General/Misc Flashcards
1
Q
Treatment for ODD
A
Parent management as first line
2
Q
Common difference between erythema multiforme and urticaria multiforme
A
Urticaria will have dermatgraphism and also facial swelling.
Urticaria multiforme= benign subtype of acute urticaria and different also from urticarial vasculitis.
Also note, out of urticaria multiforma, erythema multiforme (serum sickness like) and urticarial vasculitis- only this form ahs dermatographism.
3
Q
Alternative to pencillamine for wilsons disease
A
Trietine
‘Treatin Wilsons with antibiotics ‘
4
Q
Virus associated with long term complications of bronchiolitis obliterans
A
Adenovirus
5
Q
Which drug Binds and inhibits tubulin- apart from Vincristine.
A
Albendazole
6
Q
Common cause of haemorrhagic cystitis post stem cell transplant?
A
BK virus
7
Q
ECG changes for hypokalemia?
A
Prolonged PR, flt T waves and ST depression. U waves.
8
Q
ECG changes in hyPO vs hyPER calcemia?
A
HYPOcalcemia- prolonged qTC via longer ST segments.
HyPERcalcemia- short qTC and J waves.
9
Q
3 common bugs that colonise babies with CF within first month of life
A
S. aureus , Haemophilus influenza , or even P. aeruginosa
10
Q
Common imaging findings on first birthday for kids with CF
A
heterogeneous air trapping in of infants by their first birthday, and bronchiectasis is found in more than 10% of 1 yr olds and ∼60% of 5 yr olds.
So essentially hyperinflation
11
Q
Common pulmonary complications of CF?
A
atelectasis, hemoptysis, pneumothorax, and cor pulmonale; these usually appear in late adolescence or beyond.
12
Q
Is acute sinusitis common in CF?
A
No. Can get rhinorrhoea and nasal obstruction
13
Q
What % of kids with CF have meconium ileus at birth
A
15-20%
1/5 th
14
Q
Locus for Duschene gene
A
Xp21
15
Q
Inheritance for Duschene
A
X linked
16
Q
What does NOT improve mucosal inflammation in Crohn’s disease
A
Pred. Only stops further inflammmation
17
Q
Patchy hair loss over eyebrow, scalp and eyelashes
A
Alopecia areta (can be partial or complete.)
Autoimmune T cell driven process. Skin also weird.
18
Q
Hair pulling –> irregular and patchy loss of hair in occipital and parietal regions
A
Trichotillomania.
Will grow back eventually. Can be associated with OCD
19
Q
Massive loss of hair with brushing/combing
A
Telogen effluvium
‘Flume and your hair is gone’
20
Q
Causes of Telogen Effluvium
‘ Flume and your hair is gone’
A
Fever, pregnancy, infection, hypo/hyperthyroidism, blood donation etc tc.
Large chunks of your hair gone
21
Q
What is traction alopecia
A
Hair loss from prolonged traction ie braids.
22
Q
What is Fabry disease and inheritance?
A
X linked and overall lysosomal storage disorder.
No enzyme (GLA) to metabolise fats leading to build up.
23
Q
Classic symptoms of Fabry’s disease and complications
A
Burning hands and feet, angiokeratomas and reduced sweating. BECUASE ISSUES WITH FAT DEPOSITION
Complications: kidney disease, heart enlargement, stroke, GI upset etc.
24
Q
Which intracranial tumour commonly presents with cerebellar signs
A
Medulloblastoma
25
Which condition is strongly assocaited with giant cell astrocytoma
Tuberous sclerosis
26
Which condition is assocaited with optic glioma
NF1
27
unilateral microtia, unilateral facial muscle hypoplasia, and mandibular hypoplasia.
Goldenhar syndrome
28
These babies often have a normal cry, with severe inspiratory stridor
Bilateral vocal cord paralysis
Bilateral vocal cord paralysis produces airway obstruction manifested by respiratory distress and high-pitched inspiratory stridor, aphonatory or dysphonic sound, or inspiratory weak cry. Unilateral have less stridor symptoms
29
Most common cause of red vulva in prepubertal girls
Nonspecific vulvovaginitis is responsible for 25 to 75% of vulvovaginitis in prepubertal girls.
30
Which non-human creature is the largest carrier of salmonella?
Reptiles and amphibians are known carriers of salmonella sp, with many species having a >90% carriage rate. There is an estimated 3% increased risk of salmonella infection if living with a reptile. Salmonella may also be transmitted by birds, dogs and cats, and farm animals.
31
First line for neonatal candidiasis
Amphobterecin B
32
Sepsis and small salmon-pink papules in neonate
Listeria monocytogenes
You have CHEESE with SALMON so salmon coloured pink papules
33
A higher sweat chloride is indicative of worse prognosis for CF T or F
True.
Higher chloride in sweat assocaited with class 3 mutation which is worse
34
EEG changes similar to Panayiotopoulos syndrome. Unlike Panayiotopoulos syndrome, seizures occur during the day and EEG changes are activated by eye closure
Lennox- Gastaut syndrome
EEG: spike discharges occipital area.
Think- Kan-X = LennoX = sleep during the day and trigger seizures when eyes closed
35
A 5-year-old boy who has episodes where he is unresponsive, with reduced tone and vomiting at night. His EEG shows occipital spikes
Panayiotopoulus syndrome.
Different from Rolandic due to OCCIPITAL spikes
36
Long term risk of measles
Subacute panencephalitis - in one year
ADEM- 10 years
37
Homozygous GCK mutation
Neonatal diabetes
38
What syndrome gives you parotitis
Sjogrens
39
What is dermatitis herpetiformis?
Chronic skin conditions ASSOCIATED WITH COELIAC DISEASE
40
What medication is associated with pyloric stenosis
Erythromycin
41
Condition commonly associated with neonatal hepatic fibrosis
Autosomal recessive PKD
42
Most serious side effect of atomoxetine
Suicidal ideation
43
Most common early VISUAL finding for idiopathic cranial hypertension
reduced visaul acuity
44
Maternal uniparental disomy on chromosome 7
Russell Silver
45
Most common presenting feature of ARF
arthritis
46
Antiepileptic with highest risk of congenital malformations
Sodium valproate
47
Most common organism causing osteomyelitis in kid
Staph aureus
48
Treatment for neonatal conjunctivitis with chlamydia
Erythromycin
49
Treatment of infants in whom gonococcal ophthalmia
Ceftriaxone
Note neonates wouldn't get cefotaxime because billiary sludge. Also cef causing calcium formation in the line.
50
WHich GLUT is in the brain? And hence malfn can cause problems ie seizures
GLUT1
51
First line for acute dystonic reaction from antipsychotics OR antiemetics
Benztropine
Second line= lorazepam
52
A 7-year-old girl presents with a 4-week history of cough, shortness of breath on exertion and recent haemoptysis. Over the last 12 months she has been treated for persistent iron deficiency anaemia. She has normal renal function. A chest x-ray demonstrates diffuse alveolar opacity.
Pulmonary haemosiderosus
Remember, goodpastures can cause haemoptysis but only if kidney affected
53
Tropical island, low platlets, fevers, body aches
Dengue
54
Length of tics to be diagnosed with Tourettes
12 months
55
Recurrnce risk of autism in sibling who also had autism
10%
56
IQ for mild ID
Remember: 20 year olds are profoundly dumb
50-70
Goes down by 15 points each tier. Profound <20
(think 20 year olds are profoundly dumb)
57
Risk of growth hormone in prader willi ?
OSA
58
Psychiatric condition commonly associated with tourettes
OCD
59
RIsk of sturge weber syndrome if unilateral port wine stain on one side of V1
7-8%
60
Risk of sturge weber if bilateral port wine stain over V1
100%
61
Where is the stain in Sturge weber and what do you need to worry about
V1 distribution
Worry about brain malformations etc. MRI at 12 months sensitive
62
What is the mutation for port wine stain (and hence Sturge Weber)
GNAQ mutation
'You have a NAQ for port wine'
63
What are the two conditions caused by GNAS mutations and why?
McCune Alright and pseudopseuhohypoparathyroidism.
Due to imprinting.
64
What percentage of sturge weber have glaucoma
66%
65
Small size vasculitis
HSP
Anti C1q
ANCA
- Churg Strauss
- Wegener's
66
Medium sized vasculitis
Kawasaki disease and polyarteritis nordosa
67
Large sized vasculitis
Giant cell arteritis
Takasayu
68
What is least useful for management of eczema
Allergen immunotherapy
69
If there is vasculitis, what is the most common form in SLE
Cutaneous vasculitis
70
Name the lesion: Mass anteriorly to sternocleidomastoid with fevers
Branchial cyst, usually presents when infected
71
Soft cheek mass with overlying bruit
haemangioma
72
soft, painless masses that transilluminate. Rapid enlargement can occur with infection or haemorrhage.
Lymphatic malformation
Most common benign neck lesion
73
Midline mass on the neck
Thyroglossal cyst
74
Infections commonly associated with erythema multiforme
Precipitating factors include herpes simplex virus, Epstein-Barr virus, and histoplasmosis.
75
Gene for DMD and Beckers (locus etc)
Dystrophine
Xp21
76
What class of drugs is Imipramine
TCA
Works on both serotonin and norepinephrine....
77
Uses for Imipramine
Depression AND adjunct to noctural eneurisis
78
Main mechanisms of action for imipramine
Overarching TCA
Serotonin and norepinephrin reuptake inhibitor.
Most affinity to serotonin and so anticholinergic effects
79
Which metabolic condition is a DDx for NAI
GLutaric acidemia. Causes brain bleeds and sweaty feet.
80
How long after IVIG can you give live vaccinations vs after blood transfusion
11 months post Kwasaki
5-6 months otherwise
81
When does IgM antibody peak for Measles
peak within first week
82
Hepatitis, positive ANA/SMA antibodies
Type 1
Good prognosis
A= first alphabet, type1
83
Hepatitis, positive anti-LKM
Type 2
later in the alphabet so type 2
84
Difference between dyskeratosis congenita and hoyeraal hredidarrson syndrome
Dyskeratosis= shortening of telomeres (leukoplakia, nail dystrophy and tooth abnormalities) but hoyeral = the same but with cerebellar hypoplasia
85
Which one is confined to suture lines?
Caput secundum vs cephalohaematoma vs subgaleal
Cephalohaematoma
Caput secundum= like chignon= post birth but superficial so across suture lines
86
CHD associated with T1DM in mum?
TGA
(Have a lot of TriGlycerides in T1DM)
87
Which metabolic condition = contraindicated for keto diet
MCAD!
Cant use ketones as fatty acid utilisation defect
88
Bone tumour that looks like peeling onion
Ewing's sarcoma
'Ew bone cancer'
89
Bone cancer with starburst sign
Alveolar rhabdomyosarcoma
90
What is pulmonary alveolar proteinosis
Too much surfactant productin
91
PU1 deficiency = what in the lungs
reduced ability to clear mess by alveolar macrophages
92
Result of homozygous GCK mutation?
Neonatal diebetes.
'Glucose c Kid mutation= neonatal diabetes'
93
Interaction between Pyridostigmine and gentamycin
increased neuromuscular blockade
94
Action/Reason to not use LABA as sole agent
Downregulation of B receptors (makes SABA ineffective)
95
Antibiotic with increased risk of pyloric stenosis
erythromycin
96
Infection most associated with viral myositis
Influenza
97
What are anti histone antibodies a hallmark feature of
Drug induced lupus
98
Cells responsible for response in protein conjugated vaccine
T cell
99
Cells promoting cross linking response and production of IgM post polysaccaride vaccine
B cells
Ie in pneumonvax
100
What is hungry bone syndrome?
Common post parathyroidectomy.
Magnesium moves into bone as result of increased bone formation. Also reduced phos and ca
101
Top 3 reasons for Prader Willi syndrome (genetically)
1. Deletion from paternally contirbuted portion of chromosome 15 (this is 65-75%)
2. Maternal uniparental disomy
3. Imprinting.
102
Name 3 RASopathies
1. NF1
2. Noonan
3. Costello
4. Cardio-facial- cutaneous
5. Legios
5. LEOPARD
103
In status, what mechanism= decreased efficacy with repeated use of benzos
Internalisation of GABA receptors
104
What is Starling's law of heart?
Volume of blood ejected by the ventricle depends on the volume present at the end of diastole
105
How does morphine induce itching
Histamine release. Also causes hypotension and bronchospasm from same
106
What hormone stimulates testosterone production in first half of pregnancy?
Placental HCG.
essentially acts on leydig cells. Peak at 8-12 weeks.
After that low testostrone, controlled by FOETAL pituitary
107
Granulomatosis infanseptica, salmon pink papules and preterm labour
Listeria monocytigenes
108
Contraindications to rotavirus
Underlying conditions: short gut, meckels, hirshsprungs
SCID
Prev anaphylaxis
Intussuception
109
What is a marker for Rickets
ALP
110
What IL does Anakinra act on?
IL-1
111
What acts on Leukotrine
Montelukast
112
Dinatuximab
Anti-GD2 for neuroblastoma
113
Which variation of a tracheal ring is managed conservatively
Anomalous innominate causing few or no symptoms is managed conservatively.
114
Which bugs are commonly resistant to ampicillin secondary to beta lactamase
E. coli, P. mirabilis, Salmonella, Shigella, H. influenzae
115
ADHD risk if:
1x sibling affected
1x parent affected
sibling: 30%
parent: 57%!!!
Twins: 70-80%
116
Is Coma a sign of serotonin intoxication
No
117
Which two SSRIs cause dose dependant QT prolongation
Escitalopram, Citalopram
118
Which antiepileptic causes gingival hyperplasia
Phenytoin
119
Diagnosis?
Binge eating twice a week for 3 months
Bulimia nervosa
Altered perception of body image= anorexia nervosa
120
Next line of treatment in Kawasaki if no improvement in symptoms after 2x IVIG
Give pulse IV methylpred for 2 days and screen for cardiovascular
121
4 absolute contraindications to Infliximab
1. Untreated infection
2. Undrained abscess
3. Severe congested cardiac failure
4. Known hypersensitivity to murine protein
122
What is a relative contraindication to Infliximab
Chronic Hep B carraige
Amoungst others
123
Grimacing smile, broad and often angulated thumbs and halluces, short stature, and moderate-to-severe intellectual disability.
Rubinstein-Taybi syndrome (RSTS)
124
Absent corpus callosum, central chorioretinal lacunae, and infantile spasms.
Syndrome and inheritance
Aicardi
No clear inheritance pattern
125
Septal defect, cardiac conduction problems, absent thumbs.
Autosomal dominant
Holt-Oram
Sita is running, HOLTS because of her heart block and puts hand (without thumbs) on her forehead- o RAM.
126
Most potent oncogenic agent in humans
HTLV1
(Human T lymphotropic virus)
Seen in adult T cell lymphoma.
Mums with this SHOULD NOT BREAST FEED.
127
Genetic material shared between first, second and third degree relatives
First degree relatives share 50%
Second degree relatives share 25%
Third degree relatives (i.e. cousins) share 12.5%
128
Which bug has no pleocytosis in CSF
Parvovirus
129
Why is there an increased risk of MI in Williams
Narrowing of coronary arteries causing increased MI risk
130
What do you use to measure/test for short stature in kids? (not neonates)
Measure IGF and IGF beta- not growth hormone because it fluctuates too much in kids with pulsatile secretion
131
What disease does Echinococcus cuase
Hydatid disease
Livers
132
Which bacteria is known to cause mucositis
Strep viridans
133
What syndrome is associated with germ cell tumours?
Kleinfelters syndrome
134
Which factors in coagualtion have the longest and shortest half life?
Factor VII -- shortest
Factor XIII- longest
135
Half life of VWF
10 hours
136
Where should the UVC sit
T9
137
Where does the UAC sit
T6-T9
138
What is a marker of vWF activity/function?
Ristocretin
139
What are the subtypes for eating disorders? Hint 3 main and then sub categories
Anorexia Nervosa
- restrictive eating and bine eating subtype
- WEIGHT LOSS
Bulimia Nervosa
- binge eating AND measures not to gain weight ie laxatives, purging etc. PREVENTION OF WEIGHT GAIN.
Atypical AN or ARFID
- not eating based on specific textures or foods
140
Difference in personality types for Anorexia Nervosa vs Bulimia Nervosa?
AN: like me.
above-average intelligence and socioeconomic status who is a conflict-avoidant, risk-aversive perfectionist and is struggling with disturbances of anxiety and/or mood
BN:
impulsivity and features of borderline personality disorder associated with depression and mood swings.
141
First and second most common cause of death in anorexia?
Suicide = first
Tachycarrthmia= second
142
Which eating disorder benefits from SSRI
Bulimia.
Food= best treatment for depression in AN
143
Which treatment is the ONLY evidence based therapy in AN
Family based therapy
144
Which treatment is appropriate for Bulimia Nervosa
DBT or CBT
145
What % of AN patients recover (with appropriate treatment and therapy)
80%
Less data for BN
146
Where is the main problem in porphyria
Problems in enzymes involved in heme metabolism pathway
147
Most common porphyria in childhood/paediatrics?
Erythropoietic protoporphyria
148
4 signs or clinical manifestations of acute intermittent porphyria
4 Ps
Pain (abdominal)
Peripheral neuropathy
Psychosis
Pee - red when exposed to light
149
inheritance for acute intermittent porphyria
Aut Dominant
150
Typical presentation for congenital erythrypoietic porphyria
Red stained urine immediately after birth and then significant photosensitivity (which can be manifested by phototherapy)
151
What is the name of obsessions and delusions occurring post strep infection?
PANDAS
Can be trigger OR excacerbator in 10% of kids due to swelling of basal ganglia. Controversial ++
152
Mechanism of resistance to penicillin from strep pneumo?
Generation of penicillin binding proteins.
153
Mechanism of resistance for enterobacter to cephalosporins
Chromosomal induction of beta lactamases
154
Mechanism of CF related diabetes
Reduced insulin production.
Essentially BLOCKED pancreatic secretions causing self-digestion and reduced beta cell numbers causing reduced insulin production.
155
Treatment for Diamond-Blackfan anaemia
Corticosteroids, blood transfusions, stem cell transplant
156
Treatment for Fanconi Anaemia
Androgen, blood transfusion, stem cell transplant
157
What is the formula for minute volume
Tidal volume x RR
158
Rupture or damage to what veins can cause subdural haematoma and what is the shape of a subdural
Cortical bridging veins cause subdural.
normal
159
% of kids transitioning to ALL from transient myeloproliferative disorder in Downs
25%
160
Compared to normal, which leukemia has better survival in kids with Downs? (AML vs ALL)
AML
>80% survival
161
What is GATA 1
a transcription factor that controls megakaryopoiesis. Involved in ALL and transient myeloproliferative disorder for Downs
162
Which electrolyte is not absorbed maximally in the proximal tubule
Magnesium! Mostly in loop of Henle
163
What type of astrocytoma is Tuberous sclerosis associated with
Giant cell astrocytoma
164
Pathway in pilocytic astrocytoma
RAS-MAPK , involves BRAF mtuations
165
% of MDD kids that attempt suicide
30%. (60% think about it)
166
What carries oxygenated vs deoxygenated blood in the foetal circulation
Umbilical VEIN carries oxygenated blood and combined with SBC+IVC into Right atrium via liver.
Umbilical ARTERY carries deoxygenated blood back to placenta.
167
Two markers which are SENSITIVE for hereditary angioedema
Low c1 inhibitor AND low C4
Both act in the classical pathway
168
Should you give Naloxone to kids at risk of withdrawal
NO
Can cause withdrawal symptoms including seizures
169
What time period defines DELAYED cord separation?
21 days
170
Is PJP a fungus or virus
Fungus
171
Where is the majority of iron absorbed?
Duodenum
172
What cardiological condition is imp to think about for brain abscess?
Tetralogy of Fallot
Most brain abscesses will have underlying issue.
173
RItuximab acts on CD20, what type of B cell is that?
Memory B cell and immature B cells.
PLASMA is CD 19- expressed just after plasmoblast stage.
174
How does Diazoxide work?
Stimulation of the potassium ATP channel.
Causes hyperpolarisation via K channels and inhibition of calcium entering the cell leading to inhibition of ca release
175
Erbs Palsy with Horners, what level
T1
176
Difference between Erbs and Klumpke's?
Erbs- rotated out, C5-C6
Klumpkes claw- C8+T1
177
sodium valproate with lamotrigine increases the risk of associated adverse drug reactions via....
Competitive enzyme inhibition
