Paediatrics Flashcards

1
Q

What is vesico-ureteric reflux?

A

Backflow of urine from bladder to ureter and kidney

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2
Q

Presentation of vesico-ureteric reflux

A

Hydronephrosis on antenatal U/S
Recurrent childhood UTI
Reflux nephropathy (chronic pyleonephritis secondary to VUR)

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3
Q

Grade 1 VUR

A

Reflux to ureter only, no dilation

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4
Q

Grade 2 VUR

A

Reflux to renal pelvis on micturation, no dilation

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5
Q

Grade 3 VUR

A

Mild/moderate dilation of ureter, renal pelvis and calyces

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6
Q

Grade 4 VUR

A

Dilation of renal pelvis and calyces, moderate ureteral tortuosity

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7
Q

Grade 5 VUR

A

Gross dilation of ureter, pelvis and calyces with ureteral tortuosity

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8
Q

Management of vesico-ureteric reflux

A
Mild likely to self resolve
Prophylactic antibiotics
Management of hypertension
Gel injected into end of ureter
Surgical ureteral implantation (rare)
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9
Q

Investigations for vesico-ureteric reflux

A

Micturating cystourethrogram

DMSA scan to look for renal scarring

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10
Q

Define enuresis

A

Involuntary discharge of urine by day or night or both, in a child aged 5 years or older, in the absence of congenital or acquired defects of the nervous system or urinary tract

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11
Q

Types of noctural enuresis

A

Primary: child never had continence

Secondary: child dry for 6 months or more prior

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12
Q

What conditions do you need to exclude when seeing a child with noctural enuresis?

A

Diabetes
UTI
Constipation

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13
Q

Management of noctural enuresis

A

Check for underlying causes
Advise on fluids, diet, toileting behaviour
Star chart for behaviours (e.g. toilet before bed), not dry nights

Enuresis alarm under 7 years

Desmopressin over 7 years

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14
Q

Inheritance of homocystinuria

A

Autosomal recessive

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15
Q

What is homocystinuria?

A

Deficiency in cystathionine beta synthase

Causes increased blood and urine homocysteine

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16
Q

Presentation of homocystinuria

A

In early childhood

Tall, fair hair
Marfanoid appearance
Kyphoscoliosis
Atherosclerosis, stroke
Low IQ
Epilepsy
Glaucoma
Downwards lens dislocation
Cataracts
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17
Q

Management of homocysteinuria

A

Vitamin B6
Folate

Half of children won’t respond to B6 and will need a low methionine diet

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18
Q

What is a low methionine diet?

A

Largely vegan
Protein from nuts, lentils
Minimise protein from meats, eggs, milk

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19
Q

What is developmental dysplasia of the hip?

A

Spectrum of disease from mild acetabular dysplasia to frankly dislocated femoral head

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20
Q

Risk factors for developmental dysplasia of the hip

A
Female > Male 6:1
Breech
Family history 
First born child
Oligohydramnios
Birth weight >5kg
Congenital calcaneovalgus foot deformity
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21
Q

What percentage of children are born with developmental dysplasia of the hip?

A

1-3%

20% cases bilateral
Left hip affected more than right

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22
Q

Screening for developmental dysplasia of the hip

A

Routine ultrasound if: first degree relative of hip problems at early age, breech at or after 36 weeks, multiple pregnancy

All infants screened at birth and 6 weeks with Barlow and Ortolani

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23
Q

Examination for developmental dysplasia of the hip

A

Barlow - attempts to dislocate an articulated femoral head

Ortolani - attemps to relocate a dislocated femoral head

Symmetry of leg length

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24
Q

Imaging for developmental dysplasia of the hip

A

Ultrasound

Xray if >4.5 months

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25
Q

Management of developmental dysplasia of the hip

A

Most stabilise themselves by 3-6 weeks
Pavlik harness if under 5 months
Older children may need surgery

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26
Q

Which children tend to be affected by slipped upper femoral epiphysis?

A

Age 10-15
Males
Obese

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27
Q

What is slipped upper femoral epiphysis?

A

Displacement of the femoral head epiphysis postero-inferiorly

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28
Q

Features of slipped upper femoral epiphysis

A

Acutely after trauma or chronic symptoms

Hip, groin, medial thigh or knee pain
Limp
Loss of internal rotation of the leg in flexion

Bilateral slip in 20%

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29
Q

Investigations of slipped upper femoral epiphysis

A

Xray hip - AP and lateral (frog leg)

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30
Q

Management of slipped upper femoral epiphysis

A

Internal fixation

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31
Q

What is Perthe’s disease?

A

Avascular necrosis of the femoral epiphyses

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32
Q

Features of Perthe’s disease

A

Limp
Hip pain - progressive over a few weeks
Stiffness
Reduced ROM

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33
Q

Investigations for Perthe’s disease

A

Xray: widening of joint space, decreased femoral head size / flattening of femoral head

Bone scan or MRI if normal xray and persistent symptoms

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34
Q

Management of Perthe’s disease

A

<6 years: observe
Older children: surgery, cast, braces

Most will resolve with conservative management

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35
Q

Who is affected by Perthe’s disease?

A

Age 4-8
M>F 5:1
10% cases bilateral

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36
Q

What is ankyloglossia?

A

Tongue tie

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37
Q

Management of croup

A

Dexamethasone 0.15mg/kg
High flow oxygen
Nebulised adrenaline

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38
Q

Main cause of croup

A

Parainfluenza

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39
Q

Features prompting admission in croup

A

Moderate or severe
Under 6 months
Uncertainty of diagnosis
Known upper airway abnormalities (Downs, laryngomalacia)

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40
Q

What is the average age of adoption in the UK?

A

4 years

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41
Q

School exclusion in scarlet fever

A

24 hours after starting antibiotics

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42
Q

School exclusion in whooping cough

A

2 days after starting antibiotics

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43
Q

School exclusion in measels

A

4 days from onset of rash

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44
Q

School exclusion in rubella

A

5 days from onset of rash

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45
Q

School exclusion in chickenpox

A

Until all lesions have crusted over

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46
Q

School exclusion in mumps

A

5 days from onset of swollen glands

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47
Q

School exclusion in impetigo

A

Until lesions are crusted and healed, or 48 hours after antibiotic treatment

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48
Q

School exclusion in scabies

A

Until treated

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49
Q

School exclusion in influenza

A

Until recovered

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50
Q

Newborn hearing screening test

A

Otoacoustic emissions

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51
Q

Newborn and infant hearing test if they fail screening

A

Auditory brianstem response test

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52
Q

Hearing test age 6-9 months

A

Distraction test

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53
Q

Hearing test age 18 months-2.5 years

A

Recognition of familiar objects “where’s the teddy?”

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54
Q

Hearing test >2.5 years

A

1) Performance testing

2) Speech discrimination tests

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55
Q

Hearing test >3 years

A

Pure tone audiometry

Done at school entry

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56
Q

What screening tests are done for hearing?

A

Otoacoustic emissions at newborn

Pure tone audiometry at school entry

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57
Q

How common is an undescended teste?

A

2-3% of term infants
Higher if preterm
25% cases bilateral

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58
Q

Complications of an undescended teste

A

Infertility
Torsion
Testicular cancer
Psychological

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59
Q

Management of a unilateral undescended teste

A

Refer at 3 months if persistent, seen by urologist by 6 months
Consider orchidoplexy around 1 year

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60
Q

Management of bilateral undescended teste

A

If at birth: urgent review, endocrine/genetic testing

If at 6-8 weeks: urgent 2 week referral to paediatrics

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61
Q

Conditions associated with Wilm’s tumour

A

Beckwith-Wiedermann syndrome
WAGR syndrome
Hemihypertrophy

1/3rd cases have loss of function mutation in WT1 gene on chromosome 11

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62
Q

Features of Wilm’s tumour

A
Abdominal mass
Flank pain
Painless haematuria
UTI
Systemic features - poor appetite, fever, weight loss
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63
Q

At what age do Wilm’s tumours present?

A

Typically under 5

Median age 3 years

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64
Q

Management of Wilm’s tumours

A

Nephrectomy
Chemotherapy
Radiotherapy if advanced disease

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65
Q

Other word used for Wilm’s tumour

A

Nephroblastoma

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66
Q

What percentage of Wilm’s tumours have metastases at presentation?

A

20%

Most commonly to lung

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67
Q

Features of growing pains

A

Never present at the start of the day after waking
No limp
No limitation on physical activity
Systemically well
Normal physical examination
Motor milestones normal
Symptoms often intermittent and worse after a day with lots of activity

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68
Q

Protective factors for sudden infant death syndrome

A

Breast feeding
Room sharing
Use of dummies

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69
Q

Risk factors for sudden infant death syndrome

A

MAJOR:
Prone sleeping, Parental smoking, Prematurity, Bed sharing, Hyperthermia or head covering

OTHER: 
Male sex, Multiple birth, Social class IV and V, Maternal drug use, Winter
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70
Q

What temperature should the vaccine fridge be kept at?

A

+2 to +8 degrees

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71
Q

Prevalence of iron deficiency anaemia in children

A

10%

Higher in Asian, Afro-Caribbean, Chinese

Preterm babies at high risk

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72
Q

Causes of iron deficiency anaemia in children

A

Diet is primary
Malabsorption - check for coeliac
Bleeding
Outside the UK: malaria, parasites, HIV, TB

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73
Q

Management of iron deficiency anaemia in children

A
Preterm: 5mg elemental iron till weaned
Breast/formula milk till 1 year
Restrict cows milk 
Iron rich weaning foods
Vit C rich weaning foods
3-6mg/kg daily iron
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74
Q

Causes of Hand, Foot and Mouth Disease

A

Coxsackie A16

Less often enterovirus 71

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75
Q

Features of hand, foot and mouth disease

A

Systemic upset - fever, sore throat
Oral ulcers
Vesicles on palms and soles

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76
Q

Management of hand, foot and mouth disease

A

Symptomatic

No need for school exclusion

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77
Q

What is laryngomalacia?

A

Common benign cause of noisy breathing

Softening of the larynx, causing collapse during inspiration

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78
Q

Presentation of laryngomalacia

A

Noisy breathing/intermittent stridor
Around 4 weeks old

Usually self resolves by age 2 years

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79
Q

Cause of scarlet fever

A

Group A haemolytic streptococci

usually strep pyogenes

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80
Q

Presentation of scarlet fever

A

Incubation 2-4 days

Fever
Malaise
Tonsilitis
Strawberry tongue
Rash - fine erythema, starts on torso, facial sparing, 'sandpaper' texture
Desquamination later
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81
Q

Management of scarlet fever

A

Oral penicillin

Azithromycin if penicillin allergic

Notifiable disease

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82
Q

Complications of scarlet fever

A

Otitis media
Rheumatic fever - 20 days after infection
Acute glomerulonephritis - 10 days after infection

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83
Q

Central causes of hypotonia

A

Down’s syndrome
Prader Willi syndrome
Hypothyroidism
Cerebral palsy

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84
Q

Neurological and muscular causes of hypotonia

A
Spinal muscular atrophy
Spina bifida
Guillian-Barre syndrome
Myasthenia gravis
Muscular dystrophy
Myotonic dystrophy
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85
Q

Management of paediatric migraine

A

NSAIDS
Triptans for over 12 years - sumatriptan nasal spray

Prophylaxis:
1st line - propranolol, pizotifen
2nd line - valproate, topiramate, amitryptilline

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86
Q

Cause of roseola infantum

A

Human herpes virus 6

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87
Q

Features of roseola infantum

A

Incubation 5-15 days
High fever, lasting a few days
Maculopapular rash follows fever, starts on neck and trunk
Nagayama spots - papular enanthem on uvula and soft palate
Febrile convulsions in 10-15%
Diarrhoea
Cough

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88
Q

Management of roseola infantum

A

No school exclusion

Symptomatic management

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89
Q

Treatment options for head lice

A

Wet combing
Malathion
Dimeticone

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90
Q

Cause of head lice

A

Pediculus capitis (parasite)

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91
Q

Features of chickenpox

A

Fever initially
Itchy rash starts on head/trunk
Rash is macular then papular then vesicular
Mild systemic upset

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92
Q

Management of chickenpox

A

Supportive
Keep cool, trim nails, calamine
Infective till all lesions crusted

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93
Q

Management when immunocompromised people are exposed to chicken pox?

A

VZIG for immunocompromised or newborns exposed

If chicken pox develops then IV aciclovir

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94
Q

Infectivity of chicken pox

A

4 days before rash until 5 days after rash first developed or when all lesions have crusted

Incubation is 10 to 21 days

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95
Q

Complications of chicken pox

A

Secondary bacterial infection (NSAIDs increase the risk)
Pneumonia
Encephalitis
Disseminated haemorrhagic chickenpox

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96
Q

Patau syndrome - what is the chromosome abnormality?

A

Trisomy 13

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97
Q

Patau syndrome key features

A
Microcephalic
Small eyes
Cleft lip/palate
Polydactyly
Scalp lesions
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98
Q

Edward’s syndrome genetics

A

Trisomy 18

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99
Q

Edward’s syndrome key features

A

Micrognathia
Low set ears
Rocker bottom feet
Overlapping of fingers

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100
Q

Fragile X syndrome key features

A
Learning difficulties
Macrocephaly
Long face
Large ears
Macro-orchidism
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101
Q

Noonan syndrome key features

A

Webbed neck
Pectus excavatum
Short stature
Pulmonary stenosis

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102
Q

Pierre-Robin key features

A

Micrognathia
Posterior displacement of the tongue (may cause upper airway obustruction)
Cleft palate

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103
Q

Prada Willi key features

A

Hypotonia
Hypogonadism
Obesity

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104
Q

William’s syndrome key features

A
Short stature
Learning difficulties
Friendly, extrovert personality
Transient neonatal hypercalcaemia
Supravalvular aortic stenosis
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105
Q

Cri du chat syndrome genetics

A

Chromosome 5p deletion syndrome

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106
Q

Cri du chat key features

A
Characteristic cry due to larynx and neurological problems
Feeding difficulties, poor weight gain
Learning difficulties
Microcephaly and micrognathism
Hypertelorism
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107
Q

Measles infectivity

A

Spread by droplet
Infective from prodrone until 4 days after rash starts
Incubation period 10-14 days

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108
Q

What causes measles?

A

Measles virus

RNA paramyxovirus

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109
Q

Features of measles

A

Prodrome - irritable, conjunctivitis, fever

Koplik spots - start before rash, white spots on buccal mucosa

Rash

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110
Q

What does the measles rash look like and where does it start?

A

Discrete maculopapular rash becoming blotchy and confluent

Starts behind ears

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111
Q

Management of measles

A

Supportive
Admit if immunosuppressed/pregnant
Notifiable disease

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112
Q

How to manage contacts of measles

A

Non-immunised children who come into contact with measles should be offered MMR vaccine within 72 hours

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113
Q

Complications of measles

A

Encephalitis - 1-2 weeks after illness onset
Subacute sclerosing panencephalitis - 5-10 years later
Febrile convulsions
Giant cell pneumonia
Keratoconjunctivitis, corneal ulcer
Diarrhoea
Myocarditis

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114
Q

What type of disease is Kawasaki’s disease?

A

Systemic vasculitis

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115
Q

Diagnosis of Kawasaki’s disease

A

Clinical

Highly likely if high fever + 4 other features
OR high fever + 3 other features + ECHO changes

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116
Q

Features of Kawasaki’s disease

A
High grade fever for >5 days, resistant to antipyretics
Bilateral conjunctival injection
Red, cracked lips
Strawberry tongue
Cervical lymphadenopathy
Red palms and soles which later peel
Non-vesicular rash
Irritable child
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117
Q

Management of Kawasaki’s disease

A

High dose aspirin
IV immunoglobulin
ECHO to screen for coronary artery aneurysms

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118
Q

Complication of Kawasaki’s disease

A

Coronary artery aneurysms

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119
Q

What causes pertussis?

A

Bordetella pertussis

gram positive bacteria

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120
Q

Infectivity of pertussis

A

Incubation 10-14 days

No lifelong protection

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121
Q

Features of pertussis

A
2-3 coryzal days as prodrome
Coughing bouts - worse at night, may cause vomiting
Inspiratory whoop
Persistent cough
Apnoea in infants
Symptoms may last 10-14 days
Marked lymphocytosis
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122
Q

Diagnostic criteria for pertussis

A

Acute cough >14 days without clear cause, plus one of

Paroxysmal cough
Inspiratory whoop
Post-tussive vomit
Undiagnosed apnoea in infants

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123
Q

Management for pertussis

A

Antibiotics reduce spread but not clinical course, start if within 21 days of cough onset

Macrolide - clarithromycin, azithromycin, erythromycin

Prophylactic antibiotics to household contacts

School exclusion for 48 hours after starting antibiotics

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124
Q

Complications of pertussis

A

Subconjunctival haemorrhage
Pneumonia
Bronchiectasis
Seizures

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125
Q

Risk factors for ADHD

A

Maternal smoking, drinking, heroin during pregnancy
Low birth weight
Fetal hypoxia
Severe early psychosocial adversity

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126
Q

What is ADHD?

A

Features relating to inattention and/or hyperactivity/impulsivity that are persistent

Symptoms >6 months and in 2 or more settings (school, work, home)

Symptom onset before age 12

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127
Q

Features of ADHD

A

Inattention: doesn’t follow direction, easily distracted, difficult to sustain tasks and organise tasks, forgetful

Hyperactivity/Impulsivity: unable to play quietly, talks excessively, doesn’t wait their turn, can’t sit still, interruptive

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128
Q

Management of ADHD

A

Parental training education programmes

Psychological therapy

Drug treatment

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129
Q

Drug management of ADHD

A

1st line: Methylphenidate - need to monitor height, weight, BP, heart rate, baseline ECG

2nd line: Lisdexamfetamine - need baseline ECG

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130
Q

Management of otitis media in Down’s syndrome or cleft palate

A

Urgent referral to ENT

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131
Q

When does benign rolandic epilepsy occur?

A

Ages 4-12

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132
Q

Features of benign rolandic epilepsy

A

Seizures at night
Seizures are partial but secondary generalisation may occur
Child otherwise normal

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133
Q

EEG in benign rolandic epilepsy

A

Centro-temporal spikes

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134
Q

Management of benign rolandic epilepsy

A

Often not needed

1st line: carbamazepine

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135
Q

Prognosis in benign rolandic epilepsy

A

Excellent

Majority outgrow by age 16

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136
Q

Causes of microcephaly

A
Normal variant
Familial
Congenital infection
Perinatal brain injury e.g. hypoxic ischaemic encephalopathy
Fetal alcohol syndrome
Syndromes e.g. Patau
Craniosynostosis
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137
Q

What is the definition of microcephaly?

A

Head circumference < 2nd centile

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138
Q

How would an older child with missed developmental dysplasia of the hip present?

A

Trendlenberg gait

Leg length discrepency

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139
Q

Management of eczema

A
Topical emollient (250g per week)
Ratio of emollient to steroid is 10:1
Emollient applied 30 mins before steroid
Emollient can get contaminated with bacteria
Topical steroids
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140
Q

Contraindications to MMR vaccine

A

Severe immunosuppression
Allergy to neomycin
Received another live vaccine within 4 weeks
Avoid pregnancy for 1 month after
Immunoglobulin therapy within the last 3 months

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141
Q

Adverse effect of MMR vaccine

A

Malaise, fever rash after 1st dose
Occurs after 5-10 days
Lasts 2 days

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142
Q

Features of cow’s milk protein intolerance/allergy

A
Regurgitation and vomiting
Diarrhoea
Urticaria, atopic eczema
Colic symptoms
Wheeze, chronic cough
Rarely angioedema and anaphylaxis
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143
Q

Diagnosing cow’s milk protein intolerance/allergy

A

Generally clinical
Skin prick testing
Total IgE and specific IgE

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144
Q

Management of cow’s milk protein intolerance/allergy if bottle fed

A

1st line: extensive hydrolysed formula

2nd: amino acid based formula

10% also allergy to soya milk

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145
Q

Management of cow’s milk protein intolerance/allergy if breastfed

A

Eliminate cow’s milk from maternal diet
Mum will need calcium supplement
Extensively hydrolysed formula when weaning till 1 year

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146
Q

Difference between cow’s milk protein intolerance and allergy

A

Allergy = immediate, IgE mediated

Intolerance = delayed, milder, non-IgE mediated

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147
Q

Features of meningococcal septicaemia

A
Fever
Vomiting
Lethargy
Non-blanching rash
Prolonged cap refill
Shock
Hypotension
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148
Q

Management of meningococcal septicaemia in the community

A

Call 999

IV or IM benzylpenicillin (unless history of anaphylaxis)

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149
Q

Chondromalacia patellae

A

Softening of the patella cartilage
Teenage girls
Anterior knee pain walking up/down stairs, rising from sitting
Responds to physio

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150
Q

Osteochondritis dissecans

A

Pain after exercise

Intermittent swelling and locking

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151
Q

Osgood schlatter

A

Sporty teenagers

Pain, swelling, tenderness over the tibial tubercle

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152
Q

Patellar tendonitis

A

Athletic teenage boys
Chronic anterior knee pain that’s worse after running
Tender below the patella on examination

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153
Q

Patellar subluxation

A

Medial knee pain due to lateral subluxation of patella

Knee may give way

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154
Q

First sign of puberty in males and age of onset

A

Testicular growth
Around age 12
Testicular volume >4ml indicates puberty onset

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155
Q

First sign of puberty in females

A

Breast development

Around age 11.5

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156
Q

Management of threadworms

A

Mebendazole

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157
Q

Management of labial adhesions

A

Generally self-resolves by puberty
If problems e.g. recurrent UTI may need topical oestrogens
Severe may be require surgery

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158
Q

What are labial adhesions?

A

Fusion of the labial minora in the mid-line
Age 3 months to 3 years
Generally self-resolve by puberty

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159
Q

What conditions are tested for with the heel prick?

A
Cystic fibrosis
Hypothyroidism
Phenylketonuria
Maple syrup urine disease
Medium chain acteyl co-A dehydrogenase deficiency
Homocystinuria 
Isovaleric acidaemia
Glutaric aciduria type 1
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160
Q

In a child with frank haematuria what should you consider?

A

Wilm’s tumour

Very urgent paediatric referral

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161
Q

Features of pyloric stenosis

A
Presents at 3-6 weeks
Projectile vomiting
Palpable mass in upper quadrant
Dehydration
Constipation
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162
Q

Investigations for pyloric stenosis

A

Hypochloraemic, hypokalaemic acidosis

Diagnosed via ultrasound

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163
Q

Who is most likely to get pyloric stenosis?

A

M>F 4:1
10-15% have family history
First born more commonly affected

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164
Q

Management of pyloric stenosis

A

Ramstedt pyloromyotomy

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165
Q

Average age of diagnosis of retinoblastoma

A

18 months

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166
Q

Inheritance and genetics of retinoblastoma

A

Autosomal dominant

Loss of function of retinoblastoma tumour suppressor gene on chromosome 13

10% cases hereditary

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167
Q

Features of retinoblastoma

A

Absent red-reflex, replaced by white pupil (Leukocoria)
Strabismus
Visual problems

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168
Q

Management of retinoblastoma

A

Enucleation

Other options if not advanced: external beam radiation, chemotherapy, Photocoagulation

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169
Q

What should you think of in a baby with lack of red reflex?

A

Retinoblastoma

170
Q

What vitamins would someone on orlistat be at risk of becoming deficient in?

A

Fat soluble

A, D, E, K

171
Q

What should you do if there are weak or absent femoral pulses at the 6 week baby check?

A

Discuss with the paediatric consultant on call

172
Q

Management of umbilical granulomas

A

Salt application

Silver nitrate cautery

173
Q

What is glue ear?

A

Otitis media with effusion

174
Q

Features of glue ear

A

Peaks at 2 years

Conductive hearing loss - may present with speech/language delay, behavioural problems

175
Q

Presentation of acute otitis media

A
Sudden onset
Otalgia
Rubbing ear
Fever
URTI symptoms
Hearing loss
Ear discharge
176
Q

Findings of acute otitis media on examination

A

Bulging TM
Loss of light reflex
Erythema of TM
Otorrhoea

177
Q

Management of acute otitis media

A

Generally self limiting

5-7 days amoxicillin
Erythromycin/clarithromycin if pen allergic

178
Q

When do you give antibiotic therapy in acute otitis media?

A
If not improving by day 3
Systemically unwell
Immunocompromised
Significant co-morbidity 
<2 years and bilateral
Perforation/discharge
179
Q

Features of Down’s Syndrome

A
Upslanting palpebral fissures, Epicanthic folds, Brushfield Spots, Single palmar crease
Hypotonia
Duodenal atresia
Hirschsprung's disease
Congenital heart disease 
Visual problems
Otitis media
180
Q

Genetic abnormalities in Down’s Syndrome

A

94% Non-disjunction
5% Robertson translocation
1% Mosaicism

181
Q

Risk of recurrence of Down’s Syndrome if it is a Robertson translocation

A

10-15% if mother is the carrier

2.5% if father is the carrier

182
Q

Risk of Down’s Syndrome if maternal age is 20

A

1 in 1,500

183
Q

Risk of Down’s Syndrome if maternal age is 30

A

1 in 800

184
Q

Risk of Down’s Syndrome if maternal age is 35

A

1 in 270

185
Q

Risk of Down’s Syndrome if maternal age is 40

A

1 in 100

186
Q

Risk of Down’s Syndrome if maternal age is 45

A

1 in 50 or more

187
Q

Gold standard for antenatal Down’s Syndrome testing

A

Combined test, between weeks 11 and 13+6

Nuchal transluency (thickened in DS)
Serum beta HCG (increased)
Pregnancy associated plasma protein A (decreased)

188
Q

Antenatal Down’s Syndrome testing for late bookers

A

Weeks 15-20 triple or quadruple tes

Alpho-feto protein
Unconjugated oestriol
Human chorionic gonadotrophin

+ Inhibin A for quadruple

189
Q

Cause of rubella

A

Togavirus

190
Q

Features of rubella

A

Low grade fever, headache
Maculopapular rash - starts on face then spreads to body, gone by day 3-5
Lymphadenopathy
Forchheimer spots - red spots seen on soft palate

191
Q

Management of rubella

A

Supportive

School exclusion for 4 days after rash starts

192
Q

Features of congenital rubella syndrome

A
Sensorineural deafness
Congenital cataracts
Congenital heart disease
Growth retardation
Hepatosplenomegaly
Purpuric skin lesions
'Salt and pepper' chorioretinitis
Cerebral palsy
193
Q

Risk of congenital rubella syndrome

A

90% in weeks 8-10

Low risk after 16 weeks

194
Q

What is erythema infectiosum also called?

A

Fifth’s disease

Slapped cheek syndrome

195
Q

Cause of erythema infectiosum

A

Parvovirus B19 or erythrovirus B19

196
Q

Features of erythema infectiosum

A

Low grade fever
Bright red rash on cheeks
Lace-like rash on body
URTI

197
Q

Management of erythema infectiosum

A

Supportive

No school exclusion as not infectious after gets rash

198
Q

Erythema infectiosum during pregnancy

A

if <20 weeks need to check maternal IgG and IgM

Risk of hydrops fetalis, intrauterine death, spontaneous abortion

199
Q

Cause of mumps

A

Paramyxovirus

200
Q

Features of mumps

A
May be asymptomatic
Systemic - fever, malaise
Parotitis
Headache
Facial pain
201
Q

Management of mumps

A

Supportive
Notifiable
Infectious for 9 days after swelling starts

202
Q

Complications of mumps

A

Orchitis - 25% of postpubertal males
Hearing loss - transient, unilateral
Meningoencephalitis
Pancreatitis

203
Q

Definition of obesity in children

A

BMI > 98th centile

204
Q

Definition of overweight in children

A

BMI > 91st centile

205
Q

Paraumbilical hernia

A

Defects in linea alba
Edges well defined
Less likely to spontaneously close

206
Q

Omphalitis

A

Infection of umbilicus
Staph aureus most common
Topical and systemic antibiotics
Can spread rapidly

207
Q

Persistent urachus

A

Persistent urachus which attaches to bladder
Discharges urine
Associated with other urogenital abnormalities

208
Q

Umbilical granuloma

A

Cherry red lesion around umbilicus
May bleed on contact
Purulent discharge

209
Q

Persistent vitello-intestinal duct

A

Discharges small bowel content
Rare
Requires surgical closure

210
Q

What type of vaccine is the rotavirus vaccine?

What is the theoretical risk of rotavirus vaccine?

A

Live attenuated vaccine

Theoretical risk of intussusception

211
Q

Constipation red flags in children

A
From birth or first few weeks
Breastfed
Meconium >48 hours
Ribbon stools
Faltering growth
Weakness in legs
Abdo distension
212
Q

Management of constipation

A

Disimpaction if needed: movicol, then stimulant, then osmotic

Maintenance: movicol, then stimulant, then osmotic

Ensure enough fluids and fibre

213
Q

Management of constipation in bottle fed infants

A

Extra water between feeds
Abdominal massage
Bicycle legs

214
Q

Management of constipation in breast fed infants

A

Unusual

Consider organic cause

215
Q

Management of constipation in a weaning infant

A

Extra water, dilated fruit juice, fruit

Lactulose

216
Q

Signs of faecal impaction

A

Severe constipation
Overflow soiling
Faecal mass palpable in abdomen

217
Q

How many children will have a febrile convulsion?

A

3%

218
Q

Risk of further febrile convulsions

A

1 in 3

Apyretics do not lower risk

219
Q

Types of febrile convulsions

A

Simple
Complex
Status

220
Q

Simple febrile convulsion

A

<15 minutes
Generalised
No recurrence in 24 hours
Complete recovery in 1 hour

221
Q

Complex febrile convulsion

A

15-30 mins
Focal seizure
Repeat seizure within 24 hours

222
Q

Febrile status epilepticus

A

> 30 mins

223
Q

Which febrile convulsions should be admitted?

A

First seizure

Any features of complex seizure

224
Q

What is a cephalohaematoma?

A

Swelling on newborns head a few hours after birth
Bleeding between periosteum and skull
May cause jaundice
Up to 3 months to resolve

225
Q

What is ophthalmia neonatorum?

A

Infection of newborn eye

226
Q

What causes ophthalmia neonatorum?

A

Chlamydia trachomatis

Neisseria gonorrhoeae

227
Q

Management of ophthalmia neonatorum

A

Same day paediatric assessment

228
Q

Features of acute lymphoblastic leukaemia

A
Lethargy, pallor
Frequent severe infections
Easy bruising, petechiae
Bone pain
Spleno/hepatomegaly
Fever in 50%
Testicular swelling
229
Q

Poor prognostic factors in acute lymphoblastic leukaemia

A
Age <2 or >10
WBC >20 at diagnosis
T or B cell surface markers
Non-caucasian 
Male sex
230
Q

Features of eczema in infants

A

Affects face and trunk

231
Q

Features of eczema in young children

A

Affects extensor surfaces

232
Q

Features of eczema in older children

A

Typical distribution
Flexor surfaces
Creases of face and neck

233
Q

School exclusion in conjunctivitis

A

No exclusion

234
Q

Treatment of croup

A

Oral dexamethasone 0.15mg/kg

235
Q

Benzylpenicillin for menginococcal dose if 1-11 months old

A

300mg IM or IV

236
Q

Benzylpenicillin for meningococcal dose if 1-9 years

A

600mg IM or IV

237
Q

Benzylpenicillin for meningococcal dose if 10-17 years

A

1.2 grams IM or IV

238
Q

Benzylpenicillin for meningococcal dose if an adult

A

1.2 grams IM or IV

239
Q

Prophylactic treatment of meningitis in close contacts

A

Ciprofloxacin

240
Q

Antibiotics for meningitis <3 months

A

IV amoxicillin + IV cefotaxime

No steroids

241
Q

Antibiotics for meningitis >3 months

A

IV cefotaxime or ceftriaxone

242
Q

Who is affected by infantile spasms?

A

Age 4-8 months

M>F

243
Q

Features of infantile spasms

A

Characteristic ‘salaam’ attacks
Last 1-2 seconds but may repeat 50 times
Progressive mental handicap

244
Q

EEG in infantile spasms

A

Hypsarrhythmia

245
Q

CT in infantile spasms

A

Diffuse or localised brain disease in 70% (e.g. tuberous sclerosis)

246
Q

What is Freiberg disease?

A

Metatarsal avascular necrosis

usually second metatarsal

247
Q

Who is mainly affected by Freiberg disease?

A

Tall, athletic females

Usually adolescents

248
Q

Features of Freiberg disease

A

Pain
Swelling
Stiffness

Of the affected metatarsal (usually 2nd)

249
Q

Management of Freiberg disease

A

Limit activity, analgesia, orthotic devices
Refer if no improvement at 6 weeks
Surgery rarely needed
Recovery may be gradual and take up to 1 year

250
Q

What is calcaneal apophysitis?

A

Swelling and irritation of growth plate in the heel
Heel pain in sporty children
Pain on squeezing heel

Also called Sever disease

251
Q

What does hand dominance prior to 18 months suggest?

A

Cerebral palsy

Hemiparesis

252
Q

Management of fever in a baby under 3 months

A

Refer to hospital
Full septic screen
IV antibiotics

253
Q

Causes of early jaundice

A
Rh incompatibility
ABO incompatibility
G-6-P dehydrogenase deficiency
Spherocytosis
Infection
Autoimmune haemolytic anaemia
Crigler Najjar syndrome
Gilbert's syndrome
254
Q

Causes of jaundice 24 hours - 3 weeks

A
Physiological jaundice
Breast milk jaundice
Infection
Haemolysis
Bruising
Polycythaemia
Crigler Najjar syndrome
255
Q

Causes of prolonged jaundice >3 weeks

A
Physiological jaundice
Breast milk jaundice
UTI
Congenital infection e.g. CMV, toxoplasmosis
Hypothyroidism
Haemolytic anaemia
Obstruction
Biliary atresia
Neonatal hepatitis
256
Q

Main causes of neonatal sepsis

A

Group B streptococcus

Escheria coli

257
Q

Risk factors for neonatal sepsis

A
Maternal - previous baby with GBS, GBS colonisation, bacteruria
Intrapartum temp > 38
Membrane rupture > 18 hours
Premature < 37 weeks
Low birth weight < 2.5kg
Maternal chorioamnionitis
258
Q

Management of neonatal sepsis

A

IV benzylpenicillin + IV gentamicin

259
Q

What is Epstein’s pearl?

A

Congenital cyst in the mouth

Spontaneously resolve over a few weeks

260
Q

Inheritence of Freidrich’s ataxia

A

Autosomal recessive

Trinucleotide repeat

261
Q

Management of threadworms in pregnant women

A

1st trimester - 2 weeks strict personal hygeine

2nd/3rd trimester - can use mebendazole if necessary

262
Q

Inheritance of haemophilia A

A

X linked recessive

263
Q

Inheritance of Alport’s syndrome

A

X linked dominant

10% cases are autosomal recessive

264
Q

Inheritance of Rett’s syndrome

A

X linked dominant

265
Q

Inheritance of vitamin D resistant rickets

A

X linked dominant

266
Q

Inheritance of Fragile X

A

X linked dominant

Trinucleotide repeat

267
Q

Inheritance of Huntington’s disease

A

Autosomal dominant

Trinucleotide repeat

268
Q

Inheritance of myotonic dystrophy

A

Autosomal dominant

Trinucleotide repeat

269
Q

Should a premature baby have their immunisation schedule adjusted for gestational age?

A

No, give according to chronological age

270
Q

What is genu varum?

A

Bow legs

271
Q

What is genu valgum?

A

Knock knees

272
Q

What is the main cause of hypertension in children?

A

Renal parenchymal disease

273
Q

Causes of hypertension in children

A
Renal parenchymal disease
Renal vascular disease
Coarctation of the aorta
Phaeochromocytoma
Congenital adrenal hyperplasia
Essential hypertension
274
Q

Which condition is associated with supravalvular aortic stenosis?

A

William’s syndrome

275
Q

Vaccinations at birth

A

BCG if has risk factors

276
Q

Vaccinations at 2 months

A

‘6-1 vaccine’ - diphtheria, tetanus, whooping cough, polio, Hib and hepatitis B

Oral rotavirus

Men B

277
Q

When do children receive the Men C vaccine?

A

Used to be given at 3 months but has been discontinued

278
Q

Vaccinations at 3 months

A

‘6-1’ vaccine - diphtheria, tetanus, whooping cough, polio, Hib and hepatitis B

Oral rotavirus

Pneumococcal vaccine

279
Q

Vaccinations at 4 months

A

‘6-1 vaccine’ - diphtheria, tetanus, whooping cough, polio, Hib and hepatitis B

Men B

280
Q

Vaccinations at 12-13 months

A

Hib/Men C
MMR
Pneumococcal vaccine
Men B

281
Q

Vaccinations at 2-8 years

A

Annual flu vaccine

282
Q

Vaccinations at 3-4 years

A

‘4 in 1 preschool booster’ - diphtheria, tetanus, whooping cough, polio

MMR

283
Q

Vaccinations at 12-13 years

A

Human papilloma vaccine

284
Q

Vaccination at 13-18 years

A

‘3 in 1 teenage booster’ - tetanus, diphtheria, polio

Men ACWY

285
Q

At what ages can the rotavirus vaccine be safely given?

A

First dose not after 15 weeks
Second dose not after 24 weeks

Due to theoretical risk of intussusception

286
Q

What type of vaccine is rotavirus?

A

Oral live attenuated

287
Q

What age is the rotavirus vaccine given?

A

2 months

3 months

288
Q

When is genu varum a normal variant?

A

Bow legs

Birth to 2 years is normal

289
Q

When is genus valgus a normal variant?

A

Knock knees

3-6 years is normal

290
Q

When is flat feet a normal variant?

A

Until age 3

most will resolve by age 8

291
Q

What vaccines are in the 6-1 vaccine?

A

diphtheria, tetanus, whooping cough, polio, Hib and hepatitis B

292
Q

Inheritance of sickle cell anaemia

A

Autosomal recessive

293
Q

Epistaxis under 2 years

A

Uncommon - urgent referral for possible bleeding disorder

294
Q

Normal observations under 1

A

HR 110-160
SBP 70-90
RR 30-40

295
Q

Normal observations 1-2

A

HR 100-140
SBP 80-95
RR 25-35

296
Q

Normal observations 2-5

A

HR 95-130
SBP 80-100
RR 25-30

297
Q

Normal observations 5-12

A

HR 80-120
SBP 90-110
RR 20-25

298
Q

Normal observations >12

A

HR 80-100
SBP 100-120
RR 15-20

299
Q

When is Men C vaccine given?

A

1 year and 14 years

300
Q

When is Men B vaccine given?

A

2 months, 4 months, 1 year

301
Q

When is pneumococcal vaccine given?

A

3 months and 1 year

302
Q

When is MMR vaccine given?

A

1 year and 3-4 years

303
Q

When is rotavirus vaccine given?

A

2 months and 3 months

304
Q

When is ‘6-1’ vaccine given?

A

2 months, 3 months and 4 months

‘4 in 1 booster’ at age 4
‘3 in 1 booster’ as a teenager

305
Q

Visual problems in Down’s syndrome

A
Refractive errors
Strabismus
Cataracts
Recurrent blepharitis
Glaucoma
306
Q

Commonest cause of hypothyroidism in children

A

Autoimmune thyroiditis

307
Q

Causes of hypothyroidism in children

A

Autoimmune thyroiditis
Post total-body irradiation
Iodine deficiency

308
Q

Inheritance of sick cell anaemia

A

Autosomal recessive

309
Q

Side effect of montelukast

A

Nightmares

310
Q

Investigations for atypical or repeat UTI in infant <6 months

A

Ultrasound during infection
DMSA 4-6 months later
MCUG

311
Q

Cause of cystic fibrosis

A

defect in the cystic fibrosis transmembrane conductance regulator gene (CFTR), which codes a cAMP-regulated chloride channel

80% of CF cases are due to delta F508 on the long arm of chromosome 7

312
Q

Organisms which may colonise CF patients

A

Staph aureus
Pseudomonas aeruginosa
Burkholderia cepacia
Aspergillus

313
Q

Most common cause of inherited learning disability

A

Fragile X syndrome

314
Q

Specific exclusion for the DTP vaccine

A

Unstable or evolving neurological condition

315
Q

Features of pityriasis rosea

A

Herald patch

1-2 weeks later salmon-pink oval scaly patches in christmas tree distribution on the back

316
Q

Management of pityriasis rosea

A

Self limiting

Resolves in 6 weeks

317
Q

What is pityriasis verisocolor?

A

Superficial cutaneous fungal infection

Caused by malassezia furfur

318
Q

Predisposing factors for pityriasis versicolor

A

Immunosuppression
Malnutrition
Crohn’s

319
Q

Features of pityriasis versicolor

A
On trunk
Patches may be hypopigmented, pink or brown
More noticeable after sun exposure
Scale common
Mild pruritis
320
Q

Management of pityriasis versicolor

A

Topical antifungal with ketaconazole shampoo

If doesn’t respond: skin scrapings, oral itraconazole

321
Q

Genetics of fragile X syndrome

A

Trinucleotide repeat disorder

X linked dominant

322
Q

Features of fragile X syndrome in males

A
Learning difficulty
Large, low set ears
Long thin face
High arched palate
Macro-orchidism
Hypotonia
Autisim
Mitral valve prolapse
323
Q

Features of fragile X syndrome in females

A

Ranges from mild to normal

324
Q

How many babies are affected by cleft lip and palate?

A

1 in 1000

325
Q

Types of cleft lip and palate

A

15% isolated cleft lip
40% isolated cleft palate
45% combined cleft lip and palate

326
Q

Problems caused by cleft lip and palate

A

Feeding
Speech - 75% have normal speech with therapy
Increased risk of otitis media

327
Q

Management of cleft lip and palate

A

Cleft lip repaired 1 week to 3 months

Cleft palate repaired 6 to 12 months

328
Q

Main types of bacteria causing UTI in children

A

E. Coli (85%)
Klebsiella
Staphylococcus saprophyticus

329
Q

Presentation of UTI in infants

A
Poor feeding
Fever
Irritable
FTT
Vomiting
330
Q

Management of UTI in children

A

<3 months = admit
Upper UTI = admit
>3 months and lower UTI = oral antibiotics

331
Q

Specialist investigations for UTI in children

A

If <6 months will need renal ultrasound, plus DSMA and MCUG if recurrent or atypical infection

332
Q

Androgen insensitivity syndrome inheritance

A

X linked recessive

333
Q

Androgen insensitivity syndrome karyotype

A

46 XY

334
Q

What is androgen insensitivity syndrome?

A

End organ resistance to testosterone causes genetically male children to have female phenotype

335
Q

What is congenital adrenal hyperplasia?

A

A group of autosomal recessive conditions causing abnormal cortisol synthesis

336
Q

Most common enzyme deficiency in congenital adrenal hyperplasia

A

21-hydroxylase deficiency (90%)

337
Q

Classical congential adrenal hyperplasia features

A

Severe
Non-salt losing presents in childhood with low glucocorticoids and high androgens, males present with virilisation at age 2-4
Salt losing presents in adrenal crisis in first few weeks due to aldosterone deficiency
Females have ambiguous genitalia

338
Q

Non-classical congenital adrenal hyperplasia features

A

Milder
Later onset
Hyperandrogenism in childhood or teenage years
Females have early pubarche, infertility, hirsutism, menstrual disturbance

339
Q

5-alpha reductase deficiency genotype

A

46 XY

340
Q

5-alpha reductase deficiency interitance

A

Autosomal recessive

341
Q

What is 5-alpha reductase deficiency?

A

Inability of males to convert testosterone to dihydrotesosterone

342
Q

Presentation of 5-alpha reductase deficiency

A

Ambigous genitalia at birth
Hypospadias
Virilisation at puberty

343
Q

Male pseudohermaphroditism

A

46 XY

Testes but external genitalia is female or ambiguous

344
Q

Female pseudohermaphroditism

A

46 XX

Ovaries but external genitalia is male or ambiguous

345
Q

True hermaphroditism

A

46 XX or 46 XXY
Very rare
Both ovarian and testicular tissue

346
Q

Features of androgen insensitivity syndrome

A

Primary amenorrhoea
Undescended tests cause groin swelling
May have breast development

347
Q

What is the other term for primary hypogonadism?

A

Klinefelter’s syndrome

348
Q

Features of Klinefelter’s syndrome

A
Tall
Lack secondary sexual characteristics
Small, firm testes
Infertile
Gynaecomastia
Learning difficulties
Truncal obesity
349
Q

Genotype in Klinefelter’s syndrome

A

47 XXY

350
Q

Hormone levels in Klinefelter’s syndrome

A

High LH

Low testosterone

351
Q

Treatment for Klinefelter’s syndrome

A

Testosterone replacement

Fertility treatment

352
Q

What is the other name for hypogonadotrophic hypogonadism?

A

Kallman’s syndrome

353
Q

Inheritance of Kallman’s syndrome

A

X linked recessive

354
Q

Features of Kallman’s syndrome

A
Anosmia
Delayed puberty
Hypogonadism
Cryptoorchidism
Normal or above average height
355
Q

Hormone levels in Kallman’s syndrome

A

Low LH

Low testosterone

356
Q

Most common cause of diarrhoea and vomiting in children?

A

Rotavirus

357
Q

Cystic fibrosis inheritance

A

Autosomal recessive

358
Q

Genetics of cystic fibrosis

A

CFTR gene on chromosome 7

Most commonly delta F508 variant

359
Q

Features of cystic fibrosis

A
Meconium ileus, rectal prolapse
Recurrent chest infections
Malabsorption = steatorrhea, FTT
Liver disease
Diabetes
Nasal polyps
Pubertal delay, short stature
Subfertility
360
Q

Diagnosis of cystic fibrosis

A

Newborn heel prick
Sweat test
Genetic testing

361
Q

Colonising organisms in cystic fibrosis

A

Staph aureus
Pseudomonas aeruginosa
Burkholderia cepacia
Aspergillus

362
Q

Management of cystic fibrosis

A

chest PT, postural drainage
Multivitamin, pancreatic supplement
High calorie high fat diet
Orkambi if delta 508

363
Q

Features of rickets

A
Genu varum (bow legs) in toddlers
Genu valgum (knock knees) in chidren
'Rickety rosary' - swelling at costochondral junction
Kyphoscoliosis
Craniotabes (soft skull)
Increased infections
Impaired growth
364
Q

Blood tests in rickets

A

Low Vit D, Low calcium

High Alk phos

365
Q

Wrist xray in rickets

A

Cupping, splaying and fraying of metaphysis

366
Q

Presdisposing factors for rickets

A

Dietary deficiency of calcium
Prolonged breast feeding
Unsupplemented cows milk formula
Lack of sunlight

367
Q

Management of rickets

A

Oral vitamin D replacement

368
Q

When do flat feet resolve?

A

4-8 years

369
Q

In-toeing in children

A

Present by 1 year
Majority will self resolve
If not may need casting or surgery depending on cause

370
Q

What is genu varum?

A

bow legs

371
Q

When does genu varum resolve?

A

Presents 1-2 years

Resolves by 4-5 years

372
Q

What is genu valgum?

A

knock nees

373
Q

Out-toeing - when does it resolve?

A

Resolves by age 2

Intervene if doesn’t resolve

374
Q

Cause of acute epiglottitis

A

Haemophilus influenzae type B

375
Q

Features of acute epiglottitis

A
Rapid onset
High temperature
Stridor
Drooling of saliva
'Tripod' position
376
Q

Diagnosis of acute epiglottitis

A

Direct visualisation by anaesthestics

Lateral view xray - ‘thumb sign’

377
Q

Management of acute epiglottitis

A

May need intubation
Oxygen
IV antibiotics

378
Q

What is Bartter’s syndrome?

A

Inherited cause of severe hypokalaemia due to defective chloride absorption in the loop of henle

379
Q

Inheritance of Bartter’s syndrome

A

Autosomal recessive

380
Q

Features of Bartter’s syndrome

A
FTT
Polyuria, polydipsia
Weakness
Sensorineural deafness
Normotension
Hypokalaemia
381
Q

Blood pressure in Bartter’s syndrome

A

Normotensive

382
Q

Management of Bartter’s syndrome

A

Oral potassium

Potassium sparing diuretics

383
Q

Triad of nephrotic syndrome

A

Proteinuria (>1g/m2 in 24 hours)
Hypoalbuminaemia
Oedema

384
Q

Causes of nephrotic syndrome

A

80% due to primary glomerulonephritis

20% due to systemic disease - diabetes, SLE, amyloidosis

385
Q

Main cause of nephrotic syndrome in children

A

Minimal change glomerulonephritis

386
Q

Treatment of minimal change glomerulonephritis

A

High dose steroids

90% children respond

387
Q

Genetics of Turner’s syndrome

A

45XO or 45X

Presence of just one sex chromosome OR deletion of short arm of one of the chromosomes

388
Q

Features of Turner’s syndrome

A
Short stature
Shield chest, widely spaced nipples
Webbed neck
Primary amenorrhoea
Cystic hygroma
Multiple pigmented naevi
Lymphoedema in neonates (in feet)
Horseshoe kidney
Short 4th metacarpal
389
Q

Congenital heart disease in Turner’s syndrome

A

15% bicuspid aortic valve

10% coarctation of the aorta

390
Q

Congenital renal disease in Turner’s syndrome

A

Horseshoe kidney

391
Q

What diseases are females with Turner’s syndrome at increased risk of?

A

Autoimmune thyroiditis

Crohn’s

392
Q

Causes of macrocephaly

A
Chronic hydrocephalus
Chronic subdural effusion
Neurofibromatosis
Gigantism (e.g. Soto's syndrome)
Metabolic storage diseases
Bone problems e.g. thalassaemia
393
Q

Congenital toxoplasmosis

A
Cerebral calcifications
Chorioretinitis
Hydrocephalus
Cerebral palsy
Hepatosplenomegaly
Anaemia
394
Q

Congenital cytomegalovirus

A
Growth retardation
Purpuric skin lesions
Pneumonitis
Sensorineural deafness
Hepatosplenomegaly
Anaemia
Cerebral palsy
395
Q

McCune Albright Syndrome

A

Precocious puberty
Cafe-au-lait spots
Polyostotic fibrous dysplasia
Short stature

396
Q

Define precocious puberty

A

Development of secondary sexual characteristics before 8 years in females and 9 years in males

397
Q

Classification of precocious puberty

A

Gonadotrophin dependent - LH and FSH raised

Gondadotrophin independent (pseudo) - LH and FSH low, excess sex hormones

398
Q

Causes of precocious puberty in males

A

Bilateral enlarged testes = gonadotrophin release from intracranial lesion

Unilateral enlarged teste = gonadal tumour

Small testes = adrenal cause (tumour or adrenal hyperplasia)

399
Q

Causes of precocious puberty in females

A

Usually idiopathic/familial and follows normal puberty

Organic is rare e.g. McCune Albright Syndrome

400
Q

Asthma management age 5-16 STEP ONE

A

SABA

Salbutamol

401
Q

Asthma management age 5-16 STEP TWO

A

SABA + low dose ICS

Salbutamol + clenil 100mcg 2 puffs BD

402
Q

Asthma management age 5-16 STEP THREE

A

SABA + low dose ICS + LRTA

Salbutamol + clenil 100mcg 2 puffs BD + montelukast

403
Q

Asthma management age 5-16 STEP FOUR

A

SABA + low dose ICS + LABA

stop LRTA

404
Q

Asthma management age 5-16 STEP FIVE

A

SABA + maintenance and reliever therapy (MART) that includes low dose ICS
Salbutamol + fostair

405
Q

Asthma management age 5-16 STEP SIX

A

SABA + MART that includes moderate dose ICS

406
Q

Example of low dose ICS

A

Clenil 100mcg 2 puffs BD

407
Q

Example of moderate dose ICS

A

Clenil 200mcg 2 puff BD

408
Q

Example of LRTA

A

Montelukast

409
Q

Example of MART

A

Fostair

410
Q

Asthma management age <5 STEP ONE

A

SABA

411
Q

Asthma management age <5 STEP TWO

A

SABA+ 8 week trial of moderate dose ICS

If improves and symptoms return within 4 weeks = continue low dose ICS
If improves and symptoms return outwith 4 weeks = repeat trial

412
Q

Asthma management age <5 STEP THREE

A

SABA + low dose ICS + LTRA

413
Q

Asthma management age <5 STEP FOUR

A

Stop LRTA
Continue SABA + low dose ICS
Refer specialist clinic

414
Q

Example of LABA

A

Salmeterol

415
Q

Features of asthma

A
Cough, worse at night
Dyspnoea
Wheeze
Reduced PEFR
Atopy
416
Q

Spirometry findings in asthma

A

Reduced FEV1
Normal FVC
FEV1/FVC ratio <70%

417
Q

Moderate acute asthma

A

PEFR >50%

Normal speech

418
Q

Acute severe asthma

A

PEFR 33-50%
Can’t finish sentences
HR over threshold
RR over threshold

419
Q

Acute severe asthma - HR criteria

A

> 110 if over 12
125 if over 5
140 if 1-5

420
Q

Acute severe asthma - RR criteria

A

> 25 if over 12
30 if over 5
40 if 1-5

421
Q

Life threatening asthma

A
Oxygen <92%
PEFR <33%
PaO2 <8
Normal PaCO
Confusion
Exhaustion
Silent chest
Cyanosis 
Hypotension, bradycardia
422
Q

Steroids in acute asthma in children

A

Given to all children for 3-5 days

20mg OD if age 2-5
40mg OD if >5