hemoptysis (3) cough (4) Chest pain (5) fever
Diagnostic workup of Wegener’s Granulomatosis (1) Biopsy (2) c-ANCA (+)
An autoimmune disease characterized by antibodies against pulmonary and renal basement membranes. 90% of patients are male with median age of 21 years. Goodpasture’s Disease
Symptoms of Goodpasture’s Disease (1) Hemoptysis (94%) (2) Dyspnea on exertion (3) Gross hematuria
Prognosis of Goodpasture’s Disease Median survival is 3 months. 5% survive 3 years.
Pathophysiology of pulmonary hemorrhage secondary to Systemic Lupus Erythematosus Intra-alveolar hemorrhage attributable to acute necrotizing vasculitis involving small blood vessels of the alveolar septae
Histological findings of SLE with pulmonary involvement Granular staining for immunoglobulin and complement along the alveolar basement membranes
Idiopathic Pulmonary Hemosiderosis Recurrent hemoptysis of unknown etiology. 80% of patients are <16 years of age. No sex predilection. No renal involvement.
Created by: UVAPATH2