Paeds endo, musc, neuro, misc Flashcards Preview

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Flashcards in Paeds endo, musc, neuro, misc Deck (283)
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1
Q

What is the most common cause of CAH?

A

21-hydroxylase deficiency

2
Q

Recall the signs and symptoms of CAH

A

Virilisation of external genitalia
Salt-losing crisis
Tall stature

3
Q

What is the best initial investigation to do when there are ambiguous genitalia?

A

USS

4
Q

What is the confirmatory investigation used to diagnose CAH?

A

Raised plasma 17-alpha-hydroxyprogesterone - cannot do in a newborn

5
Q

What sodium and potassium levels are seen in a salt losing crisis?

A

Hyponatraemia, hyperkalaemia

6
Q

What is the surgical management option for CAH?

A

For girls there is corrective surgery at early puberty to make the genitalia look more female

7
Q

What is the medical management for CAH?

A

Lifelong hydrocortisone and fludrocortisone

8
Q

How should a salt-losing crisis be managed?

A

IV hydrocortisone, IV saline, IV dextrose

9
Q

What is the ‘classical triad’ of symptoms in DM?

A

Polydipsia, polyuria, weight loss

10
Q

Recall the diagnostic criteria for DM

A
  1. Symptoms + fasting >7 OR random >11.1
  2. No symptoms + fatsing >7 AND random >11.1
  3. No symptoms + OGTT >11.1
  4. HbA1c > 6.5%
11
Q

What OGTT result is considered ‘impaired’ glucose tolerance?

A

7.8-11.1

12
Q

What fasting glucose result is considered ‘impaired’ glucose tolerance?

A

6.1-7.0

13
Q

Recall the 1st and 2nd line options for insulin therapy

A

1st line = multiple daily injection basal-bolus: injections of short-acting insulin before meals, with 1 or more separate daily injections of intermediate acting insulin or long acting insulin analogue

2nd line = continuous SC insulin infusion (pump)

14
Q

Recall the names of 2 types of long acting insulin

A

Glargine, determir

15
Q

Recall the names of 3 types of short acting insulin

A

Lispro, apart, glulisine

16
Q

Why should site of SC insulin injection be regularly rotated in T1DM?

A

Avoidance of lipohypertrophy

17
Q

What should cap glucose be when fasting and after meals?

A

Fasting: 4-7

After meals: 5-9

18
Q

When does annual monitoring for retinopathy/ nephropathy/ neuropathy begin?

A

12 years

19
Q

How is DKA managed?

A
  1. Replace fluids: deficit + maintenance requirement
    Deficit volume = weight in kg x 10 x 5% for mild-mod DKA, or 10% for severe DKA
    Maintenance volume = LOWER for DKA, due to risk of cerebral oedema
    <10kg = 2mls/kg/hr
    10-40kg = 1ml/kg/hr
    40+kg = 40mls/hr
  2. After 1-2 hours, start insulin therapy - infuse at 0.05-0.1 units/kg/hour
20
Q

What are the symptoms of HHS?

A

Weakness, leg cramps, visual disturbances
N+V
MASSIVE DEHYDRATION
Focal neurology

21
Q

What is the best option for oral monotherapy in T2DM?

A

Metformin (biguanide)

22
Q

What medications can be added for tx-resistant T2DM?

A

Sulphonylureas (eg glibenclamide)

Alpha-glucosidase inhibitors (eg acarbose)

23
Q

Recall the 2 possible biochemical definitions of DKA

A
  1. Acidosis + bicarb <15

2. pH <7.3 + ketones >3

24
Q

How is DKA biochemically classified as mild/ mod/ severe?

A

Mild = pH <7.3, mod = <7.2. sev = <7.1

25
Q

Recall the volume of fluid resus needed in a DKA emergency?

A

If shocked: 20mls/kg bolus over 15 mins

If not shocked: 10mls/kg bolus over 60 mins

26
Q

How should fluids be replaced following the initial emergency in DKA?

A

Fluid deficit = 5% if mild-mod DKA, 10% if severe DKA
Deficit volume = weight x 10 x deficit%

Fluid maintenance in DKA specifically:
<10kg: 2mls/kg/hour
10-40kg: 1ml/kg/hour
40+kg: 40mls/kg/hour

27
Q

What is the maintenance requirement of fluids in non-DKA situations?

A

First 10kg = 100mls/kg/day (4mls/kg/hour)

Next 10kg = +50mls/kg/day (2mls/kg/hour)

Every kg above 20kg = +20mls/kg/day (1ml/kg/hour)

28
Q

Recall the % fluid deficit that correlates to each severity of DKA

A
Mild = 5%
Mod = 7%
Sev = 10%
29
Q

Calculate the fluid requirement of a 20kg boy in DKA, with pH 7.15, who has already received a 10ml/kg bolus (200ml) over 60 mins

A

Calculation: (deficit x weight x 10) - initial, “shocked” bolus over 48 hours

Deficit if pH = 7.15 = 5% (7 x 20 x 10) = 1000
Initial bolus - 200: 1400-200 = 1200 over 48 hours
1200/48 = 25mls/ hour
Maintenance = (10 x 100) + (10 x 50) = 1500mls over 24 hours

30
Q

When should insulin therapy be started in DKA?

A

After 1-2 hours of IV fluid replacement

31
Q

How should insulin dose be calculated?

A

0.05-0.1 units/ kg/ hour

32
Q

When should dextrose therapy be started in DKA?

A

When glucose is <14mmol/L

33
Q

Recall 2 important possible complications of DKA with their symptoms

A

Cerebral odoema: Cushing’s triad of raised ICP (bradycarida, HTN, irregular breathing)

Hypokalaemia (typically asymptomatic)

34
Q

What is the most common cause of delayed puberty in boys?

A

CDGP (constitutional delay of growth and puberty)

35
Q

Recall the parameters for referral in growth restriction

A

If >75th centile, only refer once it drops by >=3 centiles

If 25th-75th centile, only refer once it drops by >=2 centiles

If <25th centile, refer once centile drops by >=1 centiles

36
Q

What is the definition of delayed puberty in males and females?

A

Males: no testicular development (<4mL) by age of 14 years

Females: no breast development by age 13 years or no period by age 15

37
Q

Recall 3 causes of Constitutional Delay of Growth and Puberty

A

Chronic disease
Malnutrition
Psychiatric (eg depression/ AN)

38
Q

Recall some causes of hypogonadotrophic hypogonadism

A

Hypothalamopituitary disorders: panhypopituitarism, intercranial tumours
Kallman’s syndrome, PWS
Hypothyroidism (acquired)

39
Q

Recall some causes of hypergonadotrophic hypogonadism (high LH ad FSH)

A

Congenital: cryptochidism (absence of 1 or both testes from scrotum), Kleinfelter’s, Turner’s

Acquired: testicular torsion, chemotherapy, infection, trauma, AI

40
Q

How can delayed puberty be staged in boys vs girls?

A

Boys: Prader’s orchidometer
Girls: Tanner’s staging

41
Q

What non-gonadotrophin hormones are important to measure as part of initial investigation of delayed puberty?

A

TSH

Prolactin

42
Q

Recall 2 important imaging tests that may be done in investigation of delayed puberty?

A
Bone age (from wrist x ray)
MRI brain
43
Q

How should CDGP be managed?

A

1st line: reassure and offer observation - they have a fantastic prognosis

2nd line: short course of sex hormone therapy
Boys: IM testosterone (every 6 weeks for 6 months)
Girls: transdermal oestrogen (6 months) and cyclical progesterone once established

44
Q

How should primary testicular/ ovarian failure be treated?

A

Boys: regular testosterone injections
Girls: oestrogen replacement gradually

45
Q

What are the age parameters for ‘early normal’ vs precocious puberty?

A

Early normal: girls = 8-10, boys = 9-12

Precocious: girls = <8, boys = <9

46
Q

What are the different causes of gonadotrophin-dependent vs gonadotrophin-independent precocious puberty?

A

Gonadtrophin-DEPENDENT: often idiopathic, but may be due to CNS tumours

Gonadotrophin-INDEPENDENT: gonadal activation independent of HPG- may be due to ovarian (folllicular cyst, granuloma cell tumour, Leydig cell tuour, gonadoblastoma), or testicular pathology

47
Q

What is premature thelarce?

A

Isolated breast development before 8 years

48
Q

What does BL enlargement of the testicles indicate before puberty?

A

Gonadotrophin-dependent precocious puberty (intercranial lesion, ie optic glioma in NF1)

49
Q

What does UL enlargement of a testicle indicate pre-puberty?

A

Gonadal tumour

50
Q

What does small testes and precocious puberty indicate?

A

Tumour of CAH (adrenal cause)

51
Q

What is the gold standard investigation for precocious puberty?

A

GnRH stimulation test

52
Q

If CAH is suspected, what test should be done?

A

Urinary 17-OH progesterone

53
Q

What is the medical treatment for precocious puberty?

A

GnRH agonist + GH therapy if gonadotrophin-dependent

If gonadotrophin-independent: -
Testotoxicosis: ketoconazole/ cyproterone
CAH: hydrocortisone + GnRH agonist

54
Q

What are the 2 most common causes of dwarfism?

A

Achondroplasia

Hypochondroplasia

55
Q

What is the phenotype of achondroplasia?

A

Arms and legs short, normal length thorax

56
Q

What is the phenotype of hypochondroplasia

A

Small stature, micromelia (small extremities), large head

57
Q

What gene mutation is associated with achondroplasia and hypochondroplasia?

A

FGFR3 - autosomal dominant mutation

58
Q

Recall some signs and symptoms of osteogenesis imperfecta

A

Blue sclera, short stature, loose joints, hearing loss, breathing problems

59
Q

Other than short stature, what are the symptoms of achondroplasia?

A

Hydrocephalus, depression of nasal bridge, marked lumbar lordosis, trident hands

60
Q

What X ray findings may be present in achondroplasia?

A

Chevron deformity: metaphyseal irregularity

Flaring in long bones

61
Q

Recall 4 possible causes of cogenital hypothyroidism, and whether they are inherited or not

A

Thyroid gland defects (eg missing, ectopic) - not inherited

Disorder of thyroid hormone metabolism - inherited

Hypothalamopituitary dysfunction (eg tumours, ischaemic damage) - not inherited

Transient hypothyroidism (eg due to maternal carbimazole or Hashimotos, not inherited)

62
Q

Recall some signs and symptoms of congenital hypothyroidism including 3 unique symptoms

A

Unique: coarse features, macroglossia, umbilical hernia

Others: large fontanelles, low temp, jaundice, hypotonia, pleural effusion, short stature, oedema, etc

63
Q

How should congenital hypothyroidism be investigated and treated in an infant?

A

Pretty much the same as an adult

64
Q

What is the most common cause of acquired primary hypothyroidism?

A

Hashimoto’s autoimmune thyroiditis

65
Q

Recall 2 genetic risk factors for hashimoto’s thyroiditis?

A

Down’s, Turner’s

66
Q

What is the prognosis of growth for children with primary hypothyroidism?

A

Dw they can catch up :-)

67
Q

What are the signs and symptoms of hyperthyroidism in the foetus?

A

High CTG trace and foetal goitre on USS

68
Q

What are the signs and symptoms of hyperthyroidism in the neonate?

A

Same as adult

69
Q

What is the medical management of hyperthyroidism?

A

Carbimazole/ propothiouracil for 2 years

70
Q

What non-medical management options are there for hyperthyroidism in children?

A

Radioiodine treatment

Surgery

71
Q

How is severe obesity, obesity and overweightness defined in children?

A

Severely obese: 99th centile
Obese: >95th centile
Overweight: 85th-94th centile

72
Q

What causes impaired skeletal growth in rickets?

A

Inadequate mineralisation of bone laid down at the epiphyseal growth plates

73
Q

Recall 3 deficiencies that can cause rickets?

A

Calcium
Vit D
Phosphate

74
Q

What will an x ray show in Rickets?

A

Thickened and widened epiphysis
Cupping metaphysis
Bowing diaphysis

75
Q

What will be the biochemical picture in rickets?

A

Reduced calcium and phosphate
Raised

Diagnostic = calcium x phosphate <2.4

76
Q

How can rickets be treated?

A

Calcium supplements

Oral vitamin D2

77
Q

What are the most common causes of fracture in a neonate?

A

Clavicle - from shoulder dystocia

Humerus/ femur - from breech delivery

78
Q

What is the prognosis for neonatal fracture?

A

Great

79
Q

How old does a child have to be prescribed codeine and morphine?

A

16

80
Q

How should paediatric fractures be managed?

A

Pain management

Manipulation and reduction

81
Q

How old does a child need to be to get a intramedullary nail?

A

4

82
Q

What is the Ottawa ankle rule?

A

X ray only indicated if:

  • Pain in the malleolar zone AND bone tenderness at the posterior edge/ tip of the lateral malleolus OR
  • Pain in the malleolar zone AND bone tenderness at the posterior edge/ tip of the MEDIAL malleolus OR
  • An inability to bear weight both immediately and in the emergency department for four steps
83
Q

What is the Ottawa knee rule?

A

X ray only indicated if: age 55+ OR isolated patellar tenderness OR cannot flex to 90 degrees OR an inability to bear weight both immediately and in the ED for 4 steps

84
Q

What is Perthe’s disease?

A

Avascular necrosis of the femoral epiphysis from an interruption of blood supply, followed by revascularisation and reossification over 18-36 months

85
Q

In which age group/ gender is Perthe’s most common?

A

4-8 y/o boys

86
Q

Recall the signs and symptoms of Perthe’s

A

Insidious presentation: limp, knee pain, hip pain –> limb shortening

87
Q

What investigations should be done for Perthes?

A

X ray +/- MRI

Roll test

88
Q

What would an x ray show in Perthe’s disease?

A

Increased density of femoral head

89
Q

What is the roll test?

A

Patient supine, roll affected hip internally and externally –> guarding or spasm in Perthe’s

90
Q

How is Perthe’s managed?

A

Simple analgesia for pain management<6 years: observation - non-surgical containment using splints>6 years –> surgery

91
Q

What is Osgood Shlatter Disease?

A

Osteochondritis (inflammation of the cartilage/ bone) of the patellar tendon insertion at the knee

92
Q

Recall the most at-risk group for OSD?

A

10-15y who are physically active

93
Q

Recall the signs and symptoms of OSD?

A

Gradual onset knee pain after exercise that is relieved by rest

Localised tenderness and swelling over tibial tuberosity

Hamstring tightness

94
Q

What might be seen on X ray in OSD?

A

Fragmentation of the tibial tubercle and overlying soft tissue swelling

95
Q

How should OSD be managed?

A

Simple analgesic packs (intermittent)

Protective knee pads

Stretching

Reassure - this will resolve over time, but may persist until end of growth spurt

Advise stopping/ reducing all sporting activity

96
Q

What is chondromalacia patellae?

A

Anterior knee pain from degeneration of articular cartilage on posterior surface of patella

97
Q

What is the general cause of chondromalacia patellae?

A

Overuse in physical activity

98
Q

Recall the signs and symptoms of chondromalacia patellae

A

Anterior knee pain - exacerbated by movement –> painless passive movement but pain and grating sensation on repeated extension

99
Q

How should chondromalacia patellae be managed?

A

Physio

100
Q

What is osteochondritis dissecans?

A

Reduced blood flow causing cracks to form in the articular cartilage and subchondral bone –> avascular necrosis –> fragmentation of bone and cartilage with free movement of fragments –> activity related joint pain

101
Q

Recall the signs and symptoms of osteochondritis dissecans?

A

Pain after exercise - catching, locking and giving way

102
Q

What is osteomyelitis?

A

Infection of metaphysis of long bones, commonly the distal femur and proximal tibia

103
Q

What is the most common pathogen implicated in osteomyelitis?

A

Staph aureus

104
Q

What is the most common age group affected by osteomyelitis?

A

<5 years

105
Q

How does the presentation of osteomyelitis compare to the presentation of septic arthritis?

A

Usually chronic in onset and less severe than septic arthritis (over a week rather than a day)

106
Q

Recall the signs and symptoms of osteomyelitis?

A

Fever

Acute onset limb pain, immobile limb, skin swollen, tender and erythematous

107
Q

What investigations should be done for suspected osteomyelitis?

A

Septic screen
BCs and FBC
Joint aspiration and MC+S
XR –> MRI of joint (shows soft tissue)

108
Q

How should acute osteomyelitis be managed?

A

High dose IV empirical –> narrow spec Abx
1st line is flucloxacillin
Take BCs before staring IV Abx
Change to oral Abx as soon as CRP is back to normal
Surgical debridement may be necessary

109
Q

What is septic arthritis?

A

Infectious arthritis of the synovial joint (vs osteomyelitis of bone)

110
Q

Which joint is affected in 75% of cases of septic arthritis?

A

Hip

111
Q

What is the usual pathogen implicated in septic arthritis?

A

S aureus

112
Q

Recall the signs and symptoms of septic arthritis

A

Single joint warm, erythematous, tender, reduced range of movement, infants will hold the limb still

113
Q

What investigations should be done for septic arthritis?

A
SAME AS OSTEOMYELITIS
Septic screen
BCs and FBC
Joint aspiration and MC+S
XR --> MRI of joint (shows soft tissue)
114
Q

How should septic arthritis be managed?

A

Similar to osteomyelitis but not the same

IV flucloxacillin –> oral

Joint wash out and aspirated to dryness PRN

115
Q

What is Still’s disease?

A

Persistent joint swelling presenting before 16 years, in the absence of infection/ other defined cause

116
Q

What are the signs and symptoms of Still’s?

A
May be any number of joints
Gelling (stiffness after periods of rest)
Intermittent limp
Morning joint stiffness/ pain
Limited movement
117
Q

Recall some late signs of Still’s

A

Inflammation and bone expansion –> ‘knock knees’

Salmon-coloured rash (pathogenomic of systemic Still’s)

118
Q

What investigations should be done in suspected Still’s?

A

Clinical diagnosis mainly
Bloods + imaging to prove classification and prognostic info
ANA, FBC, RhF, CRP/ESR, anti-CCP
USS/ MRI

119
Q

How should Still’s be managed?

A
MDT rheuatology
NSAIDs
Corticosteroids (high to low dose)
DMARDs - if disease fails to respond to conventional treatments - methotrexate or sulfalazine
TNF alpha inhibitors if needed
120
Q

What is the prognosis for Still’s disease?

A

Most children can expect good disease control and quality of life
If poor disease control –> anterior uveitis and fractures

121
Q

What is the former name of reactive arthritis?

A

Reiter’s syndrome

122
Q

What are the most likely causative organisms in reactive arthritis?

A

Enteric bacteria - salmonella, shigella, campylobacter, yersinia

123
Q

Recall the signs and symptoms of reactive arthritis

A

Transient joint swelling (<6 weeks) following an extra-articular infection
Low grade fever

124
Q

Which joints are most typically affected by reactive arthritis?

A

Ankles/ knees

125
Q

What investigations should be done in suspected reactive arthritis?

A

A diagnosis of exclusion as no positive findings
Bloods (CRP normal or mildly elevated)
Normal XR

126
Q

How should reactive arthritis be managed?

A

Self-limting, NSAIDs will do for analgesia

127
Q

What is SUFE?

A

Displacement of epiphysis of femoral head posteroinferiorly

128
Q

Why does SUFE need prompt treatment?

A

To prevent avascular necrosis

129
Q

What age group is usually affected by SUFE?

A

10-15 years

130
Q

What is the biggest RF for SUFE?

A

Obesity

131
Q

What are the signs and symptoms of SUFE?

A

Limp/ hip pain +/- referred to the knee
Insidious or acute onset
“Loss of internal rotation of a flexed hip”
Trendelenburg gait positive

132
Q

What investigations should be done in suspected SUFE?

A

Hip XR in AP and frog-lateral view (both hips)

133
Q

How should SUFE be managed?

A

Analgesia, bed-bound

Surgical internal fixation at growth plate

134
Q

What is transient synovitis?

A

Irritable hip - 3-10 years

135
Q

What age group is affected by transient synovitis?

A

3-10 years

136
Q

What is a red flag for urgent hospital assesment in transient synovitis?

A

Age <3 with an acute limp

137
Q

What is the cause of transient synovitis?

A

Viral infection (so causes a low grade fever)

138
Q

How should transient synovitis be investigated and managed

A

Clinically - it is self-limiting

139
Q

What is DDH?

A

A spectrum of conditions affecting proximal femur and acetabulum - ranging from subluxation to frank dislocation

140
Q

What is true DDH?

A

Femoral head has a persistently abnormal relationship with the acetabulum–> abnormal bony development, premature arthritis and significant disability

141
Q

What are the 2 manoevres that are key to early detection of DDH?

A

Barlow - dislocate posteriorly out of the hip

Ortolani - relocate back into acetabulum on hip abduction

142
Q

What is the biggest RF for DDH?

A

Female

143
Q

Recall the signs and symptoms of DDH

A

Limp/ abnormal

Delayed crawling/ walking - toe-walking

Asymmetrical skin folds

Limb length discrepancy

144
Q

What age group are the Barlow and Ortolani manoevres appropriate for?

A

<6 months

145
Q

What other investigation can be done alongside B and O manoevres in suspected DDH?

A

USS

146
Q

What investigation should be done in children too old for B and O manoevres?

A

X ray

147
Q

What are the indications for USS neonatally regardless of presentation, and when is this USS done?

A

Born breech OR FHx pos of DDH

Done at 6 weeks

148
Q

How should DDH be managed in the newborn?

A
Pavlik harness (most will resolve spontaneously by 3-6w of age) 
This keeps hips flexed and abducted
149
Q

How should DDH be managed in those >6 months old?

A

Surgery if conservative measures fail or there is no progress with harness

150
Q

What is a reflex anoxic seizure?

A

Episodes of tonic clonic fitting due to cardiac asystole secondary to vagal inhibition

151
Q

What are the causes of reflex anoxic seizure?

A

Can be triggered by pain, head trauma, cold food (ice cream), fright, fever

152
Q

What is a febrile convulsion?

A

A seizure and fever in the absence of intracranial infection

153
Q

What is the age group that can be affected by febrile convulsions?

A

6m to 3y

154
Q

What are the signs and symptoms of febrile convulsions?

A

Generalised tonic clonic seizure on background of fever

155
Q

What is the difference between a simple and complex febrile seizure?

A

Simple: do not cause brain damage and no increased risk of epilepsy

Complex: focal, <15 mins, repeated in same illness, increased risk of subsequent epilepsy

156
Q

What investigations should be done following a febrile seizure?

A

Identify and manage the cause of fever

No other main investigations (don’t do an EEG)

May potentially want to screen for meningitis/ encephalitis, do a urine MC+S and blood glucose

157
Q

How should febrile convulsion be managed during the seizure?

A

Protect from injury and do not restrain

If seizure lasts <5 minutes do nothing

If seizure lasts >5 mins and no drugs are available, call an ambulance

If drugs are available: after 5 mins administer PR diazepam OR buccal midazolam

158
Q

When should an ambulance be called for febrile convulsion?

A

1st seizure - of any duration or cause
Seizure lasts >5 mins with no drugs
Breathing difficuties

159
Q

Which children should be admitted to hospital following a febrile convulsion?

A

First febrile seizure<18 months old

Diagnostic uncertainty about the cause

Complex febrile seizure

Currently on Abx

160
Q

What system is used to classify seizures?

A

International League Against Epilepsy 2017

161
Q

What is the correct name for an absence seizure these days?

A

Generalised non-motor seizure

162
Q

What is the difference in symptoms between tonic-clonic and myoclonic?

A

Tonic clonic is the big one with violent muscle contractions, eyes rollingback, tongue biting, incontinence and both aura and post-ictal phenomena

Myoclonic = brief arrhythmic muscular jerking movement

163
Q

What is the most common form of childhood epilepsy?

A

Benign Rolandic Epilepsy - myoclonic seizures in a 3-12 year old

164
Q

What are the 3 different types of myoclonic epilepsy

A

Benign rolandic (3-12y)
Juvenile myoclonic epilepsy (12-18y)
Progressive myoclonic epilepsy

165
Q

What is the other name for benign rolandic seizures?

A

Sylvian seizures

166
Q

What are the signs and symptoms of benign rolandic epilepsy?

A

Myoclonic seizures during sleep involving face and UL with hypersalivation

167
Q

What are the signs and symptoms of juvenile myoclonic epilepsy?

A

Myoclonic seizures involving upper body just after waking up and beginning after puberty

168
Q

What is progressive myoclonic epilepsy?

A

Rare syndromes of combination of myoclonc and tonic-clonic seizures, with patient deteriorating over time

169
Q

For what type of epilepsy is treatment not usually given?

A

Benign Rolandic Epilepsy

170
Q

What type of antiepileptic actually exacerbates myoclonic seizures?

A

Lamotrigine

171
Q

What type of antiepileptic actually exacerbates absence seizures?

A

Carbamazapine

172
Q

What is the 1st line for rescue therapy for prolonged epileptic seizures?

A

Buccal midazolam

173
Q

Which antiepleptic drug requires monitoring?

A

Carbamazapine

174
Q

After how long seizure-free can antiepileptic therapy be stopped?

A

2 years

175
Q

Recall 2 Side effects of valporate

A

Weight gain, hair loss

176
Q

How is status epilepticus defined?

A

1 seizure lasting >5 mins OR
>2 seizures within a 5 min period without the person returning to normal between them 1 febrile seizures lasting >30 mins

177
Q

What is the age range of peak incidence for infantile spasm?

A

3-8 months

178
Q

What is an infantile spasm?

A

Sudden, rapid, tonic contraction of trunk and limb muscles with gradual relaxation over 0.5-2 seconds

179
Q

What is a ‘Salaam’ attack?

A

Head goes down and arms go up in the air

180
Q

How should West Syndrome be investigated?

A

EEG - shows hypoarrhythmias

181
Q

What is the prognosis for West syndrome?

A

Poor

182
Q

How should West syndrome be managed?

A

Vigabatrin or corticosteroids

183
Q

What investigations should be done for vasovagal syncope?

A

Lying and standing BP with ECG if indicated

FBC (to query anaemia/ bleeding)

184
Q

What are the indications for CT head in children?

A
Head injury + at least 1 of:
- suspected NAI
- post-traumatic seizure
- GCS <14
- Suspected skull fracture
- Focal neurology 
OR 
2 other risk factors
(LOC >5 mins, abnormal drowsiness, >3 episodes of vomiting, high-impact injury, amnesia > 5 mins)
185
Q

Give 2 signs of tear of the MMA

A

Battle sign

Racoon eyes

186
Q

What are the signs and symptoms of extradural haemorrhage?

A

Lucid interval followed by deterioration

Potential focal neurology

187
Q

How should extradural haemorrhage be managed?

A

Fluid resuscitation to correct hypovolaemia

Evacuation of haematoma and arrest bleeding

188
Q

What is the cause of subdural haemorrhage?

A

Tear in vein as it crosses subdural space

189
Q

What is the main symptom of subdural?

A

Gradually decreasing GCS

190
Q

What is often the cause of subdural in infants?

A

NAI

191
Q

Which infants are most at risk of intraventricular haemorrhage?

A

Premature babies due to VLBW

192
Q

What are the 2 main causes of intraventricular haemorrhage in infants?

A

ECMO in preterm babies with ARDS
Congenital CMV infection

ECMO = extracorporeal membrane oxygenation

193
Q

Recall the signs and symptoms of intraventricular haemorrhage

A

Sleepiness and lethargy, apnoea, reduced

Moro reflex, low tone, tense fontanelle

194
Q

Which investigation is used to diagnose intraventricular haemorrhage in a baby?

A

Trans-fontanelle USS

195
Q

How should intraventricular haemorrhage be treated in a baby?

A

Fluids
Anticonvulsant
Acetazolamide (to reduce CSF) and LP
Ventriculo-peritoneal shunt if hydrocephalus

196
Q

What is the difference between communicating and non-communicating hydrocephalus?

A

Communicatig: CSF flow obstructed after it enters the ventricles
Non-communicating: flow of CSF is obstructed WITHIN the ventricles

197
Q

What are the 2 causes of communicating hydrocephalus?

A

Meningitis

SAH

198
Q

What are the possible causes of non-communicating hydrocephalus?

A

Aqueduct stenosis:
Congenital causes: Dandy-Walker malformation or Chiari malformation

Acquired: IVH/ tumour

199
Q

What is the sunset sign?

A

Eyes appear to be driven down bilaterally - sign of raised ICP

200
Q

What are the 2 key investigations for hydrocephalus?

A

Cranial USS

Measurement of head circumference

201
Q

What is the first line management for hydrocephalus?

A

Ventriculoperitoneal shunt

202
Q

What is the second line management for hydrocephalus?

A

Furosemide - to inhibit CSF production

203
Q

What % of migraines have aura?

A

10%

204
Q

Is migraine likely to be UL or BL?

A

Could be either

205
Q

What are the symptoms of cluster headaches?

A

UL (eyes/ side of face) sharp throbbing pain, causing swelling/ watering inclusters

206
Q

What are some signs that migraine is secondary to something more sinister?

A

Visual field defects, gait or cranial nerve abnormalities, growth failure, papilloedema, early morning headache

207
Q

If simple analgesia doesn’t work, what is the 2nd line pain med in migraines?

A

Nasal sumatriptan

208
Q

Recall 2 prophylactic medications for migraine

A

Topiramate

Propranolol

209
Q

What is Tourette’s?

A

Chronic and multiple tics - starting before 18y old and persisting >1 year

210
Q

What are the 3 types of tic?

A

Motor, vocal or phonic

211
Q

Recall 2 therapies that are useful in tics

A
  1. Habit reversal therapy (learn movements to ‘compete’ with tics)
  2. Exposure with response prevention - help the child get used to the unpleasant feeling before a tic so that they can stop the tic occuring
212
Q

What is the first line medication for tic disorder?

A

Antipsychotics - eg risperidone

213
Q

What is the inheritance pattern of Duchenne muscular dystrophy?

A

x-linked recessive

214
Q

At what age does duchenne muscular dystrophy present, and at what age can it be diagnosed?

A

1-3y presentation

Can be diagnosed at 5y

215
Q

Which gene is deleted in Duchenne muscular dystrophy?

A

Dystrophin

216
Q

Describe the pathophysiology of duchenne muscular dystrophy?

A

Dystophin gene connects cytoskeleton to muscle fibres to ECM through membrane

Where deficient –> influx of Ca –> calmodulin breakdown –> excess free radicals –> myofibre necrosis

217
Q

What type of gait is seen in Duchenne muscular dystrophy?

A

Waddling

218
Q

Other than gait, what else is affected in early DMD?

A

Language delay

219
Q

What is Gower’s sign?

A

The need to turn prone to rise - seen in DMD

220
Q

How is the heart affected by Duchenne muscular dystrophy?

A

Primary dilated cardiomyopathy

221
Q

What is elevated in the plasma in Duchenne muscular dystrophy?

A

CKP (creatine phosphokinase) due to myofibre necrosis

222
Q

How should Duchenne muscular dystrophy be managed?

A

No cure - often management is to alleviate the symptoms
Physiotherapy to clear lungs and exercise to help prevent contractures
Medical:
- CPAP (due to weakness of intercostals)
- Glucocorticoids (to delay need for wheelchair)
- Cardioprotective drugs

223
Q

What is the difference between Beck’s MD and DMD?

A

Same signs and symptoms but often less severe and progresses at a slower rate

224
Q

What is the genetic cause of myotonic muscular dystrophy?

A

Autosomal dominant trinucleotide repeat disorder

225
Q

When does myotonic muscular dystrophy present?

A

20s to 30s

226
Q

In what way is myotonic muscular dystrophy the reverse of duchenne muscular dystrophy?

A

It affects small muscles more than large muscles

227
Q

Which form of MEN is NF1 associated with?

A

MEN2

228
Q

Recall some signs and symptoms of NF1

A

Café au lait spots
Neurofibroma
Axillary freckles
Lisch nodule

229
Q

What is tuberous sclerosis?

A

Rare genetic condition that causes mainly benign tumours to develop in different parts of the body

230
Q

Recall 3 cutaneous features of tuberous sclerosis

A

“Ash leaf” patch
Shagreen patches (rough skin on lumbar spine)
Angiofibromata (butterfly facial distribution)

231
Q

Recall some neurological features of tuberous sclerosis

A

Infantile spasms
Focal epilepsy
Intellectual disability (often with ASD)

232
Q

Why is there a risk of hydrocephalus in tuberous sclerosis?

A

Development of subependymal giant cell astrocytoma

233
Q

Which investigations are useful for diagnosing tuberous sclerosis?

A

CT/ MRI

234
Q

What is the layman’s term for the haemangioma in Sturge Weber syndrome?

A

Port wine stain

235
Q

Where does the haemangiomatous facial lesion present in Sturge Weber Syndrome?

A

Trigeminal nerve distribution

236
Q

Recall some signs and symptoms of Sturge Weber Syndrome

A
Epilepsy 
Contralateral hemiplegia
Phaeochromocytoma
Intellectual disability
Glaucoma
237
Q

How should Sturge Weber Syndrome diagnosis be confirmed?

A

MRI

238
Q

What are the 3 symptoms that may present via somatisation?

A

Abdo pain
Reucrrent headaches
Limb pain

239
Q

What is Apley’s rule?

A

The further the pain is from the umbilicus, the more likely the pain is of an organic nature

240
Q

What is the 1st line management for somatisation?

A

Promote communication between family and children

Pain-coping skills ie relaxation techniques for headaches

241
Q

What should be done if 1st line treatments for somatisation fail?

A

Referral to CAMHS

242
Q

Which type of developmental delay has the best prognosis?

A

Isolated delay (global has association with syndromes that have poorer prognosis)

243
Q

How does the aetiology of conjunctivitis differ between children and adults?

A

In adults is usually viral or allergic, in children is more likely to be bacterial

244
Q

What is the most likely cause of sticky eyes in a <48 hours neonate?

A

Gonorrhoea

245
Q

What is the most likely cause of sticky eyes in a neonate in first 1-2w?

A

Chlamydia - often co-presents with pneumonia

246
Q

How should neonatal gonorrhoeal infection be investigated and treated?

A

Gram stain and culture

3rd gen cephalosporin (eg ceftriaxone)

247
Q

How should neonatal chlamydia infection be investigated and treated?

A

Immunofluorescent staining

Oral erythromycin

248
Q

Which is more common in children out of hypermetropia and myopia?

A

Hypermetropia

249
Q

What is the cause of Retinopathy of Prematurity?

A

Vascular proliferation leads to retinal detachment –> fibrosis and blindness

250
Q

What is the main RF for retinopathy of prematurity?

A

Uncontrolled use of high concentrations of oxygen

251
Q

What are the 2 key signs of retinopathy of prematurity?

A

Unusual eye movements

White pupils

252
Q

What is the first line treatment of retinopathy of prematurity?

A

Laser photocoagulation

253
Q

What is strabismus?

A

Abnormal alignment of eyes

254
Q

At what age is strabismus diagnosed?

A

1-4 years

255
Q

What are the 2 types of strabismus?

A

Non-paralytic (refractive error in one or more eyes)

Paralytic (squinting eye could be caused by motor nerve paralysis or SOL - 3rd nerve palsy)

256
Q

How should strabismus be managed?

A

1st line = glasses
2nd line = eye patching
3rd line = eye drops
4th line = eye muscle surgery

257
Q

Which burn pattern is typical of NAI?

A

Glove and stocking

258
Q

Which type of long bone fracture is indicative of NAI?

A

Spiral fractures

259
Q

What is the classic triad of features in shaken-baby syndrome?

A
  1. Retinal haemorrhages
  2. CT showing brain swelling/ encephalopathy
  3. CT showing subdural haematoma
260
Q

What needs to be ruled out in suspected NAI?

A

Leukaemia, ITP and haemophilia

261
Q

What is the definition of SIDS?

A

Deaths which remain unexplained after a post-mortem

262
Q

What is the peak age for SIDS?

A

2-4 months

263
Q

Recall some important risk factors for SIDS

A

Front-sleeping baby

Prematurity, LBW, male, maternal smoking, microenvironment (pillow, heat)

264
Q

For how long should parents share a room with baby?

A

6 months

265
Q

Which organisation provides support for SIDS?

A

Lullaby Trust

266
Q

For how long should a child be excluded from school if they have scarlet fever?

A

24 hours after antibiotics

267
Q

For how long should a child be excluded from school if they have whooping cough?

A

48 hours after Abx

268
Q

For how long should a child be excluded from school if they have measles?

A

4 days from onset of rash

269
Q

For how long should a child be excluded from school if they have rubella?

A

4 days from onset of rash

270
Q

For how long should a child be excluded from school if they have chickenpox?

A

Until all lesions crusted over

271
Q

For how long should a child be excluded from school if they have impetigo?

A

Until all lesions crusted over

272
Q

For how long should a child be excluded from school if they have mumps?

A

5 days from onset of swollen glands

273
Q

For how long should a child be excluded from school if they have influenza?

A

Until recovered

274
Q

When do fontanelle close by?

A

1 year usually, can be as late as 2

275
Q

What should be given before buccal midazolam in a fitting child?

A

Oxygen

276
Q

What infections are children with DiGeorge syndrome particularly at risk of and why?

A

Candidiasis

No thymus –> no T cells

277
Q

Give 2 causes of evanescent salmon pink rash

A

Listeriosis (neonate)

Juvenile idiopathic arthritis

278
Q

Why shouldn’t metoclopramide be given to children?

A

Can give oculogyric crises

279
Q

Recall 3 conditions that cause rigors in children?

A

Pyelonephritis
Influenza
Malaria

280
Q

How should burns be managed medically?

A

Cover for potential toxic shock syndrome with ceftriaxone and clindamycin

281
Q

How can the % of Total Burn Surface Area be measured OE?

A

Hand is 1% as a rough guide

282
Q

Recall the 5 points of the Fraser guidelines

A
  1. YP understands professional’s advice
  2. YP cannot be persuaded to inform parents
  3. YP is likely to begin/ continue having sex regardless of contraception
  4. Physical/ mental wellbeing likely to suffer from lack of contraception
  5. YP’s BI require them
283
Q

What % of children who have a febrile convulsion will have one again?

A

33-50%