U-world (Respiratory-Path)

Abnormal ciliary movement in the nasal epithelium could be due to abnormally thick mucus in CF, but could also be found in a primary ciliary dyskinesia, an example of this is called?

Kartagener's syndrome

Which leukotriene is the most potent chemotactic agent?

Leukotriene B4

What protein do eosinophils contain that causes significant damage to parasites?

Major basic protein

Superior sulcus tumors or mediastinal mass will cause superior vena cava syndrome

Mediastinal mass

The mechanism of caseous necrosis (TB) is not completely understood, but what is it mediated by? Describe what happens?

Mediated by Th1 lymphocytes. TH1 lymphocytes stimulate macrophages and CD8+ T cells. Ultimately, the macrophages undergo epithelioid and Langhans giant cell transformation

The most extreme case of extrapulmonary TB is called what?

Miliary tuberculosis

Greenish sputum is collected from a man with cough, what molecule accounts from the greenish color?

Myeloperoxidase (MPO)

What cell is myeloperoxidase located? Be specific?

Neutrophil; azurophilic granules

Does intraalveolar hemorrhage have any connection with acute pancreatitis?

No

What is the most common type of Hodgkin's lymphoma?

Nodular sclerosis

Describe hyperacute organ rejection?

Preformed Ab's against ABO or human leukocyte antigens (HLA) cause blood vessel spasm and diffuse intravascular coagulation with resultant ischemia

What is a normal Reid index?

0.4

Pulmonary artery hypertension follows a two-hit hypothesis?

1. Abnormal BMPR2 gene acts as the first insult and predisposes to pulmonary vascular disease 2. A second insult activates disease process resulting in vascular smooth proliferation in pulmonary vasculature

Vitamin D is obtained in 2 ways?

1. Absorbed from GI tract 2. Synthesized from precursors in skin after sunlight exposure

What are 5 different types of common inciting allergens?

1. Animal dander 2. Feathers 3. Dust mites 4. Mold 5. Pollens

Asbestos exposure increases the risk for which 2 cancers? Which is more common?

1. Bronchogenic carcinoma (more common) 2. Mesothelioma

What 4 enzymes act on arachidonic acid to create different products?

1. COX 1 2. COX 2 3. 5-lipoxygenase 4. 12-lipoxygenase

Describe acute rejection? What do X-rays show?

1. Cell-mediated with CD8 T cells playing a central role. Acute rejection causes vascular damage 2. X-ray show perihilar and lower lob opacities

What are the 4 inflammatory phases of infection with strep pneumo?

1. Congestion due to fluid extravasation into alveolar space 2. Red hepatization which is congestion due to PMNs and RBCs 3. Gray hepatization due to deposition of fibrinous material 4. Elimination of S. pneumo

Pulmonary function tests for pulmonary fibrosis reveals what about FEV1, FVC and FEV1/FVC

1. Decrease in FEV1 2. Decrease in FVC 3. Normal or increased FEV1/FVC

What is Virchow's triad?

1. Endothelial injury 2. Venous stasis 3. hyper-coagulable state

The top 3 most common causes of pulmonary fibrosis are?

1. Environmental exposure 2. Sarcoidosis 3. Collagen vascular disease

Most cases of asthma occur due to a combination of which 2 factors?

1. Genetic 2. Environmental

What are 2 examples of where intraalveolar hemorrhage would occur?

1. Goodpasture 2. Wegener's syndrome

Describe the 3 types of rejections seen in lung transplants? What is the time frame?

1. Hyperacute (minutes) 2. Acute (1-2 weeks) 3. Chronic (month or years)

Excessive Vit D supplementation may result in? (3)

1. Hypercalcemia 2. Painful bone demineralization 3. Confusion

What are 5 major causes of pulmonary artery hypertension?

1. Idiopathic (sporadic); hereditary 2. Left heart failure 3. Chronic hypoxia 4. Chronic thromboemolism 5. HIV infection

Squamous cell carcinoma shows what 2 features on histology?

1. Keratinization 2. Intercellular bridges

What are the 2 enzymes involved in the transformation of Vit D to calcitriol? Where are they located?

1. Liver: 25-hydroxylase 2. Kidney: alpha1-hydroxylase

EM of tumor cells from mesothelioma will show which 2 characteristics?

1. Long slender microvilli 2. Abundant tonofilaments

What are the 2 most common causes for superior vena cava syndrome?

1. Lung cancer 2. Non-Hodgkin lymphoma

What is the main difference between adenocarcinoma and mesothelioma?

1. Mesothelioma have long, slender microvilli 2. Adenocarcinoma have short, plump microvilli

What PG's are responsible for vasodilation and edema? (3) Vasodilation and Inhibition of platelet aggregation? (1) Vasoconstriction and platelet aggregation (2)

1. PGD2, PGE2, and PGF2 2. PGI2 3. Thromboxane

Treatment of neonatal respiratory distress syndrome involves? (2)

1. Positive pressure oxygen 2. Surfactant

The superior vena cava is formed by the union of which 2 veins?

1. Right brachiocephalic veins 2. Left brachiocephalic veins

What are 2 major risk factors for adult respiratory distress syndrome (ARDS)?

1. Sepsis 2. Shock

Why do neutrophils and macrophages release lysosomal enzymes in the setting of a lung abscess? (2)

1. Serve to digest the offending pathogens and tissue 2. Chemotactially summon additional neutrophils and macrophages

What is the main problem with squamous metaplasia within the bronchi? (2)

1. Squamous epithelium does not have cilia and goblet cells and is unable to provide mucociliary clearance 2. Squamous metaplasia can progress to squamous dysplasia

What are 2 physical findings you will find in someone with superior vena cava syndrome?

1. Swelling of the face 2. Dilated collateral veins

What are the 2 variants of scleroderma?

1. Systemic sclerosis 2. CREST syndrome

What is the main difference between transudate and exudate? What are the most common states you will see these?

1. Transudate: Low protein content--CHF 2. Exudate: High protein content--Inflammatory

Leukotriene C4, D4 and E4 function in what 3 ways?

1. Vasoconstriction 2. Bronchospasm 3. Increased vascular permeability

What is the role of bradykinin? (4)

1. Vasodilation 2. Increases vascular perm 3. Stimulates smooth muscle contraction 4. Mediates pain

When you think of asbestos exposure which occupation should you think of? (3)

1. shipbuilding 2. roofing 3. plumbing

About how long after initial infection with TB would a person mount a T cell helper response?

2-4 weeks

Interferon secretion by activated T lymphoctyes leads to macrophage activation which allows them to form epitheloid cells and improves their ability to kill intracellualr TB. This step occurs how many weeks after initial infection?

2-4 weeks

Pulmonary artery hypertension is defined as mean pulmonary artery systolic pressure of greater than?

25 mm Hg

Surfactant is produced by which types of cells?

Type II pneumocytes

What type of hypersensitivity is delayed?

Type IV

In CF pts the pancreas may become fibrotic and a deficiency in what particular vitamin may contribute to squamous metaplasia of the epithelial lining of pancreatic exocrine ducts?

Vitamin A

Pulmonary emboli often cause what characteristic infarctions?

Wedge-shaped, pleural based infarctions

PICTURE Pulmonary fibrosis

What disease state does this represent?

PICTURE Ferruginous bodies

What does this picture represent?

PICTURE Pulmonary edema; alveolar spaces

What does this picture represent? Where does the fluid accumulate?

PICTURE Hyaline; ARDS

What is lining the the alveoli in this picture; what disease state does this represent?

PICTURE Pulmonary edema; cardiomegaly, pleural effusions

What is this a picture of? Name 2 characteristics seen in this picture?

Rheumatoid arthritis + pulmonary fibrosis =

Rheumatoid lung disease

What is the only type of lung cancer that cannot be treated with surgery?

Small cell carcinoma

What is the calculation to figure out the Reid index?

Submucosal mucous gland/entire thickness (excluding cartilage)

Describe the pathophysiology of squamous metaplasia in the bronchi?

Substitution of the respiratory epithelium in bronchi with squamous epithelium occurs in response to chronic irritation, such as smoking

The immune reaction to TB is through what? This creates what type of granulomas?

TH1; caseating

Increased quantities of which type of T cell predisposes a child to asthma?

TH2

Describe the pathogenesis associated with retinal damage caused by oxygen therapy on a neonate?

Temporary local hyperoxia in the retina is thought to induce up-regulation of proangiogenic factors such as vascular endothelial growth factor

Why aren't hemosiderin-containing macrophages (siderophages-heart failure cells) seen in acute heart failure? Where are they seen?

They are seen in chronic left heart failure when erythrocytes are able to extravasate into the alveoli due to the increased pressures in the capillary bed. This takes time.

PICTURE Keratin pearls; squamous cell carcinoma

This arrow is pointing at what? What cancer is this associated with?

PICTURE Bilateral hilar adenopathy

This picture is indicative of what disease process?

PICTURE Pulmonary embolism

This type of infarction is most commonly caused by?

Increased pulmonary artery hypertension leads to?

cor pulmonale (right heart failure)

Recurrent respiratory infections and bilateral absence of the vas deferens are suggestive of?

cystic fibrosis

In tension pneumothorax where does the trachea deviate towards?

deviates away from effected lung

Minimal change disease, pathology?

effacement of the processes of glomerular podocytes

In a spontaneous pneumothorax the trachea deviates towards?

effected lung

Silicosis is often described as what on X-ray?

eggshell calcifications

Destruction of interalveolar septate would be expected in a pt with?

emphysema

Infiltrate in asthmatics will predominantly be which 2 cells?

eosinophils and mast cells (ME-mast/eosin)

Fat globule occlusion of the pulmonary vasculature is caused by?

fat embolism caused by fractures of long bones, pelvis or ribs

The clinical triad of acute-onset neurologic abnormalities, hypoxemia and a petechial rash in a patient with severe long bone and/or pelvic fractures is strongly suggestive of?

fat embolism syndrome

Trauma, non-focal neurologic disturbances and chest lesions consistent with thrombocytopenia all point in the direction of?

fat embolism syndrome

Pancreatic insufficiency caused by CF can cause a deficiency of?

fat soluble vitamins

Asbestosis is characterized by the formation of what on the parietal pleura?

fibrocalcific plaques

What is elevated in sarcoidosis?

ACE

Occasional sputum eosinophils and reduced FEV1 together suggest?

Allergen driven asthma

PICTURE Saddle embolism

An older pt undergoes hip surgery and then develops shortens of breath associated with this, what is this?

Diffuse scleroderma is associated with what antibody?

Anti-Scl-70 (anti-DNA topoisomerase I)

CREST syndrome is associated with what antibody?

Anticentromere (C for Crest and antiCentromere)

Why is there thrombocytopenia and petechiae associated with fat embolism syndrome?

Arise secondary to platelet adherence to and coating of the fat microglobules

Asbestos tends to effect what part of the lung, while silicosis and coal tend to effect?

Asbestos=base Silicosis and coal=apex Asbestos is from the roof, but effects the base Silica and coa are from the base (earth) and effect the roof (apex)

Cavities that are formed by TB frequently become infected with what other organism?

Aspergillus flavus

Which complement is important for activating neutrophils, monocytes, eosinophils and basophils?

C5a

CREST syndrome stands for?

C: Calcinosis R: Raynaud's E: Esophageal dysmotility S: Sclerodactyly T: Telangiectasia (small dilated BV near the skin)

Which infection is the most significant opportunistic infection in lung transplant recipients?

CMV

In TB a lower lobe lung lesion (Ghon focus) accompanied by ipsilateral hilar adenopathy is described as a?

Ghon complex

What other cell type, besides the columnar cells, of the respiratory tract are contained within the bronchi and secrete mucous to help trap inhaled debris?

Goblet cells

Pts with what type of inflammations frequently develop hypercalciemia?

Granulomatous disorders (sarcoidosis, TB, Hodgkin's and non-Hodgkin's lymphoma)

What is the role of myeloperoxidase (MPO)

H202-->HOCL by MPO

The main presenting pulmonary symptom in Goodpasture syndrome is?

Hemoptysis

Superior sulcus tumors (pancoast tumors) are often involved in what syndrome?

Horner's syndrome

B cell proliferation is driven by TH2 T cells which secretes what cytokine?

IL-4 (Hot T-Bone stEAk)

Nitrosamines and amides increase the incidence for which type of cancer? Be specific in the type of cancer

Intestinal stomach cancer

How are ferruginous bodies formed?

Iron that accumulates in the lung by coating asbestos fibers

Pulmonary fibrosis manifests clinically with?

gradual onset dyspena

Hepatic centrilobular necrosis is death of hepatocytes immediately surrounding what?

hepatic vein

Sarcoidosis pathophysiology?

immune-mediated, widespread noncaseating granulomas

95% of males with CF who survive to adulthood are found to be?

infertile

Primary TB infection is first established in which part of the lung?

lower

Mesothelioma usually effects what part of the lungs?

lower lobes

Once mycobacteria have proliferated in many macrophages what occurs next in an immunocompetent person?

macrophages (or dendritic cells) migrate to the lymph nodes and induce a T-cell response

Normal levels of Vit A and its metabolite, retinoic acid, are required to?

maintain orderly differentiation of specialized epithelia (including mucus-secreting columnar epithelium)

Mesothelioma is a malignant neoplasm arising from which type of cells?

mesothelial cells

What is a common complication of scleroderma in regards to the lungs?

microvascular injury of pulmonary arterioles leads to narrowing of the lumen and increased pressure in pulmonary circulation-->hypertrophy of right ventricle-->right-sided CHF

Once mycobacteria have settled in the lower lung fields what occurs next?

mycobacteria are phagocytosed by alveolar macrophages and mycobacteria proliferation occurs

Minimal change disease is classified as nephrotic or nephritic?

nephrotic

Alpha1-antitrypsin is the major inhibitor of?

neutrophil elastase

Sarcoidosis leads to development of which type of inflammatory tissue?

non-caseating granulomas

Atelectasis is diminished volume of part of the lung due to?

obstruction

Def in alpha1-antitrypsin can cause which type of emphysema?

panacinar emphysema

Bronchioalveolar carcinoma is a variant of adenocarcinoma and is usually located where in the lungs?

periphery

Sudden onset unilateral chest pain with hyperresonance and absent breath sounds implies?

pneumothorax

What is primary spontaneous pneumothorax?

pneumothorax in someone without pre-existing pulmonary disease and not caused by trauma

What is damaged in acute rejection?

pulmonary and bronchial vessels

Bilateral diffuse pattern of small, irregular opacities that are most pronounced in the lower lobes are seen in?

pulmonary fibrosis

Acute left ventricular failure with orthopnea and dyspnea can result in what?

pulmonary venous hypertension leading to transudate in alveolar lumen

Histologically, suppurative destruction of the lung parenchyma within the abscess cavity is seen and this destruction occurs due to?

release of lysosomal enzymes by neutrophils and macrophages

What are some clinical manifestations of sarcoidosis?

restrictive lung disease, erythema nodosum, bell's palsy, epithelial granulomas containing microscopic Schaumann and asteroid bodies, uveitis and hypercalcemia

One potential adverse effect of oxygen therapy is?

retinal damage

An AA presenting with constitutional symptoms, bilateral hilar adenopathy and pulmonary complaints is concerned for which disease process?

sarcoidosis

Erythema nodosum, arthralgias, hilar lymphadenopathy and elevated serum ACE levels are common findings in?

sarcoidosis

What is the most common liver finding in sarcoidosis?

scattered granulomas (noncaseating granulomas)

Asbestos exposure is the only risk factor for meothelioma, people who might have been exposed to this work at?

shipbuilding and insulation (industrial)

What is damaged in chronic rejection of a lung?

small airways

What is the most aggressive type of lung cancer?

small cell carcinoma

Exudation and alveolar hepatization are histologic features of pneumonia caused by?

strep pneumo

Does having a ghon focus/complex represent weak immunity or strong?

strong

The major contributor to wall thickening in chronic bronchitis is enlargement of which layer?

submucosal mucous gland enlargement

Compression of the superior vena cava causes?

superior vena cava syndrome

What is the demographic of those who are effected by pneumothorax?

tall thin males around the age of 20

In an obstructive lesion where does the mediastunum deviate toward?

toward the opacified lung (diseased)

During ARDS the interstitial and intra-alveolar edema, inflammation and fibrin depositon cause the alveoli to become lined with?

waxy hyaline membranes

After someone inhales TB, they are phagocytosed, walled off due to TH1 and turned into a ghon complex. Can TB survive in a dormant state within the macrophages?

yes

Does squamous bronchial metaplasia reversible upon discontinuation of smoking?

yes

PICTURE Fat embolism (used osmium tetroxide stain)

A man suffers a bone injury and develops dyspnea. A section of his lung is taken and you see this, what is it?

What is the pathophysiology behind fat embolism syndrome?

A traumatic event dislodges fat globules from the bone marrow which then occludes the pulmonary microvessels. The release of free fatty acids from the fat globules causes local toxic injury the the endothelium (ARDS)

Smoking increases the risk for which cancer in people who are exposed to asbestos?

Bronchogenic carcinoma

Which 3 complements are responsible for anaphylaxis that trigger histamine releases from mast cells?

C3a, C4a and C5a

Anergy is the inability to mount an immune response to foreign antigens and is often tested for by intradermal injection of what species? (this is because all of us have been exposed to this)

Candida

Which of the parameters is normal in acute respiratory distress syndrome? 1. Lung compliance 2. Work of breathing 3. V/Q matching 4. Capillary wedge pressure

Capillary wedge pressure

What causes capillary wedge pressure to increase?

Cardiogenic (hemodynamic) causes of pulmonary edema, such as pulmonary venous hypertension

Describe chronic rejection?

Causes inflammation of the small bronchioles (bronchiolitis obliterans). This leads to narrowing and obstruction of the effected bronchioli

High concentration of what in the sweat test is diagnostic of CF?

Cl-

What type of cells are found in the respiratory epithelium of the bronchi?

Columnar cells (possess motile cilia)

Pulmonary interstitial fibrosis causes alveolar to?

collapse

Elderly individuals who undergo hip surgery are at very high risk of developing posteroperative?

Deep venous thrombosis and pulmonary embolism

What type of hypersensitivity is elicited with TB infection?

Delayed-type hypersensitivity

What is the pathogenesis in ARDS?

Diffuse injury to pulmonary microvascular endothelium and/or the alveolar epithelium results in leaky alveolocarpillary membrane

Why do pts with granulomatous disorders develop hypercalcemia?

Due to elevated 1alpha-hydroxylase-mediated vitamin D activation in epithelioid macrophages

Why do neurological symptoms sometime exists with fat embolism syndrome?

Fat globules escape the emboli in the lungs and cause microvascular occlusion within the CNS

PICTURE right mainstem bronchus lesion; cancer that has blocked the right bronchus because he smokes

a 65 year old man comes in with progressive shortness of breath. he has decreased lung sounds over the right chest, this is his x ray, what does he have? most likely caused by?

Hyperresonance on auscultations means?

absent breath noises

Which type of rejection is successfully treated with immunosuppressants?

acute

Acute necrotizing pancreatitis is a major risk factor for which pulmonary disease?

adult respiratory distress syndrome

If a lung abscess communicates with an air passage, the semiliquid exudate within will create what type of cavity?

air containing cavity

What specific part of the lung does bronchioalveolar carcinoma grow?

alveolar septa

In the lung, the typical pathologic finding associated with secondary TB is where?

apical cavitary lesion

Though uncertain, the thought of primary pneumothorax is from rupture of?

apical subpleural blebs

Under the influence of inflammatory stimuli, cell membrane phospholipids release what, which contributes to the inflammatory response?

arachidonic acid

What is sclerodactyly?

atrophy of the skin of the hands

What is the classic feature found in pts with sarcoidosis in the lung?

bilateral hilar adenopathy

Chest X-ray for sarcoidosis is essential for diagnosis, x-ray often reveals?

bilateral hilar lymphadenopathy

Aniline dyes increase the incidence of which type of cancer?

bladder

Presence of which antibody is typical for Wegner's disease?

cANCA

What is the most active form of vitamin D?

calcitriol

Smoking causes which type of emphysema? This is prominent in the upper or lower lobes?

centriacinar; upper lobe

Examination of bronchi that reveal thickened bronchial walls, neutrophilic infiltrates, mucous gland enlargement and patchy squamous metaplasia represents?

chronic bronchitis

Periportal hepatic fibrosis is a pathologic finding found in?

chronic viral hepatits

The leading cause for chronic bronchitis is?

cigarette smoke

What induces asthma attacks in exercise-induced asthma?

cold air

Both variants of scleroderma, systemic sclerosis and CREST syndrome, develop due to increase deposition of what?

collagen in tissues