Day 6

Question 1

Common knee joint problems in children and young adults

(6)

Answer 1

Question 2

A 7-year-old boy is brought in to the GP surgery with an exacerbation of asthma.

On examination he has a bilateral expiratory wheeze but there are no signs of respiratory distress.

His respiratory rate is 24 / min and PEF around 60% of normal.

What is the most appropriate action with regards to steroid therapy?

Answer 2

3 days of prednisalone

The 2016 British Thoracic Society guidelines state the following with respect to steroid treatment in children:

Use a dose of 10 mg prednisolone for children under 2 years of age, 20 mg for children aged 2-5 years and 30-40 mg for children >5 years. Those already receiving maintenance steroid tablets

Question 3

A woman brings her daughter to general practice concerned that she hasn’t started her periods yet. Her daughter is 15 years old.

On general examination, she is in the 9th percentile of height for her age, has short ring fingers, poor breast development, and a high arched palate.

On auscultation of her heart, you note a crescendo-decrescendo murmur on the upper right sternal border which radiates to the carotids.

What is the most likely diagnosis?

What type of murmur is a crescendo-decrescendo murmur?

What is the most likely cause of this murmur?

Answer 3

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

bicuspid aortic valve (15%)

coarctation of the aorta (5-10%)

Question 4

Which cardiac deformity is caused by rheumatic fever?

What is the acute presentation of rheumatic fever?

Answer 4

Streptococcal rheumatic heart disease can cause aortic stenosis

history of polyarthritis and fever, possibly with acute cardiac involvement.

Question 5

What can cause aortic stenosis?

(2)

Answer 5

Rheumatic heart disease - streptococcal rheumatic heart disease

William’s syndrome - This is a genetic condition caused by a deletion on chromosome seven. It is associated with aortic stenosis

Question 6

What is the acute presentation of rheumatic fever?

(3)

What is a long term complication?

(1)

Answer 6

history of polyarthritis and fever, possibly with acute cardiac involvement

aortic stenosis

Question 7

William’s syndrome features

(5)

Answer 7

This is a genetic condition caused by a deletion on chromosome seven.

It is associated with aortic stenosis,

patients typically present with intellectual disability, underdeveloped cheeks, short noses, broad foreheads, and a happy affect

Question 8

A 13-year-old girl presents to the emergency department complaining of coryzal symptoms.

She has been experiencing a fever, runny nose, headache and a non-productive cough for several days.

She appears comfortable, alert and well hydrated.

Her mother is concerned because despite giving her paracetamol and ibuprofen, her temperature does not seem to be coming down.

She took the last dose of antipyretics one hour ago.

On examination, her temperature is 38.9ºC, her heart rate is 110bpm, her blood pressure is 90/70mmHg and her respiratory rate is 26 breaths per minute.

Her chest sounds clear, her throat is slightly red with no signs of tonsillitis.

Her ears are non-tender and otoscopy shows no abnormalities.

She does not have any rashes and shows no signs of photophobia or neck stiffness.

How should this patient be managed?

Answer 8

Initiate sepsis six protocol

BUFALO

Children aged over 12 have similar normal vital signs to an adult

Question 9

A 4-year-old boy presents with a 2-day history of passing loose stools and non-bilious vomiting. He has passed 5 loose stools and vomited 2 times over the last 48 hours. No visible mucus or blood seen in the stool, and urine output has not changed according to his mother. He is able to tolerate oral fluid and liquid food.

He has not travelled abroad recently and there are no sick contacts. His vaccination schedule is up-to-date and there are no concerns regarding his growth and development.

On examination, he appears well and is alert and responsive. He has warm extremities and capillary refill time is <2 seconds. His vital signs are normal. Peripheral pulses are strong and regular. There is normal skin turgor and there are no sunken eyes.

How should the patient be managed?

Answer 9

• Introduce oral rehydrating solution (ORS)
• Do not use antidiarrhoeal medications in children under 5 years old with diarrhoea and vomiting caused by gastroenteritis
• Discourage the drinking of fruit juices and carbonated drinks, especially in those at increased risk of dehydration [NICE 2009]. Fruit juice without extra water or carbonated drinks has too much sugar in it and this can draw water from the body into the gut, making the child more dehydrated.
• Do not routinely give antibiotics to children with gastroenteritis [NICE 2009] because treatment is not effective on symptoms and does not prevent complications.

Question 10

Causes of chronic diarrhoea in infants

(4)

Answer 10

• most common cause in the developed world is cows’ milk intolerance
• toddler diarrhoea: stools vary in consistency, often contain undigested food
• coeliac disease
• post-gastroenteritis lactose intolerance

Question 11

GI causes of vomiting in children

Obstructive (6)

Inflammatory (4)

CNS (4)

Answer 11

Obstructive (6)

• Foreign bodies
• Idiopathic Pyloric Stenosis
• Intussusception
• Indirect inguinal hernias
• Volvulus
• Appendicitis

Inflammatory (4)

• Gastroenteritis
• Hepatitis
• Peptic Ulcer Disease
• Other uncommon: pancreatitis, cholecystitis

CNS (4)

• Head injury
• migraine
• brain tumour
• CNS infection

Question 12

Diagnostic criteria of migraines

(5)

Answer 12

Diagnosis of exclusion; usually 3 or more of:

• abdominal pain
• nausea or vomiting
• throbbing headache, unilateral location
• associated aura (usually preceding the headache)
• relief following sleep

Question 13

A mother brings her 10-month-old child to the emergency department late at night due to respiratory distress. The mother states that the child did not eat dinner that night and has since started drooling at the mouth, looking very unwell and the child is now petrified of being moved. Questioning reveals that the child is late for their routine vaccinations for their age, but the mother is keen on completing the course.

The patient has a heart rate of 150 beats per minute, a temperature of 39.4ºC, blood pressure of 88/60mmHg (normal for age: ~90/60mmHg) and a respiratory rate of 28 breaths per minute.

What is the most likely diagnosis?

Which other symptoms are most likely to be found in this patient?

Answer 13

Acute epiglottis

Patients with acute epiglottis may adopt the ‘tripod’ position

Abrupt onset and rapid progression (within hours) of dysphagia, drooling, and distress (‘the three D’s’)

Question 14

You are junior doctor at a GP practice.

Your next patient is a 3-year-old girl who is notably irritable and teary.

Her father explains that she has had a reduced appetite for the past couple of days.

On examination you note multiple vesicles over both palms and around the mouth. She is also pyrexial.

Given the likely diagnosis, what is the most appropriate next step?

Answer 14

Hand, foot and mouth disease requires symptomatic treatment only

Question 15

You are reviewing the growth of a 4-week-old neonate. She has a length on the 35th percentile, weight on the 42nd percentile and a head circumference on the 4th percentile.

Which of the following is the most likely cause of her microcephaly?

Answer 15

Foetal alcohol syndrome - associated with microcephaly

Question 16

A 6-year-old boy is brought for review.

You can see from his records that he has been treated for constipation in the past but is otherwise fit and well.

His mother reports that he is currently passing only one hard stool every 4-5 days.

The stool is described as being like ‘rabbit droppings’.

There is no history of overflow soiling or diarrhoea.

Examination of the abdomen is unremarkable.

What is the most appropriate first-line intervention?

Answer 16

Advice on diet/fluid intake + Movicol Paediatric Plain

Constipation in children: macrogols (e.g. Movicol) is first-line

Question 17

A newborn is found to have a number of congenital abnormalities including an extra finger on each hand, a cleft palate and lip, microphthalmia and microcephaly.

Which of the following chromosomes is most likely to be affected in this child?

Answer 17

A baby is born with microcephaly, small eyes, low-set ears, cleft lip and polydactyly - Patau syndrome

Trisomy 13

Question 18

Which infection commonly causes a crop of white spots on the inside of the mouth?

Answer 18

Measles - Koplik spots

Question 19

What is the prodrome of measles? (3)

What maybe seen inside the mouth? (1)

Describe the rash distribution in measles (2)

Describe the rash appearance in measles (4)

Answer 19

Prodrome: 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 20

Key features of mumps

(2)

Answer 20

Fever, malaise, muscular pain

Parotitis (‘earache’, ‘pain on eating’): unilateral initially then becomes bilateral in 70%

Question 21

Key features of Rubella

(4)

Answer 21

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

Lymphadenopathy: suboccipital and postauricular

Question 22

Key features of Erythema infectiosum

(4)

Answer 22

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 23

A 6-month-old baby who was born in Bangladesh is brought to surgery. Around one week ago he started with coryzal symptoms. His mother reports he has not been feeding well for the past two days and has started to vomit today. Her main concern is a cough which occurs in bouts and is so severe he often turns red. No inspiratory or expiratory noises are noted. Clinical examination reveals an apyrexial child with a clear chest.

\What is the most likely diagnosis?

Answer 23

Whooping cough (pertussis)

Diagnostic criteria

Whooping cough should be suspected if a person has an acute cough that has lasted for 14 days or more without another apparent cause, and has one or more of the following features:

• Paroxysmal cough.
• Inspiratory whoop.
• Post-tussive vomiting.
• Undiagnosed apnoeic attacks in young infants.

Question 24

A 5-month-old baby is admitted with poor feeding. Over the last 24 hours, they have fed 25% of their usual amount and have had significantly fewer wet nappies than usual. The baby has been particularly irritable over this time and is not settling. There is no past medical history, no known allergies, and there has been no travel outside of the UK.

On examination, the baby appears unsettled and grouchy. There are no rashes on exposure and the fontanelles appear normal.

The physical observations reveal tachycardia and a fever of 39ºC.

When you attempt to manually flex the baby’s neck you note that they also flex the hips and knees.

Which of the following is the most appropriate empirical intravenous treatment?

Answer 24

Ceftriaxone

Initial empirical therapy for meningitis if > 3 months of age: IV 3rd generation cephalosporin

Antibiotics

< 3 months: IV amoxicillin (or ampicillin) + IV cefotaxime

> 3 months: IV cefotaxime (or ceftriaxone)

Question 25

Most common cause of croup

Answer 25

parainfluenza virus

Question 26

How is meningitis treated in kids? 5 stages

Answer 26

**1. Antibiotics** * \< 3 months: IV amoxicillin (or ampicillin) + IV cefotaxime * \> 3 months: IV cefotaxime (or ceftriaxone) **2. Steroids** * NICE advise against giving corticosteroids in children younger than 3 months * dexamethsone should be considered if the lumbar puncture reveals any of the following: frankly purulent, CSF, CSF white blood cell count greater than 1000/microlitre, raised CSF white blood cell count with protein concentration greater than 1 g/litre, bacteria on Gram stain **3. Fluids** * treat any shock, e.g. with colloid **4. Cerebral monitoring** * mechanical ventilation if respiratory impairment **5. Public health notification and antibiotic prophylaxis of contacts** * ciprofloxacin is now preferred over rifampicin

Question 27

A baby boy is delivered by emergency caesarean section at 42 weeks and 6 days because of fetal tachycardia and thick meconium-stained amniotic fluid. Intrapartum antibiotics were given as the mother was known to be colonised with group B streptococcus. The baby is cyanosed and tachypnoeic with chest wall retraction. Chest X-ray shows patchy infiltrations and atelectasis. What is the likely diagnosis? (3) What predisposes babies to it? (3)

Answer 27

* **Meconium aspiration syndrome** refers to respiratory distress in the newborn as a result of meconium in the trachea. * It is more common in post-term deliveries, with rates of up to 44% reported in babies born after 42 weeks. * It causes respiratory distress, which can be severe. * **Higher rates occur where** there is a history of maternal hypertension, pre-eclampsia, chorioamnionitis, smoking or substance abuse.

Question 28

Atelectasis definition

Answer 28

Atelectasis (at-uh-LEK-tuh-sis) is a complete or partial collapse of the entire lung or area (lobe) of the lung.

Question 29

A 4-month-old boy is failing to gain weight and has had recurrent chest infections since birth. On examination, he looks thin and tired. Auscultation of the precordium reveals a **continuous murmur heard loudest under the left clavicle**. What is the most likely diagnosis?

Answer 29

Patent ductus arteriosus Patent ductus is associated with a continuous murmur whilst Pulmonary stenosis presents with a systolic murmur.

Question 30

Patent ductus is associated with a continuous murmur is associated with which condition?

Answer 30

Patent ductus is associated with a continuous murmur whilst

Question 31

Continuous murmur is associated with which condition?

Answer 31

Patent ductus arteriossis

Question 32

Pulmonary stenosis presents with which murmur?

Answer 32

Pulmonary stenosis presents with a **systolic murmur.**

Question 33

Which murmur are VSDs associated with?

Answer 33

VSDs are associated with a **pansystolic murmur**

Question 34

Pansystolic murmur is caused by which defect?

Answer 34

pansystolic murmur is associated with VSD

Question 35

ASDs are associated with which murmur?

Answer 35

**ASD's have a fixed split S2 sound** due to the increased venous return overloading the right ventricle during inspiration and delaying closure of the pulmonary valve.

Question 36

Charlie is a 7 month old baby boy who presents to you with poor weight gain (50th to 10th centile), on examination he has an erythematous, blanching rash over his abdomen, colicky abdominal pain and vomiting after feeds. He has been breast feeding with top-ups of 'Aptamil' formula. What is the most likely diagnosis? (3)

Answer 36

**The correct answer is cows' milk protein intolerance.** * Multi-system involvement * 7 months would suggest the new introduction of top up feeds which correlates with the symptoms * Faltering growth along with the multi-system involvement would suggest cows' milk protein intolerance

Question 37

What is the classical age for pyloric stenosis?

Answer 37

2 to 8 weeks very rare above 6 months

Question 38

What is the developmental cause of Hirschprung's disease? (3)

Answer 38

Craniocaudal migration of ganglion cells of the bowel begins at 12th wk of gestation Arrest of this migration produces an aganglionic segment of bowel-absence of Aurbach's & Meissener's plexus This aganglionic segment of bowel unable to relax & peristaltic wave stops proximally- functional obstruction

Question 39

You are called to the postnatal ward to review an 8 hour old baby born by elective caesarian section at 39 weeks gestation. After reading the case notes you discover the use of maternal labetalol for high blood pressure. On examination, the baby appears jittery and hypotonic. What is the most appropriate next step? What is the diagnosis? (4)

Answer 39

A jittery and hypotonic baby may suggest **neonatal** **hypoglycaemia**. **Neonatal abstinence syndrome** (also called NAS) is a group of conditions caused when a baby withdraws from certain drugs The use of **maternal labetalol is a risk factor** and these babies must have their blood glucose measured. Neonatal abstinence syndrome may also present in this way and so the use of maternal opiates or illicit drug use in pregnancy should also be ascertained.

Question 40

You are the paediatrician reviewing a newborn who was delivered at 39+2 weeks vaginally and without any complications. The child's father has a diagnosis of achondroplasia and the parents are wondering whether their child has this condition. Aside from measuring the child's length, what physical feature might suggest achondroplasia in this child? (4)

Answer 40

Trident hands - feature of achondroplasia short limbs (rhizomelia) lumbar lordosis midface hypoplasia.

Question 41

**Match the genetic condition to the phenotype** Low set ears Saddle-gap deformity Trident hand deformity Single palmar crease Microcephaly

Answer 41

**Low set ears** - Fragile X syndrome **Saddle-gap deformity** - Down's syndrome **Trident hand deformity** - achondroplasia **Single palmar crease** - Down's syndrome **Microcephaly** - alcohol and certain drugs, chemicals and infections in the womb **Macrocephaly** - achondroplasia

Question 42

A newborn infant is noted to have a posterior displacement of the tongue and a cleft palate. What is the most likely diagnosis?

Answer 42

A baby is noted to have micrognathia and a cleft palate. He is placed prone due to upper airway obstruction. There is no family history of similar problems - **Pierre-Robin syndrome**

Question 43

Patau syndrome features (5)

Answer 43

* Trisomy 13 * Microcephalic, small eyes * Cleft lip/palate * Polydactyly * Scalp lesions

Question 44

Edward's syndrome features | (5)

Answer 44

* (trisomy 18) * Micrognathia * Low-set ears * Rocker bottom feet * Overlapping of fingers

Question 45

Fragile X features | (4)

Answer 45

Learning difficulties Macrocephaly Long face Large ears Macro-orchidism

Question 46

Noonan syndrome features (4)

Answer 46

Webbed neck Pectus excavatum Short stature Pulmonary stenosis

Question 47

Pierre-Robin syndrome features (3)

Answer 47

* Micrognathia * Posterior displacement of the tongue (may result in upper airway obstruction) * Cleft palate

Question 48

Prader-Willi syndrome features (3)

Answer 48

Hypotonia Hypogonadism Obesity

Question 49

William's syndrome (5)

Answer 49

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

Question 50

Cri du chat syndrome features (6)

Answer 50

* (chromosome 5p deletion syndrome) * Characteristic cry (hence the name) due to larynx and neurological problems * Feeding difficulties and poor weight gain * Learning difficulties * Microcephaly and micrognathism * Hypertelorism

Question 51

Answer 51

Adrenal hyperplasia FSH and LH are both low in gonadotrophin independent precocious puberty

Question 52

Precocious puberty in males and testes size

Answer 52

## Footnote **bilateral enlargement** = gonadotrophin release from intracranial lesion **unilateral enlargement** = gonadal tumour **small** **testes** = adrenal cause (tumour or adrenal hyperplasia)

Question 53

You have just helped deliver a 2 week premature baby and are asked to do a quick assessment of the current APGAR score. The baby has a slow irregular cry, is pink all over, a slight grimace, with a heart rate of 140 BPM and moving both arms and legs freely. The current APGAR score is?

Answer 53

A - Pink all over no cyanosis - 2 points P - Pulse rate over 100 - 2 points G - Grimace - 1 point A - Activity flexed arms and legs - 2 points R - Respiration slow irregular cry - 1 point **8 points** * A score of 0-3 is very low score * between 4-6 is moderate low and between * 7 - 10 means the baby is in a good state

Question 54

An 8-year-old boy is reviewed in the Enuresis clinic. He is still wetting the bed at night despite using an enuresis alarm for the past three months. There are no problems with micturition during the daytime and he passes one soft stool everyday. Which treatment is most likely to be offered?

Answer 54

Desmopressin ## Footnote particularly if short-term control is needed (e.g. for sleepovers) or an enuresis alarm has been ineffective/is not acceptable to the family

Question 55

Risk factors for surfactant deficiency lung disease (SDLD) gestation-related (2) Other (4)

Answer 55

**The risk of SDLD decreases with gestation:** * 50% of infants born at 26-28 weeks * 25% of infants born at 30-31 weeks **Other risk factors for SDLD include:** * male sex * diabetic mothers * Caesarean section * second born of premature twins

Question 56

SDLD Management (3)

Answer 56

Management * oxygen * assisted ventilation * exogenous surfactant given via endotracheal tube

Question 57

An emergency call is put out as a 9-year-old boy has collapsed in the waiting room. The receptionists have already dialled 999. He does not respond to stimulation and no signs of respiration can be found after 10 seconds. There is no obvious foreign body in the mouth. What is the most appropriate next step? (3)

Answer 57

* **give 5 rescue breaths** * **check for signs of circulation** infants use brachial or femoral pulse, children use femoral pulse * **15 chest compressions:2 rescue breaths (see above)** 1. chest compressions should be 100-120/min for both infants and children 2. depth: depress the lower half of the sternum by at least one-third of the anterior–posterior dimension of the chest (which is approximately 4 cm for an infant and 5 cm for a child) 3. in children: compress the lower half of the sternum 4. in infants: use a two-thumb encircling technique for chest compression

Question 58

You are asked to attend an elective Caesarean section for macrosomia and maternal diabetes. At what times should you assess the APGAR scores?

Answer 58

APGAR scores are assessed at 1, 5 and 10 minutes of age

Question 59

A baby born at 35 weeks gestations via normal vaginal delivery is found to be irritable 48 hours after birth and suffers a convulsion. There is no obvious head trauma or swellings. Which of the cranial injuries is most likely to have occurred? (3)

Answer 59

An **intracranial haemorrhage** refers to subarachnoid, subdural or intraventricular haemorrhages. **Subarachnoid** **haemorrhages** are common and may cause irritability and even convulsions over the first 2 days of life. **Subdural** **haemorrhages** can occur following the use of forceps. **Intraventricular** **haemorrhage** mostly affects pre-term infants and can be diagnosed by ultrasound examinations.

Question 60

What is this

Answer 60

A cephalohaematoma may occur after a spontaneous vaginal delivery or following a trauma from the obstetric forceps or the ventouse. A haemorrhage results after the presidium is sheared from the parietal bone. The tense swelling is limited to the outline of the bone. It reduces over a few weeks - months.

Question 61

A baby is born at 32 weeks gestation and transferred to the neonatal unit. Over the next few hours, the baby exhibits nasal flaring, chest wall indrawing, and appears to be jaundiced. Observations are a heart rate of 72/min, a respiratory rate of 70/min, and a temperature of 38.1ºC. Which organism is most commonly responsible for the likely diagnosis? (2)

Answer 61

**Early-onset neonatal sepsis** in the UK is most commonly caused by **group B streptococcus** infection

Question 62

A 2-year-old boy is brought to the clinic by his mother who has noticed that he has developed a small mass. On examination; a small smooth cyst is identified which is located above the hyoid bone. On ultrasound the lesion appears to be a heterogenous and multiloculated mass. What is the most likely diagnosis? (3)

Answer 62

* Dermoid cysts are usually multiloculated and heterogeneous. * Most are located above the hyoid * their appearances on imaging differentiate them from thyroglossal cysts.

Question 63

Dermoid cyst features (3)

Answer 63

* Derived from pleuripotent stem cells and are located in the midline * Most commonly in a suprahyoid location * They have heterogeneous appearances on imaging and contain variable amounts of calcium and fat

Question 64

Thyroglossal cyst features (3)

Answer 64

* Located in the anterior triangle, usually in the midline and below the hyoid (65% cases) * Derived from remnants of the thyroglossal duct * Thin walled and anechoic on USS (echogenicity suggests infection of cyst)

Question 65

Features of a Branchial cyst (5)

Answer 65

* Six branchial arches separated by branchial clefts * Incomplete obliteration of the branchial apparatus may result in cysts, sinuses or fistulae * 75% of branchial cysts originate from the second branchial cleft * Usually located anterior to the sternocleidomastoid near the angle of the mandible * Unless infected the fluid of the cyst has a similar consistency to water and is anechoic on USS

Question 66

Infantile haemangioma features (4)

Answer 66

May present in either triangle of the neck Grow rapidly initially and then will often spontaneously regress Plain x-rays will show a mass lesion, usually containing calcified phleboliths As involution occurs the fat content of the lesions increases

Question 67

Lymphadenopathy of the neck features (3)

Answer 67

* Located in either triangle of the neck * May be reactive or neoplastic * Generalised lymphadenopathy usually secondary to infection in children (very common)

Question 68

How do the types of bipolar disorder differentiate themselves in their presentation?

Answer 68

type I disorder: mania and depression (most common) type II disorder: hypomania and depression

Question 69

How is transient tachypnoea managed? | (2)

Answer 69

## Footnote observation, supportive care supplementary oxygen may be required to maintain oxygen saturations

Question 70

4 most common causes of stridor in infants

Answer 70

croup acute epiglottitis laryngomalacia inhaled foreign body

Question 71

A 2-month-old baby presents with gradually worsening noisy breathing which is especially noticeable when she eats. She is on a lower centile for weight gain and has poor food intake. What is the most likely diagnosis? (2)

Answer 71

laryngomalacia "noisy breathing" = stridor

Question 72

A 7-month-old child is referred to clinic by her general practitioner because of asymmetrical hip creases. What is your suspected diagnosis? What is the first-line investigation in this case? What about if the infant was younger than 4.5 months?

Answer 72

DDH DDH in a child \>4.5 months: x-ray is the first-line investigation If younger than 4.5 months, ultrasound

Question 73

Management of DDH | (3)

Answer 73

**Management** ## Footnote most unstable hips will spontaneously stabilise by 3-6 weeks of age Pavlik harness (dynamic flexion-abduction orthosis) in children younger than 4-5 months older children may require surgery

Question 74

Answer 74

Meningitis in children \< 3 months: give IV amoxicillin in addition to cefotaxime to cover for Listeria

Question 75

You are working in the emergency department when you assess a 6-year-old boy with a broken arm. As part of your examination, you notice some lacerations on his back. On questioning, he doesn't want to talk about it but his mother quickly reassures you this is from him playing with his friends after all, 'boys will be boys'. You are suspicious. What do you do?

Answer 75

**Put the child's arm in a cast and admit them, then contact child protection** The GMC good medical practice contains guidelines on protecting children and young people. Within this, they state that 'you must be open-minded when considering the possible cause of an injury or other signs that may suggest that a child or young person is being abused or neglected. For example, as part of the differential diagnosis, you should consider whether an uncommon condition, including a genetic condition, might have caused or contributed to the child's or young persons injury or symptoms. You must also make sure that the clinical needs of children and young people continue to be met and are not overshadowed by child protection concerns.

Question 76

You are asked to review a neonate with a postnatal diagnosis of congenital diaphragmatic hernia. They are now stable, having had initial medical management. The baby's parents have done some background reading on the condition and have some questions for you. **The outcome or prognosis of a patient with CDH is largely dependent on which 2 factors**

Answer 76

The presence of the liver in the thoracic cavity is a poor prognostic factor for CDH **The outcome or prognosis of a patient with CDH is largely dependent on 2 factors** * Liver position * Lung-to-head ratio

Question 77

Where are congenital diaphragmatic hernias most likely to occur? (3)

Answer 77

CDH is significantly more common on the left hand side. * 85% of cases occur on the left * 13% on the right * 2% bilaterally.

Question 78

What is the prognosis of congential diaphragmatic herniation (1)

Answer 78

Only around 50% of newborns with CDH survive despite modern medical intervention.

Question 79

Congential diaphragmatic herniation of newborns features (3)

Answer 79

Congenital diaphragmatic hernia (CDH) occurs in around 1 in 2,000 newborns. It is characterised by the herniation of abdominal viscera into the chest cavity due to incomplete formation of the diaphragm. This can result in pulmonary hypoplasia and hypertension which causes respiratory distress shortly after birth.

Question 80

A 4-year-old boy is brought to the emergency department by his parents who are worried he is not acting his normal self and is very tired. There has been a recent outbreak of norovirus at his school, and for the past two days, he has been having repeated episodes of diarrhoea and vomiting and is not eating or drinking. On examination, he appears lethargic and has pale, mottled skin. His hands feel cold and when you assess his skin turgor it is reduced. Peripheral capillary refill time is 4 seconds. He weighs 17kg. **Observations**: * Pulse: 160bpm (90-140) * BP: 98/68 mmHg * Respiratory rate: 34/min (25-30) * Oxygen saturation: 96% on air * Temperature: 37.5ºC What is the most appropriate volume of 0.9% NaCl to give as a bolus?

Answer 80

Start IV fluid resuscitation in children or young people with a bolus of 20 ml/kg over less than 10 minutes

Question 81

How does treatment for dehydration in kids differ from that of adults?

Answer 81

Kids, give 20ml per kilogram over 10 minutes Adults, give 500ml over 10 minutes

Question 82

Features suggestive of hypernatraemic dehydration: (5)

Answer 82

* jittery movements * increased muscle tone * hyperreflexia * convulsions * drowsiness or coma

Question 83

The next parent arrives with her 2-year-old son. He was diagnosed with cow's milk protein allergy (CMPA) when he was 3 months old and got much better after it was first recognised. He has been on a dairy excluding diet and has been fed hydrolysed milk up until 1 year of age. He was tried on the milk ladder and successfully made it onto raw milk in the past month without further reaction or diarrhoea. His mother wonders whether this is normal, or if he was misdiagnosed when he was younger. IgE testing had been done and found to be normal. What would you advise the boy's mother?

Answer 83

Most children with non-IgE-mediated cow's milk protein allergy will be milk tolerant by 3 years of age

Question 84

At what age is raw cow's milk generally tolerated? Which immune molecule generally mediates this intolderence? (2)

Answer 84

Most children with **non-IgE-mediated** cow's milk protein allergy will be milk tolerant by 3 years of age Most children with **IgE-mediated** cow's milk protein allergy will be milk tolerant by 3 years of age

Question 85

How is cows milk protein (CMPA) generally managed? (6)

Answer 85

**Management if formula-fed** 1. extensive hydrolysed formula (eHF) milk is the first-line replacement formula for infants with mild-moderate symptoms 2. amino acid-based formula (AAF) in infants with severe CMPA or if no response to eHF 3. around 10% of infants are also intolerant to soya milk **Management if breastfed** 1. continue breastfeeding 2. eliminate cow's milk protein from maternal diet. Consider prescribing calcium supplements for breastfeeding mothers whose babies have, or are suspected to have, CMPI, to prevent deficiency whilst they exclude dairy from their diet 3. use eHF milk when breastfeeding stops, until 12 months of age and at least for 6 months

Question 86

A 7-month old infant is brought to the general practitioner by his mothers. They describe a history of a runny nose with a mild fever for the past week. Today, they thought he looked paler than usual, has been increasingly drowsy, and seemed to have difficulty breathing. On examination, he is of normal colour, there is nasal flaring and moderate intercostal recession. He only wakes after rubbing his chest for 5 seconds. His pulse rate is 140 beats per minute, respiratory rate is 40 breaths per minute, oxygen saturations 94% on room air, and temperature is 37.9 ºC. Which aspect of the presentation is most worrying as a sign of serious illness?

Answer 86

Moderate or severe **intercostal recession** is a red flag in paediatric patients with a fever

Question 87

A 6-week-old infant boy born at 37 weeks gestation by caesarian section is brought to his general practitioner for a routine physical examination. During the examination, the general practitioner notes that the neonate's urethral meatus is located on the ventral aspect of the penile shaft, rather than the distal glans penis. Name this abnormal finding, What other congenital defect is this neonate at an increased risk of also having?

Answer 87

hypospadias Cryptorchidism is present in around 10% of patients with hypospadias

Question 88

# Define cryptorchidism Which condition is it assocaited with?

Answer 88

known as **undescended testis**, approximately 10% of neonates with **hypospadias** also have cryptorchidism.

Question 89

# Define posterior urethral valve Which condition is it associated with?

Answer 89

Posterior urethral valves (PUV) are obstructive membranes that develop in the urethra (tube that drains urine from the bladder), close to the bladder. It is associated with increased susceptibility to urinalysis tract infections

Question 90

What are Varicoceles Which condition are they associated with?

Answer 90

Varicoceles are associated with venous hypertension in the **pampiniform venous plexus** and are not a typical congenital defect.

Question 91

A 2-day-old neonate is being treated for transient tachypnoea of the newborn. She is being monitored on the ward, and feeding is being encouraged when she is not tachypnoeic. She was born via emergency cesarean section at 34 weeks, following maternal complications in pre-term labour. Her mother has severe asthma and during pregnancy, developed gestational diabetes. **What is the greatest risk factor for developing this condition?**

Answer 91

Delivery by caesarean section is a risk factor for transient tachypnoea of the newborn

Question 92

Which pulses are checked during the assessment of an infant's heart rate?

Answer 92

femoral and carotid

Question 93

A 6-month-old baby presents with feeding difficulties associated with a cough and wheeze and is diagnosed with bronchiolitis. What are the precipitating factor for a more severe episode of bronchiolitis and not just an increased risk of developing bronchiolitis? What is the causative agent?

Answer 93

more serious if bronchopulmonary dysplasia (e.g. Premature) congenital heart disease or cystic fibrosis respiratory syncytial virus (RSV) is the pathogen in 75-80% of cases

Question 94

Answer 94

Biliary atresia typically presents in the first few weeks of life with jaundice, appetite and growth disturbance Bile duct stenosis would present in a similar way but is less common.

Question 95

What causes biliary atresia?

Answer 95

A childhood disease of the liver in which one or more bile ducts are abnormally narrow, blocked, or absent. It can be congenital or acquired.

Question 96

A 2-hour-old baby is reviewed for increased work of breathing. He was born 2 hours ago via elective caesarean section at 39 weeks gestation. The pregnancy was uncomplicated. Currently, he has a respiratory rate of 75 breaths per minute (normal: 40-60) and an oxygen saturation of 95% (normal: \>93%). There is nasal flaring visible on examination. A chest x-ray shows hyperinflated lung fields and a line of fluid in the horizontal fissure of his right lung. What is the most likely diagnosis? What is the most appropriate management?

Answer 96

**transient tachypnoea of the newborn** Observation and supportive care +/- oxygen are the mainstays of treatment in uncomplicated transient tachypnoea of the newborn

Question 97

A 25-year-old man is counselled regarding the genetics of Huntington's disease Describes the concept of anticipation

Answer 97

**Anticipation in trinucleotide repeat disorders** = earlier onset in successive generations

Question 98

A 15-year-old known type 1 diabetic comes in with vomiting, abdominal pain, and drowsiness. On examination, you notice her breath smells of pear drops. She tells you that she can't be admitted right now as she is missing a friend's birthday party. After explaining the risks of leaving she seems to understand, can repeat what you have said, and explain her decision. Her parents say that she should stay for treatment. What should you do?

Answer 98

**Admit the patient for treatment, seeking legal advice if she continues to refuse treatment** This individual is under the age of 16 and is deemed to have capacity. Therefore, they have the right to agree to a treatment without a parent's consent, but if they choose to refuse it, you must weigh up their best interests. As the patient has diabetic ketoacidosis, the decision to leave would most likely be fatal and so it would be fair to make the decision that it is in her best interests.

Question 99

A man brings his 18-month-old daughter to your GP clinic. She has had coryzal symptoms for the last 2 days. Last night, she started with a barking cough and a mild temperature of 37.8º. On examination, there is mild stridor when mobilising, with no recessions visible. Chest sounds clear with good air entry bilaterally. The temperature today remains at 37.8º, but all other observations are normal. What is the most appropriate management?

Answer 99

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

Question 100

How is mild croup managed?

Answer 100

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

Question 101

A doctor identifies that a neonate was found to have a left subclavicular thrill, a heaving apex beat, and a continuous 'machinery like' murmur. Regarding this defect, what is the correct management?

Answer 101

Patent ductus arteriosus: indomethacin is given to the neonate in the postnatal period, not to the mother in the antenatal period

Question 102

What would the findings be of a cardiac examination of an infant with PDA? (3)

Answer 102

left subclavicular thrill a heaving apex beat continuous 'machinery like' murmur

Question 103

A 9-month-old boy has been referred to you by the out of hours GP. His parents give a 24-hour history of increased work of breathing, coryzal symptoms, lethargy and reduced oral intake. On examination, you note fine inspiratory crackles and subcostal recessions. He is pyrexial (37.9ºC) and oxygen saturations are 91% in air. What is the next most appropriate course of action? (5)

Answer 103

This child has **bronchiolitis**. A lower respiratory tract infection commonly caused by respiratory syncytial virus. The disease is usually self-limiting, but it is important to note that some children deteriorate in the first 72 hours of the illness. NICE recommend admission if any of the following criteria are present: * Apnoea (observed or reported) * Persistent oxygen saturation of \<92% in air * Inadequate oral fluid intake (\<50% of normal fluid intake) * Persisting severe respiratory distress, for example grunting, marked chest recession, or a respiratory rate of over 70 breaths/minute

Question 104

Overview of PDA | (4)

Answer 104

generally classed as 'acyanotic'. However, uncorrected can eventually result in late cyanosis in the lower extremities, termed differential cyanosis ## Footnote connection between the pulmonary trunk and descending aorta usually, the ductus arteriosus closes with the first breaths due to increased pulmonary flow which enhances prostaglandins clearance more common in premature babies, born at high altitude or maternal rubella infection in the first trimester

Question 105

PDA risk factors (3)

Answer 105

more common in premature babies, born at high altitude maternal rubella infection in the first trimester

Question 106

What is the most likely causative agent for bacterial pneumonia in children?

Answer 106

**Streptococcus pneumoniae** is the most likely causative agent of a bacterial pneumonia in children

Question 107

Treatment of S. pneumoniae in children | (4)

Answer 107

## Footnote Amoxicillin is first-line for all children with pneumonia Macrolides may be added if there is no response to first line therapy Macrolides should be used if mycoplasma or chlamydia is suspected In pneumonia associated with influenza, co-amoxiclav is recommended

Question 108

A 16-year-old male presents to his general practitioner with cough, rhinorrhoea, sore throat, fever and a rash. He has no significant past medical history and is on no medications. He has recently arrived from Romania and is a member of the Roma community. His observations are heart rate 94 beats per minute, blood pressure 120/80 mmHg, respiratory rate 18/minute, oxygen saturations 97% on room air and temperature 39.2ºC. On examination, there is a maculopapular rash affecting his face. His eyes have a serous discharge and small white lesions are noted on his buccal mucosa. There is no tonsillar exudate. There is no evidence of meningism. The cardio-respiratory and abdominal examinations are unremarkable. What is the likely diagnosis?

Answer 108

Measles is characterised by prodromal symptoms, Koplik spots. maculopapular rash starting behind the ears and conjunctivitis

Question 109

Investigations for mumps | (1)

Answer 109

Investigations IgM antibodies can be detected within a few days of rash onset

Question 110

What is the infective agent in scarlet fever?

Answer 110

The rash of scarlet fever is caused by a streptococcal infection

Question 111

A 5-year-old boy is brought to the surgery with chickenpox. His mother wants advice regarding school exclusion. What is the most appropriate advice to give?

Answer 111

Chickenpox school exclusion - until all the lesions are dry and have crusted over (usually about 5 days after the onset of the rash)