Day 1 PAEDS

Question 1

A 3-month-old infant presents with vomiting and regurgitation following feeds. The child is gaining weight normally and no abnormalities are found on examination

Answer 1

gastro-oesophageal reflux disease

Question 2

a 6-year-old girl who has sickle cell disease develops pallor and fatigue shortly after a viral illness characterized by an erythematous rash affecting her cheeks

Answer 2

Parvovirus B19

Question 3

A two-year-old child is brought to the hospital. His parents are concerned as he has had a temperature of 39ºC for the last five days. They have also noted that his eyes are red, his neck is swollen and he has a new rash.

On examination, there is a generalised maculopapular rash and the child has bilateral conjunctivitis. There is unilateral cervical lymphadenopathy. The lips are cracked and erythematous and a strawberry tongue is present.

What is the diagnosis?

What treatment should this child receive initially?

Answer 3

This child has Kawasaki disease.

Kawasaki disease is a systemic vasculitis that generally presents in the under 5s.

Typical findings, as seen in this case, include an acute febrile illness lasting over 5 days, bilateral non-purulent conjunctivitis, unilateral cervical lymphadenopathy, a polymorphic rash, and mucosal erythema with a strawberry tongue.

Swelling of the hands and feet can occur in the acute stage with desquamation in the second week. Coronary aneurysms can develop in up to one-quarter of untreated patients.

THE MAIN GOAL OF TREATMENT IS TO REDUCE CARDIAC COMPLICATIONS.

The standard treatment in the UK is intravenous immunoglobulin and high dose aspirin (despite the fact that it is usually contraindicated in children) due to the additional anti-inflammatory effects this provides.

Question 4

Answer 4

maculopapular rash

Question 5

Define febrile

Answer 5

febrile

Question 6

Define purulent

Answer 6

consisting of, containing, or discharging pus.

Question 7

Answer 7

conjunctivitis

Question 8

What is a polymorphic rash?

Answer 8

Question 9

What are the characteristics of Kawasaki’s disease?

(6)

Answer 9

Typical findings

1. acute febrile illness lasting over 5 days
2. bilateral non-purulent conjunctivitis
3. unilateral cervical lymphadenopathy
4. polymorphic rash
5. mucosal erythema
6. strawberry tongue

Question 10

What is vasculitis?

Answer 10

autoimmune inflammation, of the blood vessels

categorized by the size of the blood vessels

white blood cells confuse the normal antigens on the endothelial cells with the antigens of foreign invaders because they look similar—this is called molecular mimicry

This exposes the underlying collagen to intravascular coagulation (blood clots(

Fibrin is deposited into the walls as part of the healing process stiffening the vessels or aneurysms

Question 11

What causes Kawasaki’s disease?

Answer 11

vasculitis affecting medium-size vessels supplying organs

it affects the coronary arteries

Question 12

How is Kawasaki’s disease managed?

(4)

Answer 12

Management

• high-dose aspirin
• Kawasaki disease is one of the few indications for the use of aspirin in children. (Due to the risk of Reye’s syndrome aspirin is normally contraindicated in children)
• intravenous immunoglobulin
• echocardiogram (rather than angiography) is used as the initial screening test for coronary artery aneurysms

Question 13

What is Reye’s syndrome

(2)

Answer 13

• Encepalopaphy + liver damage following a viral infection
• mitochondria become damaged within the liver and the liver is unable to filter nitrogenous compounds, causing hepatic encepaplopathy

Question 14

A 5-week-old boy is brought into the GP by his mother with diarrhoea and vomiting for the past 4 days. He also has a new rash that is irritating him and has developed a runny nose.

There is no history of any weight loss, pyrexia, or other family members being unwell.

On further questioning, she reports that she has tried to wean him from breast to bottle this week as she is going away with work in 3 weeks time and is anxious about him not feeding well if there is a sudden change.

The GP suspects that the infant may have cow’s milk protein intolerance.

What is the next most appropriate feed to trial in this infant?

Answer 14

If a formula-fed baby is suspected of having mild-moderate cow’s milk protein intolerance then a extensively hydrolysed formula should be tried

Question 15

When is extensively hydrolysed formula milk used?

Answer 15

severe cow’s milk protein intolerance

Question 16

What are the features of cows milk intolerance in infants?

Answer 16

Features

1. regurgitation and vomiting
2. diarrhoea
3. urticaria, atopic eczema
4. ‘colic’ symptoms: irritability, crying
5. wheeze, chronic cough
6. rarely angioedema and anaphylaxis may occur

Question 17

When is Amino acid-based formula used in infants?

Answer 17

Amino acid-based formula is appropriate for infants with severe cow’s milk protein intolerance (CMPI).

This formula is less palatable, however, it is appropriate for those with severe intolerance as it is composed of free amino acids only.

Question 18

When is high protein formula used in infants?

(2)

Answer 18

High protein formula has been used to manage pre-term infants.

This is becoming an increasingly specialised use as there are increasing studies showing that high-protein feed (even in prematurity) is associated with increased long-term obesity risk.

Question 19

When is a lactose-free formula used in infants?

Answer 19

Lactose-free formula would be appropriate if the child was considered to be lactose intolerant.

The features pointing towards cow’s milk protein intolerance are:

rash and runny nose

Infants with lactose intolerance will usually have GI symptoms only.

Question 20

Cause of obesity in children

(5)

Answer 20

Cause of obesity in children

1. growth hormone deficiency
2. hypothyroidism
3. Down’s syndrome
4. Cushing’s syndrome
5. Prader-Willi syndrome

Question 21

What is Prader-Willi syndrome?

Answer 21

Prader-Willi syndrome is an example of genetic imprinting where the phenotype depends on whether the deletion occurs on a gene inherited from the mother or father:

• Prader-Willi syndrome if gene deleted from father
• Angelman syndrome if gene deleted from mother

Question 22

NICE recommendations for clinical obesity in children

(2)

Answer 22

NICE recommend

consider tailored clinical intervention if BMI at 91st centile or above

consider assessing for comorbidities if BMI at 98th centile or above

Question 23

Eight-year-old. He has dysmorphic features with:

• macro-dolichocephaly,
• down-slanting palpebral fissures pointed chin
• Parents note that he has rapid growth and challenging behaviours

What is the most likely diagnosis?

Answer 23

Sotos syndrome: a rare disorder characterised by excessive physical growth and learning disabilities

Question 24

Define dolichocephaly

Answer 24

Dolichocephaly

The most common form of craniosynostosis, where premature closure of the sagittal suture results in an impediment to the lateral growth of the skull while anteroposterior growth continues, producing a classic elongated, yet narrow, skull.

Question 25

What is Sotos syndrome?

(2)

Answer 25

Sotos syndrome is a rare genetic disorder characterised by excessive physical growth during the first 2 to 3 years of life

It is caused by a mutation in the NSD1 (Nuclear receptor-binding SET domain containing protein) gene and is inherited with in an autosomal dominant fashion

Question 26

What are the characteristics of Patau syndrome?

(4)

Answer 26

Trisomy 13

Microcephalic, small eyes
Cleft lip/palate
Polydactyly
Scalp lesions

survival beyond the neonatal period is rare

Question 27

What are the features of Edward’s syndrome?

(4)

Answer 27

Trisomy 18

Micrognathia
Low-set ears
Rocker bottom feet
Overlapping of fingers

Question 28

Marie is a 14-year-old who has come to her GP for contraception advice. She explains she has been sexually active with a 14-year-old male partner for the last 2 months.

She has researched various methods of contraception and would like to try the combined oral contraceptive pill (COCP).

She has previously had depression after being abused as a child and remains under the care of the Child and Adolescent Mental Health Services.

However, she says her mood has been much better since meeting her partner. Her parents do not know about the situation and she can not be convinced to tell them.

What is the right course of action?

Answer 28

Prescribe the COCP, providing there are no contraindications

Question 29

When can you provide contraceptive, abortion and STI advice and treatment, without parental knowledge or consent?

Answer 29

a. they understand all aspects of the advice and its implications
b. you cannot persuade the young person to tell their parents
c. the young person is very likely to have sex with or without such treatment
d. their physical or mental health is likely to suffer unless they receive such advice or treatment
e. it is in the best interests of the young person to receive the advice and treatment without parental knowledge or consent.

Question 30

A 2-day-old baby has not passed meconium yet.

Your consultant tells you they believe that the child has Hirschsprung’s disease, and asks you what your initial management would be.

Until a firm diagnosis can be made and more definite treatment given, which of these is the best initial treatment for this child?

Answer 30

The initial management in Hirschprung’s disease is rectal washouts/bowel irrigation

Question 31

What is the cause of Hirschsprung’s disease?

Answer 31

Hirschsprung’s disease is caused by an aganglionic segment of bowel due to a developmental failure of the parasympathetic Auerbach and Meissner plexuses.

Thus a segment of the bowl is paralysed.

Question 32

Possible presentations of Hirschsprung’s

(2)

Answer 32

Possible presentations

neonatal period e.g. failure or delay to pass meconium

older children: constipation, abdominal distension

Question 33

Hirschsprung’s investigations

(2)

Answer 33

Investigations

abdominal x-ray

rectal biopsy: gold standard for diagnosis

Question 34

Hirschsprung’s management

Answer 34

Management

initially: rectal washouts/bowel irrigation

definitive management: surgery to affected segment of the colon

Question 35

A mother comes in saying that she thinks her 6-month-old son has colic.

She shows you a video on her phone.

The video shows a 6-month-old baby crying, which stops abruptly and the child draws his chin into his chest, throws his arms out.

The child then relaxes and starts crying again, and over the course of the minute long video this is repeated around 10 times.

The mother also reports that the child has been referred to the community paediatric clinic due to slight delay in reaching developmental milestones.

Which one test is most appropriate for you order to help confirm your diagnosis?

Answer 35

In infantile spasms the child will become distressed between spasms, whereas in colic the child will become distressed during the ‘spasms’

EEG is needed, as hypsarrhythmia is commonly found in West’s syndrome.

Question 36

Define colic

Answer 36

Colic is when a healthy baby cries for a very long time, for no obvious reason.

It is most common during the first 6 weeks of life.

It usually goes away on its own by age 3 to 4 months. Up to 1 in 4 newborn babies may have it.

Question 37

Define Hypsarrhythmia

Answer 37

Hypsarrhythmia is very chaotic and disorganized brain electrical activity with no recognizable pattern, whereas a normal brain electrical activity

Question 38

An 11-week-old boy started with a runny nose and mild fever.

He was better for 2 days, and then now has begun coughing often, is struggling to breathe and is not taking to feeds.

On examination, there is evidence of nasal flaring.

On chest auscultation, there are bilateral crackles and an expiratory wheeze. His temperature is 38.2ºC (normal: 36.1-38.0ºC), oxygen saturations are 98% on air (normal: >96%), the heart rate is 130 beats per minute (normal: 115-160), and he has a respiratory rate of 48 breaths per minute (normal: 25-45).

What is the most likely diagnosis?

What is the most appropriate treatment?

Answer 38

Bronchiolitis does not require antibiotics

children require supportive management only

Question 39

Bronchiolitis features

(5)

Answer 39

Features

• coryzal symptoms (including mild fever) precede:
• dry cough
• increasing breathlessness
• wheezing, fine inspiratory crackles (not always present)
• feeding difficulties associated with increasing dyspnoea are often the reason for hospital admission

Question 40

Investigation of bronchiolitis

Answer 40

Investigation

immunofluorescence of nasopharyngeal secretions may show RSV

Question 41

Management of Bronchiolitis

(3)

Answer 41

Management is largely supportive

• humidified oxygen is given via a head box and is typically recommended if the oxygen saturations are persistently < 92%
• nasogastric feeding may be needed if children cannot take enough fluid/feed by mouth
• suction is sometimes used for excessive upper airway secretions

Question 42

Epidemiology of bronchiolitis

(3)

Answer 42

respiratory syncytial virus (RSV) is the pathogen in 75-80% of cases

most common cause of a serious lower respiratory tract infection in < 1yr olds (90% are 1-9 months, with a peak incidence of 3-6 months). Maternal IgG provides protection to newborns against RSV

higher incidence in winter

Question 43

A 7-year-old boy is diagnosed with Attention Deficit Hyperactivity Disorder. What is the most appropriate dietary advice to give to his parents?

Answer 43

NICE recommend a normal balanced diet unless a food diary has demonstrated a link between behaviour and certain foods.

Question 44

What is Croup?

Answer 44

• Croup is a form of upper respiratory tract infection seen in infants and toddlers.
• It is characterised by stridor which is caused by a combination of laryngeal oedema and secretions.
• Parainfluenza viruses account for the majority of cases.

Question 45

What is the Epidemiology of croup?

(2)

Answer 45

What is the Epidemiology

peak incidence at 6 months - 3 years

more common in autumn

Question 46

What are the features of croup?

(4)

Answer 46

Features

• stridor
• barking cough (worse at night)
• fever
• coryzal symptoms

Question 47

What is Laryngomalacia?

(2)

Answer 47

Congenital abnormality of the larynx.

Infants typical present at 4 weeks of age with: stridor

Question 48

What is Acute epiglottitis?

What ages does it affect?

Answer 48

Acute epiglottitis is rare but serious infection caused by Haemophilus influenzae type B.

Prompt recognition and treatment is essential as airway obstruction may develop.

Epiglottitis generally occurs in children between the ages of 2 and 6 years.

Question 49

What are the features of acute epiglottitis?

(4)

Answer 49

Features

1. rapid onset
2. unwell, toxic child
3. stridor
4. drooling of saliva

Question 50

An 11-year-old boy presents to his general practitioner with unilateral hip and knee pain of 2 weeks after being involved in a tackle whilst playing football.

He has a marked limp that has recently worsened.

On examination, there is the loss of internal rotation of the leg in flexion. He has no fever and his observations are stable.

What is the most likely diagnosis?

Answer 50

There is often the loss of internal rotation of the leg in flexion in slipped capital femoral epiphysis

typically age group is 10-15 years

Question 51

Epidemiology of slipped capital femoral epiphysis

(4)

Answer 51

• typically age group is 10-15 years
• More common in obese children and boys
• Displacement of the femoral head epiphysis postero-inferiorly
• May present acutely following trauma or more commonly with chronic, persistent symptoms

Question 52

Features of Slipped capital femoral epiphysis

(3)

Answer 52

Features

hip, groin, medial thigh or knee pain

loss of internal rotation of the leg in flexion

bilateral slip in 20% of cases

Question 53

What are the investigations and management of Slipped capital femoral epiphysis?

Answer 53

Investigation

AP and lateral (typically frog-leg) views are diagnostic

Management

internal fixation: typically a single cannulated screw placed in the center of the epiphysis

Question 54

What is Transient synovitis?

(4)

Answer 54

Transient synovitis is sometimes referred to as irritable hip. It generally presents as acute hip pain associated with a viral infection.

The typical age group is 2-10 years.

A low-grade fever is present in a minority of patients but high fever should raise the suspicion of other causes such as septic arthritis.

It is the commonest cause of hip pain in children.

Transient synovitis is self-limiting, requiring only rest and analgesia.

Question 55

What is Perthes’ disease?

What is the typical age range?

Where does it affect?

(3)

Answer 55

Perthes’ disease is a degenerative condition affecting the hip joints of children, typically between the ages of 4-8 years.

It is due to avascular necrosis of the femoral head, specifically the femoral epiphysis.

Impaired blood supply to the femoral head causes bone infarction.

Question 56

What are the features of Perthes’s disease?

(4)

Answer 56

Features

• hip pain: develops progressively over a few weeks
• limp
• stiffness and reduced range of hip movement
• x-ray: early changes include widening of joint space, later changes include decreased femoral head size/flattening

Question 57

What is the epidemiology of Perthes’ disease?

(3)

Answer 57

children, typically between the ages of 4-8 years

Perthes’ disease is 5 times more common in boys.

Around 10% of cases are bilateral

Question 58

How is Perthes’ disease diagnosed?

What are the complications?

Answer 58

Diagnosis

• plain x-ray
• technetium bone scan or magnetic resonance imaging if normal x-ray and symptoms persist

Complications

• osteoarthritis
• premature fusion of the growth plates

Question 59

You see the mother of a 6-month-old baby who was born premature (at 32 weeks) and has recently been discharged from hospital following an admission with ‘breathing problems’.

Whilst he was in the hospital he was given an injection called palivizumab, however, the mother cannot recall what this medication is for.

What is this medication and its indication?

Answer 59

Palivizumab is a monoclonal antibody which is used to prevent respiratory syncytial virus (RSV) in children who are at increased risk of severe disease.

Those at risk of developing RSV include

• Premature infants
• Infants with lung or heart abnormalities
• Immunocompromised infants

Question 60

A 32-year-old G1P0 woman, who is 32-weeks pregnant, attends her GP surgery complaining of dysuria.

The GP arranges a urine dip and culture. The urine culture grows, a Gram-positive, beta-haemolytic cocci, described in the report as ‘Group B’.

What is the most likely effect on the foetus, from the bacteria identified in the mother’s urine culture?

Answer 60

Maternal Group B Streptoccocus is a risk factor for neonatal sepsis

Question 61

Prenatal rubella infection is associated with which birth defect?

Answer 61

Congenital cataracts and Hearing impairment

Question 62

Congenital cataracts and Hearing impairment is associated with which prenatal infection?

Answer 62

Rubella infection

Question 63

What are the risk factors for neonatal sepsis?

Answer 63

low birth weight

Prolonged rupture of membranes (≥18 hours)

Question 64

A 26-year-old primiparous woman gives birth to her first child at 39 weeks.

The child is delivered via caesarean section.

The baby and mother are well post-delivery. Observations are normal and there are no postoperative complications noted.

During routine post-birth checks, a sample of umbilical cord blood is taken and analysed.

Glucose is recorded as 2.4 mmol/L.

What should the management be?

Answer 64

Encourage early feeding and monitoring blood glucose is the correct answer. Transient hypoglycaemia is common in newborns, and if asymptomatic is not a cause for concern.

In these cases, as in the situation above, encouraging early feeding (either bottle or breast) and monitoring blood glucose until normalised is all that is required.

Question 65

A 16-year-old girl with cystic fibrosis is being reviewed for her annual check-up. She was diagnosed with cystic fibrosis 15 years ago.

She has a good exercise tolerance, minimal gastrointestinal symptoms and has not been hospitalised in the past year.

Her recent investigations show an iron-deficient anaemia on her blood work, and multiple positive sputum cultures for Burkholderia species.

Her latest FEV1 is 60% of her predicted.

What feature of her history confers the greatest increase in mortality?

Answer 65

Chronic infection with Burkholderia species is known to increase mortality

This infection is an opportunistic infection which can cause loss of lung reserve, hospitalisation and disseminated infection. All of these features contribute to the increase in mortality it confers.

Question 66

A 3-month-old baby girl is diagnosed as having developmental dysplasia of the left hip following an ultrasound examination.

Clinical examination of the hip was abnormal at birth.

What treatment is she most likely to be given?

Answer 66

Pavlik harness (dynamic flexion-abduction orthosis)

most unstable hips will spontaneously stabilise by 3-6 weeks of age

older children may require surgery

Question 67

Developmental dysplasia of the hip

Clinical examinations (2)

Answer 67

Clinical examination

Barlow test: attempts to dislocate an articulated femoral head

Ortolani test: attempts to relocate a dislocated femoral head

Question 68

A 9-day-old pre-term neonate stops tolerating his cow’s milk feeds given by the nurses in the special care baby unit.

He vomited after the most recent feed and the nurse noticed bile in the vomit. Stools are normal consistency but the last stool contained fresh red blood.

On examination, he is well hydrated but his abdomen is grossly distended and an urgent abdominal x-ray is requested.

X-ray shows distended loops of bowel with thickening of the bowel wall. What is the next best step in management?

Answer 68

This scenario describes a case of necrotising enterocolitis.

Given the history and examination along with the age and prematurity of the infant, bacterial necrotising enterocolitis is the most likely diagnosis.

Due to the seriousness of this, broad-spectrum antibiotics must be commenced immediately. For this reason, answer 2 is the correct answer.

Question 69

You are performing a newborn baby check and discover during your examination that the neonate has ambiguous genitalia.

What is the most likely cause of ambiguous genitalia?

Answer 69

congenital adrenal hyperplasia is most common cause in newborns

Question 70

What is Klinefelter’s syndrome also known as?

What is the karyotype of Klinfleter’s?

What are the features? (6)

How is it diagnosed?

Answer 70

What is Klinefelter’s syndrome also known as?

primary hypogonadism

What is the karyotype of Klinfleter’s?

Karyotype 47, XXY

What are the features?

• often taller than average
• lack of secondary sexual characteristics
• small, firm testes
• infertile
• gynaecomastia - increased incidence of breast cancer
• elevated gonadotrophin levels

How is it diagnosed?
chromosomal analysis

Question 71

Why are patients with Klinefelter’s normally diagnosed during puberty?

(2)

Answer 71

Individuals with Kallman’s syndrome do not have ambiguous genitalia

They are phenotypically and genotypically male, but have hypogonadotropic hypogonadism.

This is often diagnosed at puberty.

Question 72

During routine post-birth checks, a sample of umbilical cord blood is taken and analysed. Glucose is recorded as 2.4 mmol/L.

What should the management be?

Answer 72

Neonatal hypoglycaemia: if asymptomatic then encourage normal feeds and monitor glucose

Question 73

A 9-day-old pre-term neonate stops tolerating his cow’s milk feeds given by the nurses in the special care baby unit. He vomited after the most recent feed and the nurse noticed bile in the vomit. Stools are normal consistency but the last stool contained fresh red blood. On examination he is well hydrated but his abdomen is grossly distended and an urgent abdominal x-ray is requested. X-ray shows distended loops of bowel with thickening of the bowel wall.

What is the diagnosis?

What is the next best step in management?

Answer 73

Given the history and examination along with the age and prematurity of the infant, bacterial necrotising enterocolitis is the most likely diagnosis.

Due to the seriousness of this, broad-spectrum antibiotics must be commenced immediately.

Question 74

Feverish illness in children Red Flags

Answer 74

• Pale/mottled/ashen/blue

• No response to social cues
• Appears ill to a healthcare professional
• Does not wake or if roused does not stay awake
• Weak, high-pitched or continuous cry
• Grunting
• Tachypnoea: respiratory rate >60 breaths/minute
• Moderate or severe chest indrawing

• Reduced skin turgor

• Age <3 months, temperature >=38°C
• Non-blanching rash
• Bulging fontanelle
• Neck stiffness
• Status epilepticus
• Focal neurological signs
• Focal seizures

Question 75

A 6-year-old boy is brought to the GP by his mother.

He has been experiencing coryza accompanied by a fever of 37.8C for the last 3 days.

This morning his mother noticed a red rash on both cheeks and pallor surrounding his mouth.

Which is the most likely causative organism?

Answer 75

• In this scenario, the boy presents with slapped-cheek syndrome, also known as erythema infectiosum.
• He has a typical presentation of coryza and fever followed by a red rash.
• This infection is caused by parvovirus b19

Question 76

Define coryza

Answer 76

runny nose

Question 77

Features of chickpox

(3)

Answer 77

• Fever initially
• Itchy, rash starting on head/trunk before spreading. Initially macular then papular then vesicular
• Systemic upset is usually mild

Question 78

Which disease presents with a fever initially, then developed into an itchy rash on the head/trunk before spreading.

The rash is initially macular, then papular, then vesicular.

Answer 78

Fever initially

Itchy, rash starting on head/trunk before spreading. Initially macular then papular then vesicular

Systemic upset is usually mild

Question 79

Features of measles

Answer 79

• Early symptoms: irritable, conjunctivitis, fever
• Koplik spots: white spots (‘grain of salt’) on buccal mucosa
• Rash: starts behind ears then to whole body, discrete maculopapular rash becoming blotchy & confluent

Question 80

Which childhood disease initially presents with irritability, conjunctivitis, fever.

Koplik spots are also present on the buccal mucosa

A maculopapular rash then develops behind the ears, spreading to the whole body.

Answer 80

Measles

Question 81

Features of rubella

(2)

Answer 81

• Rash: pink maculopapular, initially on face before spreading to whole body, usually fades by the 3-5 day
• Lymphadenopathy: suboccipital and postauricular

Question 82

Which common childhood disease presents with:

Lymphadenopathy: suboccipital and postauricular and pink maculopapular rash, initially on face before spreading to whole body, which usually fades by the 3-5 day

Answer 82

Rubella

Question 83

What are the features of Erythema infectiosum infection?

(4)

Answer 83

• Also known as fifth disease or ‘slapped-cheek syndrome’
• Caused by parvovirus B19
• Lethargy, fever, headache
• ‘Slapped-cheek’ rash spreading to proximal arms and extensor surfaces

Question 84

A child has slapped cheek syndrome, lethargy, fever and headache.

What is the most likely diagnosis?

(2)

Answer 84

Erythema infectiosum Caused by parvovirus B19

Question 85

What are the features of scarlet fever?

(4)

Answer 85

• Reaction to erythrogenic toxins produced by Group A haemolytic streptococci
• Fever, malaise, tonsillitis
• ‘Strawberry’ tongue
• Rash - fine punctate erythema sparing the area around the mouth (circumoral pallor)

Question 86

A young patient has fever malaise, tonsilitis and strawberry tongue.

There is also a punctate rash around the body, sparing the area around the mouth.

Answer 86

Scarlet fever

Question 87

define punctate

Answer 87

studded with or denoting dots or tiny holes.

Question 88

Which conditions present with “strawberry tongue”?

(3)

Answer 88

Kawasaki disease

Strawberry naevus

Scarlet fever

Question 89

What are the features of Hand, foot and mouth disease?

Answer 89

• Caused by the coxsackie A16 virus
• Mild systemic upset: sore throat, fever
• Vesicles in the mouth and on the palms and soles of the feet

Question 90

A 4-year-old boy is reviewed in the Paediatric Admissions Unit. He has had a fever for the past week.

On examination, he has red, sore lips and conjunctival injection. He also has swollen, red hands.

Answer 90

High fever lasting >5 days, red palms with desquamation and strawberry tongue are indicative of Kawasaki disease

Question 91

Which immunisations are included in the 6 in 1 vaccine?

At what age is this vaccine given?

Answer 91

1. Diphtheria
2. tetanus
3. pertussis
4. polio
5. Haemophilus influenzae Type B Infection
6. hepatitis B

Current guidelines suggest giving it at 8 weeks

Question 92

What is Vesicoureteral reflux?

Answer 92

abnormal flow of urine from your bladder back up the tubes

Question 93

What is a Micturating cystourethrogram?

Answer 93

a contrast x-ray of the urethra+bladder+ureters

Question 94

What is the pathophysiology of Vesicoureteric reflux (VUR)

Answer 94

Pathophysiology of VUR

• ureters are displaced laterally, entering the bladder in a more perpendicular fashion than at an angle
• therefore shortened intramural course of the ureter
• vesicoureteric junction cannot, therefore, function adequately

Question 95

You are running a Thursday afternoon clinic and a father comes in with his 12-year-old son who has developed new pustular, honey-coloured crusted lesions over his chin.

You diagnose localised non-bullous impetigo.

What is your management plan?

Answer 95

Prescribe topical hydrogen peroxide 1% cream and advise them that the child should be excluded from school until the lesions are crusted and healed.

A child with impetigo should be excluded from school until the lesions are crusted and healed or 48 hours after commencing antibiotic treatment

Question 96

What is Impetigo?

How is it managed?

Answer 96

Impetigo is a superficial bacterial skin infection usually caused by either Staphylcoccus aureus or Streptococcus pyogenes

• NICE Clinical Knowledge Summaries now recommend hydrogen peroxide 1% cream for ‘people who are not systemically unwell or at a high risk of complications’

Question 97

Which conditions require no time off school?

(7)

Answer 97

1. Conjunctivitis
2. Fifth disease (slapped cheek)
3. Roseola
4. Infectious mononucleosis
5. Head lice
6. Threadworms
7. Hand, foot and mouth

Question 98

How long should patients with scarlet fever stay off school?

Answer 98

24 hours after commencing antibiotics

Question 99

How long should patients with Whooping cough stay off school?

Answer 99

24 hours after commencing antibiotics

Question 100

How long should patients with measles stay off school?

Answer 100

4 days after commencement of the rash

Question 101

How long should patients stay off school with rubella?

Answer 101

5 days from the onset of the rash

Question 102

When can patients with chickenpox go back to school?

Answer 102

When all the lesions have crusted over

Question 103

How long should patients with mumps

Answer 103

5 days from the onset of swollen glands

Question 104

How long should patients with impetigo wait before going back to school?

Answer 104

Until lesions are crusted and healed,

or

48 hours after commencing antibiotic treatment

Question 105

When can patients with diarrhoea and vomiting go back to school

Answer 105

48 hours after the previous episode

Question 106

How long should patients with scabies wait before returning to school?

Answer 106

until treated

Question 107

How long should patients with Influenza wait before returning to school?

Answer 107

until recovered

Question 108

What is fragile X syndrome?

What are the clinical features?

Answer 108

Fragile X syndrome is a trinucleotide repeat disorder.

Features in males

• learning difficulties
• large low set ears, long thin face, high arched palate
• macroorchidism
• hypotonia
• autism is more common
• mitral valve prolapse

Question 109

How is fragile x syndrome diagnosed?

Answer 109

Diagnosis

• can be made antenatally by chorionic villus sampling or amniocentesis
• analysis of the number of CGG repeats using restriction endonuclease digestion and Southern blot analysis

Question 110

A 12-hour old baby is reviewed on the neonatal intensive care unit after his nurse noticed tremors movements of his limbs.

He’s been noted to be taking poorly to feeds, more irritable and more drowsy than usual.

He was born at 34 weeks, by emergency caesarean section for reduced foetal movements and foetal bradycardia. His mother has a relatively healthy pregnancy, apart from needing to take carbamazepine for epilepsy.

On examination, he was notably bigger than expected, adjusted for prematurity. He is visibly jittery on arm movements when moving around.

What is the most likely diagnosis?

Which part of his history puts him at risk for this?

Answer 110

Preterm birth (< 37 weeks) is a key risk factor for neonatal hypoglycaemia

This baby has the classical signs of neonatal hypoglycaemia, with the autonomic features of irritability and jitteriness, and the neuroglycopenic features of drowsiness and poor feeding. Neonatal hypoglycaemia has various risk factors, which can be thought of as due to the inability to regulate glucose or reduced glycogen stores.

A pre-term infant will not yet have developed the same glycogen reserve that a term infant will have done.

Therefore, prematurity is an important risk factor for the development of neonatal hypoglycaemia.

Question 111

A four-year-old child with poorly controlled asthma attends GP surgery with his mother due to increasing frequency of his asthma exacerbations. He is already on salbutamol inhaler as required and beclometasone inhaler 200mcg/day. He uses these devices with a spacer and has good technique.

What is the next best step in his management?

Answer 111

Child aged < 5 years with asthma not controlled by a SABA + paediatric low-dose ICS - asthma management in children < 5 years - add a leukotriene receptor antagonist

Question 112

What are leukotrienes?

(2)

Answer 112

• Leukotrienes are inflammatory chemicals the body releases after coming in contact with an allergen or allergy trigger.
• Leukotrienes cause tightening of airway muscles and the production of excess mucus and fluid.

Question 113

You see a worried mum with her 6 month old baby boy. She is concerned that his skull shape is not normal.

His occiput is flattened on the left, his left ear mildly protruding forward and his left forehead more prominent than the right.

No other abnormality is detected. What is the most appropriate management?

Answer 113

Reassurance

Plagiocephaly

• parallelogram shaped head
• the incidence of plagiocephaly has increased over the past decade. This may be due to the success of the ‘Back to Sleep’ campaign

Question 114

A 6-year-old boy is brought to the emergency department after fever for 6 days and has not been himself. He has given him paracetamol but this has had no effect.

On examination, the boy has bright red cracked lips, injection of the conjunctiva, palpable lymph nodes in the cervical region and the palms of his hands and soles of his feet are red.

What is the most likely diagnosis?

What medication should be administered?

Answer 114

High dose aspirin is indicated in Kawasaki disease, despite it usually being contraindicated in children

Question 115

How are patients with scarlet fever treated?

Answer 115

Phenoxymethylpenicillin (penicillin V)

Question 116

What is Benzylpenicillin (penicillin G) used to treat?

Answer 116

Benzylpenicillin (penicillin G) is indicated for patients with various infections, including meningitis and endocarditis.

Question 117

At what age should a child be able to sit without support?

Answer 117

7-8 months

Question 118

At what age should a child be able to crawl?

Answer 118

9 months

Question 119

At what age should a child be able to walk unsupported?

Answer 119

12-15 months

Question 120

At what age should a child be able to squat to pick up a toy?

Answer 120

18 months

Question 121

At what age should a child be able to run?

Answer 121

two years

Question 122

At what age should a child be able to walk upstairs without a rail?

Answer 122

3 years

Question 123

At what age should a child be able to hop on one leg?

Answer 123

4 years

Question 124

A 2-month-old girl is brought to surgery with poor feeding and vomiting.

The mother reports that her urine has a strong smell.

A urinary tract infection is suspected.

What is the most appropriate management?

Answer 124

Refer immediately to hospital

• infants less than 3 months old should be referred immediately to a paediatrician

Question 125

A 6-week-old boy presents to the paediatric emergency department with fever, vomiting and reduced appetite for the last 48 hours. There are no concerns regarding his growth and development so far.

On examination, the patient appears to be lethargic and unsettled. He is tachypnoeic and his temperature is 39ºC. His blood pressure and pulse rate are within the normal range. There are no signs suggesting raised intracranial pressure.

1. What test should be performed?
2. What is the likely diagnosis?
3. What is the likely treatment?

Answer 125

1. Lumbar puncture
2. Bacterial meningitis
3. Empirical antibiotics

Question 126

A 6-week old boy has meningitis.

What are the likely causative organisms for his age?

Answer 126

Group B streptococcus

E.coli

Listeria monocytogenes

Question 127

A 6-year old boy has meningitis.

What are the likely causative organisms for his age?

Answer 127

Neisseria meningitidis

Streptococcus pneumoniae

Question 128

A 7-year-old boy has a diagnosis of asthma which has been treated with salbutamol as and when required.

His mother feels that his symptoms have worsened and he now has a night time cough which is keeping him awake and affecting him at school.

The examination is unremarkable. How should you manage this?

Answer 128

In this question, as is currently common in clinical practice, the child is not taking an inhaled corticosteroid.

Question 129

You review a 10-year-old girl with increasing constipation over the past 4 years, having normal bowel habits beforehand. The rest of the history and examination are normal. She is on no medication at present.

What would be an appropriate next step in the management?

Answer 129

Osmotic laxative

An osmotic laxative should be prescribed initially as the stool is likely to be hard.

A stimulant laxative may also be required, but only once the stools are soft.

Question 130

A 4-month-old baby is brought to the Emergency Department with vomiting for the past 3 days.

His mother describes the vomiting as projectile that occurs after every feed and is not settling.

Heart rate is 140bpm, respiratory rate is 36/min, blood pressure is 90/60mmHg, capillary refill is 3 seconds and mucous membranes are slightly dry. He is afebrile.

The paediatric registrar comes down to review the baby and decides to admit him for further investigation. Which of the following investigations would be most useful to determine the diagnosis?

Answer 130

The infant in this scenario demonstrates classical signs of pyloric stenosis.

Projectile vomiting after every feed in a young baby should increase the index of suspicion for possible pyloric stenosis.

Question 131

Pyloric stenosis epidemiology

(4)

Answer 131

incidence of 4 per 1,000 live births

4 times more common in males

10-15% of infants have a positive family history

first-borns are more commonly affected

Question 132

Pyloric stenosis features

(4)

Answer 132

Features

‘projectile’ vomiting, typically 30 minutes after a feed

constipation and dehydration may also be present

a palpable mass may be present in the upper abdomen

hypochloraemic, hypokalaemic alkalosis due to persistent vomiting

Question 133

How is pyloric stenosis diagnosed and managed?

Answer 133

Diagnosis is most commonly made by ultrasound.

Management is with Ramstedt pyloromyotomy.

Question 134

A 4-month-old boy is brought to the emergency department by his concerned mother.

She says that she first noticed he had a mild fever and runny nose around 5 days ago, and over the last few days, he appears to have become more breathless and is grunting when he breathes.

She is particularly concerned because she struggles to get him to feed and reports that his nappies are not as wet as normal.

On examination, you notice chest recessions, wheeze and bilateral inspiratory crackles.

Given the likely diagnosis, what is the most appropriate treatment?

Answer 134

Bronchiolitis does not require antibiotics, children requires supportive management only

Question 135

A 2-year-old boy is taken to his GP with a 1 day history of right-sided limp.

His parents report him being otherwise fit and well apart from a recent cold and his nursery deny observing any physical trauma.

On examination, he is afebrile and evidently in pain however has a normal range of movement in the right hip.

What would be the most appropriate management at this stage?

Answer 135

Urgent assessment should be arranged for a child < 3 years presenting with an acute limp

Nice Clinical Knowledge Summaries advise that all children with an acute limp < 3 years of age should be urgently assessed in secondary care because they are at higher risk of septic arthritis and child maltreatment.

Question 136

You are asked to review a neonate on the labour ward.

They were born at 40 weeks gestation with no complications.

Parents are reluctant to administer vitamin K to the baby, stating they prefer a more natural approach.

What advice would you give to the parents regarding best practice in neonatal vitamin K?

Answer 136

Both oral and IM routes of vitamin K are licensed for neonates, but IM should be recommended to parents due to reduced concerns about compliance and shorter (one-off) treatment duration

Vitamin K is very important in preventing haemorrhagic disease of the newborn. It can be given either intramuscularly or orally.

Question 137

Why are newborns given vitamin K?

(3)

Answer 137

• Newborn babies are relatively deficient in vitamin K.
• This may result in impaired production of clotting factors which in turn can lead to haemorrhagic disease of the newborn (HDN).
• Bleeding may range from minor brushing to intracranial haemorrhages

NB: Breast-fed babies are particularly at risk as breast milk is a poor source of vitamin K. Maternal use of antiepileptics also increases the risk

Question 138

A 7-year-old with a one day history of a painful right hip. Just about able to walk but painful. Looks flushed and has a temperature of 38.7ºC

Answer 138

Septic arthritis/osteomyelitis

Question 139

An 8-month-old child is noted to have a discrepancy between the skin creases behind the right and left hips

Answer 139

Development dysplasia of the hip

Question 140

An obese 13-year-old boy presents with a two week history of right sided knee pain associated with a stiff right hip. There is no history of trauma

Answer 140

Slipped upper femoral epiphysis

Question 141

A 2-month-old boy is brought to the general practitioner by his mother.

Since birth he has been feeding poorly, is often agitated and has a blue-tinge to his lips after prolonged episodes of crying.

He was born at term, weighing 2400 grams at delivery.

Examination reveals an ejection systolic murmur at the left sternal edge.

Which of the following is the most likely diagnosis?

Answer 141

Tetralogy of Fallot: Cyanosis or collapse in first month of life, hypercyanotic spells. Ejection systolic murmur at left sternal edge

Question 142

What are the four features of tetralogy of fallot

(4)

Answer 142

ventricular septal defect (VSD)

right ventricular hypertrophy

right ventricular outflow tract obstruction, pulmonary stenosis

overriding aorta

Question 143

Secondary features of tetralogy of Fallot

(4)

Answer 143

cyanosis

causes a right-to-left shunt

ejection systolic murmur due to pulmonary stenosis (the VSD doesn’t usually cause a murmur)

chest x-ray shows a ‘boot-shaped’ heart, ECG shows right ventricular hypertrophy

Question 144

A 16-year-old girl comes to your GP surgery worried that she has not yet started her periods.

She is quite short, has a webbed neck, low set ears and widely spaced nipples.

A heart murmur is heard on auscultation.

What is the most likely diagnosis?

What type of murmur are you most likely to hear?

Answer 144

Turner’s syndrome is associated with an ejection systolic murmur due to bicuspid aortic valve

Question 145

Which murmur is associated with Turner’s syndrome?

(2)

Answer 145

ejection systolic murmur due to bicuspid aortic valve

Question 146

A 6-year-old girl presents with her mother who notices that her daughter often gets out of breath when climbing the stairs at home. .

On examination, small multiple bruises, of varying ages are seen on both her lower legs.

Cardiac examination reveals a soft systolic murmur heard on the left sternal edge. Examination of her abdomen reveals a palpable mass in the left and right hypochondriac regions.

What is the most likely diagnosis?

Answer 146

Hepatosplenomegaly and the presence of bruising together with the symptoms of anaemia (soft systolic murmur and shortness of breath on exertion) suggest ALL.

Acute lymphoid leukaemia

Question 147

The parents of a 14-month-old girl present to their GP. They have noticed that in some photos there is no ‘red eye’ on the left hand side. When you examine the girl you notice an esotropic strabismus and a loss of the red-reflex in the left eye. There is a family history of a grandparent having an enucleation as a child. What is the most likely diagnosis?

Answer 147

A congenital cataract may cause a loss of the red-reflex but is likely to have been detected at birth or during the routine baby-checks. It would also not explain the family history of enucleation.

Question 148

How would you differentiate from acute epiglottitis and croup?

Answer 148

Acute epiglottitis

• 2 and 6 years
• rapid onset
• unwell, toxic child
• stridor
• drooling of saliva

Croup

• 6 months - 3 years
• more common in autumn
• stridor
• barking cough (worse at night)
• fever
• coryzal symptoms

Question 149

Meconium ileus is a common neonatal feature of what?

Answer 149

cystic fibrosis

Question 150

When is the neonatal blood spot screening test typically performed in the United Kingdom

Answer 150

Neonatal blood spot screening (previously called the Guthrie test or ‘heel-prick test’) is performed at 5-9 days of life

Question 151

Neonatal blood spot screening tests

(9)

Answer 151

1. congenital hypothyroidism
2. cystic fibrosis
3. sickle cell disease
4. phenylketonuria
5. medium chain acyl-CoA dehydrogenase deficiency (MCADD)
6. maple syrup urine disease (MSUD)
7. isovaleric acidaemia (IVA)
8. glutaric aciduria type 1 (GA1)
9. homocystinuria (pyridoxine unresponsive) (HCU)

Question 152

A 2-year-old boy presents with a harsh cough and pyrexia. His symptoms worsened overnight and on examination stridor is noted. Which one of the following interventions may improve his symptoms?

Answer 152

Croup - A single dose of oral dexamethasone (0.15 mg/kg) is to be taken immediately regardless of severity

Question 153

A 38-year-old foster parent brings in her 5-year-old foster child to the GP. He has been complaining of pain when going to the toilet.

The foster mother explains that he often has pain when urinating and as a younger child often cried when passing urine.

He has only recently developed pain while defecating, however, the foster mother is clearly concerned and consents to the GP examining the child.

Which clinical finding is most likely to be indicative of child sexual abuse?

Answer 153

Anal fissures and recurrent UTIs in children can be indicative of sexual abuse

Question 154

Features of childhood sexual abuse can include:

(8)

Answer 154

1. pregnancy
2. sexually transmitted infections, recurrent UTIs
3. sexually precocious behaviour
4. anal fissure, bruising
5. reflex anal dilatation
6. enuresis and encopresis
7. behavioural problems, self-harm
8. recurrent symptoms e.g. headaches, abdominal pain

Question 155

A 7-week-old baby boy is brought to his GP surgery as his mum is concerned that his feeding has been poor over the last 24hours.

She has noticed he feels warm but hasn’t noticed any cough or coryzal symptoms. He is due to have his immunisations next week.

He has had a wet and dirty nappy today and she noticed his urine smelt very strongly.

On examination, he has a raised temperature at 38.9ºC. He is irritable but examination of his chest and abdomen is otherwise normal.

What is the most appropriate management for this child?

Answer 155

Children under 3 months with a suspected UTI should be referred to specialist paediatrics services

Question 156

Why do neonates have a greater risk of hyperbilirubinaemia?

Answer 156

Neonatal blood cells have a life cycle of 70 days, compared to adult red blood cells’ 120 days

there is also less efficient hepatic metabolism

Question 157

What is Kernicterus?

Answer 157

deposition of bilirubin into the brain parenchyma

about half of free bilirubin is free circulating

this can cross into the blood-brain barrier

bilirubin should be conjugated

Question 158

Which parts of the brain are particularly affected by bilirubin?

(2)

Answer 158

basal ganglia

responsible for the coordination of movement

Question 159

Why would a baby with neonatal jaundice not respond to light therapy?

Answer 159

if the bilirubin is conjugated, usually intrahepatic:

bile duct obstruction

galactosaemia

alpha-1-antitrypsin deficiency

Question 160

Which of the following is a cause of unconjugated hyperbilirubinemia in infants?

Choledochal cyst

Physiologic jaundice

Bile duct obstruction

Cystic fibrosis

Neonatal hepatitis

Answer 160

Physiologic jaundice

it is the only non-intrahepatic cause of hyperbilirubinaemia

Question 161

How might you measure neonatal jaundice?

Answer 161

blood sample

transcutaneous meter

Question 162

How does age factor into neonatal jaundice?

Answer 162

if the baby is <24 hours old, more likely to be haeomolysis

if the child is >2 weeks old (3 weeks if preterm, the problem is more likely to be an unconjugated syndrome

Question 163

Risk factors for neonatal jaundice

Answer 163

• haemolysis? if the mother is type O blood group
• if the Mediterranean, far east or afro, G6PD deficiency

Question 164

in a foetus, what is the direction of blood flow?

Answer 164

right to left

pulmonary artery to the aorta

n.b. pulmonary pressure is high, keeping the ductus arteriosis open

Question 165

ina a neonate which direction of shunt is found in patent ductus arteriosis?

Answer 165

left to right shunt

aorta to pulmonary artery

Question 166

What is the PaO2 in uterus?

What is the PaO2 in at birth?

Answer 166

25mmHg

60-80mmHg

it changes as the lungs fill with air

Question 167

What week does surfactant synthesis occur?

Answer 167

28 weeks

Question 168

Name the three main types of newborn respiratory pathologies:

(3)

Answer 168

Transient tachypnea of newborn (TTN)

Respiratory Distress Syndrom (RSD)

Persistent pulmonary hypertension (PPHT)

Question 169

Describe the mechanism of Transient tachypnea of newborn (TTN)

Which age baby is likely to get it?

What is the Mechanism

Answer 169

• tachypnoea (fast breathing)
• GRUNTING
• caused by failure to clear lung fluid
• use of accessory muscles
• nasal flaring

frequently seen in “late preterms” 34-37 weeks

Mechanism

decreased expression of amioloride-sensitive airway epithelial cell Na+ ion channels, delaying lung fluid clearance

Question 170

Describe the mechanism of Respiratory distress syndrome (RDS)

Which age baby is likely to get it?

What is the Mechanism

Answer 170

Features include:

• hypoxaemia
• blue-coloured lips, fingers and toes
• rapid, shallow breathing
• flaring nostrils
• a grunting sound when breathing

almost always seen in premature ~28 weeks babaies

caused by insufficient surfactant production; treated with steroids and CPAP

Question 171

What are the side effects of giving oxygen to a neonate?

(3)

Answer 171

O2 produces free radicals which cause

retinopathy of prematurity

and brochodysplasia

Question 172

What is port wine stain also known as?

Answer 172

Sturge–Weber syndrome

Question 173

Name three vascular birthmarks

Answer 173

port wine stain (Sturge–Weber syndrome)

salmon patches

Strawberry naevus

Question 174

What are Salmon patches?

(4)

Answer 174

Salmon patches are a kind of vascular birthmark which can be seen in around half of newborn babies they are also known as stork marks or stork bites.

They are pink and blotchy, and commonly found on the forehead, eyelids and nape of the neck.

They usually fade over a few months, though marks on the neck may persist.

Question 175

What are Strawberry naevi?

(4)

Answer 175

Strawberry naevi (capillary haemangioma) are usually not present at birth but may develop rapidly in the first month of life. They appear as erythematous, raised and multilobed tumours.

Typically they increase in size until around 6-9 months before regressing over the next few years (around 95% resolve before 10 years of age).

Common sites include the face, scalp and back. Rarely they may be present in the upper respiratory tract leading to potential airway obstruction

Capillary haemangiomas are present in around 10% of white infants.

Question 176

What is the epidemiology of strawberry naevi?

(2)

Answer 176

Female infants, premature infants and those of mothers who have undergone chorionic villous sampling are more likely to be affected

they rarely appear at birth and grow during the first month

Question 177

Potential complications of strawberry naevus

(4)

Answer 177

mechanical e.g. Obstructing visual fields or airway

bleeding

ulceration

thrombocytopaenia

Question 178

When would treatment of strawberry naevus be required?

How would it be treated?

Answer 178

If treatment is required (e.g. Visual field obstruction)

propranolol is increasingly replacing systemic steroids as the treatment of choice.

Topical beta-blockers such as timolol are also sometimes used.

Question 179

What is a cephalohematoma?

Answer 179

A cephalohematoma is an accumulation of blood under the scalp.

During the birth process, small blood vessels on the head of the fetus are broken as a result of minor trauma.