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RACP Gen Paeds > Gen Paeds > Flashcards

Gen Paeds Flashcards

1
Q

You can be diagnosed with intellectual disability and a specific learning disability? (T/F)

A

False

Criterion for an SLD is to NOT have ID - i.e. IQ<70

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2
Q

You can be diagnosed with autism and a specific learning disability? (T/F)

A

True - SLD can co-exist with autism or ADHD, as long as IQ >70

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3
Q

What age range is the WPPSI appropriate for?

A

Wechsler preschool and primary scale of intelligence

2.5 - 7 years 7 month

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4
Q

Appropriate age range for the WISC?

A

Weschler Intelligence Scale for Children
Age 6 yr to 16 yr

$500-$1000 for psychometric testing?

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5
Q

Wechsler Individual Achievement Test

Whats it for?

A

WIAT
Assess academic capabilities between age 4-85
In conjunction with full scale IQ testing can differentiate domains of difficulty - can use to diagnose Specific Learning Difficulty

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6
Q

Give examples of a specific learning disability?

A

Dyslexia
Dyspraxia
Dyscalculia
Dysgraphia
Disblity with working memory/executive funcitoning
Contested: Central auditory processing, sensory processing disorder (more just labels for symptoms that co-exist in other disorders, they instead need speech, language and psychometric assessments)

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7
Q

Describe the difference between disability and difficulty as it pertains to learning

A

Difficulty - under achieve for wide range of reasons (sensory/behavior/absent/emotional/SES)

Disability- Unexpected (per other domains) and persistent (despite interventions) difficulties in specific areas as result of underlying neuro developmental disorder

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8
Q

A child presents as “clumsy” and you suspect Developmental Co-ordination Disorder - What DDx, What O/E to perform?

A

Dx via physio assessments - formally Griffiths now Peabody or Bruinisk/Odertesky

DDx
Anterior horn Cell - SMA
Peripheral nerves -CMT
Neuromuscular junction - Juvenile myasthenia
Muscle - DMD, Congenital myopathy, Congenital muscular dystrophy, Myotonic dystrophy

Reflexes
Gowers

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9
Q

Perthes disease

Gender preference?

Age of pres?

Mx?

A

Boys 5:1

Age 4-9

Rest, analgesia, rarely femoral osteotomy, older kids GAMP to keep epiphysis within acetabulum

X-ray flattened femoral head
Bone scane/MRI for early changes

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10
Q

Rheumatic Fever - Joint Pain pattern?

A 
Migratory - 1-2/7 per joint
Knee and Ankle joints - asymmetric
Intense Pain out of keeping with expectation (As compared to oJIA for example)
Responds well with NSAIDS
Lasts less than 3/52
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11
Q

Rotavirus - Risk of Intuscception from vaccine?
Faecal lymphocytes?
Proteins involved

A

1:20,000 - 100,000
NO - Think bacterial if present
NSP 1, NSP 4

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12
Q

Perthes:
Typical age
Associations

A 
5-7 (but 3-12)
HIV
SLE
GCS
CKD
Gaucher

Bilateral in 10%

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13
Q

NAIT Anti Platelet antibodies?

A

Caucasians
HPA - 1A
HPA - 5B

Asian
HPA -4

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14
Q

T1DM Antibodies

A

Anti Insulin IAA - 1st appearing, 70%, fades with age/inversely proportional
Anti Islet Cell ICA - 70-90%
Anti Glutamic Acid Decarboxylase - GAD 80%
Anti Protein Tyrosine Phosphatease IA-2A 55-75%

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15
Q

B12 Visual Mnemonic

A

Cobalt - Cobalamin
Parrot - PARIETAL CELL - SPITTING ACID and INTRINSIC FACTOR, Gastric Epithelial
Glycoprotein - Intrinsic Factor - COGWHEEL
VACUUMING CHILD - END OF TUBE is TERMINAL ILLEUM sucking in B12 and IF
cf. Folate in Jejunum absorption
Cow’s and Pig’s mobilze gut bacterium for B12
Metal on Table - METAL EYE - METHIONINE-Synthase
Catalyses regeneration of methionine from homocysteine
THF -> gives a methyl group
Young boy breaking Millenium Falcon with Croquet
- Methylmalonyl-CoA Mutase - converts MMA-CoA to Succincyl Co-A for Haem synthesase or Citric acid pathway

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16
Q

Proprionic Acidemia Mnemonic

A

Han breaking out MMA
Propeller Acid - Propionic Acid - BUILD UP OF ACID
DEFECT in Propionyl-CoA Carboyxylase - Cardboard box and Croquet.
Millenium Falcon stuck in acid - Methylmalonic Acid
Propionyl-CoA then interconverts into PROPIONIC ACID
and DECREASED Methymalonic Acid (Because enzyme deficient)
(MMA also measured for B12 deficiency, where it is increased due to failure of next enzyme MMA->Succincyl-Coa)
Autosomal Recessive
Infant- Hypotonic Guard
Hepatomegaly
Sheaking vat, seizures

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17
Q

Xeroderma Pigmentosum - Mnemonic

A

XEROX Copier
NUCLEAR Powered - Nucleotide excision repair (For UV light)
vs. Base excision repair
UV Black Light
10c Dimes, TT- Dimers, Kinks, blocks replication, NER Fixes in healthy patients - XPA, XPB, XPC, XPD, XPE, XPF, XPG.
Extreme sensitivity to UV light
- OUTRAGEOUS sunburns with brief exposure
- Early Skin Cancer +++
Corneal ulcers

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18
Q

Woods Lamp

A

Enhances EPIDERMAL pigmentation (Not dermal)

Hypopigmentation (ASH LEAF MACULES) - Sharp borders, fluoresces blue/white or yellow/green vs. reduced blood flow (no change)

Porphyria - Red-pink fluoresnce of skin
Teeth - Erythpoetic porphyria

Tetracyclines fluoresce on skin after oral therapy

Tine - blue/green Capitis
Malassize (Including pityriasis versicolour)

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19
Q

Eating Disorder - Criteria for AN?

A

Prevalance = 1%
Loss of weight (If not to below expected then ‘atypical AN’)
Intense fear or PERSISTENT BEHAVIOURs to avoid weight gain
Undue experience/Self Evaluation of weight/fat

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20
Q

Eating Disorder - Treatment for AN

A

Family Based Therapy - Maudsley

1) Weight restoration, parent=parent, kid=kid, parent in control
Parent supervises HITH style all feeds until 85% weight
Weekly for 4/12
Siblings support distress
2) Adolescent transition
Adolescent gradually given control
Parents/siblings fade
Fornightly for 2/12
3) Adolescent issues
Time to address the other co-morbid shit put to the side
Monthyl

//

VS.

CBT for Eating Disorders (CBTe)
3 x 50 minutes with view to formulation of individuals psychopathology- ADDRESSES YOUNG PERSON

//
Antipsychotics for extreme meal distress
SSRIs for comorbid depression/anxiety only

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21
Q

Bulimia Nervosa - Prevalance/Dx

A

2-3%
Losing control with compensatory behaviours
Self evaluation

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22
Q

Causes of ARFID?

A

Avoidant Restrictive Food Intake Disorder
/
Phobia - Fear of vomit/choking
Somatising - Too many other stressors so ‘feels full’
ASD/Fussy Eating

//
FBT - UP (Unified Protocol)  + Address underlying cause
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23
Q

8 Core Principles of addressing anorexia?

A
  1. Agnostic to cause
  2. Separate client from “The Anorexia”
  3. Privildge the family’s resourcefulness
  4. Hospital is temporary
  5. Target “THE ANOREXIA” not the other family conflicts, come together for this
  6. Structural CHANGE to defeat
  7. Therapist resists expert role, transfer to parents
  8. Medical needs> adolescent needs
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24
Q

Admission Criteria for AN?

A 
HR< 50 or >30bpm increase postural
BP <80mmhg or 20mmHg drop postural
Weight <75%, or >10% in 3/12
HypoK <3.0
Hypothermia <35.5
QtC >0.45
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25
Q

Risk of vertical transmission in HBV?

A

If mum is HbE +ve (Or viral load high)
Then 90% untreated, 5% if HB-IG and HB-Vac
If HbE-Antigen -ve and treated then 1%

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26
Q

Rate of cCMV

A

congenital-CMV
1:500
Roughly equal contribution from 50% population seronegative who acquire (2%) and 50% population seropositive who reactivate (10%)

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27
Q

Antipsychotic Drugs - Which receptors?

A

Antidopaminergic - block D2 receptors
Causes parkinsonian symptoms

Anticholinergic ‘

Neuroleptic malignant

Haematological

Rashs
Skin Pigment

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28
Q

Risperidone?

A

Good to treat subacute aggression/irritability with psychotic symptoms

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29
Q

Olanzapine - good for use with?

Effects

A

Overt aggression with psychosis

Good for sedation and strong mood regulating

Also 1st line manic bipolar

AVOID in RENAL FAILURE

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30
Q

Quetiapine?

A

Second generation (Between SSRI and Antipsychotic) Antipsychotic with serotonin enhancing properties

Good for sleep
Good for anxiety

Bigger dose at night (sleep)
Smaller at day (anxiety)

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31
Q

Ziprasidone?

A

Second generation antipsychotic,
Not that effective but:

NO WEIGHT GAIN - doesn’t cause weight gain

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32
Q

Clozapine

A

Second Line (So if doesn’t improve with initial antipsychotic)- Second generation antipsychotic

Associated with CARDIOMYOPATHY
- So have to do an echo

Associated with agranulocytosis - 1%
Cf. Olaznapine 0.5%
Quetiapine 0.6%
Risperidone 0.3%

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33
Q

Aripiprazole

A

Prominent mood problem with pyschosis

ADHD + psychosis

Or for delerious PICU patient

“Third Generation Antipsychotic”

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34
Q

Treatment/Antidepressants for Depression?

A

Brief Intervention - works about 30%

CBT/IPT works about 55%

If inadequate response then intensify CBT/IPT

If no response then :Antidepressant - SSRI Fluoxetine - continue for 12/12 before

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35
Q

Vitamin E deficiency - symptoms?

A

Progressive Ataxia
Retininitis Pigmentosis
Spinocerebellar and dorsal columns (Mimics B12)
Loss of antioxidant function for RBC - haemolytic anaemia, ancanthocytes, bilirubin gallstones
Vitamin E = Tocopherols
Fat soluble vitamin

Vitamin E Excess - Excess Warfarin anticoagulation

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36
Q

Vitamin A deficiency?

Vitamin A excess

A

HAY (Vitamin A) Scene

DEFICIENT
Night Blindness
Conjuinctival “foamy” spots (due to specialised epithelial signalling)
Corneal liquifaction (Carrot - Cream)
Dry scaly skin - follicular hyperkeratosis and loss of sebaceous gland
Immunosuppression for T-cell maturation

EXCESS
Raised ICP
Papilloedma
Teratogen ++ (Cleft, Cardiac - TGA/TOF)
Dry skin ++
Hepatomegaly
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37
Q

Kleinfelters - Vmnemonic

What genes
What cells
What hormone
What signalling
What phenotype
A

Kleinfelters = Clean Filters
47 XX - Two X wrenches, BROKEN Bar (Barr body inactivation)
Sertoli (certificate), curled up tubes, seminiferous tubules (Hyalizined) and Leydig (ladybug) both less functional, may have leydig pseudoadeonmatous clusters

Low TESTOSTERONE (Aka Lestosterone from Leydig cells)
And
HIGH esTROJAN horse from Sertolis

High FSH (Fish) and High LH (Lute)from low T -> feedback

Tall, skinny/slender low muscle mass men, testicular atrophy, gynecomastia
Infertile, degenerative seminiferous and azoospermia

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38
Q

Osteo Sarcoma - Visual Menomic?

A

Bony Shark (OsetoSarcoma)
Bruce LI FRAUMENI kicking over a cement mixer (Osteoid Tumour)
Old man with a glass eye (Retinoblastoma) and a peg leg (Knee brace, knee predilection (Femur/Tibia) and metaphyseal (as in adjacent to the physeal line where bone growth happens)

Codman’s triangle on sailing ship - lifting periosteum
Sunburst pattern - Perpindicular rapid growth

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39
Q

Falling ESR despite cracking fever?

A

Consider low fibrinogen - consumed by HLH or DIC or Macrophage Activation Syndrome - as Fibrinogen required for ESR assay

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40
Q 
Vignette:
4yo Boy
Fever
Splenomegaly
Cytopaenias
EBV Positive - big nodes
LFT deranged
A

Consider HLH - Especially XLP - X-linked lymphoproliferative - Slam Associated Protein - SAP (Crucial to activate cytotoxic NK and CD8 cells)
Abberant IFN gamma signalling
Macrophages activated +++

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41
Q

What is primary HLH?

A

Haemophagocytic Lymphohistiocytosis

  • Macrophages gone crazy due to failure of normal cytotoxic cells to clear a viral infection - That is failure of NK and CD8 cells
  • Problems with granules - function, trafficking, delivery
Perforin
UNIC13D
STXBO2
STX11
Albinisms//
Griscelli RAB 27a
Chediak Higashi - LYST
XLP1 - SAP - SH2D1A
XIAP- XLP2 - BIRC4
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42
Q

Where does EBV lay dormant?

A

In B - cells

Its HHV4

The body needs the help of CD4 then cytotoxic function of CD8 and NK to clear the infected B cells

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43
Q

Erythema Toxicum

Which day
What does Bx show?

A

Day 2

Full of eosinophils

44
Q

Rash at 6/52 of age?
Forehead
Looks like acne?

A

Called milk spot, or neonatal acne but:
Pityrosporum
Seborrheic folliculitis, sebaceous glands starting to settle - crisis for flora, pustules, symmetrical

Natural history - resolution
Dilute ketoconazol 2% 1:10 and apply daily

45
Q

What is cradle cap?

What age makes you think of something else?

A

Greasy (Seborrhoeic) scale, not puruitic, analgous to dandruff

Use soap substitute, olive oil, bath oil,

DDx -Scalp ECZEMA if >3/12
Eczema scale is harsher, mor epuritic - dry harsh scale - use ointments - no role for anti yeast

46
Q

Perineal protrusion in neonate?

A

IPPP - infantile perineal pyramid protrusion- resolves

47
Q

Nappy rash that isn’t settling - what is best barrier cream?

A

COVITOL Cod liver oil in zinc cream

Bepanthan

Plain zinc

Also if cost barrier, can prescribe via olive oil 10% in zinc paste -1kg, 5x repeats.

Or 1% hydrocortisone ointment
Add in antifungal if suspicios - swab for growth

48
Q

Nappy rash and flexure involvement

A

Napkin Psoriasis

49
Q

Erytherma Multiforme?

A

Not urticaria

Needs a bullseye breaking the skin

50
Q

Zinc deficiency in infants

A

A)Inherited - genetic = acrodermatitis enteropathica
Autosomal recessive, failure of absorption, breast milk protective, presents post weaning

B) Acquiried
Multifactorial
Prematuritis
Malabsoprtion
Some mums have low zinc in breast milk
//
Sharply demarcated, eroded - peeling skin
Around fingertips/nappy/lips
Hair loss
MISERABLE
Immunosuppression
Cognitive/motor delay
//
No good Ix for Zinc stores (cf. ferritin)
Low alk phos
1-2mg/kg/day
DDx - metabolic conditions with acidosis
51
Q

Perioral dermatitis?

A

Periorofacial rosacea
Spares immediately next to lips - pustular

Occurs on withdrawal/rebound from topical steroids

52
Q

Drug eruption?

A

Widespread eruption, macular, diffuse, symmetrical

DDx viral exanthem

Not to be confused with ‘fixed drug eruption’ - circular, post inflammatory pigmentation, recurs/flares with medication

53
Q

Erythroderma - how much of skin>

Complications

A 
90% of skin
Psoriasis
Eczema
Drug eruption
Lymphoma/Leukaemia

Red all over is significant metabolic impact
Can High output cardiac failure
Can dehydrate
Can de-nutrition incl Fe, protein

54
Q

Is erythema multiforme on the SJS-TEN Spectrum?

A

Not the same as
SJS/TEN worse

Erythema multiforme - which are targets with epidermal disruption/blister to central target - but won’t coalesce
May have a focal oral lesion “target lesion in mouth”

55
Q

SJS/TEN?

A

Widespread detachement of full-thickness epidermis

Drugs - sulfonamides, anticonvulsants (Phenytoin, Phenobabitone, Carbemazepine)

Infection
Autoimmune

Prodrome - 2 days to 2/52
Fever, URTI, Milk to moderate skin tenderness
Conjunctivitis/mucositis
Signiifcant loss of skin

56
Q

Differ TEN from Staph Scalded Skin?

A

No MUCOSAL lesions in SSSS
More superficial (Subcorneal) in SSSS rather than subepidermal
More tender in SSSS

57
Q

Bullous impetigo vs. staph scalded skin

A

Bullous impetigo is local from toxin

Staph SS is Toxinemic

Think umbi stump first week

58
Q

Parvo B 19

Can cause 3 rashes?

A

Slapped cheek first

Fine lacy reticular rash to arms - immune complex phenomenon - evanescent for 2/52

Purpuric glove + stocking

59
Q

Hand foot and mouth

What does rash look like?

In what way is HSV different?

A

Classic” Oval grey blisters palm, soles, mouth,

CoxsackieA 16
SYMMETRICAL (cf. HSV)
Widespread brown scabbign to perioral extremeties and nappy
Polymorphic in nature (cf HSV)
Can be blistering, look purpuric
Manifest stronger where eczema present “eczema coxsackium”

60
Q

Kawasaki Disease

Fever and rash relationship from dermatology lecture?

A

Fever stays high with rash unlike many milder viral which improve at time of rash apperance

Palms and sole involvement rare in normal virus

Eye redness spares RIM aroudn pupil - ocular injection

Eruption of skin non-specific but often accentuates perineal/groin

61
Q

DRESS ?

A

Drug reaction Eoisonphils Syndrome
2-6/52 into Rx - ESPECIALLY ANTIEPELEPTICS

commencement
Fever
Lymphadenopathy
Variable rash

Commonly involves other organs - hepatitis/nephritis

FBE - atypical lymphocytosis that PROGRESSES TO EOSINOPHILLIA

62
Q

Measles - which day does rash appear?

A

Measles rash on DAY 4 - T-Cell mediated

Kough, Koryza, Koryza with fever BEFORE Rash

63
Q

Food allergy urticaria timecourse?

A

Less than a couple of hours

Starts on head and neck

64
Q

Antihistamine - What ages are ok?

A

Best prophylactically

From 6/12 can use non-sedating

From 24/12 can use sedating

Antihistamines WON’T affect rash of urticaria - just itch and elevation of wheal

Vs.

PRED which will CLEAR urticaria if sufficient dose

65
Q

Pityriasis Rosea?

A

Seasonal variations - therefore ?infectious trigger

Proximal joint
Herald patch in 80%
Long axis is “inverted christmas tree”
Free edge in internal - i.ie CENTRAL scale — cf. Fungal with scale at edge of lesion

Lasts 3-6/52

Rx
Topical steroids not very good
Sunlight/UVB very useful
?Erythyromycin

66
Q

Unilateral laterothoracic exanthem// asymmetrical periflexural exanthem

?

A

Discrete erythematous in hip or axilla then spread ipsilateral or over trunk

Scaly eruptions
Lasts weeks

67
Q

Giannotti-Crosti Syndrome

Or Papupal acrolocated syndrome

A

Age 6/12 to 12 yrs
Urticaria like papules on limbs/buttocks/face - outer cheeks

spares trunk

Monomorphic for patient “Papulo-vesicles”

Lasts weeks 6/52
No Rx required
Not contagious

68
Q

Eczema?

is it chronic?

Patterns

A

Tendency to sensitive skin “degree of atopy”

Triggered - multifactorial, shouldn’t be “chronic” - aim is to have no eczema most days.

Triggers - heat, dry, irritants

Think environmental if age>2 , distributed with exposed areas, HDM< grass pollen, animal dander - SPT and RAST (as well as total IgE)

Food allergen <1yo, widespread, unsettled, associated GIT or FTT, reactions to food

69
Q

Whats the relevance of total IgE in interpreting RAST testing?

A

An elevated total IgE in the thousands due to raging HDM allergy (for e.g.) will give moderate FALSE positives for anything you examine, due to non-specific binding nature of IgE

If negative total IgE then unlikey to have a type 1 allergy

70
Q

Perioral dermatitis after eating favourite foods?

A

Food intolerance -

Salicylates/Amines

Delicious
Vasoreactive
Tomatoes/strawberries/cirtus/watermelon/soya/acidi preservaive

Natural Hx grows out of

71
Q

Juicy plaques in non-atopic individual

A

Discoid/Nummular eczema?

Vicious cycles

Needs potent topical steroids and antibiotics
Wet dressing
Sunlight / UVB

72
Q

MYTHS OF TOPICAL STEROIDS

  • What things DOESN’t it cause?
A

1 Skin thinning
2 HPA Access
3 In setting of infection
4 Striae
5 Allergic contact dermaitits
6 Osteopaenia
7 Ocular effects - if eyelid hydrocortisone ok for long term, more potent for short burst
- Or pimecrolimus
Hypertrichosis - discoid eczema only, short term
Hypopigmentation - secondary to eczema/inflammatino
Purpura/telengectasia

DOSAGE is insufficient - i.e. a tube a week of methylpred in a 6/12 old is fine
DISEASE causes all this stuff
Or is TRANSIENT only

BUT:

CAN irritate periorifacial dermatitis/rosacea - rim sparing pallor, papules, rebound side effect - cortisone asthma spray/nasal if mask used
Rx with oral antibiotics - erythromycin, bactrim, or tetracyclines if oldre - may take years

PRESCRIBE AUTHORITY QUANTITY
do not write “sparingly”
Get it CLEAR

73
Q

Azathioprine - MoA/commencement/side effects

  • For eczema?
A

Purine inhibitor

Check Thiopurine MethylTransferase (TPMT) prior to start, as some people can’t metabolise

Start at 1/4 dose and get to full by 8/52, expect therapeutic effect at 12/52

GI - nausea/vomting
Bone marrow - lymphocytes - aim 1.0-1.5

74
Q

Allergic contact dermatitits - what to treat it with?

A

Known trigger, unlikely to recur, antihistamines most helpful PRIOR to exposure/prophylaxsis

Therefore give steroids - ORALLY - but also throw in some topical

75
Q

Ulcerated haemangioma Rx?

A

Bactroban/fucidin
Metronidazole
xylocaine jelly

Big mixed up dollop on top
Then occlusive dressing - like tegaderm,

Prevent with nappy rash cream +++

76
Q

Haemangiomas to worry about?

A

PHACES
Posterior Fossa
Haemangioma - Big, segmental - i.e. stops at
Arterial anomalies
Cardiac + Coarct
Eye abnormalities/ endocrine abnormalities

BEARD distribution (2/3rd involve airway)

Visual field/periocular (refractive due to pressure)

Ulcerating/disfiguring

77
Q

Advice with propanolol for haemangioma?

A

STOP WHEN FEEDING STOPS - PAUSE DURING ILLNESS - hypoglycaemia risk

Can start at 1mg/kg/day - then to 2mg/kg/day mostly, increase up to 3mg/kg/day if eye saving

Continue until achieve agenda
Don’t need to increase with weight or titration to 2mg/kg if having adequate effect

5% Sleep disturbance - +/- Atenolol

78
Q

Time for skin prick testing after drug reaction?

A

At least 4-6/52 after last reaction as you may have depleted all the IgE specific on those mast cells and need to regenerate - risk of false NEGATIVE

79
Q

Anti TNF Alpha drugs - Main thing to thing before commencing?

A

Screen for TB

80
Q

In which pH do various urine stones form?

A

They form in acid excelpt for CALCIUM PHOSPHATE.

SO ALKALINIZE; unless CaPO4

81
Q

Cortisol Levels - When highest, when lowest?

A

Highest at 0800 - screen for insufficiency - confirm with synACTHen
Lowest at 2400 - screen for excess with saliva - confirm with 24/24 urinary cortisol and/or Dexa suppression

82
Q

Why risperidone in intellectual disability?

A

Injurious/aggresive behaviour

83
Q

Why clonidine?

What is it?

A

Treats Tics
Treats hyperactivity/aggression/dyssomnias

Alpha2 adrenergic agnoist

84
Q

Ossification centres of the elbow?

A 
CRITOE, 1, 3 , 5, 7 ,9, 11
Capitellum
Radial
Internal epicondyle
Trochlear
Olectranon
External epicondyle
CRITOE
85
Q

Pertussis Treatment -

Pertussis Prophylaxsis -

A

Macrolide (Azithro/Clarithro) - Bactim if CI
antibiotic for index with <14 day cough (<21day if <6 months)
Then ALL household if a <6/12
and ALL childcare if <6/12 in same room

86
Q

Risk of Neural Tube Defects?

A

15: 10,000 conceptions, 2:10,000 live births
Risk 1:25 if parent has NTD, 1:50 if sibilng has NTD

Mother on antiepileptic - valproate and less so carbemazepine
Diabetes Mellitus in mother
Folic acid at 5mg

87
Q

Symptoms of tethered cord?
Which age?
Which group of patients

A

Due to traction of nerves
In puberty due to growth
Neural tube defect patients

Bladder, bowel Sx
Foot position
Back or lower limb pain

88
Q

Greatest historical risk of death in neural tube disorder?

A

Renal failure - obstructive due to neurogenic bladder

Now from birth daily or more CIC unless demonstrate non-contractile/high pressure system

89
Q

Options to preserve renal function in neurogenic bladder

A

Rx
CIC (Risk of LATEX allergy)
anticholinergics (Oxybutynin/Ditropan) to reduce detrusor hyper-reflexia
Surg:
Botox
Vesicostomy/Urinary diversion
Mitoffanoff (appendix conduit bladder to umbilicus)

90
Q

Precocious puberty - age definitions and risk of brain tumour

A

Girls, <8yo, 5% risk tumour

Boys, <9yo, 70% risk tumour

91
Q

Puberty onset and timing of growth spurts

A

Male -from 9; average 13 - growth at 15

Girls from 8, average 11 - growth at 11

92
Q

4 week old infant with hypocalcemic seizure?

Two differentials

A

Hypoparathyroidism

Infant of severe vitamin D def. mother

93
Q

Scrotal pigmentation in a caucasian newborn?

A

Congenital Adrenal Hyperplasia. Expected vomiting/dehydration/collapse/death day 8-14

94
Q

Noonans - physical characteristics:

A

“Male Turners”
Webbed neck, down turned palpebral fissures, wiry hair, down turned mouth

Autosomal Dominant
PTPN11 Mutation

95
Q

Midline defects

A 
Hypertelorism
Dimpled nose
Central cleft lip/palate
Bifid uvula
Central incisor in upper

Thyroid
Cardiac
Gonadal
Malrotation of gut

CHARGE

96
Q

Phenotypic shape of coeliac disease in 2-3 year old?

A

Big abdomen, skinny arms and legs, short stature

97
Q 
Low grade glioma - 
Whats the prognosis
Whats the treatment
Whats the genetics
Any novel Rx?
A

Cystic changes on MRI
Good prognosis - most common CNS tumour in kids, 90% survival
If resectable - cure
If unresectable, chronic disease
18/12 of gentle CTx (Vincristine/Carboplatin)
Often relapse and re-dose
Single pathway - MEK1/2 as final common
Therefore MEK inhibitors, orally active selumetinib, trametinib, cobimentinib

98
Q

DIPG - tumour

A

Most common high grade brain tumour
Incurable
Diffuse - ill defined spreading mass around the pons -
Short duration prior to presentation 2-6/52 -cranial nerve or long tract signs
Dead within a year

99
Q

Whats the relevance of small round blue cell tumours?

A

They are embryonal - so all the embryonal tumours “Blastomas”

Ewings
Neuroblastoma
Medulloblastoma

100
Q

Medulloblastoma

A

Most common MALIGNANT CNS in paeds
20% of brain tumours
Peak at 4 years
“Dull appearance of MRI - densely packed, full of nuclei, not full of cysts, “
2-6/12 of signs
Obstructive hydrocephalus
Cerebellar dysfunction
Average risk - M0 and mostly resectable (<1.5cm^2) - 80% cure
High risk - M1-4 or big residual 70% cure
Group 3 is terrible
Post surgical - cerebellar mutism in 25%, irritable, emotional lability, cranial nerve, resolves in months

Chemo and radiation
Terrible if relapse
Plenty of side effects

101
Q

What is Gorlin Syndrome?

A

Association with medulloblastomas and propensity to BCC - hugely so following craniospinal irradation
Also some bony/cartilage stuff - syndactyl, ears, calcified falx, palmar pits

102
Q

Radiation therapy side effects?

A

Terrible if <3 years old due to poor future cognitive development

Otherwise:
Endocrine - Growth Hormone 
Spinal growth less 
Secondary Malignancy - brain tumours
IQ and learning (less if >10 years old)
Vasculopathy and stroke
103
Q

Hearing dysfunction - Tiers of investigation?

A 
Tier 1 - MRI for uni/bilateral
CMV Testing - urine/saliva if young, otherwise Guthrie
Gene for Connexion26
Visual Acuity
Family audiograms
Tier 2
Genetic WES/Micro
ECG - for Long QT1 homozygote- Jervel Lange Nielsen
Thyroid Fx - ?Pendred
Urine micro - ?Alports
Opthal - Usher 
Renal U/S - ?Branchio/oto/renal syndrome
Metabolic if matrilineal inheritance/regression
104
Q

Ritalin - MOA

A

Dopamine and Noradrenaline reuptake inhibitors

105
Q

Atomoxitine (Strattera) MOA

A

Noradrenaline reuptake inhibitor

Steady state effect

RACP Gen Paeds (1 decks)

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