General/Misc Flashcards

1
Q

Treatment for ODD

A

Parent management as first line

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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.

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3
Q

Alternative to pencillamine for wilsons disease

A

Trietine

‘Treatin Wilsons with antibiotics ‘

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4
Q

Virus associated with long term complications of bronchiolitis obliterans

A

Adenovirus

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5
Q

Which drug Binds and inhibits tubulin- apart from Vincristine.

A

Albendazole

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6
Q

Common cause of haemorrhagic cystitis post stem cell transplant?

A

BK virus

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7
Q

ECG changes for hypokalemia?

A

Prolonged PR, flt T waves and ST depression. U waves.

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8
Q

ECG changes in hyPO vs hyPER calcemia?

A

HYPOcalcemia- prolonged qTC via longer ST segments.
HyPERcalcemia- short qTC and J waves.

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9
Q

3 common bugs that colonise babies with CF within first month of life

A

S. aureus , Haemophilus influenza , or even P. aeruginosa

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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

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11
Q

Common pulmonary complications of CF?

A

atelectasis, hemoptysis, pneumothorax, and cor pulmonale; these usually appear in late adolescence or beyond.

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12
Q

Is acute sinusitis common in CF?

A

No. Can get rhinorrhoea and nasal obstruction

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13
Q

What % of kids with CF have meconium ileus at birth

A

15-20%
1/5 th

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14
Q

Locus for Duschene gene

A

Xp21

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15
Q

Inheritance for Duschene

A

X linked

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16
Q

What does NOT improve mucosal inflammation in Crohn’s disease

A

Pred. Only stops further inflammmation

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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.

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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

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19
Q

Massive loss of hair with brushing/combing

A

Telogen effluvium
‘Flume and your hair is gone’

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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

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21
Q

What is traction alopecia

A

Hair loss from prolonged traction ie braids.

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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.

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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.

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24
Q

Which intracranial tumour commonly presents with cerebellar signs

A

Medulloblastoma

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25
Q

Which condition is strongly assocaited with giant cell astrocytoma

A

Tuberous sclerosis

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26
Q

Which condition is assocaited with optic glioma

A

NF1

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27
Q

unilateral microtia, unilateral facial muscle hypoplasia, and mandibular hypoplasia.

A

Goldenhar syndrome

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28
Q

These babies often have a normal cry, with severe inspiratory stridor

A

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

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29
Q

Most common cause of red vulva in prepubertal girls

A

Nonspecific vulvovaginitis is responsible for 25 to 75% of vulvovaginitis in prepubertal girls.

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30
Q

Which non-human creature is the largest carrier of salmonella?

A

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.

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31
Q

First line for neonatal candidiasis

A

Amphobterecin B

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32
Q

Sepsis and small salmon-pink papules in neonate

A

Listeria monocytogenes
You have CHEESE with SALMON so salmon coloured pink papules

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33
Q

A higher sweat chloride is indicative of worse prognosis for CF T or F

A

True.
Higher chloride in sweat assocaited with class 3 mutation which is worse

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34
Q

EEG changes similar to Panayiotopoulos syndrome. Unlike Panayiotopoulos syndrome, seizures occur during the day and EEG changes are activated by eye closure

A

Lennox- Gastaut syndrome

EEG: spike discharges occipital area.
Think- Kan-X = LennoX = sleep during the day and trigger seizures when eyes closed

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35
Q

A 5-year-old boy who has episodes where he is unresponsive, with reduced tone and vomiting at night. His EEG shows occipital spikes

A

Panayiotopoulus syndrome.
Different from Rolandic due to OCCIPITAL spikes

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36
Q

Long term risk of measles

A

Subacute panencephalitis - in one year
ADEM- 10 years

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37
Q

Homozygous GCK mutation

A

Neonatal diabetes

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38
Q

What syndrome gives you parotitis

A

Sjogrens

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39
Q

What is dermatitis herpetiformis?

A

Chronic skin conditions ASSOCIATED WITH COELIAC DISEASE

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40
Q

What medication is associated with pyloric stenosis

A

Erythromycin

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41
Q

Condition commonly associated with neonatal hepatic fibrosis

A

Autosomal recessive PKD

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42
Q

Most serious side effect of atomoxetine

A

Suicidal ideation

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43
Q

Most common early VISUAL finding for idiopathic cranial hypertension

A

reduced visaul acuity

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44
Q

Maternal uniparental disomy on chromosome 7

A

Russell Silver

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45
Q

Most common presenting feature of ARF

A

arthritis

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46
Q

Antiepileptic with highest risk of congenital malformations

A

Sodium valproate

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47
Q

Most common organism causing osteomyelitis in kid

A

Staph aureus

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48
Q

Treatment for neonatal conjunctivitis with chlamydia

A

Erythromycin

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49
Q

Treatment of infants in whom gonococcal ophthalmia

A

Ceftriaxone
Note neonates wouldn’t get cefotaxime because billiary sludge. Also cef causing calcium formation in the line.

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50
Q

WHich GLUT is in the brain? And hence malfn can cause problems ie seizures

A

GLUT1

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51
Q

First line for acute dystonic reaction from antipsychotics OR antiemetics

A

Benztropine
Second line= lorazepam

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52
Q

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.

A

Pulmonary haemosiderosus

Remember, goodpastures can cause haemoptysis but only if kidney affected

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53
Q

Tropical island, low platlets, fevers, body aches

A

Dengue

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54
Q

Length of tics to be diagnosed with Tourettes

A

12 months

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55
Q

Recurrnce risk of autism in sibling who also had autism

A

10%

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56
Q

IQ for mild ID
Remember: 20 year olds are profoundly dumb

A

50-70
Goes down by 15 points each tier. Profound <20
(think 20 year olds are profoundly dumb)

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57
Q

Risk of growth hormone in prader willi ?

A

OSA

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58
Q

Psychiatric condition commonly associated with tourettes

A

OCD

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59
Q

RIsk of sturge weber syndrome if unilateral port wine stain on one side of V1

A

7-8%

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60
Q

Risk of sturge weber if bilateral port wine stain over V1

A

100%

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61
Q

Where is the stain in Sturge weber and what do you need to worry about

A

V1 distribution
Worry about brain malformations etc. MRI at 12 months sensitive

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62
Q

What is the mutation for port wine stain (and hence Sturge Weber)

A

GNAQ mutation
‘You have a NAQ for port wine’

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63
Q

What are the two conditions caused by GNAS mutations and why?

A

McCune Alright and pseudopseuhohypoparathyroidism.
Due to imprinting.

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64
Q

What percentage of sturge weber have glaucoma

A

66%

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65
Q

Small size vasculitis

A

HSP
Anti C1q

ANCA
- Churg Strauss
- Wegener’s

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66
Q

Medium sized vasculitis

A

Kawasaki disease and polyarteritis nordosa

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67
Q

Large sized vasculitis

A

Giant cell arteritis
Takasayu

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68
Q

What is least useful for management of eczema

A

Allergen immunotherapy

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69
Q

If there is vasculitis, what is the most common form in SLE

A

Cutaneous vasculitis

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70
Q

Name the lesion: Mass anteriorly to sternocleidomastoid with fevers

A

Branchial cyst, usually presents when infected

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71
Q

Soft cheek mass with overlying bruit

A

haemangioma

72
Q

soft, painless masses that transilluminate. Rapid enlargement can occur with infection or haemorrhage.

A

Lymphatic malformation
Most common benign neck lesion

73
Q

Midline mass on the neck

A

Thyroglossal cyst

74
Q

Infections commonly associated with erythema multiforme

A

Precipitating factors include herpes simplex virus, Epstein-Barr virus, and histoplasmosis.

75
Q

Gene for DMD and Beckers (locus etc)

A

Dystrophine
Xp21

76
Q

What class of drugs is Imipramine

A

TCA
Works on both serotonin and norepinephrine….

77
Q

Uses for Imipramine

A

Depression AND adjunct to noctural eneurisis

78
Q

Main mechanisms of action for imipramine

A

Overarching TCA
Serotonin and norepinephrin reuptake inhibitor.
Most affinity to serotonin and so anticholinergic effects

79
Q

Which metabolic condition is a DDx for NAI

A

GLutaric acidemia. Causes brain bleeds and sweaty feet.

80
Q

How long after IVIG can you give live vaccinations vs after blood transfusion

A

11 months post Kwasaki
5-6 months otherwise

81
Q

When does IgM antibody peak for Measles

A

peak within first week

82
Q

Hepatitis, positive ANA/SMA antibodies

A

Type 1
Good prognosis
A= first alphabet, type1

83
Q

Hepatitis, positive anti-LKM

A

Type 2
later in the alphabet so type 2

84
Q

Difference between dyskeratosis congenita and hoyeraal hredidarrson syndrome

A

Dyskeratosis= shortening of telomeres (leukoplakia, nail dystrophy and tooth abnormalities) but hoyeral = the same but with cerebellar hypoplasia

85
Q

Which one is confined to suture lines?
Caput secundum vs cephalohaematoma vs subgaleal

A

Cephalohaematoma
Caput secundum= like chignon= post birth but superficial so across suture lines

86
Q

CHD associated with T1DM in mum?

A

TGA

(Have a lot of TriGlycerides in T1DM)

87
Q

Which metabolic condition = contraindicated for keto diet

A

MCAD!
Cant use ketones as fatty acid utilisation defect

88
Q

Bone tumour that looks like peeling onion

A

Ewing’s sarcoma

‘Ew bone cancer’

89
Q

Bone cancer with starburst sign

A

Alveolar rhabdomyosarcoma

90
Q

What is pulmonary alveolar proteinosis

A

Too much surfactant productin

91
Q

PU1 deficiency = what in the lungs

A

reduced ability to clear mess by alveolar macrophages

92
Q

Result of homozygous GCK mutation?

A

Neonatal diebetes.

‘Glucose c Kid mutation= neonatal diabetes’

93
Q

Interaction between Pyridostigmine and gentamycin

A

increased neuromuscular blockade

94
Q

Action/Reason to not use LABA as sole agent

A

Downregulation of B receptors (makes SABA ineffective)

95
Q

Antibiotic with increased risk of pyloric stenosis

A

erythromycin

96
Q

Infection most associated with viral myositis

A

Influenza

97
Q

What are anti histone antibodies a hallmark feature of

A

Drug induced lupus

98
Q

Cells responsible for response in protein conjugated vaccine

A

T cell

99
Q

Cells promoting cross linking response and production of IgM post polysaccaride vaccine

A

B cells
Ie in pneumonvax

100
Q

What is hungry bone syndrome?

A

Common post parathyroidectomy.
Magnesium moves into bone as result of increased bone formation. Also reduced phos and ca

101
Q

Top 3 reasons for Prader Willi syndrome (genetically)

A
  1. Deletion from paternally contirbuted portion of chromosome 15 (this is 65-75%)
  2. Maternal uniparental disomy
  3. Imprinting.
102
Q

Name 3 RASopathies

A
  1. NF1
  2. Noonan
  3. Costello
  4. Cardio-facial- cutaneous
  5. Legios
  6. LEOPARD
103
Q

In status, what mechanism= decreased efficacy with repeated use of benzos

A

Internalisation of GABA receptors

104
Q

What is Starling’s law of heart?

A

Volume of blood ejected by the ventricle depends on the volume present at the end of diastole

105
Q

How does morphine induce itching

A

Histamine release. Also causes hypotension and bronchospasm from same

106
Q

What hormone stimulates testosterone production in first half of pregnancy?

A

Placental HCG.
essentially acts on leydig cells. Peak at 8-12 weeks.
After that low testostrone, controlled by FOETAL pituitary

107
Q

Granulomatosis infanseptica, salmon pink papules and preterm labour

A

Listeria monocytigenes

108
Q

Contraindications to rotavirus

A

Underlying conditions: short gut, meckels, hirshsprungs
SCID
Prev anaphylaxis
Intussuception

109
Q

What is a marker for Rickets

A

ALP

110
Q

What IL does Anakinra act on?

A

IL-1

111
Q

What acts on Leukotrine

A

Montelukast

112
Q

Dinatuximab

A

Anti-GD2 for neuroblastoma

113
Q

Which variation of a tracheal ring is managed conservatively

A

Anomalous innominate causing few or no symptoms is managed conservatively.

114
Q

Which bugs are commonly resistant to ampicillin secondary to beta lactamase

A

E. coli, P. mirabilis, Salmonella, Shigella, H. influenzae

115
Q

ADHD risk if:
1x sibling affected
1x parent affected

A

sibling: 30%
parent: 57%!!!

Twins: 70-80%

116
Q

Is Coma a sign of serotonin intoxication

A

No

117
Q

Which two SSRIs cause dose dependant QT prolongation

A

Escitalopram, Citalopram

118
Q

Which antiepileptic causes gingival hyperplasia

A

Phenytoin

119
Q

Diagnosis?
Binge eating twice a week for 3 months

A

Bulimia nervosa

Altered perception of body image= anorexia nervosa

120
Q

Next line of treatment in Kawasaki if no improvement in symptoms after 2x IVIG

A

Give pulse IV methylpred for 2 days and screen for cardiovascular

121
Q

4 absolute contraindications to Infliximab

A
  1. Untreated infection
  2. Undrained abscess
  3. Severe congested cardiac failure
  4. Known hypersensitivity to murine protein
122
Q

What is a relative contraindication to Infliximab

A

Chronic Hep B carraige
Amoungst others

123
Q

Grimacing smile, broad and often angulated thumbs and halluces, short stature, and moderate-to-severe intellectual disability.

A

Rubinstein-Taybi syndrome (RSTS)

124
Q

Absent corpus callosum, central chorioretinal lacunae, and infantile spasms.
Syndrome and inheritance

A

Aicardi
No clear inheritance pattern

125
Q

Septal defect, cardiac conduction problems, absent thumbs.
Autosomal dominant

A

Holt-Oram

Sita is running, HOLTS because of her heart block and puts hand (without thumbs) on her forehead- o RAM.

126
Q

Most potent oncogenic agent in humans

A

HTLV1
(Human T lymphotropic virus)
Seen in adult T cell lymphoma.
Mums with this SHOULD NOT BREAST FEED.

127
Q

Genetic material shared between first, second and third degree relatives

A

First degree relatives share 50%

Second degree relatives share 25%

Third degree relatives (i.e. cousins) share 12.5%

128
Q

Which bug has no pleocytosis in CSF

A

Parvovirus

129
Q

Why is there an increased risk of MI in Williams

A

Narrowing of coronary arteries causing increased MI risk

130
Q

What do you use to measure/test for short stature in kids? (not neonates)

A

Measure IGF and IGF beta- not growth hormone because it fluctuates too much in kids with pulsatile secretion

131
Q

What disease does Echinococcus cuase

A

Hydatid disease
Livers

132
Q

Which bacteria is known to cause mucositis

A

Strep viridans

133
Q

What syndrome is associated with germ cell tumours?

A

Kleinfelters syndrome

134
Q

Which factors in coagualtion have the longest and shortest half life?

A

Factor VII – shortest
Factor XIII- longest

135
Q

Half life of VWF

A

10 hours

136
Q

Where should the UVC sit

A

T9

137
Q

Where does the UAC sit

A

T6-T9

138
Q

What is a marker of vWF activity/function?

A

Ristocretin

139
Q

What are the subtypes for eating disorders? Hint 3 main and then sub categories

A

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
Q

Difference in personality types for Anorexia Nervosa vs Bulimia Nervosa?

A

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
Q

First and second most common cause of death in anorexia?

A

Suicide = first
Tachycarrthmia= second

142
Q

Which eating disorder benefits from SSRI

A

Bulimia.
Food= best treatment for depression in AN

143
Q

Which treatment is the ONLY evidence based therapy in AN

A

Family based therapy

144
Q

Which treatment is appropriate for Bulimia Nervosa

A

DBT or CBT

145
Q

What % of AN patients recover (with appropriate treatment and therapy)

A

80%
Less data for BN

146
Q

Where is the main problem in porphyria

A

Problems in enzymes involved in heme metabolism pathway

147
Q

Most common porphyria in childhood/paediatrics?

A

Erythropoietic protoporphyria

148
Q

4 signs or clinical manifestations of acute intermittent porphyria

A

4 Ps
Pain (abdominal)
Peripheral neuropathy
Psychosis
Pee - red when exposed to light

149
Q

inheritance for acute intermittent porphyria

A

Aut Dominant

150
Q

Typical presentation for congenital erythrypoietic porphyria

A

Red stained urine immediately after birth and then significant photosensitivity (which can be manifested by phototherapy)

151
Q

What is the name of obsessions and delusions occurring post strep infection?

A

PANDAS
Can be trigger OR excacerbator in 10% of kids due to swelling of basal ganglia. Controversial ++

152
Q

Mechanism of resistance to penicillin from strep pneumo?

A

Generation of penicillin binding proteins.

153
Q

Mechanism of resistance for enterobacter to cephalosporins

A

Chromosomal induction of beta lactamases

154
Q

Mechanism of CF related diabetes

A

Reduced insulin production.
Essentially BLOCKED pancreatic secretions causing self-digestion and reduced beta cell numbers causing reduced insulin production.

155
Q

Treatment for Diamond-Blackfan anaemia

A

Corticosteroids, blood transfusions, stem cell transplant

156
Q

Treatment for Fanconi Anaemia

A

Androgen, blood transfusion, stem cell transplant

157
Q

What is the formula for minute volume

A

Tidal volume x RR

158
Q

Rupture or damage to what veins can cause subdural haematoma and what is the shape of a subdural

A

Cortical bridging veins cause subdural.
normal

159
Q

% of kids transitioning to ALL from transient myeloproliferative disorder in Downs

A

25%

160
Q

Compared to normal, which leukemia has better survival in kids with Downs? (AML vs ALL)

A

AML
>80% survival

161
Q

What is GATA 1

A

a transcription factor that controls megakaryopoiesis. Involved in ALL and transient myeloproliferative disorder for Downs

162
Q

Which electrolyte is not absorbed maximally in the proximal tubule

A

Magnesium! Mostly in loop of Henle

163
Q

What type of astrocytoma is Tuberous sclerosis associated with

A

Giant cell astrocytoma

164
Q

Pathway in pilocytic astrocytoma

A

RAS-MAPK , involves BRAF mtuations

165
Q

% of MDD kids that attempt suicide

A

30%. (60% think about it)

166
Q

What carries oxygenated vs deoxygenated blood in the foetal circulation

A

Umbilical VEIN carries oxygenated blood and combined with SBC+IVC into Right atrium via liver.
Umbilical ARTERY carries deoxygenated blood back to placenta.

167
Q

Two markers which are SENSITIVE for hereditary angioedema

A

Low c1 inhibitor AND low C4
Both act in the classical pathway

168
Q

Should you give Naloxone to kids at risk of withdrawal

A

NO
Can cause withdrawal symptoms including seizures

169
Q

What time period defines DELAYED cord separation?

A

21 days

170
Q

Is PJP a fungus or virus

A

Fungus

171
Q

Where is the majority of iron absorbed?

A

Duodenum

172
Q

What cardiological condition is imp to think about for brain abscess?

A

Tetralogy of Fallot
Most brain abscesses will have underlying issue.

173
Q

RItuximab acts on CD20, what type of B cell is that?

A

Memory B cell and immature B cells.
PLASMA is CD 19- expressed just after plasmoblast stage.

174
Q

How does Diazoxide work?

A

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
Q

Erbs Palsy with Horners, what level

A

T1

176
Q

Difference between Erbs and Klumpke’s?

A

Erbs- rotated out, C5-C6
Klumpkes claw- C8+T1

177
Q

sodium valproate with lamotrigine increases the risk of associated adverse drug reactions via….

A

Competitive enzyme inhibition