RESPIRATORY Flashcards

Question

What are some signs and symptoms of emphysema

Answer 1

- **Breathing with pursed lips (pink puffers):** prevents alveolar collapse by increasing the positive end expiratory pressure - **Barrel shaped chest**: due to air trapping and hyperinflation - **Loss of cardiac dullness:** due to hyperexpansion of lungs from emphysema - **Dyspnoea** - **Cough:** could be productive - **Weight loss:** due to energy expenditure while breathing - Signs of CO2 retention - **Drowsy** - **Asterixis** - **Confusion**

Answer 2

Clinical Dx with Spirometry Test for confirmation Spirometry shows Obstruction: FEV1/FVC ration <0.7 **Global Initiative for COPD (GOLD)** FEV1% - compared to predicted value Stage 1 - > 80% (mild) Stage 2 – 50-79% (moderate) Stage 3 – 30-49% (severe) Stage 4 - < 30% (very severe) Multiple peak flow measurements to exclude asthma

Answer 3

CXR: DLCO (diffusing capacity of CO across lung): Low in COPD, Normal in Asthma Genetic testing for A1AT Def. ABG - may show T2RF ECG - heart function CXR - flattened diaphragm Bloods - anaemia

Answer 4

Hyperexpanded, enlarged lungs, air pockets (bullae) or a flattened diaphragm, and saber sheath trachea Barrel shaped chest

Answer 5

STOP SMOKING Annual flu vaccine and the pneumococcal vaccine (this is a one off vaccine)

Answer 6

GOLD A= 1 or less exacerbations per year not requiring admission with mild symptoms GOLD B= 1 or less exacerbations per year not requiring admission severe symptoms GOLD C= 2 exacerbations per year or 1 per year requiring admission n with mild symptoms GOLD D= 2 exacerbations per year or 1 per year requiring admission with severe symptoms

Answer 7

SABA:short-acting beta-agonist (e.g. salbutamol) SAMA: short-acting muscarinic antagonist (ipratropium) LABA: long-acting beta-agonist (e.g. salmeterol) LAMA: long-acting muscarinic antagonist (e.g. tiotropium)

Answer 8

Any short or long acting bronchodilator SABA/SAMA SABA - salbutamol SAMA - ipratropium

Answer 9

long acting beta agonist (LABA) plus a long acting muscarinic antagonist (LAMA) - LABA: long-acting beta-agonist (e.g. salmeterol) LAMA: long-acting muscarinic antagonist (e.g. tiotropium)

Answer 10

combined long acting beta agonist (LABA) plus an inhaled corticosteroid (ICS) LABA (i.e. salmeterol) ICS (i.e. budesonide) . If these don’t work then they can step up to a combination of a LABA, LAMA and ICS.

Answer 11

Nebulisers - eg Salbutamol - **Phosphodiesterase-4 inhibitors** eg Roflumilast - (blocks the breakdown of cyclic adenosine monophosphate) - **Long-term oxygen therapy (LTOT)**

Answer 12

b2-adrenoceptor agonists (beta agonists) They act on b2-adrenoceptors to cause smooth muscle relaxation and bronchodilation, by increasing cAMP . Also inhibit histamine release from lung mast cell

Answer 13

LABAs possess adequate lipophilic properties to remain in the airway tissues as a depot in close vicinity to the beta2-receptor, explaining their long duration of effect.

Answer 14

They are Anti-cholinergics *(long/short-acting muscarinic* ***ant****agonist)*– promotes cGMP degradation 🡪 smooth muscle relaxation Muscarinic antagonists work by blocking the action of acetylcholine, a neurotransmitter that causes smooth muscle contraction in the airways, leading to bronchospasm and narrowing of the airways.

Answer 15

Glucocorticoids reduce number of inflammatory cells in airways by: - **Suppress production** of chemotaxic mediators - Reduce **Adhesion molecule expression** - **inhibit inflammatory cell survival in the airway** ICS also suppress inflammatory gene expression in airway epithelial cells, eg via **IKB-ALPHA**

Answer 16

worsening of symptoms such as cough, shortness of breath, sputum production and wheeze. It is usually triggered by a viral or bacterial infection.

Answer 17

H.Influenzia S.Pneumoniae TREAT BOTH WITH AMOXICILLIN

Answer 18

CO2 will make blood more acidotic. This will show as a type 2 respiratory acidosis high CO2 and low oxygen with low pH If this chronic there will be some metabolic compensation by kidneys releasing more bicarbonate to try and neutralise pH

Answer 19

Prednisolone for 7-14 days Regular inhalers or home nebulisers Antibiotics if there is presence of infection

Answer 20

Nebulised bronchodilators (e.g. salbutamol 5mg/4h and ipratropium 500mcg/6h) Steroids (e.g. 200mg hydrocortisone or 30-40mg oral prednisolone) Antibiotics if evidence of infection Physiotherapy can help clear sputum

Answer 21

IV aminophylline Non-invasive ventilation Intubation and ventilation -Doxapram can be used as a respiratory stimulant if ventilation not appropriate

Answer 22

Too much oxygen in someone that is prone to retaining CO2 can depress their respiratory drive. Venturi masks are designed to deliver specific percentage concentrations of oxygen If retaining CO2 aim for oxygen saturations of 88-92% titrated by Venturi mask If not retaining CO2 and their bicarbonate is normal (meaning they do not normally retain CO2) then give oxygen to aim for oxygen saturations > 94%

Answer 23

An infectious disease caused by Mycobacteria characterised by caseating granulomas.

Answer 24

Caused by two species: Mycobacterium tuberculosis and Mycobacterium bovis. **Spread via airborne transmission**

Answer 25

Acid Fast Rod Bacilli Non motile + non spore forming Acid fast staining (Zeihl-Neelsen stain.) - **Stains red** Doesn't take up gram stain due to Mycolic acid capsule Resistant to phagocytic killing. Slow growing (15-20 hrs)

Answer 26

IVDU, homeless, immunosuppression alcoholic, close contact with infected patients - either as living with them or as healthcare worker

Answer 27

1.7Bn people have latent TB Top infectious killer in the world Affects immunocompromised more More common in South Asia (India, China, Pakistan) and Sub-Saharan Africa

Answer 28

Macrophages struggle to clear TB due to its waxy mycolic acid capsule. Instead of being broken down and cleared, A **focal caseating granuloma** typically forms in the lower lobe known as a **Ghon focus.** The Ghon focus can then spread to the **Hilar Lymph nodes** in the lungs, which together form a **ghon complex** These ghon complexes can under go **fibrosis and calcification**, leading to the appearance of **ranke complexes** on xray

Answer 29

- occurs after primary infection, immune system encapsulates sites of infection and stop the progression of the disease. - Patients remain asymptomatic and the bacteria remains dormant, resulting in negative sputum cultures but a positive Mantoux test. - These patients are not infectious. - However, if patients are immunocompromised, the disease can progress or reactivate at a later stage to become active TB.

Answer 30

Immunocompromised patients may develop secondary TB when latent TB reactivates - Patients are infectious. - Reactivation typically occurs in the lung apex where pO2 is highest, as mycobacteria are aerobic. bacteria can spread locally, to form caseating granulomata, or systemically (miliary TB).

Answer 31

Miliary TB - Where immune system cannot control the infection and it becomes disseminated Extrapulmonary TB - where TB infects other areas

Answer 32

Lymph nodes Pleura Central nervous system Pericardium Gastrointestinal system Genitourinary system Bones and joints Cutaneous TB affecting the skin

Answer 33

Systemic Sx: Fatigue Fever + night sweats + weight loss (characteristic of TB) Lymphadenopathy Wet Cough w/haemoptysis >3 weeks Chest pain Dyspnoea Erythema Nodosum *(swollen fat under the skin causing bumps and patches that look red or darker than surrounding skin.)* TB = Wet cough, Sarcoidosis - similar symptoms, but dry cough

Answer 34

CNS - Meningism Skin rash Cardiac - TB pericarditis Sx Bone - Join pain Spinal Pain (spinal TB) GU - Epididymitis, LUTS Abdo - Ascites

Answer 35

- Auscultation: often normal; **crackles may be present** - **Clubbing:** if long-standing as bacteria is contained within granuloma and causes no Sx

Answer 36

Latent Disease - Mantoux Test Interferon Gamma release assay

Answer 37

Take blood sample, mix it with TB antigens. In a person that has had previous contact with TB **the white blood cells have become sensitised to those antigens** and will release **interferon-gamma.** More sensitive than the Mantoux test - *unlikely to be positive from the BCG vaccine*

Answer 38

indicates possible previous vaccination, latent or active TB. 0.1 ml of 1:1,000 of tuberculin is injected S/C, site is inspected 48-72 hours later - This reaction is measured as a diameter of induration that occurs across the forearm - If positive, assess for active disease, greater than 5mm

Answer 39

Nucleic-Acid Amplification Test (NAAT): rapid diagnostic test conducted on sputum or urine Microbiology: send three deep cough sputum samples; analyse with Ziehl-Neelsen stain (will turn red) Chest Xray - Latent disease may show Ghon complex Reactivated TB may show patchy or nodular consolidation with cavitation (gas filled spaces in the lungs)

Answer 40

**Patchy Consolidation** Ghon Complex Granulomatous Lesions **Hilar Lymphadenopathy** - (enlargement) Pleural Effusion

Answer 41

Doesnt necessarily need Tx If risk of reactivation then: 6 months of isoniazid with pyridoxine or 3 months of isoniazid, pyridoxine and rifampicin

Answer 42

RIPE: Combination Abx for 6-12 months R – Rifampicin for 6 months I – Isoniazid for 6 months P – Pyrazinamide for first 2 months E – Ethambutol for first 2 months Pyra zina mide Etham but ol

Answer 43

BCG vaccine is offered to patients that are at higher risk of contact with TB: Neonates born in areas of the UK with high rates of TB Neonates with relatives from countries with a high rate of TB Neonates with a family history of TB Unvaccinated older children and young adults (< 35) who have close contact with TB Unvaccinated children or young adults that recently arrived from a country with a high rate of TB Healthcare workers

Answer 44

Infection of the lung parenchyma leading to inflammation of the lung tissue and fluid exudation (sputum) collecting in the alveoli. Can be seen as consolidation on a chest x-ray

Answer 45

Community acquired Hospital acquired if it occurs more than 48 hours after admission - *(can be further classified into Elderly, Ventilator-associated, Post-op)* Aspiration pneumonia if it occurs after inhaling foreign material

Answer 46

*Think - community, so more basic/simple bacteria* Streptococcus pneumonia (50%) H. Influenzae (20%) - Staphylococcus aureus - Klebsiella pneumonia Pseudomonas aeruginosa

Answer 47

Gram-positive cocci Alpha haemolytic Optochin sensitive

Answer 48

- Bronchopneumonia: infection can be throughout the lungs involving the bronchioles as well as the alveoli. - Atypical or interstitial pneumonia: infection is mainly just outside the alveoli in the interstitium. - Lobar pneumonia: infection causes complete consolidation of a whole lobe of the lung.

Answer 49

P.aeurginosa E.coli S.aureus Klebsiella These can cause CAPs as well, but just not as common

Answer 50

Streptococcus pneumoniae (pneumococcus) =Accounts for 80% of cases -Particularly associated with high fever, rapid onset and herpes labialis A vaccine to pneumococcus is available Haemophilus influenzae Particularly common in patients with COPD Klebsiella pneumonia is classically seen in alcoholics

Answer 51

Mycoplasma pneumoniae Staphylococcus aureus Legionella pneumophilia

Answer 52

Klebsiella

Answer 53

These microbes typically multiply and cross over from the airways into the lung tissue, creating an inflammatory response. Pneumonia refers to any inflammatory reaction affecting the alveoli it is most commonly secondary to infection The tissue fills with white blood cells as well as proteins, fluid, and red blood cells if a nearby capillary is damaged in the process.

Answer 54

pneumonia caused by an organism that cannot be cultured in the normal way or detected using a gram stain. They dont respond to penicillins Tx with Macrolides, Fluoroquinolones and tetracyclines.

Answer 55

Legionella pneumophila (Legionnaires’ disease) *infected water, causes Hyponatraemia* Mycoplasma pneumoniae. *erythema multiforme, neuro signs* Chlamydophila pneumoniae *school child* Coxiella burnetii AKA “Q fever” *farmer* Chlamydia psittaci. - *bird owner* TIP: You can remember the 5 causes of atypical pneumonia with the mnemonic: “Legions of psittaci MCQs” M – mycoplasma pneumoniae C – chlamydydophila pneumoniae Qs – Q fever (coxiella burnetii) **Legionella pneumophila should be suspected in multiple otherwise fit patients who have developed pneumonia after air condition exposure. Hyponatraemia and lymphopenia are features associated with Legionella pneumophila .**

Answer 56

Pneumocystis jiroveci (PCP) Occurs in immunocompromised Px AIDS defining illness Co-trimoxazole (combination of Trimethoprim and Sulphamethoxazole)

Answer 57

Extremes of age Preceding infection (viral) Immunosuppressed IVDU Smoking CO-Morbidities - DM, HIV Respiratory conditions - asthma, COPD, CF

Answer 58

Productive cough w/purulent sputum (rusty suggests S. pneumoniae) Fever - due to infection Pleuritic chest pain- pain worse on deep breathing (sandpaper on sandpaper) Dyspnoea Malaise May cause confusion in elderly Dry cough in atypical pneumonia **Classic symptoms - Fever Sweats Rigors**

Answer 59

Reduced breath sounds Bronchial Breathing w/ coarse crepitations Crackles and Wheeze Consolidation Dull Percussion Hypoxia Tachycardia Pyrexia Confusion Cyanosis

Answer 60

Heart Failure PE Pleural effusion Bronchiectasis Acute bronchitis Drug-induced pneumonitis Cancer TB Interstitial Lung Disease

Answer 61

commonly found in the lungs of healthy people, not a cause for disease. **source of opportunistic infection** in immunocompromised people, eg HIV/AIDs, cancer, autoimmune or inflammatory conditions, and chronic lung disease The risk of PCP increases when CD4-positive T-cell levels are less than 400 cells/μL

Answer 62

Co-trimoxazole (combination of Trimethoprim and Sulphamethoxazole)

Answer 63

CRB-65 out of hospital and CURB-65 in hospital. The only difference is that out of hospital you do not count urea. C – Confusion (new disorientation in person, place or time) U – Urea > 7 R – Respiratory rate ≥ 30 B – Blood pressure < 90 systolic or ≤ 60 diastolic. 65 – Age ≥ 65

Answer 64

Score 0/1: Consider treatment at home Score ≥ 2: Consider hospital admission Score ≥ 3: Consider intensive care assessment 0 = Low risk (<1% mortality) 1-2 = Intermediate risk (1-10% mortality) 3-4 = High Risk (>10% mortality) requires in-patient admission and IV antibiotics. Normally a CAP is treated with Co-Amox and Clarithromycin (to cover for atypicals). HAPs are treated with Tazocin, however this may change depending on trust guidelines.

Answer 65

1st Line: - **CXR:** consolidation caused by inflammatory exudate within alveoli and bronchioles - Atypical pneumonia causes interstitial inflammation instead, so CXR may be normal FBC - Raised WCC U&E - urea CRP - raised due to inflammation Sputum culture: allows assessment of organism and antibiotic sensitivities Urine culutres, for Legionella Urinary Antigen - Doesn’t respond well to Abx

Answer 66

Consolidation - region of normally compressible lung tissue - filled with liquid instead of air Happens as inflammatory fluid exudate accumilates in the alveoli and broncioles in acute inflammation MUST BE PRESENT IN ORDER TO DIAGNOSE PNEUMONIA - IT IS MORE RADIO OPAQUE - WHITER THAN NORMAL LUNG TISSUE

Answer 67

Oxygen Saturation 94-98% IV Fluids if dehydrated Appropriate Analgesia - Paracetamol/NSAIDs Mild severity – CURB65 0-1 (5 days) **Amoxicillin** Allergic? – clarithromycin or doxycycline

Answer 68

Moderate severity – CURB65 2 (5 days) Amoxicillin + clarithromycin Severe CURB65 3-5 (>5 days, 14-21 if S. Aureus) IV co-amoxiclav + clarithromycin *(btw Clarithromycin is a macrolide)*

Answer 69

S. Pneumoniae Use amoxicillin, or cefuroxime *(second gen Cephalosporin)*

Answer 70

Amoxicillin + clavulanic acid - (Co-Amoxiclav) Doxycycline

Answer 71

Flucloxacillin Cefuroxime MRSA = Vancomycin

Answer 72

Co-amoxiclav Cephalosporins P. Aeruginosa Piperacillin-Tazobactam (Tazocin)

Answer 73

Macrolides/Fluoroquinolones,tetracyclines.

Answer 74

Asthma is a chronic inflammatory airway disease characterised by intermittent airway obstruction and hyper-reactivity, + inflammed bronchioles and mucous hypersecretion

Answer 75

Eosinophilic (Allergic) (70%) - Extrinsic IgE mediated T1 Hypersensitivity Non-Eosinophilic (Non Allergic) (30%) - Intrinsic non IgE mediated.

Answer 76

- Asthma is a common disease with a prevalence of almost 10% in the US - Commonly starts in childhood between the ages 3-5 years and may either worsen or improve during adolescence - Peak prevalence between 5-15 years

Answer 77

- **History of atopy**: such as eczema and allergic rhinitis (IgE-mediated atopic conditions) - **Family history** - **Allergens**: such as tobacco smoke, pets, outdoor air pollution, weeds, grass, mould pollen and dust mites - **Viral upper respiratory tract infection** - **Other triggers**: cold weather and exercise, medications e.g. beta blockers and aspirin - **Occupational exposure (10-15%)**: isocyanates are the most common cause of occupational asthma (e.g. spray painting)., or flour form baking

Answer 78

Genes controlling cytokines IL-3 -4 -5 -9 -13 ADAM33 states that early childhood exposure to particular microorganisms protects against allergic diseases by contributing to the development of the immune system. *Generally asthma before 12 is more genetic after this it is more environmental*

Answer 79

**excessive reaction from Th2 cells against specific allergens**. Allergens from environmental triggers e.g. cigarette smoke, are picked up by dendritic cells and presented to Th2 cell Cyotkines are produced - (IL-3, IL-4, IL-5, IL-10, IL-13) This leads to the production of IgE antibodies which coat mast cells and stimulate them to release granules containing things e.g. histamines, leukotrienes and prostaglandins. It also results in the activation of eosinophils which promote an immune response by releasing more cytokines and leukotrienes.

Answer 80

Smooth muscle spasm around bronchioles Increased mucus secretion. Airway wall oedema There is also an increase in vascular permeability and recruitment of additional immune cells from the blood. Initially these inflammatory changes are completely reversible but longer term leads to thickening of the epithelial basement membrane, which permanently reduces the airway diameter. ---> **also in chronic asthma - see smooth muscle hypertrophy**

Answer 81

Beta blockers - (block adrenaline from binding) Blocking these receptors leads to smooth muscle contraction, narrowing airways and increasing blood pressure. Aspirin inhibits COX1/2 leads to increase USE of LPOX pathway. Produces leukotrienes (LTB4, 5, 6) These are proinflammatory

Answer 82

Chest tightness Episodic Dyspnoea/SOB Wheeze Dry Cough (typically but can be wet)

Answer 83

Diurnal PEFR variation Dyspnoea and Expiratory Polyphonic wheeze Samter’s Triad: Nasal polyps, Aspirin sensitivity, Asthma Atopic Triad: Atopic Rhinitis, Asthma, Eczema Hyper resonant Percussion

Answer 84

Curschmann spirals: Mucus plugs that look like casts of the small bronchi Charcot-Leyden crystals: From break down of eosiophils.

Answer 85

- **Fractional exhaled nitric oxide (FeNO):** >40 ppb is positive in adults - **Spirometry:** FEV1/FVC <70% suggests obstruction if positive for obstruction, then carry out Bronchodilator reversibility (BDR) - **Peak flow rate (PEFR)**: measured multiple times a day over 2-4 weeks. Variability of >20% is diagnostic - **Airway hyperreactivity testing**: a histamine or methacholine direct bronchial challenge is performed if the investigations above are inconclusive - **Allergy testing**: immunoassay for allergen-specific IgE or skin-prick testing

Answer 86

Morning lower, evening higher. Diurnal variation

Answer 87

Looks to see if bronchodilators can **improve FEV1** In asthma, use of bronchodilators should lead to improvement of FEV1 by ≥12% and increase ≥200ml in volume post-bronchodilator An improvement of less than 12% is indicative of COPD

Answer 88

1st line - SAB2A, eg **Salbutamol**, PRN 2nd line - **SABA** ; e.g. salbutamol + **low dose ICS**e.g. beclomethasone.

Answer 89

3a. Before adding more drugs assess inhaler technique and compliance 3b. SABA + ICS + Leukotriene Receptor Antagonist (LTRA = montelukast) 4th - SABA + ICS + LABA (Salmeterol) +/- LTRA

Answer 90

SABA and MART (maintenance and reliever therapy); this is combined **fast-acting LABA and ICS** for symptomatic relief and maintenance in a single inhaler. Can increase ICS dose!!! ***ICS increase transcription of B2-receptor gene = more expression of it on cell surface receptors ---> ICS help Beta Agonists to work***

Answer 91

Fast respiratory rate Symmetrical wheeze Tight sounding chest with reduced air entry

Answer 92

Almost as tight as Abishek's chest at miniNAMs when he wore a muscle fit top a good 3 sizes to small *#pecs #gainz #gymLAD*

Answer 93

PEFR ABG: patients will initially have respiratory alkalosis. Abnormal or high PCO2 is concerning as it implies the patient is tiring

Answer 94

Moderate - PEFR 50-75% of predicted **Acute Severe** - PEFR 33-50%, Resp rate >25, HR >110, can't complete sentences Lifethreatening PEFR <33%, Sats <92%, Becoming tired, No wheeze,

Answer 95

OSHITME: **O**2 if oxygen sats are low **S**aba (Salbutamol nebulised) **H**ydrocortisone IV **I**pratropium bromide added to nebulizer *remember, ipratropium is a SAMA* **T**heophyline IV - Bronchodilator **M**gSO4 - Magnesium sulphate, **E**scalate

Answer 96

- No night-time symptoms ● Inhaler used no more than three times per week ● No breathing difficulties, cough or wheeze on most days ● Able to exercise without symptoms ● Normal lung function test

Answer 97

Cystic fibrosis (CF) is an inherited, autosomal recessive, multi-system disease affecting mucus glands. Respiratory problems most prominent, as well as pancreatic insufficiency.

Answer 98

Cystic fibrosis is the most common inherited condition in the Caucasian population, affecting 1/2500 births, whilst 1/25 of the population are carriers **AUTOSOMMAL RECESSIVE**

Answer 99

cystic fibrosis transmembrane conductance regulatory gene on **chromosome 7** **Δ-F508** is the most common mutation, where the codon for **phenylalanine** (F) in the CFTR gene is deleted, resulting in proteolytic degradation.

Answer 100

cystic fibrosis transmembrane conductance regulatory gene codes for a channel protein that pumps chloride ions into various secretions, those chloride ions help draw water into the secretions, which ends up thinning them out.

Answer 101

Misfolded CFTR protein **can't migrate from the endoplasmic reticulum to the cell membrane**, meaning there’s a lack of CFTR protein on the epithelial surface This means that it can’t pump chloride ions out, which means water doesn’t get drawn in, and the secretions are left overly thick.

Answer 102

CFTR mutation leads to **thick mucus secretions.** This causes impaired **mucociliary clearance** as the mucus is extra thick. This leads to **stagnation of mucus that contains pathogens which leads to increased infection risk.** The thicker mucus causes difficulty breathing Trapping of mucosal pathogens can cause a inflammatory reaction which leads to an increased risk of bronchiectasis

Answer 103

- Low volume thick airway secretions that reduce airway clearance, resulting in bacterial colonisation and susceptibility to airway infections, especially with: S. Aureus – flucloxacillin H. influenzae – amoxicillin Pseudomonas aeruginosa - ciprofloxacin

Answer 104

Can lead to Meconium Ileus: Stool becomes too thick to pass through the bowel leading to bowel obstruction. - **Surgical emergency** Failure to thrive

Answer 105

Thick secretions from the pancreas can lead to pancreatic duct obstruction. Pancreatic insufficiency and malabsorption of foods. Pancreas enzymes can build up in pancreas and damage it, leading to pancreatitis and fibrosis Bowel obstructions Can also lead to endocrine dysfunction - ***insulin dependant diabetes***

Answer 106

Thicker biliary secretions leads to an increased risk of biliary obstruction. Could lead to liver cirrhosis CFTR mutation can cause atrophy of the vas deferens leading to infertility

Answer 107

Chronic cough w/Thick sputum production Recurrent respiratory tract infections Loose, greasy stools (steatorrhoea) due to a lack of fat digesting lipase enzymes Abdominal pain and bloating Most common cause of Meconium Ileus in infants Parents may report the **child tastes particularly salty when they kiss them, due to the concentrated salt in the sweat** Poor weight and height gain (failure to thrive)

Answer 108

- Low weight - Nasal polyps - Finger clubbing - Crackles and wheezes - Abdominal distension

Answer 109

- It is found during the **heel-prick/Guthrie test** which screens for CF in babies by looking for serum immunoreactivity trypsinogen Immunoreactivity trypsinogen is an pancreatic enzyme that is released into the blood when the pancreas is damaged.

Answer 110

Other than **heel-prick/Guthrie test** *(looks for serum IRT)* **Sweat test:** gold standard test; induce sweating *(by placing electrodes on skin)* followed by analysis of sweat to check Cl- concentration A result of **> 60 mmol/L (sweat chloride) is positive** and requires referral to a cystic fibrosis specialist (normal value < 40 mmol/Ll) Genetic testing: Genetic testing for CFTR gene mutation can be performed during pregnancy, via amniocentesis - **Lung function tests:** obstructive pattern seen; and allows monitoring of treatment - **Sputum sample:** microbiological investigation during exacerbations Faecal elastase: test for pancreatic insufficiency

Answer 111

Patients with cystic fibrosis struggle to clear the secretions in their airways. This creates a perfect environment with plenty of moisture and oxygen for colonies of bacteria to live and replicate.

Answer 112

Exercise, no smoking! - **Airway clearance techniques:** minimum 2 times per day. Chest physiotherapy and postural drainage. - **Bronchodilator:** inhaled salbutamol for exacerbations rhDNase e.g. dornase alfa - an enzyme that breaks down material in secretions, making them less viscous - Nebulised hypertonic saline

Answer 113

- CREON tablets helps to digest fats in patients with pancreatic insufficiency (missing lipase) - fat-soluble vitamin supplements (A, D, E, K); - High calorie diet to make up for malabsorption and calories needed for respiratory effort

Answer 114

personalised genetic treatments - targets specific mutation - **F508 del,** Lumacaftor is a CFTR corrector, acts as a **Pharmalogical chaperone** to bring the F508 del CFTR protein to the cell membrane Ivacaftor, a CFTR potentiator binds to channel directly, increases the chances of it opening. primarily the targets G551D mutation. Can be used in combination with one another

Answer 115

Prognosis: Median survival is now ~ 41yrs in the UK, although a baby born today would expect to live longer. - 90% of patients with CF develop **pancreatic insufficiency** - 50% of adults with CF develop **cystic fibrosis-related diabetes** and require treatment with insulin - 30% of adults with CF develop **liver disease** - Most males are infertile due to **absent vas deferens** Lung disease is the most common cause of death in patients with cystic fibrosis

Answer 116

Bronchiectasis is the permanent dilation of bronchi due to the destruction of the elastic and muscular components of the bronchial wall. It's an **obstructive airway disease**

Answer 117

More common in women than men May develop after lung infections Pathological end point of many disease Presents at any age but incidence increase with age

Answer 118

**Primary ciliary dyskinesia**: *cilia don’t move normally which leaves mucus stuck in the airways. Bacteria trapped in mucus can lead to pneumonia and inflammation* **Cystic fibrosis:** mucus is sticky and therefore hard to sweep, can accumulate Obstruction Foreign body e.g. peanut Post TB stenosis Tumour Thick mucus AIDs, or immunoglobulin deficiency Post-infections

Answer 119

Pseudomonas aeruginosa TB Measles Pneumonia Pertussis

Answer 120

Chronic inflammation 🡪 damage to airways 🡪 elastin destruction (lung dilates) and collagen deposition 🡪 stiff, large airways that are plugged with mucous, and and fibrosis of lung parenchyma (non-reversible)

Answer 121

Chronic cough with expectoration of large quantities of **foul-smelling sputum**, sometimes it is flecked with blood (haemoptysis) Haemoptysis SOB/Dyspnoea Wheeze Chest pain

Answer 122

History of chronic productive cough and recurrent chest infections (pneumonia) Finger clubbing – especially with cystic fibrosis, due to long term hypoxia Crackles over affected areas, usually at base of lungs **Normally affects baseline of lungs**

Answer 123

**Lung function test** – shows obstructive pattern, less lung capacity and less FEV1 - FEV1/FVC = <0.7 **Chest xray** - Dilated bronchi with thickened walls – tramline and ring shadows, Multiple cysts containing fluid showing up as cystic shadows **High resolution CT - gold standard** - Thickened, dilated bronchi with cysts at end of bronchioles, Airways larger than associated blood vessels - ***Signet ring sign*** Sputum culture – to see colonisation status and exclude non-tuberculous mycobacterial disease

Answer 124

**Haemophilus influenzae - MOST COMMON** Pseudomonas aeruginosa Strep. Pneumoniae S. Aureus

Answer 125

- **Antibiotics:** for recurrent infections - **Postural drainage:** to remove excess mucus - **Chest physio** - **Mucolytics** - carbocisteine, to break up mucus - **Bronchodilators e.g. nebulised salbutamol:** useful for asthma or COPD sufferers - **Anti-inflammatory agents e.g. long term azithromycin** (macrolide) can reduce exacerbation frequency - **Surgery:** to remove physical obstruction e.g. foreign object

Answer 126

Pseudomonas aeruginosa – oral ciprofloxacin H. influenzae – oral amoxicillin, co-amoxiclav or doxycycline Some multi-resistant species needs IV cephalosporin S. Aureus – flucloxacillin

Answer 127

Airways that are full of mucous become hypoxic. Widespread hypoxia leads to widespread vasoconstriction of the pulmonary blood vessels - leading to pulmonary hypertension Pulmonary hypertension means that the right side of the heart will have to beat harder to get blood around pulmonary circulation - RV hypertrophy Known as Cor pulmonale

Answer 128

Lungs covered by thin serious layer – visceral pleural Reflected on chest wall and pericardium as parietal pleura Pleural cavity is the thin space in-between this, and the fluid here is drained into lymphatic system Allows movement of lung against chest wall Coupling system between lungs and chest Clearing fluid from pulmonary interstitium

Answer 129

Whether the fluid is: Exudative - high protein count (>30g/dL) Transudative - lower protein count (<30g/dL) *(transparent, less protein)* Lymphatic pleural effusion (chylothorax)

Answer 130

caused by inflammation, infection and malignancy. The inflammation results in protein e.g. LDH leaking out of the tissues and into the pleural space. - eg increased vascular permeability

Answer 131

Transudate effusion: caused by increased hydrostatic pressure *(pulmonary hypertension)* or low oncotic pressure. This causes fluid to move from the capillaries into the pleural sac.

Answer 132

- **Lymphatic effusion (chylothorax)** - The thoracic duct is disrupted so drainage is not efficient - lymphatic fluid accumulates in the pleural space. Eg damage to duct during surgery or due to tumours

Answer 133

Congestive heart failure: heart can't effectively pump to rest of body and so blood is backed up and increases pressure Hypoalbuminaemia: may be due to liver failure (lack of protein production), nephrotic syndrome (loss of protein in the urine) and malabsorption. Hypothyroidism Peritoneal dialysis: an acute massive effusion may occur within 48 hours of initiating dialysis due to dialysis fluid crossing the diaphragm Meig’s syndrome: this is the triad of a benign ovarian tumour with ascites and a pleural effusion

Answer 134

Malignancy: tumour infiltration of pleural capillaries and cytokines increase capillary permeability Infection: e.g. pneumonia (acute lung injury increases vascular permeability - **Trauma** - **Pulmonary embolism** - **Pancreatitis** - **Autoimmune and connective tissue disorders:** key causes include SLE, rheumatoid pleurisy and eosinophilic granulomatosis with polyangiitis Exudative pleural effusions can also be caused by Dresslers Syndrome

Answer 135

Reduced Chest expansion on affected side Reduce breath sounds on affected side Dull percussion (increased fluid) Decreased tactile or vocal fremitus (reduced vibration of chest wall when speaking) Pleural friction rub/bronchial breathing Tracheal Deviation

Answer 136

Dyspnoea Pleuritic chest pain (often exudate due to pleural inflammation/irritation) Cough Sx of underlying pathology: Peripheral oedema - Heart failure Ascites - Liver failure Cough & fever - RTI

Answer 137

CXR: Transudates usually bilateral (TB) Exudates usually unilateral (EU) Blunting of costophrenic angle Thoracocentesis - aspirate some pleural fluid, just above the rib to avoid NV bundle Transudate – clear Exudate – cloudy Lymphatic – looks like milk

Answer 138

- Exudates have a protein level >30 g/L whilst transudates have a protein level <30 g/L - If the protein level is 25-35 g/L, Light’s criteria should be applied Light’s criteria is used to distinguish between transudate and exudate effusions

Answer 139

The criteria state that an **exudate is likely** if one or more of the following criteria are met: - **Pleural fluid protein divided by serum protein is > 0.5** - **Pleural fluid LDH divided by serum LDH is > 0.6** - **Pleural fluid LDH > 2/3 the upper limits of normal serum LDH**

Answer 140

Conservative Mx: Small effusions can be treated by Tx of underlying cause (often Transudative) Small ones can you diuretics eg furosemide Thoracocentesis - needle drainage Chest Drain - remove fluid (often Exudative) Pleurodesis - injection that adheres visceral and parietal pleural together so excess fluids cannot end up between them

Answer 141

Vacuum in the pleural space created by 1. **Tension of the diaphragm and Chest wall**, to pull the thoracic cavity outwards 2. **Elastic recoil of the lungs** which pull the lungs inwards - This pull creates a vacuum in the pleural space Pleural space has a pressure of **-5 centimetres of water relative to the pressure of 0 centimetres of water in both the thoracic cavity and the lungs**

Answer 142

Air in pleural space 🡪 partial or complete collapse of lung

Answer 143

Breach in the pleura (trauma/CT disorders) Leads to air entry into pleural cavity Elastic recoil of the lung causes it to deflate eg. Subpleural bullae burst eg. Abnormal connection between pleural space and airways (due to trauma)

Answer 144

- Typical presentation: a middle-aged patient with COPD presents with sudden onset breathlessness and chest pain - There are signs of underlying disease - Due to ruptured bleb or bullae (fluid-filled sac) **secondary to lung disease** Causes - Underlying lung disease e.g. COPD, asthma, lung cancer - TB - Pneumocystic jirovecii

Answer 145

Typical presentation: a ventilated patient suddenly becomes breathless and haemodynamically unstable. This is an emergency Air is forced into the thoracic cavity without means of escape **it is a one way valve.**

Answer 146

- Trachea will deviate away, due to the increased pressure “pushing” it over to the other side - **Hyper-resonant percussion** because there is more air inside the chest - **Decreased tactile and vocal fremitus**

Answer 147

Air is drawn in to the pleural space with each breath and cannot escape. This is dangerous as it creates pressure in the thorax and will push the mediastinum across kinking the big vessels causing cardiorespiratory arrest, and put pressure on the heart

Answer 148

Sudden onset pleuritic chest pain Sudden-onset dyspnoea Sweating As pneumothorax enlarges, patient becomes more breathless and may develop pallor and tachycardia

Answer 149

Reduced breath sounds on affected side Hyper resonant percussion (increased air) Reduced chest expansion Trachea Deviation away Tachycardia/Tachypnoea Hypotension - in Tension Pneumothorax

Answer 150

- **If tension pneumothorax is suspected, don't wait for investigations!** - **Erect CXR:** first-line investigation - Demonstrates a visible visceral pleural edge with no lung markings peripheral to this line - In a tension pneumothorax, there is mediastinal shift and tracheal deviation contralaterally CT chest: gold-standard due to accurate pneumothorax size estimation, - rarely done though

Answer 151

- **Emergency!** - **Immediate needle decompression** with the insertion of a large-bore (14–16G) needle into the **second intercostal space in the midclavicular line** - **High-flow oxygen** - **Chest drain** after **aspiration** - **Repeat CXR**

Answer 152

**Rim of air <2cm** and no SOB - can heal on its own **If SOB and/or a >2cm rim of air** - **Aspiration** is usually performed at the 2nd intercostal space midclavicular line on the affected side - **Chest drain** is inserted at the 5th intercostal space mid-axillary line on the affected side within the 'triangle of safety' - **High-flow oxygen** if needed - **Repeat CXR**

Answer 153

An infected Pleural Effusion (pus collects in pleural cavity)

Answer 154

PHAL: Pus, High LDH. Acidic pH (pH < 7.2), Low glucose

Answer 155

When a patient has an improving pneumonia but new or ongoing fever. Pleural aspiration will show pus, acidic pH <7.2 ,low glucose and high LDH.

Answer 156

chest drain to remove the pus and antibiotics

Answer 157

Umbrella term to describe conditions that affect the lung parenchyma (the lung tissue) causing inflammation and fibrosis.

Answer 158

Pulmonary Fibrosis Pneumonoconiosis Sarcoidosis *(Granulomatous)* Hypersensitivity Pneumonitis

Answer 159

Generally the damage is irreversible and so supportive Tx to prevent progression. Mx options: Remove or treat the underlying cause Home oxygen where they are hypoxic at rest Stop smoking Physiotherapy and pulmonary rehabilitation Pneumococcal and flu vaccine

Answer 160

Idiopathic PF - **most common Interstitial lung disease** Drug induced PF Secondary PF

Answer 161

It describes the interstitial fibrosis of the lung parenchyma and has a number of causes. The most common cause is idiopathic

Answer 162

Amiodarone - *anti arrhythmia* Cyclophosphamide - *a type of chemo drug* Methotrexate - *DMARD* Nitrofurantoin - *antbx, often used for UTIs*

Answer 163

Alpha-1 antitrypsin Rheumatoid arthritis SLE Systemic sclerosis Asbestos

Answer 164

Can causes asbestosis - leads to Lung fibrosis Pleural thickening and pleural plaques Adenocarcinoma Mesothelioma Asbestos is fibrogenic, meaning it causes lung fibrosis. It is also oncogenic, meaning it causes cancer

Answer 165

Type 1 - helps make up air barrier, long and thin Type 2 - cuboidal, secrete surfactant In-between these there is interstitial tissue, that contains macrophages and fibroblasts

Answer 166

- **Advanced age:** the mean age at diagnosis is 60-70 years of age - **Male gender**: twice as common in men - **Smoking** - **Family history** - **Dust exposure:** raising birds, metal, wood

Answer 167

**type II pneumocytes over-proliferate** during the repair process = too many myofibroblasts, that don't undergo apoptosis so collagen accumulates **interstitial layer thickens** between the alveoli and the capillary ==> excess collagen also leads to the loss of alveoli creating cysts, surrounded by **thick walls in a pattern called “honeycombing”.**

Answer 168

**PSHCT** Pneumonoconiosis Sarcoidosis + Silicosis Hypersensitivity Pneumonitis Cystic Fibrosis Tuberculosis

Answer 169

Idiopathic Pulmonary Fibrosis Drug Induced PF Connective tissue Disorders (SLE)

Answer 170

Progressive Dyspnoea Dry cough (no sputum) Malaise

Answer 171

**IPF mainly affects the lower lobes** - Signs - **Bibasal fine end-inspiratory crackles,** predominantly in lower zones - **Clubbing** - **Cyanosis**

Answer 172

Spirometry: Restriction CXR - non specific but can be used to exclude other DDx Gold Standard: High CT thorax to confirm diagnosis = Ground glass & **Honeycombing** Lung biopsy if still unclear

Answer 173

FVC reduced so FEV1/FVC ratio >0.8 predicted value e.g. pulmonary fibrosis Decreased total lung capacity FEV1 = volume of air that can be forcefully expired in 1 second FVC = total volume of air that can be forcibly exhaled after maximum inhalation

Answer 174

Antinuclear antibodies (ANA) and rheumatoid factor (RF): ANA is positive in 30% and RF is positive in 10-20%, but this does not confirm that the fibrosis is secondary to connective tissue disease

Answer 175

Remove/treat underlying cause Home oxygen when hypoxic at rest Stop smoking Physiotherapy Pneumococcal and flu vaccine Advanced care planning and palliative care where appropriate Prednisolone for exacerbations end stage/advnaced - LUNG TRANSPLANT

Answer 176

Pirfenidone is an antifibrotic and anti-inflammatory **Nintedanib** is a monoclonal antibody inhibits **tyrosine kinase - involved in cell division/proliferation**

Answer 177

Sarcoidosis is a granulomatous inflammatory multi-systemic disease in which any organ can be affected, although the lungs are the predominantly affected organ.

Answer 178

- **Afro-Caribbean and Scandinavian ethnicity** - **Young adults**: commonly presents at 20-40 years of age - **Female gender** - **Family history** of it Associated with HLA-DRB1 and DQB1 alleles. Previous infections of **Mycobacterium Tuberculosis**,

Answer 179

A granuloma is an aggregation of macrophages that forms in response to chronic inflammation. This occurs when the immune system attempts to isolate foreign substances that it is otherwise unable to eliminate. Histiocytes (specifically macrophages) are the cells that define a granuloma. The macrophages in granulomas are often referred to as "epithelioid". This term refers to the vague resemblance of these macrophages to epithelial cells

Answer 180

A T-cell-mediated immune response to an unknown antigen attracts other immune cells, causes the **formation of granulomas** The granulomas in sarcoidosis are non-caseating which means that there is no tissue necrosis at the centre of the granuloma. The macrophages begin to release local mediators that result in inflammation, and can fuse together forming a single multi-nucleated cell called a **Langhans cell**, It can involve nearly every organ, but they most often involves hilar lymph nodes which are lymph nodes that are near the point where the bronchi meets the lung.

Answer 181

**Dry cough** - note TB has phlegm production!!** Dyspnoea Mediastinal lymphadenopathy Constitutional Sx: Weight loss, Fatigue and fever

Answer 182

Common: (PESH) Peripheral lymphadenopathy Eye – anterior uveitis Skin –Erythema nodosa, skin papule Hepatosplenomegaly Less common: Bone Heart – arrhythmias CNS Kidney

Answer 183

specific presentation of sarcoidosis. It is characteristic by a triad of: Erythema nodosum Bilateral hilar lymphadenopathy Polyarthralgia (joint pain in multiple joints) Heerfordt’s syndrome: causes facial nerve palsy, fever, uveitis and parotitis - see picture, also asossicated with sarcoidosis

Answer 184

- **Routine bloods:** inflammatory markers may be raised, can screen for other organ involvement - **Serum calcium:** hypercalcaemia (10%) (due to macrophages in non-caseating granulomas activating vitamin D) - **Angiotensin-converting enzyme (ACE):** elevated (but with poor sensitivity (60%) and specificity (70%)) - **CXR:** first-line imaging; may show hilar lymphadenopathy or bilateral infiltrates CT chest: shows hilar lymphadenopathy

Answer 185

Tissue biopsy: usually done by doing bronchoscopy with ultrasound guided biopsy of mediastinal lymph nodes. Shows characteristic **non-caseating granulomas** with epithelioid cells, as well as intracytoplasmic star-shaped or laminar calcified inclusions - **(asteroid bodies, Schaumann bodies)**

Answer 186

Tuberculosis (caseating) Lymphoma Hypersensitivity pneumonitis HIV Toxoplasmosis Histoplasmosis

Answer 187

ASx/Mild: No Tx is 1st Line: 85% of stage 1 resolve spontaneously 50% of stage 2 resolve spontaneously When Tx required: 1st Line: **Oral Prednisolone (+bisphosphonates to protect against OP)** 2nd Line: **Methotrexate/Azathioprine** Complicaiton - Pulmonary fibrosis, Pulmonary HTN, Cor Pulmonale

Answer 188

Type 3 hypersensitivity reaction to an **environmental allergen that causes parenchymal inflammation and destruction** in people sensitive to that allergen

Answer 189

Bird-fanciers lung is a reaction to proteins in bird droppings Farmers lung is a reaction to mouldy spores in hay (micropolyspora faeni) Mushroom workers’ lung is a reaction to specific mushroom antigens Malt workers lung is a reaction to mould on barley

Answer 190

**TYPE 3 HYPERSENSITIVITY REACTION** Allergen is inhaled and picked up by alveolar macrophages which take it to the nearest lymph node., and antibodies against allergen (IgG) form autoantigens These complexes get deposited in the lung tissues and alveoli. Complement is activated which causes acute inflammation of the lung tissue causing pneumonitis Chronically this can lead to pulmonary fibrosis Can be acute or chronic

Answer 191

Sx develop <12 hrs after a good amount of exposure and resolve after 48 hrs Fever Rigors Headache Myalgia Shortness of breath Cough Chest tightness

Answer 192

Sx develop over prolonged exposure (often lower levels for long time) Progressive cough Progressive Dyspnoea Fatigue Weight loss Fibrosis is evident

Answer 193

. Bronchoalveolar lavage involves collecting cells from the airways during bronchoscopy by washing the airways with fluid then collecting that fluid for testing

Answer 194

**Bronchoscopy with Bronchoalveolar lavage:** Biopsy shows raised T Lymphocytes and mast cells in bronchi, alveoli and **non-caseating granulomas** **High Res CT - Ground glass (upper lobes)** and centrilobular nodules. Reticular opacity **Spirometry/ Pulmonary function test - Restrictive** Serology - Specific Igs

Answer 195

Remove the allergen Steroids (glucocorticoids) can help with symptoms Give oxygen if necessary Long term: wear a facemask or +ve pressure helmet. Long-term steroids *Compensation (UK Industrial Injuries Act) may be payable.*

Answer 196

Interstitial lung fibrosis that occurs secondary to inhalation of occupational triggering antigens that cause an inflammatory reaction. *Penumo - con - oh - sis*

Answer 197

Silicosis - Inhalation of silicon dioxide Asbestosis - Inhalation of asbestos

Answer 198

Particles (Dust, asbestos, silica) are inhaled, they reach the terminal bronchioles and are ingested by interstitial and alveolar macrophages. Dust particles are carried by macrophages and expelled as mucus. In chronic exposure (occupation), this process is no longer functional and macrophages accumulate in alveoli, Resulting in immune system activation and lung tissue damage. Fibroblasts then arrive and deposit excess collagen to cause lung fibrosis

Answer 199

Pulmonary hypertension is increased resistance and pressure of blood in the pulmonary arteries. A mean pulmonary arterial pressure that is greater than 25 mmHg.

Answer 200

SLE, infections e.g. HIV, thyroid disorders, and inherited genetic mutations. Endothelial damage - release endothelin-1, serotonin, and thromboxane. These make the pulmonary arterioles constrict and cause hypertrophy of the smooth muscle surrounding them. The damaged endothelial cells also produce less nitric oxide and prostacyclin (which, make the pulmonary arterioles dilate and inhibit smooth muscle hypertrophy)

Answer 201

-usually due to myocardial infarction or systemic hypertension Pulmonary blood vessels are normal and undamaged, but the left side of the heart is unable to pump efficiently. This causes a backup of blood in the pulmonary veins and capillary beds, which can increase the pressure in the pulmonary artery.

Answer 202

Area in the lung is diseased and is unable to deliver oxygen to the blood, so pulmonary arteries in this area vasoconstrict Widespread disease = widespread vasoconstriction of pulmonary arterioles, = **increases pulmonary vascular resistance** This makes it **hard for the right ventricle to pump out blood.** To make the same amount of blood flow through the pulmonary arterioles, the **right side of the heart has to generate increased pressure and this results in pulmonary hypertension**.

Answer 203

Increase in Jugular venous pressure Fluid build up in liver = hepatomegaly Fluid Buildup in legs - Leg Oedema Systemic Hypertension ***Think - fluid build up in RS of heart, and before that in the systemic circulation - hence these symptoms!!***

Answer 204

Progressive breathlessness Exertional Dizziness/syncope Fatigue Sx of underlying pathology

Answer 205

Loud S2 heart sound - pulmonary side Raised JVP Pulmonary/Tricuspid regurgitation Tachycardia Hepatomegaly Peripheral oedema Right parasternal heave - due to RV hypertrophy Signs of underlying pathology

Answer 206

1st Line: ECHO: RV function, enlargement and Pressures Gold Standard (diagnostic) **Right heart Catheter** CXR - RVH, Enlarged proximal PA ECG: Right Ventricular Hypertrophy Right axis deviation Right bundle branch block

Answer 207

Primary P HTN: Phosphodiesterase-5 inhibitors (SILDENAFIL) IV prostanoids (Epoprostenol) Endothelin receptor antagonists (Macitentan) Secondary P HTN: Tx underlying cause Supportive Tx for complications: Respiratory failure, HF, arrythmias

Answer 208

Right heart failure: due to increasing pulmonary pressures (Can lead to biventricular failure) Pericardial effusion and tamponade Hepatic congestion: due to worsening right heart failure

Answer 209

Ciliated Cells - to help waft pathogens out of lungs Goblet Cells - to secrete mucin **Club Cells** - help protect bronchiolar epithelium **Basal cells** - immature cells that can differentiate into other cells Neuroendocrine cells - that can secrete hormones into the blood

Answer 210

CLASSIFY BY HISTOLOGY Small Cell lung cancer (20%) Non- Small cell lung cancer (80%)

Answer 211

- Adenocarcinoma (40%) - Squamous cell Carcinoma (20%) - Large Cell Carcinoma (10%) - Carcinoid/other (10%)

Answer 212

- **Increasing age**: adenocarcinomas are an exception, often occurring in younger patients. - **Smoking**: tobacco smoking or environmental smoke exposure. - **Other environmental exposure**: radon, asbestos, arsenic, chromium, air pollution and radiation. - **Family history:** some gene mutations are known to be associated with an increased risk of lung cancer development.

Answer 213

Secondary are More common than Primary: Lungs oxygenate 100% of blood and therefore they come into contact with all blood. This increases the risk of metastasis.

Answer 214

Mets from: Breast Kidney Bowel Bladder

Answer 215

- 15% of lung cancer cases - Location: **central lesion near the main bronchus, and commonly large** - Derived from **neuroendocrine Kulchitsky cells** Associated with Smoking

Answer 216

- Contain neurosecretory granules that can release neuroendocrine hormones. This makes SCLC responsible for multiple paraneoplastic syndromes: - SIADH → **hyponatraemia** - Ectopic ACTH → **Cushing's syndrome** - Lambert-Eaton myasthenic syndrome - releases antibodies that destroy neurons - PTH-like substance 🡪 hypercalcaemia - HCG or related hormones 🡪 gynecomastia (enlargement of male breast tissue) - Rapid growth and patients usually present in an advanced stage, metastases early, often to mediastinum or distally.

Answer 217

Commonly associated with Asbestos MOST COMMON non small cell lung cancer Affects the peripheral lung Arises from **Mucous secreting glandular epithelium** METs Common

Answer 218

Most common cell type in non-smokers, not related to smoking Paraneoplastic syndromes: - Hypertrophic pulmonary osteoarthropathy: causes inflammation of the bones and joints in the wrists and ankles, and clubbing of the fingers and toes - Gynaecomastia - swelling of breast tissue in males, erectile dysfunction

Answer 219

- Location: central lesion - Squamous, or square shaped, cells that produce keratin Most strongly associated with cigarette smoke Number of cases: 20% NSCLC

Answer 220

Hypertrophic pulmonary osteoarthropathy: causes inflammation of the bones and joints in the wrists and ankles, and clubbing of the fingers and toes PTHrP → hypercalcaemia - - Fatigue, Bone pain, Headaches, Nausea and vomiting, Constipation., polyuria Most present as obstructive lesion leading to infection

Answer 221

Number of cases: <10% NSCLC Location: Peripheral lesion commonly but can be found anywhere in lungs Originates: Lacks both glandular and squamous differentiation Paraneoplastic Syndromes - Ectopic B-hCG production - Hypertrophic pulmonary osteoarthropathy:

Answer 222

- Rare - From mature neuroendocrine cells - Paraneoplastic syndrome: Carcinoid syndrome which causes the secretion of hormones, particularly serotonin, which leads to increased peristalsis and diarrhoea, and bronchoconstriction causing asthma.

Answer 223

A tumour in the lung apex/ upper lung that commonly metastasises to the necks sympathetic plexus and can press on the sympathetic ganglion Can be caused by both squamous cell and adenocarcinomas

Answer 224

HORNERS Syndrome characterised by: Ptosis - Droopy eyelids Myosis - Excessive Pupil Constriction Anhidrosis - Lack of sweating

Answer 225

Cough w/haemoptysis Shortness of breath Pleuritic Chest pain Constitutional Sx of Cancer: Fever Night sweats Weight loss Loss of appetite Signs of Paraneoplastic syndromes!

Answer 226

Reduced Breath sounds Stony Dull Percussion - suggests malignant pleural effusion Hoarseness (press on RLN) Lymphadenopathy Clubbing Recurrent chest infections Paraneoplastic Changes

Answer 227

- **Chest X-ray:** first-line - Hilar enlargement - Lung consolidation - “Circular opacity” – a visible lesion in the lung field - Pleural effusion – usually unilateral in cancer, and collpase - **CT chest with contrast:** gold-standard imaging; requested if there is an abnormal CXR **or** persistent symptoms with a normal CXR. - **PET-CT:** if CT is suggestive of malignancy, patients should have a staging PET-CT, using 18-fluorodeoxygenase which is taken up by malignant tissue Gold standard **Bronchoscopy + Biopsy**

Answer 228

TNM classification Tumour – how big it is T1 – <3cm T2 - >3cm T3 – invades chest wall, diaphragm and pericardium T4 – invades mediastinum, heart, great vessels, trachea, oesophagus, vertebra, carina

Answer 229

Nodes – how many and where they are N0 - none N1 – Hilar nodes N2 – same side mediastinal nodes or subcarinal N3 – contralateral mediastinum or supraclavicular Metastases 0 - none 1a – tumour on same side 1b – tumour elsewhere X - unknown

Answer 230

SCLC has a poorer prognosis than NSCLC, as SCLC patients will likely have disseminated disease at the point of first presentation.

Answer 231

Often Diagnosed late and Px have metastatic disease: Therefore chemoradiotherapy with platinum-based agents, e.g. **cisplatin** palliative care

Answer 232

Early: Surgical Excision + adjuvant therapy - *lobectomy/ pneumonectomy/ Segmentectomy* Late/Metastatic: palliative treatment with immunotherapy, chemotherapy, and radiotherapy (Nb. NSCLC has poor response to chemotherapy)

Answer 233

- **Local obstruction:** - **Recurrent laryngeal nerve palsy** presents with a hoarse voice. It is caused by the cancer pressing on the recurrent laryngeal nerve as it passes through the mediastinum. - **Phrenic nerve palsy** due to nerve compression; causes diaphragm weakness and presents as shortness of breath. - **Superior vena cava obstruction** caused by direct compression of the tumour on the superior vena cava. It presents with facial swelling, difficulty breathing and distended veins in the neck and upper chest. “**Pemberton’s sign**” is where raising the hands over the head causes facial congestion and cyanosis. This is a medical emergency. - **Horner’s syndrome** is a triad of partial ptosis, anhidrosis and miosis. It is caused by a Pancoast’s tumour (tumour in the pulmonary apex) pressing on the sympathetic ganglion. Pneumothorax

Answer 234

- **Syndrome of inappropriate ADH** (**SIADH**) caused by ectopic ADH secretion by a small cell lung cancer and presents with hyponatraemia. - **Cushing’s syndrome** can be caused by ectopic ACTH secretion by a small cell lung cancer. - **Hypercalcaemia** caused by ectopic parathyroid hormone from a squamous cell carcinoma. - **Hypertrophic pulmonary osteoarthropathy:** causes inflammation of the bones and joints in the wrists and ankles, and clubbing of the fingers and toes - **Limbic encephalitis:** small cell lung cancer causes the immune system to make antibodies to tissues in the brain, specifically the limbic system, causing inflammation in these areas. This causes short term memory impairment, hallucinations, confusion and seizures. It is associated with **anti-Hu antibodies.** - **Lambert-Eaton myasthenic syndrome:** small cell carcinoma prompts the body to produce autoantibodies which bind and destroy neurons

Answer 235

CRESP: C = choriocarcinoma R = renal cell carcinoma E = endometrial carcinoma S = Synovial sarcoma P = Prostate carcinoma These will often give rise to **CANNONBALL metastases, or SNOWSTORM pattern** *Cannonball- multiple large, well circumscribed round pulmonary metastases*

Answer 236

Further left you go: <== More affinity to O2, picks up O2 easier, harder to dissociate O2 Factors that drive curve more left: (Haemoglobin has MORE AFFINTY FOR O2 - LEFT FOR LOCKS IN O2 more ) Higher pH (More alkaline), Decrease in CO2, Decrease in Temperature, Foetal Haemoglobin, Carbon Monoxide , **methemoglobinemia** = elevated methaemoglobin - *(haemoglobin with iron in Fe3+ state, that cannot bind to O2 like normal Hb with Fe2+)*

Answer 237

Further right you go ==> Less affinity for O2, dissociates more easily. Factors that drive curve to the right: (Haemoglobin will have LESS AFFINITY FOR O2) Decrease in pH (more acidic) , More CO2, Increase in Temperature

Answer 238

Respiratory syncytial virus (RSV) causes the majority of cases of bronchiolitis.

Answer 239

A malignant neoplasm of the mesothelial cells of the pleura Other sites include mesothelial cells of peritoneum, pericardium and tunica vaginalis of testes

Answer 240

asbestos exposure, with the development of the malignancy occurring 20-40 years after exposure (after a long latent period).

Answer 241

asbestos fibres make their way to the mesothelium and can get tangled up with the cell's chromosome, causes DNA damage and modification in gene expression Can also activate neutrophil and macrophages. These growths start to express a lot of calretinin, a calcium-binding protein, involved in regulating calcium levels within the cell.

Answer 242

- Signs - **Finger clubbing** - **Reduced breath sounds** - **Stony dull percussion:** suggests a pleural effusion - **Ascites:** if peritoneal disease is present - Signs of metastases: **e.g. lymphadenopathy, hepatomegaly, bone pain/ tenderness, abdominal pain/obstruction** - Symptoms - **Shortness of breath** - **Cough** - **Pleuritic chest pain or chest wall pain** - **Bloody sputum:** if blood vessels are affected - **Constitutional symptoms**: - **Fatigue, fever, night sweats, weight loss**

Answer 243

- **CXR:** unilateral pleural effusion, reduced lung volumes, pleural thickening, lower zone interstitial fibrosis for asbestos - **Contrast-enhanced CT chest:** performed following a suspicious CXR and may demonstrate ****pleural thickening, pleural plaques and enlarged lymph nodes Gold standard - biopsy Stain with selective markers that are positive in mesotheliomas, such as **calretinin** - fried egg shape

Answer 244

Surgery (if operable) otherwise Palliative: Extrapleural pneumonectomy Pleurectomy with decortication Rarely curative +/- Chemotherapy: Cisplatin Pemetrexed +/- Radiotherapy

Answer 245

A symptom of difficult or laboured breathing

Answer 246

Assess degree of baseline functional disability due to Dyspnoea Grade 0: I only get breathless with strenuous exercise Grade 1: I get short of breath when hurrying on level ground or walking up a slight hill Grade 2: On ground level I stop for breath when walking at own pace Grade 3: I stop for breath after walking about 100 yards or after a few minutes on level ground Grade 4: I am too breathless to leave the house or I am breathless when dressing/undressing

Answer 247

COPD Idiopathic Pulmonary Fibrosis Bronchiectasis

Answer 248

ACS Stable angina Chronic HF Pericarditis

Answer 249

Pleural Effusion Lung Cancer Interstitial Lung Disease (pneumonoconiosis)

Answer 250

Musculoskeletal Anxiety Metabolic acidosis: - DKA - Acute renal failure

Answer 251

Epiglottitis refers to inflammation and localised oedema of the epiglottis, which can result in potentially life-threatening airway obstruction.

Answer 252

Haemophilus Influenza B - (gram-negative coccobacillus) *Other organisms have become more common in the developed world, such as Streptococcus pneumoniae and Streptococcus pyogenes.*

Answer 253

Peak age 6-12 (can occur at any) Male gender Unvaccinated Immunocompromised **now far less common due to the introduction of HiB vaccine**

Answer 254

Rapid Onset: Dysphagia Dysphonia (stridor) Drooling Distress Stridor Tripod Position: A sign of respiratory Distress Lean forward, mouth open, tongue out = max air in) Pyrexia

Answer 255

If acutely unwell then NO Ix but immediate Tx Laryngoscopy: diagnostic and will demonstrate swelling and inflammation of the epiglottis Lateral neck radiograph: securing the airway is the priority but, once done, an x-ray can be performed looking for the thumb sign (soft tissue shadow that looks like a thumb pressed into the trachea);

Answer 256

EMERGENCY First Line: - Secure airway, Endotracheal intubation - Nebulised adrenaline - IV antibiotics - Amoxicillin, Co-Amoxiclav, Erythromycin, Doxycycline Second Line: - Dexamethasone

Answer 257

Croup, also known as laryngotracheobronchitis, is a viral upper respiratory tract infection. Children between 6 months and 2 years old are most commonly affected

Answer 258

Main cause: Parainfluenza virus Influenza Adenovirus Respiratory Syncytial Virus (RSV)

Answer 259

Usually improves in <48 hours Increased work of breathing “Barking” cough, occurring in clusters of coughing episodes Hoarse voice Stridor Low grade fever

Answer 260

Oral Dexamethasone (single dose 150mcg/kg) Remember cause is viral!

Answer 261

Upper respiratory tract infection caused by Bordetella pertussis **(Gram negative aerobic coccobacillus)**

Answer 262

The bacterium contains a surface protein, **filamentous haemagglutinin adhesin**, which binds to the sulfatides found on cilia of epithelial cells. Once anchored, the bacterium produces **tracheal cytotoxin, which stops the cilia from beating.**, and Pertussis toxin which inhibits alveolar macrophages This prevents the cilia from clearing debris from the lungs, so the body responds by sending the host into a coughing fit.

Answer 263

Catarrhal stage (1-2 weeks): - Dry, unproductive cough - Low-grade fever - Conjunctivitis - Coryzal symptoms Paroxysmal stage (1-6 weeks): - Coughing fits: typically consist of a short expiratory burst followed by an inspiratory gasp, causing the ‘whoop’ sound - Whoop sound caused sharp inhalation of breath during coughing bout - Post-tussive vomiting Convalescent stage (lasts up to 6 months): Gradual improvement in symptoms

Answer 264

Nasopharyngeal swab/aspirate: Culture/PCR Anti-pertussis toxin immunoglobulin G (IgG) serology

Answer 265

Notify PHE Hospital admission if severe Antibiotics: if Cough Sx is within 21 days Macrolids - Clarithromycin, Azithromycin School work absence: highly contagious

Answer 266

Pneumonia Encephalopathy Otitis media Injuries from coughing - pneumothorax Seizures.

Answer 267

Type 1 respiratory failure occurs when the respiratory system cannot adequately provide oxygen to the body, leading to hypoxemia.

Answer 268

Type 1 = 1 change (O2) pO2 (partial O2 pressure) is low pCO2 (partial CO2 pressure) is low or normal

Answer 269

Pulmonary Embolism

Answer 270

Type 2 respiratory failure occurs when the respiratory system cannot sufficiently remove carbon dioxide from the body, leading to hypercapnia

Answer 271

Type 2 = 2 changes (O2 & CO2) pO2 is low pCO2 is high

Answer 272

Hypoventilation: Caused by COPD Neuromuscular weakness Obesity Chest wall deformity Reduced respiratory drive (opiates)

Answer 273

Bounding pulse Flapping tremor (asterixis) Confusion Drowsiness Reduced consciousness

Answer 274

diffusing capacity of the lungs for carbon monoxide (DLCO) Transfer Coefficient of oxygen/CO the ability of the lungs to transfer gas from inhaled air to the red blood cells in pulmonary capillaries

Answer 275

Can calculate by inspiring a small amount of carbon monoxide (not too much since can kill) hold breath for 10 seconds at total lung capacity (TLC) then the gas transferred is measured

Answer 276

Pulmonary haemorrhage - can absorb O2 very efficiently due to bleeding resulting in more red blood cells being available

Answer 277

Severe emphysema Fibrosing alveolitis Anaemia Pulmonary hypertension Idiopathic pulmonary fibrosis COPD

Answer 278

An autoimmune disease characterised by Anti-GBM autoantibodies that attack the lungs and kidneys. A type of Pulmonary Vasculitis

Answer 279

n Goodpasture syndrome, autoantibodies bind to a specific part of **the α3 chain that is usually hidden deep** within the folded triple helix of **Type IV collagen.** (makes the basement membrane if you can remember phase 1) This is a type II hypersensitivity reaction - autoantibodies, usually IgG but rarely IgM or IgA, bind to the the α3 chain, and activate the complement system

Answer 280

Symptoms Typically starts with upper respiratory tract infection e.g. sneezing, nasal discharge, nasal congestion, runny nose Fever Cough Tiredness Signs Intermittent haemoptysis Anaemia – may result from persistent intrapulmonary bleeding Acute glomerulonephritis

Answer 281

Anti-GBM antibodies in blood CXR – transient patchy shadows/pulmonary infiltrates due to pulmonary haemorrhage often in lower zones Kidney biopsy – crescentic glomerulonephritis

Answer 282

Corticosteroids – prednisolone Plasmapheresis – remove blood and clean to remove anti-GBM antibodies before inserting it back Bilateral nephrectomy – in severe/unresponsive cases

Answer 283

Inflammation of the pharynx with exudate production

Answer 284

Viral- EBV and adenovirus. (rhinovirus causes tonsillitis) Bacteria- group A Strep (S.pyogenes) Staph aureus is most common for recurrent tonsillitis, due to antimicrobial resistance

Answer 285

Rheumatic fever (typically 2-4 weeks post S.pyogenes infection)

Answer 286

Viral is self-limiting Bacterial- amoxicillin/flucloxacillin

Answer 287

Infection and inflammation of the middle ear. The bacteria enter from the back of the throat through the eustachian tube. Bacterial infection of the inner ear is often preceded by a viral upper respiratory tract infection.

Answer 288

The middle ear is the space that sits between the tympanic membrane (ear drum) and the oval and round windows that connect to the inner ear. Contains The 3 auditory ossicles, the malleus, incus and stapes, and its where the eustachian tube connects to the ear It is a very common site of infection in children.

Answer 289

Bacteria- Streptococcus pneumoniae (most common), haemophilus influenzae, and staphylococcus aureus Viral- Respiratory syncytial virus, rhinovirus, adenovirus

Answer 290

Otoscopy examination will reveal a **bulging**, red/cloudy tympanic membrane - **Ear pain** often associated with holding, tugging or rubbing of the ear in children - **Reduced hearing** - **Recent upper respiratory tract infection** - **Balance issues and vertigo:** if infection affects the vestibular system - **Non-specific symptoms** - Fever - Irritability and poor feeding - Vomiting - Sore throat

Answer 291

Will usually resolve within 3-7 days Can give amoxicillin 2nd line would be Co-amoxiclav

Answer 292

Viral infection (Rhinovirus, parainfluenza virus, and influenza virus) is the most common. (As per causes URTI) Can be caused by bacterial infection (Streptococcus pneumoniae, haemophilus influenzae and staphylococcus aureus)

Answer 293

Symptoms Fontal headache Facial pain Fever Signs Purulent nasal discharge Tenderness

Answer 294

Self limiting Usually lasts <10 days and has non-purulent discharge

Answer 295

The Centor criteria give an indication of the likelihood of a sore throat being due to bacterial infection. The criteria are: Tonsillar exudate Tender anterior cervical adenopathy Fever over 38°C (100.5°F) by history Absence of cough. If 3 or 4 of Centor criteria are met, the positive predictive value is 40% to 60%. The absence of 3 or 4 of the Centor criteria has a fairly high negative predictive value of 80%.

Answer 296

Haematuria

Answer 297

Peripheral Neuropahty, **Can also be a trigger for SLE**

Answer 298

Hepatitis, Also gout, and joint pain

Answer 299

– Eye problems e.g. uveitis

Answer 300

Note RI = 2 months, PE = 6 months

Answer 301

one off dose of ciprofloxacin

Answer 302

They are lipophilic - so take longer to act as a bronchodilator

Answer 303

the classic symptoms of a pulmonary embolism are pleuritic chest pain (worse on deep breaths), shortness of breath and haemoptysis

Answer 304

Obstruction of the pulmonary vasculature, secondary to an embolus.

Answer 305

Embolus enters Right heart via IVC This causes occlusion of small blood vessels Causes V/Q mismatch as blood cannot gas exchange. Causes **Hypoxic vasoconstriction and reactive bronchoconstriction.** Narrows airways leading to Dyspnoea Hypoxic vasoconstriction **increases pulmonary vascular resistance causing Pulm HTN. This causes RV heart strain and hypertrophy. Eventually this leads to RHF** (Cor Pulmonale)

Answer 306

Anything affecting Virchow’s Triad: Endothelial Injury: Smoking, HTN, Trauma Venous Stasis: Immobility, Post surgery, AF, Obesity Hypercoagulability - Oral contraceptive pill, Polycythaemia, Malignancy

Answer 307

Hypotension Tachypnoea Tachycardia Raised respiratory rate Low grade fever Haemodynamic instability causing hypotension Raised JVP Hypoxia

Answer 308

WELLS Score: <4 Unlikely PE - perform D-dimer. - if >500ng/ml then perform CT Pulmonary Angiogram. > 4 Likely PE - Perform CTPA (GS): Will diagnose PE clot.

Answer 309

Active Cancer paralysis/recent plaster immoblisation of leg Recenlty bedridden or major surgery Local tenderness along distribution of venous system Entire leg swollen/calf swelling Pitting oedma Previous DVT All of these score +1 point Alternative diagnosis at least as likely as a DVT = -2 points

Answer 310

ECG: S1Q3T3 ***CHEST XRAY NORMALLY NORMAL IN A PE*** CTPA - GS diagnostic Bloods: D-Dimer Anti-phospholipid Abs - in unprovoked DVT/PE

Answer 311

ECG: S1Q3T3 Deep S waves in lead I Deep Q waves in lead III T waves inverted in Lead III RBBB V1-3 & Sinus Tachycardia

Answer 312

Anticoagulants: 1st Line: DOAC (Rivaroxaban, Apixaban) (LMWH if DOAC CI) eg dalteparin, 2nd Line: Warfarin If unstable/severe - Thrombolysis (clot busting) - Alteplase If fails - Catheter embolectomy

Answer 313

Compression Stockings Regular walking SC LMWH - dalteparin,

Answer 314

Right lower lobe of the lung **most vertically transposed**

Answer 315

Morning lower, evening higher. Diurnal variation