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3A > sat 7th > Flashcards

sat 7th Flashcards

1
Q

Genetics of neurofibromatosis type 1

A

chromosome 17- neurofibrimin

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2
Q

Criteria of neurofibromatosis type 1 and how to remember it

A

CRABING
C- cafe au lait spots (>15mm)
R- relative with NF1
A- Axillary and lymph node freckling
B- Bony dysplasia- leg bowing
I- Iris harmartomas (yellow brown spots in eye)
N- neurofibromas
G- gliomas in optic pathway

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3
Q

What can cause occlusion of the anterior spinal artery in anterior cord syndrome

A

iatrogenic causes- occurs during cross clamping of the aorta
severe hypotension
vasculitis
atherothrombosis
cocaine

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4
Q

What drops are used to diagnose horners

A

apraclonidine

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5
Q

what drops can help locate horners

A

hydroxyamphetamine

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6
Q

What drugs may be used in the chronic management of MS

A

natalizumab
ocrelizumab
beta interferon

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7
Q

What are some contraindications to thrombolysis following stroke?

A

GI bleeding in the past 3 weeks
LP in the past 7 days
Stroke or head trauma in the past 3 months
intracranial neoplasm
seizure at stroke onset
previous intracranial haemorrhage
oesophageal varices
uncontrolled hypertension
suspected SAH

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8
Q

Pathophysiology of lambert eaton syndrome

A

autoimmune condition associated with small cell lung cancer where there is antibodies that target the voltage gated calcium channels- leads to weakness that improves with repetitive stimulation

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9
Q

how is lambert eaton diagnosed and what does it show?

A

EMG - shows incremental response to repetitive stimulation

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10
Q

What surgical procedure is used in biliary atresia?

A

kasai portoenterostomy

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11
Q

what sign does duodenal atresia have

A

double bubble sign

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12
Q

what cancers are associated with coeliac disease

A

non-hodgkins lymphoma

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13
Q

what is a specific complication of hirschprungs

A

hirschprungs associated enterocolitis

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14
Q

what surgical procedure is used in hirschprungs

A

swenson procedure

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15
Q

what sign does intersussception have on USS

A

target sign

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16
Q

what conditions are associated with intusscusseption

A

meckel diverticulum and recurrent viral illnesses

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17
Q

what is the pathophysiology of Meckel diverticulum

A

persistence of the embryological vitelline duct

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18
Q

How does meckel diverticulum present?

A

asymptomatic
painless vaginal bleeding
volvulus or intussuception

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19
Q

What scan is used for meckels diverticulu

A

technetium-99m pertechnetate scintigraphy

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20
Q

what is the management of meckels diverticulum

A

laparotomy

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21
Q

what is the 2 rule for meckel’s diverticulum

A

it is 2 inches long
it occurs 2 feet from the ileocaecal valve
it affects 2% of the population

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22
Q

what procudure may be used to treat malrotation if risk of volvulus

A

ladds procedure

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23
Q

long term management of cows milk protein allergy

A

milk ladder- starts with malted milk biscuits

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24
Q

what sign does volvulus have on abdominal x ray

A

coffee bean sign

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25
Q

what conditions are associated with malrotation

A

exomphalos, congenital diaphragmatic hernia
and intrinsic duodenal atresia

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26
Q

how is cows milk protein allergy diagnosed?

A

skin prick test

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27
Q

how does volvulus present?

A

diffuse abdo pain
complete consitpation
green bilious vomiting

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28
Q

how do infantile spasms present on EEG

A

hypsarrhythmia

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29
Q

what might be used to treat infantile spasms

A

vigabtrin or steroids

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30
Q

how does lennox-gastraut present of EEG

A

slow spike

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31
Q

how do typical absence seizures present on EEG

A

3Hx generalise symmetrical

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32
Q

what is the recurrence rate of febrile convusions

A

30-40%

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33
Q

what defines status febrile convulsions

A

longer than 30 mins

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34
Q

what may be used to treat spasticity in cerebral palsy

A

baclofen

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35
Q

what may be used to treat drooling in cerebral palsy

A

glycopyronium bromide

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36
Q

what two types of hydrocephalus are there and what are causes of each

A

communicative- SAH, meningitis (failure of CSF absorption)
non-communicative- congenital malformations such as stenosis of the cerebral aqueduct)

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37
Q

how is hydrocephalus diagnosed in chidlren?

A

cranial USS

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38
Q

what can cause squints in children

A

idiopathic
refractive error
visual loss
ophthalmoplegia

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39
Q

what two types of squints are there

A

concominant (most common- non paralytic
non-concominant (paralyic )

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40
Q

who need to be referred to an optician for squints

A

those with paralytic squints, those with divergant squints, those over 2

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41
Q

who needs USS in during UTI

A

any age with atypical UTI
under 6 months with recurrent UTI

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42
Q

who needs USS 6 weeks after UTI

A

under 6 months with any UTI
>6 months with recurrent UTI

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43
Q

who needs DMSA scan?

A

under 3 years with recurrent or atypical UTI
over 3 years with recurrent UTI

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44
Q

what causes haemolytic uraemic syndrome?

A

GI infection with shigella producing E.coli

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45
Q

What can increase the likelihood of getting haemolytic uraemic syndrome?

A

using loperamide or antibiotics

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46
Q

how does haemolytic uraemic syndrome present?

A

prodrome of bloody diarrhoea
then:
- abdo pain
- bruising
- confusion
- haematuria
- lethargy
- decreased urine output

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47
Q

what percentage of undescended testes are bilateral?

A

25%

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48
Q

when should referral for undescended testes occur and when should surgery occur

A

referral by 3 months
surgery by 6 months

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49
Q

if a child is found to have bilateral undescended testes what should be done

A

urgent paeds referral within 24 hours

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50
Q

what are some causes of nocturnal enuresis ?

A

overactive bladder
fluid before bed (fizzy drinks)
failure to wake- undeveloped bladder signals
psychological distress

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51
Q

what conditions is hypospadius associated with?

A

undescended testes and inguinal hernia

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52
Q

at what age does surgery for hypospadius occur?

A

by 12 months

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53
Q

what symptom my those with hypospadius get due to chordee?

A

pain during sex

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54
Q

what is the inheritence of congenital adrenal hyperplasia?

A

autosomal recessive

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55
Q

what enzyme is deficient in congenital adrenal hyperplasia?

A

21 alpha hydroxylase

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56
Q

what does the skin appear like in congenital adrenal hyperplasia and why?

A

it will be highly pigmented as high ACTH

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57
Q

For how long do children with chickenpox need to stay off school?

A

5 days or until all the scabs have crusted over

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58
Q

For how long do children with mumps need to be kept of school

A

5 days since swelling

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59
Q

how long do children with impetigo need to stay off school

A

until sores have healed and crusted over
or 48 hours after antibiotics

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60
Q

for how long do children with measles and rubella need to be kepts of school

A

4 days after rash develops

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61
Q

for how long do children with scarlet fever need to be kept off school

A

24 hours after starting antibiotics

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62
Q

for how long do children with whooping cough need to be kept off school?

A

48 hours after starting antibiotics or 21 days

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63
Q

What coniditons do children not need to be kept off school for?

A

hand foot and mouth
slapped cheap syndrome
headlice
threadworms
tonsilitis
glandular fever

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64
Q

Dose of IM penicillin if:
- under 1:
- 1 to 9:
- over 9:

A
  • under 1: 300mg
  • 1 to 9: 600mg
  • over 9: 1200mg
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65
Q

treatment of meningitis in under 3 months

A

IV cefotaxime and amoxicillin

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66
Q

if meningitis is associated with massive adrenal haemorrhage and septic shock what is it called?

A

friedrich waterhouse

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67
Q

How is mumps diagnosed?

A

PCR testing

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68
Q

Management of chickenpox in pregnancy:
- before 20 weeks
- after 20 weeks

A

before: give VZV immunoglobulins
after: give aciclovir

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69
Q

what are some potential complications of chickenpox

A

pneumonia
bacterial superinfection
encephalitis
conjunctival lesions

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70
Q

how is chickenpox treated in immunocompromised?

A

aciclovir

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71
Q

what virus causes rubella ?

A

toga virus

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72
Q

how does rubella present?

A

low grade fever
maculopapular rash
lymphadenopathy

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73
Q

Whats the name of the sign that may be seen on x ray of TB?

A

coin sign

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74
Q

what are the 4 drugs used in the treatment of TB?

A

rifampicin
isoniazid
Pyrazinamide
Ethambutol

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75
Q

SE of rifampicin

A

red secretions

76
Q

SE of isoniazid

A

peripheral neuropathy

77
Q

SE of pyrazinamide

A

gout

78
Q

SE of ethambutol

A

eye problesm- optic neurtitis

79
Q

what oral sign is seen in roseola infantum

A

nagayama spots on uvula and hard palate

80
Q

complications of scartlet fever

A

rheumatic fever
otitis media
glomerulonephritis

81
Q

how does scartlet fever present?

A

fever, strawberry tongue
rash- spares pams, soles and oral region, starts on torso
rash is described as sandpaper rash

82
Q

Complications of measles

A

otitis media
pneumonia
encephalitis
subacute sclerosing panencephalitits (5-10 years after(

83
Q

How can you remember the criteria for kawasaki disease

A

CRASH and burn
Conjunctivitis (bilarteral)
Rash
Adenopathy (lymph node enlargement)
Strawberry tongue
Hand and feed peeling

Burn- fever> 39 for 5 days

84
Q

3 phases of kawasaki disease

A

acute
subacute
convalescent

85
Q

what is the pathophysiology of kawasaki disease?

A

systemic medium sized vessel vasculitis

86
Q

how is kawasaki treated ?

A

IV immunoglobulins and aspirin

87
Q

How do you differeniate polyarticular and oligoarticular JIA

A

poly= 5 or more joints in the first 6

88
Q

How is JIA defined

A

arthritis for more than 6 weeks in those under 16

89
Q

what criteria is used for septic arthritis ?

A

Kocher criteria

90
Q

what makes up the kocher criteria?

A
  1. fever >38.5
  2. refusal to weight bear
  3. WCC> 12,000 cells/mm3
  4. ECR >40 mm/h
91
Q

what is the most common cause of septic arthritis in children?

A

staph. aureus

92
Q

Who needs same day paeds assessment for transient synovitis

A

under 3 with a limp
any child with a fever and limp

93
Q

what does X ray show in perthes disease?

A

widening of the joint space

94
Q

if X ray doesn’t show changes in perthes disease what investigation should be done?

A

MRI

95
Q

what staging is used for perthes disease?

A

cattarall staging

96
Q

management of perthes :
- before 6 years
- after 6 years

A

before- observation
after - surgery

97
Q

gold standard investigation of osteomyelitis

A

bone marrow aspirate and culture

98
Q

If leg length discrepancy is found when looking for DDH what test can be done ?

A

galleazi test

99
Q

Who will have a hip USS at 6 weeks for DDH

A

those who were breech at or after 36 weeks
those with a first degree relative with DDH
multiple pregnancies

100
Q

When are all babies screened for DDH?

A

NIPE and at 6 week examination

101
Q

which hip is more common in DDH

A

left hip

102
Q

Describe neonatal rescusitation

A
  1. WARM BABY
  2. 5 inflation breaths
  3. repeat 5 inflation breaths
  4. 30 second ventilation breaths
  5. compressions:
    - rate of 3:1
    - using 2 fingers
    - 4cm deep
103
Q

what antibiotics are given in neonatal sepsis

A

IV benzypenicillin and gentamycin

104
Q

what classifies early onset neonatal sepsis

A

within the first 72 hours

105
Q

what level of blood glucose defines neonatal hypoglycaemia

A

<2.6 mmol/l

106
Q

When should dextrose be given for neonatal hypoglycaemia

A

if symptomatic or <1.5mmol/l

107
Q

Which of caput succedanium and cephalohaematoma crosses the suture lines?

A

caput succendanium

108
Q

what staging system is used to calculate the severity of hypoxic ischaemic encephalopathy

A

sanart staging system

109
Q

define physiological jaundice

A

jaundice that occurs after 24 hours and less than 2 weeks in term infants and 3 weeks in premature infants

110
Q

two treatment options for jaundice

A

phototherapy
exchange transfusion

111
Q

what are the three types of pathological jaundice

A

early onset (<24 hours)
late onset (after 2 or 3 weeks)
conjugated

112
Q

3 reasons why physiological jaundice occurs

A

high Hb concentration at birth so increased breakdown
fetal haemoglobin has a short lifespan (70 not 120)
hepatic bilirubin metabolism is less efficient in the first few days of life

113
Q

How does kernicterus present?

A

lethargy-> hypotonia-> hypertonia
poor feeding
fever
high pitched cry
opisthotonos (Arching position)

114
Q

what are some complications of kernicterus

A

athetoid cerebral palsy
deafness
low IQ

115
Q

what are some causes of prematurity

A

previous preterm birth
cervical insufficiency
multiple pregnancy
placental insufficiency
iatrogenic

116
Q

What are the different classes of prematurity

A

<28= extreme
28-32= very
32-37= moderate

117
Q

What are some early complications of prematurity

A

RDS
hypothermia
hypoglycaemia
retinopathy of prematurity
poor feeding
apnoeas and bradycardias
NEX
immature immune system and infection
neonatal jaundice

118
Q

what are some long term complications of prematurity

A

chronic lung disease of prematurity (bronchopulmonary dysplasia)
learning and behavioural difficulties
hearing and visual impairments
cerebral palsy
resp infection

119
Q

before what gestation would you give steroids for before birth

A

36 weeks

120
Q

before what gestation would you give IV mag sulf before birth

A

34 weeks

121
Q

explain the pathophysiology of retinopathy of prematurity

A

abnormal development of the blood vessels in the retina-> may occur as a result of o2 therapy as hypoxia stimulates growth of the blood vessels

122
Q

how is retinopathy of prematurity treated?

A

transpulmonary laser photocoagulation

123
Q

who is screened for retinopathy of prematurity

A

babies born less than 32 weeks and those less than 1.5kg

124
Q

what two things are seen on chest xray in transient tachypnoea of the neonate ?

A

hyperinflation
fluid in the horizontal fissure

125
Q

RF for respiratory distress syndrome

A

caesarean
hypothermia
perinatal hypoxia
meconium aspiration
maternal diabetes
preamturity (<32(

126
Q

chest x ray appearance of resp distress syndrome

A

ground glass

127
Q

short term complications of resp distress syndrome

A

pneumothorax
infection
apnoea
NEC

128
Q

long term complications of resp distress syndrome

A

chronic lung disease of prematurity

129
Q

where in the bowel is most commonly affected by NEC?

A

distal ileum and proximal colon

130
Q

when does NEC commonly occur

A

second week of life

131
Q

what are two protective factors against NEC

A

antenatal corticosteroids and breastmilk feeds

132
Q

what sign is there for pneumatosis intestinalis?

A

soap bubble/halo sign

133
Q

Key sign seen in congenital rubella

A

absent red light reflex due to cataracts

134
Q

how does congential CMV present?

A

blueberry muffin rash
deafness
eye inflammation
microcephaly
seizures

135
Q

how does congenital HSV present?

A

craniofascial malformations
rash
deafness
fetal death

136
Q

Who should be given antibiotics for otitis media?

A
  • <2 with bilateral otitis media
  • with ear discharge (otorrhoea)
  • symptoms havent resolved after 3 days
137
Q

who needs specialist assessment for otitis media

A

under 3 months with temp >38
3-6 months with temp >39

138
Q

what antibiotic is given for otitis media?

A

amoxicillin

139
Q

what bacteria most commonly causes otitis media

A

strep pneumoniae

140
Q

what is pierre robin sequence

A

a rare congenital birt hdefect characterised by underdeveloped jaw (micrognathia), backwards displacement of the tongue (glossoptosis) and airway obstruction, and u shaped cleft palate

141
Q

which is more common cleft lip or cleft palate?

A

palate

142
Q

what are some RF for cleft lip and palate?

A

maternal anti-epileptic use
maternal diabetes and obesity
patau syndrome
kallman syndrome

143
Q

what medication can be given to prevent vasoocclusive crisis in sickle cell disease?

A

hydroxycarbamide

144
Q

gene that alpha thalassaemia is inherited from ?

A

chromosome 16

145
Q

gene that beta thalassaemia is inhertied from ?

A

chromosome 11

146
Q

what birth mark is caused by vascular malformations in the dermis ?

A

naevus flammeus (port wine stain)

147
Q

what condition is associated with port wine stains?

A

sturge weber syndrome

148
Q

when do cavernous hemangiomas arise and what is another name for them?

A

usually present in first month of life (not present at birth)
also called a strawberry naevus

149
Q

describe capillary hemangiomas

A

also called stalk bites- salmon pink macules on the upper eyelid, mid forehead and neck

150
Q

How can you differentiate wilms tumours and neuroblastomas ?

A

wilms tumours will not cross the midline whereas neuroblastomas will

151
Q

How do neuroblastomas present?

A

abdominal mass
pallor
weight loss
bone pain
limp
hepatomegaly

152
Q

what can be tested for in the urine of neuroblastomas?

A

there will be raised VMA and HVA levels

153
Q

what can be seen on x ray of a neuroblastoma ?

A

calcifications

154
Q

inheritance of retinoblastomas?

A

autosomal dominant

155
Q

How do osteosarcomas present on x ray?

A

poorly defined lesion with sunburst appearance

156
Q

how do ewings sarcomas present on x ray

A

lamellated- onion skinning

157
Q

How do ewings sarcomas present on MRI

A

large mass with evidence of necrosis and small blue round cells with clear systoplasms

158
Q

How do posterior cerebral artery strokes present?

A

contralateral homonymous hemianopia with macular sparing
visual agnosia

159
Q

How does lateral pontine syndrome differ from wallenberg’s

A

it has additional deafness

160
Q

How does webers syndrome present?

A

3rd nerve palsy and contralateral weakness of upper na lower limb

161
Q

How does a posterior communicating artery stroke present?

A

painful 3rd nerve palsy

162
Q

What scoring system is used to determine the disability level post stroke?

A

Barthel’s index

163
Q

1st and 2nd line management for secondary prevention of ischaemic stroke

A

1st line= clopidogrel
2nd line= aspirin and dipyridamole

164
Q

When would you do an immediate assessment for TIA

A

if on blood thinners or has a blood disorder.

165
Q

How is myasthenic crisis treated?

A

IV immunoglobulins and plasma exchange

166
Q

how do people with huntingtons disease usually die?

A

aspiration pneumonia

167
Q

what is deficient in huntingtons

A

GABA - and inhibitory neurotransmitter

168
Q

How does idiopathic intracranial hypertension present?

A

headache- worse on lying down
blurred vision
papilloedema
enlarged blind spot
6th nerve pasly

169
Q

what drugs are associated with idiopathic intracranial hypertension?

A

COCP
steroids
vitamin A
lithium
tetracyclines

170
Q

what medications might be used in the management of idiopathic intracranial hypertension?

A

acetazolamide
topiramate

171
Q

who is donzepil contraindicated in

A

those with bradycardia

172
Q

when should someone with bells palsy be referred?

A

3 weeks

173
Q

what is the main SE of levodopa

A

excessive motor activity (dyskinesia)

174
Q

what parkinsons drug can cause pulmonary fibrosis ?

A

dopamine agonsits- cabergoline

175
Q

list some dopamine agonists

A

bromocriptine
cabergoline
ropinorole

176
Q

what two drugs can be used to treat benign essential tremor ?

A

propranolol and primidone

177
Q

define seizures

A

transient episodes of abdnomal electrical activity in the brain

178
Q

How long must epileptics not drive cars after a seizure?

A

1 year (or 6 months if just a seizure and not epilepsy)

179
Q

How long must epileptics not drive lorries after a seizure?

A

10 years

180
Q

What defines bronchopulmonary dysplasia?

A

infants who still have an oxygen requirement at 36 weeks postmenstrual age (gestational age plus chronological age)

or who need it at 28 days

181
Q

Who is mainly affected by bronchopulmonary dysplasia?

A

those born before 28 weeks who experienced RDS and required oxygen therapy at birth.

182
Q

RF for bronchopulmonary dysplasia

A

intrauterine growth restriction
male
chorioamnionitis
smokng

183
Q

What measures may be taken to prevent the development of bronchopulmonary dysplasia?

A

-using CPAP instead of ventilation where possible
-minimising ventilation associated lung injury by monitoring tidal volumes
-high calorie nutrition
-using caffeine to stimulate resp effort

184
Q

What are some complications of bronchopulmonary dysplasia?

A

serious bronchiolitis
Cerebral palsy
intraventricular haemorrhages
cardiac dysfunction

185
Q

how should raised ICP be managed?

A
  1. elevate the patients head to 30 degrees
  2. IV mannitol
  3. controlled hyperventilation (hypocapnia causes vasoconstriction of the arteries)
  4. remove CSF- drain, VP shunt, repeat LPs
186
Q

what tracts are damaged i

A

3A (42 decks)

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