L11: Restrictive Respiratory Pathology

What is the primary cause of neonatal respiratory distress syndrome?

immature type II pneumocytes leads to low surfactant production

Ration of lecithin to sphingomyelin in Neonatal respiratory distress syndrome?

L/S ratio <1.5

COPD includes which disorders

emphysema, bronchiectasis, chronic bronchitis, asthma

Pulmonary changes characterized by enlargement of air spaces distal to terminal bronchiole which results in destruction of alveolar walls

emphysema

Most common cause of emphysema

cigarette smoke

Why does cigarette smoke cause emphysema

toxic and free radical damage to respiratory epithelium leads to an inflammatory response in the airways and release of proteases from neutrophils and macrophages

What happens when cigarette smoke alters protease-antiprotease mechanism?

proteases are free to lyse elastin and other structural proteins, leading to loss of alveolar elastic tissue and loss of septal tissue

Hereditary alpha 1 antitrypsin deficiency leads to

mutation in protease inhibitor

Young patient presents with emphysema symptoms but say they have never smoked cigarettes. What is most likely the cause?

hereditary alpha 1 antitrypsin deficiency

Breathing characteristics of emphysema pts

pursed lip breathing, hyperventilation

Emphysema increases resistance in pulmonary artery blood flow, leading to pulmonary hypertension and eventually..

RV failure

Pathogenesis of bronchiectasis

obstruction -> microbial proliferation (S. aureus, K. pneumoniae) -> inflammation -> necrotizing suppurative infection -> damage to epithelia -> impair mucociliary clearance -> destruction of muscle and elastic tissue secondary to erosive inflammatory pro

Morphology of bronchiectasis

abnormally dilated airways, with mucous plug

Clinical features of bronchiectasis

chronic, productive, mucopurulent cough, foul smelling secretion, hemoptysis likely

Persistent productive cough for at least 3 months per year during a period of 2 consecutive years

chronic bronchitis

Bronchitis is caused by inhaled irritants, which leads to bronchial wall thickening caused by

goblet cell hyperplasia, submucosal glandular hyperplasia, mucus hypersecretion

Bronchitis causes a change from columnar respiratory epithelium to multiple layers of squamous epithelium

bronchial squamous metaplasia

In bronchitis, the inflammation, glandular hyperplasia, and mucous hypersecretion leads to overall

airflow obstruction

Two types of bronchial asthma

1. intrinsic/non-atopic asthma 2. extrinsic/atopic asthma

Positive family history is common in which asthma?

extrinsic/atopic asthma

In extrinsic asthma, IL-4 stimulates which cells to produce IgE that locaizes mast cells?

stimulates B cells

Il-3 and Il-5 in asthma stimulates

eosinophil activation

Asthma attack that lasts days-weeks and does not respond to therapy; life threatening

status asthmaticus

Asthma shows up on xray as

hyperinflation, diaphragmatic flattening, increased bronchial cuffing, +/- mucous plugs

Restrictive pulmonary disease is characterized by

reduced expansion of lung parenchyma, reduced total lung capacity, decreased FVC and decreased FEV1

Major causes of restrictive pulmonary disease

chest wall disorders, acute or chronic interstitial diseases

Chronic interstitial disease that would cause restrictive pulmonary disease includes

idiopathic pulmonary interstitial fibrosis, pneumoconiosis, infiltrative disease

Idiopathic pulmonary interstitial fibrosis aka

diffuse interstitial lung disease

Ground glass appearance on chest ray is associated with

idiopathic pulmonary interstitial fibrosis

Lung disease caused by inhalation of dust particles

pneumoconiosis

Rod shaped, ferruginous bodies in sputum and lung interstitium from inhalation of asbestos fibers

asbestosis

Anthracotic deposits caused by inhalation of coal dust

anthracotic deposits

Sarcoidosis of salivary glands. Leads to xerostomia and increased risk for caries

mikulicz syndrome

Bilateral lymphadenopathy on chest xray is associated with

sarcoidosis

Sarcoidosis is associated iwth increased levels of

HLA-A1 and HLA-BB, increased ACE levels, hypercalcemia, polyclonal hypergammaglobulinemia

In sarcoidosis, the exaggerated interstitial and intra-alveolar CD4+ Th1 response to the unknown antigen leads to...

macrophage activation and noncaseating granuloma formation

Sarcoidosis is associated with caseating granuloma or noncaseating granuloma?

sarcoidosis = noncaseating granuloma; tuberculosis = caseating granuloma