Important notes document

Question 1

APC tumour suppressor gene lies on

Answer 1

chromosome 5
colonic cancer

Question 2

HFE gene for haemochromatosis lies on

Answer 2

chromosome 6

Question 3

ATP7B gene for Wilsons disease lies on

Answer 3

chromosome 13

Question 4

Peutz jegher syndrome gene

Answer 4

serine threonine kinase LKB1 or STK11

autosomal dominant

Question 5

test for haemochromatosis

Answer 5

transferrin saturation >55%, best marker to monitor tx response

2y osteoarthritis (hook like osteophytes at 2nd and 3rd MCP) and slate grey skin

also hypogonadotrophic hypogonadism due to pituitary gland involvement - typically presents as impotence in men and amenorrhoea in women - irreversible

diabetes due to pancreatic beta cells damaged

liver cirrhosis due to hepatic iron overload causing fibrosis

Question 6

encapsulated bacteria in splenectomy

Answer 6

Strep pneumoniae, Hib, n meningitidis

Question 7

HNPCC lynch gene

Answer 7

MSH2 and MLH1
involved in DNA mismatch repair leading to microsatellite instability

colorectal and endometrial cancers

Question 8

elevated subtypes of Ig and corresponding hepatobiliary disease

Answer 8

alcoholic LD - IgA
PBC - IgM
autoimmune hepatitis - IgG

Question 9

MEN1

Answer 9

parathyroid (hyper)
pituitary
pancreas (insulin/gastrinoma)
adrenal and thyroid

Question 10

nicotinic acid test

Answer 10

confirms diagnosis of Gilbert’s syndrome - mutation in gene for enzyme glucuronyl transferase

prolonged increase in serum unconjugated bilirubin levels due to impairment of hepatic uptake and conjugation of bilirubin

Question 11

ammonium chloride acidification test

Answer 11

to evaluate renal tubular acidosis rather than liver function

inability to adequately acidify urine

Question 12

fulminant vs viral hepatitis

Answer 12

necrosis vs apoptosis

Question 13

SBP most common organism

Answer 13

E Coli
Klebsiella

gram +
Strep pneumo, viridans and Staph

Question 14

Diarrhoea, history of carcinoid tumour - treatment

Answer 14

octreotide

Question 15

diarrhoea and history of scleroderma

Answer 15

suggestive of bacterial overgrowth

hydrogen breath test

rifaximin tx of choice
co-amox
metronidazole

Question 16

diarrhoea and terminal ileal resection

Answer 16

bile salt malabsorption

treatment with cholestyramine

Question 17

HCC management

Answer 17

Barcelona classification

no signs of portal htn, single lesion <2cm - surgical resection

2-3 tumours <3cm or 1 <5cm without vascular invasion or extrahepatic spread - liver transplant (treat with TACE/RFA)

good performance status and evidence of vasc, lymph, extrahep spread - sorafenib (tyrosine kinase inhibitor) prolongs survival

poor candidates - symptomatic tx

Question 18

Verner-Morrison or WDHA syndrome (watery diarrhoea, hypokalaemia, achlorhydria)

Answer 18

VIPomas - non-beta islet cells of the pancreas

Question 19

Fanconi syndrome

Answer 19

impaired renal tubular function.

anaemia - rare genetic disease leading to bone marrow failure and increased risk for leukaemia

Question 20

Glasgow imrie criteria

Answer 20

paO2 <7.9
age >55
WBC >15
Ca <2
urea >16
LDH >600
albumin <32
glucose >10

Question 21

laxative to avoid in IBS

Answer 21

lactulose

Question 22

factors increasing risk of cancer in UC

Answer 22

disease >10 yrs
pancolitis
onset <15 yo
unremitting disease
poor compliance to tx

Question 23

first symptom of carcinoid syndrome

Answer 23

facial flushing

due to release of serotonin and kallikrein into systemic circuliation

Question 24

% of individuals with positive FOBT that have ca

Answer 24

10

Question 25

biliopancreatic diversion with duodenal switch

Answer 25

primarily malabsorptive procedure, reserved for BMI >60

Question 26

coeliac disease HLA assoc

Answer 26

DQ2 and DQ8

Question 27

typical iron studies in haemochromatosis

Answer 27

transferrin saturation >55% in men or >50% in women raised ferritin and iron low TIBC

Question 28

Dubin Johnson syndrome

Answer 28

conjugated hyperbilirubinaemia defect in the canalicular multispecific organ anion transporter (MRP2) benign course - no clinical impairment, intermittent jaundice only

Question 29

marker of infectivity in hep B

Answer 29

HBeAg antibodies only - vaccine only

Question 30

colorectal ca with solitary liver lesion

Answer 30

surgical resection

Question 31

pancreatic cancer risk factors

Answer 31

BRCA2 mutations KRAS gene mutation also BRCA 1, PALB2, MLH1/MSH2

Question 32

how to prevent refeeding syndrome

Answer 32

provide 50% of normal energy and protein requirements for first 2 days

Question 33

appendicitis score

Answer 33

Alvarado

Question 34

prophylaxis of oesophageal bleeding

Answer 34

NSBB propranolol - decreases portal venous pressure by reducing cardiac output and splanchnic blood flow

Question 35

amoebiasis treatment

Answer 35

metronidazole + diloxanide furoate

Question 36

human/animal bite pen allergy

Answer 36

doxy + metro

Question 37

anthrax tx

Answer 37

ciprofloxacin

Question 38

lower UTI

Answer 38

trimeth/nitro

Question 39

acute pyelonephritis

Answer 39

broad spectrum cephalosporin/quinolone

Question 40

acute prostatitis treatment

Answer 40

quinolone for 14d trimethoprim

Question 41

exacerbations of chronic bronchitis tx

Answer 41

amox tetracycline clarithromycin

Question 42

uncomplicated CAP tx

Answer 42

amox pen allergy - doxy/clari staph spp - fluclox

Question 43

atypical pneum rx

Answer 43

clari

Question 44

HAP tx

Answer 44

<5d - coamox/cefuroxime >5d - piptaz/ceftaz/cipro

Question 45

impetigo rx

Answer 45

hydrogen peroxide oral fluclox/erythromycin

Question 46

cellulitis rx

Answer 46

fluclox clari/eryth/doxy in pen allergic

Question 47

cellulitis near eyes or nose

Answer 47

co-amox

Question 48

erysipelas

Answer 48

fluclox

Question 49

throat infections

Answer 49

phenoxymethylpenicillin (also for sinusitis) pen allergy - erythromycin

Question 50

otitis media vs externa

Answer 50

amox vs fluclox

Question 51

gingivitis rx

Answer 51

metronidazole

Question 52

gonorrohoea chlamydia PID syphilis BV treatment

Answer 52

IM ceftriaxone doxy/azithro ofloxacin+metro OR IM cipro+doxy+metro benzathine penicillin OR doxy OR erythromycin oral/topical metro OR topical clindamycin

Question 53

C diff campy salmonella (non typhoid) shigella

Answer 53

vanc - fidaxo - oral vanc + IV metro clarithromycin ciprofloxacin ciprofloxacin

Question 54

Fite's faraco stain

Answer 54

m leprae

Question 55

cryptosporidium spread

Answer 55

farm animals and contaminated water modified ZN stain

Question 56

aerosolised pentamidine prophylaxis

Answer 56

often not effective spontaneous pneumothorax

Question 57

incubation period of giardia

Answer 57

3-40 days chronic intermittent explosive diarrhoea without blood

Question 58

campy incubation

Answer 58

2-4 days

Question 59

HIV treatment process

Answer 59

2 NRTI + additional third (INI, PR or NNRTI)

Question 60

maraviroc moa

Answer 60

entry inhibitor

Question 61

examples of NRTI

Answer 61

emtricitabine tenofovir disoprox

Question 62

NNRTI examples

Answer 62

efavirenz nevirapine

Question 63

PI examples

Answer 63

ritonavir lopinavir

Question 64

rare complication in atypical pneumonia

Answer 64

cardiac conduction abnormalities due to autoimmune reactions

Question 65

purpura fulminans

Answer 65

strep pneumo

Question 66

high risk wounds for tetanus

Answer 66

>6 hrs >1cm deep foreign body infection ischaemia necrosis

Question 67

pregnancy VZV

Answer 67

susceptible in first 20 weeks - VZIG susceptible in 20 weeks on - either VZIG or aciclovir from d7-14 post exposure oral aciclovir if presenting within 24 hours of onset of rash and 20+ weeks

Question 68

silver stain

Answer 68

PCP or fungi

Question 69

rose bengal stain

Answer 69

fungal keratitis or acanthamoeba

Question 70

LGV treatment

Answer 70

doxy or azithro/erythro and possible drainage/aspiration

Question 71

Loefflers syndrome

Answer 71

larvae of strongy travel around the host body including reaching the lungs causing respiratory symptoms and eosinophilia tx of strongy - ivermectin

Question 72

causes of bloody diarrhoea CASSE

Answer 72

campy amoebiasis salmonella shigella EHEC (supportive care)

Question 73

scrub typhus

Answer 73

orientia tsutsugamushi chigger vasculitis like illness with regional or generalised lymphadenopathy and an eschar hepatic and renal failure, myocarditis and meningoencephalitis treatment - doxy

Question 74

HHV3 HHV4 HHV5

Answer 74

VZV EBV CMV

Question 75

JE typically affects

Answer 75

thalamus, basal ganglia, brainstem

Question 76

how does TB cause hypoadrenalism

Answer 76

adrenalitis, haematogenous spread -> progressive destruction of the glands

Question 77

dex in bacterial meningitis

Answer 77

purulent CSF WCC >1000 raised WCC + protein count >1 bacteria on gram stain

Question 78

schisto treatment

Answer 78

praziquantel fever myalgia cough diarrhoea urticarial rash and hsmeg with eosinophilia and pulmonary isolates = Katayama fever

Question 79

Klebsiella associations

Answer 79

cardiomyopathies alcoholics upper lobe involvement

Question 80

visceral larva migrans mucocutaneous larva migrans larva currens

Answer 80

toxocara canis anylostoma braziliense strongyloides stercoralis

Question 81

differentiate MRSA

Answer 81

with rapid PCR vanc > teic > linez

Question 82

clostridia

Answer 82

tetani - spastic paralysis perfringens - skin inf and gas gangrene difficile - diarrhoea sordelii - post partum or termination sepsis

Question 83

diagnosis of bartonella

Answer 83

serology culture histopath with Warthin-Starry stain

Question 84

moa of trimethoprim

Answer 84

dihydrofolate reductase inhibitor ADR - myelosuppression transient rise in creatinine, blocks ENaC in distal nephron causing a hyperkalaemia distal RTA

Question 85

treatment of HCV

Answer 85

PI (daclatasvir + sofosbuvir OR sofosbuvir + simeprevir) with or without ribavirin

Question 86

screening for HIV

Answer 86

HIV 1/2 Ab/Ag immunoassay

Question 87

pneumonia associations

Answer 87

Pneumonia + Alcoholic + Cavitation = Klebsiella Pneumonia + cavitation = Staph, Klebsiella Pneumonia + Prior Flu = Staph Pneumonia Pneumonia + Chicken Pox Rash = Varicella Pneumonia + Hemolytic Anemia = Mycoplasma Pneumonia + Hyponatraemia + Travel History = Legionella Pneumonia + Fleeting opacities = Cryptogenic Pneumonia Pneumonia + Fits/LOC = Aspiration Pneumonia Pneumonia + HSV oral lesion = Strep Pneumonia Pneumonia + parrot = Chlamydia psitatssi Pneumonia + farm animals = Q fever (coxillea brunetii) Pneumonia + HIV = think pcp but if straight forward case strep pneumonia is still most common Pneumonia + Cystic fibrosis = consider pseudomonas/ Burkholderia Pneumonia + COPD or exac = c1::Haemophilus Influenza Commonest cause of CAP = Strep Pneumonia

Question 88

shortest replication time of non falcip malaria

Answer 88

P knowlesi -> 24 hour cyclical fever syndrome

Question 89

erythema multiforme erythema migrans marginatum

Answer 89

mycoplasma pneumonia lyme rheumatic fever

Question 90

IRIS

Answer 90

paradoxical worsening of pre-existing chronic or latent condition

Question 91

Lyme disease test

Answer 91

ELISA for IgM or G if no rash

Question 92

factors which reduce vertical transmission

Answer 92

maternal antiretroviral therapy C section neonatal ART infant bottle feeding

Question 93

neonatal ART

Answer 93

zidovudine infusion during C sec if <50 then zidovudine orally to neonate otherwise triple ART and cont for 4-6 weeks

Question 94

disseminated gonorrhoea triad

Answer 94

dermatitis migratory polyarthritis tenosynovitis

Question 95

New-delhi metallo-beta-lactamase 1 mutation that leads to

Answer 95

carbapenem resistance mx - colistin, tigecycline

Question 96

D alanyl D lactate variation

Answer 96

mechanism of VRE

Question 97

penicillin binding protein 2 alteration

Answer 97

MRSA MEC gene

Question 98

Brucellosis

Answer 98

animals or unpasteurised dairy serology (RBT) -> STA -> ELISA fluctuating temperatures, transient arthralgia, myalgia and hypergidrosis with a wet hay smell

Question 99

pus cells on urethral swab

Answer 99

NSU doxy oral

Question 100

echinococcus tx

Answer 100

albendazole

Question 101

sodium stibogluconate

Answer 101

leish

Question 102

Monod's or air crescent sign

Answer 102

aspergilloma vs improving angioinvasive aspergilloma mobile mass - prone position

Question 103

lassa fever rx

Answer 103

ribavirin

Question 104

ifn a ifn b ifn g

Answer 104

Hep B MS TB test

Question 105

TB hypersens

Answer 105

Type 4 hypersensitivity

Question 106

virus known to cause a CSF result with low glucose

Answer 106

mumps

Question 107

severe transaminitis in the first trimester

Answer 107

Hep E infection thrombocytopaenia and hx of undercooked meat

Question 108

HHVs

Answer 108

herpes simplex virus 1 and 2 -> cold sores, genital herpes —> VZV - HHV 3 -> chickenpox, shingles —> CMV (HHV 5) -> Glandular fever, CMV retinitis / colitis / oesophagitis in HIV — >HHV 6 -> Glandular fever, roseola infantum —> EBV (HHV 4) : Glandular fever, lymphoma, nasopharyngeal CA —> HHV 7 -> Glandular fever, roseola infantum —> HHV 8 -> Kaposi sarcoma

Question 109

needle stick transmission

Answer 109

Hep B 20-30% HIV 0.2% Hep C - 2% Vertical transmission Hep C - 6% Sexual intercourse Hep C - 2%

Question 110

oseltamavir moa

Answer 110

neuraminidase inhibitor flu A and B

Question 111

tetanospasmin

Answer 111

GABA

Question 112

exclude HIV seroconversion illness

Answer 112

p24 antigen

Question 113

meningitis with pen allergy

Answer 113

chloramphenicol

Question 114

MAC infection

Answer 114

clari + ethambutol rifampicin as third azithro

Question 115

which film identifies parasite burden in malaria

Answer 115

thick films

Question 116

cold agglutinin disease

Answer 116

Mycoplasma pneumoniae

Question 117

fever headaches rash on trunk and limbs with sparing of palms and soles exposure to vermin

Answer 117

typhus doxy

Question 118

non keratinised vs keratinised warts

Answer 118

podophyllum vs cryotherapy

Question 119

listeriosis

Answer 119

diarrhoea flu like illness meningoencephalitis, ataxia, seizures tumbling motility in blood cultures pleocytosis in CSF, predom lymphocytes amox/ampi IV amox + gent in elderly

Question 120

p450 ART

Answer 120

nevirapine induces PI inhibit

Question 121

IVDU with descending paralysis, diplopia and bulbar palsy, slurred speach, dysphagia, ptosis and facial muscle weakness no fever or loss of sensation or loss of awareness

Answer 121

C botulinum

Question 122

spiramycin

Answer 122

reduces risk of toxo transmission from mother to foetus

Question 123

rhabditiform larvae

Answer 123

strongy

Question 124

orf

Answer 124

contagious ecthyma Parapoxvirus

Question 125

tularaemia

Answer 125

zoonotic infection involving F tularensis rodents and ticks papulo-ulcerative lesion at site of bite with reactive and ulcerating regional lymphadenopathy doxycycline rx

Question 126

typhoid

Answer 126

abdo pain constipation delirium/encephalitis abdo perf leukopenia rose spots relative bradycardia (Faget sign) test of choice - large volume blood culture Widal test IV ceftriaxone empirical therapy chronic carrier status in the gallbladder

Question 127

adjunctive therapy for mod-severe PCP (po2 <8 or Aa gradient >35)

Answer 127

steroids

Question 128

multibacillary lep

Answer 128

rifamp+dapsone+clofazimine

Question 129

yellow fever vaccine contraindication

Answer 129

egg protein

Question 130

conjugate vaccine examples

Answer 130

Men ACWY Men B Pneumococc HIB Meningococc

Question 131

most common infective cause of diarrhoea in HIV

Answer 131

cryptosporidium

Question 132

vancomycin AEs

Answer 132

oto and nephrotoxicity red man syndrome - flushing and maculopapular rash due to non IgE mediated mast cell degranulation -> antihistamines

Question 133

most effective intervention for MRSA in hospitals

Answer 133

hand washing

Question 134

genital ulcers STIs

Answer 134

painless ulcer painless LN = syphilis Single painless ulcer painful LN = LGV Indolent painless ulcers 'regional lymphadenopathy is rare' >> Granuloma inguinale Multiple painful ulcer with ragged edges painful LN = chancroid H. ducreyi Multiple painful blisters/ulcers with tingling pain neuropathic type pain and tender inguinal lymph nodes + systemic symptoms= Genital herpes simplex

Question 135

cause of erysipelas

Answer 135

GAS - pyogenes

Question 136

acute toxo detection

Answer 136

serology with IgM rising 5-14 d, peak at 2-3mo

Question 137

vaccines always contraindicated in HIV

Answer 137

2 GI infection -> cholera, polio 2 lung infection -> BCG (TB), intranasal Influenza

Question 138

contraindicated at CD4 <200 in HIV

Answer 138

yellow fever MMR chicken pox

Question 139

drug causes of thrombocytopaenia FANQAAH

Answer 139

furosemide antibiotics (PSR) NSAIDs Quinine abciximab anti-convulsants (carb, valp) heparin

Question 140

drugs causing impaired glucose tolerance

Answer 140

thiazides, furosemide steroids IFNa tacrolimus cyclosporin nicotinic acid

Question 141

drugs causing urinary retention

Answer 141

opioids NSAIDs anti-cholinergics dipyridamole TCAs

Question 142

alpha 1 agonists

Answer 142

phenylephrine oxymetazoline

Question 143

alpha 2 agonists

Answer 143

brimonidine

Question 144

alpha 1a antagonist

Answer 144

tamsulosin

Question 145

alpha 1 antagonist

Answer 145

doxazosin

Question 146

beta 1 agonist

Answer 146

dobutamine non sel - aten/bisop

Question 147

beta 2 agonist

Answer 147

salbutamol

Question 148

dopamine agonists

Answer 148

bromocriptine cabergoline ropinirole

Question 149

dopamine antagonists

Answer 149

haloperidol metoclopramide domperidone

Question 150

GABA agonist

Answer 150

BZD baclofen

Question 151

GABA antagonist

Answer 151

flumazenil

Question 152

histamine-1 antagonist

Answer 152

antihistamines - loratadine, urticaria, DRESS

Question 153

histamine-2 antagonist

Answer 153

ranitidine

Question 154

muscarinic agonist

Answer 154

pilocarpine

Question 155

muscarinic antagonist

Answer 155

atropine ipratropium/tiotropium oxybutynin

Question 156

nicotinic agonist

Answer 156

varenicline suxamethonium

Question 157

nicotinic antagonist

Answer 157

atracurium

Question 158

oxytocin agonist

Answer 158

syntocin

Question 159

oxytocin antagonist

Answer 159

atosiban

Question 160

serotonin agonist

Answer 160

triptan ergotamine - partial agonist

Question 161

serotonin antagonist

Answer 161

ondansetron pizotifen methesergide cyproheptadine (control of diarrhoea in carcinoid)

Question 162

drugs causing lung fibrosis

Answer 162

amiodarone bleomycin, busulphan nitrofurantoin ergot derived dopamine agonists - bromocriptine, cabergoline, pergolide

Question 163

drugs causing ocular problems

Answer 163

* Cataracts - Steroids * Corneal opacities - Amiodarone (vortex keratopathy, night time glare), Indomethacin * Optic neuritis - Ethambutol (anti TB), Amiodarone, Metronidazole * Retinopathy - Chloroquine (anti malarial), Quinine (anti malarial)

Question 164

Drugs causing photosensitivity

Answer 164

* Thiazides * Tetracyclines, sulphonamides, ciprofloxacin * Amiodarone - hypothyroidism, hyperthyroidism type 1 (antithyroid drugs), hyperthyroidism type 2 (corticosteroids), photosensitivity, corneal opacities, lung fibrosis, optic neuritis * NSAIDs - Piroxicam * Psoralen’s * Sulphonylureas - Metformin

Question 165

clinical features of ecstasy overdose

Answer 165

CNS symptoms - agitation, anxiety, confusion, ataxia, dilated pupils, lock jaw, bruxism hyponatraemia hyperthermia rhabdomyolysis seizures acute renal failure acute psychosis supportive mx -> dantrolene

Question 166

ethylene glycol poisoning

Answer 166

stage 1 - confusion, slurred speech, dizziness stage 2 - metabolic acidosis with high anion gap and high osmolar gap, tachycardia, hypertension stage 3 - AKI mx - fomepizole -> inhibits alcohol dehydrogenase

Question 167

methanol poisoning

Answer 167

intoxication, nausea visual problems - blindness, optic neuropathy fomepizole ethanol haemodialysis folinic acid to reduce visual complications

Question 168

monoclonal antibodies

Answer 168

* Infliximab : anti TNF - Rh arthritis, Crohn’s * Rituximab :anti CD20-Rh arthritis, Non-Hodgkin’s Lymphoma * Cetuximab : Epidermal growth factor receptor antagonist - Metastatic colon cancer, Head & Neck cancer * Trastuzumab : HER2/neu receptor antagonist - metastatic breast disease * Alemtuzumab : anti CD52 - Chronic lymphocytic leukaemia * Abciximab : Glycoprotein IIb/IIIa receptor antagonist - Percutaneous coronary interventions Abiciximab - can cause thrombocytopenia * OKT3 : anti CD3 - used to prevent organ rejection Cetucoxib & Panitumumba - metastatic colon cancer Infliximab and Adalimumab - Crohn’s disease

Question 169

mercury poisoning albacore tuna

Answer 169

visual field defects sensorineural hearing loss paraesthesia peripheral neuropathy hyperchloraemic metabolic acidosis consistent with RTA irritability avoid chelation

Question 170

lead poisoning

Answer 170

peripheral neuropathy constipation vomiting blue lines on gums (Burtons) lead paint in house

Question 171

manganese poisoning

Answer 171

visual hallucinations and anxiety later -> mimics Parkinson's disease fume inhalation working with steel

Question 172

zinc poisoning

Answer 172

GI distress, abdo pain, nausea chronic - spastic gait, sensory ataxia

Question 173

octreotide moa

Answer 173

somatostatin analogue used in acute variceal bleeding treatment, acromegaly, carcinoid syndrome, VIPomas, refractory diarrhoea, following pancreatic surgery causes biliary stasis -> gallstones

Question 174

treatment of oculogyric crisis

Answer 174

- Benztropin / Procyclidine / Trihexiphenidyl

Question 175

zero order kinetics

Answer 175

warfarin aspirin alcohol theophylline tolbutamide phenytoin salicylates heparin ethanol

Question 176

first pass metabolism

Answer 176

* Aspirin * Isosorbide dinitrate, Isosorbide trinitrate * Glyceryl trinitrate * Lignocaine AKA Lidocaine * Propranolol * Verapamil * Isoprenaline * Testosterone * Hydrocortisone - Morphine

Question 177

Phase 1 reactions

Answer 177

oxidation reduction hydrolysis deamination dealkylation

Question 178

Phase 2 reactions

Answer 178

conjugation

Question 179

drugs affected by acetylator status

Answer 179

* Isoniazid (slow - per neur, rapid - hepatitis) * Dapsone * Procainamide * Hydralazine * Sulfasalazine

Question 180

PDE5 inhibitors

Answer 180

causes vasodilation through increase in cGMP leading to smooth muscle relaxation Sildenafil - Viagra : short acting, 1 hour before sexual activity Tadalafil - Cialis : longer acting than Sildenafil, once daily Contraindications : 1. Patient taking nitrates and related drugs such as Nicorandil 2. Hypotension 3. Recent stroke or MI - wait period of 6 months Uses - Erectile dysfunction, Pulmonary Hypertension Adverse - blue discolouration, non arteritic anterior ischemic optic neuropathy nasal congestion flushing GI side effects headache Priapism

Question 181

cause of hepatocellular damage in paracetamol OD

Answer 181

depletes glutathione stores causing accumulation of NAPQI

Question 182

medications and receptors

Answer 182

G protein coupled receptor - Opioid receptor, Beta receptors Ligand gated ion channels - nicotinic acetylcholine, GABA receptors Tyrosine kinase - Insulin Nuclear receptors - Hydrocortisone, Levothyroxine

Question 183

types of potassium sparing diuretics

Answer 183

* Epithelial Sodium Channel Blockers A.Amiloride : blocks the Epithelial Na channel in DCT; weak diuretic, usually given in combination with thiazide or loop to counter hypokalaemia B.Triamterene * Aldosterone antagonists - Spironolactone, Eplerenone a) Spironolactone : acts in cortical collections duct i) Ascites - patients with cirrhosis develop secondary hyperaldosteronism ii) Heart failure iii)Nephrotic syndrome iv)Conn’s syndrome

Question 184

drugs to avoid in renal failure

Answer 184

antibiotics - tetra, nitro NSAIDs lithium metformin

Question 185

quinine toxicity

Answer 185

tinnitus, visual blurring, flushed dry skin, abdominal pain, visual defects arrhythmia - prolonged QRS and QT -> VF hypoglycaemia flash pulmonary oedema supportive mx, bicarc, CPAP

Question 186

cipro ECG

Answer 186

QT prolongation

Question 187

salicylate overdose

Answer 187

A.Hyperventilation B.Tinnitus C.Lethargy D.Sweating, pyrexia E. Nausea, vomiting F. Hyperglycaemia & hypoglycaemia G.Seizures H.Coma mixed respiratory alkalosis + metabolic acidosis Early stimulation of respiratory centre - rest. Alkalosis Direct renal effect of salicylates with acute renal failure - acidosis MX : charcoal if within 1 hour, urinary alkalisation with IV HCO3, hemodialysis Indications for hemodialysis in salicylate overdose - * Serum concentration >700 mg/L * Metabolic acidosis resistant to treatment * Acute renal failure * Pulmonary edema * Seizures * Coma

Question 188

serotonin syndrome

Answer 188

* Neuromuscular excitation - hyperreflexia, myoclonus, rigidity * ANS excitation - hyperthermia, sweating * AMS - confusion * Increased reflexes, clonus, normal pupil size IV fluids cyproheptadine chlorpromazine

Question 189

NMS

Answer 189

* Tachycardia, hypertension, pyrexia, diaphoresis * Increased Creatine kinase * Slower onset * Decreased reflexes, lead pipe rigidity, normal pupils * Mx - Dantrolene

Question 190

side effects of anti-anginal drugs

Answer 190

1. CCB - Headache, flushing, ankle oedema; Verapamil - constipation 2. Beta blockers : bronchospasm (asthma), fatigue, cold peripheries, sleep disturbances 3. Nitrates - headache, postural hypotension, tachycardia 4. Nicorandil - headache, flushing, anal ulceration

Question 191

side effects of diabetes drugs

Answer 191

a) Metformin - GI side effects (diarrhoea, nausea), lactic acidosis Page 61 b) Sulfonylureas (glimepiride) - hypoglycaemia, increased appetite, weight gain, SIADH, liver dysfunction (cholestatic) c) Glitazones (Pioglitazone) - weight gain, fluid retention, liver dysfunction, fractures (CI in heart failure) d) Gliptins - pancreatitis

Question 192

QT shortening

Answer 192

Hypercalcemia, Hypermagnesemia, Digoxin, Thyrotoxicosis

Question 193

QT Prolongation - MATCHES OPQ

Answer 193

M - Methadone/Metocloprmaide A - Amiodarone T - TCA (Citalopram, Escitalopram); Tefenadine/Tramadol, C - Chloroquine/Clarithromycin H - Haloperidol E - Erythromycin S - Sotalol O - Ondansetron P - Procainamide Q - Quinolones

Question 194

illegal drugs ODs

Answer 194

Cocaine overdose - palpitations, agitation, chest pain, mydriatic pupils, elevated pulse rates Cocaine - sympathomimetic drug which stimulates the release of Noradrenaline and dopamine. Causes increased HR, increased BP and vasoconstriction. Coronary vasospasm - acute chest pain Cannabis overdose - relaxation, euphoria, slowed reaction time, red eyes LSD overdose - mydriasis, altered mental state Heroin - respiratory depression, mitosis, sedation, hypotension, bradycardia Ketamine - dissociative anaesthetic agent by antagonist to NMDA receptors in brain

Question 195

motion sickness preferred meds

Answer 195

hyoscine > cyclizine > promethazine

Question 196

calcineurin

Answer 196

IL-2 release

Question 197

tamoxifen ADR

Answer 197

* Menstrual disturbances - vaginal bleeding, amenorrhea * Hot flushes * Venous thromboembolism * Endometrial cancer (due to partial agonist activity on oestrogen receptors causing endometrial hyperplasia and increased risk of neoplasia)

Question 198

teratogens

Answer 198

A.ACE inhibitors - renal dysgenesis, crania-facial abnormalities B.Alcohol - cranio-facial abnormalities C.Aminoglycosides - ototoxicity D.Carbamazepine - Neural tube defects, craniofacial abnormalities E. Chloramphenicol - Gray baby syndrome F. Cocaine - IUGR, preterm labor G.Lithium - Ebstein anomaly (atrialized right ventricle) H.Maternal DM - Macrosomia, Polyhydramnios, Preterm labor, caudal regression, neural tube defects I. Diethylstilbestrol - vaginal clear cell adenocarcinoma J. Smoking - preterm labor, IUGR K.Tetracylines - discoloured teeth L. Thalidomide - limb reduction defects M.Valproate - neural tube defects N.Warfarin - craniofacial abnormalities

Question 199

monitoring for ADRs

Answer 199

Retinopathy - Hydroxychloroquine, Ethambutol, Vigabatrin, AMiodarone BP monitoring - Cyclosporin Urine analysis - Gold, Penicillamine (both to look for protein due to increased risk of membranous glomerulonephritis) Urine analysis - Cyclophosphamide (blood due to risk of hemorrhagic cystitis & bladder cancer) Therapeutic levels - Tacrolimus, Cyclosporin

Question 200

HIT diagnosis

Answer 200

serotonin release assay or Hep-PF4 ab ELISA

Question 201

macrolides AE

Answer 201

* QT prolongation * GI side effects are common * Cholestatic jaundice * P450 inhibitor * Azithromycin - hearing loss, tinnitus

Question 202

PDGFR ab

Answer 202

imatinib dasatinib

Question 203

carbon monoxide poisoning oxygen dissociation curve

Answer 203

shifts to the left downward

Question 204

amitriptyline

Answer 204

increase serotonin and noradrenaline Blocks histamine, cholinergic and alpha 1 receptors TCA - cardiotoxic prolongs QT internal and widened QRS complexes leading to ventricular arrhythmia and seizures. Also causes metabolic acidosis. Overdose of amitriptyline - anti cholinergic effects * Dilated pupils * Dry skin * Confusion * Uriary retention * Tachycardia

Question 205

organophosphate poisoning

Answer 205

over-activation of the parasympathetic nervous system - excessive urination, defaecation, borderline bradycardia, miosis, diaphoresis

Question 206

drugs worsening heart failure

Answer 206

1. Pioglitazone 2. NSAIDs 3. Non Dihydropyridene CCB - Verapamil, DIltiazem 4. Non cardio selective Beta blockers 5. Alpha blockers used for urological problems

Question 207

Cholinergic overdose symptoms - DUMBBELLS

Answer 207

* D - diarrhoea * U - Urination * M - Miosis * B - Bradycardia * B - Bronchospasm * E - Emesis * L - Lacrimation * L - Lethargy * S - Salivation, Sweating SLUDGE - Salivation, Lacrimation, Urination, Defecation, GI Upset, Emesis

Question 208

drugs causing photosensitivity

Answer 208

P - phenothiazines Q - Quinine, Quinolones, Quinidine R - Retinoids S - Sulphonamides, Sulfonylureas T - Tetracyclines, Thiazides A - Amiodarone N - NSAIDs - Piroxicam, Celecoxib D - Dapsone C - Chloroquine, Chlorpromazine N - Nalidixic acid G - Griseofulvin

Question 209

tacrolimus glucose

Answer 209

NODAT

Question 210

Flecainide moa

Answer 210

vaughan williams class 1c anti-arrhythmic blocks Nav1.5 sodium channel widened QRS and prolonged PR not to be used in structural heart

Question 211

precipitating factors for digoxin tox

Answer 211

- Hypokalaemia * Increasing age * Renal failure * Myocardial ischaemia * Hypomagnesemia * Hypercalcemia * Hypernatremia * Acidosis * Hypothermia * Hypothyroidism * Drugs - Amiodarone, Quinidine, Verapamil, Diltiazem, Spironolactone, Ciclosporin, Thiazides, Loop diuretics

Question 212

drug causes of low Mg

Answer 212

- Drugs - diuretics, PPI * TPN * Diarrhoea * Alcohol * Hypokalaemia * Hypercalcemia - secondary to hyperparathyroidism * Metabolic disorders - Gitelman’s and Bartter’s.

Question 213

osmolar gap formula

Answer 213

2xNa + glucose + urea (<10)

Question 214

thallium poisoning

Answer 214

GI upset delirium convulsions coma MODS

Question 215

iron poisoning

Answer 215

vomiting diarrhoea hepatotoxicity bowel obstruction multi organ failure myocardial siderosis metabolic acidosis (lactic) Diagnosis : * Serum iron levels >500 micro gram/dL - aggressive * Abdominal X ray may reveal radiopaque pills * Glucose - elevated due to hepatic dysfunction - can cause hypoglycaemia later * FBC - anemia * LFT - hepatic dysfunction Serum iron levels >90 —> moderate to sever toxicity Management : * Mld GI symptoms with serum concentration < 500 —> Observe * IV Fluids * IV Deferroxamine : binds ferric iron and promotes urinary excretion. Indications of Deferroxamine : 1) severe symptoms - AMS, hemodynamic instability, persistent diarrhoea and vomiting 2) Anion gap metabolic acids 3) Serum iron >500 4) Significant number of pills on radiography

Question 216

drugs precipitating AIP

Answer 216

* Barbiturates * Halothane * Benzodiazepines * Alcohol * Oral contraceptive pill (by increasing synthesis of porphyrin precursors, as they increase demand for hepatic cytochrome P450 enzyme system which in turn increases heme synthesis and depletes heme stores to provoke an attack of acute intermittent porphyria) * Sulphonamides

Question 217

nivoluman moa

Answer 217

PD-1 inhibitor hypophysitis, hypothyroidism

Question 218

EGFR PDGR VEGF ALK-1 CTLA-4

Answer 218

erlotinib olaratumab bevacizumab crizotinib ipilimumab

Question 219

adrenaline induces

Answer 219

hyperglycemia, hyperlactatemia and hypokalaemia

Question 220

monoclonal ab constant region

Answer 220

Fc

Question 221

drugs cleared by haemodialysis

Answer 221

BLAST B - barbiturate L - lithium A - alcohol S - salicylates T - theophyllines

Question 222

erythromycin effect on stomach

Answer 222

motilin receptor agonist - stimulates gastric emptying

Question 223

agranulocytosis drug causes

Answer 223

* Anti thyroid drugs - Carbimazole, Propylthiouracil * Anti psychotics - atypical anti psychotics * Anti epileptics - Carbamazepine * Antibiotics - Penicillin, Chloramphenicol, Co-trimoxazole * Anti depressant - Mirtazapine * Cytotoxic drugs - Methotrexate

Question 224

normal anion gap metabolic acidosis

Answer 224

Addison's Bicarbonate loss: GI (e.g. diarrhoea) or renal (e.g. renal tubular acidosis) Chloride excess Diuretics (e.g. acetazolamide) Diarrhoea, vomiting * Pancreatic fistula * Renal bicarbonate loss * Kidney disease * Hypoaldosteronism * Acetazolamide - causes renal sodium and HCO3 loss * Renal tubular acidosis

Question 225

allopurinol hypersensitivity

Answer 225

HLAB*5801 allele RFs - diuretic use, ethnicity, CKD DRESS syndrome

Question 226

ethambutol ocular SE

Answer 226

neuritis central scotoma red-green clind

Question 227

finasteride moa

Answer 227

5 alpha reductase inhibition

Question 228

NSAID overdose

Answer 228

seizures acidosis renal impairment GI haemorrhage CNS effects

Question 229

digoxin loading dose

Answer 229

volume of distribution

Question 230

warfarin loading dose

Answer 230

rapid onset of action

Question 231

what increases the risk of heart valve regurgitation in patients who use fluoroquinolones

Answer 231

RA

Question 232

raised anion gap metabolic acidosis

Answer 232

* Methanol poisoning * Uraemia * Diabetic ketoacidosis * Paraldehyde * Isoniazid, iron overload * Lactic acidosis (can be due to metformin) * Ethylene glycol intoxication * Salicylate overdose

Question 233

effects of anabolic steroids

Answer 233

BP elevation increased LDL decreased HDL prolonged -> haematocrit increase, prothrombotic + plasma fibrinogen decreases

Question 234

heroin withdrawal sx

Answer 234

Sweating; diarrhoea; nausea; vomiting; irritability; restlessness; muscle ache MX : methadone or buprenorphine maintenance programs to prevent dangers of heroin withdrawal

Question 235

meds and cancers

Answer 235

Ethanol - fetal alcohol syndrome Diethylstilbestrol - vaginal carcinoma Phenobarbital - cleft lip Thalidomide - phocomelia Androgen - deformities

Question 236

drugs contraindicated during breast feeding

Answer 236

* Amiodarone * Anti neoplastics * Chloramphenicol * Ergotamine * Iodides * Methotrexate * Lithium * Tetracyclines * Cabergoline * Pseudoephedrine * All recreational drugs * Clozapine (fetal agranulocytosis) * BZD - cleft lip, cleft palate

Question 237

fungal infections

Answer 237

— Oral candidiasis - Miconazole oral gel or nystatin mouthwash ; severe infection - oral fluconazole — Oesophageal candidiasis - ketoconazole — tinea infection : Terbinafine topical — invasive aspergillosis - Amphotericin B (nephrotoxic) — Aspergilloma : oral voriconazole — cryptococcal meningitis : amphotericin B

Question 238

drugs increasing concentration of ciclosporin

Answer 238

* FLuconazole * Ketoconazole * Itraconazole * Voriconazole * Methyl prednisolone high dose * Allopurinol * Protease inhibitors * Colchicine

Question 239

doxorubicin moa

Answer 239

anti tumor activity derives from triggering of topoisomerase II dependent DNA breakage Cardiotoxicity * Cardiomyopathy * Acute arrhythmia * Congestive heart failure * Dose dependent impairment of LVEF * Myocardial dysfunction.

Question 240

eplerenone adrs

Answer 240

hyperkalaemia dizziness hypotension diarrhoea nausea

Question 241

vigabatrin ADRs

Answer 241

aggression, alopecia, retinal atrophy, reduced peripheral vision

Question 242

vincristine features

Answer 242

Peripheral neuropathy - neurotoxic feature C/F : Foot drop, parenthesis, loss of ankle jerks, wrist drop. Vincristine - biochemical picture resembling syndrome of inappropriate antidiuretic hormone (SIADH) & hyponatremia. Other adverse effects - mucositis of GIT and bleeding Bleomycin - interstitial pneumonia Cyclophosphamide - hemorrhagic cystitis Doxorubicin and Daunorubicin - cardiomyopathy Methotrexate - hepatic and pulmonary fibrosis

Question 243

actions of insulin

Answer 243

* Promotes glucose uptake * Inhibits glucose generation or release * Inhibits glucose converstion into storage molecules - glycogen and triglycerides * Increases protein synthesis * Promotes glycogen synthesis * Inhibits gluconeogenesis * Promotes lipogenesis * Decreases serum potassium levels.

Question 244

amphetamine misuse

Answer 244

C/F : enhanced mood, excessive speech, increased energy, insomnia Sign : hypertension, dilated pupils, dry mucous membranes Complication : Paranoid psychosis with prolonged use Clinical uses - treating narcolepsy and ADHD

Question 245

tx for psychedelic OD

Answer 245

distorted sensory perception, hallucination, out of body experiences, panic/agitation in severe cases Mx - BZDs

Question 246

what Ca channels do CCBs work on

Answer 246

L type

Question 247

HIV lipodystrophy

Answer 247

insulin resistance pattern loss of fat on face increasing abdominal fat deposition of subcut fat on back 1) low HDL and High triglyceride levels 2) hypertension 3) impaire glucose tolerance precedes the development of type 2 DM.

Question 248

PNH mechanism

Answer 248

clonal red cell abnormality, leaves erythrocytes open to complement mediated attack. Can occur in paroxysms resulting in hemolysis. Eculizumab - orphan drug for PNH which inhibits C5 - stops complement mediated erythrocyte destruction

Question 249

TCA overdose

Answer 249

Side effects - Anti cholinergic - dry mouth, tachycardia, mydriasis Sodium channel blockade - QRS prolongation, arrhythmia, seizures Others - grossly abnormal reflexes, loss of consciousness, divergent eye movements

Question 250

theophylline adverse effects

Answer 250

nausea, vomiting, hypokalaemia, metabolic acidosis Regular smoking - u-pegulates hepatic enzyme activity and cessation of smoking causes increased theophylline concentrations. Other factors that increase theophylline concentrations - heart failure, liver cirrhosis, acute viral infection, increased age, enzyme inhibitor or inducer.

Question 251

polymorphic VT

Answer 251

* Broad complex tachycardia - numerous ventricular foci that leads to polymorphic QRS complexes * Most common type - tornadoes de pointes * Aetiology : A.Inherited B.MI C.Drug induced D.Congenital Long QT syndrome - Romano Ward, Jervell and Lange-Nielson syndrome E. Electrolyte disturbances - low K, low Mg, Low Ca * Torsades de pointes : prolonged QT interval indicates longer time needed for myocytes to repolarise due to channelopathy - causing premature after-depolarisations. * C/F : light headedness, palpitations, dizziness, sudden cardiac death. Can progress to VF or cardiac arrest. * Bidirectional VT - DIGOXIN * MX : IV Magnesium is the drugo choice : 1-2g in 30 to 60 seconds, repeated in 5-10 minutes. * Avoid these drugs A.Anti arythmie - Amiodarone, Sotalol, Procainamide B.Anti psychotics - Haloperidol C.Antidepressants - Citalopram D.Antibiotics - Erythromycin, Trimethoprim E. Antihistamines - Terfinadine F. Antifungals - Ketoconazole, Fluconazole

Question 252

digoxin specific antibodies

Answer 252

digoxin tox with severe hyperkalaemia

Question 253

diagnosis of SIADH

Answer 253

Confirming requires serum osmolality <275 and urine osmolality >100 and Urine sodium >30 - concentrated urine Na >20 - hyperosmolality >100 mOsm/k - hyponatremia plasma Na <125 mmol/L - absence of hypovolemia/edema/diuretics

Question 254

warfarin decreased efficacy

Answer 254

Pharmacokinetic factors : * Cholestyramine - decreased absorption * CY450 inducers - St Johns Wort Pharmacodynamic factors : * Excess dietary vitamin K * Oestrogen’s * Griseofulvin * 6-mercaptopurine (Azathioprine)

Question 255

tumour lysis syndrome

Answer 255

* Metabolic disorder - complication of malignant diseases * Hyperuricaemia + hyperkalemia + hyperphosphotemia * Hypocalcemia * Raised urea and creatinine levels due to acute renal failure * Hyperuricemia - crystallisation of uric acid in renal tubules - obstructive uropathy * Associated with steroid administration, monoclonal antibodies, biologics - Thalidomide, Paclitaxel, Zoledronic acid * Hypocalcemia - arrhythmia, tetany, seizures * C/F : - Dyspnea, orthopnea, tachypnea - Dyusira, flank pain, hematuria - Nausea, vomiting, seizures, tetanic spasms - Abdominal distension - Facial edema - Palpitations - Lethargy * Management : Rapid expansion of intravascular volume - IV fluids started 48 hours before initiation of chemotherapy * Allopurinol - can decrease production of uric acid - used in low risk patients * Recombinat urate oxidase : Rasuricase - treats hyperuricemia associated with leukaemia and lymphoma - used in high risk patients * Sodium Bicarbonate - for urine alkalinisation * Calcium - Calcium chloride, calcium glutinate

Question 256

drugs causing nephrogenic DI

Answer 256

* Lithium * Demeclocycline * Amphotericin * Glibenclamide

Question 257

methaemoglobinaemia cause

Answer 257

isosorbide mononitrate

Question 258

coronary artery vasospasm in cocaine OD

Answer 258

nitrates or CCB

Question 259

drugs causing hyperprolactinaemia

Answer 259

* Dopamine receptor antagonists - Domperidone, Metoclopramide * Antidepressants - TCA, MAO inhibitors, SSRI * CCB - Verapamil * Opiates * Protease inhibitors * Bezafibrate * H2 receptor antagonists * Oestrogens, anti androgens * Cocaine * Cyproheptadine

Question 260

drug induced hepatitis

Answer 260

* Febofibrate * Anti TB drugs * Methyldopa * Carbamazepine * Nitrofurantoin * Isoniazid

Question 261

Inhibitors of CYP 3A4 with CIclosporin - increased risk of nephrotoxicity

Answer 261

- Verapamil * Diltiazem * Imidazole anti fungals * Macrolide antibiotics

Question 262

green blue urine brown urine

Answer 262

* Pseudomonas urinary tract infection * Amitriptyline * Indigo carmine * IV cimetidine * IV promethazine * Methylthioninium Chloride * Triamterene * Levodopa * Metronidazole * Nitrofurantoin * Fava beans

Question 263

drugs associated with intracranial HTN

Answer 263

tetracyclines amiodarone OCP withdrawal of steroid

Question 264

carbimazole ADR

Answer 264

Carbimazole — converts into Methimazole —> inhibits iodination of tyrosine and blocks the coupling of Iodotyrosine. Methimazole : minor immunosuppressive action —> leading to reduction in serum thyroid stimulating hormone antibody (TRAb). Adverse effects - 7 to 28 days after starting treatment - stop the drug if they develop any fever or sore throat. Other rare complications : hepatitis, cholestatic jaundice

Question 265

gp2b/3a inhibitors

Answer 265

Abciximab, Eptifibatide, Tirofiban The receptor mediates platelet aggregation. Inhibited by these drugs - leads to decreased thrombus formation. ADP receptor antagonism - Clopidogrel, Prasugrel, Ticagrelor

Question 266

statin-drug interactions

Answer 266

* Antacid and anti fungal agents interact with Atorvastatin. * All statins interact with Cyclosporine and Nicotinic acid. * All statins should be used in caution with patients on fibrate therapy as the risk of rhabdomyolysis is increased.

Question 267

cytotoxic drug resistance

Answer 267

P-glycoprotein encoded by MDR1 MRP

Question 268

chemotherapy SEs

Answer 268

1. Ciclosporin - nephrotoxicity, hepatotoxicity, increased K, Na, hypertrichosis, gum hypertrophy, increased BP, fluid retention 2. Cyclophosphamide - bone marrow suppression 3. Methotrexate - pulmonary fibrosis, pancytopenia, bone marrow suppression, mucositis 4. Vincristine - neurotoxicity 5. Doxorubicin - cardiotoxicity

Question 269

SEs of salmeterol

Answer 269

hypokalaemia liver disease not used in breast feeding and preg

Question 270

phenytoin is a highly protein bound drug

Answer 270

* Toxicity happens when proportion of free to bound drug are increased. * Factors which increase this proportion - - States of hypoalbuminemia - pregnancy, malnutrition, nephrotic syndrome, malignancy - Uraemia - Neonates - Elderly individuals - Hyperbilirubinemia

Question 271

cytochrome pathways

Answer 271

CYP2CB - pioglitazone 3A4 - simvastatin 2C9 - NSAID and warfarin 2D6 - antipsychotics 3A2 - conversion of testosterone

Question 272

common drugs adrs

Answer 272

Methotrexate - pneumonitis, pulmonary fibrosis, hepatic fibrosis, folate deficiency Ciclosporin - nephrotoxic, cholestasis, hypertrichosis, gingival hyperplasia, tremor, nausea, vomiting Prednisolone - used as treatment for fibrosing alveoli’s Sulfasalazine - can cause pulmonary fiborisi, myelosuppression, decreased sperm count, hepatitis, oral ulcers, rash, haemolytic anemia. Azathiprine - dose related myelosuppression, hair loss, hypotension, cholestatic jaundice, arrhythmia, hypersensitivity reactions

Question 273

theophylline metabolic disturbance

Answer 273

* Metabolic disturbances - hypokalaemia, metabolic acidosis * Theophylline toxicity - hypotension, hypokalaemia, ventricular & supra ventricular arrhythmia

Question 274

AED effects on OCP

Answer 274

* Lamotrigine - most correct choice as it does not interfere with effectiveness of OCP * Carbamazepine - powerful enzyme inducer and decreased OCP effectiveness, and increased risk of unintended pregnancy * Clonazepam - causes sedation * Topiramate - decreases Estrogen levels - reduces pill effectiveness; also has a ten-fold risk of cleft lip compared to other people * Phenytoin - causes weight gain, powerful enzyme inducer - can cause congenital malformations - cleft lip, cleft palate, neural tube defects

Question 275

digoxin overdose

Answer 275

Cardiac features : * A and AV block * Premature ventricular contractions Slowed ventricular response * PR prolongation * scooped ST depression Non cardiac features : * GIT - nausea, vomiting, diarrhoea * Vision - blurred, yellow green discoloration * CNS - weakness, dizziness, confusion, apathy, malaise, headache, depression, psychosis * Haematological - thrombocytopenia, agranulocytosis * Gynecomastia MX : Digoxin specific antibody fragments (Fab) fragments Symptomatic bradycardia - atropine Hypotension - fluid replacement

Question 276

rosuvastatin metabolism

Answer 276

substrate for hepatic uptake transporter OATP1B1 and efflux transporter breast cancer resistance protein (BCRP) CYP3A4 inhibitors impact metabolism of Simvastatin & Atorvastatin - which are affected by CYP3A4 inhibitors like Itraconazole and Clarithromycin ciclosporin inhibitor of OATP1B1

Question 277

cranberry juice

Answer 277

increase in INR due to 2c9 metabolism liver/brassica - decrease INR

Question 278

beta agonists and AT2 lead to

Answer 278

promote photo oncogene expression - which then Stimulates protein synthesis and induces synthesis of fetal forms of actin and myosin, leading to hypertrophy of smooth muscle Beta blockers, ACE inhibitors, ARBs can be used for reversal of hypertrophy

Question 279

drug fever

Answer 279

penicillins, phenytoin, hydralazine, and Quinidine

Question 280

acetazolamide ADs

Answer 280

A.Metabolic acidosis due to HCO3 loss in PCT and DCT through inhibition of reabsorption B.Hypokalaemia C.Acute interstitial nephritis D.agranuloytosis E. Thrombocytopenia

Question 281

paracetamol metabolism key pathways

Answer 281

1) glucuronidation 2) sulfation

Question 282

drug induced haemolysis

Answer 282

* G6PD - Quinolones, dapsone, primaquine, Aspirin * Acquired autoimmune haemolytic anaemia - Methyldopa, Penicillin, Quinine, Quinidine * Lead exposure - can cause direct red cell toxicity

Question 283

antifungals

Answer 283

* Symptomatic patients - hemoptysis - surgical management by lobectomy * Alternative treatments - bronchial arterial embolisation & long term oral Itraconazole. * Pre and post operative systemic anti fungal treatment is also preferred. * Treatment options include - amphotericin with flucytosine/ itraconazole / voraconazole. Fluconazole - candidiasis and CNS infections with Cryptococcus neoformans Griseofulvin - tinea capitis in adults and children; effective against infections caused by Trichophyton tonsurans and Microsporum spp. Nystatin - oral, vaginal and enteric candidiasis; only topical; systemic route too toxic Terbinafine - superficial mycoses - dermatophyte infections

Question 284

site of action of diuretics

Answer 284

* C - carbonic anhydrase inhibitors - proximal convoluted tubule * O - osmotic diuretics - at loop of Henle * L - loop diuretics - ascending limb of loop of Henle * T - thiazide diuretics - at proximal distal convoluted tubule * P - Potassium sparing - at distal portion of distal convoluted tubule

Question 285

drugs causing mydriasis

Answer 285

1) drugs with sympathomimetic actions - cocaine, amphetamine, pseudoephedrine 2) Anticholinergic drugs - antihistamines, atropine, tricyclic antidepressants

Question 286

ciclosporin moa

Answer 286

Two main modes of action - 1. Arrest the activity of resting lymphocytes in G0 or G1 phase of growth cycle 2. Cytokine inhibition - IL-2 * Ciclosporin concetrations affected by renal function and coadministration of drugs that interfere with CYP3A4 enzyme activity. * Contraindicated drugs with Ciclosporin - Statin, Macrolides

Question 287

carbamazepine autoinduction

Answer 287

CYP3A4

Question 288

what drug protects against radiation therapy

Answer 288

amifistone increase sens? * Dactinomycin - radio sensitiser agent which increases cellular response to radiation treatment * Metronidazole * 5-Fluorouracil * Gemcitabine * Cisplatin * Hydroxyurea * Paclitaxel * Mitomycin C * Topotecan

Question 289

Gitelman syndrome metabolic

Answer 289

* Low levels of chloride * Low levels of potassium * Low levels of magnesium * Decreased calcium excretion in urine * C/F : muscle cramps, weakness, numbness, thirst, waking up at night to urinate, salt cravings * Mx : potassium sparing diuretic - Amiloride or Spironolactone

Question 290

loop and thiazide vs CA

Answer 290

metabolic alkalosis vs acidosis

Question 291

drug induced cholestasis

Answer 291

* Phenothiazines - Chlorpromazine * Erythromycin * Ciclosporin * Cimetidine * Nifedipine - Nitrofurantoin * Imipramine * Azathioprine * Dextro-propoxyphene Danazol - prolonged use - can hepatic adenomas or HCC

Question 292

amoebic liver abscess

Answer 292

- Routine Blood tests : raised TLC, deranged LFT; raised inflammatory markers - Amoebic serology : >90% sensitive - Most patients with Amoebic Liver abscess will have negative stool microscopy - Medical Rx : Metronidazole OR Tinidazole

Question 293

coag negative staph

Answer 293

epidermidis positive - aureus

Question 294

rocky mountain spotted fever

Answer 294

* RMSF : febrile illness - Rickettsia rickettsii * History of tick bite 65%, fever 100%, rash 90% of patients * C/F : fever, headache, malaise, myalgia * Macular rash - starts on hands and feet and progresses proximally towards trunk. * Macules evolves into papules, petechiae, ecchymoses. * Severe cases - necrosis & gangrene * Diagnosis - confirmed by 4-fold rise in titres over 2 week period * Treatment : Tetracycline

Question 295

lyme disease tx

Answer 295

doxy/azithro

Question 296

Acrodermatitis chronic atrophicans

Answer 296

dermal atrophy + peripheral nerve involvement + dermal sclerosis * Rare manifestations - keratitis, uveitis * MX : early stages - oral Doxycycline * Later stage - IV Ceftriaxone

Question 297

adverse effects of PI

Answer 297

Hypertrigyceridemia, diarrhoea, asthenia, oral paresthesia, impaired glucose metabolism

Question 298

actinomycosis

Answer 298

* Cervicofacial actinomycosis - most commonly seen * Risk factors : dental extractions, jaw fractures, periodontal abscess, tonsillar crypts * C/F : acute, odontogenic abscess or cellulitis of the floor of mouth, (or) as a painless reddish swelling * Subacute to chronic course : regression of central suppurative foci while the infection progresses peripherally; can spread to other parts of head and neck, can involve meninges. * Actinomyces - sulfur granules, anaerobic conditions * Nocardiosis - sulfur granules +/- aerobic conditions * Prolonged courses of Amoxicillin >6 months

Question 299

cutaneous nocardiosis

Answer 299

* Following previous trauma to skin * Presents with a painless swelling allowed by an area of ulceration * Nocardia cultures - slow growing, does not grow under anaerobic conditions. USE PARAFFIN BAIT FOR GROWING

Question 300

ribavirin common side effect

Answer 300

haemolytic anaemia teratogenic

Question 301

heterophile test

Answer 301

EBV/mono

Question 302

fungal keratitis

Answer 302

* Mycotic keratitis * Associated with trauma * Ulcerative lesion in cornea, base is raised with creamy infiltrate * Slough off the ulcer appears dry with a feathery border surrounded by a yellow line of demarcation. * R/F : patients with immunosuppression * Mx : antifungals such as Amphotericin B, Itraconazole, Natamycin

Question 303

nightmares

Answer 303

efavirenz

Question 304

peripheral neuropathy

Answer 304

stavudine

Question 305

hyperlipidaemia

Answer 305

PIs

Question 306

tenofovir

Answer 306

renal impairment OP

Question 307

epididymo-orchitis

Answer 307

Men >35 years, stable partner - caused by enteric bacteria : treat with Ofloxacin Men <35 years, multiple sexual partners, ?STI cause - treat with Ceftriaxone

Question 308

mefloquine toxicity

Answer 308

acute psychosis

Question 309

hantavirus infection

Answer 309

* Hemorrhagic fever with renal features * Endemic in Korean, Balkans, Scandinavia * C/F : fever, myalgia, shock, conjunctival haemorrhage with epistaxis, elevated creatinine, elevated urea Haemolytic Uraemia syndrome will be associated with kidney disease and thrombocytopenia + hypertension (NO SHOCK)

Question 310

Q fever

Answer 310

Coxiella burnetii - transmission to humans by inhalation of infected dust and aerosols, drinking unpasteurised milk from cows C/F - high fever, headache, malaise Complications - pneumonia, hepatitis, myocarditis

Question 311

M Tb replication time

Answer 311

18-24 hours Escherichia coli - divides every 20 minutes Staph aureus divides every 30 minutes Treponema pallidum divides every 1-2 days Mycobacterium leprae - divided every 14 days

Question 312

trypanosomes

Answer 312

* African Trypanosomiasis : A.Caused by protozoa transmitted by tsetse fly. B.West Africa - Trypanosome Brucei ambience C.East Africa - Trypanosome brucei rhodesiense D.Early C/F : fever, headaches, arthralgia, pruritis E. Later stages : meningoencephalitis, personality change, agitation, severely disordered sleep F. INV of choice : thick and thin blood fils. CSF M/C, serology G.MX : T. brucei ambiense —> Pentamidine H.MX : T. Brucei rhodisense —> Suramin I. CNS involvement Rx changes J. CNS T. brucei ambiense - Nifurtimox & Elfornithine K.CNS T.brucei rhodisense - Melasprolol * Trypanosome cruzi - South American Trypanosomiasis - Transmitted by reduviid bugs - Cardiac : cardiomyopathy dilated, myocarditis, cardiac arrhythmia - complete heart block - Mega oesophagus & megacolon - due to destruction of parasympathetic nerves; recurrent aspiration, bloating, sever constipation. - MX : Benznidazole or Nifurtimox

Question 313

criteria for diagnosis of toxic shock syndrome

Answer 313

1. Fever >38.9 2. Hypotension 3. Widespread erythematous, macular rash 4. Subsequent desquamation of affected skin areas, palms and soles 5. Involvement of at least 3 organ systems - - GIT - diarrhoea, vomiting - Muscular - severe myalgia, raised CK - Renal : raised urea, raised creatinine - Heaptic : impaired liver function - Blood : thrombocytopenia - CNS : disorientation, confusion without focal neurology - Mucous membranes : hyperaemia of conjunctiva, pharynx or vagina

Question 314

loa loa treatment

Answer 314

diethycarbamazine Sensitivity reactions to filarial antigen both local and systemic - common and can stimulate acute manifestation reactions * Treatment - 1mg/kg on first day - increasing over 3 or more days to 6mg/kg in divide dose - dose is continued for 21 days * Heavy filariasis - DEC can trigger encephalitis - treatment with Albendazole is recommended

Question 315

shiga toxin associated HUS

Answer 315

complement activation C3 and C9

Question 316

dermatophyte infections

Answer 316

1) Micosporum - single microconidia or multiseptate macroconidia 2) Trichophyton spp. - multiple small microconidia 3) Epidermophyton spp. - do not produce conidia

Question 317

brucella melitensis

Answer 317

niclosamide empty stomach * Doxycycline PO + Streptomycin IM OR * PO Quinolone + PO Rifampicin OR * PO Doxycycline + PO Rifampicin +/- Gentamicin/Cotrimoxazole

Question 318

malignant otitis externa pathogen

Answer 318

pseud

Question 319

sporotrichosis

Answer 319

* Begins as reddish, non tender, macula-papular lesion at the site of inoculation. * Similar nodules forms along proximal lymphatic channels * These break to form a row of ulcers * Diagnosis - culture of biopsy samples of Saboraud dextrose agar * Treatment - Itraconazole - sever infections : IV Amphotericin B

Question 320

infective endocarditis with an increasing PR interval

Answer 320

aortic root abscess

Question 321

strep gallolyticus endocarditis

Answer 321

colon ca biliary abnormality

Question 322

Rx cryptococcal meningitis

Answer 322

- IV Amphotericin B + Flucytosine/ Fluconazole

Question 323

cutaneous leish presentation

Answer 323

* Incubation period - few days to several months * Erythematous nodules develops at the site of infected sandfly bite * Golden crust forms - sore reaches its final size - usually 1-5 cm in diameter; over weeks or months * Crust may fall away leaving a ulcer with raised edge; satellite papules are common * After months or years, lesion starts to heal; leaving behind a depressed, mottled scar.

Question 324

diffuse infiltrative lymphocytic syndrome

Answer 324

weakness due to peripheral motor neuropathy; ascetic meningits and cranial palsies can occur too

Question 325

quinine stimulates release of

Answer 325

insulin

Question 326

culture negative IE

Answer 326

Coxiella, Brucella, Bartonella Non infective cause - malignancy, SLE (Libman Sacks endocarditis)

Question 327

ART SEs

Answer 327

* Indinavir - renal stones, asymptomatic hyperbilirubinemia * Ritonavir - P450 inhibitor Protease inhibitors - Indinavir, Ritonavir * Tenofovir - renal impairment, osteoporosis * Zidovudine - anaemia, black nails, myopathy, significant reduction in white cell count * Didanosine - pancreatitis * Abacavir - idiosyncratic hypersensitivity ass. With rash and fever; associated with HLA B5701 type * Lamivudine - skin rashes, arthralgia, peripheral neuropathy NRTIs - Zidovudine, Lamivudine, Stavudine, Tenofovir, Zalcitabione, Emtricitabine, Didanosine, Abacavir

Question 328

jock itch

Answer 328

clotrimazole

Question 329

most common organism in bronchiectasis

Answer 329

Hib

Question 330

causes of transudative effusion (<30g protein)

Answer 330

heart failure (most common transudate cause) hypoalbuminaemia liver disease nephrotic syndrome malabsorption hypothyroidism Meigs' syndrome

Question 331

exudative effusion (>30g protein)

Answer 331

infection pneumonia (most common exudate cause), tuberculosis subphrenic abscess connective tissue disease rheumatoid arthritis systemic lupus erythematosus neoplasia lung cancer mesothelioma metastases pancreatitis pulmonary embolism Dressler's syndrome yellow nail syndrome - lymphatic leak, chylothorax

Question 332

causes of occupational asthma

Answer 332

isocyanates - foams and paints soldering flux resin flour platinum salts cadmium -> emphysema/COPD

Question 333

PFTs

Answer 333

* Obstructive Lung Disease - A.FEV1 - significantly reduced B.FVC - reduced or normal C.FEV1 / FVC - reduced D.Conditions - Asthma, COPD, Bronchiectasis, Bronchiolitis obliterans * Restrictive Lung Disease - 1. FEV1 - reduced 2. FVC - significantly reduced 3. FEV1 / FVC - normal or increased 4. Conditions - Pulmonary fibrosis, Asbestosis, Sarcoidosis, ARDS, Kyphoscoliosis, Ankylosing spondylitis, Neuromuscular disorders, Severe obesity, Interstitial Lung Disease

Question 334

raised TLCO

Answer 334

- Asthma - Pulmonary Hemorrhages - Goodpastures, Granulomatosis with microscopic polyangitis - Left to right cardiac shunts - Polycythemia - Hyperkinetic states - Male gender, exercise

Question 335

reduced TLCO

Answer 335

- Pulmonary fibrosis - Pulmonary emboli - Pulmonary edema - Pneumonia - Emphysema - Anaemia - Low Cardiac output

Question 336

mesothelioma

Answer 336

chromosome 22 (TSG) drainage not recommended SV40 carcinogen

Question 337

CF presentation

Answer 337

delayed puberty nasal polyps CFRD rectal prolapse

Question 338

mycoplasma

Answer 338

haemoptysis - rise in polyclonal anti-I IgM Abs in mycoplasma pneum macrolides as no cell wall

Question 339

causes of clubbing

Answer 339

bronchiectasis abscess empyema malignancy HPOA fibrosing alveolitis asbestosis

Question 340

R heart strain

Answer 340

* Normal CTPA - no contrast in IVC * ECG of right heart strain - ST segment depression and T wave inversion in V1-V4, and inferior leads leads II, III aVF

Question 341

chest drain for infected effusion

Answer 341

A.Patients with frankly purulent or turbid/cloudy pleural fluid on sampling B.Pleural fluid pH < 7.2 with suspected pleural infection C.Poor clinical progress despite antibiotic treatment D.Presence of organisms identified by Gram stain and/or culture from a non-purulent pleural fluid sample

Question 342

Churg strauss syndrome

Answer 342

* Eosinophilia + necrotising granulomatous inflammation * Involves respiratory tract, renal system, skin and peripheral nerves * Typically presents with history of asthma, with atopy, recurrent nasal polyps * 7 out of 10 patients - have mono neuritis multiplex * Renal involvement - hematuria, proteinuria, hypertension, raised creatinine levels. * Diagnostic investigation of choice - skin biopsy : granuloma formation with small vessel arteriopathy. Mx - steroids

Question 343

Familial PAH

Answer 343

BMPR2 ECG may show P pulmonate, right axis deviation, RV hypertrophy, RV strain, RBBB, ITC prolongation

Question 344

step 2 of COPD treatment if no asthmatic features

Answer 344

LABA+LAMA

Question 345

treatment of radiation pneumonitis

Answer 345

systemic corticosteroids addition of azathioprine or cyclosporin

Question 346

carcinoid tumour of lung

Answer 346

* Tumor is normally vascular. * Histology - small polygonal cells with finely granular eosinophilic staining of cytoplasm and nuclei are small and round. * Bronchial carcinoid - derives from stem cells of bronchial epithelium - Kulchitsky cells. * 80-90% of them develop in bronchus of sub segmental size - patients often present with bronchial obstruction that has led to a lower respiratory tract infection. * Total surgical excision should be attempted if there is no evidence of metastasis. recurrent haemoptysis with segmental collapse

Question 347

lung ca appearances

Answer 347

- Adenocarcinoma : forms a gland and produced mucin. - Bronchoalveola cell carcinoma : arises from type 2 pneumocytes and spreads rapidly. - Small cell carcinoma of bronchus : arises from neuroendocrine Kulchitsky cells - composed of sheets of small, round to spindled cells with dark nuclei, scanty cytoplasm, fine granular nuclear chromatin with indistinct nucleoli. ‘Salt and pepper appearance’ - Squamous cell carcinoma : well differentiated contains keratin pearls. Poorly differentiated may stain positive for keratin, with large irregular nuclei and coarse nuclear chromatin with large nucleoli. Presence of intercellular bridging is diagnostic of squamous cell carcinoma

Question 348

Langerhans cell histiocytosis

Answer 348

proliferaton of langerhans cells in bronchiolar and bronchial epithelium, forming granulomas. upper zone pulmonary fibrosis C/F - cough, SOB on exertion, fever, fibrosis in mid to upper zones CXR - bilaterally symmetrical reticulonodular pattern Dx - incidentally on CXR Spirometry - restrictive pattern

Question 349

Macleod syndrome

Answer 349

unilateral emphysema following childhood bronchiolitis

Question 350

empyema pleural fluid analysis

Answer 350

Macroscopic pus * pH < 7.2 * Glucose < 3.3 mol/L * LDH > 1000

Question 351

haemoptysis massive

Answer 351

Massive hemoptysis + stable hemodynamics —> angiography + embolisation (step 1) later if needed —> lobar resection

Question 352

legionella and penicillin allergy

Answer 352

levofloxacin

Question 353

ARDS

Answer 353

low compliance, high elastic recoil, reduced transfer factor, low pulmonary artery wedge pressure

Question 354

primary pulmonary HTN

Answer 354

* Presents with breathlessness, fatigue, angina (due to right ventricular ischaemia), pre syncope.syncope. * Autosomal dominant pattern with incomplete penetrance of inheritance in familial cases * Physical sings include - (1) elevated JVP (2) Left parasternal heave (3) Pansystolic murmur - tricuspid regurgitation (4) Right ventricular S4 (5) peripheral edema

Question 355

L hilar mass clinical sign

Answer 355

whispering pectoriloquy

Question 356

mx of narcolepsy

Answer 356

* CNS Stimulants - First line - Modafinil, Armodafinil * Second line - Methylphenidate, Dexamfetamine * Patients with cataplexy : 1) first line - sodium oxybate 2) second line - Fluoxetine, Paroxetine, Venlafaxine

Question 357

hepatopulmonary syndrome

Answer 357

Hepatic disease can result in right-to-left shunting, secondary to intrapulmonary vasodilation mainly in lower lobes. * This is similar to Pulmonary AV malformations seen in hereditary hemorrhagic telangiectasia. * There is increase blood flow through the lower lobes when patient moves from supine to standing position. * This results in blood from the lower lobes which is poorly oxygentated, entering the left side of heart, leads to oxygen desaturation in the erect position. * This phenomenon is called orthodeoxia.

Question 358

PCP XR

Answer 358

normal X ray or b/l interstitial shadowing - perihilar in distribution

Question 359

Hypogammaglobulinemia in ataxia telangiectasia

Answer 359

* Inherited in autosomal recessive fashion * Ataxic from an early age * Oculomotor apraxia and choreathetosis later in life * Alpha-fetoprotein can be raised. * Cell defects and deficiencies in humeral immunity with defective DNA repair and IgA and IgE deficiencies result in recurrent infections and tumour susceptibility.

Question 360

hypoxia in primary pulmonary HTN

Answer 360

intra-pulmonary shunting, reduced diffusion capacity due to arterial fibromuscular hyperplasia

Question 361

Hereditary spinocerebellar degeneration

Answer 361

autosomal dominant or recessive. Associated with slowly progressive cerebellar syndromes, tremors and ataxic gait. Extrapyramidal features can be presents pyramidal signs, cortical symptoms such as behavioural disturbance, epilepsy, cognitive disturbances. NOT associated with bronchiectasis.

Question 362

Mounier-Kuhn syndrome

Answer 362

congenital deficiency of bronchial cartilage; tracheal and bronchial dilation and associated with recurrent LRTIs

Question 363

Williams-Campbell syndrome

Answer 363

deficiency of bronchial cartilage particularly in sub segmental bronchi. Leads to bronchiectasis distal to the affected bronchi.

Question 364

idiopathic pulmonary haemosiderosis

Answer 364

occurs in younger people and characterised by pallor, weakness, lethargy, dry cough and occasional hemoptysis. No abnormal immunological features. Gas transfer factor is elevated because of hemoptysis blood is already in alveoli

Question 365

bronchiectasis on HRCT

Answer 365

signet ring sign dilation and thickening of affected bronchi

Question 366

BHD syndrome

Answer 366

Pneumothorax + lung cysts + fibrofolliculoma of skin + renal cancer Due to mutant of follicular gene Family history of pneumothorax and renal cancer.

Question 367

CF infiltration of

Answer 367

neutrophils

Question 368

HAP mx

Answer 368

3rd gen cephalosporin + aminoglycoside

Question 369

forced expiratory flow

Answer 369

* Reflects the status of small airways * Effort independent * Impaired in smokers and in patients with graft v/s host disease with associated lung pathology * Impaired in bronchiolitis obliterans * Not useful for identifying large airway disease

Question 370

elevated unilateral hemidiaphragm ix

Answer 370

fluoroscopy

Question 371

cryptogenic pulmonary eosinophilia

Answer 371

malaise fever weight loss raised ESR asthma rx - 1 year steroids

Question 372

Caplan syndrome

Answer 372

RA + pneumoconiosis

Question 373

acute asthma TLC

Answer 373

increased due to gas trapping

Question 374

mendelson syndrome

Answer 374

acute pneumonia caused by regurgitation of stomach contents and aspiration of chemical material, usually gastric juices. Can cause severe bronchospasm. Pneumonia develops rapidly and within hours, patients become tachypnoeic, hypoxic, and febrile. Minimal sputum production. Commonly seen after anaesthesia when the gag reflex is depressed.

Question 375

best predictor of OSA

Answer 375

neck size

Question 376

jejunal biopsy in whipple's disease

Answer 376

infection with gram-positive actinobacteria deposition of macrophages containing PAS positive granules within villi 14 days of ceftriaxone of benzylpenicillin + year of co-trimox

Question 377

gastrin hormone

Answer 377

G cells in antrum + duodenum inhibited by low ph and somatostatin increases acid secretion by gastric parietal cells, increased secretion of pepsinogen, increased IF section, increased gastric motility, parietal cell maturation

Question 378

CCK

Answer 378

I cells in upper SI secretion of enzyme rich fluid from pancreas, contraction of gallbladder and relaxation of sphincter of Oddi, decreases gastric emptying, trophic effect on pancreatic acinar cells, inducing satiety

Question 379

what makes direct contact with L kidney

Answer 379

pancreas L suprarenal gland

Question 380

achalasia mx

Answer 380

short acting nitrates before meals CCBs Botox heller myotomy

Question 381

drug causes of pancreatitis

Answer 381

azathioprine OCP steroids anti-retrovirals fibrates thiazides

Question 382

Cullens and Gray Turners

Answer 382

umbilicus vs flanks

Question 383

drug causes of chronic pancreatitis

Answer 383

thiazides azathioprine tetracyclines hyperCa, hyperlipidaemia

Question 384

exocrine insufficiency test in chronic pancreatitis

Answer 384

pancreolauryl and serum para-amino benzoic acid testing fecal elastase

Question 385

pre vs post sinusoidal portal htn

Answer 385

normal in pre HVPG >= 5 - induced portal htn

Question 386

Wilsons disease

Answer 386

autosomal recessive ATP7B on chr13 high levels of free copper fibrosis and CNS signs reduced serum caeruloplasmin and total serum copper mx penicillamine tridentine hydrochloride liver transplant

Question 387

malignancies in coeliac

Answer 387

T cell lymphoma upper GI tract

Question 388

Type 1 cryoglobulinaemia

Answer 388

IgG or IgM - monoclonal MGUD, Waldenstrom, CLL, myeloma Raynauds

Question 389

Type 2 cryoglobulinaemia

Answer 389

mixture of polyclonal Ig with RF Hep C, Sjogrens, SLE positive RF

Question 390

Type 3 cryoglobulinaemia

Answer 390

polyclonal HCV, autoimmune disease

Question 391

Meltzer's triad

Answer 391

t2 and 3 purpura, arthralgia, weakness

Question 392

advanced Parkinsons intestinal

Answer 392

pseudo-obstruction prokinetics

Question 393

NOD2 gene mutation

Answer 393

Crohns

Question 394

HFE mutations

Answer 394

hctosis C282Y H63D increased transferrin and ferritin

Question 395

vitamin C deficiency

Answer 395

weak collagen poor wound healing and recovery from burns scurvy - haematological, abnormal osteoid and dentin formation XR - generalised bone rarefaction, metaphyseal deformity of fractures and sebperiosteal hepatomas co-factor for hydroxylation of proline and lysine

Question 396

T1 vs 2 autoimmune hepatitis

Answer 396

most common, anti SMA and ANA, inc IgG HLA DR3 and 4 children, anti LKM1 and ALC-1 HLA DQB1 and DRB

Question 397

liver biopsy in AIH

Answer 397

piecemeal necrosis and mononuclear infiltration of portal and peri portal areas

Question 398

AIH mx

Answer 398

pred azathioprine

Question 399

protein losing enteropathy dx

Answer 399

alpha 1 antitrypsin levels indicate plasma leakage of proteins in gut due to sarcoid, IBD, pseudomemb colitis, CMV colitis, TB, CTD

Question 400

bile salt malabsorption scan

Answer 400

SEHCAT

Question 401

PSC

Answer 401

ALP raised, anti sma and ANA MRCP dx cholestyramine UDO abx if cholangitis

Question 402

drugs causing hepatitis

Answer 402

* Isoniazid * Rifampicin * Methyldopa * Atenolol * Enalapril * Verapamil * Nifedipine * Amiodarone * Ketoconazole * Cytotoxics * Halothane

Question 403

drugs causing cholestasis

Answer 403

* OCP * Ciclsporin * Azathioprine * Chloramphenicol * Cimetidine * Ranitidine * Erythromycin * Nitrofurantoin * Imipramine * Ibuprofen * Hypoglycaemics

Question 404

Menetrier's disease

Answer 404

giant gastric folds, in fundus and body hyperplasia of gastric pits, gland atrophy, increase in mucosal thickness hypochlorhydria - nil gastric production hypoalbuminaemia PPI

Question 405

metformin can cause

Answer 405

bile acid malabsorption similar to ileal resection, terminal ileal disease, cholecystectomy

Question 406

PBC

Answer 406

ALP raised cholestatic jaundice AMA antibodies hallmark of PBC portal htn and varices anti mitochondrial antibody M2 raised IgM cholestyramine obeticholic acid

Question 407

angiodysplasia 2x normal colonoscopies

Answer 407

capsule endoscopy

Question 408

moa of lactulose in hepatic encephalopathy

Answer 408

acidic metabolites increase ionisation of freely diffusible ammonia into ammonium which cannot diffuse back into blood

Question 409

capecitabine advantage

Answer 409

oral administration metabolised to 5FU

Question 410

black pigment gallstones

Answer 410

irregular and radiolucent, bile pigments hereditary spherocytosis, sickle cell, thalassaemia, cirrhosis

Question 411

bleeding source of duodenal ulcer

Answer 411

posterior aspect of duodenum —> posterior superior pancreaticoduodenal artery Anterior duodenum + head of pancreas —> anterior superior pancreaticoduodenal artery Pylorus and primal duodenum —> proximal duodenal artery Stomach —> gastroepiploic artery

Question 412

fistulating crohns mx

Answer 412

anti TNF first line

Question 413

acute fulminant hepatitis

Answer 413

A and E

Question 414

can get IRIS after

Answer 414

Whipples tx relapse tx-> penicillin, ceft, doxy, hcq

Question 415

Gardner syndrome

Answer 415

AD polyps, osteomas, soft tissue tumours congenital hypertrophy of retinal pigment epithelium APC gene on chr 5q21 total colectomy

Question 416

juvenile polyposis

Answer 416

AD hamartomatous polyps throughout GIT

Question 417

Peutz-Jegher

Answer 417

AD intestinal hamartomas and peri oral

Question 418

Boyce's sign

Answer 418

swelling on L side of cheek which gurgles on palpation

Question 419

obstetric cholestasis

Answer 419

30-36 weeks of preg pruritis of palms and soles assoc with UTIs risk of foetus prematurity raised transaminases and bile acids

Question 420

contraindications to liver transplant

Answer 420

untreated HIV irreversible pulmonary disease ongoing alcohol abuse extra-hepatic malignancy

Question 421

Crohn's like enterocolitis

Answer 421

mmf adverse effect

Question 422

contraindications for liver biopsy

Answer 422

increased INR >1.5 thrombocytopaenia <50 severe ascites difficult body habitus haemangiomas echinococcus

Question 423

Menkes disease

Answer 423

X linked recessive ATP7A gene copper deficiency failure to thrive, seizures, hypotonia, neuro dys

Question 424

Dubin johnson syndrome

Answer 424

AR defective secretion of conjugated high direct bili dark granular pigments on liver biopsy rotor same but biopsy n

Question 425

criggler najar syndrome

Answer 425

familial unconjugated hyperbili T1 - birth 2 - later

Question 426

GIT polyps with highest malignancy potential

Answer 426

adenomas serrated villous

Question 427

GAVE - gastric antral valvular ectasia

Answer 427

watermelon stomach bleeds RFA at endoscopy

Question 428

UC indications for colectomy

Answer 428

stool >8x colon diameter >5.5 CRP >45

Question 429

systemic sclerosis diarrhoea

Answer 429

SIBO E coli and bacteroides vit B12 def metro and cipro

Question 430

acquired factor 8 def

Answer 430

phenytoin penicillin sulfa drugs pregnancy diabetes psoriasis SLE MG MS

Question 431

drug causes of chronic hepatitis

Answer 431

ketoconazole methyldopa nitrofurantoin isoniazid

Question 432

which hep C genotype most difficult to treat

Answer 432

3

Question 433

MALT

Answer 433

paraprotein and pseudohypoproteinemia origin from B cells eradicate with H pylori tx

Question 434

liver drug SEs

Answer 434

1) Cholestatis liver injury - Chlorpromazine, Azathiprine, Captopril, Cyclosporine, Penicillamine, Erythromycin, COCP, Flucloxacillin 2) Hepatitis picture : Paracetamol, Phenytoin, Ethanol, Rifampicin, Allopurinol, Isoniazid 3) Mixed cholestasis / hepatitis : Co-Amoxiclav, Sulphonamides, Sulfasalazine, Carbamazepine

Question 435

Turcot syndrome

Answer 435

polyps and 1y brain tumour HNPCC - glioblastoma

Question 436

target cells

Answer 436

sickle cell thalassaemia IDA hyposplenism liver disease

Question 437

tear drop poikilocytes

Answer 437

myelofibrosis

Question 438

spherocytes

Answer 438

HS AIHA

Question 439

basophilic stippling

Answer 439

lead poisoning thalassaemia sideroblastic anaemia myelodysplasia

Question 440

schistocytes

Answer 440

cold AIHA mechanical heart valve DIC

Question 441

pencil poikilocytes

Answer 441

IDA

Question 442

burr cells

Answer 442

uraemia pyruvate kinase deficiency

Question 443

acanthocytes

Answer 443

abetalipoproteinemia

Question 444

HSMNs

Answer 444

megaloblastic anaemia

Question 445

irradiated blood products

Answer 445

depletion of T lymphocytes and used avoid transfusion-associated gvhd

Question 446

breast cancer gene mutation

Answer 446

p53

Question 447

Burkitt lymphoma

Answer 447

c-myc translocation EBV - african form starry sky appearance - lymphocyte sheets interspersed with macrophages containing dead apoptotic tumour cells rasburicase

Question 448

tumour lysis syndrome

Answer 448

hyperkalaemia hyperphosphataemia hypocalcaemia hyperuricaemia acute renal failure

Question 449

commonest UK cancers

Answer 449

breast > lung > colorectal > prostate > bladder

Question 450

indications for CLL tx

Answer 450

* Progressive marrow failure - development or worsening of anaemia and/or thrombocytopenia * Massive >10 cm or progressive lymphadenopathy * Massie > 6 cm or progressive splenomegaly * Progressive lymphocytosis : >50 % increase over 2 months or lymphocyte doubling time < 6 months * Systemic symptoms - weight loss >10 % in last 6 months, extreme fatigue, fever > 38 degree for > 2 weeks, night sweat * Autoimmune cytopenia - ITP

Question 451

CLL mx

Answer 451

FCR ibrutinib

Question 452

cyclophosphamide

Answer 452

MOA : causes cross linking in DNA Adverse effects : hemorrhagic cystitis, myelosuppression, transitional cell carcinoma Hemorrhagic cystitis - treated with Mesna and hydration; cyclophosphamide toxic metabolite —> Acrolein which can cause damage to urothelium; treatment with Mesna removes the Acrolein

Question 453

bleomycin

Answer 453

degrades preformed DNA - lung fibrosis (lower zone) * Anthracyclines Doxorubicin - stabilises DNA-topoisomerase II complex which inhibits DNA & RNA synthesis Adverse : Cardiomyopathy

Question 454

antimetabolites

Answer 454

* Methotrexate : inhibits dihydrofolate reductase and thymidylate synthesis; Adverse - myelosuppression, mucositis, liver fibrosis, lung fibrosis lower zones * 5-Flurorouracil : Pyrimidine analogues inducing cell cycle arrest and apoptosis by blocking thymidylate synthase (works during S phase) ; adverse - myelosuppression, mucositis, dermatitis * 6-Mercaptopurine : purine analogue activated by HGPRTase decreasing purine synthesis; adverse - myelosuppression * Cytarabine : Pyrimidine antagonist - interferes with DNA synthesis at S phase and inhibits DNA polymerase Adverse - myelosuppression, ataxia

Question 455

acts on microtubules

Answer 455

* Vincristine, Vinblastine : inhibits formation of microtubules; Adverse - Vincristine —> Peripheral neuropathy ( reversible ), paralytic ileus Adverse - Vinblastine —>. Myelosuppression * Docetaxel - prevents microtubule depolymerisation & disassembly decrease free tubulin; adverse - neutropenia

Question 456

topoisomerase inhibitors

Answer 456

Irinotecan : inhibits topoisomerase I which prevents relaxation of supercoiled DNA - adverse : myelosuppression

Question 457

other cytotoxics

Answer 457

Cisplatin - causes cross linking of DNA (MOA similar to cyclophosphamide); adverse - ototoxicity, peripheral neuropathy, hypomagnesemia * Hydroxyura (hydroxycarbamide) - inhibits ribonucleotide reductase, decreasing DNA synthesis; adverse - myelosuppression

Question 458

drug causes of pancytopaenia

Answer 458

cytotoxics antibiotics - trimethoprim, chloramphenicol anti-rheumatoid drugs - gold, penicillamine carbimazole - agranulocytosis anti-epileptics - carbamaz sulphonylureas - tolbutamide

Question 459

Fanconi anaemia

Answer 459

* Autosomal recessive * Haematological - aplastic anaemia, increased risk of AML * Neurological * Skeletal : short stature, thumb/radius abnormalities * Cafe au lait spots

Question 460

haematological malignancy genetics

Answer 460

- t(9;22) - Philadelphia Chromosome * >95% of patients with CML * ABL proto-oncogene fusion with BCR gene n chromosome 22 * BCR-ABL fusion gene - fusion protein which has excessive tyrosine kinase activity * Poor prognostic factor in ALL - t (15 ; 17) * Seen in acute promyelocytic leukaemia AML-M3 subtype * Fusion of PML and RAR-alpha genes - t ( 8 ; 14 ) * Burkitt’s lymphoma * MYC oncogene translocated into an immunoglobulin gene - t ( 11 ; 14 ) * Mantle cell lymphoma * Deregulation of cyclin D1 (BCL-1) gene - t (14 ; 18) * Follicular lymphoma * Increased BCL-2 transcription

Question 461

hereditary angioedema

Answer 461

AD low C1INH during attack low C2 and C4 levels during and between attacks serum C4 - screening tool mx IV C1 inhibitor conc, FFP danazol

Question 462

histological classification of hodgkins

Answer 462

* Nodular sclerosing : most common ~70%, good prognosis; associated with lacunar cells, F > M * Mixed cellularity : ~20%, good prognosis, associated wit high number of RS cells * Lymphocyte predominant : ~5% of total cases, BEST prognosis * Lymphocyte depleted - rare, WORST prognosis] NC anaemia eosinophilia (IL5) raised LDH RS - multinucleated or bilobed nucleus with prominent eosinophilic inclusion like nucleoli

Question 463

Ann arbor staging

Answer 463

- Stage I : single lymph node - Stage II : 2 or more lymph nodes / regions on same side of diaphragm - Stage III : nodes on both sides of diaphragm - Stage IV : spread beyond lymph nodes Each stage divided into A or B - * A = no systemic symptoms other than pruritus * B = weight loss > 10% in 6 months / night sweats / fever > 38

Question 464

chemo for Hodgkins

Answer 464

1) ABVD - Doxorubicin, Bleomycin, Vinblastine, Dacarbazine 2) BEACOPP - Bleomycin, Etoposide, Doxorubicin, Cyclophosphamide, Vincristine, Procarbazine, Prednisone

Question 465

methaemoglobinaemia

Answer 465

Congenital causes - 1) hemoglobin variants - HbH, HbM 2) NADH Methaemoglobin reductase deficiency Acquired causes - 1) Drugs - Sulphonamides, Nitrates, Dapsone, Sodium nitroprusside, Primaquine 2) Chemicals - aniline dyes Aniline dyes - bladder cancer Primaquine - G6PD Features of Methaemoglobinemia - 1) chocolate cyanosis 2) Dyspnoea 3) Headache 4) Anxiety 5) Severe forms - acidosis, arrhythmia, seizures, coma 6) Normal pO2 but decreased oxygen saturations Management : - Enzyme deficiency of NADH Methaemoglobin reductase —> ascorbic acid - IV Methylthionium chloride (Methylene blue) for acquired causes

Question 466

causes of normocytic anaemia

Answer 466

chronic disease CKD aplastic anaemia hyltic anaemia blood loss

Question 467

contra indications to platelet transfusion

Answer 467

chronic bm failure autoimmune thrombocytopaenia HIT TTP

Question 468

tx of PRV

Answer 468

aspirin venesection hydroxyurea and phosph-32 ruxolitinib

Question 469

PCT tx

Answer 469

chloroquine

Question 470

chronic granulomatous disease (neutrophils)

Answer 470

lack of NADPH oxidase reduces ability of phagocytes to produce ROS recurrent pneumonias and abscess - S aureus and fungi negative nitroblue-tetrazolium test abnormal dihydrorhodamine flow cytometry test

Question 471

Chediak higashi syndrome (neutrophils)

Answer 471

microtubule polymerisation effect -> decrease in phagocytosis albinism and peripheral neuropathy recurrent bacterial infections giant granules in neutrophils and platelets

Question 472

leukocyte adhesion deficiency (neutrophils)

Answer 472

- defect of LFA-1 integrin (CD18) protein on neutrophils - Recurrent bacterial infections - Delay in umbilical cord sloughing may be seen - Absence of neutrophils/pus at sites of infection

Question 473

common variable immunodeficiency (B cells)

Answer 473

- low antibody levels - IgG, IgM, IgA - Recurrent chest infections - Can predispose to autoimmune disorders and lymphoma

Question 474

Bruton's congenital agammaglobulinaemia (B cells)

Answer 474

- X-linked recessive - Defect in Burton’s tyrosine kinase (BTK) gene that causes severe block in B cell development - Recurrent chest infections - Absence of B cells with reduced immunoglobulins of all classes

Question 475

selective IgA def (B cells)

Answer 475

- maturation defect in B cells - Most common primary antibody deficiency - Recurrent sinus and throat infections - A/W coeliac disease and may cause false negative coeliac antibody screen - Sever transfusion reactions may occur due to anti-IgA antibodies —> anaphylaxis

Question 476

Di George syndrome (T cells)

Answer 476

- 22q11.2 deletion - Failure to develop 3rd and 4th pharyngeal pouches - C/F : congenital heart disease (TOF), learning difficulties, hypocalcemia, recurrent viral/fungal diseases, cleft palate

Question 477

combined B and T cell disorders

Answer 477

1) Severe combined immunodeficiency - X-linked defect due to defect in common gamma chain - Common gamma chain : protein used in the receptor for IL-2 and other interleukins - Other causes : adenosine deaminase deficiency - Recurrent bacterial, viral and fungal infections - Reduced T cell receptor excision circle 2) Ataxic telangiectasia - defect in DNA repair enzymes - Autosomal recessive - C/F : cerebellar ataxia, telangiectasia, recurrent chest infections - 10% risk of developing leukaemia or lymphoma 3) Wiskott-Aldrich syndromes - defect in WASP gene - X linked recessive - C/F : recurrent bacterial infections, eczema, thrombocytopenia - Low IgM levels - Increased risk of autoimmune disorders and malignancy 4) Hyper IgM syndromes - mutations in CD40 gene - Infections - Pneumocystic pneumonia - Hepatitis - Diarrhoea

Question 478

AML

Answer 478

BM failure and thrombocytopaenia accumulation/rapid proliferation of poorly differentiated myeloblasts in blood anaemia, low WBC (infections, fever, lymphaden), thrombocytopaenia, hepatosplenomegaly, organ infiltration (spleen) * FBC - anaemia, macrocytosis, leukocytosis, neutropenia, thrombocytopenia * Peripheral smear - Auer rods, blasts * Increased uric acid, increased LDH * BM - hyper cellular bone marrow Mx - cytarabine, idarubicin/daunorubicin hydroxycarbamide, allopurinol, rasburicase

Question 479

pancytopenia, liver dysfunction, diarrhoea, rash after transfusion

Answer 479

GVHD

Question 480

hairy cell leukemia

Answer 480

activating point mutation of BRAF isoform of RAF (BRAF V600E) has been implicated in the pathogenesis. Hairy cell leukaemia expresses CD103. HCL - can also express range of B cell markers - CD19, CD20, CD22, CD25.

Question 481

phases of trials

Answer 481

Phase I trial - pharmacokinetics, human toxicity of the drug Phase II - to study optimal dosing, common adverse effects, activity of drug, efficacy of drug against disease Phase III - to compare new drug with conventional therapy Phase IV - determines long term toxicity of the drug

Question 482

mx of vwd

Answer 482

* Tranexamic acid for mild bleeding * Desmopressin (DDAVP) - raises levels of vWF by inducing release of vWF from Weibel-Palade bodies in endothelial cells * Factor VIII concentrate

Question 483

sickle crises

Answer 483

1) Thrombotic / vaso-occlusive / painful crises * Precipitated by infection, dehydration, deoxygenation * Infarcts occur in vaious organs including the bones (e.g. avascular necrosis of hip, hand-foot syndrome in children, lungs, spleen, brain). 2) Acute Chest syndrome * Vase-occlusion within the pulmonary vasculature —> infarction in lung parenchyma * C/F : dyspnea, chest pain, pulmonary infiltrates on CXR, low PO2 * Mx - pain relief, oxygen therapy, antibiotics, transfusion which improves oxygenation ->> most common cause of death after childhood 3) Aplastic crises * Caused by sudden infection with Parvovirus B19 * Sudden fall in Hemoglobin * Bone marrow suppression - reduced reticulocyte count 4) Sequestration crises * Sickling within organs like spleen or lungs causes pooling of blood and worsening of anaemia * Increased reticulocyte count

Question 484

commonest tumour in anterior mediastinum

Answer 484

thymoma MG red cell aplasia dermatomyositis SLE SIADH

Question 485

Waldenstrom's macroglobulinaemia

Answer 485

* Lymphoplasmacytoid malignancy characterised by secretion of monoclonal IgM paraprotein (1)Systemic upset - weight loss, lethargy (2)Hyperviscosiy syndrome - visual disturbance - pentameric configuration og IgM increases serum viscosity (3)Hepatosplenomegaly (4)Lymphadenopathy (5)Cryoglobulinemia - Raynaud’s * Investigations - Monoclonal IgM paraproteinemia * BM biopsy —> diagnostic : infiltration of BM with lymphoplasmacytoid lymphoid cells * Mx : rituximab-based combination chemotherapy

Question 486

maethaemoglobinaemia

Answer 486

metabolic acidosis due to lactic acid build-up due to tissue hypoxia chocolate cyanosis

Question 487

TTP

Answer 487

* Abnormally large and sticky multimers of vWF causes platelets clumping in blood vessels * TTP - deficiency of ADAMTS13 which breakdown the large vWF 1. Typically adult females 2. Fever 3. Fluctuating neurological signs due to micro emboli 4. Microangiopathic haemolytic anaemia 5. Thrombocytopenia 6. Renal failure * Causes - (a)Post infection - urinary, gastrointestinal (b)Pregnancy (c)Drugs - cyclosporin, Cop, clopidogrel, aciclovir (d)Tumour (e)SLE (f) HIV

Question 488

AIHA

Answer 488

* Warm AIHA - antibody titre is usually IgG - extravascular hemolysis * Causes of Warm AIHA - 1) idiopathic 2) autoimmune disease - SLE 3) neoplasia - lymphoma, CLL 4) drugs - methyldopa * Cold AIHA - IgM - hemolysis at 4 degree; intravascular hemolysis; can have symptoms of Raynaud’s and acrocyanosis. * Causes : - Neoplasia : lymphoma - Infections, Mycoplasma, EBV * SLE and lymphoma : mixed warm-cold hemolytic anaemia

Question 489

gram positive coagulase negative

Answer 489

staph epidermidis

Question 490

haemolytic anaemia associated with mycoplasma

Answer 490

therapeutic plasmapheresis

Question 491

duffy blood group antigen alleles

Answer 491

Fya and Fay

Question 492

microcytic anaemia with disproportionate MCH and MCV. Also associated with increase iron and increased ferritin and increased TIBC.

Answer 492

BTT

Question 493

Prolonged APTT = factor VIII deficiency —>

Answer 493

intrinsic

Question 494

Factor VII deficiency = prolonged PT —>

Answer 494

extrinsic

Question 495

Factor X deficiency

Answer 495

= prolonged PT and APTT

Question 496

blood films

Answer 496

1) hyposplenism - post splenectomy, coeliac disease a) Target cells b) Howell Jolly bodies c) Pappernheimer bodies d) Sideritic granules e) Acanthocytes 2) Iron deficiency Anaemia A.Target cells B.Pencil poikilocytes C.Combined with B12 / folate —> mixed microcytic + microcytic picture 3) Myelofibrosis - tear drop poikilocytes 4) intra vascular hemolysis - schistocytes

Question 497

malignancies and associated infections

Answer 497

1) EBV - Hodgkin’s lymphoma, Burkkitt’s lymphoma, Nasopharyngeal carcinoma 2) HTLV-1 : Adult T cell leukaemia/lymphoma 3) HIV-1 : High grade B cell lymphoma 4) H.pylori : MALT 5) Malaria : Burkitss’s lymphoma

Question 498

leukemioid reaction

Answer 498

- high leukocyte alkaline phosphatase score - Dohle bodies - toxic granulations in white cells - Left shift of neutrophils = three or fewer segments of nucleus

Question 499

TACO vs TRALI

Answer 499

TA circulatory overload —> hypertension TA acute lung injury —> hypotension

Question 500

alteplase moa

Answer 500

tPA reversal - FFP + IV TXA

Question 501

CLL mx

Answer 501

FCR ibrutinib venetoclax

Question 502

t9:22

Answer 502

philadelphia good prog in CML poor in AML/ALL

Question 503

AT3 deficiency

Answer 503

AD * Inhibits several clotting factors - thrombin, factor X, factor IX. * It mediates the effect of heparin * Heparin works by binding to antithrombin III - enhancing anticoagulant effect of heparin by inhibiting formation of thrombin and other clotting factors. * C/F - recurrent venous thromboses * Mx - lifelong warfarin, heparin during pregnancy, antithrombin III concentrates during surgery or childbirth

Question 504

where is BRCA2

Answer 504

chr 13

Question 505

other useful mutations

Answer 505

ALK gene mutation - adenocarcinoma of lung; found on chromosome 2 C-KIT mutation - GIST and AML; chromosome 4 EFGR mutation - renal cell carcinoma, non small cell lung carcinoma; EFGR gene on chromosome 7 KRAS mutation - pancreatic cancer, chr 12

Question 506

CALR

Answer 506

calreticulin essential thrombocytosis

Question 507

universal donor of FFP

Answer 507

AB RhD -ve

Question 508

CA125

Answer 508

ovarian primary peritoneal

Question 509

sideroblastic anaemia

Answer 509

* Delta aminolevulinate synthase 2 deficiency * Acquired causes - A.Myelodysplasia B.Alcohol C.Lead D.Anti TB medications * Investigations : (1)FBC - hypo chromic microcytic anaemia (2)Iron studies - high ferritin, high iron, high transferrin saturation (3)Blood film - basophilic stippling of red cells (4)Bone marrow - Prussian blue stain will show ringed sideroblasts * Management - supportive, Pyridoxine may help

Question 510

Wald v MM

Answer 510

Waldenstrom's macroglobulinaemia - Organomegaly with no bone lesions Multiple myeloma - Bone lesions with no organomegaly

Question 511

aprepitant

Answer 511

anti-emetic NK1 receptor

Question 512

bladder ca aromatic amines

Answer 512

benzidine and beta naphthylamine

Question 513

Perl's stain

Answer 513

ring sideroblasts

Question 514

most common HL

Answer 514

nodular sclerosing

Question 515

ALL prognosis

Answer 515

Good Prognosis - 1) FAB L1 type 2) Common ALL 3) pre-B phenotype 4) Low initial WBC 5) Del (9p) 6) Trisomy 4, 7 and 10 7) Hyper diploidy 8) t(12;21) - also known as TEL-AML1 fusion protein 9) Translocation (1;19) Poor prognosis - 1. Hypodiploidy 2. FAB L3 type 3. T or B cell surface markers 4. Philadelphia translocation t(9;22) 5. Age < 2 years or > 10 years 6. Male sex 7. CNS involvement 8. High initial WBC >100,000 9. Non caucasian

Question 516

testicular teratoma markers

Answer 516

AFP and bHCG

Question 517

least likely ca to be inherited

Answer 517

gastric

Question 518

most common bleeding disorder

Answer 518

VWD

Question 519

most common hypercoag state

Answer 519

Factor V Leiden

Question 520

Burkitts transloc

Answer 520

8:14

Question 521

aflatoxin

Answer 521

HCC

Question 522

nitrosamine

Answer 522

gastric and oesophageal ca

Question 523

arsenic

Answer 523

lung and liver angiosarcoma

Question 524

benzene

Answer 524

leukaemia

Question 525

chromosome assocs

Answer 525

- chromosome 4 = Huntington’s disease, PKD - Chromosome 8 = gamma globin genes in foetal HB synthesis - Chromosome 12 = Rett syndrome, bladder cancer - Chromosome 16 = alpha thalassemia, alpha globulin genes - Chromosome 20 = Creutzfeldt Jacob disease, Niemann Pick disease type C

Question 526

tumour markers

Answer 526

- CA 125 —> ovarian cancer - CA 19-9 —> pancreatic cancer - CA 15-3 —> breast cancer - PSA —> prostate cancer - AFP —> HCC, teratoma testicular - CEA —> colorectal cancer - S-100 —> melanoma, schwannoma - Bombesin —> Small cell lung carcinoma, gastric carcinoma, neuroblastoma

Question 527

staging and prognosis of mm

Answer 527

beta 2 microglobulin

Question 528

HIT

Answer 528

* Caused by IgG antibodies that recognise multi molecular complexes of platelet factor 4 and heparin. * Received heparin within 100 days - at risk of fall in platelets. * DVT, PE, Major limb artery thrombosis, Stroke, MI, B/L adrenal hemorrhagic necrosis can occur

Question 529

rasburicase

Answer 529

uric acid oxidation - it is an enzyme that breaks down uric acid. Urine testing for patients on Rasburicase - falsely elevated uric acid levels - transport sample on ice cube to freeze action

Question 530

febrile reaction due to

Answer 530

donor neutrophils

Question 531

Cabot rings

Answer 531

megaloblastic anaemia, haemolytic anaemia, lead poisoning Heinz bodies - haemolytic anaemia Pappenheimer bodies - haemolytic anemia, sideroblastic anaemia, sickle cell disease Russell bodies - Ig containing inclusion bodies found in plasma cells Howell Jolly bodies - basophilic nuclear remnants in circulating RBCs

Question 532

phases and drugs

Answer 532

S phase drug : MTX, 5-FU G2 phase : Bleomycin M phase : Docetaxel G1 phase : Etoposide vincristine - meta

Question 533

aplastic anaemia

Answer 533

If a suitable HLA donor i.e., sibling present —> anti-thymocyte globulin is preferred treatment If no mention of suitable donor —> haematopoietic stem cell transplant

Question 534

positive acute phase reactants

Answer 534

TNFa IL6 ferritin negative - albumin

Question 535

protein C deficiency

Answer 535

* Acts to inactivate forms of pro-coagulant factors Va and VIIIa * Deficiency of protein C causes increased risk of venous thromboses. * Protein C —> vitamin K dependent serine protease * Thrombin activate protein C when bound to thrombomodulin. * Skin necrosis on administration of warfarin occurs * Autosomal codominant condition

Question 536

mycosis fungoides

Answer 536

* Indolent lymphoma of mature T-cells predominantly involving skin * Patient has circulating abnormal T cells called Sezary cells * Sezary cells + erythroderma = Sezary syndrome * Sezary syndrome —> severe pruritic, psoriasis like lesions. * Mx : Chemo

Question 537

nephrotic syndroem

Answer 537

protein S deficiency

Question 538

gross splenomegaly

Answer 538

= CML & Myelofibrosis. In CML —> white cell count > 100,000. Myelofibrosis —> WCC mildly raised leucoerythroblastic blood picture with teardrop cells typical of diagnosis of myelofibrosis

Question 539

Neurofibromatosis type 2 chromosome 22q12.2

Answer 539

B/L acoustic neuroma juvenile posterior sub capsular lenticular opacities

Question 540

risk of relapse in AML

Answer 540

most important prognostic marker is bone marrow karyotype Low risk of relapse - t(8;21) or chromosome 16 inversion High risk of relapse - deletion of chromosome 5 or 7

Question 541

breakdown times of cells

Answer 541

RBC 120 days Neutrophils 5.4 days Platelets 8-9 days

Question 542

alpha thalassaemia

Answer 542

* Pallor * Jaundice * Haemolytic anaemia * Hepatosplenomegaly * Frontal bossing of skull * Dysrythmas * Cardiac failure - myocardial siderosis

Question 543

abs of paraneoplastic syndromes

Answer 543

1) Anti Yo antibodies - ovarian and breast Paraneoplastic cerebellar degeneration Paraneoplastic cerebellar degeneration occurs when tumor cells express proteins that are normally expressed in cerebellum triggering autoimmune reaction : dysarthria, ataxia, nystagmus. 2) Anti Hu antibodies - small cell lung cancer 3) Anti Ro antibodies - associated with SLE and neonatal heart block 4) Anti-Tr antibodies - Hodgkins lymphoma 5) Anti-Zic4 - small cell lung cancer

Question 544

colorectal ca FOLFOX

Answer 544

- Folinic Acid, Flurouracil & Oxiplatin

Question 545

lytic lesions of MM

Answer 545

Low intensity on T1 weighted fission MRI High intensity on T2 weighted diffusion MRI

Question 546

hypernephroma

Answer 546

- clear cell or renal adenocarcinoma - Hematogenous spread to bone causes lytic lesions and hypercalcemia - Classic train : hematuria + loin pain + abdominal mass - Other C/F : night sweats, left varicocele due to compression of renal vein

Question 547

warfarin and protein C and S

Answer 547

vit K dependent, one month off warfarin before screen

Question 548

cisplatin SEs

Answer 548

Ototoxicity, Nephrotoxicity, Neurotoxicity, Hypomagnesemia, Hypokaleia, Hypocalcemia

Question 549

cisplatin moa

Answer 549

promotes cross-linking to form DNA adducts —> leads to promotion of intra-cellular signalling via pathways such as p53, p73, and MAP kinase leading to increased cellular apoptosis

Question 550

T315KD

Answer 550

ponatinib

Question 551

important syndromes

Answer 551

1) Felty’s syndrome - neutropenia + chronic arthritis + splenomegaly 2) Curran syndrome - association of kidney, hand and foot abnormalities 3) Di George syndrome - genetic disorder - heart defects, learning difficulties, cleft palate and thymus anomalies 4) Kostmann syndrome - severe congenital neutropenia 5) De Grouchy syndrome - 18q deletion syndrome, problems from childbirth

Question 552

meds for specific ca

Answer 552

Imatnib for GIST —> inhibition of c-kit Imatinib for CML —> inhibition of tyrosine kinase B-raf inhibitor —> malignant melanoma - Vemurafenib PDGFR alpha inhibitor —> also tyrosine kinase inhibitor of imatinib

Question 553

aplastic anaemia

Answer 553

Pancytopenia with hypo cellular bone marrow in the absence of an abnormal infiltrate or marrow fibrosis - Congenital causes - Fanconi anaemia, Dyskeratosis congenita, Reticular dysgenesis - Acquired causes : 1. Cytotoxic drugs and cancer treatment 2. Drugs - anti epileptics ( Carbamazepine, Phenytoin), antibiotics ( Sulphonamides, Chloramphenicol), NSAID, Gold 3. Virus - Parvovirus B19, HIV, Hepatitis 4. PNH, Myelodysplastic syndrome - Fanconi anaemia : autosomal recessive or X-linked; mutations in genes that code for nuclear complex involved in DNA damage response - GATA2 deficiency : dysfunction in zinc finger transcription factors involved in hematopoiesis; cytopenia, myelodysplastic syndromes, acute leukaemia, infection, immunodeficiency, lymphoedema. Clinical Features - recurrent infections - Fatigue - Pallor - Bleeding - Easy bruising - Tachycardia - Dyspnoea - Fanconi anaemia : hearing loss, pigmentation abnormalities, urogenital abnormalities

Question 554

MEN2a

Answer 554

Medullary thyroid cancer + pheochromocytoma + primary hyperparathyroidism

Question 555

MEN2b

Answer 555

Medullary thyroid cancer + Pheochromocytoma + Marfaboid habitus + mucosal/digestive neurofibromatosis

Question 556

RET proto-oncogene

Answer 556

* Found on chromosome 10 * Codes for receptor Tyrosine kinase * Loss of function mutations associated with Hirschsprung’s disease * Gain of function mutations associated with MEN-2 and medullary thyroid carcinoma

Question 557

nephrogenic DI sickle

Answer 557

cell trait or disease

Question 558

AIP kidneys

Answer 558

SIADH

Question 559

ITP severe

Answer 559

Severe ITP —> platelets less than 30,000 : treat with IV Immunoglobulins IVIG IVIG >> Methylprednisolone — if patient is diabetic and has uncontrolled blood sugars No IVIG in options —> choose Methylprednisolone

Question 560

protein C deficiency test

Answer 560

copperhead snake venom

Question 561

chemo for SCLC

Answer 561

cisplatin and etoposide or carboplatin

Question 562

absence seizures

Answer 562

3Hz spike and wave pattern sodium valproate and ethosuximide

Question 563

extensor plantars

Answer 563

UMN

Question 564

absent ankle jerk

Answer 564

LMN

Question 565

ataxia telangectasia

Answer 565

AR ATM gene most common inherited combined immunodeficiency cerebellar ataxia telangiectasia - spider angiomas IgA deficiency -> recurrent chest infections 10% risk of malignancy or blood ca

Question 566

causes of autonomic neuropathy

Answer 566

* Diabetes * GBS * Multisystem atrophy (MSA) * Shy Drager syndrome * Parkinsons’s * Infections - HIV, Chagas disease, neurosyphilis * Drugs - anti hypertensives, TCA * Craniopharyngioma

Question 567

benign rolandic epilepsy

Answer 567

* Form of childhood epilepsy that typically occurs between 4-12 years Features : * Seizures occur mostly at nights * Typically partial seizures [paraesthesia affecting face] * Secondary generalisation of seizures may occur * EEG - centretemporal spikes * Seizures stop by adolescence

Question 568

parietal lobe lesions

Answer 568

sensory inattention apraxia astereognosis inf hom quadr Gerstmanns - alexia, acalculia, finger agnosia and R-L disorientation

Question 569

occipital lobe lesions

Answer 569

HH with mac spar cortical blindness visual agnosia

Question 570

temporal lobe lesions

Answer 570

* Wernicke’s aphasia - word substitution, neologism, speech fluent * Superior homonymous quadrantanopia * Auditory agnosia * Prospoagnosia = difficulty recognising faces

Question 571

frontal lobe lesions

Answer 571

* Broca’s apahsia * Disinhibits * Perseveration = repetition of articular process such as word, phrase regardless of absence or cessation of stimulus * Anosmia * Inability to generate list

Question 572

cerebellum lesions

Answer 572

* Midline lesions - gait and truncal ataxia * Hemisphere lesion - intention tremor, past pointing, dysdiadokinesis, nystagmus

Question 573

where do Wernicke and Korsakoff syndrome affect

Answer 573

medial thalamus and mamillary bodies of the hypothalamus

Question 574

which area causes hemiballismus

Answer 574

sub thalamic nuclei of BG

Question 575

Huntingtons chorea affected region

Answer 575

striate (caudate nucleus) of BG

Question 576

Kluver bucy syndrome

Answer 576

amygdala hypersexuality, hyperorality, hyperphagia, visual agnosia

Question 577

CADASIL

Answer 577

AD NOTCH3 mutation on chr 19 migraines + strokes + dementia

Question 578

hyponatraemia with carbamazepine

Answer 578

SIADH

Question 579

raised lymphocytes in CSF

Answer 579

- viral meningitis/encephalitis - TB meningitis - Lyme disease - Behcets disease - SLE - Lymphoma - Leukaemia

Question 580

raised protein in CSF

Answer 580

- GBS - TB meningits - Bacterial meningits - Viral meningitis - Groin’s syndrome - increase CSF protein below a spinal canal blockage like tumor, disc, infection

Question 581

cervical spondylitic myelopathy

Answer 581

motor weakness sensory loss bladder/bowel dysfunction neck pain wide ataxic spastic gait UMN weakness in LL - hyper-reflexia, hypertonia, upgoing plantars

Question 582

drug causes of peripheral neuropathy

Answer 582

Amiodarone - Isoniazid - Vincristine - Nitrofurantoin - Metronidazole

Question 583

DVLA rules

Answer 583

epilepsy/seizures - first one, 6 months - established multiple - free for 12 mo - none for 5 years - license back - no driving for 6 months after last dose of AED syncope - single, explained - 4 weeks - unexplained - 6 months - 2 or more - 12 months stroke/TIA - 1 month off - multiple -> 3 months - craniotomy - 1 year - pituitary tumour - 6 months chronic -> inform dvla

Question 584

dystrophinopathies

Answer 584

XLR mutation in gene encoding dystrophin on xp21 - protein which connects muscle membrane to actin DMD - frameshift mutation causing one or both binding sites to be lost - 5 years onwards Beckers - non frameshift, milder, 10 years onwards

Question 585

infantile spasms

Answer 585

vigabatrin and steroids

Question 586

lennox-gastaut syndrome

Answer 586

extension of infantile spasms atypical absences, falls, seizures EEG - slow spike ketogenic diet

Question 587

JME/Janz syndrome

Answer 587

teenage, F>M infrequent generalised seizures following sleep deprivation valproate (levetirac)

Question 588

pregnancy and epilepsy

Answer 588

lamotrigine - ?dose increase monotherapy - Sodium valproate : associated with neural tube defects - Phenytoin : associated with cleft palate, give vit K

Question 589

GTCS mx

Answer 589

valproate - m leve/lamot - f

Question 590

focal seizures

Answer 590

lamotrigine carbamazepine

Question 591

carbamazepine exacerbates

Answer 591

absence seizures

Question 592

moa of ethosuximide

Answer 592

blocks T type Ca channels in thalamic neurons

Question 593

BL facial nerve palsy causes

Answer 593

sarcoid GBS Lyme BL acoustic neuromas

Question 594

UL facial palsy

Answer 594

LMN - Bell’s palsy, Ramsay hunt syndrome, Acoustic neuroma, HIV, Parotid tumor, MS, DM LMN - affects all facial muscles UMN u/l facial palsy - Stroke. UMN lesion - - spares upper face

Question 595

foot drop and weakness of hip abduction radiculopathy

Answer 595

L5

Question 596

Friedrich's ataxia

Answer 596

most common cause of early onset hereditary ataxias AR - TNR disorder - GAA on X25 on chr9 (frataxin) Clinical features : * Absent ankle jerks / extensor planters * Cerebellar ataxia * Optic atrophy * Spinocerebellar tract degeneration * HOCM - most common cause of death * High arched palate * Diabetes mellitus * Kyphoscolisos

Question 597

GBS cross reaction of

Answer 597

abs with gangliosides in the PNS correlation between the anti-GM1 and clin features

Question 598

LP in GBS

Answer 598

increase in protein with normal WBC

Question 599

NCS in GBS

Answer 599

decreased velocity, prolonged distal motor latency, increased F wave latency

Question 600

Miller Fisher syndrome

Answer 600

- variant of GBS - Ass. With - ophthalmoplegia, areflexia, and ataxia - Descending paralysis - anti-GQ1b antibodies are present in 90% of cases

Question 601

GBS mx

Answer 601

IVIG or PExc

Question 602

poor prognostic factors in GBS

Answer 602

- age > 40 years - Poor upper extremity muscle strength - Previous history of diarrhoea illness - High anti-GM1 titre - Need for ventilatory support

Question 603

HSMN1 genetics

Answer 603

AD PMP22 gene - myelin motor dx wasting of distal muscles, pes cavus, clawed toes foot drop and leg weakness

Question 604

Huntingtons genetics

Answer 604

AD TNR disorder - CAG repeat chr4

Question 605

lateral medullary syndrome - PICA

Answer 605

cerebellar features - ataxia, nystagmus brainstem - ipsilateral -> dysphagia, facial numbness, CN palsy brainstem - contralateral -> limb sensory loss

Question 606

MND mx

Answer 606

Management : - Riluzole - prevents stimulation of glutamate receptors, main use in ALS, prolongs life by about 3 months - Respiratory care - BiPAP - prolongs life around 7 months - Nutrition - Percutaneous gastrostomy tube

Question 607

familial ALS

Answer 607

chr 21 superoxide dismutase

Question 608

natalizumab

Answer 608

- recombinant monoclonal antibody; - antagonist to alpha -4 beta - 1 integrin found on surface of leucocytes - Inhibits migration of leucocytes across the endothelium and into the BBB - Given IV

Question 609

ocrelizumab

Answer 609

antiCD20

Question 610

fingolimod

Answer 610

Sphingosine 1 phosphate receptor modulator, ORAL administration; MOA : prevents lymphocytes from leaving lymph nodes

Question 611

glatiramer acetate

Answer 611

immune decoy in MS

Question 612

drugs exacerbating MG

Answer 612

- penicillamine - Quinidine - Procaiamide - Beta blockers - Lithium - Phenytoin - Antibiotics - gentamicin, macrocodes, quinolones, tetracycline

Question 613

myotonic dystrophy

Answer 613

* Autosomal dominant, trinucleotide repeat disorder * Two main types - DM1 and DM2 * DM1 : CTG repeat at end of DMPK gene - chromosome 19; distal weakness more prominent * DM2 : repeat expansion of ZNF9 gene on chromosome 3; proximal weakness more prominent General features : - myotonic facies : long haggard appearance - Frontal balding - Bilateral ptosis - Cataracts - Dysarthria Other features : - myotonia - tonic spasm of muscle - Weakness of arms and legs - Mild mental impairment - Diabetes mellitus - Testicular atrophy - Cardiac - heart block, cardiomyopathy - Dysphagia

Question 614

where does CSF absorption take place

Answer 614

arachnoid villi

Question 615

otosclerosis inheritance

Answer 615

AD

Question 616

CNS paraneoplastic sx

Answer 616

LES - SCLS, pre-synaptic VGCC anti-Hu - SCLC/neuroblastoma, cerebellar syndrome, sensory neuropathy, encephalomyelitis anti-Yo - ovarian/breast, cerebellar syndrome anti-GAD - breast/colorectal/SCLS, Stiff person's syndrome or diffuse hypertonia anti-Ri - breast/SCLS, ocular opsoclonus-myoclonus Purkinje cell ab - peripheral neuropathy, breast ca

Question 617

differentiate essential tremor from PD

Answer 617

123 Iodine-FP-CIT single photon emission CT (SPECT) scan

Question 618

dopamine receptor agonists AE

Answer 618

hallucinations impulse control disorders excessive daytime somnolence

Question 619

MAO-B

Answer 619

selegiline inhibits breakdown of dopamine

Question 620

COMT inh

Answer 620

entacapone/tolcapone

Question 621

conductive hearing loss

Answer 621

Rinne's Bone>Air Webers to affected ear

Question 622

sensorineural hearing loss

Answer 622

air>bone Webers to unaffected

Question 623

valproate AEs

Answer 623

teratogenic P450 inhibition alopecia ataxia tremor hepatotoxicity pancreatitis thrombocytopenia hyponatraemia hyperammoneic encephalopathy - Give L carnitine for tx

Question 624

Brown Seqard

Answer 624

IS spastic paresis IS loss of proprioception and vibration CL loss of pain and temp

Question 625

SACD

Answer 625

BL spastic paresis loss of proprioception and vibration limb ataxia

Question 626

Friedrichs ataxia

Answer 626

same as above but with cerebellar signs

Question 627

types of cerebellar nystagmus

Answer 627

oscillopsia - alteranti nystagmus jerk nystagmus direction of fast and slow components changes every 2 minutes lesion - flocculus and nodulus

Question 628

optic chiasma nystagmus

Answer 628

see saw nystagmus

Question 629

topiramate urinary stones

Answer 629

calcium phosphate due to formation of alkaline urine due to reduction of citrate excretion

Question 630

VHL

Answer 630

AD predisposes to neoplasia VHL gene - chr 3

Question 631

VHL features

Answer 631

- cerebellar hemangiomas - can cause SAH - Retinal hemangiomas - can cause vitreous hmmge - Renal cysts - premalignant cysts - Pheochromocytoma - Extra renal cysts : epididymal cyst, pancreatic cyst, hepatic cyst - Endolymphatic sac tumor - Clear cell renal cell carcinoma

Question 632

Foster Kennedy syndrome

Answer 632

ipsilateral optic atrophy and papilloedema of the contralateral optic nerve AVMs and juvenile nasopharyngeal angiofibroma optic atrophy - SOL

Question 633

INO

Answer 633

MLF in paramedian area of midbrain and pons impaired adduction on same side horizontal nystagmus on opposite side

Question 634

meningioma surgery visual defect

Answer 634

pie in the sky - R superior homonymous quadrantanopia

Question 635

neuromyelitis optica

Answer 635

optic neuritis and acute myelitis NMO-IgG seropositive aquaporin 4 antigen brain MRI not MS-like

Question 636

* Mutation in NOTCH3 gene * Multi infarct dementia * Recurrent TIAs and strokes * Neuro-cognitive decline : depression, low mood, * Presents with migraine and depression * Family history ++ * MRI - multiple widespread hyper intense lesion in white matter, basal ganglia, thalamus and pons

Answer 636

CADASIL

Question 637

what is contraindicated in absence seizures

Answer 637

carbamazepine

Question 638

fluent speech intact comprehension poor repetition

Answer 638

conduction aphasia arcuate fasciculus

Question 639

paraneoplastic opsoclonus-myoclonus-ataxia syndrome (POMAS)

Answer 639

Antibodies to RNA-binding protein Nova-1 are not responsible for this patient's findings. Presents with involuntary eye movements (opsoclonus), jerking movements of the limbs (myoclonus), and unsteady gait (ataxia). POMAS is also linked to small cell lung carcinoma but has a distinct clinical presentation from LEMS.

Question 640

Holmes-Adie

Answer 640

* Post viral degeneration in the ciliary ganglion * Pupil is initially dilated with a tonic reaction to light and sectorial vermiform movements * Pupil sphincter is impaired by accommodative spasm —> Miosis (constricted) * Holmes-Adie = Adie’s pupil + hyporeflexia

Question 641

medications precipitating myasthenic crisis

Answer 641

aminoglycosides corticosteroids

Question 642

MG mx

Answer 642

- Cholinesterase inhibitor - Pyridostigmine - MMF, Azathiprine, Cyclophosphamide can be used - Plasmapheresis & IVIG - Rituximab

Question 643

anterior spinal artery occlusion

Answer 643

supplies the anterior 2/3rds of the spinal cord. - segments of the core in watershed areas : T2-T4 are at high risk of ischaemia. - Sudden pain in back and distribution of the affected segment associated with b/l flaccid weakness and dissociated sensory loss - Impaired pinprick sensation and temperature sensation below the level of lesion. - Intact joint position and vibration sense.

Question 644

CO poisoning eye finding

Answer 644

Roth spots

Question 645

hockey stick sign MRI

Answer 645

vCJD

Question 646

paroxysmal hemicrania mx

Answer 646

indomethacin

Question 647

Miller-Fisher syndrome check

Answer 647

IgA levels opthalmoplegia + areflexia + ataxia

Question 648

sudden severe headache +bitemporal hemianopia

Answer 648

pituitary apoplexy elevated prolactin hyponatraemia

Question 649

how does riluzole work

Answer 649

reduces release of glutamate

Question 650

fibrosis neuro drugs

Answer 650

ergot derived dopamine receptor agonists 5-HT2B receptors pergolide -> heart valvular disease

Question 651

gene mutation associations

Answer 651

NOD2/CARD15 mutations are associated with Crohn’s FXN mutations are associated with Friedreich's ataxia GLA mutations with Fabry disease SCN5a mutations are associated with Brugada Syndrome

Question 652

Wernicke's

Answer 652

CAN OPEN

Question 653

GBS poor prognostic factors

Answer 653

age >40 poor upper muscle strength hx of diarrhoeal illness high anti-GM1 ab titre need for ventilatory support

Question 654

dentate nucleus lesion

Answer 654

ataxia

Question 655

red nucleus lesion

Answer 655

tremor

Question 656

* Acute flaccid weakness * Reduced motor function and tone of affected limb * Reduced tendon reflexes of affected limb * Atrophy of affected limb * GIT - nausea, vomititng, abdominal pain, constipation, diarrhoea * Bulbar paralytic poliomyelitis - respiratory muscle atrophy * Aseptic viral meningitis - fever, headache, vomiting

Answer 656

polio Investigations : - viral culture from CSF, stool and pharynx - CSF - lymphocytosis & increased protein - Antibodies to poliovirus Management : - prevention through vaccines : OPV and IPV - injection form - Supportive management

Question 657

FTD/Picks

Answer 657

2 genes - MAPT, GRN most common genetic - hexanucleotide repeat expansion in C9orf72

Question 658

CNS tumours

Answer 658

- Glioblastoma - M/C : regional heterogeneity within a single lesion, highly anapaestic glial cells, vascular proliferation and thrombosis, necrosis. - Glioblastoma - macroscopic : greyish ill defined mass, areas of necrosis & haemorrhage ANaplastic Astrocytoma : - Microscopic : astrocytes, nuclear atypic - Macroscopic : white ill defined mass, extending into gyrus Meningioma : - M/C : pacification, Psammoma bodies, sheets of spindle shaped cells - Gross : rubbery, round lobulated mass, firmly attached to dura Clinical Features : - raised ICP due to mass effect - Seizures

Question 659

- progressive ataxia - early sign - Absence of deep tendon reflexes - Spasticity - Peripheral sensory neuropathy - Dysarthria - early sign of disease - Difficulty swallowing - Muscle weakness - Progressive kyphoscolisosi - Pes cavus - Hammer toes - Heart - hypertrophic cardiomyopathy, myocardial fibrosis, CHF and cardiac arrhythmia - most common cause of death - Pancreas - diabetes mellitus, impaired glucose tolerance - Eyes - optic atrophy - Hearing loss - later stages of disease

Answer 659

Friedrichs ataxia

Question 660

FA mx

Answer 660

coenzyme Q10 and vit E

Question 661

medial medulla lesion

Answer 661

Dejerine syndrome hypoglossal - deviation of tongue weakness of limps due to disruption of descending CS fibres

Question 662

VOR

Answer 662

brainstem reflex -> bs death

Question 663

levetiracetam in liver disease

Answer 663

half if severe phenytoin and valproate contraindicated lamotrigine - massive reduction

Question 664

negri bodies

Answer 664

rabies

Question 665

mallory bodies

Answer 665

alcoholic liver disease

Question 666

diagnostic scan —> Lumbar puncture - RT-QuIC peptide assay 95.8% sensitivity and 100% specificity

Answer 666

for sporadic CJD

Question 667

spinal bulbar muscular atrophy - Kennedy

Answer 667

- proximal weakness and cramps - Wasting - Fasciculations - Weakness - Hyporeflexia - Perioral fasciculations *** - Androgen insensitivity - gynecomastia , infertility - X-linked recessive : CAG trinucleotide repeat

Question 668

palatal myoclonus

Answer 668

central tegmental tract lateral medullary syndrome

Question 669

optic neuritis

Answer 669

if one lesion -> 50% MS if none 25%

Question 670

unilateral dilated pupil which constricts very slowly in response to light + loss of deep tendon reflexes

Answer 670

HA pupil Ross syndrome - also hypohidrosis

Question 671

muscle weakness of quadriceps and long finger flexors asymmetry of signs presents with falls serum CK normal

Answer 671

IBM

Question 672

MMN

Answer 672

Conduction block present - MCB : failure of nerve action potential to propagate across a segment of intact myelinated nerve fibre - MCB —> reduction in amplitude and area of Compound muscle action potential after stimulation - CMAP is normal distal to the affected segment - MCB ==> ulnar and median nerves Causes progressive, asymmetrical and distal upper limb weakness anti-GM1 abs

Question 673

- blurred vision, diplopia, and ptosis secondary to CN III, IV and VI palsies - Dysarthria and dysphagia due to oculobulbar weakness - cranial nerves IX, X and XII - Symmetric descending paralysis - Absent deep tendon reflexes - Infants - hypotonia, weak cry, feeding difficulties - Autonomic dysfunction - urianry retention, postural hypotension, dry mouth

Answer 673

botulism

Question 674

ornithine aminotransferase

Answer 674

atrophy of choroid and retina

Question 675

REM sleep disorder in PD

Answer 675

melatonin

Question 676

syringomyelia can be associated with

Answer 676

T1 chiara malformation Elongated cerebellar tonsils - displaced into upper cervical canal through the foramen magnum

Question 677

Bell's palsy before the geniculate ganglion

Answer 677

hyperacusis and loss of lacrimation

Question 678

natalizumab moa

Answer 678

- Antagonist to alpha 4 - beta 1 integrin found on surface of leucocytes - Inhibits migration of leucocytes across endothelium across the BBB - IV administration

Question 679

fingolimod

Answer 679

- sphingosine 1 phosphate receptor modulator - Prevents lymphocytes from leaving the lymph nodes - Oral formula available

Question 680

global wasting of hand muscles

Answer 680

damage to T1 nerve root

Question 681

phenytoin half life

Answer 681

22 hours 5-14 days to reach steady conc wait 2 weeks after dose change to check serum phenytoin level 3-4mg/kg

Question 682

Binswanger’s disease : cerebral white matter disorder

Answer 682

- occurring in those with a multitude of vascular problems - MRI : diffuse small occlusive vessel disease - C/F : multiple TIAs or lacunar strokes - Complications : dementia, pseudobulbar palsy and gait apraxia

Question 683

Moya Moya disease : occlusive vasculopathy

Answer 683

Occlusion of terminal segments of intra cranial internal carotid arteries and extensive collateral vascularisation gives a typical hazy appearance on cerebral angiograms. C/F : recurrent headache, recurrent TIA and seizures

Question 684

Horner syndrome causes

Answer 684

1st order neuron causes / central causes - anhidrosis of face, arm and trunk 1) syringomyelia 2) Multiple sclerosis 3) Encephalitis 4) Brain tumors 5) Lateral medullary syndrome 2nd order neuron / preganglionin - anhidrosis of face 1) cervical rib 2) Thyroid carcinoma 3) Thyroidectomy 4) Bronchogenic carcinoma 5) Trauma 6) Thoracic artery aneurysm 3rd order - no anhidrosis 1) cluster headache 2) Carotid artery dissection 3) Cavernous sinus thrombosis 4) Middle ear infection

Question 685

tx for Tourette

Answer 685

risperidone

Question 686

types of nystagmus

Answer 686

Downbeat nystagmus - lesion at foramen magnum —> Arnold Chiari malformation- downward gaze — oscillopsia of vision Upbeat nystagmus —> brainstem lesion - lesion between midbrain and medulla Brun’s nystagmus : mild horizontal nystagmus in one direction of gaze and coarse horizontal nystagmus in opposite direction Brainstem compression —> cerebello pontine angle tumors Convergence retraction nystagmus : Parinaud syndrome - dorsal midbrain lesions Pendular nystagmus —> multiple sclerosis

Question 687

Increased signal in pulvinar of thalamus

Answer 687

variant CJD

Question 688

drugs worsening MG

Answer 688

1) D-penicillamine 2) Succinylcholine 3) Aminoglycosides 4) Fluoroquinolones 5) Quinidine, Procainamide 6) Propranolol 7) Lithium 8) Macrolides, Tetracyclines, Gentamicin, Quinolones

Question 689

ADP inhibitors

Answer 689

ADP inhibitors - Clopidogrel, Prasugrel, Ticagrelor, Ticlodipine - MOA : Adenosine Diphosphate inhibitors - P2Y1 and P2Y12 inhibitors - Aspirin 75 mg + Clopidogrel (75mg) / Prasugrel (10mg) / Ticagrelor (90mg) for 12 months followed by aspirin alone - Ticargrelor - dyspnoea : due to reduced Adenosine clearance - Clopidogrel x PPI - Omeprazole, Esmoprazole —> reduced anti platelet effect - Absolute CI to Prasugrel use : 1. Patients with prior stroke or TIA 2. High risk of bleeding 3. Prasugrel hypersensitivity - Ticagrelor contraindicated in : (a)patients with high risk of bleeding (b)History of intracranial haemorrhage (c)Severe hepatic dysfunction (d)Caution in patients with Asthma/COPD - due to higher risk of dyspnea

Question 690

amiodarone in ALS

Answer 690

300 after 3 shocks 150 after 5 shocks

Question 691

amiodarone moa

Answer 691

- Class III anti-arrhythmic drug - Treatment of atrial, nodal and ventricular tachycardias - MOA : Blocks K channel —> inhibits repolarisation and prolongs action potential. - Can also block sodium channels class Ia activity

Question 692

aortic dissection backwards v forwards

Answer 692

- backward tear : Aortic regurgitation, aortic incompetence - Backward tear can give rise to inferior MI - right coronary artery involvement - Forward tear will give rise to unequal BP in arms, stroke, renal failure

Question 693

- narrow pulse pressure - Slow rising pulse - Delayed ESM - Absent S2 - S4 preset - Thrill - LVH can be LVfailure

Answer 693

aortic stenosis

Question 693

ARVC

Answer 693

AD - INV : ECG abnormalities in V1-V3 : T wave inversions. - Epsilon wave in 50% of patients - terminal notch in QRS complex - ECHO - enlarged, hypo kinetic right ventricle with thin free wall - Mx : Sotalol, Catheter ablation to prevent V-tac; implantable cardioverter-defibrilaltor

Question 694

CHADSVASC C - congestive heart failure : 1 point H - hypertension : 1 point A2 - age >= 75 years : 2 points age 65-74 years. : 1 point D - Diabetes : 1 point S2 - prior stroke / TIA / thromboembolism : 2 points V - vascular disease - IHD and PAD : 1 point S - female : 1 point

Answer 694

- score 0 = no treatment - Score 1 = males : consider anticoagulation; females - no treatment - Score 2 or > 2 : anticoagulation

Question 695

Holt Oram syndrome

Answer 695

Ostium secundum - associated with Holt-Oram syndrome : tri-phalangeal thumbs ECG - RBBB with Right axis deviation Osmium primum - presents earlier than ostium secundum; associated with abnormal AV valves ECG : RBBB with LAD, prolonged PR interval

Question 696

Bicuspid aortic valve assoc with

Answer 696

Left dominant circulation ( posterior descending arises from LCX and not RCA) and Turner’s syndrome Complications - AR, AS High risk of aortic dissection and aneurysm formation of ascending aorta

Question 697

bivalirudin

Answer 697

reversible direct thrombin inhibitor

Question 698

features suggestive of VT and not SVT

Answer 698

1) AV dissoc 2) Fusion or capture beats 3) Positive QRS concordance in chest leads 4) Marked LAD 5) History of IHD 6) QRS > 160 ms 7) No response to adenosine or vagal manoeuvres

Question 699

Brugada

Answer 699

- autsomal dominant - Cause of SCD - SCN5A gene - encodes for myocardial sodium ion channel - ECG : convex ST segment election > 2mm in >1 out of V1 - V3 chest leads followed by a negative T wave inversion - Partial RBBB - ECG changes more apparent after giving Flecainide or Ajmaline

Question 700

Brugada

Answer 700

- autsomal dominant - Cause of SCD - SCN5A gene - encodes for myocardial sodium ion channel - ECG : convex ST segment election > 2mm in >1 out of V1 - V3 chest leads followed by a negative T wave inversion - Partial RBBB - ECG changes more apparent after giving Flecainide or Ajmaline

Question 701

cardiac catheterisation

Answer 701

Atrial Septal Defect : - RA, RV, PA - 85% oxygen - LA, LV, Aorta - 100 % oxygen Ventricular septal defect : oxygenated blood mixed in RV and RV onwards increased saturation - RA : 70% oxygen - RV, PA - 85% oxygen - LA, LV, Aorta - 100% oxygen Patent Ductus arteriosus : higher pressure aorta with lower pressure PA - RA, RV : 70% - PA - 85% - LA, LV, Aorta 100% VSD with Eisenmenger’s : - RA, RV, PA : 70% - LA : 100% - LV, Aorta - 85% PDA with Eisenmenger’s : - RA, RV, PA - 70% - LA, LV - 100% - Aorta - 85% ASD with Eisenmenger’s : - RA, RV, PA - 70% - LA, LV, Aorta - 85%

Question 702

HOCM mutation

Answer 702

beta myosin heavy chain

Question 703

ARVC mutation

Answer 703

desmosome

Question 704

causes of dilated cardiomyopathy

Answer 704

1) Alcohol 2) Coxsackie B virus 3) Wet beri beri 4) Doxorubicin

Question 705

causes of restrictive cardiomyopathy

Answer 705

1) amyloidosis 2) Post radiotherapy 3) Loeffler’s endocarditis

Question 706

acquired and secondary cardiomyopathy

Answer 706

Acquired Cardiomyopathy : 1) Peripartum cardiomyopathy —> late month of pregnancy to 5 months postpartum; common with multiple pregnancies 2) Takutsubo cardiomyopathy —> stress induced cardiomyopathy; patient develops chest pain and heart failure. Apical ballooning of myocardium. Secondary cardiomyopathies : 1. Infective : Coxsackie B virus, Chagas disease 2. Infiltrative - amyloidosis 3. Storage : Hemochroamtosis 4. Toxicity - Doxorubicin, Alcoholic 5. Endocrine - DM, Thyrotoxicosis, Acromegaly 6. Inflammatory - Sarcoidosis 7. Neuromuscular - Friedrich’s ataxia, DMD, Myotonic dystrophy 8. Nutritional deficiencies : Wet beri beri 9. Autoimmune - SLE

Question 707

catecholaminergic polymorphic VT

Answer 707

- autosomal dominant, causes SCD. Autosomal dominant —> Defect in Ryanodine receptor RYR2 in the myocardial sarcoplasmic reticulum Autosomal recessive forms may be present which involves mutation of calsequestrin 2 gene C/F - exercise or emotion induced Polymorphic VT causing syncope, SCD Mx - Beta blockers, Implantable cardioverter-defibrillator

Question 708

centrally acting hypertensives

Answer 708

methyldopa monoxidine conidine - a2

Question 709

eosinophilia purpura renal failure livedo reticularis

Answer 709

cholesterol embolism

Question 710

cardiac resync therapy in HF

Answer 710

heart failure and wide QRS; biventricular pacing, Reduces hospitalisation in NYHA Class III patients

Question 711

dipyridamole

Answer 711

inhibits Phosphodiesterase —> elevates cAMP levels —> reduced intracellular Ca levels Also reduced cellular uptake of adenosine and inhibition of thromboxane synthesis

Question 712

DVLA cardiology

Answer 712

HTN >180 - no HGV angioplasty - 1 week ACS - 4 weeks PPM - 1 week ICD - arrhythmia 6mo, prophy 1 mo permanent gr2 ban catheter ablation - 2 days aortic aneurysm >6.5 -> disqualified heart transplant - 6 weeks

Question 713

LAD

Answer 713

- left anterior hemiblock - LBBB - Inferior MI - WPW syndrome - right sided accessory pathway - Hyperkalemia - Ostium primum ASD, Tricuspid atresia - Minor LAD in obese people

Question 714

RAD

Answer 714

- right ventricular hypertrophy - Left posterior hemiblock - Lateral MI - CLD - cor pulmonale - Pulmonary embolism - Osmium secundum ASD - WPW - left accessory pathway - Minor RAD in tall people

Question 715

anteroseptal

Answer 715

V1-4 LAD

Question 716

anterolateral

Answer 716

2,3,avf RCA

Question 717

lateral

Answer 717

1, avl, V5 and 6 LCx

Question 718

posterior

Answer 718

V1-3 LCx and RCA

Question 719

reciprocal changes of STEMI in posterior MI

Answer 719

- horizontal ST depression - Tall broad R waves - Upright T waves - Dominant R wave in V2 Posterior infarct confirmed by ST elevation and Q waves in posterior leads (V7 - V9)

Question 720

digoxin ECG

Answer 720

- down sloping of ST segment - never tick sign or scooped out sign - Flattened / inverted T waves - Short QT interval - AV block, bradycardia - LBBB

Question 721

ECG hypothermia

Answer 721

bradycardia J wave 1st degree heart block - PR prolongation long QT atrial and ventricular arrhythmia

Question 722

short PR interval

Answer 722

WPW

Question 723

S1

Answer 723

- Soft S1 : prolonged PR, MR - Loud S1 : MS, Left to right shunt, short PR interval, atrial premature beats, hyper dynamic states

Question 724

S2

Answer 724

Soft S2 - aortic stenosis * Loud S2 - 1. Hypertension 2. hyper dynamic states 3. ASD w/o pulmonary hypertension * Fixed split in the middle : Atrial septal defect * Widely split S2 : - Deep inspiration - Pulmonary stenosis - Severe MR - RBBB * Reversed / Paradoxical split { P2 occurs before A2 } - LBBB - Severe AS - Right ventricular pacing - WPW - type B — causes early P2 - PDA

Question 725

S3

Answer 725

- due to diastolic filling of ventricle - Heard in LVF (dilated cardiomyopathy), constrictive pericarditis (pericardial knock) and mitral regurgitation.

Question 726

S4

Answer 726

- aortic stenosis, HOCM, Hypertension - Atrial contraction against stiff ventricle - coincides with P wave on ECG - HOCM - double apical impulse due to a palpable S4

Question 727

hydralazine contraindicated in

Answer 727

SLE CVA IHD increased cGMP causing smooth muscle cell contraction

Question 728

palmar xanthoma

Answer 728

remnant hyperlipidaemia

Question 729

eruptive xanthoma

Answer 729

familial hypertriglyceridemia, Lipoprotein lipase deficiency

Question 730

tendon xanthoma, tuberous xanthoma, xanthelasma

Answer 730

familial hypercholesterolemia, remnant hyperlipidemia Xanthelasma - yellowish papule and plaques cased by localised accumulation of lipid deposits on the eyelid. Mx : surgical excision / topical trichloroacetic acid / laser therapy / electrodesiccation

Question 731

poor prognostic factors in IE

Answer 731

S aureus prosthetic valve culture -ve low complement levels

Question 732

indications for surgery in IE

Answer 732

1. Severe valvular incompetence 2. Aortic abscess - indicated by a lengthened PR interval 3. Infections resistant to Abx and anti-fungals 4. Cardiac failure refractory to standard medical treatment 5. Recurrent emboli after antibiotic therapy

Question 732

IE mx

Answer 732

initial blind - amox and gent pen allergy/MRSA -> vanc and gent prosthetic valve - vanc + rif + gent native Staph - fluclox pen allergy-> vanc and rif prosthetic Staph - benpen pen allergy -> vanc+rif+gent endocarditis by sensitive species of strep viridans - benpen pen allergy -> vanc+gent less sens streptococci - benpen + gent pen allergy -> vanc + gent

Question 733

non pulsatile JVP

Answer 733

SVCO

Question 734

Kussmaul's sign

Answer 734

paradoxical rise of jvp constrictive pericarditis

Question 735

large a wave on JVP

Answer 735

TS/PS/pulm htn

Question 736

cannon a wave on JVP

Answer 736

CHB, V tac, ventricular ectopics, nodal rhythm, single chamber ventricular pacing

Question 737

large v waves on jvp

Answer 737

TR

Question 738

long qt syndrome

Answer 738

inherited conditions associated with delayed repolarization of ventricles. - common defects are LQT1 and LQT2 gene mutations - affects the slow delayed rectifier potassium channels - LQT1 : Exertional syncope - swimming - LQT2 : emotion / exercise / auditory stimuli - LQT3 : at rest or at night

Question 739

congenital long QT

Answer 739

1. Jervell-Lange-Nielsen syndrome : includes deafness 2. Romano Ward syndrome - no deafness

Question 740

other causes of long QT

Answer 740

amiodarone sotalol class 1a anti-arrhythmic TCA SSRI methadone chloroquine terfenadine erythromycin haloperidol ondansetron hypoCa hypoK hypoMg acute MI myocarditis hypothermia SAH Mx - BB, ICD

Question 741

loop diuretics AEs

Answer 741

1) Hypotension 2) Hyponatremia 3) Hypokalaemia 4) Hyomagnesemia 5) Hypocalcemia 6) Hypochloraemic alkalosis 7) Ototoxicity 8) Renal impairment - dehydration + direct toxic effect 9) Hyperglycaemia 10)Gout

Question 742

Ebstein's anomaly

Answer 742

adherence of septal and posterior leaflets of tricuspid valve to underlying myocardium - Downward (apical) dispalvemnt of functional annulus of tricuspid valve - Dilation of atrailised right ventricle with varying degree of hypertrophy and thinning of wall - Redundancy, fenestration and tethering of anterior leaflet of tricuspid valve - Dilation of right atrioventricular junction

Question 743

clopidogrel inhibition

Answer 743

CYP2C19 by omeprazole

Question 744

moxonidine moa

Answer 744

imidazoline receptors -> reduced sympathetic outflow

Question 745

key diagnostic tests to identify patients likely to benefit from cardiac resynchronisation therapy

Answer 745

TTE ECG

Question 746

treatment of prinzmetal angina

Answer 746

felodipine

Question 747

ESM louder on inspiration vs expiration

Answer 747

PS, ASD - ins AS, HOCM - exp TOF also has ESM

Question 748

PSM types

Answer 748

MR - high pitched, blowing TR - inc on insp VSD - harsh

Question 749

late systolic murmurs

Answer 749

MVP, coarctation

Question 750

early diastolic murmur

Answer 750

AR - high pitched, blowing Graham steel - PR - high pitched, blowing

Question 751

mid-late diastolic murmur

Answer 751

MS - rumbling Austin flint - severe AR

Question 752

most common cause of death after MI

Answer 752

cardiac arrest due to VF

Question 753

bradyarrhythmias after MI

Answer 753

inferior - AV block

Question 754

pericarditis in first

Answer 754

48 hours after transmural MI

Question 755

Dresslers syndrome

Answer 755

2-6 weeks after MI, autoimmune reaction to antigenic proteins give NSAIDs

Question 756

persistent ST elevation and LVF following MI

Answer 756

LV aneurysm inc risk of stroke

Question 757

LV free wall rupture

Answer 757

1-2 weeks afterwards, acute HF 2y to cardiac tamponade - JVP, pulsus paradoxus, diminished HS pericardiocentesis urgently

Question 758

VSD

Answer 758

within first week -> acute HF associated with PSM

Question 759

acute MR

Answer 759

infero-posterior infarct early-mid systolic murmur acute hypotension, pulmonary oedema

Question 760

MI 2y prevention

Answer 760

DAPT ACEI BB statin

Question 761

for 4 weeks after MI

Answer 761

avoid sexual activity avoid PDE5 inhibitors WITH nitrates, nicorandil

Question 762

thrombolysis med moa

Answer 762

tissue plasminogen activator

Question 763

ECG within how long of thrombolysis

Answer 763

90 minutes to assess if >50% reduction in ST elevation if no -> rescue PCI

Question 764

cardiac resync therapy

Answer 764

BiV pacing RA LA LV

Question 765

which BB not used in AF

Answer 765

sotalol

Question 766

fondaparinux moa

Answer 766

AT3 activator inhibits factor Xa

Question 767

bivalirudin

Answer 767

direct thrombin inhibitor

Question 768

risk factors for asystole in brady

Answer 768

- complete heart block with broad complex QRS - Recent asystole - Mobitz type II AV block - Ventricular pause > 3 seconds

Question 769

naftidofuryl oxalate

Answer 769

vasodilator in patients with poor QoL

Question 770

cilotazol

Answer 770

PDE3 inhibitor - both antiplatelet and vasodilator effects

Question 771

vasodilator testing for PAH

Answer 771

oral CCBs if +ve response -ve response -> prostacycline analogues endothelin receptor antagonist selective receptor A antagonist phosphodiesterase inhibitor

Question 772

pulsus paradoxus

Answer 772

greater than normal (>10mm Hg) fall in BP during inspiration Faint or absent pulse on inspiration Dx - severe asthma, cardiac tamponade

Question 773

pulsus alternans

Answer 773

Pulsus alterans : regular alteration of the force of arterial pulse Seen in severe LVF

Question 774

bisferiens pulse

Answer 774

double pulse two peaks in systole Seen in mixed aortic valve disease Can also be seen in HOCM

Question 775

nicorandil promotes efflux of

Answer 775

K+

Question 776

. Cardiac enzyme affected early on in myonecrosis : specific to cardiac muscle

Answer 776

-> glycogen phosphorylase isoenzyme BB, increased 1-3 hours after myocardial event

Question 777

haemolytic anaemia with which valve type

Answer 777

mechanical more than bioprosthetic

Question 778

complete heart block after inferior MI

Answer 778

not for pacing is if anterior

Question 779

mitral stenosis opening snap

Answer 779

leaflet mobility

Question 780

features of severe AS

Answer 780

narrow pulse pressure slow rising pulse delayed ESM soft/absent S2 S4 thrill duration of murmur LVH/F

Question 781

antithrombotic therapy in prosthetic heart valves

Answer 781

bio - aspirin mech - warfarin and aspirin INR target: aortic 3 mitral 3.5

Question 782

Keshan disease

Answer 782

selenium deficiency -> dilated CM

Question 783

most common cause of restrictive CM

Answer 783

amyloid

Question 784

neprilysin

Answer 784

ANP and BNP are inactivated by this hence inhibitor - sacubitril increases risk of angioedema

Question 785

hyperlipoproteinemia

Answer 785

- type I hyperlipoproteinaemia : hyper chylomicronaemia - associated with eruptive xanthomas. Complications include occlusion of retinal vein, acute pancreatitis, steatosis, organomegaly and lipaemia retinals. - Type II hyperlipoproteinaemia : associated with xanthoma tendinosum - nodular swellings on tendons. Type II hyperliproteinaemia classified further into types IIa and IIb : whether increase in triglyceride occurs in addition to increase in lLDL-colesterol. - Type III hyperlipoproteinaemia : tuboeruptive xanthomas - due to high numbers of chylomicron and high levels of intermediatedensity lipoprotein. - Most common cause : apolipoprotein E2/E2 genotype. - Type III is due to cholesterol rich VLDL. - Associated with hypercholesterolaemia and hypertriglyceridemia, normal concentration of apolipoprotein B - Two types of skin signs - 1) palmar xanthomata or orange discolouration of skin palmar creases 2) tuboeruptive xanthomata on elbows and knees. - Associated with early onset CVD and PVD - type IV hyperlipoproteinaemia : associated with eruptive xanthomas - Type V hyperlipoproteinaemia : mixed hyperlipoproteinaemia familiar or mixed hyperlipidemia. High levels of VLDL + high chylomicron. Associated with glucose intolerance and hperuricemia.

Question 786

sick sinus syndrome

Answer 786

periods of sinus brady sinus arrest SA+AV vonduction defect SVT possible PPM

Question 787

cardiac catheterisation and thyroid

Answer 787

* Cardiac catheterisation requires the use of an iodine-containing contrast. * This may worsen hyperthyroidism caused by toxic multinodular goitre, whereas it may improve the symptoms in patients with Grave's disease (Wolff-Chaikoff effect).

Question 788

epsilon waves

Answer 788

RV conduction delay ARVC

Question 789

commonest site of obstruction in coarctation

Answer 789

distal to origin of L subclav

Question 790

LQT1 syndrome

Answer 790

- LQTI gene = KCNQ1 on chromosome 11 - KCNQ1 codes for voltage gated potassium channel KvLQT1 and is highly expressed in heart - This is a slow delayed rectifier potassium channel LQT2 - KCNH2

Question 791

amiodarone only given through

Answer 791

central line

Question 792

S1

Answer 792

R wave - Quiet S1 : MR, Long PR interval, Obesity, 1st degree heart block, LBBB - Loud S1 : MS, Hyperdynamic states, Tachycardia, low BMI, Atrial Myxoma - Widely split S1 - RBBB, Ebstein’s anomaly, Ventricular tachycardia

Question 793

S2

Answer 793

Loud S2 - hypertension - Quiet S2 - aortic stenosis - Wide split S2 - RBBB, Pulmonary stenosis, VSD, MR - Fixed split S2 - ASD - Paradoxical split S2 - LBBB, PDA, Aortic stenosis. HOCM

Question 794

S4

Answer 794

- Left sided S4 : HTN, hypertrophic cardiomyopathy, aortic stenosis - Right sided S4 : pulmonary stenosis, Pulmonary HTN

Question 795

thiazide diuretics in pregnancy

Answer 795

neonatal thrombocytopenia, bone marrow suppression, jaundice, electrolyte disturbances, hypoglycaemia

Question 796

Eisenmenger complications

Answer 796

- polycythemia - bleeding disorder - cerebral embolism or abscess - Avoid pregnancy - Syncopal episodes can precede death

Question 797

restrictive cm

Answer 797

secondary to Amyloidosis with immunocyte dyscrasia - Right heart failure, raised JVP, deep Y descent - Heart size commonly is normal - Apex frequently non palpable due to thick pericardium - CXR : pericardial calcifications - ECG : diffusely diminished voltages - ECHO - small, thick ventricles, and thick intertribal septum due to amyloid deposit which leaves a granular sparkling appearance

Question 798

lutembacher syndrome

Answer 798

Ostium secundum (ass. With RBBB with RAD) in combination with Rheumatic mitral stenosis

Question 799

early graft occlusions after CABG

Answer 799

5-10% within 30 days aspirin reduces

Question 800

primary pum htn gene

Answer 800

BMPR2 ALK3, ENG, SMAD9, CAV1, KCNK3

Question 801

tricuspid regurg

Answer 801

loss of x descent and fusion of c and v to create -> giant v

Question 802

defib voltage

Answer 802

monophasic 360J biphasic 150-200J

Question 803

PPM indications

Answer 803

- persistent symptomatic bradycardia - Trifasicular block - Mobitz Type 2 AV block - Sinus pause > 3 seconds

Question 804

stabbing chest valve

Answer 804

pulmonary as anterior

Question 805

contraindications to carotid sinus massage

Answer 805

- myocardial infarction - TIA in the last 3 months - Cerebrovascular accident in last 3 months - Carotid artery occlusion - Previous ventricular arrhythmia

Question 806

chemo assoc with CVD

Answer 806

Doxorubicin, Daunorubicin, Idarubicin, Mitoxantrone

Question 807

SVT in pregnancy

Answer 807

use metoprolol

Question 808

adenosine moa

Answer 808

purine nucleoside - rapid IV injection - slows conduction through AV node by acting as a G protein-coupled receptor agonist of Adenosine A1 receptor on AV node - inhibition of adenylate cyclase —> reduction cAMP

Question 809

subclavian steal syndrome

Answer 809

occlusion or stenosis of proximal SCA leading to decreased flow in the IL vertebral artery

Question 810

drug induced TdP

Answer 810

- Antiarrhythmic drugs (e.g. sotalol, quinidine, disopyramide, procainamide, flecainide) - Certain non-sedating antihistamines (e.g. terfenadine and astemizole) - Antibiotics - Erythromycin, clarithromycin, azithromycin, levofloxacin, moxifloxacin, gatifloxacin, trimethoprim-sulfamethoxazole, clindamycin, pentamidine, chloroquine - Antifungals - Ketoconazole, itraconazole - Certain psychotropic medications (e.g. risperidone, haloperidol, phenothiazines, thioridazine, trifluoperazine, sertindole, zimeldine, ziprasidone) - Tricyclic and tetracyclic antidepressants - Certain gastric motility agents (e.g. cisapride) - Lithium.

Question 811

sotalol has both

Answer 811

Class III agents : blocks cardiac potassium channels Class II - beta blockers

Question 812

broad complex irregular tachy in structurally normal heart

Answer 812

procainamide

Question 813

S wave occurs between

Answer 813

S1 and S2 T wave - occurs between S2 and S3 U wave - depolarisation of Purkinje fibers

Question 814

statins monitoring

Answer 814

LFTs baseline 3 12 if 3x ULN then switch

Question 815

hydralazine moa

Answer 815

increases cGMP leading to smooth muscle relaxation in arterioles more than veins

Question 816

pt on warfarin undergoing emergency surgery

Answer 816

4 factor PCC

Question 817

area of the heart causing a flutter

Answer 817

tricuspid valve isthmus - ablate

Question 818

romana's sign

Answer 818

trypanosomiasis - chagas

Question 819

valsalva increases murmur in

Answer 819

HOCM MVP

Question 820

valsalva quietens murmurs in

Answer 820

AS, PS, TR

Question 821

squatting increases murmurs of

Answer 821

VSD, AS, aortic insufficiency and mitral insufficiency Squatting causes quiet murmurs in HOCM

Question 822

drugs causing pulmonary hypertension

Answer 822

methamphetamine fenfluramine

Question 823

carcinoid heart disease

Answer 823

TS and R due to semi-closed valve Liver metastasis of carcinoid syndrome —> releases serotonin and kallikrein which travels through liver sinusoids —> central vein of each lobule —> hepatic veins —> IVC and right heart Increased serotonin and kvllikrein — bronchospasm, diarrhoea, flushing

Question 824

HOCM mutations

Answer 824

MYH7 MYBPC3

Question 825

EF equation

Answer 825

EDV-ESV / EDV

Question 826

allergic myocarditis to medication

Answer 826

co-trimoxazole

Question 827

Type A vs B aortic dissection sx

Answer 827

hypotension and syncope vs hypertension

Question 828

AIN

Answer 828

drug causes - penicillin, rifampicin, NSAIDs, furosemide, allopurinol interstitial oedema and infiltrates in connective tissue between renal tubules

Question 829

pre-renal vs ATN

Answer 829

ATN has more urine sodium, poor response to fluid challenge, normal urea:creatinine, brown granular casts pre-renal -> kidneys hold on to sodium to preserve volume

Question 830

ADPKD genes

Answer 830

T1 - chr 16 2 - chr 4, later presentation

Question 831

most common form of amyloidosis

Answer 831

AL nephrotic syndrome, cardiac and neurological involvement, macroglossia, periorbital ecchymoses

Question 832

amyloidosis where renal involvement is most common

Answer 832

AA amyloid

Question 833

normal anion gap metabolic acidosis

Answer 833

- GI bicarbonate loss - diarrhoea, ureterosigmoidostomy, fistula - RTA - Drugs - Acetazolamide - Ammonium chloride injection - Addison’s disease

Question 834

Goodpasture's mx

Answer 834

PEx steroids cyclophosphamide

Question 835

CKD iron

Answer 835

Elevate hepcidin levels leads to decreased iron absorption from gut and impaired release of stored iron from macrophages and hepatocytes This causes reduced iron levels available for erythropoiesis In CKD - metabolic acidosis inhibits the conversion of Fe3+ to Fe2+ in the duodenum leading to reduced iron absorption.

Question 836

CKD bone disease

Answer 836

low vit D due to low alpha-1 hydroxylation in kidneys high phosphate low Ca due to lack of vit D and high phosphate 2y hyperPTH - due to low calc, high phosph and low vit D

Question 837

CKD bone disease mx

Answer 837

* Phosphate binders: - calcium based binders : problems include hypercalcemia and vascular calcification - Sevelamer : non calcium based binder, binds to dietary phosphate and prevents its absorption. - Sevelamer can also reduce uric acid levels and improves lipid profiles of patients with CKD * Vitamin D : alfacalcidiol, calcitriol

Question 838

renal transplant rejection

Answer 838

hyperacute - immediate acute - mononuclear cells predominate chronic - HLA mismatch, vascular changes with myointimal proliferation leading to organ ischaemia

Question 839

complications of renal transplant

Answer 839

* Renal artery thrombosis : sudden loss of urine output; immediate surgery, delays > 30 minutes high rate of graft loss * Renal artery stenosis : uncontrolled hypertension, allograft dysfunction and oedema; Mx - angioplasty * Renal vein thrombosis : pain and swelling over graft site, hematuria and oliguria * Urine leaks : diminished urine output, rising creatinine, fever, abdominal pain; USG - perigrafj collection, necrosis of ureter tip and anastomosis revision is needed * Lymphocyte : presents as a mas; drained with percutaneous technique and sclerotherapy

Question 840

cystinuria

Answer 840

AR * Recurrent formation of renal stones * Defect in membrane transport of cysteine, ornithine, lysine and arginine COLA * Chromosome 2 - SLC3A1 gene * Chromosome 19 - SLC7A9 gene * Recurrent renal stones * Yellow and crystalline, semi-opaque on X-ray * Dx : Cyanide - nitroprusside test * Mx : hydration, D-penicillamine, urinary alkalisation

Question 841

genetic nephrogenic DI

Answer 841

ADH receptor high plasma low urine osm

Question 842

adverse effects of EPO

Answer 842

- accelerated hypertension —> encephalopathy & seizures - Bone aches - Flu-like symptoms - Skin rashes, urticaria - Pure red cell aplasia - reduced risk with Darbepoietin - Raised PCV increases risk of thrombosis -Iron deficiency secondary to increased erythropoiesis

Question 843

Fanconi syndrome

Answer 843

- generalised reabsoprtive disorders of renal tubular transport in PCT - Type 2 RTA - Polyuria - Aminoaciduria - Glucosuira - Phophaturia - Osteomalacia

Question 844

most common cause of fanconi in children

Answer 844

cystinosis

Question 845

mixed nephrotic/nephritic presentation

Answer 845

- diffuse proliferative glomerulonephritis - post streptoccolal GN in children, most common form of renal disease in SLE - MPGN = mesangiocapillary - type 1 —> cryoglobulinemia, hepatitis C; type 2 : Partial lipodystrophy

Question 846

nephrotic syndrome

Answer 846

- MInimal change disease : child with nephrotic syndrome, causes - Hodgkin’s, NSAIDs; good response to steroids - Membranous glomerulonephritis - proteinuria, nephrotic syndrome, chronic kidney disease; causes : infection, rheumatoid drugs, malignancy; 1/3 develop CKD, 1/3 resolve, 1/3 respond to cytotoxic - Focal segmental glomerulosclerosis : secondary to HIV, heroin; presentation - proteinuria, nephrotic syndrome , chronic kidney disease

Question 847

HUS

Answer 847

triad of AKI, MAHA, thrombocytopenia primary/atypical - complement dysreg -> eculizumab *C5 inhibitor mab

Question 848

HSP

Answer 848

IgA mediated small vessel vasculitis mx - analgesia

Question 849

IgA nephropathy

Answer 849

Associated with - - alcoholic cirrhosis - Coeliac disease - Dermatitis herpetiformis - Henoch Schonlein purpura - due to mesangial deposition of IgA immune complexes - Mesangial hypercelluairty, +ve IF for IgA & C3 - young male, recurrent macroscopic hematuria - Associated with recent URTI - 25% patients develop ESRF - Good prognosis - frank hematuria - Poor prognosis : male gender, proteinuria, HTN, smoking, hyperlipidemia, ACE genotype D

Question 850

Membranoproliferative GN / Mesangiocapillary GN

Answer 850

- nephrotic syndrome / hematuria / proteinuria - Type 1 - cryoglobulinemia, hepatitis C - Renal Bx : tram track appearance due to subendothelial and mesangial immune deposits - Type 2 - dense deposit disease - Caused by partial lipodystrophy, factor H deficiency - Persistent activation of complement pathway - low circulating levels of C3 - C3b nephritic factor can also be found in 70% of cases - C3b nephritic factor stabilises C3 converts - Renal Bx : EM - intramembranous immune complex deposits with dense deposits - type 3 : hepatitis B, hepatitis C

Question 851

membranous glomerulonephritis

Answer 851

* Sub epithelial deposits - spike and dome appearance * Idiopathic - anti-phospholipase A2 antibodies * Infections : hepatitis B, malaria, syphilis * Malignancy * Drugs - gold, penicillamine, NSAIDs * SLE, thyroiditis Good prognosis : female sex, young age, asymptomatic proteinuria at time of presentation

Question 852

peritoneal dialysis infection rx

Answer 852

vanc/teic + ceftaz

Question 853

complications of plasma exchange

Answer 853

* Hypocalcemia * Metabolic alkalosis * Coagulation factor depletion * Immunoglobulin depletion

Question 854

bicalutamide moa

Answer 854

non steroidal anti-androgen, blocks androgen receptor Cyproterone acetate - steroidal anti androgen, prevents DHT binding from intra -cytoplasmic protein complexes Abiratone - androgen synthesis inhibitor

Question 855

pollakiuria

Answer 855

risperidone

Question 856

renal cell ca paraneoplastic effects

Answer 856

- erythropoietin = polycythemia - PTH related peptide = hypercalcemia - Renin - Paraneoplastic hepatic dysfunction syndrome = cholestasis, hepatosplenomegaly - Varicocele - left sided, due to tumor compressing veins - Stauffer syndrome - cholestasis/hepatosplenomegaly, secondary to increased levels of IL-6 - Receptor tyrosine kinase inhibitors - Sorafenib, Sunitinib - better efficacy than IFN-alpha

Question 857

when to use percutaneous nephrolithotomy

Answer 857

>20mm renal stone

Question 858

renal stones prevention

Answer 858

Renal stones prevention - - calcium stones : high fluid intake, thiazide diuretics - Oxalate stones : CHolestyramine, pyridoxine - Uric acid stone - allopurinol, urinary arlkalinization Urate stones R/F : gout, ileostomy Drug causes of renal stones : loop diuretics, steroids , Acetazolamide, Theophylline

Question 859

what causes hyperacute rejection

Answer 859

antibodies against ABO or HLA antigens ; type II hypersensitivity reaction

Question 860

method of acute graft rejection

Answer 860

mismatched HLA - cell mediated - cytotoxic T cells ; CMV can also cause acute graft rejection

Question 861

membranous nephropathy

Answer 861

commonest type in adults associated with SLE, malignancies of the bowel and Bronchus, penicilliamine therapy, and hepatitis B infection. Can also be associated with plasmodium malaria infection in the tropics. Strong association with HLA-DR3 Commonly seen in males

Question 862

what type of lupus nephritis

Answer 862

diffusive proliferative type

Question 863

testicular tumour markers

Answer 863

- seminomas : hCG - Non seminomas : AFP and/or hCG - Germ cell tumors - LDH

Question 864

COLT Pee

Answer 864

C : Carbonic anhydrase - PCT O : Osmotic diuretics - loop of Henle L : Loop diuretics : ascending limb of loop of Henle T : Thiazide diuretics - early DCT P : Potassium sparing diuretics - distal section of DCT

Question 865

Type 2 MCGN

Answer 865

idiopathic or after measles persistent complement pathway activation low circulating C3 mesangial cell proliferation partial lipodystrophy and factor H deficiency

Question 866

Type 1 MCGN

Answer 866

tramline appearance C/F : hematuria, proteinuria, nephrotic syndrome or frank kidney disease Low C3, C4. Infections with Hepatitis B, hepatitis C, cryoglobulinemia

Question 867

IgA nephropathy

Answer 867

Hematuria , Hypretension Recurrent episodes of micro- or macro-hematuria. IgA nephropathy - familial. Elevated IgA levels Circulating IgA immune complexes and abnormal IgA glycosylation IgA deposition may occur in cirrhosis and gluten enteropathy

Question 868

ATN dx

Answer 868

1) urine osmolality < 350 mOsm / kg 2) Urinary sodium > 40 mmol/L 3) FeNa > 1%

Question 869

abdominal aortic aneurysm

Answer 869

- thought to be an auto allergic response from the leak f ceroid material out of atherosclerotic plaques - Ureters become embedded in dense retroperitoneal fibrous tissue —> unilateral or bilateral ureteric obstruction. - May extend from L2 to pelvic brim - Excretion Urography - ureteric obstruction at level of pelvic brim - Mx : corticosteroids and surgical stenting of ureteric obstruction

Question 870

TB urinary tract

Answer 870

CT urography -> cavitating lesions in renal papillary areas commonly with calcification hydronephrosis

Question 871

cystinuria profile

Answer 871

- defective renal tubular reabsorption and jejunal absorption of cysteine and other dibasic amino acids - Ornithine, Lysine, Arginine - SLC3A1 - chromosome 2 - SLC7A9 - chromosome 19 - Mx : hydration, Penicillamine, urinary arlkalinization

Question 872

Bartter vs Gitelman

Answer 872

inc vs reduced excretion of Ca

Question 873

aristolochic acid nephropathy

Answer 873

Chinese herb nephropathy and Balkan endemic nephropathy Anaemia, rapid renal atrophy and fibrosis, proteinuria and high incidence of genitourinary cancer.

Question 874

Fabrys

Answer 874

- X linked lysosomal storage disorder - Myelin deposits in tubular epithelium and vascular epithelium causing ischaemic nephropathy - Defect in enzyme alpha glucosidase - Dx : slit lamp examination of cornea - M/C of urie sedan - Maltese cross lipid globules - Skin angiokeratomas - Decreased sweating - Leg lymphoedema - Peripheral neuropathy - Cardiac - complete heart block, left ventricular hypertrophy, restrictive cardiomyopathy

Question 875

LIDDLE

Answer 875

Low Hydrogen ions - metabolic acidosis I - high BP D - autosomal dominant D - drugs - amiloride L - low potassium E - ENaC activation * Increased Na levels, increased water retention, hypertension * Low Potassium and low hydrogen ions * Hypokalaemia, metabolic alkalosis, with HTN and volume overload

Question 876

RTAs

Answer 876

T1 - distal tubule hypoK nephrocalcinosis and renal stones RA, SLE, Sjogrens, ampho B, analgesics T2 - proximal hypoK osteomalacia Fanconi, Wilsons, cystinosis, tetracyc, CA inhib T3 - mixed CA 2 deficiency hypoK T4 -proximal ammonium, reduced aldosterone hyperK hypoaldosteronism, diabetes

Question 877

tremors renal med

Answer 877

tacrolimus

Question 878

HIVAN

Answer 878

rapidly advancing nephropathy large echogeneic kidneys on USS light microscopy - FSGS - podocyte proliferation with marked focal collapse of the glomerular basement membrane

Question 879

abiraterone acetate

Answer 879

cytochrome P450 17 alpha hydroxylase treating metastatic castration resistant (hormone-relapsed) prostate cancer in adult men who are asymptomatic or mildly symptomatic after failure of androgen deprivation therapy in whom chemotherapy is not yet clinically indicated or progression despite docetaxel regimen

Question 880

dialysis related amyloidosis

Answer 880

* Due to accumulation of beta-2 micro globulin * Component of class 1 HLA and is normally filtered at the glomerulus, reabsorbed and metabolised in the PCT. * Accumulation of beta-2 micro globulin in CKD patients occur in HD patients after at least 5 years. * C/F : Carpal tunnel syndrome, chronic arthropathy of shoulders b/l, followed by knees, wrists and small joints of hands; periarticular soft-tissue swelling may be seen.

Question 881

renal transplant immunosuppression

Answer 881

- Cardiovascular disease : Tacrolimus and Ciclosporin can cause hypertension and hyperglycaemia. - Tacrolimus - hyperlipidemia - Monitor patients for accelerated cardiovascular disease - Renal failure : nephrotoxic effects of Tacrolimus and Ciclosporin; graft rejection; recurrence of original disease in transplanted kidney - Tacrolimus - hyperkalemia common side effect - MMF - hyperkalemia is seen, lesser incidence than with Tacrolimus - Valganciclovir - reduces renal function, not as common as hyperkalemia with Tacrolimus Immunosuppression : 1. INITIAL : Ciclosporin / Tacrolimus + monoclonal antibody 2. MAINTENANCE : Ciclosporin / Tacrolimus + MMF 3. Add steroids if more than one steroid responsive acute rejection episode Monoclonal antibody = selective IL-2 inhibitors —> Daclizumab, Basilximab

Question 882

haemodialysis most common malnutrition

Answer 882

protein calorie

Question 883

where is EPO released

Answer 883

interstitial fibroblasts in renal cortex extra-renal -> perisinusoidal cells in liver

Question 884

Goodpasture's lung biopsy

Answer 884

haemosiderin laden macrophages

Question 885

hepatorenal syndrome

Answer 885

splanchnic vasodilation, renal vasoconstriction. Splanchnic vasodilation releases Nitric oxide which dilates the splanchnic and systemic due to portal hypertension. This causes arteriolar underselling, increasing bacterial translocation. Ultimately, systemic vascular resistance falls and renal vasoconstriction occurs. Medical management : Midodrine, Somatostatin, Dopamine

Question 886

diabetes renal changes

Answer 886

renal glomerulosclerosis Also prone to papillary necrosis. Thickening of GBM and glomerulosclerosis - Kimmelsteil Wilson lesions

Question 887

indications for emergency dialysis

Answer 887

hyperkalemia - Refractory pulmonary edema - Refractory metabolic acidosis pH < 7.2 - Uraemic complications - pericarditis, encephalopathy

Question 888

patients with lithium related DI

Answer 888

may benefit from taking amiloride 5-10mg/day

Question 889

Patient with chronic alcohol and raised anion gap —> check Na and K

Answer 889

If normal —> points towards lactic acidosis If abnormal —> alcohol ketoacidosis - excessive vomiting would cause loss of Na, K and Cl

Question 890

causes of large kidneys

Answer 890

amyloidosis diabetic neph stg1 hydronephrosis acromegaly renal vein thrombosis ADPKD

Question 891

loin pain-haematuria syndrome

Answer 891

- poorly defined disorder characterised by loin / flank pain - severe nature + hematuria - Co-existent thin basement membrane disease - Bleeding into loin and obstruction causes the pain - Dependency on narcotics is common

Question 892

cystinuria crystals

Answer 892

hexagon shaped

Question 893

what stimulates renin secretion

Answer 893

low Na low blood volume

Question 894

haemodialysis processes

Answer 894

solutes - diffusion water and fluids - ultrafiltration

Question 895

CKD diminishes activity of

Answer 895

one alpha hydroxylase, which leads to failure of converting CholeCalciferol to its active metabolite 1,25 - dihydroxycholecalciferol

Question 896

where does Na get reabsorbed in the nephron

Answer 896

PCT

Question 897

xanthogranulomatous pyelo

Answer 897

macrophage increase related to malakoplakia palpable unilateral flank mass - anorexia feveres pain CT - replacement of renal parenchyma with rounded, low density areas surrounded by ring of enhancement later - AA amyloid pigment laden macrophages

Question 898

negative acute phase proteins

Answer 898

albumin transthyretin transferrin retinol binding protein cortisol binding protein

Question 899

positive acute phase reactants

Answer 899

CRP procalcitonin ferritin fibrinogen alpha1antitryp caeruloplasmin amyloid haptoglobulin complement

Question 900

androgen insensitivity syndrome

Answer 900

* X-linked recessive * End organ resistance to testosterone in genotypical male children 46XY to have female phenotype * Complete androgen insensitivity syndrome = testicular feminisation syndrome * C/F : primary amenorrhoea, little or no pubic and axillary hair, undescended tests, breast development due to increased conversion of testosterone to estradiol chromosome analysis - 46XY

Question 901

autoimmune polyendocrinopathy syndrome

Answer 901

APS 1 - AIRE1 on chr 21 - chronic mucocutaneous candidiasis, Addison's disease, primary hypoparathyroidism APS 2 - HLA DR3/4 - Addisons, T1DM, thyroid

Question 902

carbimazole moa

Answer 902

blocks thyroid peroxidase from coupling and iodinating the tyrosine residues

Question 903

salt wasting crisis

Answer 903

dehydration hypotension electrolyte imbalance can occur in CAH

Question 904

gold standard for non-salt losing CAH

Answer 904

corticotrophin stimulation test

Question 905

management of Cushings

Answer 905

- Metyrapone : normalises cortisol levels where surgery is not possible - Mitotane - adjunct to chemotherapy - Primary treatment - surgical resection of tumor - B/L Adrenalectomy —> Nelson’s syndrome : high ACTH from removal of negative feedback of Hpothalamic - pituitary - adrenal axis ; causes skin pigmentation Insulin stress test - to differentiate between true Cushing’s syndrome and Pseudo-Cushing’s

Question 906

antidiabetic drugs

Answer 906

1) Insulin - direct replacement of endogenous insulin - Sub-Q * Main side effects : hypoglycaemia, weight gain, lipodystrophy 2) Metformin * increases insulin sensitivity, decreases hepatic gluconeogenesis * GI upset, lactic acidosis type B * First line * CI in renal failure with eGFR < 30 mL/min 3) Sulfonylureas * Stimulate pancreatic beta cells to secrete more insulin * Glimeperide, Gliclazide * Adverse effects : weight gain, fluid retention * CI - heart failure 4) Thiazolidinediones * Activates PPAR-gamma receptor in adipocytes to promote adipogenesis and fatty acid uptake * Adv - weight gain, fluid retention * Pioglitazone * CI - heart failure 5) DPP-4 inhibitors - Vidagliptin, Sitagliptin * Gliptins * Increases incretin levels which inhibits glucagon secretions * Increases incretin levels by decreasing their breakdown * Well tolerated * Risk of acute pancreatitis 6) SGLT-2 inhibitors - Canagliflozin, Dapagliflozin, Empagliflozin * Inhabits reabsoprtion og glucose in kidney * Reversible inhibiton of SGLT-2 in renal PCT to decrease glucose reabsorption and increases glucose urinary secretion * UTI, Fournier gangrene * Normoglycaemic ketoacidosis * Increased risk of lower limb amputation * Typically results in weight loss 7) GLP-1 agonists * -tides * Incretin mimetic which inhibits glucagon secretion * Subcutaneous * Adv - nausea, vomiting, pancreatitis * Weight loss GLP-1 agonists / mimetics = Exenatide , Liraglutide - increases insulin secretion - Decreases glucagon secretion - Weight loss - Sub-Q injection within 60 minutes before morning and evening meals Consider adding Exenatide to Metformin + Sulfonylurea if : - BMI > 35 - BMI < 35 and insulin is not advised or weight loss is beneficial

Question 907

MODY Types

Answer 907

Glucokinase MODY 2 - mild, rarely any complications HNF 1AE MODY 3 - progressive beta cell failure but sensitive to sulfonyureas HNF 4AE MODY 1 - neonatal hyperinsulinusim HNF 1B MODY 5 - associated with renal anomalies or cysts IPF-1 MODY 4, MODY 6-11, MODY X SUR1, Kir6.2 Hyperinsulinism in infancy and beta cell failure in adulthood

Question 908

ezetimibe used in

Answer 908

primary heterozygous familial and non familial hypercholesterolaemia - Used in patients where statins are contra-indicaed. - In combination with statin, Ezetimibe is given if serum total or LDL cholesterol is not well controlled - inhibits cholesterol receptor on enterocytes —> decreases absorption of cholesterol on small intestine

Question 909

familial hypercholesterolaemia

Answer 909

* Autosomal dominant condition * High LDL cholesterol —> early cardiovascular disease and death * Mutation in gene which encodes LDL receptor protein. Clinical Diagnosis : - Total cholesterol > 7.5 mmol / L - LDL > 4.9 mmol / L - Children TC > 6.7 mmol / L and LDL > 4 mmol / L - Definite FH : tendon xanthomas in patients or 1st or 2nd degree relatives or DNA based evidence of FH - Possible FH : family history of. MI < 50 years in 2nd degree relatives, below 60 years in 1st degree relative or family history of raised cholesterol high dose statins screen relatives

Question 910

when are fibrates used

Answer 910

particularly raised triglycerides Activating PPAR alpha receptors causing increase in LPL activity reducing triglyceride levels Adv - GI side effects, increased risk of thromboembolism

Question 911

galactosaemia

Answer 911

* Autosomal recessive * Absence of Galactose-1-phosphate Uridyl transferase enzyme * Causes increased accumulation of galactose-1-phosphate C/F : - jaundice - FTT - Hepatomegaly - Cataracts - Hypoglycaemia after exposure to Galactose - Fanconi’s syndrome urine reducing substances galactose free diet

Question 912

Hypokalaemia with alkalosis

Answer 912

1. Vomiting 2. Thiazide and loop diuretics 3. Cushing’s syndrome ( increased cortisol -> activation of mineralocorticoid receptors in distal tubules —> increased reabsorption of sodium and excretion of hydrogen & K ions —> alkalosis) 4. Conn’s syndrome - Primary hyperaldosteronism

Question 913

Hypokalaemia with acidosis

Answer 913

Diarrhoea - non anion gap metabolic acidosis - Renal tubular acidosis - Acetazolamide ( inhibits CA enzyme —> inhibits reabsorption of HCO3 in proximal tubule —> increased bicarbonate excretion in urine —> acidosis and hypokalaemia) - Partial treatment DKA

Question 914

primary hypoPTH

Answer 914

decreased secretion low Ca, high phosphate alfacalcidol CF - hypoCa symptoms

Question 915

pseudohypoPTH

Answer 915

- target cells insensitive to PTH - Due to abnormality in G protein - Associated with : low IQ, short stature, short 4th and 5th metacarpals - Dx INV : urinary cAMP and phosphate levels following infusion of PTH - Type 1 - no increase in cAMP / Phosphate - Type 2 - increase only in cAMP - Hypoparathyroidism true - increase in both cAMP and Phosphate

Question 916

glucagonoma

Answer 916

necrolytic migratory erythema - erythematous rash in the groin, extending to lower limbs, buttocks and perineum Other features of glucagonoma - NC NC Anaemia, weight loss, angular stomatitis

Question 917

MEN1

Answer 917

parathyroid (high) pituitary pancreas adrenal and thyroid

Question 918

MEN2

Answer 918

2a medullary thyroid ca parathyroid phaeochromocytoma RET oncogene 2b similar mucosal neuromas marfanoid

Question 919

Pendred's syndrome

Answer 919

* Autosomal recessive * Pendred syndrome gene - chromosome 7 * B/L sensorineural deafness, mild hypothyroidism and goitre. * Progressive hearing loss and delay in academic progression. * Defect in organification of iodine —> leading to dyshormonogenesis * MRI brain : 1.5 turns of cochlea ( normal - 2.5 turns ) * Mx : thyroid hormone replacement and cochlear implants

Question 920

primary hyperaldosteronism

Answer 920

* Adrenal adenoma = Conn’s syndrome * Most common cause - b/l idiopathic adrenal hyperplasia * C/F : hypertension, hypokalaemia, muscle weakness * 1st line investigation - plasma aldosterone/renin ratio - high aldosterone levels and low renin levels * CT abdomen and adrenal vein sampling is used to differentiate between unilateral and bilateral causes * Mx : adrenal adenoma => surgery * B/L adrenocortical hyperplasia => aldosterone antagonist Spironolactone

Question 921

primary hyperPTH

Answer 921

* Express secretion of PTH causing hypercalcemia * 85% solitary adenoma C/F : - polydipsia, polyuria - depression - Anorexia, nausea, constipation - Peptic ulceration - Pancreatitis - Bone pain/fracture - Renal stones - HTN A/W : HTN, MEN I and II. X-ray : pepper pot skull, osteitis fibrosis cystica Mx : total parathyroidectomy

Question 922

drug causes of raised prolactin

Answer 922

metoclopramide domperidone haloperidol phenothiazines SSRI opioids

Question 923

Remnant hyperlipidemia / Fredrickson type III hyperlipidemia / broad-beta disease / dysbetalipoproteinemia

Answer 923

Associated with apo-E2 homozygosity High incidence of IHD and PAD C/F : yellow palmar creases, palmar xanthomas, tuberous xanthomas Mx : fibre

Question 924

High urine osmolality > 100 mOsm / kg Urine sodium > 40 mol / L due to action of ADH on renal tubules Mx : Fluid restriction, Demeclocycline, Vasopressin R antagonists

Answer 924

SIADH

Question 925

medullary thyroid carcinoma

Answer 925

C cells derived from neural crest raised calcitonin

Question 926

papillary thyroid ca

Answer 926

- mixture of papillary and colloid filled follicles - Tumors have papillary projections and pale empty nuclei - Lymph node metastases present

Question 927

neutrophil left shift

Answer 927

Prednisolone can cause neutrophilic through * Demargination of neutrophils via the endovascular lining. * Delayed migration of neutrophils into tissue. * Release of immature neutrophils from bone marrow. During bacterial infection, there is an increased 'left shift' seen on microscopy. In 'left shift' neutrophils are produced and released from the bone marrow at an equal rate to their consumption in the circulation. With steroid use, 'left shift' generally does not occur due to increased production and release of neutrophils from the bone marrow but no neutrophil consumption.

Question 928

initial treatment of myxoedema coma

Answer 928

hydrocortisone and levothyroxine

Question 929

insulinoma

Answer 929

* Unusual symptoms such as visual disturbances, irritability, abnormal behaviour, confusion, amnesia, paraesthesiae, drowsiness * Symptoms arise after an overnight fast or before meals as a result of hypoglycaemia * Hypoglycaemic symptoms predominate - weakness, confusion, sweating, tachycardia * INV : gold standard - 72 hour supervised fasting * Blood glucose, insulin levels, C-peptide test. * Mx : surgical excision due to malignant potential of tumors Glucagon stimulation test : to assess pituitary function - GH production Sulphonyllurea assay test : helpful in conjunction with 72 hour fast in Insulinoma Calcium stimulation test : used when USG, CT and MRI often fails to localise the tumor in Insulinoma Exercise provocation test : rarely used

Question 930

alcohol and ADH

Answer 930

ADH suppression in posterior pituitary gland; picture is similar to cranial diabetes insipidus - hypernatremia, raised serum osmolality, decreased urine osmolality

Question 931

PCT

Answer 931

* Acquired deficiency in Uroporphyrinogen decardoxylase UROD * Leads to accumulation of highly carboxylate porphyrinogens in liver which are oxidised to porphyrins and enters the plasma and excreted in urine * Photosensitive porphyrins are transported to skin and causes photo damage on exposure to light * This causes derma-epidermal separation and blister formation * Excess porphyrins in urine causes urine to become reddish or dark C/F : - Blistering skin lesions on sun exposed areas of body - blisters contain serous or serosanguinous fluid - Increased susceptibility to infections - Rupturing leads to scarring with hypo- or hyperpigmentation - Hypertrichosis - cheeks and forearms - Scarring alopecia - Red urine due to porphyrins - Fragile skin Labs : A.Elevated plasma and total porphyrins levels B.LFT - mildly elevated AST and ALT, iron overload Mx : * Avoid exacerbating factors - sunlight, excess alcohol, smoking, oestrogen use * Repeated phlebotomy * Low dose Hydroxy chloroquine if ferritin > 600 ng/mL * Reflectant sunscreens containing titanium dioxide or zinc oxide * Low dose HCQ * HIV anti retro viral * Antiviral therapy for Hepatitis C

Question 932

acromegaly increased risk of ca

Answer 932

colon

Question 933

HAART lipodystrophy

Answer 933

These include a lipodystrophy-type syndrome that is characterised by the loss of peripheral and facial subcutaneous fat, but with increased abdominal and visceral fat deposition. There is also an increase in the size of the dorsocervical fat pad (buffalo hump). This fat redistribution is associated with a group of abnormalities that is usually associated with insulin resistance, such as impaired glucose tolerance, low high-density lipoprotein (HDL) cholesterol levels and high triglycerides. Glitazones can be used to treat the condition, although some small trials have proved equivocal.

Question 934

ix for retrosternal thyroid extension

Answer 934

CT scan

Question 935

hypothyroid pericarditis

Answer 935

silent pericarditis : pericardial effusion has high cholesterol content, unusual secondary pericarditis with cholesterol deposits of gold paint appearance. Pericardial effusion very rarely needs to be treated in its own right and subsides with thyroid replacement therapy

Question 936

Carney complex

Answer 936

1) spotty skin pigmentation 2) Myxoma 3) Endocrine tumors - primary pigmanet nodular adrenocortical disease (commonest), Sertoli cell tumors, GH / PRL producing tumors, pituitary adenomas, thyroid adenomas and ovarian cysts 4) Psammomatus melanotic Schwannoma

Question 937

VIPoma

Answer 937

watery diarrhoea and normal anion gap metabolic acidosis

Question 938

indications for total thyroidectomy

Answer 938

- medullary cancer > 5mm - All other cancers > 4 cm - Metastasis + - Extra - thyroid spread + - Lymph node involvement - B/L spread - Familial disease

Question 939

sex hormone binding globulin

Answer 939

reduced in insulin resistance and PCOS

Question 940

GLUT4

Answer 940

GLUT-4 : glycoprotein coded on short arm of chromosome 19. Insulin binding to GLUT-4 => tyrosine kinase activation and initiation of cascade of secondary intracellular process. GLUT-4 => channel through which glucose is taken up into muscle and adipose tissue. GLUT - 1 : non insulin stimulated glucose into cells GLUT - 2 : transports glucose into beta cells GLUT - 3 : non-insulin mediated glucose uptake into brain neurone GLUT - 5 : fructose transporter expressed on apical border of enterocytes into small intestine

Question 941

Sturge weber syndrome

Answer 941

phaeo and port wine stain

Question 942

hypokalaemic periodic paralysis

Answer 942

related to muscle voltage gated calcium channel mutation CACLN1A3 CACNA1 - calcium channel SCN4A - alpha sub unit of sodium channel

Question 943

hypercalcaemia VITAMINS TRAP

Answer 943

V - Vitamins A & D I - Immobilisation T - thyrotoxicosis A - Addison’s disease M - Milk alkali syndrome I - Inflammatory disorders N - Neoplasia S - Sarcoidosis T - Thiazides R - Rhabdomyolysis A - AIDS P - Paget’s disease, Parenteral nutrition, Parathyroidism

Question 944

saline suppression test

Answer 944

2y confirmation of hyperaldosteronism

Question 945

Primary hypertriglyceridemia (w/o hypercholesterolaemia)

Answer 945

- elevated VLDL : which is associated with even higher risk than elevated LDL elevated triglyceride levels, NORMAL cholesterol levels. Due to excess hepatic production of VLDL Heterozygous for lipoprotein lipase gene inactivating mutations Autosomal dominant disorder. Accompanied by insulin resistance, obesity, hyperglycaemia, hypertension and hyperuricaemia - Lipoprotein lipase and Apoprotein CII deficiency - elevated triglycerides - Apoprotein CII deficiency : hyperlipoproteinemia type IB / familial apolipoprotein CII deficiency - C/F : eruptive xanthomas, lipaemia retinalis, retinal vein thrombosis, pancreatitis and hepatosplenomegaly. - Chylomicrons can be detected in fasting blood sample/

Question 946

Primary mixed hyperlipidemia

Answer 946

- elevated cholesterol and Triglycerides. - Premature atherosclerosis - Remnant hyperlipidemia (= type 3 hyperlipidemia) - rare cause f mixed hyperlipidemia - Associated with Apoprotein E2

Question 947

bipolar in preg

Answer 947

stop lithium use lamotrigine/carbamazepine

Question 948

commonest cause of ESRD in UK

Answer 948

diabetes

Question 949

somatostatin receptor

Answer 949

7-transmembrane GPCR

Question 950

what drives development of 2y sexual characteristics

Answer 950

DHT also prostate/seminal

Question 951

rapidly growing thyroid mass with regional lymphadenopathy and tracheal compression

Answer 951

anaplastic thyroid carcinoma

Question 952

sarcoidosis vitamin D

Answer 952

increased hydroxylation as can occur outside the kidney mainly in macrophages in granulomas

Question 953

insulin inhibits

Answer 953

pyruvate decarboxylase upregulates G6PD, glucokinase, pyruvate dehydrogenase, glycogen synthetase

Question 954

teprotumumab

Answer 954

IGF1 inhibitor thyroid eye disease

Question 955

ovulation pattern

Answer 955

rise in LH and progesterone

Question 956

1y vs 2y ovarian failure

Answer 956

elevated FSH LH vs low

Question 957

HLA DR27

Answer 957

minimal change disease A28 - schizo

Question 958

glucagon moa in BB overdose

Answer 958

bypassing the blocked Beta blocker in beta blocker overdose and activates adenyl cycles and promotes formation of cAMP from ATP. cAMP in turn exerts a direct Beta stimulant action on heart.

Question 959

melatonin

Answer 959

synthesised and secreted by pineal gland from Serotonin - N-acetyl transferase + acetyl CoA converts serotonin to N-acetyl serotonin. - N-acetyl serotonin is converted to melatonin by hydroxyindoleO-methyltransferase - Exposure to darkness causes activation of hypothalamus by retinohypothalamic nerves - Results in increased Noradrenaline secretion by post-ganglionic sympathetic nerves (Nervi conarii) that innervate the pineal gland - Noradrenaline acts by alpha receptors in the pineal gland to increase intracellular cAMP levels which then causes an increase in serotonin N-acetyltransferase activity

Question 960

Riedel's thyroiditis

Answer 960

dense fibrosis replacing parenchyma extends beyond thyroid capsule hoarseness, stridor, dysphagia mx steroids and tamoxifen

Question 961

screening for MEN2

Answer 961

pentagastrin calcitonin calcium phaeo - 24 hr catechol

Question 962

exenatide cannot be used in

Answer 962

patients with CrCl <30 liraglutide - not in IBD semaglutide - safe

Question 963

familial hypocalciuric hyperca

Answer 963

AD CaSR gene - in parathyroid and kidney tissue

Question 964

Gordon's syndrome

Answer 964

- aka pseudo-hypoaldosteronism - that mimics hypoaldosteronism - Due to failure of response to aldosterone and levels of aldosterone are elevated due to a lack of feedback inhibition. - C/F : short stature, severe hypertension, low FeNa, normal renal function, hyperchloraemic metabolic acidosis, low renin and elevated aldosterone levels. - Hyperkalemia **

Question 965

surgery indications for 1y hyperPTH

Answer 965

hyperCa impaired renal function renal stones nephrocalcinosis reduced BMD elevated urinary Ca excretion

Question 966

deficiency of cystathione synthase

Answer 966

type 1 homocystinuria

Question 967

branched chain ketoacid dehydrogenase deficiency

Answer 967

MSUD

Question 968

if CT/MRI inconclusive in phaeo

Answer 968

MIBG scan

Question 969

hormone responsible for fusion of epiphyseal plates and cessation of growth and skeletal maturation

Answer 969

oestrogen

Question 970

increased prolactin inhibits

Answer 970

GnRH -> decreased FSH and LH

Question 971

metoclopramide binds to

Answer 971

D2 receptor on pituitary lactotrophes

Question 972

glucagon activates

Answer 972

adenyl cyclase

Question 973

sulphonylureas moa

Answer 973

mimicking role of ATP on potassium-ATP channels from the outside block -> open VGCC -> insulin release

Question 974

sipuleucel-T

Answer 974

autologous cellular immunotherapy - prostate ca

Question 975

fibrate drugs increase risk of

Answer 975

DVT

Question 976

evolocumab

Answer 976

prevents PCSK9-mediated LDL receptor degradation

Question 977

canagliflozin inc risk of

Answer 977

foot amputations

Question 978

hypothyroidism lipid

Answer 978

hyperchol rather than triglycerides

Question 979

Mullerian duct agensesis

Answer 979

Mayer-RokitanskyKuster-Hauser (MRKH) syndrome - congenital absence or underdevelopment of the uterus and upper two-thirds of the vagina in females with normal 46,XX karyotype and ovarian function. Although primary amenorrhoea would be expected in these individuals, they would typically have normal secondary sexual characteristics including pubic hair growth, which is inconsistent with the clinical presentation described in the question.

Question 980

carbenoxolone

Answer 980

glycyrrhizinic acid peptic ulcer mx hypokalaemia

Question 981

tomatoes CKD

Answer 981

hyperK

Question 982

false negative renin:aldosterone ratio results

Answer 982

ACEI ARB direct renin inhibitors - aliskiren aldosterone antagonists

Question 983

alcoholic liver disease depleted

Answer 983

glycogen storres

Question 984

achondroplasia

Answer 984

AD short stature FGFR3 - fibroblast growth factor receptor abnormal cartilage A.Short limbs (rhizomelia) and shortened fingers (brachydactyly) B.Large head with frontal bossing and narrow foramen magnum C.Midface hypoplasia with flattened nasal bridge D.Trident hands E. Lumbar lordosis

Question 985

alkaptonuria

Answer 985

* Autosomal recessive * Disorder of Phenylalanine and tyrosine metabolism due to lack of enzyme homogentisic dioxygenase * Accumulation of toxic homogentisic acid C/F : - pigmented sclera - Urine turns black when exposed to air - Intervertebral disc calcification —> back pain - Renal stones Mx : high dose vitamin C, dietary restriction of phenylalanine and tyrosine

Question 986

ADH

Answer 986

aquaporin 2 in CD supraoptic nuclei of hypothalamus -> posterior pituitary

Question 987

arachidonic acid metabolism

Answer 987

- PGI2 : vasodilation, decreased platelet aggregation, decreases uterine tone - PGE2 : increased pain, increases temperature, increase uterine tone, increases gastric mucus - PGE2 : decreased gastric acid - Thromboxane TXA2 : vasoconstriction, increases platelet aggregative - LTB4 : neutrophil chemotaxis - LTA4, LTC4, LTD4, LTE4 : increases bronchoconstriction

Question 988

ANP

Answer 988

secreted by myocytes of RA and RV (also LA but less) in response to inc blood volume 28 AA peptide - acts by cGMP Na excretion lowers BP aldosterone and AT2 antag

Question 989

otosclerosis complication inheritance

Answer 989

non-penetrance

Question 990

spontaneous mutation

Answer 990

achondroplasia

Question 991

AD vs AR quick

Answer 991

structural vs metabolic except hyperlipidaemia T2, hypokalaemic periodic paralysis (AD) Hunters, G6PD (XLR) ataxia tel and FRAX (AR)

Question 992

glioblastoma multiforme

Answer 992

A.Most common primary brain tumor in adults B.Poor prognosis C.Imaging : solid tumors with central necrosis and a rim enhances with contrast D.Causes disruption of BBB and associated with vasogenic oedema E. M/C : pleomorphic tumor cells border necrotic area F. Mx : Surgical, post op Chemo/Radio; Edema = Dexamethasone

Question 993

meningioma

Answer 993

* Second most common primary tumor in adults * Benign, extrinsic tumors of CNS * Arises from : Arachnoid cap cells of meninges and typically located next to dura * Causes symptoms by compression * M/C : spindle cells in concentric whorls, calcification of Psammoma bodies

Question 994

BL acoustic neuroma

Answer 994

NF2 Antoni A or B patterns, Verocay bodies - acellular areas surrounded by nuclear palisades

Question 995

paediatric brain tumours

Answer 995

Pilocytic astrocytoma * M/C - Rosenthal fibers - corkscrew eosinophilic bundle * Most common primary brain tumor in children Medulloblastoma * Aggressive paediatric brain tumor * Spreads through CSF system * M/C : Small, blue cells, Rosenthal pattern of cells with many mitotic figures Ependymoma - seen in 4th ventricle - Can cause hydrocephalus - M/C : Perivascular pseudo-rosettes Oligodendroma - benign slow growing tumor found in frontal lobes - M/C : Calcifications with fried egg appearance

Question 995

haemangioblastoma

Answer 995

vascular VHL foam cells

Question 996

drugs CI in breast feeding

Answer 996

I. Antibiotics - Ciprofloxacin, Tetracyclines, Chloramphenicol, Sulphonamides II. Psychiatric : Lithium, Benzodiazepines III.Aspirin IV.Carbimazole V. Methotrexate VI.Sulfonylureas VII.Cytotoxic drugs VIII.Amiodarone IX.Antipsychotics - Clozapine

Question 997

cardiac action potential

Answer 997

Phase 0 - rapid depol - Na influx Phase 1 - early repol - efflux of K and Cl Phase 2 - plateau - slow Ca influx Phase 3 - final repol - K efflux Phase 4 - restoration - slow entry of Na NaK ATPase

Question 998

LVEF

Answer 998

(SV/ED LV vol) * 100%

Question 999

CO =

Answer 999

SV * HR

Question 1000

pulse pressure

Answer 1000

SBP-DBP

Question 1001

SVR =

Answer 1001

MAP/CO

Question 1002

cell cycle

Answer 1002

G1 - inc in size determines length of cycle p53 Cyclin D/DK4/DK6/R/DK2 S - synth of DNA/RNA/histone centrosome duplication cyclin A/DK2 G2 - inc in size cyclin B/DK11 M mitosis - cell division shortest phase of cell cycle

Question 1003

phases of mitosis

Answer 1003

prometaphase - nuclear membrane breaks down allowing microtubules to attach to chromosome metaphase - chromosomes aligned in middle of cell anaphase - paired chromosome separate at kinetochores and more to opposite sides of cell telophase - chromatids arrive at opposite poles of cell cytokinesis - actin-myosin complex in centre of cell contracts resulting in it being picked into two daughter cells

Question 1004

rough endoplasmic reticulum

Answer 1004

translation and folding of new proteins manufacture of lysosomal enzymes N-linked acetylation

Question 1005

smooth ER

Answer 1005

steroid and lipid synthesis

Question 1006

Golgi apparatus

Answer 1006

modifies, sorts and packages molecules destined for cell secretion addition of mannose-6-phosphate to protein designates transport to lysosome

Question 1007

mitochondria

Answer 1007

aerobic respiratory circular DNA

Question 1008

nucleus

Answer 1008

DNA maintenance RNA transcription RNA splicing

Question 1009

lysosome

Answer 1009

breakdown of large molecules such as proteins and polysaccharides

Question 1010

nucleolus

Answer 1010

ribosome production

Question 1011

peroxisome

Answer 1011

catabolism of v long chain fatty acids and amino acids formation of hydrogen peroxide

Question 1012

proteasome

Answer 1012

degradation of protein moleculres tagged with ubiquitin

Question 1013

Patau vs Edwards syndrome

Answer 1013

trisomy 13 - polydactyly, cleft lip and palate, microcephaly Trisomy 18 - micrognathia, low set ears, rocker bottom foot, overlapping fingers

Question 1014

Prader Willi

Answer 1014

hypogonadism hypotonia obesity

Question 1015

cri du chat

Answer 1015

5p deletion larynx and neuro problems microcephaly hypertelorism

Question 1016

collagen defects

Answer 1016

T1 - OI 3 - vEDS 4 - Alports, Goodpastures 5 - EDS

Question 1017

complement deficiencies

Answer 1017

* C1 - INH deficiency : CI inhibitor deficiency - hereditary angioedema * C1q, C1rs, C2, C4 deficiency : classical pathway components, predisposes to immune complex disease - SLE, Henoch Schonlein purpura * C3 deficiency : recurrent bacterial infection * C5 deficiency : Leiner disease - recurrent diarrhoea, wasting and seborrhoeic dermatitis * C5 - C9 deficiency : enodes the membrane attack complex (MAC) ; prone to Neiserria meningitides infection

Question 1018

congenital infections

Answer 1018

rubella - sensorineural deafness, cataracts, PDA, HSmeg, purpuric lesions, salt and pepper chorioretinitis, micrcophthalmia, CP toxo - calcification, chorioretinitis, hydrocephalus, hsmeg, CP, anaemia CMV - lbw, purpuric lesions, all issues + pneumonitis and jaundice

Question 1019

cytokines

Answer 1019

IL-1 Macrophages Acute inflammation, induces fever IL-2 Th1 cells Stimulates growth and differentiation of T cell response IL-3 Activated Th1 cells Stimulates differentiation and proliferation of myeloid progenitor cells IL-4 Th2 cells Stimulates proliferation and differentiation of B cells IL-5 Th2 cells Stimulates production of eosinophils IL-6 Macrophages, Th2 cells Stimulates differentiation of B cells, induces fever IL-8 Macrohages Neutrophil chemotaxis IL-10 Th2 cells Inhibits APCs and Th1 cytokine production AKA human cytokine synthesis inhibitory factor Anti inflammatory cytokine IL-12 Dendritic cells, macrophages, B cells Activated NK cells and stimulates differentiation of naive T cells into Th1 cells TNF-alpha Macrophages Induces fever, neutrophil chemotaxis Interferon-gamma Th1 cells Activates macrophages

Question 1020

DiGeorge syndrome

Answer 1020

T-cell microdel 22q11.2 AD CATCH 22 C - Cardiac abnormalities (endocardial cushion defect most common ie - AV septal defect) A - Abnormal facies T - thyme aplasia C - Cleft palate H - hypocalcemia, hypoparathyroidism 22 - Chromosome 22 deletion single palmar crease, pronounced sandal gap, hypotonia, heart defects, duodenal atresia

Question 1021

endothelin

Answer 1021

- potent, long acting vasoconstrictor and broncho-constrictor - Prohormone converted to endothelin-1 by enzyme Endothelin converting enzyme - Acts via G-protein liked to phospholipase C leading to calcium release promotes - AT2, ADH, hypoxia, shearing inhibits - NO, prostacyc

Question 1022

erythropoeisis

Answer 1022

* Red bone marrow found in flat bones - vertebrate, sternum, ribs, proximal ends of long bones * Fonts - erythrocytes produced in liver * RBC lifespan - 120 days * Levels of erythrocytes = hematocit

Question 1023

Fabry disease

Answer 1023

XLR alpha galactosidase A burning pain/parasthesia in childhood angiokeratomas lens opacities proteinuria CVD early

Question 1024

Fragile X is a

Answer 1024

TNR disorder analysis of number of CGG repeats using restriction endonuclease digestion and Souther blot analysis

Question 1025

HIV immune cell reactions

Answer 1025

- Reduction in CD4 count * Increase in beta - 2 micro globulin * Decreased IL-2 production * Polyclonal B cell activation * Decrease in NK cell function * Reduced delayed hypersensitivity responses

Question 1026

type 1 hypersensitivity

Answer 1026

Antigen reacts with IgE bound to mast cells Anaphylaxis Atopy - asthma, eczema, hay fever

Question 1027

type 2 hypersensitivity

Answer 1027

cell bound IgG or IgM binds to antigen on cell surface Autoimmune haemolytic anaemia Goodpasture’s syndrome ITP Pernicious anaemia Acute haemolytic transfusion reaction Rheumatic fever Pemphigus vulgairs Bullus pemphigoid

Question 1028

Type 3 hypersensitivity

Answer 1028

Free antigen and antibody (IgG, IgA) combine Serum sickness SLE Post - strept GN Extrinsic allergic alveolitis

Question 1029

Type 4 hypersensitivity

Answer 1029

T - cell mediated Tuberculosis/ Tuberculin skin reaction Graft v/s host disease Scabies Allergic contact dermatitis Multiple sclerosis EAA - chronic phase GBS

Question 1030

Type 5 hypersensitivity

Answer 1030

Antibodies that recognise and bind to cell surface receptors - either stimulates them or blocks ligand binding Myasthenia gravis Graves’ disease

Question 1031

IL1 actions

Answer 1031

* Acts as a co-stimulator of T cell and B cell proliferation. * Increases expression of adhesion molecules on the endothelium. * Stimulates the release of endothelium of vasoactive factors such as PAF, NO and prostacyclin. * Causes vasodilation and increases vascular permeability. * One of the mediators of shock in sepsis * IL-1, IL-6, TNF -> acts on hypothalamus and causes pyrexia. * Anakinra - IL-1 receptor antagonist; management of rheumatoid arthritis * Canakinumab : monoclonal antibody targeted at IL-1 beta; used in systemic JIA and adult onset Still’s disease

Question 1032

innate immunity

Answer 1032

neutrophils - primary phagocytic cell in acute inflammation - granules contain myeloperoxidase and lysozyme - multi lobed nucleus eosinophil - protozoan and helminth - bi lobed nucleus monocytes - differentiate into macrophages - kidney shaped nucleus mast cells - present in tissue - release histamine, contain heparin - expressed IgE receptors on cell surface macrophages - phagocytosis of debris and pathodens - APC

Question 1033

NK cells

Answer 1033

induces apoptosis in infected and tumour cells

Question 1034

adaptive immunity

Answer 1034

helper T cells * Involved in cell mediated immune response * Recognises antigen presented by class II MHC molecule * Expressed CD4, CD3, TCR, CD28 * Major source of IL-2 * Mediates acute and chronic graft rejection cytotoxic T cells * Recognises antigens presented by MHC Class I molecules * Expressed CD8, TCR, CD3 * Mediates acute and chronic organ rejection B cells Major cell of humeral immune response * Acts as APC * Mediates hyper acute rejection * Differentiated from plasma cells * Produces large amout of antibody specific to particular antigen

Question 1035

most abundant antibody

Answer 1035

IgG

Question 1036

most commonly produced Ab

Answer 1036

IgA

Question 1037

least abundant Ab

Answer 1037

IgE

Question 1038

breast milk ab

Answer 1038

IgA protection on mucosal membranes, transported via transcytosis

Question 1039

ab activating B cells

Answer 1039

IgD

Question 1040

ab synthesised by plasma cells

Answer 1040

IgE

Question 1041

glycogen storage disorders

Answer 1041

Von Gierke’s disease - Type 1 Glucose 6 phosphatase Hepatic glycogen accumulation C/F : hypoglycaemia, lactic acidosis, hepatomegaly Pompe’s disease - type 2 Lysosomal alpha 1,4 glucosidase Cardiac, hepatic and muscle glycogen accumulation, cardiomegaly Cori disease - type 3 Alpha - 1,6 - glucosidase debranching enzyme Hepatic, cardiac glycogen accumulation. Muscle hypotonia McArdle’s disease Glycogen phosphorylase (myophosphorylase) Skeletal muscle glycogen accumulation. C/F : myalgia, myoglobinuria with exercise

Question 1042

lysosomal storage disorders

Answer 1042

Gaucher’s disease Beta glucocerebrosidase Most common; accumulation of glucocerebrocidase in brain, liver, spleen; C/F : hepatosplenomegaly, aseptic necrosis of femur Increased levels of Acid phosphatase Miglustat - recombinant glucosidase and glucosylceramide inhibitors Tay-Sachs disease Hexosaminidase A Accumulation of GM2 ganglioside within lysosomes. C/F : developmental delay, cherry red spot on macular, normal size liver and spleen Niemann - Pick disease Spnhingomyelinase Hepatosplenomegaly Cherry red spot on macula Fabry disease Alpha-galactosidase A Accumulation of ceramide trihexoside C/F : angiokeratomas, peripheral neuropathy, renal failure Krabbe’s disease Galactocerebrosidase Peripheral neuropathy, optic neuropathy, globoid cells Metachromatic leukodystrophy Arylsulfatase A Demyelination of CNS & PNS

Question 1043

mucopolysaccharide syndromes

Answer 1043

Hurler syndrome - type 1 Alpha - 1 - Iduronidase Accumulation of GAG (heparin and derma tan sulphate) C/F : Gargoylism (large head and grotesque facial appearance), Hepatosplenomeglay, YES corneal clouding Hurler Syndrome - type 2 Iduronate sulfatase Accumulation of GAGs; C/F : coarse facial features, behavioural problems, learning difficulties, short stature, NO corneal clouding

Question 1044

femoral nerve damage

Answer 1044

loss of knee extension and sensory loss to anterior and medial aspect of thigh following stab injury

Question 1045

obturator nerve injury

Answer 1045

damaged nerve anterior hip dislocation - unable to adduct thighs

Question 1046

Lateral cutaneous nerve of thigh : compression at ASIS —> meralgia paraesthetica

Answer 1046

pain, tingling, numbness in distribution of lateral cutaneous nerve —> lateral and posterior surfaces of thigh

Question 1047

tibial nerve issues

Answer 1047

foot plantar flexion and inversion - loss of sensation over sole of foot. Injured in popliteal laceration, posterior knee dislocation

Question 1048

common peroneal nerve

Answer 1048

loss of foot dorsiflexion and eversion, cannot use extensor hallucis longus; injury at neck of fibula, tightly applied plaster cast - foot drop

Question 1049

superior gluteal nerve

Answer 1049

positive Trendelenburg sign : misplaced IM injection, Pelvic #, posterior hip dislocation - cannot abduct hips

Question 1050

inferior gluteal nerve

Answer 1050

hip extension and lateral rotation - injury causes difficulty in getting up from sitting position, cant jump / climb stairs

Question 1051

Mc-cune albright

Answer 1051

random somatic mutation in GNAS gene Precocious puberty, cafe au lait spots, polyostotic fibrotic dysplasia, short stature Polyostotic fibrous dysplasia = bone replaced by fibrous tissue - weak bones, deformity, uneven growth

Question 1052

ligand gates ion channels

Answer 1052

* Mediates fast response * Nicotinic acetylcholine * GABA-A, GABA-C * Glutamate

Question 1053

tyrosine kinase receptors

Answer 1053

* Insulin * Insulin-like growth factor -1 IGF-1 * Epidermal growth factor EGF non-receptor: * Prolactin * Immunomodulators - IL-2, IL-6, IFN * Growth Hormone * G-CSF * Erythropoietin * Thrombopoietin

Question 1054

granulate cyclase receptors

Answer 1054

* Contains intrinsic enzyme activity * ANP, BNP

Question 1055

GPCR

Answer 1055

* Slow transmission, affects metabolic processes * Activated by wide variety of signals * 3 main sub-units - alpha, beta, gamma * G proteins are named according to the alpha subunit Gs : * Stimulates adenylcyclase —> increases cAMP —> activates protein kinase A * Beta 1 receptors - NA, Adrenaline, Dobutamine * Beta 2 receptors : Epinephrine, Salbuterol * H2 receptors - histamine * D1 receptos - Dopamine * V2 receptors - Vasopressin * Receptors for ACTH, LH, FSH, PTH, Glucagon, Calcitonin, Prostaglandins Gi : * Inhibits adenyl cyclase —> decreased cAMP —> inhibits protein kinase A * M2 receptors - Acetylcholine * Alpha 2 receptors - epinephrine, norepinephrine * D2 receptors - Dopamine * GABA-B receptors Gq: * Activates phospholipase C —> splits PIP2 to IP3 + DAG —> activates protein kinase C * Alpha 1 receptors : Epinephrine, norepinephrine * H1 receptor - histamine * V1 receptors - vasopressin * M1, M3 receptors - acetylcholine

Question 1056

microtubule function

Answer 1056

* Helps guide movement during intracellular transport and also binds internal organelles. * Found in all cells except blood cells. * Cylindrical structure - composed of alternating alpha and beta subunits which polymerises to form protofilaments * Microtubules are polymerised - have a negative or positive charge * Attachment protein = dyenin and kinesin : helps moves the microtubules facilitating the movement of various cell organelles. * Dyenin - retrograde fashion - down the microtubule - towards the centre +ve —> -ve * Kinesin : anterograde fashion - up the microtubule way from the centre : -ve —> +ve

Question 1057

molecular biology

Answer 1057

SNOWDROP Southern blot - detects DNA Northern blot - detects RNA Western blot - detect proteins; uses gel electrophoresis to separate native proteins by 3D structure; EG - Confirmatory HIV test ELISA - antigens and antibodies, initial HIV test

Question 1058

lack of NO

Answer 1058

hypertrophic pyloric stenosis atherosclerosis sepsis - increased NO causes shock

Question 1059

Noonans coagulation issues

Answer 1059

factor XI deficiency

Question 1060

Noonans heart

Answer 1060

pulmonary stenosis

Question 1061

oncogenes

Answer 1061

ABL - CML n-MYC - neuroblastoma BCL-2 - follicular lymphoma c-MYC - burkitt's lymphoma RET - MEN 2a and 2b RAS - pancreatic ERB-B2/HER-NEU - breast/ovarian

Question 1062

oncoviruses

Answer 1062

EBV -> Burkitts, HL, post transplant lymphoma, NPC Hep b and c - HCC HTLV-1 -> adult T cell leukaemia, tropical spastic paraparesis

Question 1063

p53 is a

Answer 1063

TSG breast colon lung ca prevents entry into S phase until DNA checked and repaired Li Fraumeni due to mutation in this

Question 1064

primary hyperPTH

Answer 1064

* PTH - elevated * Calcium - elevated * Phosphate - Low * Urine calcium : creatinine clearance ration > 0.01 * Recurrent abdominal pain - pancreatitis, renal colic * Changes to emotional or cognitive state Causes : 80% due to solitary adenoma

Question 1065

Secondary hyperPTH

Answer 1065

* PTH - elevated * Calcium - low or normal * Phosphate - elevated * Vitamin D levels - low * May have few symptoms * Complications : bone disease, osteitis fibrosa cystica, soft tissue calcifications * Parathyroid gland hyperplasia due to low calcium levels * Chronic renal failure Secondary hyperparathyroidism Usually managed with medical therapy. Indications for surgery in secondary (renal) hyperparathyroidism: * Bone pain * Persistent pruritus * Soft tissue calcifications

Question 1066

Tertiary hyperPTH

Answer 1066

* Calcium - normal or high * PTH - elevated * Phosphate - decreased * Vitamin D - normal or decreased * ALP - elevated * Due to - metastatic calcification * Nephrolithiasis * Pancreatitis * Hyperplasia of all 4 glands causes this Tertiary hyperparathyroidism Allow 12 months to elapse following transplant as many cases will resolve The presence of an autonomously functioning parathyroid gland may require surgery. If the culprit gland can be identified then it should be excised. Otherwise total parathyroidectomy and reimplantation of part of the gland may be required.

Question 1067

PKUria

Answer 1067

AR phenylalanine hydroxylase defect - converts to tyrosine learning difficulties and seizures chr 12 C/F : * Developmental delay * Fair hair and blue eyes * Learning difficulties * Seizures - infantile spasms * Eczema * Musty odour due to urine and sweat (sweat = secondary to phenyl acetate, which is a phenylketone) Labs : - hyper-phenylalaninaemia - Phenylpyruvic acid in urine

Question 1068

reverse transcriptase is

Answer 1068

DNA polymerase enzyme : involved in transcribing ss RNA into lengths of ds DNA

Question 1069

xanthine oxidase

Answer 1069

catalyses the oxidation of hypoxanthine to xanthine and also further catalyse the oxidation of xanthine to uric acid. Xanthine oxidase inhibitors - treatment option in gout. Cause of gouty tophi - firm nodules over the extensor surfaces of fingers, hands, forearms, ears.

Question 1070

Klumpke's paralysis

Answer 1070

- C8, T1 nerve roots - weakness of intrinsic muscles of hand, long extensors of fingers Supplies motor supply to all intrinsic hand muscles (except LOAF) LOAF - Lateral 2 lumbricals, Opponens pollis, Abductor pollis brevis, Flexor pollis brevis LOAF muscles supplied by median nerve C6, C8, T1

Question 1071

gastric adenocarcinoma

Answer 1071

signet ring cels >> mutinous >> papillary >> tubular in descending order of prognosis

Question 1072

where are G proteins located

Answer 1072

in the cytoplasm coupled with GPCR on cell membrane ligand binding site expressed outside the surface through the plasma membrane TSH, ACTH, GABA-B

Question 1073

Lys18 deletion mutation form

Answer 1073

HOCM trop I - high risk of SCD if trop T - milder

Question 1074

nerve damage during parotidectomy

Answer 1074

tendency of food and fluids to collect in buccal sulcus after meals due to paralysis of buccinator muscle Action of buccinator muscle : empty the buccal sulcus during mastication. Peripheral injuries of facial nerve - no loss of taste sensation as the chord tympanii fibers pass from the lingual nerve to facial nerve just below the skull and remains intact during peripheral injuries of facial nerve

Question 1075

intraglomerular thrombi of immunoglobulins

Answer 1075

malignant lymphoma deposition of amorphous material in glomerular capillary lumens

Question 1076

lung surface markers

Answer 1076

* Anterior border of left lung - moves laterally to level of fourth costal cartilage to form the cardiac notch , before moving down to 6th costal cartilage. * Inferiorly - lung reaches upto the 8th rib at mid clavicular levels - 10th rib at mid axillary level and 12th rib posteriorly at level of scapula line * Oblique fissure - level of T12 vertebra on posterior side to 6th costal cartilage on the front

Question 1077

pores of kohn

Answer 1077

intra-alveolar communication

Question 1078

bronchial veins

Answer 1078

drain into azygous on R and hemi on L

Question 1079

lymphatic drainage of lung

Answer 1079

BP LNs at hilum

Question 1080

phase 0 of cardiac potential channels

Answer 1080

L type Ca channels

Question 1081

Class 1 anti-arrhythmic

Answer 1081

Na channel blockers - effect on phase 0 of potential

Question 1082

class 3 antiarrhythmics

Answer 1082

amiodarone sotalol phase 3 - K channel blockers

Question 1083

class 4

Answer 1083

verapamil phase 2 of potential

Question 1084

CN3 nuclei location

Answer 1084

midbrain at level of superior colliculus

Question 1085

surfactant composition

Answer 1085

phospholipids such as DPPC, cholesterol and apolipoproteins water least present

Question 1086

where is ventilation controlled

Answer 1086

resp centre in medulla peripheral and central chemoreceptors DKA inc resp drive 2y to metabolic acidosis control of normal vent - central carotid chemoreceptors controls 2/3rd of response

Question 1087

3 elements of nucleotide which combine to form repeating unit making up DNA

Answer 1087

phosphate group sugar group nitrogenous base

Question 1088

RNA splicing

Answer 1088

by enzymes spliceosomes - composed of 5 small nuclear RNAs and associated proteins - leads to removal of introns and joining together of exons

Question 1089

ribosomes

Answer 1089

translate mRNA into proteins using tRNA

Question 1090

Golgi apparatus

Answer 1090

protein transport

Question 1091

hypoxia effect on VQ

Answer 1091

vasoconstriction in pulmonary vessels - improves ratio acute response due to NO

Question 1092

biceps femoris nerve supply

Answer 1092

common fibular branch of sciatic nerve

Question 1093

peroneus muscle

Answer 1093

superficial peroneal nerve

Question 1094

Absent knee reflex, loss of sensation over anterior and medial thigh

Answer 1094

femoral nerve damage

Question 1095

first biochemical change in hypothyroidism

Answer 1095

increase in serum TSH

Question 1096

ANP

Answer 1096

28 aa peptide released in response to atrial stretch dilates the AFFERENT, constricts the EFFERENT, increases the eGFR

Question 1097

acute attacks of AIP

Answer 1097

IV haematin/haem alginate suppresses the hepatic synthesis 4 day course

Question 1098

bleomycin

Answer 1098

degrades preformed DNA single DNA strand scission

Question 1099

leptin increase

Answer 1099

release of MSH, CRH, GnRH stimulation production of cortical bone, decreases cancellous bone formation

Question 1100

amylin

Answer 1100

slows gastric emptying

Question 1101

onset of puberty other hormons

Answer 1101

leptin kisseptin

Question 1102

stellate cells of liver

Answer 1102

role in fibrosis have fat droplets

Question 1103

activation of Ito cells

Answer 1103

important mechanism for control of sinusoidal perfusion

Question 1104

bile duct epithelial cells

Answer 1104

pathology of PBC and PSC

Question 1105

Kupffer cells

Answer 1105

produces mediators which stimulate stellate cells to drive fibrosis

Question 1106

normal lung volumes

Answer 1106

Peak expiratory flow - 520-700 L / min Total lung capacity - 5-6.5 L Functional residual capacity - 2-3 L Tidal Volume - 500-700 mL Inspiraotry Reserve Volume - 3.3 L

Question 1107

genetics

Answer 1107

* Human genome project- 20,000 genes * Exons = coding sequences of DNA which code for peptides produced from mRNA * Introns = exons have spaces of DNA between them called introns; these code for switches that control gene expression in splicing * Human genome have a higher number of segmental duplications vs other mammals

Question 1108

PPOX porphyria gene

Answer 1108

AD chr 1

Question 1109

CFTR gene

Answer 1109

chromosome 7

Question 1110

chromosome 11

Answer 1110

BWS Ewings

Question 1111

Shock classification

Answer 1111

1 - 15% 750mL 2 - 15-50% 750-1000mL tachycardia 3 - 30-40% 1500-2000mL hypotension tachycardia fall in UO 4 - >40% >2000mL anuria and severe shock

Question 1112

thumb abduction

Answer 1112

APB median nerve

Question 1113

wrist drop

Answer 1113

radial extensors radial nerve

Question 1114

inability to abduct and adduct fingers flex little finger

Answer 1114

ulnar nerve

Question 1115

hoarse voice, paralysis of posterior cricoarytenoid

Answer 1115

recurrent laryngeal

Question 1116

interarytenoid muscle

Answer 1116

L and R recurrent laryngeal

Question 1117

renin secretion by

Answer 1117

juxtaglomerular cells reduced by - BB, NSAIDs

Question 1118

aldosterone released in response to

Answer 1118

raised AT2, hyperK, raised ACTH retention of Na in exchange for K/H in distal tubule

Question 1119

neurons in resting state

Answer 1119

+ve external -ve internal At rest - neuron cell membranes are less permeable to Na+ than to K+. Inside of neuron is rich in chloride ions and contains lesser amount of potassium ions. Outside of neurone = rich in sodium ions and positive on outside.

Question 1120

Parkinson's increased protein aggregation

Answer 1120

due to proteasome dysfunction bortezomib - proteasome inhibitor

Question 1121

most accurate methods for testing GFR

Answer 1121

inulin clearance chromium labelled EDTA lohexol

Question 1122

nephron

Answer 1122

PCT - 50% of filtered sodium reabsorbed within the prox tubule Na/K ATPase pump Na/H antiporter - 90% of bicarb and NaCl reabsorption All filtered glucose and amino acids reabsorbed in PCT, damage -> albuminuria and proteinuria, hypokalaemia LOH - thick ascending limb impermeable to water 40% of sodium vua Na/K/2Cl pump Distal - 5% sodium Na/Cl transporter H+ ions secreted thiazide diuretics CD - Na channels spironolactone H+ secreted acidifying urine

Question 1123

BRCA1

Answer 1123

repair of double stranded breaks in DNA if mutated, breaks accumulate

Question 1124

DNA polymerase mutation

Answer 1124

DNA replication colorectal cancers

Question 1125

RB gene

Answer 1125

freezing of cell cycle associated with impaired checkpoint regulation between G1 and S phase

Question 1126

bradykinin

Answer 1126

potent vasoactive peptide

Question 1127

Hermansky-Pudlak syndrome

Answer 1127

mutation of AP3 gene, responsible for trafficking specific glycoproteins from the Golgi body AR

Question 1128

normal breathing

Answer 1128

diaphragm drops down by 1 cm, creating an intro thoracic pressure drop of 1-3 mmHg, and air intake of 500 mL Accessory muscles of respiration relax during passive expiration. Diaphragm drops by 10 cm - during exercise Rib cage recoils during passive expiration.

Question 1129

cervical rib

Answer 1129

selective weakness of APB C8/T1 nerve roots

Question 1130

liver supply

Answer 1130

75% portal vein 25% hepatic artery

Question 1131

intrinsic factor

Answer 1131

parietal cells in fundus cardia -> mucus

Question 1132

Paget's disease can have low ALP if

Answer 1132

low Mg in highly active disease

Question 1133

Organ of Corti

Answer 1133

cochlear duct contains potassium rich endolymph produces nerve impulses in response to sound vibrations

Question 1134

high vs low frequency sound

Answer 1134

scala vestibuli - base - high scala tympani - apex - low contain perilymph normal 20-2000Hz

Question 1135

diffusion

Answer 1135

fat soluble molecules - e.g. Glycerol - diffused through semi permeable cell membrane. - they dissolve in the phospholipid bilayer and pass through in direct of concentration gradient. - Endocytosis : active process where particles are captured within the cell membrane and this can be receptor or non receptor mediated. - Exocytosis : export of particles out of the cell via secretory vesicles. - Osmosis : diffusion of water across a membrane in which water diffuses into a solution having a greater solute concentration - Phagocytosis : active process by which bacteria or other antigenic material is engulfed by macrophages.

Question 1136

regional variations in lung

Answer 1136

greater vol of blood to base alveoli at base compressed - smaller but greater compliance apex - less blood flow and ventilation V/Q ratio at apex is greatest, increased PaO2 and decreased PCO2

Question 1137

congenital long QT

Answer 1137

AD K+ channels BB 2nd line - ICD, stellate gangliotomy ICD first line if cardiac arrest

Question 1138

atrial myxoma and freckles

Answer 1138

Carney complex = atrial myxoma + freckles - high cortisol levels independent of ACTH - Testicular, thyroid, pancreatic carcinomas are common - Mutation in PRKAR1A gene on chromosome 17q23-q24

Question 1139

ethylene glycol electrolyte imbalance

Answer 1139

hypoCa end product of ethylene glycol is oxalic acid combines with Ca to form Ca oxalate -> acidosis and kidney disease

Question 1140

dextrose 50% - 1700kcal

Answer 1140

40% - 1360 60% - 2040

Question 1141

dextrose 50% and lipid emulsion 20%

Answer 1141

2000kcal

Question 1142

ulnar nerve injury at the elbow

Answer 1142

Ulnar nerve injury at the elbow - inability to grip a sheet of paper between his fingers when the hand is placed flat on the table - test for paralysis of the Palmar interossei muscles - inability to adduct the fingers. Loss of sweating over the ulnar border of left hand due to damage to sympathetic interruption 4th and 5th digits are held in clawed position if ulnar nerve lesion is at the wrist. Elbow lesions of ulnar nerve leads to paralysis of long flexors only - NO CLAWING IN ULNAR ELBOW LESION.

Question 1143

precursor to cortisol

Answer 1143

11-deoxycortisol

Question 1144

precursor to corticosterone

Answer 1144

deoxycorticosterone

Question 1145

precursor to aldosterone

Answer 1145

corticosterone

Question 1146

Dubin Johnson test

Answer 1146

coproporphyrin excretion pattern

Question 1147

inhalation of fluids -> disordered gas exchange

Answer 1147

respiratory acidosis metabolic acidosis - due to volume depletion, hypotension and tissue hypoxia therefore mixed acidosis

Question 1148

DNA Proofreading

Answer 1148

DNA polymerase travels along a double stranded piece of DNA - when a mismatched pair is recognised, incorrect base is excised - Recombination - DNA recombination brings together DNA from multiple source - Replication : DNA replications is the process of producing two identical copies of DNA from one template molecule - Retro-transposition : transposition via a RNA intermediate - Transposition : movement of genetic element from one site to another in a DNA molecule - Splicing : removal of introns from the primary transcript of a discontinuous gene

Question 1149

ciliary dyenin

Answer 1149

converts chemical energy from ATP into movement -> ciliary beating 100mL mucus bronchioles no cartilage in walls diameter 1mm mucociliary escalator moves at 2cm/min

Question 1150

EEG waves

Answer 1150

* alpha waves : 8-13 Hz * Delta waves : < 4 Hz * Theta waves : 4-7 Hz * Beta waves : >14 Hz

Question 1151

hyperphagia nucleus

Answer 1151

ventromedial

Question 1152

control of BP and HR nucleus

Answer 1152

dorsomedial

Question 1153

BP reg, shivering, vasopressin nucleus

Answer 1153

posterior

Question 1154

factors maintaining pressures within the arterial system

Answer 1154

1) peripheral arterial elasticity 2) Ventricular systole 3) Recoil of elastic aorta

Question 1155

monoclonal antibodies target

Answer 1155

plasma cells

Question 1156

targets for monoclonal therapy in asthma

Answer 1156

1) IgE - in allergic phenotype 2) To target eosinophil maturation via IL-4/IL-5/IL-13 - in the eosinophilic phenotype - NICE recommends adding monoclonal IgE antibody therapy after 4 or more trials of oral corticosteroids have been completed in the previous year with optimised standard therapy - Example - Omalizumab - severe eosinophilic asthma - Il-5 antibody therapy - Mepolizumab / Reslizumab / Benralizumab - Il-4/13 antibody therapy - Dupilumab

Question 1157

cytokines involved in fever/sepsis

Answer 1157

IL1 -> vasodilation and hypotension IL6 TNFa

Question 1158

FiSH

Answer 1158

fluorescent DNA or RNA probe to bind to specific gene interest for direct visualisation of chromosomal abnormalities

Question 1159

Eastern blotting

Answer 1159

post translational modifications of proteins such as addition of lipids and phosphates

Question 1160

renal biopsy findings

Answer 1160

diabetic nephropathy - Kimmelsteil Wilson nodules - nodular glomerulosclerosis, basement membrane thickening, capillary obliteration, mesangial widening apple green birefringence - amyloid enlarged and hypercellular glomeruli - PSGN crescent moon shaped glomeruli - RPGN wire looping of capillaries - DPGN (SLE)

Question 1161

vitamin C - scurvy

Answer 1161

impaired collagen synthesis and disordered connective tissue * Follicular hyperkeratosis * Perifollicular haemorrhage * Ecchymosis * Easy bruising * Poor would healing * Gingivitis with bleeding and receding gums * Arthralgia * Edema * Weakness, malaise, anorexia, depression

Question 1162

Lesch-Nyhan syndrome

Answer 1162

XLR juvenile gout! defect in purine salvage pathway due to absence of HGPRT enzyme conversion of hypoxanthine to IMP conversion of guanine to GMP hyperuricaemia aggression, self-mutilation, intellectual impairment

Question 1163

XLD disorders

Answer 1163

Fragile X hypophosphataemic rickets

Question 1164

early response in asthma

Answer 1164

mast cell degranulation in response to IgE already produced by B cells - 10-20 min peak

Question 1165

late response in asthma

Answer 1165

eosinophils recruited by IL13 produced by lymphocytes 3-5 hours attraction of phagocytes

Question 1166

pemphigus vulgaris

Answer 1166

antibodies against desmoglein 3 - cadherin type epithelial adhesion molecule

Question 1167

claudin disorder

Answer 1167

tight junctions neonatal sclerosing cholangitis

Question 1168

connexins

Answer 1168

protein channels between two adjacent cells to allow passage of solutes up to 1000 kDa oculodendrodigital dysplasia

Question 1169

salicylate and theophyllines cause

Answer 1169

respiratory alkalosis TCA -> resp depression, coma, fits

Question 1170

hypophosphataemia worsens

Answer 1170

cardiac failure by decreasing LV contractility

Question 1171

neuralgic amyotrophy

Answer 1171

= Brachial neuritis = Brachial plezitis * Autoimmune inflammatory aetiology * Often occurs after infection, vaccination, traumatic injury, surgery, childbirth * Intense pain around shoulder and rapid patchy weakness of the affected limb * ** winging of scapula (long thoracic nerve C5-C7 * Loss of elbow flexion - Musculocutaneous nerve C5-C7 * CSF : mild raised protein, mild lymphocytosis.

Question 1172

methods to predict 3D structure of protein

Answer 1172

1) X-ray crystallography - cannot give information about how the conformation of protein may change over time. 2) Nuclear magnetic resonance spectroscopy 3) Cryogenic electron microscopy Information about conformational changes of protein : 1) Dual position inferometry; 2) nuclear magnetic resonance

Question 1173

encapsulation in stem cell derived islet cell transplants

Answer 1173

prevent autoimmune attachment by introducing barrier (alginate) to prevent access by cells of immune system mainly T lymphocytes

Question 1174

increased levels of plasminogen activator inhibitor-1

Answer 1174

insulin resistance

Question 1175

riboflavin is involved in

Answer 1175

* Involved in redox process involving the hydrogen transfer chain in mitochondria * Production of ATP

Question 1176

constipation in enteral feeding due to

Answer 1176

inadequate fluid replacement diarrhoea more common - - hyperosmolar feeds - Bacterial contamination - Low feed temperature - Reduced intestinal absorptive capacity - Too rapid or irregular administration - Lactose intolerance

Question 1177

disorders of anterior mediastinum

Answer 1177

thymus teratoma terrible lymphoma thyroid

Question 1178

supply of skin over first web space and EPB/EPL

Answer 1178

radial nerve via posterior interosseous branch and superficial branch

Question 1179

endothelin actions

Answer 1179

constricts afferent arteriole vasoconstriction stimulates RAAS release of ANP ET-A - vasoconstriction ET-B - vasodilation due to NO production

Question 1180

ulnar nerve roots

Answer 1180

C8/T1 all intrinsic except LOAF (median) wrist flexion FCU/FDP ulnar head hypothenar eminence damaged in medial epicondyle fracture and claw hand Jeanne sign - positive test in ulnar nerve damage due to hyperextension of 1st MCP Front's thumb sign - APB weak card test egawa test - palm faced flat

Question 1181

median nerve roots

Answer 1181

C6. C8, T1 damage at wrist - CTS elbow - loss of power in forearm flexors + weak wrist flexion -> hand of benediction, ape hand

Question 1182

T2DM triglycerides pattern

Answer 1182

normal LDL low HDL elevated triglycerides

Question 1183

insulin receptor type

Answer 1183

tyrosine kinase on cell membrane involved in intracellular signalling by phosphorylation of intra cellular proteins IRS-1 upregulates GLUT4 Donohue syndrome - leprechaunism = AR, non functioning insulin receptor

Question 1184

thiazide diuretics imbalance

Answer 1184

hypokalaemic alkalosis

Question 1185

Gaucher disease enzymes elevated

Answer 1185

acid phosphatase ACE

Question 1186

restrictive lung disease lung function tests

Answer 1186

- diffusion capacity is reduced - FEV 1 / FVC - normal or increased - TLC - reduced

Question 1187

obstructive airway test results

Answer 1187

- FEV 1 / FVC : reduced - TLC - normal or increased

Question 1188

what electrolyte lost a lot through ileostomy

Answer 1188

Mg -> impairs PTH secretion -> hypocalcaemia resistant to giving Ca

Question 1189

azygous lobe of lung

Answer 1189

reverse comma sign

Question 1190

mainstay of locating mutated oncogenes

Answer 1190

PCR

Question 1191

bile salt production is catalysed by

Answer 1191

cholesterol-7-alpha-hydroxylase Total bile salt pool - 2.5-5 grams Total fecal loss of bile salts = 20% Bile acids concentration in gall bladder = 100-300 mmol / L Bile salt reabsorption - 80% over 6-8 cycles

Question 1192

adrenaline is secreted by

Answer 1192

adrenal medulla

Question 1193

noradrenaline is secreted by

Answer 1193

adrenal medulla nerve terminals of the sympathetic NS

Question 1194

ubiquitination

Answer 1194

degraded by the proteasome

Question 1195

rough vs smooth ER

Answer 1195

* Rough ER : translation and folding of new proteins * Manufacture of lysosomal enzymes * Site of N-linked glycosylation * E.G of cells with extensive rough ER = pancreatic cells, goblet cells, plasma cells. Smooth ER : * Steroids and lipid synthesis * E.g. : extensive smooth ER = adrenal cortex, hepatocytes, testes, ovaries.

Question 1196

classic hereditary haemochromatosis is characterised by

Answer 1196

incomplete penetrance and variable expressivity women less affected due to menstruation

Question 1197

ampho B is a recognised cause of

Answer 1197

Fanconi syndrome - This is due to a defect in the cells of the proximal convoluted tubule. - The proximal tubular cells fail to reabsorb bicarbonate, leading to type 2 (proximal) renal tubular acidosis. T - he proximal tubular cells also fail to reabsorb glucose and amino acids, explaining the findings of protein ++ and glucose +++ on the patient's urinalysis, in spite of normoglycaemia. - The patient's blood tests, showing depletion of potassium, calcium and phosphate due to failed proximal tubular reabsorption of electrolytes, further support the diagnosis of Fanconi syndrome

Question 1198

HIV immunological changes

Answer 1198

A.Reduction in CD4 count B.Decreased IL-2 production C.Decreased NK cell function D.Reduced delayed hypersensitivity response E. Increased Beta-2-microglobulin F. Polyclonal B-cell activation

Question 1199

glucose dependent insulinotropic polypeptide GIP

Answer 1199

incretin hormone that increases the gut transit time and has central effect on reducing obesity

Question 1200

peptide YY

Answer 1200

PYY incretin appetite suppressant delays gastric emptying

Question 1201

liver cirrhosis

Answer 1201

metabolic alkalosis

Question 1202

teriparatide

Answer 1202

PTH analogue increases osteoblast activity procollagen T1 propeptides - CICP PINO OC bone turnover - CTX C terminal cross linked polypeptide

Question 1203

p51

Answer 1203

colon cancer recurrence

Question 1204

p52

Answer 1204

prostate cancer nf kappa b2

Question 1205

hilum of kidney

Answer 1205

level of L1 - R and L renal arteries aorta through diaphragm at T12

Question 1206

senescence - arrest of mitosis

Answer 1206

due to DNA damage - Apoptosis : intrinsic pathway - mitochondrial dysfunction; extrinsic pathway - cell receptor mediated cell death. DNA fragmentation occurs. - Cellular necrosis : occurs due to autolysis and Is a marker for premature cell death - Shortening of Telomeres : progressive shortening of telomeres is one of the mechanism of senescence.

Question 1207

UV radiation leads to

Answer 1207

formation of pyrimidine dimers due to energy absorbed by DNA

Question 1208

triple repeat primed PCR

Answer 1208

Friedrichs ataxia

Question 1209

alkaptonuria HLA

Answer 1209

DR7

Question 1210

PCR process

Answer 1210

- denaturing dsDNA - 95 degree C - Annealing of primer 50-70 degree celsius - Extension of dsDNA molecules - 70 degree C - RT-PCR Is able to identify transcripts of a given gene by detecting the mRNA coding for the gene - Cycling of reaction - mixture temperature enable sequential denaturation of the new DNA strand - Taq polymerase is bacterial origin - Thermus aquaticus

Question 1211

pyramidal tract lesion

Answer 1211

extensors in arms and flexors of lower limbs are affected weakness of arm extensors and hip flexors leg held in extension with foot drop arms across body with elbow and wrist flexed

Question 1212

genetic cloning

Answer 1212

enucleated oocyte fused with the nucleus to form a donor cell. Process is known as somatic nuclear transfer

Question 1213

long distance running leads to

Answer 1213

fatty acid oxidation

Question 1214

internal capsule blood supply

Answer 1214

lenticulostriate vessels fibres of optic radiation - in posterior limb lentiform nuclei - outer putamen and inner globus pallidus

Question 1215

sarcoid granuloma

Answer 1215

- asteroid bodies, Longhand Giant cells, Schaumann bodies Lymph nodes sarcoidosis - Hamazaki - Wesenberg bodies - inclusion bodies of lysosomes with protein, glycoprotein and iron.

Question 1216

CSF production by

Answer 1216

modified ependymal cells in choroid plexus

Question 1217

astrocyte function

Answer 1217

maintains biochemical stasis and provides brain micro-architecture

Question 1218

microglia function

Answer 1218

tissue macrophages

Question 1219

oligodendrocytes function

Answer 1219

forms the myelin sheath that coats axons within the CNS

Question 1220

Schwann cells

Answer 1220

forms the myelin sheet that covers axons in PNS

Question 1221

TPN requirements

Answer 1221

- Reduced food - Nitrogen 0.15-0.2 g N/kg/day; Calories - 25-30 kcal /kg / day - Moderate injury / sepsis : Nitrogen 0.2-0.3; Calories - 30-35 kcal/ kg/day - Severe injury : 0.3-0.35 g N/kg/day; Calories 35-40 kcal/kg/day

Question 1222

salt intake

Answer 1222

6g per day

Question 1223

saturated fat intake

Answer 1223

20-30g per day

Question 1224

anaemia occurs in lead poisoning due to

Answer 1224

inhibition of ferrochelatase enzyme

Question 1225

where does cartilage receive its nutrition from

Answer 1225

diffusion from synovial fluid chrondrocytes secrete collagen and proteoglycans

Question 1226

foam cells

Answer 1226

CD68 derived from monocytes

Question 1227

botulinum toxin prevents release of

Answer 1227

acetylcholine immunogenic type A neurotoxin

Question 1228

Froment's sign

Answer 1228

paper pulled ulnar nerve

Question 1229

MMP

Answer 1229

gelatinase - MMP9 - neutrophil action, angiogenesis, wound repair stromelysin - MMP3/10/11 - degrading a number of tissue constituents including serum collagen subtypes, proteoglycans, fibronectin, laminin, elastin enamelysin - MMP 2 - cleave enamel matrix proteins epilysin - MMP 28 - degrade casein and plays role in tissue homeostasis and wound repair interstitial collagenase - MMP 1 - RA

Question 1230

what correlates well with outcomes from high risk surgery

Answer 1230

cardiac index oxygen delivery - pulmonary artery cactheter

Question 1231

macula is rich in

Answer 1231

cones

Question 1232

optic papilla

Answer 1232

opening of optic nerve

Question 1233

femoral hernia

Answer 1233

lateral and inferior to pubic tubercle

Question 1234

gas exchange happens in

Answer 1234

last 7 divisions of bronchi - Equilibriation of gases takes about 0.25 seconds in the resting lung, decreasing in high cardiac output states as red cell transit time decreases. - Initial 16 branches of bronchial tree = conduction zone.

Question 1235

intense activity with reduced lactic acid

Answer 1235

decreased muscle glycogenolysis McArdle's disease

Question 1236

decreased aortic compliance ->

Answer 1236

increased pulse pressure

Question 1237

blood buffers

Answer 1237

- cell and plasma protein - Hemoglobin - protein and has imidazole side chains and can buffer H+ ions. Deoxy-Hb better buffer than Oxy-Hb - Phosphates - Bicarbonate ions - Carbonic acid

Question 1238

alpha vs B/G actins

Answer 1238

cytoskeleton globular protein found in microfilaments of cytoskeleton and in thin contractile filaments of muscle cells

Question 1239

cell cycle checkpoint

Answer 1239

G1/S G2/M

Question 1240

obese patient CVC subclavian landmark

Answer 1240

2 cm below mid point of clavicle, and 1 cm laterally

Question 1241

PCT

Answer 1241

AD uroporphyrinogen decarboxylase * Blistering skin lesions on sun exposed parts of Boyd * Hypertrichosis on cheeks and forearms * Scarring alopecia * Red urine due to large quantities of porphyrins * Labs : mild elevates AST, ALT; Elevated plasma and urine total porphyrins, faporphyrins * Mx : avoid exacerbating factors, * Repeated Phlebotomy - preferred over low dose hydroxychloroquine if ferritin > 600 ng/mL or homozygosity or compound heterozygosity for HFE mutations * Low dose hydroxychloroquine

Question 1242

irinotecan

Answer 1242

topoisomerase inhibitor blocks the breaking and rejoining of phosphodiester backbone of DNA strands

Question 1243

vinca alkaloids

Answer 1243

inhibits microtubule assembly

Question 1244

BCR-ABL tyrosine kinase inhibitor

Answer 1244

imatinib

Question 1245

fludarabine

Answer 1245

competition inhibition of nucleoside synthesis interferes with DNA synthesis and blocks replication

Question 1246

tetanus toxin

Answer 1246

cleaves the specific sites of synaptobrevin (VAMP) * Synaptic fusion complex = Synaptobrevin + Syntax + Synaptosome-associated protein SNAP-25 * This fusion complex serves to join the membranes of ACh vesicle and nerve cell. Botox cleaves Types A and E - SNAP 25

Question 1247

VLDL

Answer 1247

* VLDL - transports endogenous products - TGs, Phospholipids, Cholesterol and cholesterol esters. * Chylomicrons : transport of exogenous products * VLDL particles are continuously excreted in the liver and contains most of the TGs in the body. * Main source of energy during prolonged fasting. * Apolipoprotein C-II on its surface * Binds to capillary endothelium * Allows for TGs to be progressively removed by LPL * LPL leaves a particle without TGs and Apolipoprotein C-II —> this particle is known as IDL. Chylomicrons are synthesised post prandially in small intestinal enterocytes. HDL - takes up cholesterol from cells.

Question 1248

riboflavin AE

Answer 1248

Co-factor of Flavin adenine dinucleotide (FAD) and Flavin mono nucleotide (FMN) * Adverse effect : Angular cheilitis * Formed from Ribitol - alcohol derived from pentose sugar ribose * Dietary sources : liver, milk, cheese, eggs, beer * Involved in redox process involving hydrogen-transfer chain in mitochondria and production of ATP

Question 1249

glucose transporters

Answer 1249

GLUT 1 - fetal cells, in adults in erythrocytes and endothelial barrier cells. Bidirectional transporter. GLUT 2 - transport of monosaccharides, expressed in hepatocytes, renal tubular cells, pancreatic beta cells and small intestinal epithelial cells. Bidirectional GLUT 3 - neurons and placenta GLUT 4 - insulin mediated glucose uptake in muscles and adipose tissue, glitazones increase cell surface expression GLUT 14 - testes, poor prognosis in gastric adenoca

Question 1250

HH liver biopsy

Answer 1250

iron overload in Kupffer cells

Question 1251

liver transplant metabolic imbalance

Answer 1251

metabolic alkalosis

Question 1252

GIST and CML tx

Answer 1252

imatinib

Question 1253

caspases

Answer 1253

- in extrinsic pathway of apoptosis - death domains attract intracellular adapter proteins - CD95/FADD = MORT-1, which in turn attracts Procaspase 8 - Procaspase 8 is then modified to form a complex of two small and two large heterodimers - constituting active caspase 8. - Caspase 8 activates downstream caspases like caspase 3 (executioner caspase) and causes host cell undergoing apoptotic changes - chromatin condensation and cell fragmentation - Caspase 3 = executioner caspase - Death domain - attracts intracellular adapter protein CD95/FADD - MORT1

Question 1254

human mitochondria contain

Answer 1254

circular dsDNA nucleus - linear dsDNA nucleolus - RNA

Question 1255

phagocytosis

Answer 1255

involves chemotaxis, ingestion within a phagosome, intracellular enzymatic degradation and exocytosis. - Endocytosis of antigenic material - step in antigen presentation

Question 1256

Wilson's disease confirmation

Answer 1256

liver biopsy ATP7B gene

Question 1257

prostaglandin

Answer 1257

synthesised from straight chain C20 fatty acids, arachidonic acid precursor binds to GPCR

Question 1258

inferior alveolar nerve

Answer 1258

branch of mandibular division of trigeminal nerve - all the teeth are anaesthesised in inferior alveolar block - Mental branch of inferior alveolar nerve also supplies the chin and lower lip which becomes numb in nerve block. - Inferior alveolar nerve supplies all the teeth of semi-mandible on the affected side including lower incisor teeth.

Question 1259

heparin inhibits

Answer 1259

aldosterone secretion -> hyperK

Question 1260

isolate hypertriglyceridaemia

Answer 1260

alcohol

Question 1261

where are melanocytes

Answer 1261

basal layer of epidermis no desmosome - small dark nuclei and clear cytoplasm

Question 1262

cholera endotoxin

Answer 1262

binds to GM1 receptor located on small intestinal mucosal cells. Enter of A1 subunit into secretory cells leads to conitnouscAMP activation and increase in cAMP levels leads to massive secretion of water and electrolytes in small intestine.

Question 1263

Tay Sachs disease

Answer 1263

harmful quantities of ganglioside GM2 accumulate in tissues and nerve cells beta hexosaminidase A deaf blind cannot swallow muscles atrophy and paralysis dementia seizures increased startle cherry red spots in macula AR

Question 1264

metabolic response to trauma

Answer 1264

catecholamines cortisol glucagon GH adrenaline and glucagon most rapid - stimulates glycogenolysis

Question 1265

interferons

Answer 1265

alpha and beta interferons same cell receptor induce upregulation of molecules that inhibits viral production of RNA and DNA induces MHC Class 1 expression leading to enhanced lysis of cells IFN - alpha : activates macrophage and neutrophil killing cells. * IFN - gamma : induces expression of MHC Class II * IFN - alpha and beta : induces expression of MHC Class I * IFN - alpha : also upregualtes the host response and improves the chances of clearance of atypical mycobacterial diseases

Question 1266

tacrolimus

Answer 1266

calcineurin inhibitor

Question 1267

basiliximab

Answer 1267

IL2 inhibitor promotes T cell differentiation and proliferation

Question 1268

daclizumab

Answer 1268

IL2 inhibitor

Question 1269

MMF inhibits

Answer 1269

monophosphate dehydrogenase - blocks the de novo pathway of purine synthesis ; adverse ; GI upset and marrow suppression

Question 1270

sirolimus inhibits

Answer 1270

receptor - dependent signal transduction of IL-2 and other cytokines via its action on mTOR (mammalian target of Rapamycin) - Adverse effect - causes hyperlipidemia

Question 1271

spinal cord terminates at

Answer 1271

L1-L2 (18in, 45cm) dural sac -> S2 spinal extradural space - fat for diffusion of local anaesthetic and vertebral venous plexus LP is at L3-4 or L4-5

Question 1272

acute pancreatitis

Answer 1272

NG feed persistent vomiting - NJ later parenteral

Question 1273

clearance of drug =

Answer 1273

urine concentration x urine volume production / plasma concentration

Question 1274

C peptide levels

Answer 1274

decreased in insulin OD increased in SU abuse

Question 1275

common femoral artery lies on

Answer 1275

lateral side of femoral vein and divides 3 cm distal to inguinal ligament. * Superficial femoral artery then becomes popliteal artery by passing through hiatus in adductor Magnus * Posterior tibial artery is felt behind medial malleolus * Femoral artery thrombosis - PC intervention with angioplasty, locally delivered thrombolytics.

Question 1276

familial lipoprotein lipase deficiency

Answer 1276

rare and causes marked develatio of TG, eruptive xanthomas and recurrent pancreatitis

Question 1277

Inferior / Recurrent laryngeal nerve —> supplies sensation

Answer 1277

to larynx inferior to vocal cords - All laryngeal muscles are supplied by recurrent laryngeal nerve except cricothyroid muscle which is supplied by superior laryngeal nerve - branch of vagus nerve. - Paralysed vocal cord - paralytic position - adducted or lateral from midline and does not move on phonation - Inferior portion of larynx is anaesthetised.

Question 1278

maturation and remodeling of wound - can last up to

Answer 1278

a year acute inflammation 3 days initiation factor from platelets activated by exposed mature collagen platelets aggregate and release active agents fibrin clot develops scabs * After the initial acute inflammation, macrophages become active as the main agents of demolition removing unwanted fibrin, dead cells, bacteria, and creating fluid fill spaces for granulation tissue. * Macrophages also release factors that stimulate the formation of new capillary buds during this phase and later, they initiate and control fibroblast activity during repair * Within the connective tissue randomly oriented, collagen begins to form after a few days, reaching a peak of activity after 5 to 7 days * Acute Inflammation is the first phase of wound healing. * Macrophage proliferation is the hallmark of demolition phase * Eternal cells at the edge of the wound, start to proliferate after 24 hours and this can last for up to 3 weeks.

Question 1279

posterior gastric ulcer

Answer 1279

splenic or GD

Question 1280

lesser curve of stomach artery

Answer 1280

L gastric greater = L GE

Question 1281

rapid correction of hyponatraemia

Answer 1281

CPM or osmotic demyelination

Question 1282

fleshy lumps over elbows and fat deposits in palmar creases and elevation of cholesterol and TG concentration to almost the same value

Answer 1282

familial dysbetalipoproteinaemia * Labs will show : elevated Total cholesterol, elevated TGs, elevated VLDL and elevated IDL. * Significant risk of developing coronary heart disease * Presence of palmar xanthoma —> pathognomic

Question 1283

defect in DNA helicase

Answer 1283

xeroderma pigmentosum Cockayne syndrome trichothiodystrophy

Question 1284

enzymes cell

Answer 1284

* Nuclease : enzymes that degrade nucleic acid molecules by cleaving the phosphodiesterase bonds between nucleic acid subunits * Phosphodiesterase : inactivation of intracellular 2nd messengers such as cGMP and cAMP. * Restriction endonuclease : cuts DNA molecules at a limited number of specific nucleotide sequences * Telomerase : enzymes that maintain the ends of eukaryotic chromosomes by synthesising telomeric repeat sequences

Question 1285

MCA termination of

Answer 1285

ICA MMA is a branch of maxillary MCA passes through the lateral sulcus of cerebrum between the temporal and frontal lobes

Question 1286

involuntary vs voluntary incontinence

Answer 1286

* Involuntary faecal incontinence is controlled by internal anal sphincter * Voluntary faecal incontinence is controlled by external anal sphincter

Question 1287

PDA supplies

Answer 1287

posterior portion of IV septum and posterior L ventricular free wall

Question 1288

facial veins

Answer 1288

Cavernous sinus - lies on either side of body of sphenoid and receive blood through facial veins * Facial veins = superior and inferior ophthalmic veins * sphenoid viens, middle cerebral veins also drain into cavernous veins. * Anterior facial veins - drains the face and upper lip which is a high risk of spread of infection * Occipital vein drain directly into jugular venous system * Venous drainage of chin directly into jugular venous system * Pinna of ear- maxillary vein - and drains into jugular venous system * Skin over parotid gland - maxillary vein —> jugular venous system

Question 1289

active form of aciclovir

Answer 1289

acyclovir triphosphate which is formed by host cell kinases allows it to incorporate into viral DNA polymerase resulting in chain termination viral DNA polymerase unable to function and replication of virus halted peak conc 1-2 hours HSV - thymidine kinase

Question 1290

BRCA1 inheritance

Answer 1290

AD

Question 1291

most common EDS

Answer 1291

hypermobile

Question 1292

latex allergy food

Answer 1292

kiwi avocado banana chestnut papaya potato tomato

Question 1293

female carriers of DMD develops

Answer 1293

symptomatic cardiomyopathy

Question 1294

VWD chr

Answer 1294

12

Question 1295

Hurler syndrome type 1

Answer 1295

autosomal recessive due to defective chromosome 4p. C/F : intellectual disability, corneal clubbing, cafe au lait spots, cardiac disease - death common in the first decade

Question 1296

scromboid

Answer 1296

biogenic amines like histamine

Question 1297

cryoglobulinaemia

Answer 1297

* Type I : Monoclonal - IgG or IgM - Waldestrom’s Macroglobulinaemia (IgM), Multiple Myeloma (IgM) * Type II : Mixed monoclonal and polyclonal - Hepatitis C, HIV, Rheumatoid arthritis, Sjogren’s syndrome, Lymphoma * Type III - only polyclonal - Sjogren’s, Rheumatoid arthritis * Type II & III —> Rheumatoid factor +ve * Hepatitis B - not associated with Cryoglobulinaeia * Raynaud’s phenomenon - ONLY Type I Cryoglobulinaemia * Other C/F : vascular purpura, distal ulceration, arthralgia, renal - diffuse GN * Labs - Low C4, Raised ESR * Mx : Immunosuppression, Plasmapheresis

Question 1298

Turner syndrome

Answer 1298

45XO karyotyping

Question 1299

SCID

Answer 1299

X linked IL2 receptors - ADA enzyme : eliminates deoxyadenosine which is toxic to lymphocytes and is generated when DNA is broken down - ADA also converts Deoxyadenosine to Inosine - Mutations in ADA gene —> reduces or eliminates activity of ADA and allows for build up of deoxyadenosine - Immature lymphocytes are vulnerable to deoxyadenosine - C/F : current bacterial, Viral and fungal infections - Reduced T cell receptor excision circles - Stem cell transplantation may be successful

Question 1300

P1 receptor

Answer 1300

GPCR preferentially bound by adenosine mast cell function and eosinophil apoptosis brake in activated immune system

Question 1301

partial lipodystrophy

Answer 1301

type 2 MPGN intramembranous immune complexes with dense deposits on renal biopsy low C3 high C3b nephritic factor induces lysis of adipocytes * Associated with Type 2 MPGN, SLE, POEMS = polyneuropathy, organomegaly, endocrinopathy,monoclonal immunoglobulin, skin changes syndrome * Also associated with Diabetes

Question 1302

opsonisation

Answer 1302

coating a target with complement to promote elimination T cells humoral only identifies endotoxin - triggers immune system through linkage with CD14 on monocytes and macrophages

Question 1303

protein losing enteropathy

Answer 1303

low total cholesterol leakage of individual serum proteins is independent of molecular weight For this reason - low cholesterol levels are seen in protein losing enteropathy as cholesterol molecules are having hig

Question 1304

Hyper-IgM syndrome

Answer 1304

CD40 ligand deficiency B cell defect with recurrent sinpulmonary infections PCP ++ risk chronic cryptosporidial infections inc risk of malignancy

Question 1305

vecuronium

Answer 1305

anaesthetic allergies ammonium group patch test

Question 1306

pemphigus gestationalis

Answer 1306

2/3rd trimester urticarial lesions and blisters on anterior abdominal wall surrounding the umbilicus linear C3 deposition at derma-epidermal junction

Question 1307

MGUS

Answer 1307

presence of serum monoclonal protein (M-protein) at a concentration < 3 g/dL, bone marrow with < 10% of monoclonal plasma cells and absence of end organ damage { lytic bone lesions, anaemia, hypercalcemia, renal insufficiency, hyper viscosity}

Question 1308

AIP

Answer 1308

bulbar paresis urinary uroporphobilinogen serum delta ALA and porphobilinogen hyponatraemia IV haemin dextrose haem alginate

Question 1309

variegate porphyria

Answer 1309

AD protoporphyrinogen oxidase South Africans

Question 1310

treatment of C1inh deficiency

Answer 1310

C1 inhibitor or icatibant (bradykinin inhibitor)

Question 1311

XLR

Answer 1311

only males affected -> unaffected sons and carrier daughter - Heterozygous carrier female —> A.50% chance of male child being affected B.50% chance of female being carrier. C.25% chance of all children being affected D.25% chance of carrier children

Question 1312

selective IgA deficiency

Answer 1312

IgG2 deficiency recurrent bacterial infections risk of pernicious anaemia and gastric ca

Question 1313

birch apple syndrome

Answer 1313

apple celery oranges - asthma

Question 1314

periodic fever syndromes

Answer 1314

FMF TRAPS Hyper IgD lack high titre abs or Ag specific T cells

Question 1315

low TNF levels

Answer 1315

cachexia

Question 1316

HPA axis activation

Answer 1316

TNF and IL1

Question 1317

isotope suppression levels more a feature of

Answer 1317

myeloma than WM IgA and IgG ++ in Wald only IgM

Question 1318

HHT

Answer 1318

AD endoglin deficiency - TGFb receptor

Question 1319

increased ghrelin in

Answer 1319

prader willi from arcuate nucleus and hypothalamus

Question 1320

IgG2 deficiency

Answer 1320

infections with polysaccharide coated bacteria including Hib -> otitis media and resp tract infections

Question 1321

linkage analysis

Answer 1321

quite common for a gene mutation not to be found in a family member even if gene for condition is known. * Linkage analysis can be performed using micro satellite markers. * Testing both affected and unaffected members —> allele carrying the mutation can be tracked through the family.

Question 1322

microarray analysis

Answer 1322

gene expression

Question 1323

Kearns-Sayre syndrome

Answer 1323

mitochrondrial SNHL, RP, weak, ataxis, cardiomyopathy, dtrokes

Question 1324

Usher syndrome

Answer 1324

hearing loss and RP

Question 1325

Behcets affects

Answer 1325

post capillary venalities ANCA assoc - small-med PAN - med Takayasu - large

Question 1326

majority of ANA

Answer 1326

IgG type

Question 1327

elevation of ESR without CRP

Answer 1327

typical of SLE lymphopaenia is also typical

Question 1328

B cells can undergo

Answer 1328

somatic hypermutation isotope switching have surface bound IgG

Question 1329

hyperacute rejection of renal transplant HLA

Answer 1329

HLA-C IgG antibodies CW5 subtype

Question 1330

vitamin D resistant rickets

Answer 1330

XLD

Question 1331

isolated IgD def

Answer 1331

no tx Basque

Question 1332

CF

Answer 1332

AR - Population carrier fluency —> 1/25 chance of father being carrier. - Chance of father passing the gene - 1/25 x 1/2 = 1/50 - Mother of child had a sister with CF who died. - Parents of the mother are carriers Xx - CF gene = X - Normal gene = x - If mother is healthy —> she has 2 out 3 chances of being a carrier. 1/3 times she is normal. - Chances of passing gene is 2/3 multiplied with 1/2 = 1/3. - Chances of couple having cild with CF = 1/3 x 1/5- = 1/150.

Question 1333

X-linked adrenoleukodystrophy

Answer 1333

ABCD1 gene codes for ALD peroxisomal membrane transporter protein misdiagnosed as MS assay of long chain fatty acid elevated mx - Lorenzo's oil AR leukodyst - neonates fatal by 20 -> dev delay, deafness, visual impairment

Question 1334

HbS

Answer 1334

valine substitution for glutamic acid at position 6 of beta globin chain

Question 1335

LGI 1 encephalitis

Answer 1335

encephalitis

Question 1336

KRAS codes for

Answer 1336

GTPase icthyosis and cancers -p16 : 30% of patients with chronic pancreatitis - NRAS mutation : malignant melanoma - p53 : associated with pancreatic cancer in only 50% of pancreatic adenocarcinoma - Rb gene : Retinoblastoma, bladder cancer

Question 1337

Canavan's disease

Answer 1337

progressive neuro degen due to defect in aspartoacylase

Question 1338

Tendon xanthomata, total cholesterol elevated > 7.5, LDL > 4.9

Answer 1338

= heterozygous familial hypercholesterolaemia * Familial HDL deficiency : premature cardiovascular disease with normal LDL cholesterol levels. * Familial mixed hyperlipidemia : rise in LDL and triglycerides * Heterozygous familial hypertriglycerihdemia : rise in TGs * Homozygous familial phypercholesterolaemia : total cholesterol in the range of 12-15 or more and very early CVD

Question 1339

homozygous C1q deficiency

Answer 1339

SLE Anti-C1q antibodies : associated with hypocomplementemic urticarial vasculitis - rare autoimmune disease - recurrent urticaria - nettlerash; Dx confirmed by at least 2 of the following features : venulitis on skin biopsy, arthritis, ocular inflammation, abdominal pain, positive Cq1 antibodies to immune complexes

Question 1340

WFS1 gene

Answer 1340

Wolfram DIDMOAD AR

Question 1341

Huntington repeat

Answer 1341

CAG

Question 1342

mycophenolate is

Answer 1342

anti purine selectively depletes B and T cells - neutropaenia rare

Question 1343

2 most common mutations of HHch

Answer 1343

C282Y and H63D

Question 1344

eye of the tiger sign on MRI

Answer 1344

deposition of iron in the BG AR * Pyramidal and extra pyramidal signs * Dementia * Optic atrophy

Question 1345

calcineurin inhibitors stop production of

Answer 1345

IL2 —> inhibits calcineurin —> decreased IL-2 production —> less T lymphocyte mediated transplant rejection —> also reduced t-regulatory cell activity

Question 1346

Dubin johnson syndrome

Answer 1346

AR iranian jews cMOAT conjugated hyperbilirubinaemia

Question 1347

follicular ca associated with

Answer 1347

Gardners and Cowdens Screening for medullary thyroid cancer - Pentagastrin and calcium infusion test and measurement of calcitonin * Family history of MEN2 —> total thyroidectomy in childhood to prevent development of carcinoma is recommended * Follicular carcinomas - associate with Gardner’s syndrome and Cowden’s syndrome * Papillary carcinoma - familial papillary carcinoma of thyroid - mutations on chromosomes 1 and chromosomes 8. * Papillary cancer of thyroid - also associated with Gardner’s syndrome, and Cowden’s syndrome.

Question 1348

EBV quiescence

Answer 1348

In B-lymphocytes —> virus is quiescent ; allows it to hide from Tlymphocyte surveiilance.

Question 1349

HS deficiency

Answer 1349

spectrin and ankyrin

Question 1350

XP defect

Answer 1350

in nucleotide excision repair AR * Characterised by photosensitivity with severe sunburn in infancy * Development of numerous spots resembling freckles, larger atrophic lesions, associated with telangiectasia and multiple solar keratosis. DNA damaged by UV radiation not repaired

Question 1351

allergic reaction

Answer 1351

early phase - Due to release of histamine and mast cell degranulation, production of leukotrienes, Pgs, cytokines due to allergenspecific IgE molecules binding to mast cel receptors late phase - act on endothelial cells and causes endothelial cells to express molecules such as vascular cell adhesion molecule and selectins. * This results in recruitment and actuation of leukocytes from the blood into the site of allergic reaction. * Th-2 cells and the cytokines they produce leads to further recruitment of mast cells and eosinophils and class switching happens in plasma cells to IgE isotype.

Question 1352

homocystinuria mutation

Answer 1352

G307S

Question 1353

complement cascade

Answer 1353

C1q binds to IgM/G complexed with antigen -> formation of membrane attach complex which is responsible for disrupting the cell membrane of bacteria - C2 complement is responsible for joining with C4 to form C3 convertase. - C3b component of C3 is responsible for joining with C4b2a to form C5 convertase - C4 is responsible for koining with C2 to form C3 convertase - C5 is cleaved to form C5a - potent anaphylatoxin and C5b - which recruits the components of MAC.

Question 1354

alternative complement pathway

Answer 1354

C5-9 meningitis

Question 1355

Remnant hyperlipidaemia / familial dyslbetalipoproteinaemia / remnant hyperlipidaemia —>

Answer 1355

fibrate and statin

Question 1356

vWD having major surgery

Answer 1356

DDAVP and TXA

Question 1357

missense mutation

Answer 1357

lateration in nucleotide sequence that converts a codon for one amino acid into a codon for a different amino acid —> protein that functions abnormally

Question 1358

repeat mutation

Answer 1358

short DNA sequences are repeated a number of times, results in abnormal protein function

Question 1359

nonsense mutation

Answer 1359

alteration in nucleotide sequence that changes a triplet coding for amino acid into a termination codon

Question 1360

point mutation

Answer 1360

single nucleotide change in DNA molecule

Question 1361

placental syncytiotrophoblasts

Answer 1361

Fc IgG receptors facilitates transport into circulation' IgA in breast milk

Question 1362

EDS genes

Answer 1362

COL3A1 = vascular Ehler Danlos COL5A1 = classical Euler Danlos PLOD1 gene = kyphoscolisosis Ehler Danos

Question 1363

Brodie's abscess

Answer 1363

* X-ray features : lytic lesion with sclerotic margins in upper tibial metaphysis * Localised abscess present deep within the bone near the site of metaphysis * Deep boring pain

Question 1364

Takayasu

Answer 1364

Chronic autoimmune inflamamtor condition - large and medium sized aretireis * Transmural fibrosis and stenosis with occlusion * Pulseless disease * CD4+ and CD8+ cells aid in the inflammatory process forming granulomas and prompting chronic gradual process of inflammation C/F : * Renal artery stenosis - hypertension * Carotid artery involvement - stroke, dizziness, headaches * Jaw claudaition, mesenteric ischaemia, heart failure Labs : * ESR > 20 * Clinical basis diagnosis

Question 1365

anti U1 RNP ab

Answer 1365

MCTD

Question 1366

D penicillamine can cause

Answer 1366

pancytopaenia

Question 1367

chest pain, dyspnoea, low BP and small voltage cardiac complexes on ECG in RA

Answer 1367

cardiac tamponade

Question 1368

HLA DRW4

Answer 1368

Felty

Question 1369

HLA B8DR3

Answer 1369

Sjogrens

Question 1370

HLA B8

Answer 1370

PBC

Question 1371

rosary sign on angiography

Answer 1371

PAN

Question 1372

what is RF

Answer 1372

* Antibody against epitomes of Constant region Fc portion of IgG1 * Active rheumatoid arthritis —> RF complexes are present in synovial fluids and IgG producing B cells

Question 1373

psoriasis on mtx and ssz

Answer 1373

add adalimumab

Question 1374

safe drugs in pregnancy for RA

Answer 1374

HCQ NSAID TNF a inhibitors

Question 1375

Non productive cough and dyspnoea. CXR - may show military or diffuse nodular pattern.

Answer 1375

bronchiolitis obilterans in RA

Question 1376

Caplan syndrome

Answer 1376

pneumoconiosis and rheumatoid nodules

Question 1377

synovial fluid in RA

Answer 1377

marked vascular proliferation on synovial membrane —> thickening of synovial membrane and proliferation into folds and fronds may also be seen. Synovial fluid in RA is turbid with neutrophil - NOT a diagnostic feature. Viscosity of synovial fluid decreased in RA - increased in OA.

Question 1378

Premier's disease

Answer 1378

BL idiopathic avascular necrosis

Question 1379

raised ALP and low Ca

Answer 1379

osteomalacia Looser lines on XR and pseudofrac

Question 1380

single most important influencing factor for attaining peak bone mass density

Answer 1380

genetic factors

Question 1381

lupus nephritis mx

Answer 1381

steroids + cyclo/MMF

Question 1382

RA pleural effusion

Answer 1382

low glucose, exudative, low ph, high LDH

Question 1383

elevated levels of TNF-alpha, IL-1, IL-6, FGFR, PDGF. Il-6 can be targeted by Tocalizumab

Answer 1383

RA

Question 1384

anky spond not responding to nsaid

Answer 1384

adalimumab

Question 1385

WEGENERS

Answer 1385

cough haemoptysis nasal, epistaxis, saddle nose mononeuritis multiplex, proptosis

Question 1386

PAN criteria

Answer 1386

1) weight loss of 4 kg or more 2) Lived reticularis 3) Testicular pain / weakness 4) Mononeuropathy / polyneuropathy 5) DBP > 90mmHg 6) Elevated urea and creatinine 7) Hepatitis B infection 8) Abnormality on arteriography 9) Bx - polymorphonuclear neutrophils

Question 1387

chair raise test

Answer 1387

tennis elbow

Question 1388

Scheuermann's disease

Answer 1388

kyphosis *** Congenital absence of neural arch —> kyphoscoliosis. *** Anterior displacement of one thoracic vertebra upon another occurs in spondylolisthesis *** Osteophyte formation on posterior facet joints is seen in osteoarthritis

Question 1389

earliest xray feature of AS

Answer 1389

blurring of upper and/or lower vertebral rims at the sacroiliac junction - best seen on a lateral x-ray. This is caused by an enthesitis at the insertion of intervertebral ligaments. Persistent enthesitis —> can cause bony spurs - syndesmophytes

Question 1390

Paget's spine

Answer 1390

lumbar

Question 1391

TNFa

Answer 1391

- raised levels leads to increased insulin resistance due to phosphorylation of Insulin receptor Substrate - 1 IRS-1 - Neutrophil chemoattracts, stimulates macrophage phagocytosis and helps drive the acute phase response - TNF alpha acts on wide variety of cells - hypothalamus, liver, neutrophils, macrophages - Its gene is present on chromosome 6 - Lipopolysaccharide increases TNF-alpha production

Question 1392

gull-wing of inverted T pattern of erosions

Answer 1392

erosive inflammatory OA

Question 1393

pyrazinamide

Answer 1393

ATT - storng urate retention agent - causes >80% reduction in renal clearance of uric acid Metabolite of Pyrazinamide —> Pyrazinoic acid - oxidised by xanthine oxidase and is likely responsible for hyperuricaemic effect

Question 1394

eosinophilic fascitis

Answer 1394

* Swelling and tenderness of forearms with induration of skin * Carpal tunnel syndrome * Flexion contractures of fingers * Peripheral blood eosinophilia * Hyper-gammaglobulinaemia

Question 1395

how does anti Ro cause HB

Answer 1395

recognises the cardiac 5-HT 4 serotonin receptor - and inhibits serotonin - activated calcium L channels

Question 1396

Capgras and Fregoli

Answer 1396

replaced by imposter same person in disguise

Question 1397

C9orf72 - hexanucleotide repeat expansion of GGGGCC in C9orf72 gene

Answer 1397

frontotemporal dementia

Question 1398

TNR

Answer 1398

CGG repeat - FMR1 gene - fragile X CTG - DMPK gene - myotonic dystrophy GCG - PABPN1 gene - oculopharyngeal muscular dystrophy GAA - FXN gene - Friedrich’s ataxia

Question 1399

anorexia raised

Answer 1399

GH glucose salivary glands cortisol cholesterol carotinaemia