Questions Flashcards

1
Q

A 4-year-old child presents with a high fever, difficulty breathing, and a muffled “hot potato” voice. The child is sitting upright, drooling, and appears anxious, with inspiratory stridor noted on examination.

Upon examination he has a throat that looks like this.

What is the dx and tx? [2]

A

Inflamed epiglottis, suggestive of acute epiglottitis

Management:
- The priority of acute epiglottitis treatment is airway management by an experienced clinician trained in paediatric airways
- Abx
- IV steroids and adrenaline nebulisers
- IV fluids
- Hib Vaccination

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2
Q
A
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3
Q

Haemophilia A is an [] disease. This means that all female offspring of affected men will be [] and have a []% chance of these females passing the gene on and []% chance of these males passing the gene on

A

Haemophilia A is an X-linked recessive disease. This means that all female offspring of affected men will be carriers
50% chance of these females passing the gene on
0% chance of males passing it on .

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4
Q
A
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5
Q

An oral rotavirus vaccine has recently been introduced into the NHS immunisation schedule. When should it be given?

2 months + 4 months
2 months + 3 months
3 months + 12-13 months
3 + 4 months
4 months + 12-13 months

A

2 months + 3 months

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6
Q

[2] are commonly associated with malrotation.

A

Exomphalos and diaphragmatic herniae are commonly associated with malrotation.

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7
Q

At what age would the average child acquire the ability to sit without support? [1]

A

6-8months

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8
Q

Which of the following is most likely to be ANA+ve?

Systemic JIA
Polyarticular JIA (RF-negative and RF-positive)
Oligoarticular JIA
Enthesitis-Related Arthritis (ERA)
Psoriatic JIA

A

Oligoarticular JIA

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9
Q

Describe a skin manifestation of Turner’s syndrome [1]

A

Melanocytic naevi

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10
Q

Which of the following conditions also exhibit genetic anticipation?

Marfan syndrome
Duchenne muscular dystrophy
Myotonic dystrophy
Homocystinuria
Trisomy 21

A

Myotonic dystrophy

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11
Q

How do you differentiate between the presentation of Hirschprungs and a meconium ileus via XR? [2]

A

Hirschprungs:
- The delayed passage of meconium together with distension of abdomen is the usual clinical presentation
- A plain abdominal x ray will demonstrate dilated loops of bowel with fluid levels and a barium enema can be helpful when it demonstrates a cone with dilated ganglionic proximal colon and the distal aganglionic bowel failing to distend

Meconium ileus:
- his condition presents during the first days of life with gross abdominal distension and bilious vomiting.
- x Ray of the abdomen shows distended coils of bowel and typical mottled ground glass appearance. Fluid levels are scarce as the meconium is viscid.

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12
Q

[] is the most common cause of primary headache in children

A

Migraine is the most common cause of primary headache in children

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13
Q

Describe what a venous hum sounds like [2]

A

Venous hum is a benign murmur heard in children and sounds like a continuous blowing noise heard below the clavicles

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14
Q

You diagnose her with hand, foot and mouth disease. The mother asks if she needs to stay off school. What advice will you give her regarding school exclusion?

Stay off school for 48 hours from onset of symptoms
No need to stay off school if she feels well
Stay off school for 48 hours from onset of rash
Stay off school until rash has subsided
Stay off school until all symptoms have subsided

A

No need to stay off school if she feels well

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15
Q

What is a good way of remembering school exclusions for:
- Chickenpox
- D&V
- Mumps
- Rubella
- Measles
- Whooping cough

A

This helps me remember things kinda so hope this helps you too:
- Chicken pox –> Off school til lesions have crusted (typically ~5 days)

  • Diarrhoea and Vomiting –> 2 things so can only come back to school 2 days symptom free
  • Mumps –> 5 letters in mumps so 5 days after swelling onset (If part of MMR so now can remember these are all ~ around 5 days after a sx onset)
  • Rubella –> Part of MMR so can remember 5 days after rash onset
  • Measles –> Part of MMR so can remember ~5 days after rash onset (it’s actually specifically 4 lmao)
  • Whooping cough (Pertussis) –> ‘whoop whoop, sound of the alarm’, 2 whoops so can return to school 2 days after antibiotics
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16
Q

How do you tx PDA depending on when baby is born / timing of tx? [2]

A

In term infants with moderate to large PDAs, transcatheter closure is the treatment of choice according to current UK guidelines.

While prostaglandin inhibitors like ibuprofen are effective for PDA closure in premature infants, they have very limited efficacy in term infants, especially by 3 months of age.

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17
Q

Upper and lower pole renal scarring associated with cortical thinning → ?[]

A

Upper and lower pole renal scarring associated with cortical thinning → ?reflux nephropathy

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18
Q

Which virus causes croup? [1]

A

crouP - Parainfluenza - saw this as a comment on another question, helped me remember since

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19
Q
A

This image demonstrates grade V vesicoureteric reflux - gross dilatation of the ureter, pelvis and calyces with ureteral tortuosity. A DMSA scan is needed to identify renal scarring.

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20
Q

Describe how you tx Perthes depending on age [2]

A

If less than 6 years = observation
Older = surgical management with moderate results

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21
Q

Asides from suppresion of appetite, what are common side effects of methlyphenidate? [2]

A

insomnia, suppression of appetite and depression.

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22
Q

A 13-year-old female attends the General Practitioner (GP) with her mother. Her mother is concerned as she has noticed scratch marks on her daughter’s arms, and after a long chat her daughter has admitted to cutting herself repeatedly. The GP agrees to refer the girl to CAMHS (Child and Adolescent Mental Health Services).

Which of the following is the most suitable intervention to reduce the risk of recurrence of self-harm?

Citalopram
Cognitive behavioural therapy
Counselling
Family therapy
Sertraline

A

Cognitive behavioural therapy

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23
Q
A
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24
Q

A 33-year-old mother had a natural birth at home. Seven days later, she brought her newborn to the Emergency Department, complaining of a musty odour of the skin and urine. Examination reveals hypopigmentation and eczema. Genetic testing revealed an autosomal recessive genetic disorder.

Which of the following is most likely linked to the condition of the newborn?

Defect in the transporter for cysteine
Decreased α-ketoacid dehydrogenase
Defect in phenylalanine hydroxylase
Deficiency in homogentisic acid oxidase
Defect in tyrosinase

A

A 33-year-old mother had a natural birth at home. Seven days later, she brought her newborn to the Emergency Department, complaining of a musty odour of the skin and urine. Examination reveals hypopigmentation and eczema. Genetic testing revealed an autosomal recessive genetic disorder.

Which of the following is most likely linked to the condition of the newborn?

Defect in phenylalanine hydroxylase
- This is a typical presentation of phenylketonuria. An excess of phenylalanine leads to an excess of phenylketones in the urine. A musty odour of the skin and hair is due to phenylacetate (a phenylketone) accumulation.

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25
Q

A two-year-old child is admitted to the paediatric Emergency Department with malaise, rash, vomiting and fever. Her mother reports that she has been off her food for 24 hours and has been running a low-grade temperature. She also points to a partial thickness burn over her arm that has been de-roofed and treated with dressings by her General Practitioner.

What is the most likely diagnosis? [1]

A

An unwell child with an unhealed burn must be treated for toxic shock syndrome until proven otherwise. Treatment will require management in the intensive care department with input from a paediatric consultant and the plastic surgery team. Circulatory support with cryoprecipitate may be necessary.

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26
Q

A 12-year-old child developed headaches, vomiting and a staggering gait. A cerebellar neoplasm was diagnosed.

Which is the most common cerebellar neoplasm of childhood?

Ependymoma
Glioblastoma multiforme
Neuroblastoma
Astrocytoma
Oligodendroglioma

A

Astrocytoma

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27
Q

This child has an atypical UTI, given that it was proven to be secondary to Enterococcus infection (non-E. coli) and took 72 hours to respond to the antibiotic treatment. They are 5 months old.

Which investigations (and when) should be initiated?

A

Urgent USS during the acute infection with routine DMSA and MCUG
- in children younger than 6 months of age with an atypical UTI, an urgent USS is required during the acute infection, looking for any structural abnormalities in the urinary tract. Once the acute infection has resolved, 4–6 months later, a routine DMSA is performed (looking for a scar in the kidney, renal agenesis or a duplex system) and an MCUG (looking for vesico-ureteric reflux or a posterior urethral valve).

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28
Q

A concerned mother visits her General Practitioner to discuss her six-week-old baby. She is concerned that he is not feeding well, his urine has a musty smell, and he has very dry skin all over his trunk, which does not respond to regular emollients

What is the most likely dx? [1]

Describe the basic pathophysiology [2]

A

Phenylketonuria (PKU) is an autosomal recessive condition which affects amino acid metabolism. There is deficiency of the enzyme phenylalanine hydroxylase. If not treated with a specific diet, those affected may have behavioural problems, seizures or learning disability. Patients may also present with eczema that does not respond to standard treatment, and parents may notice a musty smell in the child’s urine or breath. This condition is screened for with the newborn heel prick test, but further investigation may be indicated if symptoms or family history support a diagnosis.

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29
Q

A 7-year-old child is seen in paediatrics for episodes of making incomprehensible noises while asleep, occurring twice in the last three months and resolving spontaneously. He also experienced slurred speech, left-sided facial twitching, and lip-tingling one morning. He has no notable medical history. His older sister had febrile convulsions as a child.

What is the most likely diagnosis?

Benign rolandic epilepsy
Juvenile myoclonic epilepsy
Nightmares
Night terrors
Pseudo-seizures

A

A 7-year-old child is seen in paediatrics for episodes of making incomprehensible noises while asleep, occurring twice in the last three months and resolving spontaneously. He also experienced slurred speech, left-sided facial twitching, and lip-tingling one morning. He has no notable medical history. His older sister had febrile convulsions as a child.

What is the most likely diagnosis?

Benign rolandic epilepsy
Juvenile myoclonic epilepsy
Nightmares
Night terrors
Pseudo-seizures

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30
Q

[] is the most appropriate initial investigation in a child with delayed puberty.

Why? [1]

A

Bone age assessment is the most appropriate initial investigation in a child with delayed puberty to determine if their skeletal maturation is consistent with their chronological age. A delayed bone age compared to chronological age can suggest a constitutional delay in growth and puberty, which is the most common cause of delayed puberty. It can also help identify any endocrinological issues affecting growth.

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31
Q

Describe the advice should give for impetigo treatment and school exclusion [2]

A

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

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32
Q

At their 1st birthday party…
* gross motor: [1]
* fine motor: [2]
* social: [1]
* speech and language: [2]

A

At their 1st birthday party…
* gross motor: walk/cruise
* fine motor: pincer grip their cake and bangs their new toys together
* social: stranger anxiety (meeting family for 1st time)
* speech and language: knows and responds to their own name + can say muma/dada

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33
Q

You are doing the six week check on a baby girl. Which one of the following best describes the Barlow test for developmental dysplasia of the hip?

Attempts to relocate a dislocated femoral head
Upward pressure on the femur with the hip flexed at 90 degrees
Observation of the relative height of the knees with the hips flexed at 90 degrees
Observation for buttock crease asymmetry with the hips flexed at 90 degrees
Attempts to dislocate an articulated femoral head

A

Attempts to dislocate an articulated femoral head

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34
Q

Infantile colic normally improves around [] months of age and resolves around [] months of age

A

Infantile colic normally improves around 3-4 months of age and resolves around 6 months of age

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35
Q

The Men B vaccine is given at [3] months.

A

The Men B vaccine is given at 2, 4 and 12-13 months.

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36
Q

Neonate; 39 weeks

Chest X-ray: hyperinflation and a thin fluid line in the right horizontal fissure.

Given the likely diagnosis, what is the most significant risk factor? [1]

A

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

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37
Q

the neonatal team decide on the most appropriate step in management as they are concerned about hypoxic brain injury.

Which intervention is most important for this baby?

A sodium bicarbonate correction
Blood transfusion
Skin to skin contact with mum
Therapeutic cooling
Vitamin K administration

A

Therapeutic cooling at 33-35 degrees attempts to reduce the chances of severe brain damage in neonates with hypoxic injury

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38
Q

A 6-year-old boy is noted to have pectus excavatum and pulmonary stenosis during a cardiorespiratory exam. What is the most likely diagnosis?

Noonan syndrome
Pierre-Robin syndrome
Edward’s syndrome
William’s syndrome
Patau syndrome

A

A young boy is noted to have a webbed neck, pulmonary stenosis, ptosis and short stature. The karyotype is normal - Noonan syndrome

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39
Q

Unilateral undescended testicle - review at [] months - if persistent refer

What is the management for bilateral? [1]

A

Unilateral undescended testis
* referral should be considered from around 3 months of age, with the baby ideally seeing a urological surgeon before 6 months of age

Bilateral undescended testes
* Should be reviewed by a senior paediatrician within 24hours as the child may need urgent endocrine or genetic investigation

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40
Q

His temperature is 39.5ºC and he has erythema, warmth, tenderness, and blistering on his right leg. Light palpation causes severe pain and there is crepitus beneath the skin when examining the surrounding tissues.

Which option is the most likely causative organism? [1]

A

Chickenpox is a risk factor for invasive group A streptococcal soft tissue infections including necrotizing fasciitis
- steptococcus pyogenes

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41
Q
A
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42
Q

At what age would the average child start to smile?

Birth
2 weeks
6 weeks
3 months
4 months

A

6 weeks

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43
Q

You review a 7-year-old girl who has cerebral palsy. She is having ongoing problems with spasticity in her legs which is causing pain and contractures. On speaking to her mother you ascertain that she is having regular physiotherapy, using the appropriate orthoses and has tried oral diazepam in the past. Which one of the following treatments may she be offered to try and improve her symptoms?

Baclofen
Clozapine
Clonidine
Dantrolene
Glyceryl trinitrate

A

The correct answer is Baclofen. Baclofen is a muscle relaxant and antispastic agent, which works by inhibiting the release of excitatory neurotransmitters in the spinal cord. It is commonly used to manage spasticity in patients with cerebral palsy, particularly when it causes pain or functional impairment. Baclofen can be taken orally or administered intrathecally for more severe cases.

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44
Q

Hypospadias surgery is typically performed at around [] months of age

A

Hypospadias surgery is typically performed at around 12 months of age

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45
Q

Congenital rubella presents with which two key characteristics? [2]

A

Congenital rubella
* sensorineural deafness
* congenital cataracts

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46
Q

Ambiguous genetalia - [] is most common cause in newborns

A

Ambiguous genetalia - congenital adrenal hyperplasia is most common cause in newborns

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47
Q

A [abx] should be used for children with pneumonia if mycoplasma is suspected

A

A macrolide e.g. erythromicin should be used for children with pneumonia if mycoplasma is suspected
- MycoPLASMAs lack a cell wall. Penicillins are b-lactam antibiotics so inhibit cell wall synthesis, therefore would be ineffective.

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48
Q

On examination you note a smooth midline lesion which is round and located just below the hyoid bone. It measures 2.5 cm x 2 cm and rises on protrusion of the tongue

What is the dx? [1]

A

rises on protrusion of the tongue = thyroglossal cysts

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49
Q

Bow legs in a child < [] is a normal variant and usually resolves by the age of [] years

A

Bow legs in a child < 3 is a normal variant and usually resolves by the age of 4 years

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50
Q

If a newborn baby has an abnormal hearing test at birth they are offered the []
- What does a positive result mean? [1]

A

If a newborn baby has an abnormal hearing test at birth they are offered the auditory brainstem response test
- The procedure entails placing a small, soft-tipped earpiece in the baby’s ear to emit gentle clicking sounds. An audible soft echo suggests a functioning cochlea. However, in this instance, the absence of an echo on two occasions raises concerns about potential hearing impairment.

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51
Q

On examination, she is noted to have microcephaly, moderate hepatosplenomegaly and a petechial rash. Shortly after admission to the neonatal intensive care unit, she has a seizure.

What is the likely infective agent that caused this presentation? [1]

A

Congenital CMV manifests with hearing loss, low birth weight, petechial rash, microcephaly and seizures

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52
Q

A respiratory rate of >[] per minute (at any age) is a red flag according to the NICE paediatric traffic light system

A

A respiratory rate of >60 per minute (at any age) is a red flag according to the NICE paediatric traffic light system

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53
Q
A
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54
Q

At what age should a child be able to pincer grip? [1]

What other fine motor activity should they be able to do at this age? [1]

A

12 months:
- pincer grip
- Bang toys together

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55
Q

At 3 months, which fine motor activites should a child be able to achieve? [4]

A
  • Reaches for object
  • Holds rattle briefly if given to hand
  • Visually alert, particularly human faces
  • Fixes and follows to 180 degrees
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56
Q

At what age should a child be able to hold in palmar grasp? [1]

A

6 months

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57
Q

At what age should a child be able to pass objects from one hand to another? [1]

A

6 months

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58
Q

At what age should a child be able to look around in every direction? [1]

q

A

6 months

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59
Q

What fine motor activities are expected of a 6 month old? [3]

A

Follow every direction
Pass objects from one hand to another
Holds in palmar grasp

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60
Q

What fine motor activities are expected of a 9 month old? [2]

A

Point with finger
Early pincer

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61
Q

Of the following, which is more common in child physical abuse?

Humeral fracture
Scaphoid fracture
Tibial fracture
Pelvic fracture
Ankle fracture

A

Humeral fracture

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62
Q

The most common fractures associated with child abuse are [3]

A

The most common fractures associated with child abuse are:
- Radial
- Humeral
- Femoral

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63
Q

What advise do you give to parents of CF about interacting with other kids with the same conditon? [1]

Why? [1]

A

patients with CF should try to minimise contact with each other to prevent cross infection with Burkholderia cepacia complex and Pseudomonas aeruginosa

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64
Q

patients with CF should try to minimise contact with each other to prevent cross infection with [2]

A

patients with CF should try to minimise contact with each other to prevent cross infection with Burkholderia cepacia complex and Pseudomonas aeruginosa

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65
Q

A 7-year-old girl presents to the emergency department accompanied by her mother. While trying to wake her up this morning, her mother noticed her face twitching and mouth drooling. This episode lasted for 30 seconds and the girl was fully aware of what was happening. The girl was drowsy and confused for the subsequent 15 minutes.

She has been well in herself and has no conditions. The pregnancy and vaginal birth have been uncomplicated. Her mother is worried that she has been tired as she has been going to bed later than usual for the past couple of weeks.

What is the most likely diagnosis? [1]

A

Benign rolandic epilepsy is characterised by partial seizures at night

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66
Q

Partial seizures at night time in childhood = ? [1]

A

Benign rolandic epilepsy

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67
Q

Describe the features of Benign rolandic epilepsy [3]

A

Seizures that occur at night / during sleep:
- Hemifacial parasthesias
- Oropharyngeal manifestations (strange noises)
- Hypersalivation

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68
Q

State three cardiac complications of 45XO [3]

A

Bicuspid aortic valve
CoA
Aortic dissection

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69
Q

Mother has bipolar that is treated during pregnancy

What cardiac complication might be seen in newborn? [1]

A

Ebsteins abnormality

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70
Q

What would indicate an admission of croup? [1]

A

Audible stridor at rest

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71
Q

How do you adapt the position of a child when giving rescue breathes depending on the age of the child? [1]

A

use neutral position for rescue breath for infant, if over 1 use head tilt and chin lift

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72
Q

Wh

What is the most common complication of roseola infantum? [1]

A

Febrile convulsions

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73
Q

What are three key features of Fragile X syndrome? [3]

A

Fragile X
- XL Head
- XL Balls
- XL ears

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74
Q

What would indicate a baby to have an US of hips at 6 weeks? [2]

A

Born in breech position at 36+ weeks
Required external cephalic version

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75
Q

What is this? [1]

A

Epstein pearl
- congenital cyst found in the mouth
- commonly on hard palate
- can be mistaken for erupting tooth if found on gum
- will spontaneously resolve

Pearls relate to palate

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76
Q

What is this? [1]

A

Bohns nodule
- found in inner labial aspect of maxillary alveolar ridges

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77
Q

Describe key features of Pierre-Robin syndrome [3]

A

‘Robin’ me of my airway’
- Small mandible & Tongue retraction –> airway obstruction

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78
Q

Which investigation is used to confirm a dx of pertussis? [1]

A

Per nasal swab

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79
Q

At a NIPE a boy is detected as having an undescended teste.

What is the management plan for this baby? [3]

How would this mx plan change if the baby had bilateral undescended testes? [1] Why? [1]

A

Review at 3 months
- If still has undescended teste refer to urology due to risk of torsion or hernia

NB: if bilateral undescended testes - referral to senior paediatrician within 24hrs as child may need endocrine or genetic investigation

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80
Q

A 10-year-old boy is found to have haemophilia A following investigation for a haemoarthrosis. Which one of his relatives is most likely to have the condition?

Father
Mother’s brother
Father’s sister
Mother
Father’s brother

A

The correct answer is Mother’s brother (maternal uncle). Haemophilia A is an X-linked recessive condition

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81
Q

How do you manage neonatal hypoglycaemia depending on whether the child is symptomatic or not? [2]

A

Asymptomatic:
- encourage normal feeding
- monitor blood glucose

Symptomatic:
* admit to the neonatal unit
* intravenous infusion of 10% dextrose

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82
Q

Sign? [1]
Dx? [1]

A

Bowel loops can be seen in the left side of the thoracic cavity
= Congenital diaphragmatic hernia

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83
Q

Which FBC finding (alongside the clinical picture) would indicate Kawasaki’s disease? [1]

A

Raised platelets

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84
Q

What is a mneumonic for remembering red flag symptoms for paeds? [5]

A

Really Sick Babies Get Antibiotics
1. Recessions (moderate or severe chest wall recessions)
2. Skin turgor reduced
3. Blue or mottled appearance
4. Grunting
5. Asleep (does not wake if aroused)

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85
Q

A baby is detected as having exomphalos antenatally.

How would you manage this situation? [2]

A

Caesarian section and staged repair
- caesarian reduces the risk of sac rupture, and surgery is non-urgent.

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86
Q

Under which situations would you offer a baby a 6 week US for DDH? [1]

A

All breech babies at or after 36 weeks gestation require USS for DDH screening at 6 weeks regardless of mode of delivery

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87
Q

What is the triad that makes up shaken baby syndrome? [3]

A

Shaken Baby Syndrome triad: ERS

Encephalopathy
Retinal haemorrhages (bilateral)
Subdural Haematoma

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88
Q

Chickenpox is a risk factor for invasive [] infections including necrotizing fasciitis

A

Chickenpox is a risk factor for invasive group A streptococcal soft tissue infections including necrotizing fasciitis

GAS - strep pyogenes

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89
Q

Lecture

What fluid bolus do you give paeds. patients in shock? [1]

A

Give fluid bolus – 10ml/kg 0.9% Sodium Chloride

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90
Q

Lecture

What GCS would you consider intubation in a child? [1]

What treatment would you give for a child with blood sugar < 3mmol/L? [2]

A

If GCS < 8 (P or less on AVPU scale) – consider intubation
* 2ml/kg 10% Dextrose if blood sugar < 3 mmol/L

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91
Q

Lecture

What are respiratory [6] and cardiac [6] causes of children arrest?

A
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92
Q

Describe the treatment plan for tet spells [4]

A
  • Initial measures include positioning the infant on their back and flexing their knees.
  • Oxygen should be administered in hospitals.
  • Prophylactic use of propranolol is practised in some centres.
  • Severe ‘Tet spells’ (exacerbation of Tetralogy of Fallot symptoms during periods of distress) can be managed with a vaso-constrictive agent e.g. phenylephrine, as a ‘last line’ medical therapy.
    *
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93
Q

How do you differentiate between a reflex anoxic seizure and a vasovagal episode?

A

**Reflex anoxic seizures **are paroxysmal self limited periods of asystole that are triggered by pain, fear or anxiety
- During these episodes the child becomes extremely pale, there may also be clonic movements, upward eye deviation, urinary incontinence and rigidity

Vasovagal Syncope
- history of feeling light headed and sweaty is classically seen during vasovagal episodes
- brief convulsions may occur if there is hypoperfusion to the brain due to a sudden drop in blood pressure.

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94
Q

A 3 month old boy, corrected for gestational age, born in May, with trisomy 21 has a ventricular septal defect. He is currently on a high-calorie nastrogastric feed and furosemide.
What prophylactic therapy is indicated for this patient?

Penicillin

Spironolactone

Palivizumab

Co-trimoxazole

Aspirin

A

Palivizumab
- This is a premature boy under the age of six months (corrected) approaching bronchiolitis season. He has significant acyanotic heart disease, requiring furosemide to prevent from him being symptomatic. He meets the indications for RSV prophylaxis. Palivizumab is a monoclonal antibody that minimises the risk of infection by RSV. It is given subcutaneously once a month during bronchiolitis season

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95
Q

Which of the following complications is most commonly associated with sickle cell disease in children?

Acute chest syndrome
1

Vaso-occlusive crisis
2

Stroke
3

Aplastic crisis
4

Priapism
5

A

Vaso-occlusive crisis

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96
Q

What heart sounds would indicate TGA? [1]

A

heart sounds are normal apart from a loud S2.

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97
Q

An 11 year old girl is brought to the GP because her mother is ‘beyond despair with her’.
The mother reports that her daughter is consistently hostile and angry towards her. She always does the opposite of what she asks her to do, shouts at her mother if she tries to get her to do anything, and insults her mother at every opportunity. Otherwise, the girl is fit and well. She has not yet reached puberty. She is getting on well at school, although occasionally her report card mentions that she talks back to teachers. She has lots of friends and gets on well with her younger brother.

What is the most likely diagnosis?

A

Oppositional defiant disorder

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98
Q

[], is suspected. This is confirmed with an oral contrast study.

A

Malrotation with small bowel obstruction, is suspected. This is confirmed with an oral contrast study.

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99
Q

Describe the difference in conduct and oppositional defiant disorder [2]

A

Conduct disorder is characterised by repetitive and persistent behavioral patterns that** violate the rights of others and social norms**, and aggressive behavior towards people and animals. Substance use and delinquency are common associated features of the condition

Oppositional defiant disorder is characterised by a pattern of negative, hostile, and defiant behavior towards authority figures without violating the rights of others. Children with ODD may argue, refuse to comply with requests, and deliberately annoy others. Substance use and delinquency are less common associated features of ODD.

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100
Q

Describe the UTI Mx in children [3+]:
- Under 6 months
- 6 months - 3 years
- 3+ yrs

A

Under 6 months
- all children get an ultrasound within 6 weeks.
- If it is atypical UTI or recurrent you do ultrasound during infection.
- Atypical and recurrent UTIs also receive DMSA scan 4-6 months after for renal scarring as well as MCUG.

6months - 3 yrs
- children with atypical UTI have ultrasound during infection.
- Children with recurrent UTI have ultrasound 6 weeks after infection.
- Both children with atypical or recurrent receive DMSA 4-6 months after.
- Dont typically offer MCUG unless suspicious.

3yrs+
- atypicals have ultrasound during infection.
- Recurrent have ultrasound at 6 weeks.
- Recurrent also have DMSA 4-6 months after. Dont typically offer MCUG.

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101
Q

Malrotation with small bowel obstruction, is suspected. This is confirmed with an oral contrast study.

What is the definitive management of this condition? [1]

A

Ladd’s operation
- This is performed, either open or laparoscopically, by untwisting the midgut volvulus, fixing the malrotated bowel in the correct location, removing Ladd’s bands (congenital adhesions) and performing an appendicectomy

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102
Q

The diagnosis is Developmental Dysplasia of the Hip with a subsequent leg length discrepancy.

[] is used to indicate whether the shortening is femoral or tibial

A

Galeazzi’s test is used to indicate whether the shortening is femoral or tibial

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103
Q

A child has ?TLS due to their chemotherapy for ALL.

Which electrolyte changes would you suspect to see? [4]

A

PUKE Calcium

Phosphate
Uric acid
K
Elevated

Low Calcium (binds to the free phosphates)

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104
Q

A 10 year old boy with a diagnosis of attention-deficit hyperactivity disorder (ADHD) attends the outpatient psychiatry clinic for review. He was recently started on Methylphenidate to control his symptoms. His behaviour has improved, however, he has developed facial tics which he finds distressing.
Which of the following is the most appropriate drug to switch to for long-term management of his condition?

Atomoxetine

Ritalin

Sertraline

Risperidone

Melatonin

A

Atomoxetine is a noradrenaline reuptake inhibitor. It is recommended for ADHD refractory to Methylphenidate or in those who it is not appropriate due to risk factors or development of side effects. Development of facial tics is an indication to change medications

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105
Q

ild ha

A
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106
Q

A 21-day old baby presents with jaundice. He was full-term, normal birth weight and appeared well until recently. Blood results show a conjugated hyperbilirubinaemia.

What is the most likely diagnosis? [1]

A

This neonate is jaundiced with a conjugated hyperbilirubinaemia. Cystic fibrosis (CF) may also cause these two in conjunction but would likely represent with weight loss or failure to thrive and/or other ciliary disorder symptoms. Biliary atresia is therefore the correct answer. Incidence is approximately 1 in 10 000 and may be associated with other congenital malformations such as intestinal atresia and imperforate anus. It is surgically treated with the Kasai procedure. Many will also go on to require a liver transplant

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107
Q

In a jaundiced neonate presenting with lethargy and feeding difficulties, [] should be performed urgently

A

In a jaundiced neonate presenting with lethargy and feeding difficulties, a septic screen should be performed urgently - sepsis is a potentially life-threatening condition that can present nonspecifically.

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108
Q

A patient takes ciprofloxacin.

5 days later he has very dark urine.

Blood examinations demonstrate a reduced haemoglobin and an elevated urea and bilirubin, alongside deranged liver function tests.

What is the likely dx? [1]

A

G6PD deficiency.

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109
Q

A patient takes ciprofloxacin.

5 days later he has very dark urine.

Blood examinations demonstrate a reduced haemoglobin and an elevated urea and bilirubin, alongside deranged liver function tests.

What is the likely blood film results? [2]

A

Red-cell fragments and Heinz bodies

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110
Q

Name this finding [1]

A

Brushfield spots are small, white-grey aggregations of connective tissue at the peripheral iris

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111
Q

Which genetic condition are Brushfield spots most likely to be found in? [1]

A

Trisomy 21

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112
Q

painless rectal bleeding in children - suspect? [1]

A

Meckel’s diverticulum

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113
Q

A child is started on Methylphenidate.

Which of the following is the most appropriate monitoring while on this medication?

Measure blood pressure every 3 months

Monitoring BMI

Measure height every 6 months

ECG every 3 months

Measure weight every 6 months

A

This medication can stunt growth through appetite suppression, and so NICE advise the following: checking height every 6 months

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114
Q

What do NICE recommend how to monitor patients if they’re on. methlyphenidate? [+]

A
  • measure height every 6 months in children and young people
  • measure weight every 3 months in children 10 years and under
  • measure weight at 3 and 6 months after starting treatment in children over 10 years and young people, and every 6 months thereafter, or more often if concerns arise
  • measure weight every 6 months in adults
  • plot height and weight of children and young people on a growth chart and ensure review by the healthcare professional responsible for treatment
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115
Q

The most sensitive view for SUFE is the [] view as typically the slip is [anterior/posterior]

A

The most sensitive view for SUFE is the frog-leg view as typically the slip is posterior

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116
Q

Children with SCD should recieve which vaccines? [3]

A

Meningococcal conjugate vaccine
annual pneumococcal immunisations
Get the influenza vaccine annually from 6 months of age

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117
Q

How do you differentiate between Edwards and Patau syndromes? [+]

A

Patau syndrome:
- microcephaly, cleft lip and palate, polydactyly, and cardiac defects.

Edwards syndrome
- clenched hands, rocker-bottom feet, and low-set ears.

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118
Q

In Paediatrics, the Pierre Robin sequence is characterised by a [3]

A

In Paediatrics, the Pierre Robin sequence is characterised by a cleft palate, retracted tongue, and a small lower jaw

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119
Q

A child has ?ALL

What would be diagnostic of this on bone marrow biopsy? [1]

A

The key diagnostic feature of ALL is the presence of greater than 20% blast cells in the bone marrow. This significant blast count differentiates it from other conditions like myelodysplastic syndromes, where the blast count is usually lower.

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120
Q

he decision is made to start Ritalin.
Which of the following regular investigations should be performed to monitor patients on this drug?

Height and weight measurement
1

Neurological examination
2

Echocardiogram
3

Full Blood Count
4

ECG and BP measurement

A

Height and weight measurement

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121
Q

A patient has ADHD.

They have regular 6 month height and weight checks for their ritalin use.

What would indicate a change of medication? [1]

A

A change that crosses 2 percentile lines should prompt consideration of a drug holiday to allow catching up, or a medication change.

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122
Q

A neonate has grunting, nasal flaring and a RR of 70.

They have a CXR.

What is the most likely diagnosis based off this clinical picture? [1]

A

Transient tachypnoea of the newborn

Imaging Findings:
* Hyperinflation of the lungs
* Fluid in the fissures
* Laminar effusions
* Fuzzy vessels

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123
Q

Evidence of bowel sounds in a respiratory exam of a neonate in respiratory distress should make you consider a []

A

Evidence of bowel sounds in a respiratory exam of a neonate in respiratory distress should make you consider a diaphragmatic hernia

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124
Q

What is the pathophysiology of congenital diaphragmatic hernia? [1]

How does it present? [2]

A

usually represents a failure of the pleuroperitoneal canal to close completely
- 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.

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125
Q

Describe the managment of CDH [3]

A

Initial management is through the insertion of a nasogastric tube with the aim of keeping air out of the gut.

Therefore for cyanosed pt the best way to assist breathing is to intubate and ventilate. The child needs definitive management in the form of surgical repair of the diaphragm.

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126
Q

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

1, 2 minutes
2, 5 minutes
0, 5 minutes
1, 5 minutes
2, 4 minutes

A

1, 5 minutes

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127
Q

What are the components of the APGAR score? [5]

A

Pulse, respiratory effort, colour, muscle tone, reflex irritability

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128
Q

[polyhydramnios / oligohydramnios] causes developmental dysplasia of the hip (DDH).

Why? [1]

A

The correct answer is oligohydramnios. Polyhydramnios refers to a spectrum of disorders that range from subtle acetabular dysplasia to complete dislocation of the hip.
- Oligohydramnios, which is a condition characterised by low amniotic fluid in pregnancy, can lead to DDH as it restricts foetal movement and thus normal development of the hip joint.

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129
Q

You are working on the paediatric ward and are called to see a child who the nurses have noticed is persistently ‘floppy’. You assess the child and find no acute cause for concern and wonder whether there might be an underlying issue.

Which of the following is most likely to be the underlying cause of neonatal hypotonia in this case?

Pierre-Robin syndrome
Becker Muscular Dystrophy
Duchenne Muscular Dystrophy
Congenital Talipes Equinovarus
Prader-Willi syndrome

A

Prader-Willi syndrome
- floppy baby can be due to prader-willi, cus think of a floppy willy

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130
Q

A diagnosis of croup is made and treatment is commenced. What is the most suitable first-line treatment for this child?

Nebulised salbutamol
Oral dexamethasone
Humidified oxygen
IV hydrocortisone
Broad spectrum antibiotics

A

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

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131
Q

Describe the difference in timelines for management for Exomphalos vs Gastroschisis? [2]

A

Exomphalos should have a gradual repair to prevent respiratory complications
- Staged closure starting immediately with completion at 6-12 months.

Gastroschisis requires urgent correction
- newborns should go to theatre as soon as possible after delivery, e.g. within 4 hours

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132
Q

Describe how you distinguish between NRDS and TTN with regards to presentation time [1] and CXR findings [1]

A

Neonates with NRDS
- usually present with respiratory distress shortly after birth which usually worsens over the next few days.
- CXR in NRDS the characteristic features are a diffuse ground glass lungs with low volumes and a bell-shaped thorax

TTN
- usually presents with tachypnoea shortly after birth and often fully resolves within the first day of life
- In TTN the CXR depicts a heart failure type pattern (e.g. interstitial oedema and pleural effusions) but key distinguishing features from congenital heart disease are a normal heart size and rapid resolution of the failure type pattern within days.

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133
Q

You detect a continuous machinery murmur over the upper left sternal edge. The murmur does not vary with position or radiate

Which of the following is this murmur most likely to reflect?

Innocent murmur
Patent ductus arteriosus
Pulmonary stenosis
Coarctation of the aorta
Tetralogy of Fallot

A

Patent ductus arteriosus: machinery murmur at the upper left sternal edge

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134
Q

At what age do you suggest that nocturnal enuresis can be managed from reassurance and advice to an alarm? [1]

A

Children under the age of 5 years who have nocturnal enuresis can be managed with reassurance and advice
- Offer an enuresis alarm system usually reserved for children over the age of 7 years who have persistent nocturnal enuresis

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135
Q

[] should be given to maintain a patent ductus arteriosus in cyanotic congenital heart diseases

A

Prostaglandin E1 (AKA alprostadil) should be given to maintain a patent ductus arteriosus in cyanotic congenital heart diseases

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136
Q

What is the most appropriate way to confirm a diagnosis of pertussis?

Blood cultures
Sputum culture
Per nasal swab
Urine for serology
Throat swab

A

What is the most appropriate way to confirm a diagnosis of pertussis?

Blood cultures
Sputum culture
Per nasal swab
Urine for serology
Throat swab

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137
Q

Which ear testing is performed at school? [1]

How does it work? [1]

A

Pure tone audiometry is done at school entry in most areas of the UK
- This involves wearing headphones and asking the child to raise a hand or press a button when they hear a beep. Usually, the sounds played will vary in pitch and be played in each ear to assess left and right hearing.

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138
Q

A baby of 8 hrs presents with jaundice.

What would be your initial investigation? [1]
Why not a different one? [1]

A

Measure serum bilirubin within 2 hours

transcutaneous bilirubinometer
* Very important info that < 24hrs transcutaneous Billi can’t be measured.

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139
Q

Which one of the following best describes the typical distribution of atopic eczema in a 10-month-old child?

Nappy area and flexor surfaces of arms and legs
Face and trunk
Nappy area and trunk
Flexor surfaces of arms and legs
Scalp and arms

A

Face and trunk
- This distribution is due to the fact that these areas are more exposed to environmental allergens and irritants. The cheeks, forehead, and extensor surfaces of the limbs are common sites. The nappy area is usually spared because it is covered and protected from these factors.

140
Q

A baby is born with micrognathia, low-set ears, rocker bottom feet and overlapping of fingers -[] syndrome

A

A baby is born with micrognathia, low-set ears, rocker bottom feet and overlapping of fingers - Edward’s syndrome

141
Q

[] criteria is used to assess the probability of septic arthritis in children

A

Kocher’s criteria is used to assess the probability of septic arthritis in children

142
Q

What are 4 signs of non-innocent murmurs? [4]

A

Signs of NOT innocent murmrs:
- Symptomatic
- Pan-systolic or diastolic
- Harsh, loud, thrills
- Radiates

143
Q

What are the 8’S of innocent murmurs? [8]

A

8S’s of an innocent murmur:
- Soft
- Systolic
- Short
- S1 & S2 normal
- Symptomless
- Special tests normal
- Standing/sitting (postural variation)
- Sternal depression (i.e. pectus excavatum apparently)

144
Q

Infantile spasms - classically characterised by repeated [] followed by []

How old are patients generally? [1]

A

Infantile spasms - classically characterised by repeated flexion of head/arms/trunk followed by extension of arms
- This is a type of epilepsy presenting at 4-8 months

145
Q

How would infantile spasms present on EEG? [1]

A

On EEG, this would show a dramatic hypsarrhythmia appearance.

146
Q

What is the exact anatomical position of where a hypospadias is most likely to be? [1]

A

On the distal ventral surface of the penis

147
Q

What Ix choice is used to dx vesicoureteric reflux [1] and the complications associated with it such as renal scarring? [1]

A

vesicoureteric reflux:
micturating cystourethrogram (MCUG)
- fluoroscopic visualisation of the bladder and urethra during voiding after instillation of contrast material into the bladder through a catheter

renal scarring in a child with vesicoureteric reflux: Radionuclide scan using dimercaptosuccinic acid (DMSA)
- administering a radiopharmaceutical agent, dimercaptosuccinic acid (DMSA), which is taken up by healthy renal tubular cells and allows for imaging of the kidneys.
- Areas that do not take up DMSA represent scars or non-functioning kidney tissue.

148
Q

Indomethacin and ibuprofen are both used to treat PDA.

What would indicate indomethacin / contraindicate ibuprofen? [1]

A

significant hyperbilirubinaemia: increasing the risk of kernicterus.
- Ibuprofen can displace bilirubin from albumin-binding sites, leading to increased free bilirubin, which can cross the blood-brain barrier and cause neurotoxicity.

149
Q

Turner’s syndrome is associated with an [] murmur due to [heart defect]

A

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

150
Q

Management of a SCFE: [3]

A

Management of a SCFE: internal fixation (this prevents the slip from worsening)
- A slipped upper femoral epiphysis is an emergency, as there is a risk of avascular necrosis of the femoral head.
- In the meantime, the child should not weight bear and should be made comfortable with analgesia

151
Q

A child falling over and having a head injury x vomiting is normal.

When would NICE rec. that a CT is indicated? [1]
How quickly should you try and give? [1]

A

Head injury in a child: Vomiting is common - NICE recommend an immediate CT scan if 3 or more episodes of vomiting

152
Q

On examination, the child appears well and there is erythema and excoriation around the anus or perineal area with no other abnormalities noted.

Given the likely diagnosis, what is the causative organism?

Enterobius vermicularis
Molluscum contagiosum birus
Pediculus humanus capitis
Trichophyton rubrum
Staphylococcus aureus

A

Enterobius vermicularis (threadworms)

153
Q

[] is first line therapy for treatment of threadworm
- Do you treat the indiviudal or whole house? [1]

A

Mebendazole is first line therapy for treatment of threadworm
- Treat household

154
Q

What is the first sign of puberty in boys?

Development of axillary hair
Height spurt
Development of pubic hair
Increase in penis length
Increase in testicular volume

A

increase in testicular volume.

155
Q

whats a good mneumonic for remembering the order of development of puberty in boys [4] and girls [4]

A

Boys:
* Grapes (testicles)
* drapes (hair)
* grow
* blow (ejaculation)

Girls:
* Boobs
* Pubes
* Grow
* Flow

156
Q

TOF
- What feature of his condition would most determine the degree of cyanosis and clinical severity?

Overriding aorta
Patent ductus arteriosus
Right ventricular hypertrophy
Right ventricular outflow obstruction
Ventricular septal defect

A

Right ventricular outflow obstruction
- If there is significant stenosis, deoxygenated blood entering the right ventricle can only travel into the arterial circulation via the overriding aorta (i.e. it cannot travel into the pulmonary circulation like normal due to the stenosis of the pulmonary valve). This will lead to an increased amount of deoxygenated blood in the arteries, causing a bluish-tinge to the skin (i.e. cyanosis). If the degree of pulmonary stenosis is less, deoxygenated blood entering the right ventricle can travel into the pulmonary circulation and not into the aorta, thus allowing oxygenation and less severe cyanosis.

157
Q

Describe the treatment plan for umbilical hernias in children [2]

A

Umbilical hernias:
- Usually self-resolve, but if large or symptomatic perform elective repair at 2-3 years of age.
- If small and asymptomatic peform elective repair at 4-5 years of age.

158
Q

If a newborn baby has an abnormal hearing test at birth they are offered the [] test

A

If a newborn baby has an abnormal hearing test at birth they are offered the auditory brainstem response test

159
Q

On examination, the child is slim built but has disproportionately large calves. When asked to walk across the room he does so on his tiptoes. Gowers test is positive.

What is the most likely Dx [1] and initial Ix? [1]

A

Duchenne muscular dystrophy:
- genetic testing rather than a muscle biopsy is now used to make a diagnosis

160
Q

When would you consider a pneumonia versus bronchiolitis in a presenting infant? [2]

A

.NICE state the following:
Consider a diagnosis of pneumonia if the child has:
* high fever (over 39°C) and/or
* persistently focal crackles.

NB: A low-grade fever is typical in bronchiolitis.

161
Q

[respiratory sign] is a red flag in paediatric patients with a fever [1]

A

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

162
Q

What are the red signs for NICE traffic light system? [5]

A
  • Moderate or severe chest wall recession
  • Does not wake if roused
  • Reduced skin turgor
  • Mottled or blue appearance
  • Grunting
163
Q

A breastfed baby with GORD should have a trial [] first line

A

A breastfed baby with GORD should have a trial an alginate (e.g. .Gaviscon) first line

164
Q

Roseola infantum is most likely to be caused by:

HHV-4
HHV-5
HHV-6
HHV-7
HHV-8

165
Q

Which cause of a rash is most likely to be associated with suboccipital lymphadenopathy? [1]

Rubella
Measles
Roseola
Hand, foot and mouth
Chickenpox

A

Which cause of a rash is most likely to be associated with suboccipital lymphadenopathy? [1]

Rubella
Measles
Roseola
Hand, foot and mouth
Chickenpox

166
Q

Low vitamin A is a risk factor for which of the following?

Rubella
Measles
Roseola
Hand, foot and mouth
Chickenpox

167
Q

A rash followed by acute kidney symptoms 10 days after the rash started is most likely caused by which pathology? [1]

A

Scarlet Fever

168
Q

A patient has vesicular rash.

They then present with symptoms of a pneumonia.

On CXR they have calcifications in the lung

What is the most likely cause? [1]

A

VZV induced pneumonia

169
Q

Rash x thrombocytopenia = ? [1]

170
Q

[] can be used for short term control of enuresis

A

Desmopressin can be used for short term control of enuresis

171
Q

The key investigation in pyloric stenosis is []

A

The key investigation in pyloric stenosis is ultrasound

172
Q

Which vaccines are live? [7]

A

MIBOOTY - MMR, influenza (intranasal), BCG, oral polio, oral rota, typhoid, yellow fever

173
Q

State 5 key physical features of foetal alcohol syndrome [5]

A

Foetal alcohol syndrome is a common cause of microcephaly, with other physical features including: smooth philtrum (flat area of skin between the upper lip and the base of the nose, without a ridge in the middle), hypoplastic upper lip and epicanthic folds (skin fold of the upper eyelid that covers the inner corner (canthus) of the eye)

174
Q

If foetus is detected to have exomphalos.

What would best management be for the baby? [1]

A

C-section at 37 weeks

175
Q

Whilst talking to his parents, you observe the son has a bubbly outgoing personality, and contemplate whether this might be a case of William’s syndrome.

What physical feature would most support this diagnosis?

Rocker-bottom feet
Flattened philtrum
Tall, slender stature
Webbing of the neck
Elfin facies

A

Whilst talking to his parents, you observe the son has a bubbly outgoing personality, and contemplate whether this might be a case of William’s syndrome.

What physical feature would most support this diagnosis?

Elfin facies

Will turner = legolas = Elf

176
Q

[] is present in around 10% of patients with hypospadias

A

Cryptorchidism is present in around 10% of patients with hypospadias
- This reflects an underlying defect in embryological urogenital migration thought to be associated with endocrine disturbances, such as low serum androgens, during pregnancy.

177
Q

Aside from measuring the child’s length, what physical feature might suggest achondroplasia in this child?

Low set ears
Sandal-gap deformity
Trident hand deformity
Single palmar crease
Microcephaly

A

Trident hands - feature of achondroplasia

178
Q

Non-bilious vomiting in first few weeks of life is consistent with pyloric stenosis

You run a gas to look at their ion picture.

What would you expect? [1]

A

Hypochloremic hypokalemic metabolic alkalosis
- Due to vomiting up stomach contents which is acidic (hydrogen chloride - HCl), the patient will be hypochloraemic. Potassium is also lost in the vomitus.

179
Q

What marker would indicate that a baby has exomphalos (and what would two differentials be) [3]

A

Raised AFP
- But also in multiple gestation and NTD

180
Q

ToF has which murmur? [1]

A

Ejection systolic (the VSD is so big that it doesn’t cause a murmur)

181
Q

Turner’s syndrome has which congenital heart defect as most likely? [1]

A

Bicuspid aortic valve [1]

182
Q

Development / disruptive behaviour x long ears x protruding ears = ? [1]

A

Fragile X syndrome

183
Q

Fever that resolves in an 1 year old, followed by a rash. They then go onto develop a seizure.
What is the most likely dx? [1]

A

Roseola infantum

184
Q

What are the critical features to note in Roseola infantum? [3]

A

6months - 2 yrs (Infantum)
Fever that resolves, but then a rash occurs
Risk of febrile seizures

185
Q

What would make you suspect toddlers diarrhoea in a patient / presenting case? [1]

A

chronic diarrhoea in infants
- diarrhoea that contains lots of undigested food.

186
Q

Which vitamins are cystic fibrosis patients most likely to be deficient in? [3]

A

ADEK - fat soluble vitamins (due to pancreatic sufficiency)

187
Q

He receives a diagnosis of Attention Deficit Hyperactivity Disorder (ADHD) and is started on first-line pharmacological therapy for this.
Which will be the most important side effect to monitor for during the course of treatment with this drug?

Excessive sleepiness

Constipation

Urinary retention

Growth impairment

Psychosis
5

A

Growth impairment
- First-line pharmacological therapy for ADHD is Methylphenidate, a central nervous stimulant. One of the most important and common side effects of Methylphenidate is appetite suppression and subsequent growth suppression. This may also be associated with weight loss

188
Q

‘halo sign’ in the oesophagus indicates what has happened? [1]

What is the next step in management? [1]

A

halo sign suggest that this child has swallowed a button battery.
- The next best management step is to perform an urgent endoscopy under general anaesthetic to remove the battery and decide if further treatment is needed, for example, if the battery has caused a tracheo-oesophageal fistula.

189
Q

Which pathogen are the most common cause of:
- early neonate sepsis (first 72hrs)
- late neonate sepsis (after 72hrs)

A

Early neonate:
- S. aureus is the most common cause of late-onset neonatal sepsis (after 72 h of life).

Late neonate
- S. agalactiae, or group B streptococcus

190
Q

You are assessing a baby’s pulse because they are unresponsive.

Which is the most appropriate pulse to feel if:
- the child < 1 year [1]
- the child > 1 year? [1]

A

brachial pulse if < 1 year

The carotid pulse is the standard site for assessing circulation in children older than one year and adults.

191
Q

An ultrasound scan shows concentric echogenic and hypoechogenic bands.

What does this likely indicate? [1]

A

Intussusception

An ultrasound scan shows concentric echogenic and hypoechogenic bands
- aka target sign

192
Q

What is the age limit of normal for sitting up unsupported? [1]

A

if beyond 9months and can’t sit upright by themselves - gross motor incompetence

193
Q

If a child is young, having stridor and is foreign - what should you suspect? [1]

A

Epiglottis

194
Q

An 18-year-old man was evaluated for social anxiety and repetitive speech patterns.
On examination, he had a long face, large ears, a prominent jaw, and hypermobile joints. The family history reveals other male relatives with similar behavioural issues and learning difficulties.

What is the diagnosis? [1]
What is the underlying genetic abnormality? [1]

A

Fragile X syndrome
- expanded CGG repeat in the FMR1 gene

195
Q

What is the initial investigations for oesophageal atresia? [2]

A

the initial investigation of choice is NG tube insertion followed by a chest X-ray to visualise the level of the tube tip and confirm the atresia.

196
Q

On examination of the baby, there is upslanting palpebral fissures and a sandal toe gap.

What is this most likely to suggest? [1]

A

Trisomy 21

198
Q

A newborn baby is noted to have low-set ears, rocker bottom feet and overlapping of her fingers. What is the most likely diagnosis?

Patau syndrome
Edward’s syndrome
William’s syndrome
Fragile X
Pierre-Robin syndrome

A

A newborn baby is noted to have low-set ears, rocker bottom feet and overlapping of her fingers. What is the most likely diagnosis?

Patau syndrome
Edward’s syndrome
William’s syndrome
Fragile X
Pierre-Robin syndrome

199
Q

NICE has also recently recommended in view of emerging research that [] should be the first line investigation in all children presenting with global developmental delay

A

NICE has also recently recommended in view of emerging research that genetic karyotyping should be the first line investigation in all children presenting with global developmental delay

200
Q

A CT head shows a heterogeneous single mass in both the right and left frontal lobes. Which of the following is the most likely diagnosis?

Craniopharyngioma
1

Pilocytic astrocytoma
2

Ependymoma
3

Meningioma
4

Glioblastoma multiforme

A

Glioblastoma multiforme
- A frontal mass on CT that crosses the midline, in a patient presenting with a seizure, is most likely to be a grade 4 astrocytoma (known as glioblastoma multiforme).

201
Q

What is the 4D, 3C of measles? [1]

A

4 days of fever
Cough, Conjunctivitis, Coryzal symptoms
+ Rash that starts behind ears

202
Q

This baby with prolonged jaundice (>14 days) with pale stools, dark urine and a conjugated hyperbilirubinaemia is highly suspicious for []

A

This baby with prolonged jaundice (>14 days) with pale stools, dark urine and a conjugated hyperbilirubinaemia is highly suspicious for obstructive jaundice

203
Q

This baby with prolonged jaundice (>14 days) with pale stools, dark urine and a conjugated hyperbilirubinaemia is highly suspicious for obstructive jaundice.

The ultrasound findings of echogenic hepatic fibrosis suggest []

A

This baby with prolonged jaundice (>14 days) with pale stools, dark urine and a conjugated hyperbilirubinaemia is highly suspicious for obstructive jaundice. The ultrasound findings of echogenic hepatic fibrosis suggest biliary atresia

204
Q

Biliary atresia is definitively diagnosed with [], which will fail to show the biliary tree

A

Biliary atresia is definitively diagnosed with cholangiography, which will fail to show the biliary tree

205
Q

What’s the mneumonic for Kawasaki’s presentation? [+]

A

CRASH and BURN

Conjuctivitis (bilateral, bulbar, non-purulent)
Rash (anything but vesicles or bullae
Adenopathy (cervical, > 1.5cm)
Strawberry tongue (other skin changes - lip cracking)
Hand and feet swelling (and peeling)

BURN - 5 days of fever

206
Q

A 2-week-old premature infant presents with abdominal distension, feeding intolerance, and bloody stools. Abdominal X-ray shows pneumatosis intestinalis.
What is the most likely diagnosis?

Malrotation

Intussusception

Necrotising enterocolitis

Gastroesophageal reflux disease

Hirschsprung disease

A

A 2-week-old premature infant presents with abdominal distension, feeding intolerance, and bloody stools. Abdominal X-ray shows pneumatosis intestinalis.
What is the most likely diagnosis?

Malrotation

Intussusception

Necrotising enterocolitis

Gastroesophageal reflux disease

Hirschsprung disease

red arrows = pneumatosis intestinalis
yellow arrows = Riger sign

207
Q

A patient has headlice. Do they need school exclusion? [1]

208
Q

Given the underlying congenital syndrome (trisomy 21), which of the following ENT manifestations would you expect in this patient?

Choanal atresia

Micrognathia

Large ears

Cleft lip/palate

Flat nasal bridge

A

ENT manifestations in Down’s syndrome include: small nose with a flat nasal bridge, small and low set ears, hearing issues and ear infections, and hypothyroidism

209
Q

Describe the difference in deterioration of sx in bronchiolitis vs viral wheeze [1]

A

Viral wheeze is more immediate, bronchiloitis has worse sx at day 3-5

210
Q

Which genetic conditions increase the liklihood of a cleft palate? [2]

A

DiGeorge syndrome and Patau syndrome (trisomy 13)

211
Q

Large ears are a feature of [genetic condition]

A

Large ears are a feature of Fragile X syndrome.

212
Q

. Micrognathia (undersized jaw) occurs in congenital syndromes such as [1]

A

Edward’s syndrome (trisomy 18)

213
Q

[] is now the first-line investigation for suspected asthma
- What would be a positive result in children? [1]

A

FeNO (fractional exhaled nitric oxide) is now the first-line investigation for suspected asthma
- In children, a positive value is a ppb of ≥ 35.

214
Q

What are the doses of adrenaline for < 6 months, 6month-6years, 6-12years and 12+ for anaphylaxis? [4]

A

Child > 12 years: 500 micrograms IM (0.5 mL)
Child 6-12 years: 300 micrograms IM (0.3 mL)
Child 6 months - 6 years: 150 micrograms IM (0.15 mL)
Child < 6 months: 100-150 micrograms IM (0.1-0.15 mL)

215
Q

all children with croup should receive a dose of [1]

A

all children with croup should receive a dose of oral dexamethasone if tolerated (otherwise nebulised or IM).

216
Q

Ptx with ADHD:
What area of the brain shows reduced function in the boy’s condition?

Frontal lobe

Parietal lobe

Hippocampus

Wernicke’s area

Broca’s area

A

The theory is that, as executive function controls the ability to focus attention and inhibit impulsive behaviours, a reduced function of the frontal lobe results in inattention and impulsivity

217
Q

Describe what is meant by a reflex anoxic seizure? [1]

A

A reflex anoxic seizure is caused by overactivity of the vagus nerve, causing vasodilation and a collapse from a temporary reduction in cerebral perfusion

218
Q

A 7-year-old presents to the paediatric accident and emergency department complaining of “pain in my tummy”. His father says his son had a sore throat a few days ago. On examination there is pain in the right iliac fossa, but there is no guarding. Urine dipstick is normal.

What is the most likely diagnosis?

219
Q

Given the likely diagnosis of acute epiglottitis, which of the following is the best next step in the management of this patient?

Prescribe intravenous broad-spectrum antibiotics

Perform an ENT exam

Prescribe oral Dexamethasone

Endotracheal intubation

Give intramuscular Adrenaline

A

Endotracheal intubation

220
Q

A child has anosmia.

Which genetic conditon are they likely to have? [1]
Name some other presenting features [2]

A

Kallmann syndrome
- hypogonadotropic hypogonadism
- amenorrhoea
-

221
Q

Which of the following is a hearing, speech and language milestone for a 6-month-old baby?

Saying 1-3 words
1

Startles to noise
2

Responding to own name
3

Turns head towards loud sounds
4

Saying ‘mama’ or ‘dada’

A

Turns head towards loud sounds

222
Q

Paediatric intestinal malrotation with volvulus → treatment? [1]

A

Paediatric intestinal malrotation with volvulus → Ladd’s procedure (includes division of Ladd bands and widening of the base of the mesentery)

223
Q

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?

Anorectal Pull through
Bowel Irrigation
Full Thickness Rectal Biopsy
Lactulose
Vancomycin

A

Bowel Irrigation

If Hirschsprung;s disease is suspected, a full thickness rectal biopsy should be performed as investigation. However, while this is being organised etc, bowel washouts should be performed to allow the baby to pass meconium, so this is the initial treatment. Once a diagnosis has been made, an anorectal pull through is generally performed as definitive management.

224
Q

What are exam findings in bronchiolitis? [2]

A

Widespread polyphonic wheeze
Crepitations

225
Q

At what age would the average child start to say ‘mama’ and ‘dada’?

3 months
4-5 months
6-7 months
9-10 months
13-14 months

A

9-10 months

226
Q

Turner’s syndrome is associated with an [] murmur due to [heart condition]

A

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

227
Q

The consultant suspects the patient may have vesicoureteral reflux.
Which investigation is used to confirm the diagnosis? [1]

A

Micturating cystourethrogram
- injecting dye through a urinary catheter to visualise the direction of flow of urine.

DMSA scan is used to confirm if renal scarring has occurred

228
Q

In neonates, [1] is the most common cause of cardiac arrest

A

In neonates, **respiratory arrest **is the most common cause of cardiac arrest

229
Q

In neonates, respiratory arrest is the most common cause of cardiac arrest.

How do you manage this? [1]

A

Necessitating immediate administration of inflation breaths for resuscitation
- Five gentle breaths with a resuscitation device establish lung inflation, stimulate breathing, and open airways before further interventions if needed.

230
Q

A 6 year old patient is recovered from a local swimming pool and is found to be unconscious, and not breathing.
What is the most appropriate initial management of this patient?

2 rescue breaths

Chest compressions 30:2

Chest compressions 15:2

Place in recovery position

5 rescue breaths

A

5 rescue breaths
- Children are more likely to suffer a respiratory arrest, especially in cases of drowning.
- Therefore, prior to starting chest compressions, the first treatment in these patients is five rescue breaths
- Chest compressions and breaths thereafter are done in a ratio of 15:2 in paediatric patients

231
Q

A 15-year-old boy initially presented with shin pain, attributed to growing pains. The pain persisted, and he developed intermittent fevers, night sweats, and swelling in his left shin. A plain film showed a lamellated periosteal reaction and reduced bone matrix in the proximal tibia. He is referred for MRI and biopsy.
The results are reported below:

MRI:
- A mass is noticeable in the proximal left tibia which is 4cm in diameter. A small area of necrosis is visible at the centre. There is minimal evidence of cortical damage in the proximal tibia
.
Bone biopsy:
- Histology: Small blue round cells are present and have a clear cytoplasm on haematoxylin and eosin staining.
What is the most likely diagnosis?

A

Ewing’s sarcoma typically presented with the lamellated (onion skinning) periosteal reaction which is visible on x-ray. MRI typically shows a large mass with evidence of necrosis and on histology small blue round cells are visible with clear cytoplasms on haematoxylin and eosin staining

232
Q

A two-week-old boy is admitted following a five day history of vomiting, abdominal distension and passage of blood stained stools. He was born at 32 weeks due to ruptured membranes.

Dx? [1]

A

Necrotising Enterocolitis

The clinical signs described and prematurity point towards a diagnosis of necrotising enterocolitis (NEC). Pneumatosis intestinalis or gas in the gut wall is pathognomonic

233
Q

Severe pyloric stenosis can lead to [acid/base change] due to excessive vomiting

A

Severe pyloric stenosis can lead to hypochloraemic hypokalaemic metabolic alkalosis due to excessive vomiting - so hypokalaemia may be present.

234
Q

Due to the breech birth, this child is at higher risk of []

A

Due to the breech birth, this child is at higher risk of having developmental dysplasia of the hip

235
Q

juvenile idiopathic arthritis (JIA) patients are at risk of which complications?

A

chronic anterior uveitis

Flexion contractures: May require physical therapy and splinting.

Joint destruction: May necessitate prostheses at a young age.

Growth failure: This can occur as a result of chronic disease and steroid use.

Macrophage Activation Syndrome (MAS) - Hyperinflammation syndrome of fever, hepatosplenomegaly, encephalopathy, raised liver enzymes and high ferritin.

Osteoporosis

236
Q

In an adolescent, localised pain of several months duration with no traumatic event or associated injury and an associated mass on examination is highly suggestive of [].

A

In an adolescent, localised pain of several months duration with no traumatic event or associated injury and an associated mass on examination is highly suggestive of osteosarcoma.

237
Q

A 4 year old boy is referred for an urgent MRI due to ongoing headaches and nausea. He is otherwise well, with no past medical history. He is growing well and hitting all of his developmental milestones. The MRI report says there is a cerebellar lesion that is most likely benign.
What is the most likely diagnosis?

Ependymoma

Medulloblastoma

Pilocytic Astrocytoma

Craniopharyngioma

Glioblastoma

A

Pilocytic astrocytoma is the most common benign brain tumour in children, often arising in the cerebellum.

238
Q

How do pilocytic astrocytomas present on an MRI? [1]

A

It presents with signs of raised intracranial pressure (headaches and nausea) and is frequently detected on imaging as a well-demarcated cerebellar lesion. These tumours typically have a good prognosis with surgical resection.

239
Q

Pilocytic Astrocytoma is a tumour seen in children, often found in the cerebellar.

Name another tumour often found in the cerebellar and how you would distinguish between them?

A

Medulloblastoma is the most common malignant brain tumour in children and arises in the posterior fossa, often involving the cerebellum
- **raised intracranial pressure **but is more likely to cause ataxia and other cerebellar signs
- distinguishable on MRI

240
Q

Describe the symptoms that a craniopharyngioma would present with [3] and why this occurs? [1]

A

Craniopharyngiomas arise from Rathke’s pouch remnants and typically present with symptoms of endocrine dysfunction (e.g., growth failure due to hypothalamic or pituitary involvement) or visual disturbances due to proximity to the optic chiasm.

241
Q

Which type of brain tumours commonly cause hydrocephalus in children ? [1]

A

Ependymomas are glial tumours that often arise from the ependymal lining of the ventricles, particularly in the posterior fossa in children. These tumours may obstruct cerebrospinal fluid flow, causing hydrocephalus

242
Q

[] is the most common cause of hip pain in children

A

Transient synovitis is the most common cause of hip pain in children
- Limited range of motion, specifically extension and internal rotation of the hip can be found. It is confirmed using a hip x-ray and ultrasound, which shows synovitis.
- It is treated using NSAIDs and bedrest. It usually clears in 7-10 days.

243
Q

A newborn born at 38+2 weeks by normal vaginal delivery, with complications of umbilical cord prolapse.

Which of the following is the most appropriate management to reduce the risk of secondary reperfusion injury?

Mechanical ventilation

Therapeutic cooling to 33–34 °C

Phenobarbital 20 mg/kg

High-flow oxygen supplementation

Fluid restriction to 40 ml/kg/day

A

Therapeutic cooling to 33–34 °C
- Therapeutic cooling slows cerebral metabolism and subsequently slows neurotransmitter and oxygen free-radical release. The reduction in cerebral oxygen requirements is thought to reduce the risk of secondary neuronal death.

244
Q

Meningococcal infection is caused by Neisseria meningitidis, a gram-negative intracellular diplococcus.

Describe the basic pathophysiology that this causes [2]

A

The pathological process is triggered by bacteria entering the circulation, initiating inflammatory processes leading to capillary leakage and intravascular thrombosis. This consumes the clotting factors rendering people coagulopathic
- This coagulation leads to consumption of platelets, rather than decreased production

245
Q

Which x-ray changes would you expect to see in rickets? [1]

A

In rickets, as there is an excess of non-mineralised osteoid bone, there is widening of the growth plates, commonly seen in the wrist.

246
Q

How do you differentiate between transient tachypnea of the newborn and NRDS?

A

Neonatal respiratory distress syndrome:
- presents with increased work of breathing shortly after birth, and the chest x-ray demonstrating diffuse granular opacities is characteristic of neonatal respiratory distress syndrome

TToN:
- Transient tachypnea of the newborn is commonly seen after C-section deliveries due to delayed resorption of lung fluid.
- It is also more common in term babies

247
Q

Frequent attendence to which point of care would point towards child abuse? [1]

A

Frequent attendance to the A&E department, rather than GP, may point towards child abuse as parents presume they will see a different doctor each time, making it less likely suspicions will be aroused

248
Q

What are features of growing pains? [+]

A
  • never present at the start of the day after the child has woken
  • no limp
  • no limitation of physical activity
  • systemically well
  • normal physical examination
  • motor milestones normal
  • symptoms are often intermittent and worse after a day of vigorous activity
249
Q

[] is generally used first-line for nocturnal enuresis if general advice has not helped

A

An enuresis alarm is generally used first-line for nocturnal enuresis if general advice has not helped

250
Q

Describe what is meant by Toddler’s diarrhoea [1]

A

Toddler’s diarrhoea is a benign condition that causes the child no problems. It is** due to the fast transit through their digestive system and often contains undigested food**. It requires no treatment

Toddler + diarrhoea = Toddler’s diarrhoea

251
Q

A boy is noted to have a webbed neck and pectus excavatum

Which childhood syndrome is this? [1]

A

Noonan syndrome

252
Q

An infant is found to have small eyes and polydactyly

Which childhood syndrome is this? [1]

A

Patau syndrome

253
Q

A 7-year-old boy with learning difficulties and macrocephaly

Which childhood syndrome is this? [1]

254
Q

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

A

If a formula-fed baby is suspected of having mild-moderate cow’s milk protein intolerance then a extensive hydrolysed formula should be tried
- This formula is tolerated by 90% of infants with cow’s milk protein intolerance. In this formula, the proteins that trigger allergy are hydrolysed into peptides which make them less allergenic.

255
Q

Patent ductus arteriosus - which three key signs? [3]

A

Patent ductus arteriosus - large volume, bounding, collapsing pulse

256
Q

The most appropriate management for primary nocturnal enuresis (bedwetting) in this case is the use of desmopressin.

What is the MoA? [1]

A

ADH analogue

257
Q

When can phototherapy be stopped for hyperbilirubinaemia? [1]

A

Once bilirubin levels have fallen over 50 micromoles/L below treatment threshold, phototherapy can be stopped.

258
Q

Question3/20
A 5 year old girl with Trisomy 21 is seen in the paediatric cardiology clinic. She has no cardiac symptoms, and on auscultation, a systolic murmur is heard at the upper left sternal edge which radiates to the back. There is fixed splitting of the second heart sound.
What is the most likely cause of her examination findings?

Mitral stenosis

Pulmonary stenosis

Tetralogy of Fallot

Ventricular septal defect

Atrial septal defect

259
Q

You are in a genetics clinic and explaining to a mother and father the reasoning why their son has Prader-Willi syndrome. What is the term we use to describe the mode of inheritance for Prader-Willi syndrome?

Autosomal recessive
Autosomal dominant
Imprinting
Pleiotropy
Variable expressivity

A

Imprinting
- In the case of prader-willi the father’s copy of the gene is not present, and the mother’s copy of the gene is present but epigenetically silenced and therefore not expressed, resulting in the prade-willi phenotype.

260
Q

When is hand preference abnormal in a developing child? [1]

A

Hand preference before 12 months is abnormal

261
Q

At what age are children expected to share toys by? [1]

A

Playing alone is normal at this age. Children are only expected to begin to share toys by 3 years of age.

262
Q

How long should you exclude a child from school for whooping cough? [1]

A

48hrs following commencement of abx

263
Q

Describe the presenting features of roseola infantum [3]
Which organism causes it? [1]

A

Roseola infantum is a common viral illness that causes a characteristic 3 day fever and then emergence of a maculopapular rash on the 4th day, following the RESOLUTION of the fever.
- the fever is typically rapid onset and can often predispose to febrile convulsions.
- rash doesn’t scab over
- The rash typically starts on the trunk and limbs (this is different to chickenpox which is typically a central rash)
- Caused by human herpes virus 6

264
Q

Why can febrile fits occur after roseola infantum? [1]

A

HHV6 is neurotropic (attacks the nervous system) and thus a rare complication is encephalitis and febrile fits (after cessation of the fever).

265
Q

How would you determine a child is suffering from whooping cough and not bronchiolitis [2]

What are the othe features? [+]

A

whooping:
- coughs in bouts that are so severe turns red. Vomiting.

No wheeze is then unlikely bronchiolitis

266
Q

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: [4]

A

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

267
Q

A 2-day-old infant presents to the emergency department with tachypnea & poor feeding. Examination reveals pronounced cyanosis, weak pulses, and signs of circulatory failure. Echocardiography confirms a congenital heart disease and surgery is scheduled for 4 days later.

What medication will the patient be given to maintain circulation until surgical correction?

Ibuprofen
Indomethacin
Prostaglandin E1 (PGE1)
Prostaglandin E2 (PGE2)
Prostaglandin F2α (PGF2α)

A

Prostaglandin E1 (PGE1)

268
Q

[] is the investigation of choice for intussusception

A

Ultrasound is the investigation of choice for intussusception
- may show a target-like mass

269
Q

A patient with PDA that is haemodynamically significant.

What initial treatment should be started to manage this condition?

Emergency surgery
Indomethacin
Beta-blockers
Intravenous fluids
Prostaglandins

A

Preterm infants with haemodynamically significant patent ductus arteriosus 1 week after birth - give ibuprofen/indomethacin to promote duct closure

270
Q

A 9-year-old girl is brought to surgery as her mother is concerned that she is too fat. This has now been a problem for over two years and mum feels this is holding her back at school. What is the most appropriate method to ascertain how obese she is?

Body mass index
Body mass index percentile adjusted to age and gender
Weight plotted on percentile chart
Mother’s perception
Waist circumference

A

Body mass index percentile adjusted to age and gender

271
Q

According to the UK immunisation guidelines, all babies, including those born prematurely, should receive their first set of vaccinations at [] weeks from birth

A

According to the UK immunisation guidelines, all babies, including those born prematurely, should receive their first set of vaccinations at 8 weeks from birth
- ‘Give as per normal timetable’
- This is because premature babies are at higher risk of infections and therefore need protection provided by vaccines as soon as possible

272
Q

This child has scarlet fever and has initiated antibiotic therapy. According to current guidelines, children with scarlet fever are advised to return to school [] hours after starting antibiotics.

A

This child has scarlet fever and has initiated antibiotic therapy. According to current guidelines, children with scarlet fever are advised to return to school 24 hours after starting antibiotics.

273
Q

[] is the most common cause of painless massive GI bleeding requiring a transfusion in children between the ages of 1 and 2 years

A

Meckel’s diverticulum is the most common cause of painless massive GI bleeding requiring a transfusion in children between the ages of 1 and 2 years

274
Q

A 12 week baby is being fed formula feed, but is experiencing episodes of regurgitation and vomiting after most feeds and ongoing diarrhoea which contains significant amounts of mucus.

What is the next appropriate step? [1]

A

Trial of extensively hydrolysed formula is the best option here as the baby’s symptoms of vomiting mucus stool and ‘colic’-type episodes fit most with cow’s milk protein allergy. If the extensively hydrolysed formula resolves the symptoms then this essentially confirms this diagnosis and must be continued instead of normal formula milk. If it does not, amino acid-based formula (AAF) may be trialled and/or further investigations may be required.

275
Q

What bone marrow biopsy results would you expect to see in ALL? (the most common type of childhood leukaemia)

A

characterised by pancytopaenic symptoms and bone marrow with blast predominance (>20%).

276
Q

You suspect a patient has transient synovitis.

Which age group is this most likely in? [1]

A

Pre-puberty children

277
Q

You suspect a patient has transient synovitis.

What causes this condition? [1]

Describe the classical presentation [2]

A

It occurs when a viral infection, such as an upper respiratory infection, moves to and settles in the hip joint

Limited range of motion, specifically extension and internal rotation of the hip can be found.

278
Q

How would you confirm a case of transient synovitis? [1]

How do you tx? [1]

A

It is confirmed using a hip x-ray (which will be normal) and ultrasound which shows synovitis.

It is treated using NSAIDs and bedrest for up to 6 weeks

279
Q

In young children presenting with leukocoria (white pupillary reflection), what is the most common [1] and serious [1] causes?

A

Most common: congenital cataracts

Most serious: retinoblastoma

280
Q

A 7-year-old boy presents to the GP clinic with a 1-month history of progressive weakness, fever and weight loss of 4 kg. His parents also reported 2 episodes of nosebleeds without any apparent injury. On physical examination, he has non-palpable pinpoint macules on his skin and a palpable spleen 2 cm below the left costal margin.

What is the most likely genetical abnormality causing this presentation? [1]

A

acute lymphoblastic leukaemia (ALL). The most common cytogenetic abnormality in paediatric ALL is the t(12:21) translocation

281
Q

How long to return to school after Abx treatment for impetigo? [1]

282
Q

A young child presenting with fever, ear rubbing, and bilateral otorrhea is most likely to have [1]

What is the treatment? [1]

A

A young child presenting with fever, ear rubbing, and bilateral otorrhea is most likely to have acute otitis media, requiring antibiotic treatment with amoxicillin.

283
Q

[] is now the most common cause of acute onset of vomiting and diarrhoea in children.

A

Norovirus gastroenteritis is now the most common cause of acute onset of vomiting and diarrhoea in children.

284
Q

Describe a typical social developemntal milestone for a 2.5 yr old child [1]

A

Parallel play, where children play alongside each other without much interaction, is a typical social developmental milestone for a 2 and a 1/2-year-old child

285
Q

What age is having imaginary friends seen as normal social development? [1]

A

having imaginary friends is more common among slightly older children, around the ages of 4 to 6.

286
Q

A neonate fails to pass meconium within first week of life.

How do you determine if this is from Hirschprungs vs cystic fibrosis from the clinical picture? [1]

How would you confirm this? [1]

A

Hirschsprung’s is more likely if the patient fails to pass stool unless a finger is passed per rectum
- A rectal suction biopsy is the gold standard for diagnosis because it allows direct sampling of the rectal mucosa to check for the absence of ganglion cells, which is diagnostic of Hirschsprung’s disease.

287
Q

Describe what is meant by a Ladd’s procedure and what is indicated for [2]

A

Ladd’s procedure is where the surgeon straightens out the bowel and divides any abnormal peritoneal bands (Ladd bands).
- for intestinal malrotation

288
Q

Ptx with MCD doesn’t respond to prednisolone.

What is the next management step? [1]

A

High-dose steroids are the first-line treatment for most children with NS, as the majority respond well to corticosteroids. However, in this case, the patient has not responded to high-dose steroids, indicating steroid-resistant nephrotic syndrome (SRNS).

The next appropriate step in managing SRNS is to stop steroids and start an alternative immunosuppressant like ciclosporin, which is a calcineurin inhibitor

289
Q

What is the first and second line treatment for [2]

A 6 day old baby was born prematurely at 33 weeks. He has been suffering from respiratory distress syndrome and has been receiving ventilatory support on NICU. He has developed abdominal distension and is increasingly septic. Ultrasound of the abdomen shows free fluid and evidence of small bowel dilatation. His blood pressure has remained labile despite inotropic support

A

necrotising enterocolitis and whilst this is often initially managed medically a laparotomy is required if the situation deteriorates.

290
Q

What is the first line treatment for the clinical scenario below? [1]

A 5-year-old child has been unwell with a sore throat and fever for several days. He progresses to develop periumbilical abdominal discomfort and passes diarrhoea. This becomes blood stained. The paediatricians call you because the ultrasound has shown a ‘target sign’.

A

The correct answer is: Pneumatic reduction under fluoroscopic guidance
- This child has an intussusception. The lymphadenopathy will have initiated it. A target sign is seen on ultrasound and is the side on view of multiple layers of bowel wall. Reduction using fluoroscopy with air is the usual first line management. Ileo-colic intussceceptions are generally most reliably reduced using this method, long ileo-ileal intussceceptions usually result in surgery.

291
Q

Describe a typical case of paediatric intestinal malrotation [+]

A

A 1-year-old child is brought to the emergency department by his parents. The parents have noticed that he is clutching his stomach. He has not eaten or drank any fluids for the entire day and he has vomited twice. His mother states the vomit was green in colour.

292
Q

What is the first line tx for paediatric intestinal malrotation with volvulus? [1]

A

Ladd’s procedure

293
Q

A child is still bedwetting. Describe how age would help determine their treatment plan after advise about lifestyle measures such as decreasing fluid intake and toileting before bed have already been tried? [2]

A

The current first-line treatment is an enuresis alarm. If the child is aged under 7, it must be tried before other measures are considered.

Desmopressin may be used first-line if the child is over the age of 7 and does not wish to use the enuresis alarm or if a short term solution is required. This is not the case in this scenario and so would not be the correct answer.

294
Q

Which genetic syndrome is haemophilia linked with? [1]
Why? [1]

Turner’s syndrome
Down’s syndrome
Ataxia telangiectasia
Hunter’s syndrome
Coeliac disease

A

Which genetic syndrome is haemophilia linked with? [1]

Turner’s syndrome
- Haemophilia is a X-linked recessive disorder and would hence be expected only to occur in males. As patients with Turner’s syndrome only have one X chromosome however, they may develop X-linked recessive conditions

295
Q

[] test is used to screen newborns for hearing problems

A

Otoacoustic emission test is used to screen newborns for hearing problems
- The OAE test is used to find out how well your inner ear, or cochlea, works. It measures otoacoustic emissions, or OAEs. These are sounds given off by the inner ear when responding to a sound. There are hair cells in the inner ear that respond to sound by vibrating. The vibration produces a very quiet sound that echoes back into the middle ear. This sound is the OAE that is measured.
- If you have normal hearing, you will produce OAEs. If your hearing loss is greater than 25–30 decibels (dB), you will not produce these very soft sounds.

296
Q

Cardiology reports a ventricular septal defect (VSD). His mother becomes very anxious when she finds out and is keen to find out more about VSD. She asks about the risks to her son.

Which one of the following is he at a high risk of? [1]
Explain your answer [1]

Essential hypertension
Aortic aneurysm
Carotid dissection
Endocarditis
Papillary muscle atrophy

A

Cardiology reports a ventricular septal defect (VSD). His mother becomes very anxious when she finds out and is keen to find out more about VSD. She asks about the risks to her son.

Which one of the following is he at a high risk of?

Endocarditis
- Blood moves at high velocity across defects such as VSD’s, stenotic or leaky valves, and PDA’s. This high velocity flow facilitates the sticking of the blood’s clotting elements to these defects. If bacteria are in the blood stream, they become enveloped in the clot adhering to the defect and endocarditis may develop there.

297
Q

A child presents with bowed legs.

When is this a normal variant till? [1]

A

Bow legs in a child < 3 is a normal variant and usually resolves by the age of 4 years

298
Q

A patient presents like this - what is the usual age of presentation? [1]

When is this a normal variant till? [1]

A

Knock knees (genu valgum) - usually present at 3rd-4th year and spontaneously recover by 8 years old

299
Q

A patient presents like this.

What is the name for this presentation? [1]
When does it usually present? [1]
What is the usual cause? [1]
When is intervention required? [1]

A

Out toeing
- Common in early infancy and usually resolves by the age of 2 years
- Usually due to external tibial torsion
- Intervention may be appropriate if doesn’t resolve as increases risk of patellofemoral pain

300
Q

A patient presents like this.

What is the name for this presentation? [1]
When does it usually present? [1]
What are the usual causes? [3]

A

Usually presents in 1st year

Possible causes:
* metatarsus adductus: abnormal heel bisector line. 90% of cases resolve spontaneously, severe/persistent cases may require serial casting
* internal tibial torsion: difference the thigh and foot ankle: resolves in the vast majority
* femoral anteversion: ‘W’ sign resolves in around 80% by adolescence, surgical intervention in the remaining not usually advised

301
Q

A patient presents with flat feet.

What is the usual age of presentation? [1]
How do you manage? [1]

A

Presents at all ages
* Typically resolves between the ages of 4-8 years
* Orthotics are NOT recommended
* Parental reassurance appropriate

302
Q

Following an emergency Caesarian-section for foetal distress, the consultant obstetrician hands the paediatrician a normal term male neonate. You note that the neonate is apnoeic, floppy and blue in colour.

What should be your first action?

Administer atropine
Initiate cardiopulmonary resuscitation
Give two rescue breaths
Intubate and ventilate
Dry the neonate

A

Dry the neonate

303
Q

Describe in neonatal guidelines for resuscitation [+]

A

Neonatal resuscitation guidelines
* Birth: Dry the baby, remove any wet towels and cover and start the clock or note the time.
* Within 30 seconds: Assess tone, breathing and heart rate.
* Within 60 seconds: If gasping or not breathing - open the airway and give 5 inflation breaths
* Re-assess: If no increase in heart rate look for chest movement
* If chest not moving: Recheck head position, consider 2-person airway control and other airway manoeuvres, repeat inflation breaths and look for a response.
* If no increase in heart rate look for chest movement
* When the chest is moving: If heart rate is not detectable or slow (< 60 min-1) - start chest compressions with 3 compressions to each breath.
* Reassess heart rate every 30 seconds. If heart rate is not detectable or slow (< 60 beats per minute) consider venous access and drugs
*

304
Q

Pulmonary hypoplasia is a term used for newborn infants with underdeveloped lungs

Causes include
[2]

A

Pulmonary hypoplasia is a term used for newborn infants with underdeveloped lungs

Causes include
oligohydramnios
congenital diaphragmatic hernia

305
Q

What are the two most common types of cardiac conditions seen in Turner’s syndrome and how would their murmurs present? [2]

A

Bicuspid aortic valve (most common)
- Aortic stenosis caused by valve causes ejection systolic murmur

Coarctation of aorta
- mid-systolic murmur
- also radio-femoral delay

306
Q

A patient has DKA and is in shock.

She is 20kg.

What bolus fluids what you prescribe? [1]

A

200ml 0.9% NaCl
- initial 10 ml/kg of 0.9% sodium chloride as a bolus (NICE), to children in shock with DKA,

307
Q

Fluid replacement in children
Fluid for shock:
- [] ml/kg of 0.9% NaCl

DKA:
- [] ml/kg of 0.9% NaCl In less than 10 mins

A

Fluid replacement in children
Fluid for shock:
* 20ml/kg of 0.9% NaCl

DKA 10ml/kg of 0.9% NaCl
* In less than 10 mins

308
Q

What are the cardinal signs of Wilm’s tumour? [4]

A

haematuria, hypertension, fever and an abdominal mass.

309
Q

[] is the definitive treatment for pyloric stenosis
- Desribe this procedure [1]

A

Pyloromyotomy is the definitive treatment for pyloric stenosis
- The procedure involves dividing the hypertrophied pylorus muscle down to the level of the mucosa whilst leaving the mucosa intact.

310
Q

Neonatal Respiratory Distress Syndrome is treated with [mode of delivery] of artificial surfactant.

A

Neonatal Respiratory Distress Syndrome is treated with intratracheal instillation of artificial surfactant.

311
Q

You suspect someone has transient synovitis.

What investigation do you need to rule a key ddx? [1]

A

Joint aspiration with microscopy, culture and sensitivities may be necessary to distinguish between transient synovitis and septic arthritis.

312
Q

Describe the potential electrolyte changes of untreated vesicoureteric reflux in childhood [2]

A

Untreated vesicoureteric reflux in childhood can result in kidney damage leading to chronic kidney disease with impaired renal function, manifested by elevated creatinine and urea levels, as well as low calcium and high phosphate due to impaired excretion.

313
Q

[], a selective-serotonin-reuptake inhibitor, is the only antidepressant medication licensed for children and has the best evidence base for use in children.

A

Fluoxetine, a selective-serotonin-reuptake inhibitor, is the only antidepressant medication licensed for children and has the best evidence base for use in children.

314
Q

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.

What is the dx and tx? [2]

A

Hand, foot and mouth disease requires symptomatic treatment only

315
Q

Describe a sign with regards to hand preferances that would indicate cerebral palsy [1]

A

Hand preference before 12 months is abnormal - it could be an indicator of cerebral palsy

316
Q

Describe what innocent murmurs in children are caused by [1]
How do they present [2]

A

Venous hums
- Due to the turbulent blood flow in the great veins returning to the heart.
- Heard as a continuous blowing noise heard just below the clavicles

Still’s murmur
- Low-pitched sound heard at the lower left sternal edge

317
Q

A young child is vomiting.

Describe how you would differentiate if this was caused by pyloric stenosis, malrotation or intestinal atresia [3+]

A

Pyloric stenosis
- Projectile, non-bilious vomiting at 4-6 wks old

Malrotation
- Bilious vomiting, abdominal pain, acutely unwell

Intestinal atresia
- Bilious vomiting if distal to ampulla of Vater, usually hours-days old

318
Q

Jaundice x 6 week old baby = ? [1]
- On exposure, you note multiple small bruises on the torso.

A

Biliary atresia
- The most concerning underlying condition here is biliary atresia. The jaundice (later than that of physiological, breast milk or breastfeeding jaundice) and bruising support a diagnosis of biliary atresia. Measurement of total bilirubin (which would be elevated) and conjugated bilirubin (which would also be elevated as it is going into the bloodstream instead of the bowel lumen) would support this diagnosis.

319
Q

According to the 2024 NICE guidelines, children aged 5 to 11 with asthma that remains uncontrolled on twice-daily low-dose ICS and PRN SABA should have their treatment escalated with [1]

A

According to the 2024 NICE guidelines, children aged 5 to 11 with asthma that remains uncontrolled on twice-daily low-dose ICS and PRN SABA should have their treatment escalated to a paediatric low-dose MART (maintenance and reliever therapy) regimen

320
Q

Which of the following emerging blood tests is most specific to bacterial sepsis?

Potassium

Red blood cell count

Lactate

Procalcitonin

White cell count

A

Procalcitonin
- Procalcitonin is an emerging blood test that shows specificity for inflammatory markers and can help detect bacterial sepsis.

321
Q

[] is a recognised risk factor for slipped capital femoral epiphysis (SCFE)

A

Growth hormone supplementation is a recognised risk factor for slipped capital femoral epiphysis (SCFE)
- SCFE is associated with increased body weight, adolescent growth spurts, and sometimes with endocrine disorders or exogenous growth hormone therapy, all of which can increase stress on the developing growth plate, leading to epiphyseal slippage.

322
Q

The most common type of heart block in children is [1]

A

The most common type of heart block in children is complete heart block, also called third-degree heart block.

323
Q

What are classic presenting features of NEC? [4]

A

patient’s premature birth, bilious vomiting, reduced feeding, and blood in stools are classic features of necrotising enterocolitis (NEC)

324
Q

What investigation would you perform for suspected NEC and what results would indicate this pathology? [3]

A

Abdominal X-ray is the first-line investigation for suspected NEC. Classic findings include pneumatosis intestinalis (gas within the intestinal wall), portal venous gas, and possibly free air if there is bowel perforation

325
Q

A 4 year old girl presents to the GP with her mother after developing a rash. Her mother reports that the patient has had a fever for the past 48 hours and has been complaining of a sore throat. On examination she has a fine punctate erythematous rash across her trunk which spares her palms. The GP notes that, although her face appears flushed, the area around her mouth appears pale.
What is the best treatment? [1]

A

A 10-day course phenoxymethylpenicillin is the first-line treatment for scarlet fever

326
Q

A patient has ?whooping cough.

What is the best first line investigation? [1]

327
Q

A 4 year old girl presents to the GP with her mother after developing a rash. Her mother reports that the patient has had a fever for the past 48 hours and has been complaining of a sore throat. On examination she has a fine punctate erythematous rash across her trunk which spares her palms. The GP notes that, although her face appears flushed, the area around her mouth appears pale.

What is the first line treatment? [1]

A

A 10-day course phenoxymethylpenicillin is the first-line treatment for scarlet fever

328
Q

A newborn baby is examined by the paediatrician after birth. The main findings on examination are low-set ears, microcephaly and omphalocele.

Also has cleft palate and polydactyly.
What is the most likely diagnosis? [1]

A

Patau syndrome

329
Q

A new mother presents to the GP with her 6-week-old infant for her 6-8-week health check. The infant looks well and is meeting all developmental milestones so far. However, the mother is concerned about a red rash on her daughter’s scalp. She explains that it is often flaky and is a little bit scaly. It does not seem to be bothering the child.

Dx and Tx? [2]

A

infantile seborrhoeic dermatitis (cradle cap)
- self-limiting condition which usually resolves by 4 months of age.
- The first line treatment is applying baby oil, vegetable oil or olive oil and then gently brushing the scalp, then washing the oil off.

330
Q

At what age does pyloric stenosis most likely occur? [1

A

typically presenting around 3-6 weeks of age.

331
Q

Because of abnormal expression of genes on chromosome 21, patients with Down’s syndrome are also at increased risk of developing [2]

A

Because of abnormal expression of genes on chromosome 21, patients with Down’s syndrome are also at increased risk of developing Alzheimer’s disease and acute lymphoblastic leukaemia

332
Q

A 6-month-old exclusively breastfed infant presents with bruising, bleeding from gums and nose, and blood in the diaper.

What is the most likely cause of his symptoms?

333
Q

The medical team is concerned that the infant has developed an acute bilirubin encephalopathy.

What is the basic pathophysiology of this condition? [1]

334
Q

Describe the difference in treating bullous and non-bullous impetigo [2]

A

Bullous impetigo typically requires systemic antibiotics due to the deeper involvement of the skin and the risk of bacterial spread.
- Flucloxacillin is the first-line treatment
- Clarithromycin

Non-bullous:
- hydrogen peroxide 1% cream is first line
- If widespread: topical (fusidic acid/mupirocin) or oral antibiotics for 5 days, such as flucloxacillin

335
Q

X-ray signs include hyperinflation, and fluid in the horizontal fissure

What does this indicate? [1]

A

Transient tachypnoea of the newborn (TTN)

336
Q

This baby has which genetic condition? [1]

A

Edwards - Rocker bottom feet

337
Q

What does this image show. [1]

Which condition can they be considered to be in? [1]

A

Cafe au lait spots
- Seen in Neurofibromatosis

338
Q

Which genetic condition is this person most likely to have? [1]

A

Down’s syndrome

339
Q

Which condition does this baby have? [1]

A

Edwards:
- Rocker bottom feet
- Small chin
- Fixed overlapping fingers

340
Q

Which genetic condition does this child have? [1]

A

Pataus
Cleft palate
Polydactyl
Small eyes

341
Q

A baby is detected as having gastroschisis via antenatal US.

What would be the managemnt plan? [1]

A

vaginal delivery with immediate repair
- NB: exomphalos is C section x stage repair

342
Q

Chickenpox is a risk for which subsequent skin infections? [2]

A

GAS which can lead to necrotising fasciitis

343
Q

When is MMR vaccine typically given? [1]

344
Q

3month x acute limp = [next step?]

A

Immediate assessment
- concerned about septic arthritis

345
Q

Neonate born at term.
Their O2 sats at 87%.
What is next step? [1]

A

Observe for 10mins
- can be supoptimal in first 10 mins for healthy neonate

346
Q

How long should you give prednisolone for after acute asthma in children? [1]