MRCP Part 2 Written Flashcards

Question

Which renal disease has the highest recurrence rate in kidney transplant?

Answer 1

``` Membranoproliferative Glomerulonephritis (30-90%) Focal Segmental Glomerulosclerosis (40%) Membranous Glomerulonephritis (30%) ```

Answer 2

Cortisol should reach over 550 by 30 mins failure to do this suggest adrenal insufficiency

Answer 3

Associated with small cell lung cancer and neuroblastoma Associated with sensory neuropathy (Often painful), cerebellar syndrome, and encephalomyelitis.

Answer 4

Associated with ovarian and breast cancer Associated with cerebellar syndrome

Answer 5

Opsoclonus Myoclonus, ataxia

Answer 6

Older age at diagnosis Use of multiple antivconvulsants History of Myoclonic or tonic-clonic seizures. Previous abnormal imaging or EEG

Answer 7

- Occuring frequently (10 or more beats/hour or 6 or more beats /min) - PVE in bigeminal rhythm - PVE in short runs of VT - PVE exhibiting R-on-T phenomenon - PVE associated with serious organic heart disease such as in the context of MI or LV decompensation

Answer 8

``` Allopurinol Cimetidine Ciprofloxacin Corticosteroids Diltiazem Erythromycin Frusemide Isoprenaline Oral Contraceptives Thiabendazole Verapamil ```

Answer 9

Deletion of long arm of chromosome 7, Elfin like face, intellectual disability, outgoing personality. Associated with Supravalvular Aortic stenosis

Answer 10

Pulmonary Stenosis Overriding Aorta Inter-ventricular septal defect Right ventricular hypertrophy.

Answer 11

Duchenne: - Absent/Non functional dystrophin gene - 1/3600 Male births incidence - Onset 3-5 yrs - Fast disease progression - Mean life expectancy mid 20s - Onset of cardiomyopathy is after skeletal progression. Becker’s: - Partially functional dystrophin gene. - 6/100 000 male births incidence - Onset 12 yrs - Slow disease progression - Mean life expectancy 40s - Onset of cardiomyopathy May present before skeletal symptoms.

Answer 12

Type 1: monoclonal usually IgM. E.g. Waldenstroms macroglobulinaemia or multiple myeloma Type 2: monoclonal IgG ‘mixed cryoglobulin’. Associated HIV + Hep C Type 3: polyclonal associated connective tissue disease especially Sjögren s syndrome. Both type 2 and 3 can have rheumatoid factor activity so may be associated with positive RhF

Answer 13

Around 1 in 6 patients taking amiodarone develop thyroid dysfunction. Amidarone-induced hypothyroidism: Occurs in patients with a previously normal thyroid gland. This is due to the autoregulatory phenomenon where high levels of circulating iodine reduces thyroid hormone synthesis and is known as the Wolff-Chaikoff effect. Amiodarone-induced Thyrotoxicosis: Two main types: - Type 1 -Usually occurs in patients with either pre-existing Thyroid disorders or iodine deficiency. Patients with underlying thyroid disorder or iodine deficiency cannot auto regulate effectively and so there is increased sensitivity and increased production and hyperthyroidism. This is known as the Joe-Basedow phenomenon. There is a goitre and it is managed through carbimazole or potassium perchlorate -Type 2 amiodarone-related destructive thyroiditis, goitre absent and managed by corticosteroids. Elevated IL 6.

Answer 14

10% of thymoma develop t + B cell deficiency (good syndrome)

Answer 15

They are more at risk of anaphylaxis with blood products.

Answer 16

Enzyme Inducer

Answer 17

Type 1: - Defect on AIRE Gene - Mucocutaneous Candiasis - Hypoparathyroidism - Hypoadrenalism Type 2: - Schmidt’s syndrome - Hypothyroidism - Hypoadrenalism - other e.g. Coeliac, T1DM, Vitiligo, Myasthenia, Pernicious anaemia, Ovarian failure.

Answer 18

False +ve in 2-12%. Dexamethasone metabolised by p450, Enzyme inducers e.g. Phenobarbital, Carbemazepine, Rifampicin, Smokers, Chronic alcohol abuse can lead to false positives.

Answer 19

Hypokalaemia with hypertension: - Cushing’s syndrome - Conn’s Syndrome - Liddles Syndrome - 11-beta hydroxylase deficeincy - Carbenoxolone - Liqourice excess Hypokalaemia without hypertension: - Diuretics - GI loss (e.g. D+V) - Renal tubular acidosis (type 1 + 2) - Bartter’s syndrome - Gitelman’s syndrome

Answer 20

MODY2 (GCK subtype) is caused by mutation in the glucokinase gene on chromosome 7. Usually there is mild asymptomatic stable hyperglycaemia from birth. Microvascular disease is rare and drugs are rarely needed. - MODY3 (HNF1A subtype) is the most common type. It is caused by a defect on chromosome 12 leading to progressive decrease in insulin production. It features severe hyperglycaemia after puberty which often leads to a diagnosis of type 1 DM. Diabetic retinopathy and nephropathy often occur. - MODY5 (HNF1B subtype) is associated with pancreatic atrophy, renal abnormalities and genital tract malformations. - MODY1,4,6 also exist but are very rare.

Answer 21

Prolonged non bloody diarrhoea

Answer 22

Bloody diarrhoea associated with vomiting and abdominal pain

Answer 23

Transudate Causes: - Cirrhosis - Portal Hypertension - Cardiac failure - Budd-chiari - myxoedema Exudate Causes: - Hepatic/Peritoneal Malignancy - Intraabdominal TB - Pancreatitis

Answer 24

O-DEVICES: - Omeprazole - Disulfiram - Erthromycin - Valproate/ Verapamil - Isoniazid - Cimetidine/Ciprofloxacin - Ethanol intoxication - Sulphonamides

Answer 25

Good pasture syndrome ANCA positive vasculitis with AKI or pulmonary haemorrhage Idiopathic crescentic GN Cryoglobuliaemia Myeloma with hyperviscosity TTP (HUS)

Answer 26

Paracetamol-induced liver failure: - arterial pH less than 7.3 24h after ingestion or all of the following - Prothrombin time greater than 100s - creatinine over 300umol/L - Grade III or IV encephalopathy Non-Paracetamol Liver Failure - PT over 100s OR 3 of 5 of the following: - Drug induced liver failure - less than 10yrs old or over 40 - at least 1wk between jaundice to encephalopathy - PT over 50s - Bilirubin over 300umol/L

Answer 27

``` SLUDGE Salivation Lacrimation Urination Diaphoresis Gastrointestinal upset Emesis, Miosis ```

Answer 28

HLA B1502 is associated with Stevens-Johnson in patient of chines origin taking phenytoin HLA A3101 is associated with Stevens-Johnson syndrome in European or Japanese patients treated with carbamazepine HLA B5701 is assoacited with abacavir hypersensitivity, which resulted in the development of HLA screening before the medication was initiated for HIV. HLA B5801 is associated with allopurinol hypersensitivity

Answer 29

Types: - AIH 1: Positive ANA and SMA, Anti-actin, elevated in IgG - AIH 2: Positive LKM-1 (Tyically female children/teenagers, rare in adults) - AIH 3: Positive Soluble liver antigen antibodies.

Answer 30

Lateral I, aVL, V5, V6 Circumflex artery

Answer 31

Inferior II, III, aVF Right Coronary Artery

Answer 32

Septal V1, V2 Left Anterior Descending Artery.

Answer 33

Two or more of the following: - Six or more Cafe-au-last macules larger than 5mm (prepubertal) or 15mm (post-pubertal) - two or more neurofibromas of any type or one plexiform - axillary or groin freckling - optic glioma - two or more lisch nodules on the iris - distinctive osseous lesion e.g. Sphenoid dysplasia, pseudo-arthrosis - a first degree relative with Type 1 NF

Answer 34

Multiple small round opacities typically 1-10mm in diameter mostly in the upper lobes

Answer 35

Multiple small nodules scattered throughout the lungs mainly upper zones and egg-shell calcification of the hilar nodes

Answer 36

Progressive linear interstitial fibrosis of upper zones

Answer 37

1-alpha-hydroxylase is activated by macrophages in sarcoid granulomas and Converts 25-hydroycitamin D3 to 1,25 dihydroxyvitamin D3, the active form of the vitamin leading to absorption of calcium from the gut and increased renal calcium absorption from the distal tubule.

Answer 38

Type A which indicates a left sided accessory pathway, and show a dominant R wave in V1 and tall R waves and inverted T waves in V1-V3 mimicking right ventricular hypertrophy. Type B indicates a right sided accessory pathway, there is a dominant S wave in V1 and tall R waves and inverted T waves in inferior leads and V4-V6 mimicking left ventricular hypertrophy.

Answer 39

Bradycardia or SVT

Answer 40

``` Haemodynamic instability Life-threatening arrhythmias Serum potassium over 5 in acute toxicity Plasma digoxin level over 12nmol/L Ingestion of more than 10mg in adults and 4mg in children Recurrent bradycardia ```

Answer 41

AKA dystrophia myotonica, an inherited autosomal dominant, myopathy with features developing at around 20-30 years old. It affects skeletal, cardiac and smooth muscles. Types: There are two main types DM1 and DM2. - DM1 present with more distal weakness caused by trinucleotide CTG repeat at the end of DMPK gene on chromosome 19 - DM2 is more proximal, caused by a tetranucleotide repeat expansion of the (CNBP) ZNF9 gene on chromosome 3. Symptoms: -DM1 (Distal weakness, Autosomal Dominant, Diabetes, Dysarthria) General Features: Myotonic fancies (long, haggard appearance), frontal balding, bilateral ptosis, cataracts, dysarthria, myotonia (tonic spasm of muscle), weakness of arms and legs, mild mental impairment, diabetes Mellitus, testicular atrophy, heart block, cardiomyopathy, dysphagia.

Answer 42

Pathognomic of whipples Pendular vergance oscillations of the eyes associated with simultaneous jaw contraction

Answer 43

- modify risk factors e.g. Stop smoking, encourage excercise, weight loss, control hypertension and diabetes. - aspirin + statin - sublingual glyceryl trinitrate for acute attacks - b-blocker or Ca blocker first line - consider dual therapy if fails - if unable to tolerate the above dual therapy consider long-acting nitrat, ivabradine, nicorandil, or ranolazine. - if steal symptomatic refer for PCI or CABG.

Answer 44

Most patients can be managed with Percutanous mitral balloon valvotomy unless they have contraindications such as: - moderate to severe mitral regurgitation - heavily calcified mitral valve - Left atrial thrombus - Concomitant coronary artery or other valve disease requiring surgery In these groups patients should undergo mitral valve replacement

Answer 45

Most common of the early-onset hereditary ataxias. Autosomal Recessive, trinucleotide repeat disorder characterised by a GAA repeat in the X25 gene on chromosome 9. Unusual in trinucleotide repeat disorders in not demonstrating anticipation. Typical age of onset is 10-15yrs. Gait ataxia and kyphoscoliosis are most common presenting features: Neurological features: - Absent ankle jerks/extensor plantars - Cerebellar ataxia - Optic atrophy - Spinocerebellar tract degeneration - Decreased vibration and proprioception, pyramidal weakness Other features: - HOCM (90%, most common cause of death) - Diabetes Mellitus (10-20%) - High-arched palate - deafness

Answer 46

Drugs that can be cleared: BLAST - Barbiturate - Lithium - Alcohol (inc methanol, ethylene glycol) - Salicylates - Theophyllines (charcoal haemoperfusion is preferable) Drugs that cannot be cleared: - Tricyclics - Benzodiazepines - Dextropropoxyphene (Co-Proxamol) - Digoxin - Beta-blockers

Answer 47

Craniopharyngioma

Answer 48

Paget’s disease of the bone Hereditary multiple ostochondromas Retinoblastoma Li-Fraumeni Syndrome

Answer 49

12hrs. Most secondary reaction occur by 10hrs.

Answer 50

Hairy Cell Leukaemia

Answer 51

Acute Myeloblastic Leukaemia

Answer 52

Acute lymphoblastic leukaemia

Answer 53

Tends to be raised in Polycythaemia Rubra Vera and Myelofibrosis and decreased in chronic myeloid leukaemia.

Answer 54

``` Chronic Myeloid leukaemia Malaria Leishmaniasis Myelofibrosis Gaucher's dIsease (Lysosomal storage disease) ```