2017 remembered Flashcards

1
Q

Spinal muscular dystrophy

A.	Neurological migration dysfunction
B.	Neurological myelination dysfunction
C.	Channel dysfunction
D.	Enzyme dysfunction
E.	Energy dysfunction
A

C. Channel dysfunction

protein necessary for survival of motor neurons

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2
Q

Federeich ataxia

A.	Neurological migration dysfunction
B.	Neurological myelination dysfunction
C.	Channel dysfunction
D.	Enzyme dysfunction
E.	Energy dysfunction
A

D. Enzyme dysfunction

frataxin protein

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3
Q

Baby has hepatosplenomegaly, IUGR, interstitial pneumonitis, jaundice, and petechiae

Toxoplasmosis
CMV
Rubella
Syphilis
Varicella
Parvovirus
A

CMV

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4
Q

Baby has hydrocephalus, intracranial calcification, chorioretinitis

Toxoplasmosis
CMV
Rubella
Syphilis
Varicella
Parvovirus
A

Toxoplasmosis

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5
Q
2.	Ivacaftor is used for management of G551D. Which receptor does it work on?
A.	Class I
B.	Class II
C.	Class III
D.	Class IV
E.	Class V
A

C. Class III

regulation problem

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6
Q
3.	What is the mechanism of clonidine?
A.	Dopamine inhibitor
B.	Alpha 2 adrenergic agonist
C.	GABA inhibitor
D.	Serotonin inhibitor
A

B. Alpha 2 adrenergic agonist

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7
Q
  1. What is the mechanism of action of bisphosphonate pamidronate?
    A. Inhibit osteoclast
    B. Activate osteoblast
A

A. Inhibit osteoclast

decrease bone resorption –> dec Ca

used to treat HI Ca levels

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8
Q
5.	Which is the most common site injured post PEG insertion?
A.	Duodenum
B.	Spleen
C.	Gall bladder
D.	Pancreas
E.	Transverse colon
A

E. Transverse colon

more likely:

  • colon
  • small bowel
  • spleen
  • liver
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9
Q
6.	Pinworms is common in children. Which of this is a pinworm?
A.	Ascaris lumbricoides
B.	Ancylostoma duodenale
C.	Enterobius vermicularis
D.	Trichuris trichuria
A

C. Enterobius vermicularis

A. Ascaris lumbricoides - round worm
B. Ancylostoma duodenale - nematode
C. Enterobius vermicularis - pinworm
D. Trichuris trichuria - whipworm

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10
Q
  1. What enzyme is dysfunctional for methemoglobinemia?
A

A. Cytochrome b5 reductase

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11
Q
8.	How to differentiate Crohns from Ulcerative Colitis?
A.	Crypt abscess
B.	Granuloma
C.	Paneth cell
D.	Lymphocytic infiltrate
E.	Superficial mucosal ulcer
A

B. Granuloma

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12
Q
  1. What is an exome?
    A. Formed by intron and exon
    B. Formed by exons
    C. Protein coding exons
A

B ?C

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13
Q
  1. Why is the gentamicin dose lengthened in neonates?
A

A. Decreased renal elimination

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14
Q
  1. What is the possible serious adverse effect of surfactant?
    A. Pneumothorax
    B. Necrotising enterocolitis
    C. Pulmonary haemorrhage
A

C. Pulmonary haemorrhage

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15
Q
12.	What is the cell lining the esophagus?
A.	Squamous
B.	Non keratinising stratified squamous
C.	Columnar
D.	Gastric
E.	Transition
A

A. Squamous

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16
Q
  1. What is the most important determinant of neonatal cardiac output?
    A. Heart rate
    B. Resistance
    C. Blood pressure
A

A. Heart rate

CO = HR x SV

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17
Q
  1. What is the main determinant to help with neonatal ventilation?
    A. Tidal volume
    B. Minute ventilation
    C. Resistance
A

B. Minute ventilation

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18
Q
  1. What is the definition of hypertension?
    A. Systolic and diastolic BP > 95th centile
    B. Systolic and diastolic BP > 99th centile
    C. Systolic BP > 99th centile + 5mmHg
    D. Systolic BP > 95th centile + 5mmHg
    E. Systolic BP > 95th centile
A

?A. Systolic and diastolic BP > 95th centile

Systolic +/- diastolic BP >/= 95th centile

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19
Q
  1. ADHD associated with
    A. ODD
    B. Mood disorder
A

A. ODD

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20
Q
19.	What is a negative symptom with schizophrenia?
A.	Anhedonia
B.	Thought disorder
C.	Psychomotor agitation
D.	Flight of Ideas
A

A. Anhedonia

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21
Q
20.	Which complement is associated with SLE?
A.	C1q
B.	C3
C.	C4
D.	Factor H
E.	Properdin
A

A. C1q

deficiency

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22
Q
  1. Which organism is associated with complement deficiency?
    A. Encapsulated organism
    B. Gram negative cocci
A

B. Gram negative cocci

Neisseria

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23
Q
22.	What is derived from the pulmonary mesenchyme?
A.	Alveolar epithelium
B.	Smooth muscle
C.	Type 1 cell
D.	Type 2 cell
A

B. Smooth muscle

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24
Q
23.	Which cell is important for mucocutaneous candidiasis?
A.	TNF alpha
B.	IL 1 
C.	IL 6
D.	IL 2
E.	IL 17
A

E. IL 17

disorder of T cells
Th17 –> IL17 –> inflam and WBC

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25
Q
24.	Where is erythropoietin derived from?
A.	DCT
B.	PCT
C.	Collecting tubule
D.	Interstitial cells
A

D. Interstitial cells

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26
Q
25.	Where does the DDAVP act?
A.	PCT
B.	DCT
C.	Ascending limb of Loop
D.	Collecting tubule
E.	Descending limb of Loop
A

D. Collecting tubule

retain H2O

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27
Q
  1. What does the p value mean?
    A. The chance of something happening
    B. Size effect
A

A. The chance of something happening

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28
Q
  1. Child got bitten by a cat on the finger and now has lytic lesion which is confirmed to be osteomyelitis. What is the responsible organism?
    A. Bartonella Henselae
    B. Kingaelae Kingae
A

A. Bartonella Henselae

Cat scratch fever –> OM

K. kingae - common cause of OM

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29
Q
  1. Picture of child with enlarged, red, unilateral lymph node swelling

A. Mycobacterium avium intracellulare
B. Staph aureus

A

A. Mycobacterium avium intracellulare

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30
Q
  1. Patient had splenectomy and has received meningococcal vaccine and pneumovax. Which other vaccines should he have?
    A. Influenza
    B. Pneumococcal polysaccharide
    C. Hemophilus influenza
A

C. Hemophilus influenza

pneumococcal, mening, HiB

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31
Q
32.	What medication to give in narcolepsy?
A.	Dopamine
B.	Serotonin
C.	Orexin
D.	Cortisol
A

B. Serotonin

stimulants
SSRIs/SnRIs
TCA

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32
Q
33.	Which pituitary hormone is destroyed in cranial radiation?
A.	ADH
B.	GH
C.	FSH
D.	LH
E.	TSH
A

B. GH

  • most common

then gonadrotropins, ACTH, TSH

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33
Q
34.	A girl with 11 beta OH deficiency will have
A.	Salt wasting
B.	Hypertension and virilisation
C.	Hirsutism
D.	Hypotension
A

B. Hypertension and virilisation

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34
Q
  1. Difference genetically between DMD and BMD
    A. Intergenic deletion
    B. Frameshift deletion
A

B. Frameshift deletion

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35
Q
  1. Selective mutism is associated with
    A. Anxiety
    B. Depression
A

A. Anxiety

36
Q
  1. Which is associated with acanthosis nigricans?

A. Increased ACTH
B. Increased insulin
C. Increased GH

A

B. Increased insulin

37
Q
38.	What is the composition of 1g of fat?
A.	4 cal
B.	9 cal
C.	10 cal
D.	25 cal
E.	32 cal
A

B. 9 cal

4 cal = carb, protein

38
Q
  1. What causes DRESS?
    A. Antipsychotic
    B. Antiepileptic
    C. Antibiotic
A

B. Antiepileptic

39
Q
  1. Which condition has the best evidence for microbiota transplant?
    A. C diff
    B. Celiac
    C. IBD
A

A. C diff

40
Q
  1. What is the 10% glucose concentration in 0.9% N/Saline?
    A. 10g glucose in 100mL 0.9% N/Saline
    B. 10g glucose in 1000mL 0.9% N/Saline
    C. 10mmol glucose in 100mL 0.9% N/Saline
    D. 100mmol glucose in 100mL 0.9% N/Saline
    E. 10g glucose in something
A

A. 10g glucose in 100mL 0.9% N/Saline

calculation:
(Concentration %/100) x Volume mL = Y (Dosage Amount in g)

0.9 NaCl = 150 mmol of Na and 150 mmol of Cl
10% dextrose in 1L NaCL = 100 g/L glucose

41
Q
  1. Difference in DMD versus BMD
A

CK

Frameshift deletion

42
Q
  1. Which gene caused APACED?
A

A. AIRE

43
Q
  1. 16 year old patient becomes jaundice virus with unconjugated hyperbilirubinemia.
A

A. Gilbert’s

44
Q
28.	Which of this is the first symptom to present in rheumatic heart disease?
A.	Arthritis
B.	Myocarditis
C.	Subcutaneous nodules
D.	Erythema marginatum
E.	Subcutaneous nodules
A

A. Arthritis

45
Q
  1. Patient has anti TTG positive and has a rash. What is it most likely associated with?
A

A. Dermatitis herpertiformis

46
Q
  1. Borderline personality associated with?
    A. Mood disorder
    B. Substance abuse
A

?A. Mood disorder

47
Q
1.	3 year old boy, having 3/7 of fevers, looking miserable. ?more details
A.	Epiglotitis
B.	Bacterial tracheitis
D.	Paratonsillar abscess
E.	Retropharyngeal abscess
A

Differences between all:

48
Q
  1. Girl swallowed 10 cent coin 1 hour ago. Initially had trouble breathing, but is now well. Coin stuck at GE junction.
    A. Reassure and discharge home
    B. Re-Xray in 24 hours
    C. Urgent endoscopy to remove
A

A. Reassure and discharge home

49
Q
  1. Girl presented with abdominal pain and bilious vomiting. She fell over her bicycle handlebar 3/7 ago. What is the pathology?
    A. Liver laceration
    B. Splenic laceration
    C. Duodenal haematoma
A

C. Duodenal haematoma

50
Q
  1. CT difference between:

A. Extradural
B. SAH
C. Subdural
D. IVH

A

differences:

51
Q
  1. Patient with tardive dyskinesia. What to do?
    A. Lower the medication dose
    B. Change to a different antipsychotic
A

B. Change to a different antipsychotic

52
Q
  1. Girl was taking paroxetine and presented with tachycardia, hyperthermia, dilated pupils and hyperreflexia. She was recently prescribed St Johns Warts.
    A. Serotonin syndrome
    B. Neuroleptic malignant syndrome
    C. Serotonin withdrawal syndrome
A

A.. Serotonin syndrome

53
Q
7.	A child has some allergy to eggs when he was 9 months old and is now 12 months old tolerating some cooked eggs. What should he do with the influenza and MMR vaccines?
A.	Give both influenza and MMR
B.	Give MMR and withhold influenza
C.	Do not give both
D.	Give both in hospital setting
A

?B. Give MMR and withhold influenza

54
Q
  1. What is the predictor for renal function in Familial Mediterranean Fever?
    A. CK
    B. Renal US
    C. Amyloidosis
A

C. Amyloidosis

55
Q
  1. Best measure of NASH
    A. ALT
    B. Albumin
    C. Coag
A

A. ALT

56
Q
  1. What is the glargine activity?
A

peal at two hrs, then plateaus for 20-24 hr

57
Q
14.	A 9 year old boy had splenomegaly and received a blood transfusion recently. He is short and poorly grown. His 8 month old brother also has splenomegaly. 
WCC 15 (high)
Macrophage (high)
Red cells Hb 97 (low)
MCV (about normal)
Platelets low

A. Malaria
B. Hereditary spherocytosis
C. Leukemia of some sort

A

?B. Hereditary spherocytosis

58
Q
15.	Febrile neutropenia with strenotrophomas maltophilia on single cefipime. What should we change it to?
A.	Vancomycin
B.	Piperacillin tazobactam
C.	Trimethoprim-sulfamethoxazole
D.	Tobramycin
A

B. Piperacillin tazobactam

59
Q
  1. 6 year old child voids right after going to the toilet which happens only in school but not at night. She is otherwise well.
    A. Dysfunctional voiding
    B. Psychogenic voiding
    C. Urinary frequency
A

A. Dysfunctional voiding

60
Q
  1. What helps to differentiate a child with ASD from other children with pragmatism
    A. Difficulty interacting with others
    B. Difficulty forming conversation of interest
    C. Repetitive and selective interest in a particular topic
A

A. Difficulty interacting with others

61
Q
  1. Child has inverted nipples and abnormal subcutaneous fat pads. Which is associated with this condition?
    A. Glycogen storage disorder
    B. Congenital disorder of glycosylation
    C. Zelleweger
A

?B. Congenital disorder of glycosylation

62
Q
  1. Mum has breast cancer, uncle as adrenal carcinoma and the child has some osteosarcoma. Which is the most common association?
    A. Li fraumeni
    B. Blooms syndrome
A

A. Li fraumeni

63
Q
  1. What is the best use of 17 hydroxyprogesterone test
    A. Virilised girl with salt wasting
    B. Virilised girl with no salt wasting
A

A. Virilised girl with salt wasting

64
Q
  1. Patient with maculopapular rash with something….

X linked lymphoproferative disorder x2

A

X linked lymphoproferative disorder x2

65
Q
22.	Child has decreased consciousness with automatisms of the left hand and then had right sided dystonia. Which part of the brain affected?
A.	Right frontal
B.	Right temporal
C.	Left cortex
D.	Left frontal
E.	Left temporal
A

E. Left temporal

66
Q
  1. Child awakes with morning stiffness which improves with movement.
    A. Arthritis
    B. IBD
A

A. Arthritis

67
Q
  1. Boy with multiple joint pains and …..
    A. Polyarteritis Nodosa
    B. ?IBD
A

A. ?Polyarteritis Nodosa

68
Q
  1. Patient receiving Factor VIII inhibitor via port without problem initially but now has L shoulder problem? X ray present.
    A. Extravasation
    B. Shoulder hematoma
    C. Pneumothorax
A

?A. Extravasation

complications:

69
Q

Types of Mody

A

Types:

70
Q
31.	Patient has bifid scrotum with incomplete male genitalia. Also has uterus and vagina under US. Patient has XY chromosome. Gonads on US are not that well appreciated. What is this?
A.	Denys drash
B.	WAGR
C.	Persistent mullarian duct
D.	CAH
A

?Denys drash

71
Q
  1. 10 month old boy presents with unilateral undescended testes. What should be the next step?
    A. Contact the surgeon for an orchidopexy
    B. Reassure
    C. Review in 6 months
A

A. Contact the surgeon for an orchidopexy

72
Q
  1. Something to do with ?cerebral salt wasting – low sodium, low potassium…. something head surgery or something….
A

?SIADH

73
Q
  1. Which is associated with disrupted circadian rhythm?
    A. Noonan
    B. Trisomy 21
A

B. Trisomy 21

74
Q
35.	Patient has water deprivation test
Initial serum Na 135
Glucose 5.1
				                         DDAVP
Urine osmolality	220	230	240	260
Urine Na	                80	85	88	

A. Central DI
B. Nephrogenic DI
C. Diabetes melitus
D. Psychogenic

A

B. Nephrogenic DI

75
Q
36.	Child with murmur LLSE, softer when sitting up, what is this?
A.	ASD
B.	TOF
C.	Vibratory murmur
D.	Venous hum
A

C. Vibratory murmur

76
Q
  1. Lithium and teratogenic effects?
A

A. Ebstein’s anomaly

77
Q

92.
pH 7.35
CO2 63
HCO3 34

Chronic neonatal lung disease
Hyperventilation
Salicylate poisoning

A

CLD

78
Q

93.
pH 7.4
CO2 32
HCO3 26

Chronic neonatal lung disease
Hyperventilation
Salicylate poisoning

A

resp alkylosis = salicylate poisoning

79
Q
94.
pH 7.1
CO2 35
HCO3 16 
High anion gap

Urea cycle defect
Ketotic hypoglycaemia
Aminoaciduria
Organic aciduria

A

?urea cycle

organic aciduria

80
Q

95.
Ketones +ve
Insulin normal
Low BSL

Urea cycle defect
Ketotic hypoglycaemia
Aminoaciduria
Organic aciduria

A

ketotic hypoglycaemia

81
Q
  1. Rash which is face sparring with MRI meningoencephalitis

Paraechovirus
Neiserria gonorrhoea
Chlamydia

A

paraecho

82
Q
  1. D10 baby with bilateral watery conjunctivitis, – bilat conj

Paraechovirus
Neiserria gonorrhoea
Chlamydia

A

chlamydia

83
Q
99. Day 1 - Long systolic mumur LUSE with ECG - LAD and RAH with peak P wave…. ? 
tricuspid atresia
TOF
Tricuspid atresia
AVSD
TGA
A

TA

84
Q
  1. 6mo, cyanosis,

TOF
Tricuspid atresia
AVSD
TGA

A

?AVSD

85
Q

Smith Magenis Syndrome

A

17p11.2 microdeletion
a developmental disorder that affects many parts of the body. The major features of this condition include mild to moderate intellectual disability, delayed speech and language skills, distinctive facial features, sleep disturbances, and behavioral problems.

Disrupted sleep patterns

  • beginning early in life
  • very sleepy during the day, but have trouble falling asleep and awaken several times each night, due to an inverted circadian rhythm of melatonin

Engaging personalities, but all also have a lot of behavioral problems

  • frequent temper tantrums, meltdowns and outbursts, aggression, anger, fidgeting, compulsive behavior, anxiety, impulsiveness, and difficulty paying attention.
  • Self-harm, including biting, hitting, head banging, and skin picking, is very common
  • Repetitive self-hugging

Other symptoms:

  • short stature, abnormal curvature of the spine (scoliosis), reduced sensitivity to pain and temperature, and a hoarse voice.
  • ear abnormalities that lead to hearing loss
  • eye abnormalities that cause nearsightedness (myopia), strabismus, and other problems with vision.