RACP

Question 1

What is Cotinine?

Answer 1

Metabolic product of nicotine - provides quantifiable method of assessing nicotine exposure (via smoking, passive, or NRT). Detectable in blood, saliva or urine.

Question 2

What is the DECAF score?

What are its components?

Answer 2

Score used to assess COPD exacerbations ?home vs inpatient management.
Score 0-1 –> early dc
Score 2 = intermediate
Score >= 3 –> admit / palliate

Components:

• eMRC dyspnoea scale on typical day (2 points if too dyspnoeic to leave house)
• eosinophils
• consolidation on CXR
• acidaemia (pH <7.30)
• presence of AF

Question 3

What is Loeffler Syndrome?

Answer 3

Transient passage of helminth larvae (mainly Ascaris) from blood to alveoli through to proximal airways and then into digestive tract. Manifest on CXR as migrating / self-resolving opacities. Associated with blood eosinophilia.

Question 4

Which cells produce surfactant?

Answer 4

Type 2 pneumocytes

Question 5

Cause of non-infective endocarditis

Answer 5

Libman-sacks disease (from SLE)

Question 6

Bohr Effect

Answer 6

Increase in acidity (decrease pH) or increased pCO2 in a tissue causes RIGHT shift –> decreased O2 affinity –> increased O2 delivery to metabolically-active tissue.

Question 7

Haldane Effect

Answer 7

Increased oxygen binding makes CO2/H+ bind less well to Hb molecule (increases CO2 delivery to the lungs)

Question 8

Zero Order Kinetics

Answer 8

Clearance by easily saturated enzyme system. Once saturated, the rate of clearance plateaus, and does not vary no matter how much drug is present. Results in a constant rate of elimination predisposing to high levels of the drug and toxicity.
Examples: phenytoin, alcohol and salicylates.

Question 9

First Order Kinetics

Answer 9

Linear process, where the rate of elimination is proportional to the drug concentration. This means that the higher the drug concentration, the higher its elimination rate.

Question 10

What is Pompholyx?

Answer 10

Eczema of soles of hands / feet.

Often triggered by heat / humidity.

Question 11

Amiodarone-induced Thyrotoxicosis:

Type 1 vs Type 2

Answer 11

Type 1:
Excess iodine load –> excess thyroid hormone made
Goitre present
Rx = carbimazole

Type 2:
Destructive thyroiditis
No goitre
Rx = prednisone

Question 12

Key Stain for Lymphoblasts (ALL)

Answer 12

TdT (staining for DNA polymerase)

Question 13

Key surface markers for B cells

Answer 13

CD10, CD19, CD20

Question 14

Acute Promyelocytic Leukaemia

• Marker
• Key Receptor
• Key Complication
• Treatment

Answer 14

t(15;17)

Retinoic acid receptor

Widespread DIC

All-trans retinoic acid (ATRA) –> encourages cell differentiation

Question 15

Auer Rods

Answer 15

Crystal ‘rod’ seen in myeloblasts in AML

Question 16

Definition of ‘pulmonary nodule’

Answer 16

<3cm lesion, completely surrounded by lung parenchyma.

Larger lesions called ‘masses’

Question 17

Most common causes of benign lung nodules (2)

Answer 17

Infectious granulomas

Pulmonary harmartomas

Question 18

Criteria for not further following up a pulmonary nodule (2)

Answer 18

Initial size <6mm (i.e. =<5mm) (and no other high risk features)

OR

No growth over 2 years

Question 19

Diagnosing BK Nephropathy:
- Typical approach

• Gold standard approach

Answer 19

1. Viral load (via PCR) in context of allograft dysfunction

2. Renal biopsy (especially if DDx includes transplant rejection, which has opposite management approach…)

Question 20

Eculizumab:
- MoA

• Indications

Answer 20

Binds C5 (i.e. the terminal complement component) - prevents formation / activation of C5a and C5b

PNH, aHUS, NMO (if anti-AQP4 +)

Question 21

Emicizumab
- Indication

• MoA

Answer 21

Haemophilia A

Cross-links F9a and F10 –> leading to F10a formation. (F10a then converts prothrombin –> thrombin, (along with F5)).

Question 22

Clopidogrel Mechanism

Answer 22

Irreversibly inhibits P2Y12 of ADP receptors on platelets → prevents activation of GPIIb/IIIa complex → no fibrinogen cross-linking → no platelet aggregation

(Whereas ticagrelor is reversible)

Question 23

HIV Binding Molecules

Answer 23

GP 120 on virus –> CD4 on host

Then GP41 –> CCR5 (acute) or CXCR4 (chronic)

Question 24

HIV Protective HLA and mechanism

Answer 24

HLA B57*01

Allows binding of MHC-1 to HIV core protein (which can not be mutated) –> strong CD8 response –> slower progression to AIDS (and less hypersensitivity to Abacavir)

Question 25

HIV Prophylaxis cut-offs and drug to use - PJP - Cryptococcus

Answer 25

PJP; give cotrimoxazole if CD4 count <200 Cryptococcys; give fluconazole if CD4 count <100

Question 26

HIV pre-exposure prophylaxis medications

Answer 26

Tenofovir + emtracitibine given daily

Question 27

HIV post-exposure prophylaxis (3)

Answer 27

Must be started <72h, 28 day course If risk <1 / 1,000 --> 2x drug therapy (tenofovir + emtracitabine / lamivudine) If risk >1 / 1,000 --> 3x drug therapy (2x drug Rx + dolutegravir)

Question 28

Rasburicase - mechanism, caution scenario

Answer 28

Supplies uricase enzyme Converts uric acid to allantoin (water soluble --> renal clearance) - I.e. can acutely LOWER urate concentration (whereas allopurinol just stops production) Caution in G6PD deficiency

Question 29

Azathioprine Metabolism

Answer 29

Active compound = 6-TGN - Levels > 235 a/w better clinical response (but more myelotoxicity) Inactive metabolite = 6-MMP - Levels > 5,700 a/w hepatotoxicity Conversion to 6-MMP can be reduced by allopurinol - give for 'shunters' / hypermethylators Check TPMT enzyme beforehand

Question 30

Binding site in Heparin-induced Thrombocytopaenia (HIT)

Answer 30

Antibodies against heparin-platelet factor 4 complex | which can also activate platelets, leading to thrombotic tendency

Question 31

Kings College Criteria for Liver Transplant (Paracetamol OD)

Answer 31

Arterial pH <7.30 Or all of: - PT >100sec (INR > 6.5) - Creatinine > 300 - Grade 3 or 4 encephalopathy (Whereas PT >100sec = absolute criteria for non-paracetamol liver failure)

Question 32

G6PD Inheritance Pattern

Answer 32

X-linked recessive

Question 33

Tocilizumab Mechanism

Answer 33

Binds soluble- and membrane-bound IL-6 receptors

Question 34

Rheumatoid Arthritis Susceptibility Genes

Answer 34

HLA-DRB1 Subtypes (i.e. different alleles of DRB1): HLA-DRB1*04:01 and HLA-DRB1*04:04

Question 35

RA: Shared epitope concept

Answer 35

HLA alleles that confer increased risk for RA all have similar amino acid sequence at position 70-74 (even in completely different HLA subtypes). (QKRAA)

Question 36

RF vs ACPA; which has stronger correlation with extraarticular disease severity?

Answer 36

RF - e.g. ILD, vasculitis | Whereas ACPAs predict more severe/erosive articular damage

Question 37

Antiphospholipid Abs

Answer 37

Anti-cardiolipin Lupus anticoagulant Anti-beta-2-glycoprotein (Need to be present in serum on 2x occasions at least 12 weeks apart)

Question 38

Most specific auto-antibody for SLE

Answer 38

Anti-Sm

Question 39

Auto-antibody that goes up/down with SLE disease severity e.g. nephritis, vasculitis

Answer 39

Anti-dsDNA

Question 40

Th1 cytokines

Answer 40

IFNy IL-2 IL-3

Question 41

Th2 cytokines

Answer 41

``` IL-4 IL-5 IL-6 IL-10 IL-13 TGF-beta ```

Question 42

Tumour markers: - Ovarian cancer - Breast cancer - Pancreatic cancer

Answer 42

CA12-5 CA15-3 CA19-9

Question 43

Half life formula

Answer 43

T1/2 = 0.693 x Vd/CL | Can be rearranged to give CL value, units L/h

Question 44

Anti-phospholipase A2 Receptor Antibodies are associated with which GN? (Anti-PLAR2)

Answer 44

Idiopathic Membranous GN

Question 45

Key CJD features (3)

Answer 45

Rapid dementia Myoclonus Periodic, triphasic sharp waves on EEG

Question 46

VZV post-exposure prophylaxis

Answer 46

Immunocompetent --> VZV vaccine Immunocompromised --> VZV Ig (within 10 days)

Question 47

Medications causing acute interstitial nephritis (3)

Answer 47

PPIs Antibiotics NSAIDs

Question 48

Key test for urea cycle disorders

Answer 48

Elevated ammonia (with normal BSL and anion gap)

Question 49

Aprepitent receptor target

Answer 49

Neurokinin 1 Receptor --> blocks substance P | Used for pruritis and chemo-induced N+V

Question 50

BRCA gene inheritance

Answer 50

Autosomal dominant

Question 51

Syphilis cardiolipin tests / 'non-treponemal' tests

Answer 51

VDRL and RPR Become negative after treatment, used for monitoring treatment / recurrence

Question 52

Syphilis 'treponemal' tests (3,1)

Answer 52

E.g. Treponema pallidum haemagglutination test (TPHA) Enzyme immunoassay (EIA) T. pallidum particle agglutination test (TPPA) Stay positive after treatment

Question 53

Most common cause of inherited thrombophilia

Answer 53

Factor V Leiden (activated protein C resistance) Due to mutation, protein C can not inactivate factor 5 --> procoagulant status (Protein C normally inactivates F5 and F8)

Question 54

Extra-intestinal IBD features associated with disease activity (4)

Answer 54

Pauciarticular arthritis (asymmetric) / large joint Erythema nodosum Episcleritis Osteoporosis

Question 55

Extra-intestinal IBD features NOT associated with disease activity (5)

Answer 55

``` 'APUPS': Axial / ank spond Pyoderma gangrenosum Uveitis PSC Symmetric, small joint (polyarticular) arthritis ```

Question 56

Key areas for lesions on MRI in MS diagnosis

Answer 56

Periventricular - ‘Dawson’s fingers’ Juxtacortical - ‘U fibre’ / cortical Infratentorial (cf. vascular) Spinal cord

Question 57

ESCAPPM Organisms - Definition - Transmission of resistance - Management (2) - Organisms

Answer 57

ESBL Subgroup with inducible beta-lactamase activity AmpC chromosomally-mediated Management: Carbapenems Piptaz ``` Enterobacter species Serratia spp Citrobacter freundii (and braakii) Acinetobacter and Aeromonas spp Proteus (not mirabilis) Providencia spp Morganella morganii ```

Question 58

Ann Arbor Staging

Answer 58

I - single node II - 2x nodes on same side of diaphragm III - nodes on either side of diaphragm IV - extra-lymphatic organ involved A - no systemic Sx (other than pruritis) B - >10% weight loss, fever, night sweats

Question 59

AML poor prognosis markers (2)

Answer 59

Deletions of chromosomes 5 or 7, | FLT3

Question 60

Pendred syndrome

Answer 60

Autosomal recessive Bilateral SNHL + hypothyroidism + goitre MRI shows 1.5 turns of cochlea (cf 2.5) Rx = thyroxine and hearing aids

Question 61

In what ILD would biopsy show hyaline membranes?

Answer 61

Acute interstitial pneumonia

Question 62

What is the main determinant of drug plasma concentration at steady state?

Answer 62

Drug clearance

Question 63

MODY3 mutation

Answer 63

HNF1a | Most common form of MODY

Question 64

MODY2 mutation

Answer 64

glucokinase

Question 65

MODY1 mutation

Answer 65

HNF4a

Question 66

Define nonsense mutation

Answer 66

Base change that causes a stop codon

Question 67

Mutation in MDS with good prognosis

Answer 67

Deletion (5q) - responds to lenalidomide

Question 68

Gitleman syndrome

Answer 68

Defect in distal tubule Na/Cl transporter, like being on a thiazide

Question 69

Bartter syndrome

Answer 69

Defect in thick ascending limb Na-K-2Cl transporter, like being on frusemide

Question 70

Liddle syndrome (3)

Answer 70

Gain of function mutation --> ENaC can no longer be broken down --> excess sodium absorption / potassium loss --> HTN / hypo-K / alkalosis Hyper-aldosterone-like state, (but low renin AND low aldosterone levels (cf. Conn's)) Spironolactone not effective in Rx (use amiloride or triamterene which block ENaC). Salt restriction also effective at maintaining normal sodium / bicarb levels. Autosomal dominant

Question 71

Gefitinib mechanism

Answer 71

EGFR inhibitor - used in lung adenoca.

Question 72

Well's criteria for PE (7) + scores

Answer 72

``` PE leading DDx - 3 Limb swelling - 3 HR > 100 - 1.5 Immobilisation - 1.5 Previous PE - 1.5 Haemoptyisis - 1 Malignancy - 1 ```

Question 73

Acute Interstitial Nephritis urine findings

Answer 73

+ve white cells - ve red cells - ve protein

Question 74

Ipilimumab mechanism

Answer 74

Inhibition of CTLA-4 co-stimulator on T-cells / Treg cells

Question 75

MRI 'hummingbird sign' is seen in...

Answer 75

PSP (due to midbrain atrophy)

Question 76

Pre-op insulin: prior to major surgery

Answer 76

Give IV insulin at variable rate

Question 77

True/False: correcting vitamin D deficiency may help prevent SLE flares?

Answer 77

True

Question 78

Anion gap formula

Answer 78

(Na + K) - (Cl + HCO3) Ref range 4 - 12 If high, implies presence of unaccounted for anions (that have consumed bicarb)

Question 79

True/False: osteoarthritis is a risk factor for osteoporosis?

Answer 79

False (may even be protective)

Question 80

Predictor of reactive arthritis recurrence or persistance

Answer 80

HLA-B27 gene

Question 81

ACEi benefits: - Renal - Ophthal

Answer 81

If microalbuminuria, then ACEi/ARB slow progression to proteinuria (may initially see a creatinine rise) If no microalbuminuria, there is no renal benefit but protects against retinopathy

Question 82

Implication of decreasing insulin requirement in GDM

Answer 82

Could suggest placental failure (the placenta is often a cause of insulin resistance) --> needs obstetric input

Question 83

What agent causes cold-aggravated neuropathy?

Answer 83

Oxaliplatin (via voltage-gated Na channels)

Question 84

Maternal / peripartum HIV management

Answer 84

Mum should be on ART regardless of HIV load Intrapartum Zidovudine if HIV RNA > 1,000 Infant should receive PEP No breast feeding C-section if maternal HIV RNA >400 at 36/40

Question 85

SAAG interpretation

Answer 85

SAAG > 11 = portal HTN | i.e. despite high oncotic pressure, there is still ascites forming indicating the presence of hydrostatic pressure

Question 86

Acanthocytes - AKA - Describe appearance - Commonly found in...

Answer 86

AKA spur cells Large red cell with spike-like projections Severe liver disease

Question 87

Light microscopy: 'sub-epithelial deposits' are seen in:

Answer 87

Membranous nephropathy | AKA spike and dome appearance

Question 88

Gram negative cocci species (2)

Answer 88

Neisseria | Moraxella

Question 89

Horner's / eye sympathetic chain anatomy / neuron orders

Answer 89

1st order: posterior hypothalamus to spinal cord (ciliospinal centre of Budge) at C8-T2 2nd order: fibres exit at T1, cross lung apex, ascend in cervical sympathetic chain, terminate at superior cervical ganglion at level of bifurcation of common carotid artery (C3-4) 3rd order: ascend along internal carotid artery to eye

Question 90

Mifepristone: - Mechanism - Use

Answer 90

Glucocorticoid receptor antagonist Hyperglycaemia of Cushing's

Question 91

Ketoconazole use in adrenal disease: | - Mechanism

Answer 91

Inhibits androgen synthesis

Question 92

Ambrisentan mechanism

Answer 92

Endothlin A + B ANTAGONIST

Question 93

Sildenafil mechanism

Answer 93

Inhibits PDE-5

Question 94

Riociguat mechanism

Answer 94

Sensitises guanlate cyclase to NO / activates gunalate cyclase

Question 95

Iloprost mechanism

Answer 95

Prostaglandin analogue

Question 96

Cell type activated in HLH

Answer 96

Macrophages (failed elimination by NK cells / CTLs)

Question 97

Define: fraction excreted unchanged (Fe)

Answer 97

Proportion of active drug cleared renally in healthy person.

Question 98

Genetic defect in Autoimmune Polyendocrine Syndrome type 1 (AKA APECED)

Answer 98

AIRE - involved in regulating T-cell selection in the thymus

Question 99

Cell surface markers of Reed-Sternberg cells

Answer 99

CD15 (75%) and CD30 (100%) | RS cell is a 'crippled' germinal center B cell that has non-functional IgG gene arrangement

Question 100

Pembrolizumab and Nivolumab mechanism

Answer 100

Inhibits PD-1

Question 101

Adrenal gland zones / hormone synthesised (4)

Answer 101

Zona glomerulosa - mineralocorticoids Zona fasciculata - glucocorticoids Zona reticularis - androgens Medulla - catecholamines

Question 102

PBC associated diseases (2)

Answer 102

Sjogrens | Autoimmune thyroid disease

Question 103

MRCP / ERCP 'string of beads' pattern refers to:

Answer 103

PSC | Multiple strictures in the biliary system

Question 104

Red Man Syndrome mechanism

Answer 104

Direct mast cell stimulation by vancomycin --> histamine release

Question 105

Castleman disease - characteristic finding:

Answer 105

Elevated IL-6

Question 106

Treatment of latent TB (3)

Answer 106

3/12 of rifampicin + isoniazid (+pyridoxine) OR 9/12 isoniazid (+pyridoxine) OR Rifapentine weekly for 3/12

Question 107

Treatment of active TB

Answer 107

2/12 of 'RIPE' (+pyridoxine) then 4/12 of rifampicin + isoniazid

Question 108

Antibody target in ITP

Answer 108

GP IIb/IIIa complex

Question 109

CHARTS Acronym for upper zone fibrosis

Answer 109

``` Coal worker's pneumocosis Hypersensitivity pneumonitis (AKA extrinsic allergic alveolitis) / histiocytosis Ank spond Radiation Tb Silicosis / sarcoidosis ``` OR: 'everything you inhale except for ank. spond. and sarcoidosis'

Question 110

Ventilation strategy for patients with ARDS

Answer 110

Low tidal volume (4-8ml/kg) with PEEP

Question 111

Smouldering myeloma criteria | AKA 'asymptomatic myeloma'

Answer 111

1. Paraprotein >30 OR Plasma cells >10% 2. AND no myeloma-relate end-organ impairment

Question 112

MRI findings in CJD

Answer 112

Caudate and putamen T2 hyperintensity and diffusion restriction on DWI/ADC

Question 113

Proven benefit(s) of pre-endoscopic PPI

Answer 113

Reduced endoscopic intervention (i.e. no difference in mortality, re-bleeding or surgery)

Question 114

Home oxygen indications for COPD

Answer 114

PaO2 <55mHg (7.3 kPa) OR PaO2 <60mmHg (7.7 - 7.8 kPa) if cor pulmonale / right HF / erythrocytosis / pulmonary HTN

Question 115

Group of patients that might particularly benefit from CABG (rather than PCI)

Answer 115

Diabetics

Question 116

Treatment for BRAF V600E mutation in melanoma

Answer 116

BRAF inhibitor AND MEK inhibitor combination e.g. Dabrafenib + Trametinib

Question 117

Treatment for cerebral venous sinus thrombosis

Answer 117

Clexane + warfarin, then warfarin once INR therapeutic (even if small intracranial bleed) NO evidence for DOACs

Question 118

Skin complication of anti-TNF Rx

Answer 118

Psoriasis

Question 119

MDR-TB definition

Answer 119

TB resistant to Rifampicin and Isoniazid

Question 120

XDR-TB definition

Answer 120

MDR-TB + resistance of moxifloxacin and amikacin

Question 121

5-FU-related chest pain mechanism

Answer 121

Coronary artery spasm | Usually associated with first infusion

Question 122

Antibiotic inactivated by surfactant

Answer 122

Daptomycin (due to aggregating with phospholipids in surfactant)

Question 123

Antibiotic with large volume of distribution, and so not useful in sepsis

Answer 123

Tigecycline

Question 124

Hemibalism site of lesion

Answer 124

Contralateral sub-thalamic nucleus or basal ganglia | One interesting cause includes hyperglycaemia

Question 125

Anti-epileptic that can worsen absence seizures

Answer 125

Carbamazepine

Question 126

Anti-GAD Abs - Found in: - Results in (syndrome)

Answer 126

Breast, colorectal, SCLC Stiff man syndrome (diffuse hypertonia)

Question 127

Anti-Hu - Found in: - Results in:

Answer 127

SCLC, neuroblastoma Limbic encephalitis, rapid peripheral neuropathy

Question 128

Anti-Ma - Found in: - Results in:

Answer 128

Testicular cancer Rhombencephalitis

Question 129

Anti-Yo - Found in: - Results in:

Answer 129

Breast, ovarian Cerebellar degeneration

Question 130

Anti-Ri - Found in: - Results in:

Answer 130

Breast, SCLC Ocular opsoclonus myoclonus, rhombencephalitis

Question 131

Anti-amphiphysin - Found in: - Results in:

Answer 131

Breast Stiff person syndrome

Question 132

LGI1 Encephalitis - What is it - 2x key features

Answer 132

A subtype of anti-VGKC-mediated encephalitis Rapid memory decline Faciobrachial and dystonic seizures

Question 133

Osteoprotegerin functions (2)

Answer 133

AKA Osteoclastogenesis Inhibitory Factor (OCIF) Acts as decoy for RANK-L (thereby reducing stimulation of osteoclastic maturation) Acts as decoy for 'TRAIL' (thereby inhibiting apoptosis of osteoclasts)

Question 134

Hb-O2 curve: causes for RIGHT shift

Answer 134

E.g. in muscle: - Increased temp - Increased pCO2 - Lower pH (increased H+) - Increased 2,3-BPG

Question 135

Bohr Effect

Answer 135

Increased pCO2 / H+ make O2 bind Hb with less affinity (thereby increasing its delivery to metabolically-active tissue)

Question 136

Components of HbA

Answer 136

2x alpha + 2x beta chains, i.e. normal adult Hb

Question 137

Components of HbF

Answer 137

2x alpha + 2x gamma chain (foetal Hb)

Question 138

What is HbS?

Answer 138

Sickle cell Hb - due to point mutation in beta chain Mutated beta chains join with normal alpha chains to form HbS If homozygous --> sickle cell anaemia HbS has reduced solubility when deoxygenated → undergoes polymerisation → distorted shape → reduced flow/deformability + increased adhesion to endothelial cells

Question 139

What is HbE?

Answer 139

Beta gene mutation causing structurally abnormal Hb Weak union beta alpha/beta --> unstable Hb (behaves like beta-thal trait) Implications if one parent has HbE and the other beta-thal

Question 140

What is HbH?

Answer 140

Moderate-severe form of alpha-thal where only 1x functional alpha gene is present (i.e. -a / --) HbH is made from beta chains (beta-4) that combine to each other

Question 141

What is HbB?

Answer 141

Hb Barts Hb made of 4x gamma chains Seen in alpha-thal major (i.e. no functional alpha genes present)

Question 142

Components of 4T score for HIT probability calculator

Answer 142

Thrombocytopaenia Timing Thrombosis Other causes present

Question 143

What is Gaucher Disease (1) Most common manifestations (4)

Answer 143

Most common lysosomal storage disease; abnormal glycolipid build-up in macrophage lysosomes ('lipid-laden macrophages') due to deficiency in glucocerebrosidase enzyme. Splenomegaly Thrombocytopaenia Neurological deficit 'Flask' appearance of long bones Rx = enzyme replacement therapy OR substrate reduction therapy

Question 144

Components of 'Fried Frailty Tool' (AKA Frailty Phenotype) (5)

Answer 144

``` Weight loss >5% Fatigue Reduced grip strength Reduced mobility speed Decreased weekly physical activity (based on energy expenditure) ```

Question 145

What organism causes erythrasma?

Answer 145

Corynebacterium | pink/brown patches in groin/axillae

Question 146

Interventions that improve COPD mortality (3)

Answer 146

``` Stop smoking Home O2 (if meets criteria) Lung reduction surgery in selected cases ```

Question 147

True / False: Obesity increases BNP

Answer 147

False - along with standard HF Rx, obesity also decreases BNP

Question 148

Gestational diabetes diagnosis thresholds for BSL: - Fasting - After 2h

Answer 148

>= 5.6 >=7.8

Question 149

Muscarinic Receptors

Answer 149

G-protein coupled M1 (Gq) - CNS M2 (Gi) - Heart M3 (Gq) - Other organs: gut, glands, eyes, blood vessels

Question 150

Nicotinic receptors

Answer 150

Ligand-gated ion channels N1 / Nm - NMJ N2 / Nn - post-ganglionic cell body / adrenal gland

Question 151

Th17 cytokines

Answer 151

IL-17 IL-22 (Small amount of IL-10)

Question 152

What type of growth factor is produced by myxomas?

Answer 152

VEGF

Question 153

Most common type of malignant primary cardiac tumour

Answer 153

Sarcomas | But metastatic cardiac tumours 20x more common

Question 154

Example of ergot-derivative meds (2) Mechanism Key side-effects (2)

Answer 154

Bromocriptine, cabergoline - Work as dopamine receptor agonists Pulmonary / cardiac / retroperitoneal fibrosis Impulse control disorders

Question 155

In utero lithium exposure increases risk of the following cardiac abnormality:

Answer 155

Ebstein's anomaly (low insertion of the tricuspid valve --> large RA, small RV) See prominent TR and a-waves

Question 156

Pathergy test: - What is it? - What condition is it found in?

Answer 156

Exaggerated skin injury after mild trauma Behcet's disease (also in pyoderma gangrenosum and Sweet syndrome)

Question 157

What is the EMA test for?

Answer 157

Diagnosis of hereditary spherocytosis. Test measures amount of fluorescence from a cell.

Question 158

Lights criteria for exudative effusion

Answer 158

1 of: Pleural:serum protein >0.5 Pleural:serum LDH >0.6 Pleural LDH >2/3 of serum LDH ULN

Question 159

Haemorrhagic bullae are caused by what organism?

Answer 159

Vibrio vulnificus Further info: G-, seafood ingestion or contaminated wound, severe sepsis, particularly liver disease or haemochromatosis, tetracycline + 3rd gen cephalosporin

Question 160

Key urine finding in ATN

Answer 160

'Muddy-brown' granular, epithelial cell casts and free renal tubular epithelial cells (that have been 'shed')

Question 161

AML: cytogenetic changes with good prognosis (4, 1)

Answer 161

t(8;21) (which generates the RUNX1-RUNX1T1 fusion gene) t(15;17) (APML) inv(16) NPM1 mutation For these cases, can try cytaribine consolidation (rather than SCT)

Question 162

AML: cytogenetic changes with poor prognosis (5)

Answer 162

-5, -7, del (5q), abnormal 3q, FLT3

Question 163

PD-1 inhibitors

Answer 163

Pembrolizumab | Nivolumab

Question 164

PD-1L inhibitors

Answer 164

Atezolizumab | Durvalumab

Question 165

CTLA-4: - Mechanism - Examples of blockers

Answer 165

Preferentially binds co-stimulatory molecule CD80/86 (AKA B7) --> loss of T-cell activation Ipilimumabc

Question 166

MELD score components

Answer 166

``` Dialysis Creatinine Bilirubin INR Sodium ```

Question 167

Child pugh components

Answer 167

``` Bilirubin Albumin INR Ascites Encephalopathy ```

Question 168

Enterococcus endocarditis Rx (2)

Answer 168

Gent-sensitive / standard: Amoxicillin 6/52 + gentamicin 2/52 (synergy) Gent-resistance: Amoxicillin + ceftriaxone (6/52 for both) (synergy) - Amox covers PBP4 and 5 - Cef covers PBP2 and 3

Question 169

Organisms that do not produce nitrites

Answer 169

Gram positives: Enterococcus Staph. saprophyticus

Question 170

Riociguat - Use - Mechanism

Answer 170

Medical therapy of CTEPH (in rare cases where surgical intervention (thrombendarterectomy) is not done). Guanalate cyclase agonist (which enhances the effects of NO binding)

Question 171

What is erythromelalgia?

Answer 171

Intermittent red/hot extremities. Associated with MDS Conservative cooling measures and aspirin

Question 172

Peritoneal dialysis-associated peritonitis management overview (5)

Answer 172

Most common organism: S. epidermidis Either continuous or intermittent (at least 6h dwell) administration. Empiric Rx = Gentamicin + Cefazolin If vancomycin required, replace cefazolin with it If suspected diverticulitis / intestinal perf, add metronidazole If gentamicin contraindicated, use single-agent cefepime regimen Duration 14-21 days

Question 173

Spirometry: fixed obstruction flow-volume loop description

Answer 173

Plateaued inspiratory and expiratory phases

Question 174

Spirometry: variable intrathoracic obstruction flow-volume loop description

Answer 174

Plateau of expiratory phase | Normal inspiratory phase

Question 175

Spirometry: variable extrathoracic obstruction flow-volume loop description

Answer 175

Normal expiratory phase | Plateau of inspiratory phase

Question 176

LADA one sentence description

Answer 176

Slowly progressive destruction of beta cells - gradual insulin deficiency (not as rapid as ‘traditional’ T1DM, but still a subtype of T1) (NOTE: Starting insulin early may help to preserve beta cell function)

Question 177

MODY one sentence description

Answer 177

Monogenetic cause of diabetes (with variable penetrance) due to genetic defect in beta cell function

Question 178

Hepatitis B infection phases

Answer 178

1. Immune tolerance - High DNA, high HBe antigen 2. Immune clearance - Falling DNA, +/- falling HBe antigen, rising AST 3. Immune control - Anti-HBe +, low DNA, normal LFTs 4. Immune escape - Rising DNA, rising LFTs, HBe still -ve

Question 179

When to treat hep B: (2)

Answer 179

Liver injury and DNA > 20,000 (and +ve E antigen) OR DNA > 2,000 and negative HBe antigen (i.e. immune escape phase)

Question 180

Contraindications for interferon therapy (4)

Answer 180

Thyroid disease Psychosis Child-Pugh B/C (can trigger decomp) Autoimmune disease

Question 181

Tenofovir complications (2,1)

Answer 181

Low BMD Fanconi syndrome / renal impairment (But safe in pregnancy)

Question 182

Type of adenoma that secretes mucous and can result in diarrhoea with a rare ALKALOSIS

Answer 182

Villous adenoma

Question 183

Cardiac action potential steps

Answer 183

``` 0 (depol) - rapid Na influx 1 (notch) - K+ efflux 2 (plateau) - Ca++ influx, K+ efflux 3 (descent) - K+ efflux 4 (resting) - K+ influx (Na/K ATPase) ```

Question 184

RANZCOG cutoffs for GDM after 2h OGTT

Answer 184

Fasting >5.1 - 6.9 (>7 = diabetes) 1h > 10 2h > 8.5 - 11 (>11.1 = diabetes) (Different to NZ screening guide) Note: Aus and WHO recommend one-step test with 75g OGTT (not 50g test as in NZ)

Question 185

Osmolar gap formula

Answer 185

Osmolar gap = Osmolality (measured) – Osmolarity (calculated) Normal <10 If high, implies presence of an abnormal solute present in significant amounts e.g. methanol, ethylene glycol

Question 186

Bullous pemphigoid pathophysiology

Answer 186

IgG against hemidesmosomal proteins BP 180 and BP 230 (deep) --> eosinophil infiltrate --> dermal / epidermal splitting

Question 187

Pemphigus vulgaris pathophysiology

Answer 187

IgG against desmoglein 1 & 3 --> loss of cell adhesion in epidermis (superficial) --> easily rupturing blisters

Question 188

Bortezomib / carfilzomib mechanism / use

Answer 188

26s proteosome inhibitor (--> build-up of intracellular M-protein --> apoptosis) Myeloma induction (along with lenalidomide and dexamethasone)

Question 189

Daratumumab mechanism (and 1x laboratory complication)

Answer 189

mAb to CD38 on multiple myeloma cells (used in relapsed disease) (Can cause pan-agglutination on antibody screen when looking for a cross-match, i.e. pan-reactive red cell antibody)

Question 190

Chromosome translocation: follicular lymphoma / (some) DLBCL

Answer 190

t(14;18) --> BCL-2 | BCL-2 inhibits apoptosis

Question 191

Chromosome translocation: CML

Answer 191

t(9;22)

Question 192

Chromosome translocation: Burkitt lymphoma

Answer 192

t(8;14) --> Myc

Question 193

Disease with 'starry sky' appearance on node biopsy

Answer 193

Burkitt Lymphoma

Question 194

AVRT - current directions and associated ECG appearance

Answer 194

Orthodromic - narrow complex | Antidromic - wide complex (due to LV being activated first)

Question 195

SVT / IV adenosine special scenarios (3)

Answer 195

1. Contraindicated in asthma 2. Need higher dose if on theophylline 3. Need lower dose if on dipyridamole

Question 196

Typical vs atypical AVNRT

Answer 196

Typical: slow-->fast conduction, short R-P interval 'Short and slow' Opposite in atypical AVNRT

Question 197

R-factor: - Formula - Interpretation

Answer 197

ALT / ULN : ALP / ULN ``` <2 = cholestatic --> US >5 = hepatocellular --> liver screen 2-5 = mixed --> biopsy ```

Question 198

Effect of trimethoprim on potassium

Answer 198

Can increase K+ (usually in AIDS patients) due to inhibition of ENaC

Question 199

Perhexiline prescribing considerations

Answer 199

7-10% of caucasians are slow metabolisers due to deficient CYP2D6 - check metabolite level 3/7 after starting; if no peak, change to 100mg per WEEK dosing. Can also cause hypoglycaemia

Question 200

Beta-blocker with additional effect of potentiating the vasodilatory effect of NO

Answer 200

Nebivolol

Question 201

Beta-blockers that have low lipid solubility (don't cause CNS effects)

Answer 201

Atenolol Nadolol Celiprolol Sotalol

Question 202

Drugs to avoid in WPW + AF (pre-excited AF)

Answer 202

AV node blockers e.g. CCBs, BBs, adenosine

Question 203

One MET 4 METs 10 METs

Answer 203

Resting metabolic demands Climbing 2x flights of stairs Strenuous sports

Question 204

Hallmark finding of ASD on exam:

Answer 204

Wide and FIXED splitting of S2

Question 205

Mutation in familial pulmonary HTN

Answer 205

Bone morphogenetic protein receptor type 2 (BMPR2) (in the TGF-beta family) (Also often found in 10% of 'sporadic PAH')

Question 206

Role of hypocretin/orexin

Answer 206

A wakefulness promoting protein, deficient in narcolepsy. Coded by orexin gene.

Question 207

FEV1 (volume) cut-off for lobectomy

Answer 207

>1.5L usually used

Question 208

Implication of blood in pleural fluid (in context of cencer)

Answer 208

Pleural invasion - hence also not curative via surgery

Question 209

Mining exposure that increases TB risk

Answer 209

Silica (via impairment of macrophage function)

Question 210

Main risk factors for chronic lung allograft dysfunction (2)

Answer 210

Acute cellular rejection Lymphocytic bronchiolitis These cause bronchiolitis obliterans syndrome May be contributed to by silent aspiration

Question 211

Idiopathic pulmonary fibrosis histology findings (1,3)

Answer 211

Pattern = usual interstitial pneumonitis (UIP) Minimal inflammatory round cell infiltrate Widening of alveolar septa Fibrosis / fibroblastic foci

Question 212

Bronchoalveolar lavage finding in pulmonary alveolar proteinosis (2)

Answer 212

Grossly turbid exudates Periodic acid-Schiff (PAS)-positive material (Disease due to abnormal accumulation of surfactant in alveoli and impaired GM-CSF function)

Question 213

HRCT finding in pulmonary alveolar proteinosis

Answer 213

'Crazy paving'

Question 214

What does faecal immunochemical test bind to?

Answer 214

Globin part of Hb More sensitive/specific than gFOBT Doesn't detect upper GI blood, but does react with lower GI bleed (commonly haemorrhoids or diverticular bleed)

Question 215

'Interface hepatitis' or bridging necrosis on histology refers to:

Answer 215

Autoimmune hepatitis (See plasma cells, rather than lymphocytes (as in chronic hep)). (Can also see 'piecemeal' necrosis)

Question 216

Immune-mediated destruction of intrahepatic bile ducts on histology refers to:

Answer 216

PBC

Question 217

Deficiency in wet/dry beriberi

Answer 217

Thiamine (B1)

Question 218

NAFLD weight loss effects: 3-5% 10%

Answer 218

Improves steatosis | Improves necro-inflammation

Question 219

Plummer-Vinson syndrome

Answer 219

Iron deficiency and (keratinised) oesophageal webs

Question 220

Periodic acid-Schiff-stained macrophages on small bowel biopsy indicates...

Answer 220

Whipple disease (Tropheryma whipplei)

Question 221

Reason for low B12 but high folate in small intestinal bacterial overgrowth

Answer 221

Bacteria consume B12, but produce Folate

Question 222

Foot process effacement is seen in...

Answer 222

Minimal change disease

Question 223

Where in renal tract does ANP act to reduce sodium reabsorption?

Answer 223

Inner medullary collecting ducts

Question 224

Which part of the tubule is most often involved in drug secretion?

Answer 224

Proximal tubule

Question 225

Effect of FGF23 on phosphate

Answer 225

Lowers levels by inhibiting reabsorption in PCT

Question 226

Hb Target in CKD

Answer 226

100-115 Higer levels have risk of: - Stroke - Thrombosis / VTE - HTN - Headache - CHF

Question 227

What causes dialysis-related amyloidosis?

Answer 227

Inability clear (or dialyse) beta-2 microglobulin which then deposits. Most common manifestations of DRA are carpal tunnel syndrome and shoulder pain. Also see cystic lesions on end of long bones.

Question 228

Risk factors for aHUS (3)

Answer 228

Deficiency in factors H and I, Low thrombomodulin Activators of factors B and C3

Question 229

Complication of gadolinium in CKD

Answer 229

Nephrogenic systemic fibrosis (hence may need haemodialysis after MRI) (See lots of CD-34 fibrocytes)

Question 230

Post-transplant lymphoproliferative disorder: - Main risk factor - Most common transplant affected - Management

Answer 230

EBV mismatch Heart-lung or bowel (both 10-20%) Reduce immunosuppression

Question 231

Which glomerulonephritis is most likely to rapidly recur in a renal allograft?

Answer 231

Primary FSGS

Question 232

Type of glomerulonephritis associated with highest VTE risk.

Answer 232

Idiopathic membranous nephropathy

Question 233

What cell does PTH target in bone?

Answer 233

Osteoblast (which can then lead to osteoclast activation if constant PTH stimulation is present, but not intermittent PTH presence)

Question 234

Effects of GLP-1 (4)

Answer 234

Stimulates insulin Inhibits glucagon Inhibits gastric emptying Reduces appetite

Question 235

Eyebrow-related sign in hypothyroidism

Answer 235

Loss of the outer 1/3 of the eyebrow

Question 236

Does neurofibromatosis type 1 or type 2 typically present with vestibular schwannoma?

Answer 236

Type 2 (due to Merlin protein, AKA schwannomin), defect)

Question 237

Gene / protein for Tay-Sachs disease

Answer 237

``` HEXA gene Hexosaminidase A (usually breaks down phospholipids / gangliosides) ```

Question 238

Areas targeted by deep brain stimulation

Answer 238

Subthalamic nucleus | Internal segment of globus pallidus

Question 239

Symptoms that respond to deep brain stimulation (4)

Answer 239

Levodopa-responsive Sx, Tremor, On-off fluctuations, Dyskinesia

Question 240

Alpha synucleinopathies (3)

Answer 240

Parkinson's Lewy body dementia MSA

Question 241

Management of myasthenic crisis

Answer 241

Plasmapheresis or IVIg Regular bedside checks of ventilation Oral prednisone

Question 242

On nerve conduction study, prolonged/dispersed F-wave latency indicates...

Answer 242

Demyelinating disease

Question 243

Examples of non-ergot dopamine agonists

Answer 243

Pramipexole, ropinorole | Risk of impulse control disorders

Question 244

Neutrophil antigens that can cause false-positive pANCA result on indirect immunofluoroscopy (3)

Answer 244

Bactericidal permeability inhibitor (BPI) Cathepsin G Lactoferrin

Question 245

'Shared epitope' predisposing to RA

Answer 245

Common amino acid motif: QKRAA Usually found in HLA-DRB1, but sometimes in other alleles

Question 246

Wide mouth diverticulae are pathognomonic for...

Answer 246

Scleroderma

Question 247

Polyarteritis nodosa: what sized vessels are affected?

Answer 247

Medium (muscular) and small arteries (but no glomerulonephritis)

Question 248

Polyarteritis nodosa: biopsy findings

Answer 248

Pan-mural necrosis, prediliction for bifurcations. Can lead to aneurysms/stenosis of arteries.

Question 249

True/false: Polyarteritis nodosa affects the lungs?

Answer 249

False: affects all organ systems other than lungs Suspect in systemic vasculitis, ANCA-negative, orchitis and abnormal renal angiography

Question 250

Factors associated with a GOOD prognosis for RA (3)

Answer 250

Acute onset Restriction to small number of large joints Sero-negative

Question 251

In patient with RA presenting with shooting pains / hyper-reflexia / hypertonia, consider...

Answer 251

Atlantoaxial subluxation (due to erosion/stretching of the transverse ligament) (Often worsened by forward flexion of neck, which has anaesthetic implications)

Question 252

Most common type of lymphoma associated with Sjogren's

Answer 252

MALToma

Question 253

True/False: Alopecia is included in diagnostic criteria for SLE.

Answer 253

False

Question 254

Sunburn damage mechanisms: - UVB - UVA

Answer 254

UV B causes radiation to epidermis - the main cause of direct sunburn - SPF is based on UV B blocking ability UV A causes free radical generation (and can penetrate below the dermis)

Question 255

Key features of fixed drug reaction (2)

Answer 255

Residual hyperpigmentation Recurrence at previously-affected sites

Question 256

Biopsy showing panniculitis and inflammation of septa in subcutaneous fat is suggestive of...

Answer 256

Erythema nodosum

Question 257

Role of p53 / where it acts in cycle

Answer 257

Tumour suppressor via arrest of cell cycle at G1 (to allow DNA repair before progressing) and stimulation of apoptosis. I.e. stops entry into S-phase. (Note, can have elevated levels of mutated p53 in cancer cells due to slow turnover)

Question 258

True/False: Oestrogen receptor positivity is a favourable prognostic factor for breast cancer?

Answer 258

True | While HER2+ is negtive factor

Question 259

Deficiency in nucleotide excision repair confers increased sensitivity to which type of chemo agents?

Answer 259

Platinum (due to reduced capacity to repair interstrand cross-links)

Question 260

How does nucleotide excision repair work?

Answer 260

Removes helix-distorting adducts on DNA, e.g. those caused by UV or tobacco smoke

Question 261

Main side effects of bevacizumab (2)

Answer 261

Hypertension (VEGF inhibitor) (Can be managed with standard BP Rx) Poor wound healing

Question 262

When to give adjuvant chemo in breast cancer

Answer 262

Node positive Or tumour >0.5cm (Or other adverse prognostic features e.g. young age, negative hormone receptor status, high-grade tumour)

Question 263

When to give RTx in breast cancer

Answer 263

All breast-conserving surgery Or if tumour >5cm / node positive

Question 264

Breast screening for women with BRCA mutation

Answer 264

Should start age 25-30, and may include MRI in addition to mammography

Question 265

Cetuximab MoA

Answer 265

mAb to EGFR | Only works in wild-type KRAS, i.e. won't work if there is a KRAS mutation

Question 266

Erlotinib mechanism

Answer 266

TKI targeting EGFR (used in NSCLC and pancreatic cancer)

Question 267

Risk group for EGFR-positive lung cancer (4 features)

Answer 267

Asian Never smoker Women Adenocarcinoma (cf squamous) (Also unlikely to be effective if there is a KRAS mutation)

Question 268

Main side-effect of erlotinib

Answer 268

Acneiform rash (face/neck) | Correlation between severity of skin reaction and survival!

Question 269

Sunitinib MoA

Answer 269

VEGF inhibitor (as well as PDGFR B, FLT-3 and c_KIT) | Used in RCC

Question 270

Intracellular bacterial pathogens (5)

Answer 270

``` Listeria Mycobacterium Brucella Rickettsia Chlamydia ```

Question 271

Name the only Gram-positive bacterium to produce endotoxins

Answer 271

Listeria monocytogenes

Question 272

What cell does JV virus affect in the brain to cause PML?

Answer 272

Oligodendrocyte

Question 273

What does CD34 staining correspond to?

Answer 273

Haematopoietic progenitor cells

Question 274

Mechanism of Cholera toxin

Answer 274

Persistent activation of adenylate cyclase --> increase cAMP --> increased chloride secretion / decreased sodium absorption --> secretory diarrhoea

Question 275

Aflatoxin associations

Answer 275

Mycotoxin produced by Aspergillus, associated with: - HCC - p53 mutation

Question 276

PCR marker for dengue

Answer 276

NS1 protein

Question 277

HAART drug most responsible for lipodystrophy

Answer 277

Zidovudine

Question 278

Treatment for Strongyloides

Answer 278

Ivermectin (Consider Dx in patients with enteric bacterial pathogen with pulmonary/GI system involvement, particularly if co-existent immunosuppression or HTLV-1 present)

Question 279

How does G-CSF facilitate collection of stem cells for transplantation?

Answer 279

Activates neutrophils --> Release MMPs --> mobilises stem cells from marrow into periphery --> collected

Question 280

How does thrombopoietin (TPO) regulate platelet levels?

Answer 280

Via effect on megakaryocytes (promotes survival and differentiation). (Binds to TPO receptor in bone marrow, with higher free TPO levels being present if there are low peripheral platelets present to bind it)

Question 281

Roles of vWF (2)

Answer 281

Binds F VIII, reducing breakdown (increases T1/2 5-fold) Helps platelets bind to exposed collagen in blood vessels (via GP1b)

Question 282

Which factor activates protrhombin?

Answer 282

Factor X (in complex with factor V) (the prothrombinase complex) F X is inactivated by protein-Z protease

Question 283

Role of prostacyclin

Answer 283

Inhibits platelet activation. | Also acts as vasodilator

Question 284

Gram positive rods

Answer 284

``` Acinetobater Bacillus Clostridium Diphtheriae Listeria ```

Question 285

Lesion of angular gyrus causes...

Answer 285

Gerstman syndrome

Question 286

'Onion skin' fibrosis on liver biopsy refers to...

Answer 286

PSC | Periductal fibrosis, intra- and extra-hepatic ducts

Question 287

What cell releases surfactant?

Answer 287

Type 2 pneumocytes (main component = DPPC)

Question 288

What is strongest -ve predictor for CLL | Medication to use if this is present.

Answer 288

Del 17p Use ibrutinib or venetoclax if this mutation (or p53 mutation) is present

Question 289

What is the mechanism of fomepizole

Answer 289

Competitive inhibitor of alcohol dehydrogenase (used in ethylene glycol and methanol poisoning)

Question 290

What is found in Brodman's area 22?

Answer 290

Wernicke's area

Question 291

At what level of factor 8 deficiency does APTT become prolonged?

Answer 291

<45%

Question 292

APTT mixing studies that initially correct but then prolong again after 1-2h incubation due to...

Answer 292

Weak autoantibodies to factor 8

Question 293

CLL cell surface CD markers (4)

Answer 293

CD 5 (aberrant, normally on T cells) + CD 19 + CD20 + CD23

Question 294

Hereditary spherocytosis; affected proteins (3)

Answer 294

Ankyrin Spectrin Band 3 red cell protein (Usually AD inheritance)

Question 295

Key lineage to distinguish between MDS and aplastic anaemia

Answer 295

Megakaryocytes

Question 296

Condition that aplastic anaemia overlaps with nearly 50% of the time

Answer 296

PNH

Question 297

Which type of cryoglobulinaemia is hyperviscosity syndrome associated with?

Answer 297

Type I cryoglobulinaemia (Which is associated with Waldenstrom's, myeloma and CLL) (Sx usually at viscosity > 4.0 centipoise)

Question 298

Main factors guiding dosage of iron-chelating therapy (3)

Answer 298

Presence of cardiac iron overload Rate of transfusional iron Body iron burden (Best measured by periodic MRI and ferritin)

Question 299

What is Evan syndrome

Answer 299

Autoimmune haemolytic anaemia with ITP seen in pregnancy

Question 300

Gene mutation causing PNH

Answer 300

Phosphatidylinositol glycan class A (PIGA) This is required for synthesis of GPI-anchored proteins including CD55 and CD59. CD 55/59 are complement regulators. If deficient, excess complement-mediated intravascular haemolysis of affected cells occurs

Question 301

Main cause of death in PNH

Answer 301

Thrombosis e.g. abdominal or cerebral veins

Question 302

Main presentations of GvHD (3)

Answer 302

Rash, GI tract, liver

Question 303

Cell marker for plasma cells

Answer 303

CD138

Question 304

Risk factor for warfarin-induced skin necrosis

Answer 304

Protein C deficiency (due to exaggeration of initial hypercoagulable state)

Question 305

Malignancies associated with EBV (3)

Answer 305

Nasal NK cell lymphoma Burkitt's lymphoma T-cell lymphoma

Question 306

Common complication / differential in Sickle Cell disease

Answer 306

Salmonella osteomyelitis (which may be masked / mimicked during vaso-occlusive crises)

Question 307

Key features of Autoimmune polyendocrine syndrome type I

Answer 307

2 of 3 of: - Mucocutaneous candidiasis - Hypo-PTH - Addison disease Likely due to AIRE mutation Predisposed to many other AI conditions

Question 308

Conditions associated with thymoma (2)

Answer 308

Myasthenia gravis Pure red cell aplasia (see anaemia / absent erythroblasts on BM but normal myeloid cells and megakaryocytes)

Question 309

Chromosome for HLA

Answer 309

Ch 6

Question 310

Which HLA encodes MHC-II

Answer 310

HLA-D | And expresses slightly longer peptides 15-25 amino acids in length

Question 311

Which HLA encodes MHC-I

Answer 311

HLA-A, -B and -C | And expresses shorter peptides, 8-10 amino acids

Question 312

Which TLR is activated by lipopolysaccharide (LPS)?

Answer 312

TLR4 | LPS is the 'prototypical' PAMP

Question 313

Difference between IgA1 and IgA2

Answer 313

Their heavy chains

Question 314

Main location for: - IgA1 - IgA2

Answer 314

Airways and serum Colon (Similar levels in small intestine)

Question 315

NK cell surface markers

Answer 315

CD56 + | CD3 -

Question 316

Mechanism of immunotherapy

Answer 316

Alters T-cell reactivity to antigen, causing reduction in cytokine release (no longer thought to be due to reduction in IgE or from induction of 'blocking IgG Abs

Question 317

Macrophage-released cytokines

Answer 317

IL-1 (responsible for stimulating hypothalamus causing fever) TNF

Question 318

Basiliximab - Use - Target

Answer 318

Immune suppression induction Targets CD25 (part of the IL-2 receptor) (Can also be used during periods of acute rejection)

Question 319

What does icatibant do?

Answer 319

Bradykinin antagonist, used in hereditary angioedema

Question 320

True / False: tryptase is more useful in venom- or medication-related anaphylaxis that food-related anaphylaxis.

Answer 320

True

Question 321

Type of tryptase found after mast cell degranulation

Answer 321

Beta-tryptase (the mature form) Usual circulating tryptases are pro-alpha and pro-beta tryptase

Question 322

Tests in hereditary angioedema (2)

Answer 322

Low C1inh level or function Low C4 (due to persistent breakdown by C1 esterase, which isn't being inhibited)

Question 323

What measurement is used to calculate / compare a drug's bioavailability?

Answer 323

Area under the curve

Question 324

True / False: the dose of a drug affects its bioavailability.

Answer 324

False

Question 325

Key pharmacodynamic property that guides efficacy of beta-lactams and lincosamides

Answer 325

Time the concentration is above the MIC (T > MIC) I.e. time-dependent killing

Question 326

Key pharmacodynamic property that guides efficacy of aminoglycosides and fluoroquinolones

Answer 326

Ratio of area under the curve over 24-h dosing (AUC0–24) to minimum inhibitory concentration (MIC); AND the ratio of maximal concentration (Cmax) to MIC I.e. concentration-dependent killing Related to volume of distribution and clearance

Question 327

Key pharmacodynamic property that guides efficacy of vancomycin (glycopeptides)

Answer 327

Total body exposure to the antibiotic (ratio of AUC0-24 to MIC) (Also relevant to aminoglycosides) Related to VoD and clearance

Question 328

Is rifampicin a CYP inducer or inhibitor?

Answer 328

Inducer E.g. reduces antiepileptic levels

Question 329

Key CYP enzyme for warfarin (and examples of drugs that affect it)

Answer 329

CYP2C9 - which gets inhibited by: - Omeprazole - Metronidazole - Cimetidine - Amiodarone

Question 330

How does ivabridine work

Answer 330

Slows heart rate via action on If channel in sinus node

Question 331

What 2 parameters affect steady state drug concentration during an constant rate infusion?

Answer 331

Dose rate Clearance

Question 332

Caspofungin mechanism

Answer 332

Inhibits synthesis of fungal cell wall beta(1-3)-D-glucan component (Low oral bioavailability, must be given IV)

Question 333

Amphotericin B mechanism

Answer 333

Forms artificial pores in membranes by binding to ergosterol

Question 334

'Azole' mechanisms

Answer 334

Inhibit CYP system, thereby stopping synthesis of ergosterol from lanosterol

Question 335

Morphine receptors (4)

Answer 335

Mu - analgesia, euphoria, miosis, respiratory depression Kappa - analgesia, miosis, respiratory depression, sedation Sigma - dysphoria, hallucination, psychosis Delta - Unclear

Question 336

Mechanism of Tacrolimus

Answer 336

Binds FK506-binding protein --> inhibits calcineurin --> stops T-cell activation and IL-2 release

Question 337

Mechanism of Cyclosporin

Answer 337

Activates cyclophilin --> inhibits calcineurin --> stops T-cell activation / IL-2 release

Question 338

Which immunosuppressant causes cytokine-release syndrome

Answer 338

Anti-thymocyte globulin

Question 339

Immunosuppressant that causes pneumonitis

Answer 339

Sirolimus (mTOR inhibitor, which stops cytokine signaling from activating the cell) Also binds FK506 (like tacrolimus but doesn't inhibit calcineurin).

Question 340

Role of tRNA

Answer 340

Small RNA chain that transfers a specific amino acid to a growing polypeptide chain during translation.

Question 341

What do microRNAs do?

Answer 341

Bind to complementary mRNA, reducing their expression or translation.

Question 342

Which DNA nucleotide is the target for methylation / inactivation?

Answer 342

Cytosine

Question 343

What is heteroplasmy?

Answer 343

The tendency for a mitochondrial mutation to be present in only a proportion of the cell's mitochondrial genome copies.

Question 344

Familial Adenomatous Polyposis: - Gene - Inheritance pattern - When to start screening - How frequently to sceen

Answer 344

APC (tumour suppressor) Autosomal dominant Sigmoidoscopy at age 12 Annually

Question 345

Scenario with peripheral neuropathy, angiokeratomas, corneal whirls and murmur/hypertrophy is likely to refer to...

Answer 345

Fabry disease (X-linked lysosomal storage disorder with accummulation of glycosphingolipid) (Can also see renal failure and clots in the brain)

Question 346

Diseases displaying anticipation (5)

Answer 346

``` Huntingtons Fragile X syndrome Spinocerebellar ataxia Myotonic dystrophy Friedreich ataxia ```

Question 347

Huntingtons allele and number of copies causing disease

Answer 347

CAG Normal <34 repeats Variable penetrance 35-39 Disease > 40 Early onset >60

Question 348

Acute intermittent porphyria: - Inheritance pattern - Enzyme affected - Accummulated products

Answer 348

Autosomal dominant Porphobilinogen deaminase ALA and PBG

Question 349

von Willebrand's disease inheritance: - Type 1 - Type 2 - Type 3

Answer 349

Autosomal dominant Autosomal dominant Autosomal recessive

Question 350

Disease with co-dominant inheritance pattern

Answer 350

Alpha-1-anti-trypsin deficiency

Question 351

Largest gene

Answer 351

Dystrophin | On x-chromosome

Question 352

Location of magnesium absorption

Answer 352

Small intestine (via passive uptake) | Uptake is dependent on Mg levels, rather than intake

Question 353

Role of magnesium in muscle contraction

Answer 353

Stimulates calcium reuptake by the sarcoplasmic reticulum

Question 354

Site of Magnesium reabsorption

Answer 354

Thick ascending limb of loop of Henle

Question 355

Mechanism of cocaine

Answer 355

Inhibits reuptake of: - Dopamine (euphoria) - Serotonin (confidence) - Norepinephrine (energy)

Question 356

Mechanism of ezetimibe

Answer 356

Inhibits transport of cholesterol across intestinal wall (increasing cellular LDL-R expression --> increased LDL uptake)

Question 357

Mechanism of fibrates

Answer 357

Activates PPAR --> alter lipoprotein synthesis and catabolism

Question 358

Gordon syndrome - Mechanism - Inheritance - Key features (4) - Treatment

Answer 358

WNK4 mutation --> increased sodium-chloride co-transporter expression ('opposite of Gittleman') Autosomal dominant HTN, hyper-K+, low renin, NORMAL aldosterone Thiazide diuretic

Question 359

Examples of drugs that inhibit CYP2D6 (3)

Answer 359

Fluoxetine, paroxetine, bupropion

Question 360

Drugs that rely on CYP2D6 for activation (2)

Answer 360

Codeine, | Tamoxifen

Question 361

Main ECG finding in hypothermia

Answer 361

J-waves (AKA Osbourne waves) | Other findings include absent P-waves, PR interval prolongation, prolonged QRS, prolonged QT

Question 362

Toxic metabolic product of methanol

Answer 362

Formaldehyde --> formic acid --> retinal / optic nerve damage

Question 363

Toxic metabolic product of ethylene glycol

Answer 363

Glycolic acid --> acidosis / calcium crystals --> AKI

Question 364

Which feature of Wernicke's encephalopathy is first to resolve with thiamine administration?

Answer 364

Ocular palsies (within hours)

Question 365

Smoking effect on clozapine / which CYP

Answer 365

Smoking induces CYP1A2 which increases clozapine clearance - may need up to double dose. (Reverse applies with CYP1A2 inhibitors e.g. theophylline, cipro)

Question 366

Which antiepileptic can cause encephalopathy secondary to high ammonia levels?

Answer 366

Valproate

Question 367

How to calculate Variance (stats)

Answer 367

Standard deviation squared

Question 368

Type I error

Answer 368

Null hypothesis is falsely rejected, i.e. a difference is found where it does not actually exist. Alpha (p-value)

Question 369

Type II error

Answer 369

Null hypothesis is falsely accepted, i.e. the study was unable to detect a difference that does actually exist Beta (1 - power)

Question 370

What statistical test to use to assess dependence between two variables (correlation)

Answer 370

Spearman's rank correlation

Question 371

Receptors affected by noradrenaline (3)

Answer 371

Alpha-1, alpha-2 and beta-1 (agonist for all) --> increased vasoconstriction and cardiac output Cf. adrenaline which also has a beta-2 effect, thereby causing vasodilatin Adverse effect via agonism of alpha 2 causing excessive vasoconstriction and organ hypoperfusion

Question 372

Ventilation strategy for ARDS

Answer 372

Low tidal volume ventilation +/- prone positioning NOT high frequency oscillatory ventilation

Question 373

Antidote for cyanide poisoning

Answer 373

Hydroxycobalamin

Question 374

Normal pulmonary artery wedge pressure (PAWP)

Answer 374

6-15 mmHg Estimates LA pressure (and LV end-diastolic pressure if no mitral disease)

Question 375

Main rhythm complications during pulmonary artery catheters (2)

Answer 375

New RBBB Ventricular or supraventricular tachycardia

Question 376

What is degludec?

Answer 376

Ultra-long-acting form of insulin (T1/2 = 25h) - i.e. longer than glargine (Due to multi-hexamer chains that slowly release monomers)

Question 377

Key organ for AL amyloidosis

Answer 377

Kidney (disrupted GBM) Also highest risk form of amyloid for heart disease

Question 378

Key organ for AA amyloidosis

Answer 378

Kidney - nephrotic syndrome | Also GI tract and thyroid. Spares heart

Question 379

Key organs for AF amyloidosis

Answer 379

Familial cardiomyopathy and familial neuropathy Key protein = transthyretin

Question 380

Tau-opathies (4)

Answer 380

PSP (no REM sleep disorder) Corticobasal degeneration Alzheimers Fronto-temporal dementia

Question 381

Joints affected in haemochromatosis arthritis

Answer 381

2nd and 3rd MCPs

Question 382

Dopamine agonism 'stages' (as an ICU drug)

Answer 382

Low dose --> DA receptors --> improves renal flow Medium --> beta-1 receptors --> increased cardiac output High --> alpha-1 receptors --> vasoconstriction

Question 383

Cause of 'loss of y descent' in JVP

Answer 383

Tamponade (equal pressures across chambers, so no rapid flow into RV) (y descent = atrial emptying) TAMponade = TAMpaX (i.e. absent y)

Question 384

Causes of 'rapid y descent' in JVP (3)

Answer 384

Constrictive pericarditis Restrictive cardiomyopathy Severe TR (Conditions that lead to rapid RV filling)

Question 385

Chromosome / protein for Presenilin 1 (PSEN1)

Answer 385

Chromosome 14 Increased alpha-beta plaques - most common cause of early-onset familial AD

Question 386

Chromosome for Presenilin 2

Answer 386

Ch 1

Question 387

Which tumour marker is increased by smoking?

Answer 387

CEA

Question 388

Receptor for substance P

Answer 388

Neurokinin 1 (antagonised by apripetant)

Question 389

Why do patients with diastolic heart failure, e.g. HOCM, deteriorate rapidly in AF?

Answer 389

High reliance on atrial contraction during late diastole to fill the ventricle.

Question 390

Chromosome for CFTR

Answer 390

Ch 7

Question 391

Most common cystic fibrosis mutation

Answer 391

Delta F508 | Phenotype II - CFTR is produced by abnormal trafficking of channel to Golgi

Question 392

A-a gradient formula

Answer 392

A-a = PA02 - PaO2 PAO2 = FiO2 x (Patm - H2O) - (pCO2 / 0.8) In normal setting, simplifies to 150 - 1.25(pCO2) (H2O = 47mmHg)

Question 393

True / False: H. pylori is a risk for GORD

Answer 393

False

Question 394

HIV type in which to use Maraviroc

Answer 394

Dominant CCR5-tropic virus

Question 395

Type of mutation most likely to disrupt production of a protein:

Answer 395

Nonsense mutation

Question 396

Features of fascicular ventricular tachycardia (3)

Answer 396

QRS 100-140ms RBBB pattern Short RS interval (Most common type of idiopathic VT from LV)

Question 397

True / False: G-CSF reduces risk of febrile neutropaenia.

Answer 397

True | also some evidence that it increases the future risk of AML

Question 398

Follow-up screening for normal colonoscopy:

Answer 398

FOBT 2 yearly or repeat scope at 10 years

Question 399

Follow-up screening for low risk colonoscopy e.g. 1-2 fully-resected polyps

Answer 399

Colonoscopy at 5 years

Question 400

Follow-up screening for high risk colonoscopy

Answer 400

Colonoscopy at 3 years

Question 401

Follow-up screening if poor bowel prep or incomplete resection of polyps

Answer 401

Colonoscopy in 1 year

Question 402

Anti-alcohol drug contraindicated in IHD

Answer 402

Disulfiram - alcohol + disulfiram reaction can cause cardiovascular instability

Question 403

Cause of S4 heart sound

Answer 403

Active filling with atrial contraction into a stiff ventricle

Question 404

Cause of S3 heart sound

Answer 404

Rapid flow deceleration / blood striking compliant LV / forceful mitral valve opening

Question 405

Antibodies in neuromyelitis optica

Answer 405

Anti-AQP4-IgG4

Question 406

Subcut morphine potency to oral morphine

Answer 406

Subcut is ~2.5x stronger than PO | Similar to IV

Question 407

True / False: OSA is well-associated with restless leg syndrome

Answer 407

False

Question 408

Best screening test for partner for beta-thalassaemia if considering pregnancy (and one person is known carrier)

Answer 408

Mean corpuscular volume <75 | If low, then go for genetics

Question 409

Mechanism of teriparatide

Answer 409

Chronically elevated PTH will deplete bone stores. However, intermittent exposure to PTH will activate osteoblasts more than osteoclasts. Thus, once-daily injections of teriparatide have a net effect of stimulating new bone formation leading to increased bone mineral density.

Question 410

Malignancies with extra high VTE risk

Answer 410

Stomach | Pancreas

Question 411

Suffix for DPP-4 inhibitors

Answer 411

'-gliptin' | Prevent breakdown of incretins GLP-1 and GIP

Question 412

Two most common causes of metabolic alkalosis

Answer 412

Gastric losses (vomiting or NG) Diuretics

Question 413

Narcolepsy HLA association

Answer 413

HLA-DQB1

Question 414

Salicylate poisoning acid-base disturbances (2)

Answer 414

Early: respiratory alkalosis Later: metabolic acidosis (Often see mixed acid-base picture of these two)

Question 415

Chromosome translocation: Mantle Cell Lymphoma / Myeloma

Answer 415

t(11;14) - good prognosis - cyclin D1 product | everything else bad in myeloma

Question 416

Investigation to consider in patient with MS with urinary incontinence

Answer 416

Ultrasound - to assess for extent of bladder emptying

Question 417

Basophilic stippling on blood film suggests (2)

Answer 417

Lead poisoning Thalassaemia

Question 418

Anti-GM1 antibodies associated with (2)

Answer 418

Multiple sclerosis | Multifocal motor neuropathy

Question 419

Anti-GQ1b antibodies associated with...

Answer 419

Miller-Fisher syndrome

Question 420

Cyclophosphamide / chlorambucil: - Class - Mechanism

Answer 420

Alkylating agents DNA cross-linking

Question 421

Doxorubicin, epirubicin: - Class - Mechanism - Key side-eeffect

Answer 421

Anthracyclines Intercalates DNA / Inhibits topoisomerase II --> no DNA/RNA synthesis Dose-dependent cardiomyopathy

Question 422

Paclitaxel, docitaxel: - Class - Mechanism - Key side-effect

Answer 422

Taxanes Overstabilises microtubules / no disaggregation (no mitosis) Neuropathy, hair loss

Question 423

Vincristine: - Class - Mechanism - Key side-effect

Answer 423

Vinca alkaloid Inhibits mitotic spindle formation / no aggregation (no mitosis) Neuropathy, hair loss

Question 424

Bleomycin: - Class - Mechanism - Key side-effect

Answer 424

Peptide 'antibiotic' DNA strand breakage Pneumonitis

Question 425

Cisplatin, oxaliplatin: - Class - Mechanism - Key side-effect

Answer 425

Platinum-based DNA cross-linking Neuropathy, hearing loss, nephrotoxic

Question 426

5-FU, AZA, MTX: - Class - Mechanism - Key side-effect

Answer 426

Anti-metabolites Inhibits purine/pyrimidine synthesis Hand-foot disease, mucositis

Question 427

Ironotecan, etoposide: - Class - Mechanism - Key side-effect

Answer 427

Topoisomerase 1 inhibitors Topoisomerase normally rejoins DNA during synthesis) D/V

Question 428

Cetuximab; key side-effect

Answer 428

Acneiform rash

Question 429

Lapatinib: - Mechanism - Use

Answer 429

Dual TKI (EGFR and HER2) Breast cancer

Question 430

Palbociclib, ribociclib: - Mechanism - Use

Answer 430

CDK4/6 inhibitors --> stops inactivation of tumour suppressor Rb --> reactivated Rb prevents progression of cell cycle past G1 HR+ breast cancer (along with aromatase inhibitors)

Question 431

Trastuzumab-emtansine mechanism

Answer 431

Antibody-drug (DM1) conjugate - used in breast cancer

Question 432

Olaparib: - Mechanism - Use

Answer 432

PARP inhibitor --> prevents DNA base excision repair --> cell accumulates damage / double-stranded breaks (due to deficient BRCA repair) --> apoptosis = concept of 'synthetic lethality' BRCA-mutated ovarian/peritoneal cancers

Question 433

Average COVID incubation period

Answer 433

4-5 days

Question 434

Key CT chest change in COVID (2)

Answer 434

Ground glass opacification (86%) +/- mixed consolidation (58%) Peripheral or lower distribution typically

Question 435

COVID virus type

Answer 435

Positive sense, single-stranded RNA virus

Question 436

Host receptor for COVID entry

Answer 436

ACE2 (which binds to 'spike' protein of virus) | same as for other SARS-causing viruses

Question 437

COVID ventilation strategy

Answer 437

Low tidal volume (<6ml/kg) with PEEP. If this fails, then try prone ventilation.

Question 438

Medications to consider in COVID patients: - If severe / not on O2 - If severe and on O2 - ICU patients

Answer 438

Remdesivir (RNA polymerase inhibitor) Low dose dexamethasone + remdesivir Dex, no remdesivir, +/- IL-6 inhibitors

Question 439

Axonal pathology effect on NCS amplitude

Answer 439

Lower amplitude - i.e. fewer axons excited with a given stimulus

Question 440

What sort of disease causes slowed velocity / increased latency on NCS?

Answer 440

Demyelinating or compression e.g. carpal tunnel

Question 441

What is 'temporal dispersion' on NCS

Answer 441

When the CMAP becomes 'spread out' due to patchy demyelination and signals arriving at different times to recording electrode

Question 442

Conditions that cause a conduction block on NCS (2)

Answer 442

Multifocal motor neuropathy | Severe demyelination

Question 443

What causes fibrillation / 'positive sharp waves' on EMG?

Answer 443

Denervation of muscle fibre (which then increases expression of ACh-R and becomes sensitive to 'background' ACh levels)

Question 444

What causes 'dive bomber' sound on EMG?

Answer 444

Myotonia

Question 445

EMG changes in neurogenic pathology: - Amplitude - Recruitment

Answer 445

Increased (one nerve now supplying many muscle fibres) Decreased

Question 446

EMG changes in myopathic pathology: - Amplitude - Recruitment

Answer 446

Reduced amplitude Increased / early recruitment - trying to get more muscle fibres involved

Question 447

What does burst suppression on EEG indicate?

Answer 447

Induced coma or post-cardiac arrest.

Question 448

Normal frequency of brain waves

Answer 448

8-12 Hz (alpha waves)

Question 449

Gene implicated in juvenile myoclonic epilepsy

Answer 449

ICK (found in 7%)

Question 450

Juvenile myoclonic epilepsy: - EEG findings - Medications

Answer 450

4-6 Hz spike or spike-wave discharges Valproate or lamotrigine

Question 451

Mechanism of levetiracetam

Answer 451

SV2A modulator

Question 452

Seizure type associated with post-ictal psychosis

Answer 452

Focal impaired awareness seizures , temporal lobe | see onset 1-3 days after cluster of seizures

Question 453

3Hz spike-wave discharges associated with...

Answer 453

Childhood/juvenile absence epilepsies

Question 454

What HLA allele to test for before starting carbamazepine?

Answer 454

HLA-B15:02

Question 455

Mutation in what allele has increased risk for statin-induced myopathy?

Answer 455

OATP1B1 (reduced hepatic clearance)

Question 456

What HLA allele increases SJS risk in people taking allopurinol?

Answer 456

HLA-58:01

Question 457

Lamotrigine mechanism

Answer 457

Glutamate antagonist

Question 458

Key features for type I (distal) RTA (3)

Answer 458

Urine pH >5.5 (despite acidosis) Hypocitraturia Hypercalciuria / stones

Question 459

Common causes of type II RTA / Fanconi syndrome (3)

Answer 459

Monoclonal light chain disease Medications (tenofovir, acetazolamide, topiramte) Sjogren's syndrome

Question 460

What is Kussmaul's sign / what does it demonstrate?

Answer 460

Absence of inspiratory decline in JVP Seen in constrictive pericarditis or restrictive cardiomyopathy, RV infarction, severe RV dysfunction e.g. large PE

Question 461

What disease shows LV-RV interdependence (i.e. SAI > 1.1)

Answer 461

Constrictive pericarditis | increase in RV pressure with inspiration, decreased LV pressure

Question 462

What happens to LV pressure during inspiration in restrictive cardiomyopathy?

Answer 462

No change | But RV pressure decreases

Question 463

What disease is associated with a pericardial knock / mechanism.

Answer 463

Constrictive pericarditis (stiff pericardium results in sudden arrest of ventricular filling) (Corresponds with y-descent)

Question 464

What does a Qp:Qs ratio of >1.5 indicate?

Answer 464

The presence of a shunt between pulmonary and systemic circulations (ratio should be 1.0)

Question 465

Calculating RV pressure | which ~ mean pulmonary artery pressure

Answer 465

Pressure (RV) = Pressure (RV-RA) + Pressure (RA) (RA pressure measured by looking at IVC) (In absence of RVOT obstruction, RV pressure = pulmonary artery systolic pressure

Question 466

Calculating pulmonary vascular resistance

Answer 466

(mPAP - LA) / Qp (Where LA is measured by the PCWP) (Where Qp ~ Cardiac output) (Where Qp ~ Qs if no intracardiac shunt) >3 woods units = pulmonary HTN

Question 467

Calculating systemic vascular resistance

Answer 467

(Ao - RA) / Qs I.e. change in pressure / flow. Where: Q ~ CO Ao ~ MAP

Question 468

Procedure of choice for rheumatic mitral stenosis

Answer 468

Percutaneous transvenous mitral commissurotomy (PTMC) AKA percutaneous mitral balloon valvotomy

Question 469

Management for methaemoglobinaemia - Acquired - Congenital

Answer 469

Methylene blue Ascorbic acid

Question 470

Reversible complications of haemochromatosis (2)

Answer 470

Cardiomyopathy Skin bronzing

Question 471

Irreversible complications of haemochromatosis

Answer 471

Arthritis Diabetes Hypogonadism Cirrhosis

Question 472

'Classic' salmonella features (3)

Answer 472

Relative bradycardia Pulse-temperature dissociation Rose spots on trunk

Question 473

Typhoid fever Rx (3)

Answer 473

Most: azithromycin OR ciprofloxacin OR ceftriaxone If acquired in Pakistan --> carbapenem Erradication with 4/52 cipro

Question 474

Most sensitive test for diagnosing typhoid fever

Answer 474

Bone marrow culture (~90%)

Question 475

Role of FGF23/Klotho and 2x mechanisms for this.

Answer 475

Lowers phosphate via 2x mechanisms: Promotes phosphate excretion at PCT Inhibits alpha-1-hydroxylase --> stops vit D activation --> stops phosphate absorption

Question 476

Key tumours of MEN1

Answer 476

Parathyroid (anterior) Pituitary (esp. prolactinoma) Pancreas (or other GI cancers e.g. Z.E. syndrome)

Question 477

MEN2a vs MEN2b

Answer 477

Both have medullary thyroid cancer Both have phaeochromocytoma Men2a also has hyper-PTH Men2b has more neuromas/gangliomas/more aggressive

Question 478

Calcitonin can be used to monitor for what type of cancer recurrence?

Answer 478

Thyroid medullary carcinoma

Question 479

Thyroglobulin can be used to monitor for what type of cancer recurrence?

Answer 479

Thyroid papillary or follicular. Note: need to also measure anti-thyroglobulin at the same time.

Question 480

CT brain signs of cerebral venous thrombosis (3)

Answer 480

Dense triangle sign Empty delta sign Cord sign

Question 481

Most common genes in familial motor neuron disease (2)

Answer 481

C9ORF72 - 40% | SOD1 - 15%

Question 482

Riluzole mechanism

Answer 482

Inhibits glutamate release / promotes reuptake --> reduced stimulation of glutamate receptors.

Question 483

Frequency of immunoglobulins in blood (most common to least common)

Answer 483

``` IgG IgA IgM IgD IgE ```

Question 484

Free vs total PSA in prostate cancer

Answer 484

In prostate cancer, there is a LOWER proportion of free PSA to total PSA (Larger fraction of PSA can escape proteolysis by 'leaking out' of malignant cells, thereby not forming 'free PSA')

Question 485

Types of von Willebrand's disease (3)

Answer 485

Type 1 - deficient production, but existing factors are normal (most responsive to DDAVP) Type 2 - non-functioning vWF Type 3 - deficiency in vWF

Question 486

What blood group is associated with low vWF levels?

Answer 486

Group O (30% lower)

Question 487

Haemophilia A severity levels

Answer 487

Based on factor activity levels: Severe - <1% - only see spontaneous bleeds in this category Moderate 1 - 5% Mild 5 - <40%

Question 488

Alpha-1-anti-trypsin - concept of 'gain of function' vs 'loss of function' toxicity

Answer 488

Loss - lung pathology due to loss of protection against elastase Gain - liver pathology due to accumulation in liver cells of unsecreted AAT protein

Question 489

Calculating RV systolic pressure using Bernoulli equation

Answer 489

RV pressure = 4(TR peak velocity)squared + RA pressure

Question 490

Management of pulmonary oedema in pregnancy due to mitral stenosis

Answer 490

Beta-blocker | Usually a high flow state, so by slowing HR --> improves (reduces) pressure gradient across valve

Question 491

'Hockey stick' deformity on ECHO indicates

Answer 491

Mitral stenosis

Question 492

Formula for valve area

Answer 492

A1 x V1 = A2 x V2

Question 493

What is low flow / low gradient stenosis?

Answer 493

In conditions with low flow, e.g. cardiomyopathy, severe hypertrophy, the ventricle can't generate enough pressure to open valve. Makes surface area look small ('severe') but is actually only moderate disease. Investigate with dobutamine test which should increase contractility and increase flow/gradient. If true 'anatomic' stenosis, then valve area shouldn't change.

Question 494

What is dimensionless index?

Answer 494

Ratio of LV outflow (V1) : velocity across valve (V2) | Ratio <0.25 = area <1/4 normal, i.e. severe

Question 495

'Fish mouth' valve is suggestive of...

Answer 495

Severe mitral stenosis in rheumatic heart disease (fusion of commisures)

Question 496

'Beads on a string' on MRCP/ERCP suggests...

Answer 496

PSC

Question 497

'Sausage-shaped' pancreas on CT suggests...

Answer 497

Autoimmune pancreatitis (a/w IgG4 disease) | Further supported by absence of surrounding inflammation or peripancreatic fluid

Question 498

Mechanism of mepolizumab

Answer 498

anti-IL-5

Question 499

Conditions in which pregnancy is 'contraindicated' / WHO risk group IV (7)

Answer 499

``` Pulmonary arterial hypertension LVEF <30% Previous peripartum cardiomyopathy with ANY residual dysfunction Severe MS or AS Marfans with aorta >45mm Aorta >50mm if bicuspid valve Native severe coarctation ```

Question 500

True / False: Teriparetide reduces hip fractures

Answer 500

False. But it does reduce vertebral and other non-vertebral fractures

Question 501

Mutation causing x-linked agammaglobulinaemia

Answer 501

Defect in Bruton's tyrosine kinase

Question 502

Frequency of colonoscopy in patients with PSC and UC

Answer 502

Annually

Question 503

True / False: High dose vitamin D supplementation can increase falls.

Answer 503

True

Question 504

bDMARDs that work in ank spond (2)

Answer 504

Anti-TNF | Anti-IL-17 (Secukinumab)

Question 505

Mechanism of hydroxyurea in sickle cell disease

Answer 505

Increases HbF production

Question 506

Listeria meningitis treatment option if patient is penicillin-allergic

Answer 506

IV cotrimoxazole

Question 507

Changes in types of valve disease in rheumatic fever with time

Answer 507

Initial MR is most common, then MS becomes more common in 30's due to fibrosis, then multivalvular in 50's.

Question 508

Medical options for pituitary Cushing's disease (2)

Answer 508

Cabergoline | Pasireotide

Question 509

Medical options for adrenal Cushing's syndrome (4)

Answer 509

Ketoconazole Metyrapone (inhibits 11-hydroxylase) Etomidate (emergency) Mitotane (irreversible)

Question 510

Australia pneumonia recommendations: - Mild - Moderate - Severe

Answer 510

Amoxicillin OR doxy Benpen + doxy/clarithromycin Ceftri + azithromycin

Question 511

Which anti-fungal typically has therapeutic drug monitoring applied during use?

Answer 511

Voriconazole

Question 512

What is released by D cells?

Answer 512

Somatostatin

Question 513

What is released by I cells?

Answer 513

Cholecystokinin

Question 514

B and T-cell deficient disorders ('WASH')

Answer 514

Wiskott-Aldrich syndrome Ataxic telangiectasia SCID Hyper IgM syndromes

Question 515

What are 'eosinophilic intracytoplasmic inclusions' more commonly known as?

Answer 515

Lewy Bodies (alpha synucleinopathy)

Question 516

What is the mechanism(s) of golimumab / certolizumab?

Answer 516

Anti-TNFa

Question 517

Osteophyte location in DISH spine x-ray

Answer 517

Anterior spine

Question 518

Hormone profile associated with BRCA1

Answer 518

Triple negative | Higher a/w ovarian cancer

Question 519

Hormone profile associated with BRCA2

Answer 519

HR+ (and PALB2 gene) | Higher a/w luminal / male breast cancer

Question 520

Causes of raised DLCO (5)

Answer 520

``` Asthma Pumonary haemorrhage L-to-R shunts Polycythaemia Hyperkinetic states e.g. exercise ```

Question 521

Causes of reduced DLCO (6)

Answer 521

``` 'FAPPLE': Fibrosis Anaemia Pneumonia PE Low cardiac output Emphysema ```

Question 522

Raised KCO with normal/reduced DLCO (3)

Answer 522

Pneumonectomy / lobectomy Scoliosis / kyphosis/ankylosis Neuromuscular weakness

Question 523

Causes for false-positive VDRL / RPR (Cardiolipin tests)

Answer 523

SLE, TB, Malaria, HIV, Leprosy, anti-phospholipid syndrome, pregnancy 'SomeTimes Mistakes Happen in LAParoscopy'

Question 524

Organism that is a contraindication for CF patients to get lung transplant.

Answer 524

Burkholderia cepacia - cepacia syndrome

Question 525

Which glomerulonephritis is associated with malignancies?

Answer 525

Membranous

Question 526

What disease is suggested by 'interface hepatitis' on histology

Answer 526

Autoimmune hepatitis | See piecemeal necrosis / bridging necrosis in severe disease

Question 527

Main cell types seen on liver biopsy for: - Autoimmune hepatitis - Chronic hep C - Alcoholic hepatitis

Answer 527

Plasma cells Lymphocytes Neutrophils

Question 528

What trinucleotide repeat is seen in myotonic dystrophy

Answer 528

CTG

Question 529

Treatment regimens: - TB - MAC

Answer 529

'RIPE' | 'RiCE' - rifampicin, clarithromycin, ethambutol

Question 530

'Stork leg' is a sign/complication of what disease?

Answer 530

Charcot-Marie-Tooth disease | most common inherited peripheral neuropathy, predominantly motor Sx

Question 531

Spinal Muscular Atrophy inheritance pattern

Answer 531

Autosomal recessive

Question 532

Only valve that (normally) is bicuspid

Answer 532

Mitral

Question 533

Most common mutation causing resistance to EGFR tyrosine kinase inhibitors

Answer 533

T790M

Question 534

Causes of cyanotic heart disease (5)

Answer 534

``` "5 T's" Truncus arteriosus Tricuspid atresia Tetralogy of Fallot Total anomalous pulmonary vascular return Transposition of great arteries ``` (Other non-cyanotic conditions only cause cyanosis once the shunt reverses with Eisenmenger's)

Question 535

If lamivudine resistance develops, which subsequent antiviral has low long-term resistance rates?

Answer 535

Tenofovir | While entecavir has up to 50% resistance by 5 years

Question 536

CD surface marker for monocytes/macrophages

Answer 536

CD14

Question 537

True/False: Primary spontaneous PTX primarily occurs during exercise.

Answer 537

False

Question 538

'Storiform' pattern on biopsy is suggestive of what disease?

Answer 538

IgG4-related disease

Question 539

Inheritance pattern for fragile x syndrome

Answer 539

X-linked dominant

Question 540

Aortic stenosis: order of clinical findings by prognosis (best to worst) (3)

Answer 540

Angina - 5 year survival Syncope - 3 year survival Heart failure - 2 year survival

Question 541

What is platypnoea-orthodeoxia and a common cause of it?

Answer 541

Oxygenation better when lying flat Hepatopulmonary syndrome

Question 542

Effect/Outcome of: - Single nucleotide deletion on reading frame - Substitution of 1st or 2nd nucleotide in a codon - Substitution of 3rd nucleotide in a codon

Answer 542

Frameshift mutation Will change 1x amino acid No change to amino acid - AKA degeneracy

Question 543

Alzheimer's CSF levels, what happens to: - A-beta42 - Tau

Answer 543

Decreased | Increased

Question 544

True / False: beta-lactams have high PO bioavailability?

Answer 544

False

Question 545

Therapy for Cryptococcus neoformans or Cryptococcus gatii

Answer 545

Amphotericin B + Flucytosine | Gatii is found in Australia and affects immunocompetent hosts

Question 546

Direction of eye movement by: - Superior oblique - Inferior oblique

Answer 546

SO4 - 'SIN' - trochlear nerve - Superior oblique intorts (i.e. down and in) Inferior oblique normally goes up and in (hence '3 strikes (CN3) and you're down and out')

Question 547

Cells that make myelin: - In the CNS - In the PNS

Answer 547

Oligodendrocytes Schwann cells

Question 548

Spasticity vs rigidity (in terms of velocity)

Answer 548

Spasticity is velocity-dependent Rigidity is tense throughout entire range of passive movement

Question 549

Mechanism of p-glycoprotein pump

Answer 549

Acts as efflux pump, reducing the absorption of drugs across the intestinal wall, increasing excretion into bile, increasing renal secretion, preventing entry into CSF.

Question 550

True / false: in type 1 respiratory failure, INITIAL treatment with high flow oxygen has lower mortality than NIV

Answer 550

True | And associated with lower intubation rates, especially in ARDS

Question 551

What type of nodule is most likely to be malignant: solid vs part-solid vs ground glass nodule?

Answer 551

Part-solid

Question 552

Distinguishing features of type 2 narcolepsy vs type 1 (2)

Answer 552

Type 2 narcolepsy: - No cataplexy - High CSF hypocretin/orexin levels

Question 553

Main organ NOT involved in scleroderma

Answer 553

Brain | Essentially every other organ system affected, particularly skin, lung and GI tract

Question 554

On liver CT with contrast, what does a 'double target' sign or 'cluster sign' refer to?

Answer 554

Liver abscess

Question 555

BRCA-associated cancers (4)

Answer 555

``` 'BOPP': Breast Ovary Pancreas Prostate ```

Question 556

Which myositis antibody has the strongest association with malignancy?

Answer 556

Anti-TIF1y (AKA anti-p155)

Question 557

True / False: acromegaly causes hypercalcaemia

Answer 557

True

Question 558

Ustekinumab mechanism

Answer 558

Anti-IL-12 / IL-23

Question 559

Vedolizumab mechanism / use

Answer 559

Anti alpha-4-beta-7 integrin Gut-specific mAB used in moderate-severe UC. Safer in older patients at increased risk of infection or malignancy.

Question 560

True / false: the following molecular profile has a good risk profile in AML? NPM1 mutated / FLT3 wild-type

Answer 560

True I.e. a mutation in FLT3 has adverse prognostic implication

Question 561

In pregnancy, how is the minute ventilation increased?

Answer 561

Predominantly through an increased tidal volume

Question 562

"Florid duct lesions" refers to...

Answer 562

PBC (granulomatous process) Degeneration of interlobular and segmental bile ducts --> 'ductopenia'

Question 563

Agents that REDUCE the effect of warfarin (6)

Answer 563

``` 'CRASHA': Cholestyramine Rifampicin Anti-epileptics St John's wort High vit K foods Azathioprine ``` For the exam, unless in this list, assume that it otherwise increases anticoagulation.

Question 564

How to remove macroprolactin from blood assay

Answer 564

Pretreat with polyethylene glycol

Question 565

aFP and b-hCG is elevated in which category of testicular germ cell tumours?

Answer 565

Non-seminomatous germ cell tumours | which is also more likely to present as metastatic disease compared to seminomas

Question 566

Distinguishing between TSH-producing adenoma vs resistance to thyroid hormone as causes of secondary hyperthyroidism: which of the two entities will have a positive response to TRH stimulation?

Answer 566

Resistance to thyroid hormone syndrome | Which will also usually not have too high of a TSH level

Question 567

Class of antivirals not recommended in decompensated liver disease (CP B or C)

Answer 567

Protease inhibitors E.g. marivet

Question 568

POEMS procedure, for achalasia, is associated with what adverse effect (compared to traditional myotomy)?

Answer 568

Higher rates of GORD/oesophagitis

Question 569

Source of faecal calprotectin

Answer 569

Phagocytes (neutrophils and monocytes, calcium and zinc-binding protein) (While faecal lactoferrin tests for leucocytes)

Question 570

Which cells release Ghrelin?

Answer 570

P/D1 cells (most abundantly found in the gastric fundus)

Question 571

Commonest type of incontinence in older people

Answer 571

Urge incontinence (due to detrusor muscle overactivity)

Question 572

True / False: smoking is a significant risk factor for haemorrhagic stroke?

Answer 572

False

Question 573

'Smooth, diffuse meningeal thickening' on MRI is suggestive of what disease?

Answer 573

POEMS Syndrome

Question 574

Which component of the 'metabolic' syndrome is least associated with development of neuropathy?

Answer 574

Hypertension

Question 575

True / False: MRI findings in idiopathic intracranial hypertension are predictive of risk of long-term vision loss.

Answer 575

False. Key predictors of adverse outcome: - High grade papilloedema - Vision loss at presentation - Transient visual obscurations prior to presentation

Question 576

True / False: Transient global amnesia can have small findings on diffusion-weighted imaging.

Answer 576

True - usually seen after 12h

Question 577

Reduced CD157 / FLAER binding on flow cytometry is suggestive of which disease?

Answer 577

PNH

Question 578

When to add midostaurin in AML

Answer 578

If FLT3 mutation present (a membrane-bound tyrosine kinase that gets inhibited by midostaurin)

Question 579

Consolidation medication in AML if good prognostic features present.

Answer 579

Cytarabine | If poor prognosis, consider allogeneic BMT

Question 580

Ibrutinib: - Mechanism - Main side-effect

Answer 580

Bruton TK inhibitor - used in CLL, esp with del17p Bruising / bleeding

Question 581

Key side effects of venetoclax (2)

Answer 581

High risk of tumour lysis syndrome | Neutropaenia - common to use GCSF

Question 582

Components of Tetralogy of Fallot

Answer 582

``` 'PROV': Pulmonary stenosis RV hypertrophy Over-riding aorta VSD ```

Question 583

Cause of large V waves on JVP

Answer 583

Tricuspid regurgitation

Question 584

Common ECG change with TAVI

Answer 584

New LBBB seen in 20% due to proximity of valve to bundle of His - 40% resolve spontaneously

Question 585

Chemo agents that cause lots of nausea

Answer 585

Platinum agents Doxorubicin Alkylating agents

Question 586

Which territory MI has biggest risk for LV thrombus formation?

Answer 586

Anterior, especially if LV <30% or apical akinesis seen. | Rx = warfarin, NOT DOAC

Question 587

2x main complications of catheter ablation treatment for AF

Answer 587

Atrio-oesophageal fistula (chest pain, fever, neuro from septic emboli) - Posterior wall of LA is very close to oesophagus Pulmonary vein stenosis

Question 588

POTS diagnostic criteria

Answer 588

HR rising >30 from baseline on standing/tilt, or rising to >120bpm (without significant change in BP)

Question 589

True / false: food allergies can be effectively managed by desensitisation immunotherapy.

Answer 589

False | But very effective for stinging/biting insects

Question 590

Agents which reduce vertebral but not hip fracture (3)

Answer 590

Teriparetide SERMs Calcitonin (While bisphosphonates, denosumab and oestrogen/progesterone reduces both classes)

Question 591

What is efficacy vs effectiveness for a drug?

Answer 591

Efficacy = drug effect in ideal circumstances e.g. trial Effectiveness = how well drug performs in real world

Question 592

Which enzyme is affected by Lumacaftor/Ivacaftor

Answer 592

Massive induction of CYP3A4 - cannot give azoles

Question 593

BODE Index: - What is it? - Components ('BODE')

Answer 593

Scale for predicting COPD survival / hospitalisation. BMI Obstruction (FEV1) Dyspnoea (mMRC scale) Exercise capacity (6MWT)

Question 594

Between PKD1 and PKD2, which has worse prognosis for progressive renal disease?

Answer 594

PKD1 (associated with truncating mutations)

Question 595

Lupus nephritis: - Which classes to treat - Induction regimen

Answer 595

Class III, IV +/- V Steroids, MMF or cyclophosphamide (reassess at 6 months)

Question 596

True / False: In CKD, the goal for PTH is to have it in normal range.

Answer 596

False - goal is to have it less than 2-9x ULN If it is normal, there is a high risk of adynamic bone disease

Question 597

Side effects: - Tenofovir disoproxil fumarate - Tenofovir alafenamide

Answer 597

Renal impairment / Fanconi's + lower BMD Weight gain

Question 598

Key finding of MERINO trial

Answer 598

In ESBL sepsis (E. coli or Klebsiella resistant to ceftriaxone), carbapenems had better outcomes than PipTaz

Question 599

How to distinguish between 'probable' and 'possible' CVID

Answer 599

Both have reduced IgG and reduced vaccine response. Possible --> normal IgA/IgM Probable --> low IgA/IgM

Question 600

Drug that increases mucormycosis infection risk

Answer 600

Deferoxamine | See 'reverse halo' sign on CT - ground glass change surrounded by wall of necrosis

Question 601

True / False: Mucormycosis will not have positive galactomannan or 1,3-beta-glucan tests

Answer 601

True (need Dx via culture or histopathology)

Question 602

Which cytokines are affected in MSMD (Mendelian Susceptibility to Mycobacterial Diseases)

Answer 602

IFNy and IL-12 --> no Th1 response --> intracellular infections Prototypic event = lymphadenitis / systemic infection after BCG vaccine

Question 603

Key findings of TULIP2 trial (re: SLE)

Answer 603

Treatment with Anifrolumab (anti-IL-1-R) associated with better composite outcome at 52 weeks than placebo (And higher rates of herpes zoster)

Question 604

Key findings of PLEXIVAS trial (re: ANCA-associated vasculitis) (2)

Answer 604

Plasma exchange did NOT reduce death or ESRF. | Low-dose steroids was non-inferior to standard dose (and had fewer infections).

Question 605

What disease is anti-NT5C1a antibodies associated with?

Answer 605

Inclusion body myositis

Question 606

What disease and complication is anti-MDA5 antibody associated with?

Answer 606

Dermatomyositis complicated by interstitial lung disease

Question 607

What is the most common myositis-associated antibody?

Answer 607

Anti-Jo-1 | a subtype of aminoacyl-transfer RNA (tRNA) synthetase antibodies

Question 608

What disease is anti-Mi-2 antibodies associated with?

Answer 608

Dermatomyositis | May also indicate group at low risk of malignancy

Question 609

Bronchoprovocation testing: what is the difference between methacholine and mannitol?

Answer 609

Methacholine = ‘direct’ stimulus, more sensitive for pick-up asthma Mannitol = ‘indirect’ stimulus, more specific. Also better association with exercise.

Question 610

What will excess Biotin levels do to troponin or TSH results?

Answer 610

False decrease (by occupying streptavidin binding sites instead of the anti-TSH Ab)

Question 611

Type 1 Amiodarone Induced Thyrotoxicosis: Mechanism Treatment

Answer 611

Excessive thyroid hormone synthesis due to excess iodine substrate (Jod-Basedow effect) Carbimazole, radio-ioidine, surgery

Question 612

Type 2 Amiodarone Induced Thyrotoxicosis: Mechanism Treatment

Answer 612

Direct toxic effect on follicular cells (destructive thyroiditis - Type 2 AIT) → excess release of preformed T4/3 Steroids

Question 613

What is the Wolff-Chaikoff effect?

Answer 613

Due to excess iodine load, there is inhibition of thyroid hormone synthesis (People with underlying thyroid disease may not be able to overcome this)

Question 614

Mechanisms of amiodarone-induced hypothyroidism (2)

Answer 614

Reduced conversion of T4 → T3 Wolff-Chaikoff effect

Question 615

Angelman syndrome genetic pathophysiology

Answer 615

Maternal UBE3A (ch 15) gene is mutated while the paternal copy is imprinted (Or from uniparental disomy)

Question 616

Prader-Willi syndrome genetic pathophysiology

Answer 616

Deletion of paternal gene cluster on Ch 15 while maternal copies are imprinted

Question 617

What cutaneous side-effect is seen with EGFR inhibitors? (And name some examples of drugs)

Answer 617

Acneiform rash E.g. erlotinib, gefitinib, cetuximab

Question 618

What is Osimertinib used for?

Answer 618

EGFR+ positive lung adenocarcinoma if progression on erlotinib or gefitinib demonstrated AND T790M mutation present

Question 619

Drugs to use for ALK-positive lung adenocarcinoma (2)

Answer 619

Alectinib (first line) or crizotinib

Question 620

What is the most common cause of secondary IgA nephropathy?

Answer 620

Alcoholic cirrhosis

Question 621

True / False: there is no data demonstrating a benefit for statin therapy in CKD patients on dialysis.

Answer 621

True (except possibly in subgroups with very high LDL)

Question 622

What cell is CTLA-4 on? | What does CTLA-4 bind to?

Answer 622

``` T cells (constitutively active on Tregs, induced on Th) B7 (CD80/86) - binds with higher affinity than CD28, thereby outcompeting it → inhibition ```

Question 623

What is CD28? | Which cell expresses it, what does it bind to?

Answer 623

CD28 = co-stimulatory molecule on T cells | Binds to B7 (CD80/86)

Question 624

True / false: the benefit of SGLT-2i’s in heart failure death/hospitalisation is only seen in patients with diabetes.

Answer 624

False | EMPEROR-reduced trial

Question 625

Bortezomib: Mechanism Key side effect

Answer 625

26S Proteasome inhibitor → build up of cellular materials → apoptosis (used in multiple myeloma) Painful peripheral neuropathy (30%)

Question 626

Marfan’s inheritance pattern

Answer 626

Autosomal dominant (FBN1 gene)

Question 627

TGFBR1 / TGFBR2 mutations are associated with which condition?

Answer 627

Loey-Dietz syndrome

Question 628

What does secukinumab target? | Indication

Answer 628

IL-17 | Associated with WORSE rates of IBD and no effect in RA - only used in psoriatic arthritis

Question 629

Fracture risk cut-offs for starting osteoporosis treatment according to Aus guidelines: Hip fracture risk Any fracture

Answer 629

If 10 year risk for hip fracture is >3%, or risk for any fracture >20% → start anti-osteoporosis medication

Question 630

Which BRCA has the greatest increased risk of male breast cancer?

Answer 630

BRCA2

Question 631

Which BRCA has the greatest increased risk of ovarian cancer?

Answer 631

BRCA1

Question 632

Gain of function / loss of function of SCN5A gene causes which syndromes respectively?

Answer 632

Gain - long QT syndrome | Loss - Brugada syndrome

Question 633

What type of seizure are anti-LGI-1 antibodies associated with (autoimmune encephalitis Abs)

Answer 633

Facio-brachial dystonic seizures (looks like face / shoulder / arm myoclonus)

Question 634

True / false: female carriers of muscular dystrophy mutations can have muscle weakness.

Answer 634

True - often mild/moderate.

Question 635

Most common cause of primary nephrotic syndrome in adults?

Answer 635

Membranous nephropathy

Question 636

What is the best technique for measuring core temperature (hierarchy)?

Answer 636

Central venous temp > oesophageal > bladder > rectal > tympanic

Question 637

Best drug to manage depression/hallucination in Alzheimer's

Answer 637

Citalopram

Question 638

Key ECG lead in RV ischaemia

Answer 638

V4R

Question 639

Empiric Rx for native valve endocarditis (3)

Answer 639

Benzylpenicillin + Flucloxacillin + Gentamicin | If prosthetic valve, replace benpen with vancomycin

Question 640

Treatment for cytokine release syndrome

Answer 640

Tocilizumab

Question 641

Diagnosis of Ross River Virus (3x modalities)

Answer 641

Haemagglutinin inhibition (HI) antibody test - 4-fold rise = diagnostic - titre >1:1280 = recent infection ELISA Virus-specific IgM (persist for months) - titres can reflect acute infection

Question 642

What lymphoma is associated with 'pan B cell markers'?

Answer 642

Diffuse Large B-cell Lymphoma CD19 + CD20 + CD22 + CD79a Rx with ABC, worse outcomes with R-CHOP

Question 643

Components of R-CHOP

Answer 643

``` Rituximab Cyclophosphamide Doxorubicin Vincristine Prednisone ```

Question 644

Mechanism of Brentuximab (and uses (2))

Answer 644

Anti-CD30 mAb coupled to a cytotoxic agent Used in relapsed DLBCL and Hodgkin lymphoma (i.e. CD30+ conditions)

Question 645

Nintedanib: - Key side-effects (2) - Mechanism

Answer 645

Diarrhoea Deranged LFTs (Multi-tyrosine kinase inhibitor, including inhibition of fibrogenic growth factors)

Question 646

Pirfenidone: - Key side-effects (2) - Mechanism

Answer 646

Photosensitivity Nausea (Inhibitor of TGF-beta --> anti-fibrotic effects)

Question 647

Myeloma therapy: - Induction agents (3) - Maintenance after autologous stem cell transplant (1)

Answer 647

Bortezomib + lenalidomie + dexamethasone (Then autologous stem cell transplant after melphalan conditioning) Lenalidomide for maintenance

Question 648

What are helmet cells (AKA schistocytes) seen in?

Answer 648

Microangiopathic diseases E.g. TTP, HUS, DIC (Due to fibrin strands 'cleaving' the RBC)

Question 649

How are 'bite cells' formed?

Answer 649

Oxidative disease e.g. G6PD deficiency --> Hb denatures --> forms Heinz body --> Heinz body gets 'bitten out' by splenic macrophage

Question 650

What diseases are 'tear drop' cells associated with? (2)

Answer 650

Myelofibrosis, thalassaemia

Question 651

Most common congenital heart defects in Downs Syndrome (top 3)

Answer 651

1. Complete AVSD (37%) 2. VSD (31%) 3. ASD (15%) CHD seen in up to 50% of Down's patients, and up to 23% have 2x abnormalities

Question 652

What is the significance of the NAP1 Clostridium strain?

Answer 652

Hypervirulent strain that produces and additional binary toxin, produces larger amounts of toxins A/b, and has increased antibiotic (esp. quinolone) resistance / decreased cure rates

Question 653

Which cytokine is associated with increased C. difficile diarrhoea?

Answer 653

IL-8

Question 654

First line medication for mild Parkinson's

Answer 654

MAOb inhibitors e.g. Selegiline | Prevent dopamine metabolism

Question 655

Abatacept target

Answer 655

Binds CD80/86 on APCs - prevents activation of T-cells (used in RA)

Question 656

True / false: alcohol consumption is protective against renal cell carcionma

Answer 656

True

Question 657

Scenarios in which to use tetanus immunoglobulin (2)

Answer 657

In a tetanus-prone wound: - If there is no/unclear history of vaccination - If the patient is immune compromised

Question 658

Oestrogen-dependent cancers (2)

Answer 658

Endometrial | Breast

Question 659

Hormone replacement therapy: contraindication to using oestrogen-only therapy (either oral or transdermal)

Answer 659

Presence of endometrial tissue (due to risk of cancer). I.e. so only use if they've had a hysterectomy

Question 660

HRT: when to use: - Oestrogen and continuous progesterone - Oestrogen and cyclical progesterone

Answer 660

Post-menopausal Peri-menopausal

Question 661

Key MMSE domain deficit in Lewy Body dementia

Answer 661

Visiospatial e.g. copying or drawing objects

Question 662

What is the best test to monitor the treatment of secondary hypothyroidism?

Answer 662

T4

Question 663

What are the treatment options for catatonia?

Answer 663

Lorazepam ECT (NOT anti-psychotics initially - can precipitate NMS)

Question 664

What can female carriers of X-linked adrenoleucodystrophy present with? (2)

Answer 664

Myelopathy | Polyneuropathy

Question 665

At what stage to offer CABG instead of PCI?

Answer 665

Two vessel disease involving LAD Triple vessel disease (I.e. two vessel disease but just RCA and circumflex still gets PCI instead)

Question 666

Summarise ECOG classes (0-5)

Answer 666

0: fully fit / independent 1: reduced physical strenuous activity 2: ambulatory + self care, mobile > 50% of day 3: limited self-care, mobile <50% of day 4: fully disabled 5: dead

Question 667

What components of psoriatic arthritis is MTX useful for? (3)

Answer 667

Arthritis Skin IBD

Question 668

Key arrhythmias of Brugada

Answer 668

AF VF Polymorphic VT (While most other cases of cardiomyopathy have monomorphic VT as rhythm).

Question 669

Empiric regimen for necrotising fasciitis. + if water-immersed

Answer 669

Meropenem OR PipTaz AND Clindamycin OR Lincomycin If water-immersed wound, add ciprofloxacin (for Aeromonas)

Question 670

Which thyroid test is elevated during sick euthyroid?

Answer 670

Reverse T3 | While 'regular' T3 is almost always low

Question 671

Chemo agents with >90% emetogenicity

Answer 671

Cyclophosphamide Cisplatin Carmustine

Question 672

Which type of peptic ulcers need follow-up scope in 8-12 weeks?

Answer 672

Gastric ulcers | Duodenal ulcers have very low malignancy risk

Question 673

True / False: DMARDs can be used for axial manifestations of ankylosing spondylarthritis

Answer 673

False (DMARDs are only used as second line for PERIPHERAL manifestations) For axial Sx: First = NSAIDs and conservative Rx. Second = anti-TNF or anti-IL-17

Question 674

What is the mode of inheritance for C9ORF72 and SOD1?

Answer 674

Autosomal dominant

Question 675

Hypersensitivity allele for abacavir

Answer 675

HLA-B*5701

Question 676

Hep B post-exposure prophylaxis consists of: Scenarios / indications.

Answer 676

Vaccination AND immunoglobulin as soon as possible But only indicated in case where: - Source is hep B positive or unknown status - AND recipient is not immune If source is negative, but recipient is also negative, then just immunise No action if recipient is known to be immune

Question 677

How to remember non-nucleoside reverse transcriptase inhibitors

Answer 677

'-vir' in the middle of the name e.g. efavirenz, nevirapine

Question 678

How to remember protease inhibitor drug names

Answer 678

'-navir' at the end of the drug name ('never (navir) tease a PRO) (Class associated with increased lipids)

Question 679

How to remember integrase inhibitor drug names

Answer 679

End with '-gravir' | Class associated with increased weight

Question 680

HIV fusion inhibitors (2)

Answer 680

Maraviroc | Enfuvirtide

Question 681

How to remember nucleoside reverse transcriptase inhibitors

Answer 681

Everything else not covered by other mnemonics

Question 682

Which hep B drugs are safe during pregnancy?

Answer 682

Tenofovir or lamivudine, not entecavir

Question 683

What does the NOD2 gene predispose to?

Answer 683

Ileal crohn's disease

Question 684

What is the mechanism of ocrelizumab?

Answer 684

Anti-CD20 (used in MS)

Question 685

Obinutuzumab mechanism of action

Answer 685

Anti-CD20 (used in follicular lymphoma and CLL)

Question 686

What targeted agent to use in right-sided, RAS/RAF mutated colorectal cancer?

Answer 686

Bevacizumab (VEGF inhibitor)

Question 687

What targeted agent to use in left-sided, RAS/RAF wild type colorectal cancer?

Answer 687

Cetuximab, panitumumab (EGFR inhibitors)

Question 688

Causes of 'Erlenmeyer Flask Deformity' on x-ray (4)

Answer 688

Gaucher's disease (lysosomal storage disorder) Niemann-Pick disease Osteopetrosis Heavy metal poisoning

Question 689

Formula for VO2max

Answer 689

VO2max = CO(CaO2 - CvO2) I.e. cardiac output x oxygen utilised

Question 690

What is the E/a ratio? (3)

Answer 690

Ratio of early diastolic filling (passive) to late atrial filling (active). Ratio <1 indicates impaired heart relaxation due to reduced early filling But in severe disease see ratio >2.0 (due to restrictive filling)

Question 691

What is the E/e' ratio?

Answer 691

Assessment of LV filling pressure. Value >15 indicates LV diastolic dysfunction.

Question 692

Positive likelihood ratio formula

Answer 692

+LR = sensitivity / (100 - specificity)

Question 693

Negative likelihood ratio formula

Answer 693

-LR = (100 - Sensitivity) / specificity

Question 694

Post-test odds formula

Answer 694

Post test odds = pre-test odds * likelihood ratio

Question 695

Odds ratio formula

Answer 695

OR = odds in exposure group : odds in control group

Question 696

What is the 500 rule in diabetes?

Answer 696

Estimates insulin-carb ratio: 500 / total daily insulin = number of carbs covered by a unit of insulin

Question 697

What is insulin sensitivity factor + how to calculate

Answer 697

How much one unit of short-acting insulin will lower BSL ISF = 100 / total daily insulin Can then use ISF to calculate correction dose: E.g. if current BSL is 10mM higher than target, then can give 10/ISF units (on top of normal insulin)

Question 698

Dialysable drugs

Answer 698

``` 'BLAST': Barbiturates Lithium Alcohol/ethylene glycol Salicylates Theophyline ```

Question 699

Muscles supplied by median nerve

Answer 699

``` 'LOAF': Lateral 2x lumbricals Opponins pollicis Abductor pollicis brevis Flexor pollicis brevis ```

Question 700

Statistical to test to use for: | Qualitative data / binomial data

Answer 700

Chi square

Question 701

Statistical to test to use for: | Quantitative, one group before/after intervention, normal distribution

Answer 701

Paired t-test

Question 702

Statistical to test to use for: | Quantitative, one group before/after intervention, not normal distribution

Answer 702

Wilcoxon test (non-parametric test with ONE word)

Question 703

Statistical to test to use for: | Quantitative, two groups compared, normal distribution

Answer 703

Unpaired t-test

Question 704

Statistical to test to use for: | Quantitative, two groups compared, not normal distribution

Answer 704

Mann-Whitney test (non-parametric test with TWO words)

Question 705

Statistical to test to use for: | Quantitative, more than 2 groups

Answer 705

ANOVA - if normal distribution | Kruskal-Wallis - not normal distribution

Question 706

Hormones that increase appetite (3)

Answer 706

Ghrelin NPY AgRP

Question 707

Markers of bone formation (3)

Answer 707

Bone-specific ALP (BSAP) N-terminal PROpeptide of type 1 collagen (PINP) Osteocalcin (i.e. not TELOpeptides, the rest indicate bone resorption)

Question 708

Alcohol withdrawal; at what time do seizures peak?

Answer 708

After ~36h

Question 709

CYP system inducers (6)

Answer 709

``` Carbamazepine Rifampicin Phenytoin/phenobarbitone St John's wort Smoking ``` (R, P and S also induce p-glycoprotein system)

Question 710

Ank spond: most common extra-axial joint involved

Answer 710

Hip

Question 711

What cell marker on B cell gets bound to induce isotype switching?

Answer 711

CD40

Question 712

Cell surface marker for T cells

Answer 712

CD3, 4, and 5 +/- CD4 and 8

Question 713

Cell surface marker for B cells

Answer 713

CD 19, 20

Question 714

What cell is FOXP3 found on?

Answer 714

Treg | Deficient in IPEX syndrome

Question 715

What is CD13 found on?

Answer 715

Myeloid cells e.g. AML

Question 716

Cell surface markers for CLL

Answer 716

CD5, 19

Question 717

HIV medication that affects mood

Answer 717

Efavirenz

Question 718

Class-wide side effect of nucleoside reverse transcriptase inhibitors

Answer 718

Lactic acidosis

Question 719

Systemic sclerosis: antibody associated with ILD

Answer 719

Anti-Scl-70 (diffuse SSc)

Question 720

Antibody associated with polymyositis

Answer 720

Anti-SRP

Question 721

Antibody associated with anti-synthetase syndrome

Answer 721

tRNA synthetases (including Jo-1)

Question 722

Antibodies in type 1 autoimmune hepatitis

Answer 722

ANA Smooth muscle antibodies Anti-actin Anti-SLA/LP (specific, severe) (Rarely see pANCA)

Question 723

Antibodies in type 2 autoimmune hepatitis

Answer 723

Anti-LKM | Anti-LC1

Question 724

ASCA: positive or negative in Crohn's?

Answer 724

Positive (and negative pANCA) Opposite in UC

Question 725

Name the circulating factor in FSGS

Answer 725

suPAR

Question 726

What do microRNAs do?

Answer 726

Small ssRNAs that bind mRNA and lead to their degradation --> can cause impaired protein production

Question 727

Which MHC does beta-2 microglobulin go with?

Answer 727

MHC-1

Question 728

Which Ambler class (re: antibiotic resistance) requires Zinc to work?

Answer 728

Class B (Thus, also called metallo-beta-lactamase) (Other classes need serine) (Class B is treated with aztreonam + ceftazadime/avibactam, or polymyxin regimen e.g. colistin + tigecycline)

Question 729

'Gold standard' test for measuring GFR

Answer 729

Inulin (completely cleared by glomerular filtration - no tubular secretion or reabsorption)

Question 730

Alzheimers prognostic association of: - APOE2 - APOE4

Answer 730

Reduced risk | Increased risk

Question 731

Gene association with Charcot-Marie-Tooth disease

Answer 731

PMP22

Question 732

Best genetic test for: - Chromosomal duplication / deletions e.g. Downs - Reciprocal translocation e.g. BCR-Abl - Microduplications/microdeletions e.g. Charcot-M-T

Answer 732

Karyotyping FISH Array comparative genomic hybridisation

Question 733

Chemo drug classes affecting M-phase (mitosis) (3)

Answer 733

Taxanes Vinca alkaloids Etoposide (also affects G2)

Question 734

Chemo drug classes affecting G2 phase (1)

Answer 734

Bleomycin

Question 735

Chemo drug classes that are independent of cell cycle stage (2)

Answer 735

Platinum agents | Alkylating agents

Question 736

Chemo drug classes that affect S-phase (3)

Answer 736

Antimetabolites Hydroxyurea Topoisomerase 1 inhibitors

Question 737

Using what drug has high association with Nocardia infection?

Answer 737

Leflunomide

Question 738

Organisms included in TB PCR complex (3)

Answer 738

M. Tb M. bovis M. Africanum

Question 739

Use of ritonavir in HIV

Answer 739

As a protease inhibitor 'booster' (inhibits CYP3A4)

Question 740

HIV medication associated with lipodystrophy

Answer 740

Zidovudine and protease inhibitors

Question 741

Key side-effects of nevirapine (2)

Answer 741

SJS, severe hepatitis

Question 742

What did the GEMINI study demonstrate (re: HIV Rx)

Answer 742

2x drug combo might be OK - if well-controlled can step down to Dolutegravir and Lamivudine

Question 743

Which condition requires liver biopsy for diagnosis

Answer 743

Autoimmune hepatitis

Question 744

What is obeticholic acid used for?

Answer 744

Second line Rx for PBC if ursodeoxycholic acid ineffective ('Fernesoid X receptor agonist)

Question 745

What is the most common type of lymphoma?

Answer 745

DLBCL (31%) - intermediate/aggressive Follicular (22%) - indolent

Question 746

What type of Hodgkin lymphoma has: - Worst prognosis? - Best prognosis?

Answer 746

Lymphocyte depleted Lymphocyte predominant (classical)

Question 747

Route of spread of Hodgkin lymphoma

Answer 747

To contiguous nodal groups (usually above diaphragm)

Question 748

Chromosomal translocation for DLBCL / new product

Answer 748

t(3;14) --> bcl-6 (new mutant allows DNA errors to accumulate without apoptosis)

Question 749

Follicular lymphoma: initial treatment regimen

Answer 749

Can watch and wait (GELF criteria) Otherwise: rituximab OR obinutuzumab + chemo: CVP vs CHOP vs bendamustine)

Question 750

Management of diffuse large b cell lymphoma - Initial - Relapse

Answer 750

R-CHOP If relapse: brentuximab or pembrolizumab

Question 751

Which 'cell of origin' type of DLCBL lymphoma has: - Good prognosis - Poor prognosis

Answer 751

Germinal centre B-cell = good Activated B cell = poor

Question 752

Neutropaenic fever: - Usual antibiotic - Antibiotics if shocked / needing ICU

Answer 752

PipTaz (NO added G-CSF acutely) Gentamicin + Vancomicin + PipTaz (or other anti-pseudomonal)

Question 753

Renal stone types; order of most-to-least common (5)

Answer 753

``` Calcium-oxalate (70-80%) Calcium-phosphate (15%) Uric acid (8%) Cystine (1-2%) Struvite (1%) ``` Struvite = Mg + P + NH4

Question 754

What are the tests sCD25 and CXCL9 associated with?

Answer 754

Haemophagocytic lymphhistiocytosis | sCD25 = soluble IL-2 receptor

Question 755

What is anti-MAG associated with?

Answer 755

Distal acquired demyelinating symmetric neuropathy | A CIDP-like disease with high monoclonal IgM (either MGUS or Waldenstroms

Question 756

First line drug for Aspergillus treatment

Answer 756

Voriconazole | Followed by caspofungin, then amphotericin

Question 757

General treatment for invasive Candidiasis: - First line - Step down Rx - Resistant disease

Answer 757

Echinocandin Fluconazole Amphotericin

Question 758

What type of radiological findings are seen in UIP? (4)

Answer 758

Sub-pleural reticulation (i.e. peripheral lung) Disease worse at lung bases Traction bronchiectasis Honeycombing

Question 759

What type of radiological changes are seen in NSIP? (4)

Answer 759

Ground glass change Sub-pleural sparing In common with UIP: Traction bronchiectasis Basal predominance

Question 760

What type of pattern is seen in idiopathic pulmonary fibrosis?

Answer 760

UIP

Question 761

Mutation associated with idiopathic pulmonary fibrosis

Answer 761

MUC5b (50%)

Question 762

What type of ILD pattern does rheumatoid arthritis have?

Answer 762

UIP | While most other connective tissue diseases will have NSIP or COP

Question 763

Why is Dravet Syndrome a resistant form of epilepsy?

Answer 763

Mutation in sodium channels (SCN1A gene) so that sodium-channel blockers have no effect

Question 764

What is the 'Telomeropathy Syndrome'? and give two examples

Answer 764

Mutation in genes that maintain telomeres --> early age organ dysfunction. Autosomal dominant with anticipation phenomenon, can often affect different organ systems across generations. Pulmonary fibrosis Dyskeratosis congenita Aplastic anaemia

Question 765

What cell cycle phase do hormonal agents act in?

Answer 765

G1

Question 766

What protein won't dipstick urine detect

Answer 766

Non-albumin protein e.g. light chains | Use sulfosalicylic acid test

Question 767

Key cytokine in gout pathogenesis

Answer 767

IL-1-beta | Mediated by neutrophils / inflammasome

Question 768

How does BCL-2 work?

Answer 768

Inhibits cell apoptosis

Question 769

What does streptococcal M-protein mimic?

Answer 769

Cardiac myosin

Question 770

Steroid effect on bone: - Acute - Chronic

Answer 770

Increase resorption (inhibits OPG and stimulates RANK) Decreases formation (reduces proliferation/differentiation and increases apoptosis of osteoblasts)

Question 771

dsDNA staining pattern

Answer 771

Homogenous

Question 772

Which insulins bind to albumin (2)

Answer 772

Detemir Degludec (ultra-long-acting)

Question 773

How does lactulose reduce encephalopathy

Answer 773

Substrate for bacteria --> reduced pH --> increased NH4+ (which is non-absorbable)

Question 774

What part of internal capsule is the corticospinal tract in?

Answer 774

Posterior limb

Question 775

What chromosome is amyloid precursor protein on?

Answer 775

Ch 21

Question 776

Which interleukin is associated with Mendelian Susceptibility to TB

Answer 776

IL-12

Question 777

Nerves supplying bladder (3)

Answer 777

Pelvic nerve (PNS) --> ACh to M3 Hypogastric nerve (SNS) --> NA to beta-3 and alpha 1 Pudendal nerve (somatic) --> ACh to nicotinic receptor

Question 778

What causes depletion of substance P

Answer 778

Capsaicin

Question 779

What does Ki-67 indicate?

Answer 779

Cell proliferation

Question 780

Cerebellopontine angle lesions affect which CN's

Answer 780

V, VII and VIII E.g. Schwannoma or meningioma

Question 781

Pizotifen: - Mechanism - Side-effect

Answer 781

Serotonin blocker Weight gain

Question 782

Essential thrombocytosis mutations, common to least common (3)

Answer 782

JAK2 (40%) CAL-R (15-30%) MPL (4-8%)

Question 783

Key criteria (Ghent criteria) for Marfan's

Answer 783

2 of 3 of: - Aortic distension/dissection - Ectopia lentis (up / out) - FBN1 gene

Question 784

Key lesion to distinguish L5 lesion vs common peroneal

Answer 784

Ankle inversion weakness (=L5)

Question 785

Cells in HLH pathogenesis (2)

Answer 785

NK cells that fail to inactivate over-stimulated macrophages

Question 786

Cell type in clozapine myocarditis

Answer 786

Eosinophils

Question 787

Cranial nerves with parasympathetic fibres

Answer 787

III (pupil constriction) VII (lacrimal / salivary glands) IX (parotid gland) X (vagus)

Question 788

Type B nerve fibres carry

Answer 788

Preganglionic signals to autonomic nervous system

Question 789

True / False: transthyretin causes renal amyloidosis

Answer 789

False

Question 790

Cytokine implicated in periodic fever syndromes

Answer 790

IL-1

Question 791

Best test for working memory

Answer 791

Digit span forward

Question 792

Medication hierarchy to treat akathisia (3)

Answer 792

1. Beta blocker 2. Benztropine 3. Benzodiazepine

Question 793

'Storiform fibrosis' refers to what diagnosis

Answer 793

IgG4 deposition disease

Question 794

Most common side-effect of oseltamivir

Answer 794

Nausea / vomiting (15%)

Question 795

Length of PPI therapy for NSAID-induced ulcer

Answer 795

8-12 weeks

Question 796

UTI treatment in pregnancy (3)

Answer 796

Nitrofurantoin Cephalexin Trimethoprim (2nd/3rd trimester)

Question 797

Most common gentamicin toxicities (2)

Answer 797

Oscillopsia, imbalance | Rare to have hearing loss, mainly tinnitus

Question 798

Type III MI definition

Answer 798

MI resulting in death without biomarkers

Question 799

What is the primary tyrosine kinase in systemic mastocytosis?

Answer 799

Kit (usually lost in other blood cancers)

Question 800

Most common mutation in Duchenne's

Answer 800

Exon deletions

Question 801

Enzyme affected in acute intermittent porphyria Inheritance How does Hemin work?

Answer 801

Porphobilinogen deaminase (Autosomal dominant) Rx = Hemin - reduces ALAS1 activity --> reduced build-up of haem precursors

Question 802

Why does 3rd nerve palsy give bigger ptosis than Horner's?

Answer 802

3rd nerve palsy affects levator palpebrae superioris, while in Horners, only the superior tarsal muscle is affected.

Question 803

Features suggesting midline cerebellar lesion (7)

Answer 803

``` Gait ataxia Imbalance Truncal ataxia Leg dysmetria Ocular signs Head bobbing Vertigo ``` (Other symptoms caused by hemispheric lesion)

Question 804

Signalling pathway of PTH to cause increased resorption

Answer 804

1. PTH released 2. Acts on osteoblast 3. Osteoblast increased RANK-L 4. Binds to RANK on osteoclast, leading to differentiation / activation Osteoblast also reduces OPG in response to PTH

Question 805

Normal role of osteoprotegerin

Answer 805

Inhibits / decoy against RANK-L, thereby reducing osteoclast activation

Question 806

Molecule mutated in eczema

Answer 806

Fillagrin

Question 807

Common site for metastatic lesions for rectal cancer

Answer 807

Lungs (due to drainage into IVC, rather than portal system)

Question 808

Warm autoimmune haemolytic anaemia - Antibody - Location

Answer 808

IgG Extravascular

Question 809

Cold autoimmune haemolytic anaemia - Antibody - Location

Answer 809

IgM / C3 Intravascular

Question 810

Causes of warm AIHA (4)

Answer 810

Primary / Idiopathic Lymphoproliferative disease e.g. CLL, NHL Connective tissue disorders Methyldopa, other drugs

Question 811

Causes of cold AIHIA (4)

Answer 811

Primary / idiopathic Mycoplasma EBV Lymphoma

Question 812

Antibiotics that inhibit 30S subunit (2)

Answer 812

Aminoglycosides | Tetracyclines

Question 813

Antibiotics that inhibit 50S subunit

Answer 813

Macrolides Clindamycin (lincosamide) Linezolid (oxazolid) Chloramphenicol

Question 814

Mechanism of Amphotericin / Polyenes

Answer 814

Impairs membrane function by binding to ergosterol --> increased permeability

Question 815

Mechanism of 'azole' anti-fungals

Answer 815

Prevent ergosterol synthesis (via inhibition of C-14-alpha demethylase)

Question 816

When to use tetanus Ig (2)

Answer 816

No prior immunisation Immunocompromised (No vaccine needed if last one given <5 years ago)

Question 817

Hormone replacement therapy, when to use: - E2 and continuous P - E2 and cyclical P - Unopposed E2

Answer 817

Post-menopause Peri-menopause Only if prior hysterectomy (due to risk of endometrial cancer if unopposed estrogen exposure)

Question 818

How to monitor secondary hypothyroidism treatment

Answer 818

T4 levels

Question 819

True / False: CD20 is expressed on plasma cells

Answer 819

False - hence why rituximab doesn't cause depletion of immune memory / can maintain normal IgG level

Question 820

What type of GN has highest association with renal vein thrombosis

Answer 820

Membranous

Question 821

What symptoms can female carriers of x-linked adrenoleucodystrophy have (2)

Answer 821

Myelopathy | Neuropathy

Question 822

Treatments for catatonia (2)

Answer 822

Benzo's ECT

Question 823

Most common cause of genetic ALS

Answer 823

C9ORF72 | Associated with FTD / tau-opathy

Question 824

Long-term complication after repair of tetralogy of Fallot

Answer 824

Pulmonary incompetence

Question 825

Medication to treat pan-resistant ESBL

Answer 825

Colistin (alongside tigecycline)

Question 826

During abiraterone treatment, what concurrent medication needs to be given?

Answer 826

Glucocorticoids

Question 827

What does Lugol's iodine do?

Answer 827

Prevents release of pre-formed thyroid hormone

Question 828

What condition causes a thyroid bruit?

Answer 828

Grave's disease

Question 829

What outcomes are improved by using CPAP? (4)

Answer 829

Less MVAs Improved QoL Better sleep scores / readings Less GORD

Question 830

Ratio for oxynorm to PO morphine

Answer 830

1.5

Question 831

Most sensitive test for diagnosis of TB / hierarchy of tests for sensitivity

Answer 831

3x consecutive TB sputum cultures | Culture > NAAT > smear

Question 832

Medications for essential tremor (2)

Answer 832

Primidone | Propranolol

Question 833

Route of breast cancer spread if: - ER+ - HER2+

Answer 833

To bones To viscera

Question 834

Infective cause of coronary artery aneurysms

Answer 834

Syphilis

Question 835

Best ACEi to use in diffuse scleroderma

Answer 835

Captopril (not ARBs)

Question 836

Medications for orbital cellulitis (2)

Answer 836

Flucloxacillin AND ceftriaxone

Question 837

What does a linear lucency through the lateral cortex of the subtrochanteric region suggest?

Answer 837

Atypical femoral fracture

Question 838

What is the 'double contour sign' on US diagnostic of?

Answer 838

Gout | Can also see cloudy area on US

Question 839

Core features of Lewy Body Dementia (3)

Answer 839

Cognitive fluctuation Visual hallucinations REM sleep disorder

Question 840

What electrolyte abnormality can trimethoprim cause?

Answer 840

Hyper-K+

Question 841

Which Parkinson Plus syndrome is associated with axial rigidity and early falls

Answer 841

PSP

Question 842

Joints affected in haemochromatosis

Answer 842

2nd and 3rd MCPJs (can have erosive arthritis)

Question 843

True / False: Having diabetes is protective against development of a new AAA

Answer 843

True | Smoking = greatest risk factor

Question 844

What is the gender difference for developing / extension of AAA

Answer 844

Male - higher risk of getting AAA | Female - higher risk of extension of AAA

Question 845

What is a bilateral vestibular Schwannoma associated with?

Answer 845

NF2 (mutation in merlin/schwannomin protein which is normally a tumour suppressor)

Question 846

What condition is dual energy CT used for to diagnose?

Answer 846

Gout

Question 847

On ECHO, what does fractional shortening indicate?

Answer 847

Systolic failure (a correlate of ejection fraction)

Question 848

Mechanism of Ivacaftor

Answer 848

Increases CFTR activity / improves Cl- gating

Question 849

Mechanism of Lumacaftor

Answer 849

Improves folding of CFTR protein

Question 850

What is the most effective complement-fixing Ab

Answer 850

IgM

Question 851

What drug inhibits inosine monophosphate dehydrogenase?

Answer 851

Mycophenolate

Question 852

What does Muromonab bind to?

Answer 852

CD3 (on T cell surface) | Used as immune suppressant after solid organ transplant

Question 853

'Trauma' medication that may be useful in hereditary angioedema.

Answer 853

Tranexamic acid (by reducing amount of fibrin breakdown products)

Question 854

Features of NF1 (6)

Answer 854

``` Cafe au lait Axillary freckling Lisch nodules (most common sign) Bone lesions Tumours e.g. fibromas Neurologic symptoms ```

Question 855

True/false: activated charcoal is effective in lithium poisoning.

Answer 855

False - heavy metals, alcohol and inorganic ions do not adsorb well to charcoal

Question 856

What does bilateral absence of the vas deferens (in CF) result in?

Answer 856

Obstructive azoospermia

Question 857

Antibiotic class that interacts with tacrolimus

Answer 857

Macrolide

Question 858

Normal Pulmonary Capillary Wedge Pressure (PCWP)

Answer 858

6-15mmHg

Question 859

Adverse effect of Mg2+ in resus

Answer 859

Hypotension and bradycardia

Question 860

How does abiraterone work in prostate cancer?

Answer 860

Inhibits synthesis of testosterone (at level of adrenals and at level of cancer cells)

Question 861

Aspergillus prophylaxis

Answer 861

Posaconazole or itraconazole

Question 862

Below what spinal level do people no longer get autonomic dysreflexia / why

Answer 862

T6 - due to compensatory vasodilatation of splanchnic circulation

Question 863

Outcomes of NIV in motor neuron disease: - Bulbar disease - Non-bulbar disease

Answer 863

Increased QoL Improved survival

Question 864

CrCl cut-offs for anti-coagulants: - Warfarin - Dabigatran - Rivaroxaban - Apixaban

Answer 864

No limit <30 <15 <15

Question 865

Most common cancers in Peutz-Jeghers syndrome

Answer 865

Colon, pancreas, breast

Question 866

Peutz-Jeghers inheritance

Answer 866

Autosomal dominant (STK11 gene)

Question 867

Indications for acute ERCP in pancreatitis (3)

Answer 867

Cholangitis CBD obstruction Increasing LFTs (Otherwise no role in first 24h, fluids have greatest survival benefit)

Question 868

Features of essential tremor (5)

Answer 868

``` Bilateral Action tremor No other neuro Fine/high frequency Worse with stress, better with EToH ```

Question 869

Urease-producing organisms (2)

Answer 869

Proteus | Klebsiella

Question 870

Antibiotic for pre-orbital cellulitis

Answer 870

Flucloxacillin

Question 871

Type of malignancy associated with BRAF-inhibitor use (as in, a side-effect)

Answer 871

Skin squamous cell carcinoma

Question 872

3x most common cardiac abnormalities in Turner Syndrome

Answer 872

Most common = bicuspid AV 2nd = coarctation 3rd = pulmonary stenosis

Question 873

What does a U-wave correlate with?

Answer 873

Hypokalaemia | Also hypothermia or hypocalcaemia

Question 874

T-cell maturation: - Positive selection - Negative selection

Answer 874

Binds MHC-1/-II --> survive | Apoptosis if binds self-antigens

Question 875

Factors involved in small initiation of clotting cascade

Answer 875

Tissue factor and VII

Question 876

Factors involved in amplification of clotting cascade

Answer 876

Xa and V

Question 877

Cough reflex: - Afferent fibre - Synapse point - Efferent fibre

Answer 877

Vagus nerve Medulla Vagus + phrenic + spinal

Question 878

Goserilin / Leuprolide - Mechanism - What to watch out for in prostate cancer - Cancer-related contraindications (2)

Answer 878

GnRH agonist 'Flare' phenomenon in first two weeks - manage with concurrent androgen receptor blocker e.g. bicalutamide Urinary tract obstruction, painful bone mets

Question 879

Degarelix mechanism

Answer 879

GnRH antagonist - avoids flare phenomenon

Question 880

What to give alongside abiraterone therapy

Answer 880

Glucocorticoids (due to increased shunting towards mineralocorticoid synthesis)

Question 881

Cancer associated with exposure to unopposed oestreogen

Answer 881

Endometrial, breast

Question 882

Enzyme that is weakly inhibited by paracetamol

Answer 882

COX-1 | Thus can cause flare of aspirin-exacerbated respiratory disease in some people

Question 883

Upper limb critical actions: - C5 - C6 - C7 - C8 - T1

Answer 883

Elbow flexion Wrist ext Elbow ext Finger flex Finger abduction

Question 884

Draw diagram of sciatic nerve divisions and motor/sensory supply

Answer 884

Sciatic (L4-S3) - knee flexion, sensation below knee Tibial (posterior) branch --> plantarflexion / inversion / sole of foot Common peroneal - Deep branch --> dorsiflexion / 1st webspace - Superficial branch --> eversion / dorsum / lateral leg

Question 885

Summary table for foot drop: - Sciatic - L4/5 - Common peroneal

Answer 885

Sciatic - loses eversion, inversion and ankle jerk L4/5 - preserved ankle jerk (it is S1/2) Common peroneal - only loses eversion

Question 886

Motor component of ankle jerk reflex

Answer 886

S1/2

Question 887

Venetoclax mechanism / use

Answer 887

Binds / inhibits BCL-2 Used in CLL with del(17p)

Question 888

Target of Benralizumab

Answer 888

IL-5R (used in asthma)

Question 889

Interventions with a proved benefit in reducing falls (4)

Answer 889

Group or home exercises (balance, strength) Home safety interventions Multi-factorial assessment Tai Chi

Question 890

Size cut-off of lesions for liver transplantation for HCC

Answer 890

3x lesions, less than 3cm OR 1x lesion <5cm (Milan) Or Cumulative tumour <8.5cm (UCSF criteria)

Question 891

TKIs for HCC (2)

Answer 891

Sunitinib Lenvatinib Used in stage C disease

Question 892

Metformin - Mechanism - Key ADRs (3)

Answer 892

Activation of AMP-activated protein kinase (AMPK) → increases insulin sensitivity GI upset, low B12 (can be reversed by giving calcium supplementation), lactic acidosis

Question 893

Sulfonylureas - Mechanism - Key ADRs (2)

Answer 893

Close ATP-sensitive K+ channels → stimulate insulin secretion Hypoglycaemia, weight gain

Question 894

Thiazolidinediones ('glitazones') - Mechanism - Key ADRs (2)

Answer 894

PPARy agonist in adipocytes Weight gain, fluid retention (contraindicated in CHF)

Question 895

DPP-4 inhibitors ('gliptins') - Mechanism - Key ADRs (1)

Answer 895

Inhibits DPP-4 which normally breaks down incretins GLP-1 and GIP Pancreatitis

Question 896

Which SGLT-2i is associated with highest euDKA risk?

Answer 896

Canagliflozin (HR3.58)

Question 897

Genetic causes of phaeochromocytoma (4)

Answer 897

MEN2 VHL SDH mutation (paraganglioma) NF1

Question 898

How to monitor for phaeochromocytoma recurrence after surgery (2)

Answer 898

Chromogranin A | Metanephrines

Question 899

Follow-up for adrenal incidentaloma with initial negative work-up (2)

Answer 899

Annual hormone screen for 5 years | Imaging at 3-6 months, then annually for up to 2 years

Question 900

What happens to V/Q ratio from apex to base?

Answer 900

``` Both V and Q increase as you go to base. Q increases more than V, thus the V/Q ratio lowers. At apex, it is ~2.1 At middle, ~1.0 At base, ~0.3 ```

Question 901

Layers of the epidermis from out to in (5)

Answer 901

``` Stratum corneum Stratum lucidum Stratum granulosum Stratum spinosum Stratum basale ```

Question 902

Muscle controling active expiration

Answer 902

Internal intercostals

Question 903

Herbal OTCs that cause liver injury (4)

Answer 903

Black cososh (menopausal Sx) Valerian (sleep, relaxation) Chinese herbal meds Green tea

Question 904

Where / what makes ACE in sarcoidosis?

Answer 904

Macrophages in granulomas

Question 905

Type of genetic inheritance diseases that can be 'cured' with gene therapy

Answer 905

Autosomal recessive - replaced loss of function

Question 906

What hormone mediates sweating?

Answer 906

Adrenaline

Question 907

Key parameter to monitor in GBS re: respiratory crisis

Answer 907

FVC - intubate when <20ml/kg

Question 908

Agent to use in severe serotonin syndrome

Answer 908

Cyproheptadine

Question 909

Agent to use in severe NMS

Answer 909

Bromocriptine

Question 910

Indications for ICS in COPD (3)

Answer 910

Frequent exacerbations/hospitalisations Eosinohilia Asthma component

Question 911

Brain region to consider if pure motor stroke

Answer 911

Internal capsule (blood supply from lenticulostriate arteries)

Question 912

Brain region to consider if pure sensory stroke

Answer 912

Thalamus (blood supply from PCA)

Question 913

'Bulbar' nerves

Answer 913

IX, X, XI, XII - exit at the medulla

Question 914

How to exclude medial rectus muscle lesion as being the cause of INO

Answer 914

Get patient to converge eyes - now just need bilateral CN III signal

Question 915

What do nerve roots exiting the spinal cord carry? - Anterior root - Posterior/dorsal root

Answer 915

Motor (cell bodies live in the anterior horn) Sensory (cell bodies of sensory cells live in the dorsal root ganglion)

Question 916

Where does the spinothalamic tract decussate?

Answer 916

1-2 levels above where they enter the spinal cord

Question 917

'Cape' or 'vest' sensory loss distribution is suggestive of...

Answer 917

Central cord lesion e.g. syringomyelia

Question 918

Outcome of anterior cord syndrome

Answer 918

1x anterior spinal artery supplies anterior 2/3 of cord --> ischaemia affects everything other than dorsal columns --> all modalities affected except proprioception and vibration

Question 919

Features that make a drug dialysable (3)

Answer 919

Low protein binding Low Vd Small molecular weight (Also note, increasing plasma clearance will DECREASE dialysis clearance rates)