ENZYMES, GROWTH FACTORS, PROTEINS, AND RECEPTORS

Surfactant phospholipids

What substance is biosynthesized by Type II cells of the alveoli?

Type II (surfactant-secreting) cell

What specific alveolar epithelial cell is responsible for the biosynthesis of surfactant phospholipids?

Angiotensin I

What specific substance is metabolized by the pharmacokinetic function of the respiratory system?

Serotonin

What monoamine is metabolized by the pharmacokinetic function of the respiratory system?

NE (Norepinephrine)

What catecholamine is metabolized by the pharmacokinetic function of the respiratory system?

Prostaglandins

What lipid compounds are metabolized by the pharmacokinetic function of the respiratory system?

Surfactant proteins

What components of surfactant are first detected toward the end of the pseudoglandular period (12-14 weeks)?

Glucocorticoids

What substance accelerates surfactant synthesis and mesenchymal thinning during the Canalicular period?

Type I alveolar epithelial cells

What cell type, derived from acinar tubules during the Canalicular period, becomes squamous in shape?

Hydrogen ions (H+)

The concentration of what ion determines the pH level and acid-base balance in arterial blood?

Bicarbonate (HCO3)

What chemical buffer is produced by the kidneys to neutralize acid in the arterial blood?

Bicarbonate (HCO3)

What component of the ABG represents the metabolic component used to assess acid-base balance?

Bicarbonate (HCO3)

What substance's reabsorption is increased in the proximal convoluted tubules of the kidneys as compensation for respiratory acidosis?

Bicarbonate (HCO3)

What substance's reabsorption is decreased by the kidneys as compensation for respiratory alkalosis?

Lactate Dehydrogenase (LDH)

What pleural fluid component is measured and compared to its serum concentration in Light’s Criteria?

Pleural fluid protein

What substance in pleural fluid is measured and compared to its serum concentration in Light’s Criteria?

Streptokinase

What fibrinolytic agent is instilled intrapleurally alongside DNAse to lyse fibrin septations in a loculated pleural effusion?

DNAse

What enzyme is instilled via a chest tube with a fibrinolytic agent to reduce the viscosity of fluid in a loculated pleural effusion?

Talc or doxycycline

What medications are instilled during pleurodesis to induce a sterile local inflammatory response?

Phrenic nerve

Which nerve, located near the cervical lymph nodes, is susceptible to injury during a scalene node biopsy?

Internal carotid artery

What major vascular structure is susceptible to injury during cervical lymph node biopsy?

Subclavian vein

What major vascular structure is susceptible to injury during cervical lymph node biopsy?

Endoscopic stapler

What specialized tool is used during VATS to resect and suture a biopsied lung area simultaneously?

Radium needles

What objects are inserted via rigid bronchoscopy for brachytherapy in endobronchial malignancies?

IL-4, IL-5, IL-13

What interleukins are produced by type 2 CD4+ T-helper cells that drive Type 2 Inflammation in asthma?

IgE

What key antibody produced by B cells binds to mast cells and leads to the release of mediators upon allergen encounter in asthma?

Histamine

What mediator is released from mast cells after IgE binding in response to an allergen?

Anti-IL5

What targeted therapy is available to treat eosinophilic inflammation in asthma regulated by IL-5?

β2
-agonists

What class of bronchodilators is the agent of choice used for measuring the reversibility of airflow obstruction?

Salbutamol

What is an example of a β2-agonist bronchodilator commonly used in spirometry reversibility testing?

Beta-blockers

What class of heart medications can induce bronchoconstriction in some patients, potentially triggering asthma symptoms?

Bradykinin

The accumulation of what chemical is responsible for the cough induced by ACE inhibitors?

TNF-alpha

What cytokine is associated with cachexia seen in severe COPD or exacerbation?

Elastase

What enzyme is released by inflammatory cells during COPD pathogenesis that causes damage to the extracellular matrix?

MMP12 (Matrix metalloproteinase 12)

What key molecule in the Protease/Antiprotease pathway leads to extracellular matrix destruction in COPD pathogenesis?

Neutrophil Elastase

What enzyme is listed as a key molecule causing extracellular matrix destruction via the Protease/Antiprotease pathway in COPD?

SERPINA1

What molecule, associated with Alpha-1 antitrypsin deficiency, is involved in the Protease/Antiprotease pathway?

NF KappaB

What key molecule in the Oxidant/Antioxidant pathway contributes to chronic inflammation in COPD pathogenesis?

NRF2

What key molecule in the Oxidant/Antioxidant pathway is implicated in the chronic inflammation seen in COPD pathogenesis?

SOD3

What key molecule in the Oxidant/Antioxidant pathway is involved in chronic inflammation in COPD pathogenesis?

HDAC2

What key molecule in the Oxidant/Antioxidant pathway is implicated in the development of chronic inflammation in COPD pathogenesis?

Rtp801

What molecule is listed as a key molecule in the Apoptosis pathway, resulting in structural cell death in COPD pathogenesis?

Ceramide

What lipid molecule is listed as a key molecule in the Apoptosis pathway leading to cell death in COPD pathogenesis?

TGFBeta

What growth factor is listed as a key molecule in the Lung Repair pathway leading to ineffective repair in COPD pathogenesis?

Elastin

What extracellular matrix component undergoes disordered repair, contributing to air space enlargement and emphysema?

ICS (Inhaled Corticosteroids)

What class of medication is the cornerstone of asthma treatment?

Omalizumab (Anti-IgE therapy)

What specific biologic is considered at Step 5 of asthma management for patients with uncontrolled symptoms?

Montelukast (Leukotriene antagonist)

What medication can be added as adjunctive therapy for asthma at any step if symptoms remain uncontrolled?

P. aeruginosa (Pseudomonas aeruginosa)

What bacterium commonly colonizes airways in bronchiectasis and contributes to chronic neutrophilic inflammation?

H. influenzae (Haemophilus influenzae)

What bacterium is commonly targeted with antibiotics in the treatment of bronchiectasis?

Acetylcysteine

What mucolytic agent is given as part of bronchial hygiene to help loosen phlegm in bronchiectasis?

PAS-positive lipoproteinaceous material

What amorphous substance accumulates in the distal air spaces, characterizing Pulmonary Alveolar Proteinosis (PAP)?

Surfactant phospholipid and apoproteins

What components accumulate in the alveoli in PAP due to impaired clearance by macrophages?

Anti–GM-CSF antibodies

What causes Autoimmune PAP by impairing the maturation and function of alveolar macrophages?

GM-CSF (Granulocyte-macrophage colony-stimulating factor)

Inhibition of what cytokine causes defective macrophage function leading to Autoimmune PAP?

GM-CSF therapy

What noninvasive treatment is used for moderate Autoimmune PAP to restore macrophage function?

Rituximab (CD20 inhibitor)

What biologic is used for the management of refractory PAP?

Rituximab

What biologic (CD20 inhibitor) is used in the immunosuppressive treatment of underlying connective tissue diseases (CTD) causing ILD?

Nintedanib

What antifibrotic medication is used in the management of ILD to control the progression of fibrosis in the lungs?

Pirfenidone

What antifibrotic medication is used in ILD management to control the scarring in the lungs?

Cyclophosphamide

What cytotoxic agent is used in the immunosuppressive treatment of underlying CTD causing ILD?

MMF (Mycophenolate Mofetil)

What cytotoxic agent is used in the management of underlying CTD that leads to ILD?

Anti–acetylcholine receptor (AChR) antibodies

What antibodies, found in approximately 85% of generalized MG cases, characterize this autoimmune disorder?

Anti–MuSK antibodies

What less common antibodies are found in 5–8% of MG patients and are associated with prominent bulbar weakness?

Endothelin

What substance, overexpressed in PAH, promotes proliferation and vasoconstriction of smooth muscle cells?

Pro-endothelin-I

What upstream precursor molecule in the Endothelin pathway is targeted by ERAs?

Nitric oxide (NO)

What molecule, underexpressed in PAH, promotes vasodilation and antiproliferation?

Prostacyclin

What naturally produced potent vasodilator is decreased in patients with PAH?

Endothelin Receptor Antagonists (ERAs)

What drug class blocks vasoconstriction and proliferation in the Endothelin pathway, used for Group 1 PAH?

Bosentan/Ambrisentan

What specific Endothelin Receptor Antagonists (ERAs) are used for Group 1 PAH?

PDE-5 Inhibitors

What drug class causes pulmonary vasodilation via the NO-sGC-cGMP pathway, used for Group 1 PAH?

Sildenafil/Tadalafil

What specific PDE-5 Inhibitors are used for Group 1 PAH?

sGC (soluble guanylate cyclase) Stimulator

What drug class causes pulmonary vasodilation and antiproliferation in the NO-sGC-cGMP pathway, used for Group 1 PAH?

Riociguat

What specific sGC Stimulator is used for Group 1 PAH, and also for inoperable Chronic Thromboembolic Pulmonary Hypertension (CTEPH)?

Prostacyclin Analogs

What drug class causes pulmonary vasodilation and inhibits platelet aggregation in the Prostacyclin pathway, used for Group 1 PAH?

Epoprostenol/Treprostinil/Iloprost

What specific Prostacyclin Analogs are used for Group 1 PAH?

Prostacyclin Receptor Agonist

What drug class stimulates the IP receptor in the Prostacyclin pathway, used for Group 1 PAH?

Selexipag

What specific Prostacyclin Receptor Agonist is used for Group 1 PAH?

Plasma heart-type fatty acid-binding protein

What serum marker is released due to RV microinfarction and increases in acute pulmonary embolism (PE)?

Serum troponin

What cardiac biomarker increases due to RV microinfarction in PE, reflecting myocardial injury?

D-dimer

What coagulation factor breakdown product is used primarily to rule out PE in patients with a low probability of disease?

Brain natriuretic peptide (BNP) / NT-pro-brain natriuretic peptide (NT proBNP)

What peptides are released by myocardial stretch and can provide clues to disease severity in PAH?

Antithrombin

What molecule does unfractionated heparin bind directly to in order to prevent additional thrombus formation in PE treatment?

Unfractionated heparin

What anticoagulant binds directly to antithrombin and is the foundation for successful PE treatment?

Recombinant RTPA

What substance is used for systemic thrombolysis in patients with massive PE presenting with hypotension?

EGFR (Epidermal Growth Factor Receptor)

What driver mutation is the most common subtype identified in lung adenocarcinoma in East Asia?

ALK (Anaplastic Lymphoma Kinase)

What driver mutation/fusion is commonly tested for in NSCLC adenocarcinoma?

ROS-1

What driver mutation is commonly tested for in NSCLC adenocarcinoma?

PD-L1 (Programmed death-ligand 1)

What biomarker status determines candidacy for immunotherapy in NSCLC?

KRAS

What driver mutation is common in lung adenocarcinoma in USA/Europe?

HER2

What driver mutation is associated with lung adenocarcinoma?

MET

What driver mutation, sometimes presenting as exon 14 skipping, is associated with lung adenocarcinoma?

NRAS

What driver mutation is associated with lung adenocarcinoma?

NTRK1

What driver mutation is associated with lung adenocarcinoma?

PIK3CA

What driver mutation is associated with lung adenocarcinoma?

RET

What driver mutation is associated with lung adenocarcinoma?

AKT1

What driver mutation is associated with lung adenocarcinoma?

BRAF

What driver mutation is associated with lung adenocarcinoma?

MEK1

What driver mutation is associated with lung adenocarcinoma?

Osimertinib, Erlotinib, Gefitinib, Afatinib, Dacomitinib

What targeted therapies are options for patients with EGFR mutation in NSCLC?