Miscellaneous

Question 1

What is the investigation of choice to confirm a microbiological diagnosis of TB infection?

Answer 1

Gastric washings on 3 consecutive mornings for microscopy for acid and alkaline fast bacilli and a culture of mycobacterium tuberculosis

Question 2

BCG induration of >15mm is considered positive in who?

Answer 2

Individuals who have no known risk factors for TB, including those with previous BCG vaccination

Question 3

BCG induration of >10mm is considered positive in who?

Answer 3

Recent migrants from high risk areas
Children <4 years old
IVDU

Question 4

BCG induration of >5mm is considered positive in who?

Answer 4

HIV infected individuals
A recent contact of an individual w/ TB
Individuals w/ fibrotic changes on CXR consistent with previous TB
Patients who have undergone organ transplants
Individuals who are immunosuppressed for other reasons

Question 5

What is the mainstay of treatment in autoimmune encephalitis?

Answer 5

IV Immunglobulins
IV corticosteroids
Plasma Exchange

Question 6

Which EEG changes are suggestive of Encephalopathy?

Answer 6

High voltage rhythmic slow wave activity with prominent spikes in the left parietal region

Question 7

How long should treatment be continued in the management of iron deficiency anaemia?

Answer 7

Until at least 3 months after the Hb level reaches the normal value for that age

Question 8

How do you calculate PO morphine dosing in palliative patients?

Answer 8

Add up the total amount of oramorph used in 24 hours and increase by 25-30%, then divide this into 2 doses

Question 9

How is breakthrough analgesia dosing calculated?

Answer 9

Take the amount of opioid used in the previous 24 hours and then give one sixth of this for breakthrough pain

Question 10

What is the biggest side effect of methylphenidate?

Answer 10

Decreased appetite and weight loss
Reduced height velocity

Question 11

What is the mechanism of action of Methylphenidate?

Answer 11

Acts by increasing dopamine levels in the frontal lobe

Question 12

How does TCA overdose present?

Answer 12

Metabolic acidosis with or without respiratory compensation

Question 13

How does aspirin overdose present?

Answer 13

Mixed metabolic acidosis and respiratory alkalosis

Question 14

How does opiate overdose present?

Answer 14

Respiratory depression causing a respiratory acidosis

Question 15

Scurvy is a deficiency of what?

Answer 15

Ascorbic Acid

Question 16

What are the symptoms of scurvy?

Answer 16

Deficiency of ascorbic acid results in a fragility of the capillary walls resulting in petechiae in the skin, mucosa and subperiosteal haemorrhages in bones

Question 17

What is a possible cause of worsening respiratory symptoms in patients with CF?

Answer 17

Aspergillosis

Question 18

What is the treatment of choice for IIH?

Answer 18

Acetazolamide

Question 19

What are the CXR findings in Pneumocystis Jerovecii?

Answer 19

Classically bilateral interstitial andWh alveolar shadowing (may also be normal)

Question 20

What are the investigations of choice in Hirschsprungs Disease?

Answer 20

Bariam Enema
Rectal suction biopsy

Question 21

Which condition presents with a history of painless blistering on hands and feet which starts when crawling?

Answer 21

Epidermolysis Bullosa Simplex

Question 22

Which layer of the skin does Epidermolysis Bullosa affect?

Answer 22

Intraepidermal

Question 23

Which layer of the skin is affected in Dystrophic Epidermolysis Bullosa?

Answer 23

Uppermost dermis - babies are described as butterfly babies because their skin in so fragile

Question 24

What are the side effects of Amphotericin B?

Answer 24

Hypomagnesaemia
Hypokalaemia

Question 25

Describe the formal visual assessments in children of different ages?

Answer 25

4 weeks - visual evoked potentials 6 weeks - optokinetic nystagmus 3 months - fixes and follows 10 months - identifies and picks up small objects 2-3 years - identification of miniature toys at 3m 3-4 years - optotype matching 5 years - snellen chart

Question 26

What is the investigation of choice for brainstem death testing?

Answer 26

Clinical diagnosis

Question 27

What is the investigation of choice for Chlamydia in boys?

Answer 27

First-catch urine sampling for NAAT testing

Question 28

Delayed cord separation and multiple skin abscesses?

Answer 28

Leucocyte Adhesion Deficiency (Established by testing flow cytometry for CD11b)

Question 29

What are the ECG findings in WPW syndrome?

Answer 29

Short PR interval Delta Waves (specifically in lead II) Broad QRS complex

Question 30

What are the symptoms of Phenytoin Toxicity>

Answer 30

CNS dysfunction - nystagmus, diplopia, ataxia, slurred speech

Question 31

When assessing limb BP, what difference in mmHg is significant?

Answer 31

20mmHg

Question 32

How long should be left before giving a live vaccination to immunosuppressed patients?

Answer 32

3 months following stopping oral steroids 6 months following chemotherapy or radiotherapy

Question 33

Which organism causes a rapid deterioration in respiratory symptoms in patients with CF?

Answer 33

Burkholderia Cepacia

Question 34

What is the treatment for B. Cepacia in patients with CF?

Answer 34

Ceftazidime and Aminoglycoside

Question 35

What is the classical presentation of Wilson's disease?

Answer 35

Pyschiatric (depression/ neurotic behaviour) Neurological (extrapyramidal symptoms) Hepatic (elevated transaminases/ asymptomatic hepatomegaly)

Question 36

Saddle shaped nose =

Answer 36

Fetal alcohol syndrome

Question 37

What are the features of Smith-Lemli-Opitz syndrome?

Answer 37

Microcephaly Low set ears Ptosis Micrognathia Synodactyli Hypospadias

Question 38

What is the recommended investigation for SMA type 1?

Answer 38

Molecular genetic testing with targeted mutation analysis for SMN1 gene

Question 39

Which condition is characterised by a herald patch (oval, pink rash with collarette of scales)?

Answer 39

Pityriasis Rosea

Question 40

What are the exclusion criteria for slapped cheek disease (Parvovirus B19)?

Answer 40

Avoid pregnant women Exclusion is ineffective because patients are infectious during the prodrome Notification is not necessary

Question 41

What is the investigation of choice for Pertussis?

Answer 41

Pernasal swab

Question 42

What criteria would suggest a diagnosis of Diabetes Insipidus?

Answer 42

Urine osmolality < 300 Serum osmolality > 300

Question 43

What are the possible complications of Neurofibromatosis Type 1?

Answer 43

Eyes: Glaucoma, Hamartomas Neurological: Learning difficulties, spinal compression Predisposition to tumours: Astrocytomas, phaeochromacytoma, Wilm's tumours Renal artery stenosis leading to hypertension

Question 44

Diarrhoea with high temperatures, relative bradycardia and rose spots on chest and abdomen?

Answer 44

Salmonella

Question 45

What is renal genesis?

Answer 45

A condition in which one or both of the kidneys fail to develop

Question 46

Which medications can cause drug induced aplastic anaemia?

Answer 46

Phenytoin Chloramphemicol Gold Phenylbutazone

Question 47

What is the treatment for listeria (gram positive bacillus)?

Answer 47

Ampicillin - should be suspected in a neonate with meconium stained liquor

Question 48

What are second line antibiotics in neonates?

Answer 48

Vancomycin and Gentamicin

Question 49

William's,syndrome is associated with which cardiac conditions?

Answer 49

Supravalvular Aortic Stenosis Peripheral pulmonary stenosis

Question 50

Melanosis Coli is seen in which type of laxative abuse?

Answer 50

Senna abuse

Question 51

What blood levels should be checked in patients on IVIG replacement?

Answer 51

Liver function Trough Immunoglobulin levels Hepatitis C status

Question 52

What are the diagnostic criteria for cystic fibrosis?

Answer 52

Sweat test result with chloride levels >60 Neonatal serum IRT >900 (Guthrie test) Stool elastase levels (normal is >200)

Question 53

IgA nephropathy presents how many days following viral URTI?

Answer 53

2-3 days

Question 54

How many days post URTI does Post-streptococcal GN present?

Answer 54

1-2 weeks

Question 55

What is the treatment for Neiserria Meningitidis?

Answer 55

Ceftriaxone Cefotaxime (if patient is on TPN/ calcium infusions)

Question 56

Presents with acute encephalopathy with hepatic involvement?

Answer 56

Reye's Syndrome

Question 57

What are the main side effects of methotrexate?

Answer 57

Mucositis Hepatotoxicity Myelosuppression Pulmonary Fibrosis

Question 58

Pyridoxine deficiency features?

Answer 58

Often caused by B6 deficiency Typically causes seizures which are unresponsive to anti-epileptic medications

Question 59

What is beri-beri syndrome a deficiency of?

Answer 59

Thiamine

Question 60

What are the symptoms of Beri-Beri disease?

Answer 60

Irritability Vomiting Seizures Endemic in Indonesian

Question 61

Lyme disease can present as ??

Answer 61

Meningitis

Question 62

What is the investigation of choice for patients with hereditary angioedema?

Answer 62

C1 Inhibitor levels C4 complement levels

Question 63

Which age group is Pneumococcal Conjugate vaccine given in?

Answer 63

Under 2 years

Question 64

Which age group is Pneumococcal polysaccharide vaccination given in?

Answer 64

Over 2 years

Question 65

What are the features of Still's disease aka systemic juvenile idiopathic arthritis?

Answer 65

Arthralgia/ Arthritis High fever that often occurs in the evening Serositis Fleeting salmon coloured, macular rash Anaemia Elevated acute phase reactants

Question 66

Which conditions can cause renal complications despite renal transplantation?

Answer 66

Alport's syndrome HUS HSP Cystinosis Nephrotic syndrome IgA Nephropathy

Question 67

Which rash typically consists of multiple target lesions?

Answer 67

Erythema Multiforme

Question 68

Which condition is erythema multiform associated with?

Answer 68

Mycoplasma

Question 69

What are the possible causes of mixed upper and lower motor neurone signs?

Answer 69

Fredreich's Ataxia MND Vitamin B12 deficiency

Question 70

What is the first line anti-convulsant choice in neonates?

Answer 70

Phenobarbital

Question 71

Prolonged QTc and associated sensorineural deafness?

Answer 71

Jervell-Lange-Neilson

Question 72

What is Haptoglobin?

Answer 72

Haptoglobin is a protein present in the bloodstream which binds to free Hb and removes it from the bloodstream

Question 73

What are the haematological findings during the acute phase of haemolysis in G6PD?

Answer 73

Decreased haptoglobin Increased plasma methaeme albumin Haemoglobinuria Heinz bodies present (denatured haemoglobin and are seen in acute haemolysis)

Question 74

What are the criteria for diagnosis of NF1?

Answer 74

Six or more cafe-au-lait spots (>5mm pre-pubertal, >15mm post-pubertal) Two or more neurofibromas Axillary or inguinal freckling Optic pathway glioma Two or more lisch nodules Osseous lesions (thinning of the cortex of long bones, sphenoid dysplasia) 1st degree relative with NF1

Question 75

What are the features of Galactoassaemia?

Answer 75

Oil drop cataracts Neonatal conjugated jaundice Proximal renal tubular acidosis Hepatomegaly Hypoglycaemia

Question 76

What do oil drop cataracts signify?

Answer 76

Galactoassaemia

Question 77

What is the diagnostic investigation of choice for galactosaemia?

Answer 77

Red cell enzyme levels

Question 78

What is posterior fossa syndrome?

Answer 78

Dysarthria + Emotional Lability + Ataxia + Mutism

Question 79

What is the treatment of choice for Pertussis?

Answer 79

Azithromycin

Question 80

What are the features of Cystinosis?

Answer 80

Deposition of cysteine in the eyes, kidneys and thyroid gland - causing retinitis pigmentosa, hypothyroidism and renal tubular acidosis (hyperchloraemic metabolic acidosis) + polyuria and polydipsia

Question 81

What is the treatment of choice for Hypoplastic left heart syndrome?

Answer 81

Norwood Procedure

Question 82

What are the investigations of choice for post-varicella cerebellitis?

Answer 82

MRI head LP

Question 83

What is Reye syndrome?

Answer 83

Triad of encephalopathy, fatty liver degeneration and elevated transaminases in the post-infectious period, often associated with aspirin ingestion

Question 84

What are the classical findings on blood gas of pyloric stenosis?

Answer 84

Hypochloraemic hypokalaemia metabolic alkalosis

Question 85

Which conditions are coloboma seen in?

Answer 85

CHARGE syndrome Rubenstein-Taybi Syndrome Trisomy 13 Goldenhar Syndrome