Term
| Name the four stages of lung development and when they occur during gestational age. |
|
Definition
| pseudoglandular (1-15), canalicular (15-25), terminal sac (25-40), alveolar (40-8yrs old) |
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Term
| During what phase is surfactant produced? |
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Definition
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|
Term
| During which phase are early respiratory efforts seen? when is respiraiton effective? |
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Definition
|
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Term
| What happens during the alveolar phase? |
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Definition
| alveoli continue to divide and grow |
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Term
| Inspired object is likely to go down which mainstem bronchus? |
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Definition
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Term
| How many alveoli per lung? |
|
Definition
|
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Term
| Compare/contrast type I and II pneumocytes. |
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Definition
type I is >95% of surface area and resonsible for gas exchange, can't replace themselves
type II are more numerous. They produce surfactant and responsible for repair by dividing and replacing type I pneumocytes |
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Term
| The interstitium of the lung is made up of... |
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Definition
| septal wall, collagen, elastin, capillaries, alveolar epithelium, lymphocytes |
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Term
| What are the two blood supplies of the lung? |
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Definition
| pulmonary artery from RV and bronchial arteries from aorta |
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Term
| What is pulmonary hypoplasia? |
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Definition
| decreased lung weight to body weight ratio |
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|
Term
| What percent of neonates have pulmonary hypoplasia? |
|
Definition
|
|
Term
| What are some common causes of pulmonary hypoplasia? |
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Definition
| fetal abdominal mass (kidney), diaphragmatic hernia, impaired fetal respiratory efforts (ligohydramnios, maternal uterine space occupying mass), dwarfism syndromes |
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|
Term
| How do you diagnose pulmonary hypoplasia? |
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Definition
| radiology (PN U/S), and clinical with respiratory distress and small bell-shaped chest; lung weight at autopsy confirms |
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|
Term
| What is the mortality rate of pulmonary hypoplasia? |
|
Definition
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Term
| What are the five main types of tracheoesophageal fistulas? |
|
Definition
| EA with distal TEF (87%), isolated EA (8%), isolated TEF (4%), EA with proximal TEF (1%), EA with double TEF (1%) |
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|
Term
| Obstructive and restrictive lung diseases are both types of _____. |
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Definition
| diffuse Pulmonary Diseases |
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Term
|
Definition
| collapse of previously inflated lung so lung is perfused but not ventilated |
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Term
| What is a major complication of Atelectasis? |
|
Definition
|
|
Term
| What are the three types of atalectasis? |
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Definition
| resorption due to airway obstruction (#1 type!), compression due to outside compression, and contraction due to scarred diseased lung |
|
|
Term
| Is Atalectasis reversible? |
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Definition
| resorption and compression atelectasis are reversible but contraction is not |
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|
Term
| A decreased FEV1/FVC is characteristic of what type of lung disease? |
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Definition
|
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Term
| Give examples of an obstructive lung disease of the bronchi. |
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Definition
| asthma, crhonic bronchitis/bronchiolitis, bronchiectasis |
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|
Term
| Emphysema is an obstruction at the level of the ______. |
|
Definition
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|
Term
|
Definition
| clinical grouping of smoking related obstructive lung diseases (chronic bronchitis, emphysema, bronchiolitis) that affects 10% of US and is the fourth leading cause of death. IRREVERSIBLE |
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|
Term
| What is chronic bronchitis? |
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Definition
| defined clinically as a productive cough x 3 mo for 2 yrs accompanied by hypoxemia and hypercapnia. "blue bloater" Caused by mucus in airway |
|
|
Term
| What are the histological characteristics of lung with chronic bronchitis? |
|
Definition
| increase mucus glands and increased glbolet cells |
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|
Term
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Definition
| determines thickness of submucosal glands and thickness of bronchial wall (basement membrane to cartilage) |
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|
Term
| What is the definition of emphysema? |
|
Definition
| defined histopathologically as the destruction and abnormal enlargement of airspaces distal to the terminal bronchiole (acini) |
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Term
| What is the process that leads to alveoli damage in emphysema? |
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Definition
| cigarette smoke --> elastase release from PMNs --> tissue damage |
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|
Term
| What are the two main forms of emphysema? |
|
Definition
| centriacinar and panacinar |
|
|
Term
| How do you clinically diagnose emphysema? |
|
Definition
progressive dyspnea, weight loss
gas exchange is normal until end stage "pink puffers" |
|
|
Term
| Characterize centriacinar emphysema. |
|
Definition
| #1 type (>95%), upper lobes and patchy as it progresses, highly linked to cigarates (anthracotic pigment deposition is a clue) |
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|
Term
| Characterize panacinar emphysema? |
|
Definition
| 5% of emphysema, diffuse but most severe in lower lobes, associate with alpha1-antitrypsin deficiency (autosomal recessive, chrom 14) |
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|
Term
|
Definition
| permanently dilated bronchi due to destruction of surrounding support tissues such as muscle, elastin |
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|
Term
| hat causes bronchiectasis? |
|
Definition
|
|
Term
| What are the clinical symptoms of bronchiectasis? |
|
Definition
|
|
Term
| What do you need to diagnose bronchiectasis? |
|
Definition
| clinical story + radiological findings |
|
|
Term
| What types of diseases can cause bronchiectasis? |
|
Definition
| CF, immunodeficiencies, Kartagener |
|
|
Term
| What percent of kids have asthma? |
|
Definition
|
|
Term
| Atopic versus non-atopic asthma. |
|
Definition
| atopic accounts for 70% of asthma and equates to TH2 mediated immune response to an environmental trigger |
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|
Term
| What are the clinical symptoms of asthma? |
|
Definition
|
|
Term
| What are the main features of asthma? |
|
Definition
| reversible airway bronchospasm, eosinophils, increased mucus production |
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|
Term
| What is the gross appearance of asthmatic lungs? |
|
Definition
| hyperexpanded with mucus plugs |
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|
Term
| What is the histological appearance of asthma? |
|
Definition
| mucus plugs in spirals (curshmann spirals), hyalinized bronchiolar basement membrane, increased peribronchial lsmooth muscle, eosinophils, charcot leyden crystals |
|
|
Term
| What are intestitial lung diseases? |
|
Definition
| heterogenous group of d/os involving diffuse small deposits of fibrosis (scar) |
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|
Term
| "ground glass infiltrates" on CXR indicates that the pt has... |
|
Definition
|
|
Term
| What is the gross description of a lung with ILD? |
|
Definition
|
|
Term
|
Definition
| 35% idiopathic, 25% pneumoconioses, 20% sarcoidosis, 10% collagen vascular disease, 10% drug induced |
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|
Term
| What is Idiopathic Pulmonary Fibrosis? |
|
Definition
| best characterized idiopathic ILD. happens to 40-70 yo; 3 yr survival rate. Grossly has a cobblestone lung. Histologically has "usual interstitial pneumonia UIP" with fibrosis, smooth muscle deposits, arterial thickening |
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|
Term
| What are the most dangerous particulates that cause pneumoconoises? |
|
Definition
| coal, silica quartz, and asbestos |
|
|
Term
|
Definition
| lung disease resulting from inhalation of particulates fumes or vapors in the environment |
|
|
Term
| What is the progression of carbon coal dust causing lung disease? |
|
Definition
| asymptomatic athracosis ---> anthracotic emphysema--> progressive massive fibrosis (PMF) with anthracosis in < 10% |
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|
Term
| Who is at risk for silicosis? |
|
Definition
| sandblasting, miners, stone cutters |
|
|
Term
| What is the progression of pneumoconioses caused by silica? |
|
Definition
| hard, UL nodules --> superimposed TB --> progressive massive fibrosis (PMF) |
|
|
Term
| What is the number one cause of pneumoconioses? |
|
Definition
|
|
Term
| Who are at risk for asbestos related pneumoconioses? |
|
Definition
| miners, insulators, shipyard workers |
|
|
Term
| Describe the progression of pneumoconioses caused by asbestos. |
|
Definition
| pleural plaques -> asbestosis PMF --> malignancy |
|
|
Term
| Does asbestos cause cancer? which types? |
|
Definition
asbestos= 5x risk of lung cancer (+ smoking = 55x)
asbestos = 1000x mesothelioma risk (smoking no additive effect) |
|
|
Term
| Which types of lung diseases end in cor pulmonale? |
|
Definition
|
|
Term
| What is sarcoidosis? Who is at high risk for sarcoidosis? |
|
Definition
| systemic immune related disease of unknown cause that affects Lung (95%), LN, spleen and liver. between 20-40 yrs old B>W and F>M |
|
|
Term
| What is classic radiological finding on CXR for sarcoidosis pt? |
|
Definition
| bilateral hilar LAD in 90% of cases |
|
|
Term
| What is the prognosis of sarcoidosis? |
|
Definition
| 2/3 of pts recover all function. 1-5% mortality prob due to ILD |
|
|
Term
| What is the histological appearance of sarcoidosis? |
|
Definition
| systemic non-caseating granulomata associated with lymphatics and vessels (schaumann bodies and asteroid bodies) |
|
|
Term
| How do you diagnose sarcoid? |
|
Definition
| diagnosis of exclusion= rule out other granulomatous conditions (fungal and TB) |
|
|
Term
| What are the collagen vascular disorders that can cause ILDs? What increases your risk? |
|
Definition
| lupus, rhumatoid arthritis, systemic sclerosis (pregnancy and smoking increase risk) |
|
|
Term
| What drugs can cause ILD? |
|
Definition
| amiodarone, methotrexate, bleomycin |
|
|
Term
| What is the number one cause of pulmonary edema? |
|
Definition
| cardiogenic (left sided CHF causes increased PV pressure and pulmonary edema) |
|
|
Term
| What are other causes of pulmonary edema besides CHF? |
|
Definition
| ARDS, pnumonia, drugs, high altitude |
|
|
Term
| What are the clinical signs of pulmonary edema? |
|
Definition
| SOB, orthopnea, PND, LE edema, crackles (same as for those in CHF) |
|
|
Term
| How do you tell there is pulmonary edema on gross lung? |
|
Definition
| wet, heavy lungs with frothy fluid in bronchi and parenchyma on cut-section |
|
|
Term
| How do you tell pulmonary edema histologically? |
|
Definition
| pink, semitransparent fluid in airspaces |
|
|
Term
|
Definition
| clnical syndrome of rapid respiratory insufficiency with severe hypoxemia. Equivalent to diffuse alveolar dammage (DAD) Due to vascular injury more than alveoli epithelial damage |
|
|
Term
|
Definition
Aspiration/Acute pancreatitis
eR (trauma)
Drowning/Drugs
Sepsis |
|
|
Term
| How long does it take ARDS to occur after trigger? |
|
Definition
|
|
Term
| What are the three phases of DAD? |
|
Definition
| exudative (wk 1), organizing (wk 2-3), fibrotic (>wk 3) |
|
|
Term
| The exudative phase of DAD is characterized by.. |
|
Definition
| hyaline membrane, intraalveolar edema and hemorrhage |
|
|
Term
| What characterizes the organizing phase of DAD? |
|
Definition
| hyperplasia of type II pneumocytes |
|
|
Term
| What causes Pumonary Thromboemboli (PTE)? |
|
Definition
| > 95% from large veins in legs. true thrombi (5%) occur only in significant CHF or primary arterial hypertension |
|
|
Term
| What are the situations/conditions that predispose one to a PTE? |
|
Definition
| post-operative, obesity, OCPs, cancer >> factor V Leiden, APL syndrome |
|
|
Term
| Medium PTE cause... (symptoms) |
|
Definition
| pulmonary infarct with peripheral wedge-shaped and hemorrhagic due to dual blood supply with transient chest pain, SOB, + CXR |
|
|
Term
| What is the recurrence risk of PTE? |
|
Definition
|
|
Term
| What are the symptoms of large ("saddle") PTEs? |
|
Definition
| sudden death (>50,000 deaths/year) 30% of autopsies |
|
|
Term
| What is pulmonary arterial hypertension? |
|
Definition
| when the pressure in the pulmonary artery rises to > 1/4 the systemic BP (normal- Pulmonic BP= 1/8 Systemic BP) |
|
|
Term
| Most PAH is secondary to various conditions. Name a few. |
|
Definition
| recurrent PTE, left-sided CHF, diffuse airspace disease (obstructive or restrictive), MV stenosis |
|
|
Term
| Idiopathic PAH is common in what population? |
|
Definition
|
|
Term
| Where is the gene, what is the inheritance, and what is the penetrance of familial PAH? |
|
Definition
| BMPR2 (chrom 2), AD, < 20% penetrant |
|
|
Term
| What is the gross pathology of PAH? |
|
Definition
| atheromas, RVH (>.4cm), RV and RA dilatation |
|
|
Term
| What is the histological pathology of PAH? |
|
Definition
| medial hypertrophy and plexiform lesions in extreme form |
|
|
Term
| What is diffuse alvolar hemorrhage? |
|
Definition
| bleeding into the alveolar space |
|
|
Term
| What are the classic symptoms of diffuse alveolar hemorrhage? |
|
Definition
| hemoptysis, anemia, diffuse pulmonary infiltrates (may see hemosiderin if more chronic/recurrent bleeding) |
|
|
Term
| What are the two main causes of Diffuse Alveolar Hemorrhage? |
|
Definition
| goodpasture syndrome and Wegener granulomatosis |
|
|
Term
| What is Goodpasture syndrome? |
|
Definition
| AI attack on alvolar septal basement membrane and glomerular basement membrane by anti-GBM antibody. |
|
|
Term
| Describe the typical Goodpasture's patient. |
|
Definition
|
|
Term
| What is Wegener's Granulmatosis? |
|
Definition
| AI attack on vasculature by ANCA antibody causes vascultitis and granulomata in lung and problems in sinuses, kidneys and joints |
|
|
Term
| What is the typical patient with Wegener Granulomatosis? |
|
Definition
|
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