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3a > Paeds > Flashcards

Paeds Flashcards

1
Q

Name 4 paediatric brain tumours

A
  1. Astrocytoma
  2. Glioblastoma
  3. Medulloblastoma
  4. Ependymoma
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2
Q

What is an astrocytoma?

A
  • primary tumour of brain arising from astrocytes

- most common primary brain tumour in children

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3
Q

clinical features of an astrocytoma

A
  • altered mental state
  • headache
  • N+V
  • gait
  • ataxia
  • weakness
  • seizures
  • visual disturbances
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4
Q

describe the histology you would see of a pilocytic astrocytoma

A

Rosenthal fibres (corkscrew eosinophilic bundle)

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5
Q

initial treatment of astrocytoma

A
  • dexamethasone +/- mannitol
  • temporary hyperventilation
  • levetiracetam/phenytoin (seizures)
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6
Q

What is a glioblastoma?

A
  • most common primary tumour in adults

- poor prognosis (1yr)

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7
Q

describe the histology you would see of a glioblastoma

A
  • pleomorphic tumour cells border necrotic areas
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8
Q

what would imaging show of a glioblastoma

A
  • solid tumours w/ central necrosis
  • rim that enhances w/ contrast
  • vasogenic oedema: disruption of blood brain barrier
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9
Q

what is a medulloblastoma?

A
  • aggressive paediatric brain tumour
  • arises within the infratentorial compartment
  • spreads through CSF system
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10
Q

describe the histology of a medulloblastoma

A
  • small blue cells

- Rosette pattern of cells w/ many mitotic figures

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11
Q

What is an ependymoma

A
  • tumour arises from ependymomas (tissue of the CNS)
  • Paeds: intracranial, 4th ventricle
  • adults: spinal
  • may cause hydrocephalus
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12
Q

describe the histology of an ependymoma

A

perivascular pseudorosettes

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13
Q

name a neuroendocrine tumour

A

Neuroblastoma

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14
Q

what is a neuroblastoma

A
  • top 5 causes of cancer in children
  • tumour arises from neural crest tissue of the adrenal medulla + sympathetic NS
  • median age of onset: 20 months
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15
Q

clinical features of a neuroblastoma

A
  • ABDOMINAL MASS
  • pallor, weight loss
  • bone pain, limp
  • hepatomegaly
  • paraplegia
  • proptosis
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16
Q

investigations for a neuroblastoma

A
  • ↑ urinary vanillylmandelic acid (VMA)
  • ↑ homovanillic acid (HCA)
  • X-ray: calcifications
  • Biopsy
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17
Q

name bone tumours

A
  • osteosarcoma

- Ewings sarcoma

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18
Q

what is an osteosarcoma

A
  • mesenchymal cells
  • w/ osteoblastic differentiation
  • incidence: 5 / 1,000,000
  • peak age: 15-30yrs
  • commoner in males
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19
Q

clinical features of an osteosarcoma

A
  • worsening pain over weeks to months
  • mass/swelling: firm, sometimes tender + warm
  • antalgic gait
  • uncommon: hx of trauma
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20
Q

investigations of an osteosarcoma

A
  • conventional radiographs
  • ↑ alkaline phosphate
  • ↑ lactate dehydrogenase
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21
Q

what is Ewings Sarcoma

A
  • symptoms of osteosarcoma + fever, night sweats
  • incidence: 0.3 / 1,000,000
  • onset: 10-20yrs
  • commoner in males
  • commonest site: femoral diaphysis
  • blood borne metastasis is common
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22
Q

describe the histology of Ewings Sarcoma

A

small round tumour

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23
Q

Name a kidney tumour

A

nephroblastoma aka Wilms’ tumour

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24
Q

what is a nephroblastoma/Wilm’s tumour

A
  • most common childhood malignancy

- presents <5yrs

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25
Q

clinical features of a nephroblastoma

A
  • abdo mass (most common)
  • painless haematuria
  • flank pain
  • anorexia
  • fever
26
Q

nephrotic syndrome is diagnosed by:

A

1) Proteinuria > 1g/m2/24 hours
2) Serum albumin < 25
3) peripheral oedema

27
Q

cause of nephrotic syndrome in children?

A

minimal change disease

28
Q

what is minimal change disease

A
  • effacement of foot processes

- protein leaks out –> hypoalbuminaemia –> oedema –> hypercholesterolaemia

29
Q

presentation of nephrotic syndrome in children

A
  • facial swelling
  • peripheral oedema
  • occasionally infection
30
Q

inx for nephrotic syndrome

A
  • FBC, U&Es, LFTs
  • Immunoglobulins
  • Complement levels
  • Varicella titres
  • Blood pressure

Consider need for:

  • Autoantibodies, hep B serology
  • Renal USS
  • Renal biopsy
31
Q

management for nephrotic syndrome

A
  • Fluid balance
  • Prednisolone
  • Penicillin prophylaxis
  • Pneumoccocal vaccination
  • Consider albumin infusion

Consider
Levamisole
Cyclophosphamide
Cyclosporin

32
Q

kid develops maculopapular rash after amoxicillin. What could they have?

A

Infectious mononucleosis

33
Q

triad of Haemolytic Uraemic Syndrome

A
  1. normocytic anaemia
  2. AKI
  3. thrombocytopenia
34
Q

symptoms of haemolytic uraemic syndrome

A
  • BLOODY DIARRHOEA
  • no fever
  • abdo pain
35
Q

cause of haemolytic uraemic syndrome

A

Shiga producing toxin E.coli (contaminated food)

36
Q

treatment of haemolytic uraemic syndrome

A

blood transfusion

37
Q

what is Stevens-Johnson Syndrome?

A
  • type IV hypersensitivity reaction

- cytotoxic T-cells inappropriately attack + kill epithelial cells in the mucosa + skin epidermis

38
Q

what is the difference between Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis?

A
  • SJS: <10% total body surface area

- TEN: >30% total body surface area

39
Q

Name 4 features of Stevens-Johnson Syndrome

A
  1. sudden maculopapular rash w/ target lesions
  2. mucosal involvement
  3. Fever, arthralgia
  4. Nikolsky’s sign
40
Q

what is Nikolsy’s sign?

A

epidermal layer easily sloughs off when pressure is applied to affected area (evident in Toxic Epidermal Necrolysis)

41
Q

Name some causes of Stevens-Johnson Syndrome

A
  1. penicillin
  2. sulphonamides
  3. lamotrigine, carbamazepine, phenytoin
  4. allopurinol
  5. NSAIDs
  6. OCP
42
Q

What is the definitive diagnosis for Stevens-Johnson Syndrome?

A

Skin biopsy: keratinocyte apoptosis w/ detachment of epidermal from dermal layer

43
Q

Management for Stevens-Johnson Syndrome

A
  1. Withdraw causative agent
  2. Prophylactic anticoagulation: enoxaparin
  3. PPI: omeprazole prevents stress related gastritis + intestinal ulceration

4, dressings + topical antibacterial agents + emollients
5. Ophthalmological exam

44
Q

Describe Infantile spasms (West’s syndrome)

A
  • brief spasms at 4-6m
  • M>F
  • usually 2nd to serious neurological abnormality (e.g. TS, encephalitis, birth asphyxia) or may be cryptogenic
45
Q

Treatment for infantile spasms (west syndrome)

A

1st line: Vigabatrin

steroids

poor prognosis

46
Q

Features of infantile spasms (West’s syndrome)

A
  1. Flexion of head, trunk, limbs → extension of arms (Salaam attack)
  2. last 1-2 secs, repeat up to 50 times
  3. Progressive mental handicap
  4. EEG: hypsarrhythmia
47
Q

what are Typical (petit mal) absence seizures

A
  • onset 4-8 yrs
  • F>M
  • stare momentarily + stop moving
  • duration few-30 secs; no warning, quick recovery; often many per day
  • EEG: 3Hz generalized, symmetrical
48
Q

treatment for absence seizures

A
  • sodium valproate, ethosuximide

- good prognosis: 90-95% become seizure free in adolescence

49
Q

what is Lennox-Gastaut syndrome

A
  • multiple seizure types
    -mostly drop attacks (astatic seizures), tonic seizures + atypical absences
  • neurodevelopment arrest or regression + behaviour disorder. severe mental handicap
    -may be extension of infantile spasms (50% have hx)
    onset 1-5 yrs
  • EEG: slow spike
50
Q

treatment for Lennox-Gastaut sydrome

A

ketogenic diet may help

prognosis is poor

51
Q

what is Benign rolandic epilepsy

A
  • tonic clonic seizures in sleep
  • or simple focal seizures with abnormal
    sensation in tongue/ face
    -most common in childhood, M>F
  • paraesthesia (e.g. unilateral face), usually on waking up
  • EEG: focal sharp waves from the Rolandic or centrotememporal area
52
Q

what is Juvenile myoclonic epilepsy (Janz syndrome)

A
  • Myoclonic seizures but generalised tonic cloonic and absence seizures may occur
  • shortly after wakening
  • clumsy in morning
  • onset: teens; F:M = 2:1
53
Q

treatment for Juvenile myoclonic epilepsy (Janz syndrome)

A

usually good response to sodium valproate

54
Q

how do you know from CSF if it is bacterial or viral meningitis?

A

bacterial: ⬆️ polymorphs
viral: ⬆️lymphocytes

55
Q 
learning difficulties 
big head 
long face
large ears
large testes
A

fragile x syndrome

56
Q 
learning difficulties 
short
friendly extrovert personality 
star shaped irises
elfin like facies
transient neonatal hypercalcaemia
supravalvular aortic stenosis
A

william syndrome

57
Q

webbed neck
pectus exavatum
short stature
pulmonary stenosis

A

noonan syndrome

58
Q 
small head
small eyes
cleft lip/palate 
polydactyly 
scalp lesions
A

Patau syndrome (trisomy 13)

59
Q

hypotonia
hypogonadism
obesity

A

prader willi syndrome (chromosome 15)

gene deleted from father

60
Q

what is the 1st, 2nd and 3rd line treatment for ADHD

A

1st: methylphenidate
2nd: Lisdexamfetamine
3rd: Dexamfetamine

3a (5 decks)

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