2017 remembered Flashcards
(85 cards)
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
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
3
Q
Baby has hepatosplenomegaly, IUGR, interstitial pneumonitis, jaundice, and petechiae
Toxoplasmosis CMV Rubella Syphilis Varicella Parvovirus
A
CMV
4
Q
Baby has hydrocephalus, intracranial calcification, chorioretinitis
Toxoplasmosis CMV Rubella Syphilis Varicella Parvovirus
A
Toxoplasmosis
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
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
7
Q
- 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
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
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
10
Q
- What enzyme is dysfunctional for methemoglobinemia?
A
A. Cytochrome b5 reductase
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
12
Q
- What is an exome?
A. Formed by intron and exon
B. Formed by exons
C. Protein coding exons
A
B ?C
13
Q
- Why is the gentamicin dose lengthened in neonates?
A
A. Decreased renal elimination
14
Q
- What is the possible serious adverse effect of surfactant?
A. Pneumothorax
B. Necrotising enterocolitis
C. Pulmonary haemorrhage
A
C. Pulmonary haemorrhage
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
16
Q
- 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
17
Q
- What is the main determinant to help with neonatal ventilation?
A. Tidal volume
B. Minute ventilation
C. Resistance
A
B. Minute ventilation
18
Q
- 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
19
Q
- ADHD associated with
A. ODD
B. Mood disorder
A
A. ODD
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
21
Q
20. Which complement is associated with SLE? A. C1q B. C3 C. C4 D. Factor H E. Properdin
A
A. C1q
deficiency
22
Q
- Which organism is associated with complement deficiency?
A. Encapsulated organism
B. Gram negative cocci
A
B. Gram negative cocci
Neisseria
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
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
25
```
24. Where is erythropoietin derived from?
A. DCT
B. PCT
C. Collecting tubule
D. Interstitial cells
```
D. Interstitial cells
26
```
25. Where does the DDAVP act?
A. PCT
B. DCT
C. Ascending limb of Loop
D. Collecting tubule
E. Descending limb of Loop
```
D. Collecting tubule
retain H2O
27
28. What does the p value mean?
A. The chance of something happening
B. Size effect
A. The chance of something happening
28
29. 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. Bartonella Henselae
Cat scratch fever --> OM
K. kingae - common cause of OM
29
30. Picture of child with enlarged, red, unilateral lymph node swelling
A. Mycobacterium avium intracellulare
B. Staph aureus
A. Mycobacterium avium intracellulare
30
31. Patient had splenectomy and has received meningococcal vaccine and pneumovax. Which other vaccines should he have?
A. Influenza
B. Pneumococcal polysaccharide
C. Hemophilus influenza
C. Hemophilus influenza
pneumococcal, mening, HiB
31
```
32. What medication to give in narcolepsy?
A. Dopamine
B. Serotonin
C. Orexin
D. Cortisol
```
B. Serotonin
stimulants
SSRIs/SnRIs
TCA
32
```
33. Which pituitary hormone is destroyed in cranial radiation?
A. ADH
B. GH
C. FSH
D. LH
E. TSH
```
B. GH
- most common
then gonadrotropins, ACTH, TSH
33
```
34. A girl with 11 beta OH deficiency will have
A. Salt wasting
B. Hypertension and virilisation
C. Hirsutism
D. Hypotension
```
B. Hypertension and virilisation
34
35. Difference genetically between DMD and BMD
A. Intergenic deletion
B. Frameshift deletion
B. Frameshift deletion
35
36. Selective mutism is associated with
A. Anxiety
B. Depression
A. Anxiety
36
37. Which is associated with acanthosis nigricans?
A. Increased ACTH
B. Increased insulin
C. Increased GH
B. Increased insulin
37
```
38. What is the composition of 1g of fat?
A. 4 cal
B. 9 cal
C. 10 cal
D. 25 cal
E. 32 cal
```
B. 9 cal
4 cal = carb, protein
38
40. What causes DRESS?
A. Antipsychotic
B. Antiepileptic
C. Antibiotic
B. Antiepileptic
39
42. Which condition has the best evidence for microbiota transplant?
A. C diff
B. Celiac
C. IBD
A. C diff
40
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. 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
10. Difference in DMD versus BMD
CK
| Frameshift deletion
42
12. Which gene caused APACED?
A. AIRE
43
27. 16 year old patient becomes jaundice virus with unconjugated hyperbilirubinemia.
A. Gilbert’s
44
```
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. Arthritis
45
29. Patient has anti TTG positive and has a rash. What is it most likely associated with?
A. Dermatitis herpertiformis
46
17. Borderline personality associated with?
A. Mood disorder
B. Substance abuse
?A. Mood disorder
47
```
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
```
Differences between all:
48
2. 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. Reassure and discharge home
49
3. 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
C. Duodenal haematoma
50
4. CT difference between:
A. Extradural
B. SAH
C. Subdural
D. IVH
differences:
51
5. Patient with tardive dyskinesia. What to do?
A. Lower the medication dose
B. Change to a different antipsychotic
B. Change to a different antipsychotic
52
6. 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.. Serotonin syndrome
53
```
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
```
?B. Give MMR and withhold influenza
54
8. What is the predictor for renal function in Familial Mediterranean Fever?
A. CK
B. Renal US
C. Amyloidosis
C. Amyloidosis
55
9. Best measure of NASH
A. ALT
B. Albumin
C. Coag
A. ALT
56
13. What is the glargine activity?
peal at two hrs, then plateaus for 20-24 hr
57
```
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
?B. Hereditary spherocytosis
58
```
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
```
B. Piperacillin tazobactam
59
16. 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. Dysfunctional voiding
60
17. 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. Difficulty interacting with others
61
18. 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
?B. Congenital disorder of glycosylation
62
19. 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. Li fraumeni
63
20. What is the best use of 17 hydroxyprogesterone test
A. Virilised girl with salt wasting
B. Virilised girl with no salt wasting
A. Virilised girl with salt wasting
64
21. Patient with maculopapular rash with something….
| X linked lymphoproferative disorder x2
X linked lymphoproferative disorder x2
65
```
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
```
E. Left temporal
66
23. Child awakes with morning stiffness which improves with movement.
A. Arthritis
B. IBD
A. Arthritis
67
24. Boy with multiple joint pains and …..
A. Polyarteritis Nodosa
B. ?IBD
A. ?Polyarteritis Nodosa
68
26. 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. Extravasation
complications:
69
Types of Mody
Types:
70
```
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
```
?Denys drash
71
32. 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. Contact the surgeon for an orchidopexy
72
33. Something to do with ?cerebral salt wasting – low sodium, low potassium.... something head surgery or something….
?SIADH
73
34. Which is associated with disrupted circadian rhythm?
A. Noonan
B. Trisomy 21
B. Trisomy 21
74
```
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
B. Nephrogenic DI
75
```
36. Child with murmur LLSE, softer when sitting up, what is this?
A. ASD
B. TOF
C. Vibratory murmur
D. Venous hum
```
C. Vibratory murmur
76
37. Lithium and teratogenic effects?
A. Ebstein’s anomaly
77
92.
pH 7.35
CO2 63
HCO3 34
Chronic neonatal lung disease
Hyperventilation
Salicylate poisoning
CLD
78
93.
pH 7.4
CO2 32
HCO3 26
Chronic neonatal lung disease
Hyperventilation
Salicylate poisoning
resp alkylosis = salicylate poisoning
79
```
94.
pH 7.1
CO2 35
HCO3 16
High anion gap
```
Urea cycle defect
Ketotic hypoglycaemia
Aminoaciduria
Organic aciduria
?urea cycle
| organic aciduria
80
95.
Ketones +ve
Insulin normal
Low BSL
Urea cycle defect
Ketotic hypoglycaemia
Aminoaciduria
Organic aciduria
ketotic hypoglycaemia
81
96. Rash which is face sparring with MRI meningoencephalitis
Paraechovirus
Neiserria gonorrhoea
Chlamydia
paraecho
82
98. D10 baby with bilateral watery conjunctivitis, – bilat conj
Paraechovirus
Neiserria gonorrhoea
Chlamydia
chlamydia
83
```
99. Day 1 - Long systolic mumur LUSE with ECG - LAD and RAH with peak P wave…. ?
tricuspid atresia
TOF
Tricuspid atresia
AVSD
TGA
```
TA
84
100. 6mo, cyanosis,
TOF
Tricuspid atresia
AVSD
TGA
?AVSD
85
Smith Magenis Syndrome
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.
