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1

What infections are transmitted through the birth canal at delivery?

GBS

E. Coli

Listeria

Hep B

HIV

2

What congenital infections can be transmitted transplacentally?

TORCHSHPV

Toxoplasmosis

Other

Rubella

CMV

HSV

Syphillus (treponema pallidum)

Hep B

Parvovirus B19

Varicella zoster

3

What do the placentally transmitted congenital infections cause?

4

What is the management of placentally transmitted congenital infections?

Generally, the earlier in gestation the foetus congenitally acquires a TORCH infection, the higher the risk of severe congenital abnormalities and developmental delay.

A conversation is therefore usually had with mothers (especially in the first trimester) about the option of termination.

 

Some TORCH infections can be treated in the neonate, but for the majority of TORCH infections the treatment is supportive and includes only management of complications.

5

What screening tests for congenital infections should be offered/recommended to pregnant women?

All mums - SARaH

Syphillus (assays)

Asymptomatic bacteruria (mid stream urine culture, treat empirially to avoid pyelonephritis)

Rubella (anti-Rubella antibodies)

HIV (Western blot and ELISA, give anti-retrovirals to mum, deliver by C-section)

Hep B (HB-sAG, vaccinate and give IgG in delivery room)

GBS (after 37 weeks)

High risk mums - SARaH + 1G, 3T, 4C

Gonorrheoa

Trichomoniasis, Toxoplasmosis & Thyroid function

Chlamydia, Hepatitis C, CMV and cervical abnormalities (pap smear)

 

6

What are the effects of toxoplasmosis infection on a foetus?

If 1st trimester low risk of transmitting to foetus but high rate of severe complications

If 3rd trimester high risk of transmitting the infection but low rate of severe complications

 

Diffiuse calcifications throughout the brain visible on USS

Seizures

Plus the general effects of TORCHSHPV

  • Anaemia
  • Hepatosplenomegaly
  • Symmetrical IUGR (microcephaly)
  • Developmental delay
  • Sensorineural deafness
  • Visual problems

7

What effect does congenitally acquired Rubella have on a foetus?

Outcome depends on gestation age at which it was transmitted.

  • < 12/40 --> congenital rubella syndrome (>90%)
  • 12-18/40 --> sensorineural deafness (20%)
  • >18/40 --> complications rare

8

What effect does congenitally acquired CMV have on the foetus?

Periventricular calcifications

Symmetrical IUGR with microcephaly

Developmental delay

Hepatospleenomegaly

Anemia

Sensorineural hearing loss

Visual problems

9

What is the clinical picture of a baby who has contracted syphillis?

Rhinitis develops at 1 week and worsens. Initially clear then progressively purulent and blood stained.

 

Neurosyphillis

Bone problems

Maculopapular rash
 

10

What is the clinical picture of a foetus that has contracted Parvovirus B19?


Severe anaemia --> high output cardiac failure --> hydrops foetalis
 

11

How do you approach the causes of neonatal sepsis? What are the risk factors and bugs?


EARLY <5 days = SOMETHING TO DO WITH DELIVERY/PREGNANCY
Vertical transmission --> GBS, E coli, Listeria
Risk factors

  • Maternal infection: UTI, GBS+
  • Previous child with GBS sepsis
  • Prolonged rupture of membranes (>18h)
  • Preterm labour
  • Febrile during labour

LATE >5 days = ACQUIRED AFTER BIRTH

Staphylococcus, streptococcus (plus above pathogens)

Risk factors

  • NICU stay (just like ICU stay causes infections)
  • Foreign bodies eg ETT, catheters
  • Malformations

12

What is the management of neonatal sepsis?

13

What tests would you do at the first antenatal assessment?

Every visit

  • BP
  • Weight
  • Urine dipstick
  • Foetal auscultation (after 12weeks)
  • Foetal movements (after 18 weeks)
  • Fundal height measurement (after 20 weeks)

Because it's the first visit

  • LNMP for dating (+ 1 year, - 3 months, + 7 days)
  • ABO, Rh, Rh antibody levels
  • FBE to screen for anaemia
  • EPNDS
  • Routine torch screening --> SARaH
    • Syphilis
    • Asymptomatic bacteruria (MSU culture)
    • Rubella
    • HIV
    • Hep B
  • If high risk, additional TORCH screening --> 1G, 3T, 4C
    • Gonorrheoa
    • Trichomoniasis, Toxoplasmosis & Thyroid function 
    • Hep C, chlamydia, CMV, cervical abnormalities (pap smear)

14

What are the diagnostic criteria for encopresis?

  • < 2 or fewer defecation in toilet per week
  • at least 1 episode of faecal incontinence per week
  • history of retentive posturing
  • large and/or painful and/or hard bowel movements
  • large faecal mass in rectum 

15

What are the potential complications of GORD in infants?

oesophagitis 
failure to thrive 
aspiration

16

What is colic?

Crying is normal physiological behaviour in young infants. At 6 - 8 weeks age, a baby cries on average 2 - 3 per 24 hours. Excessive crying is defined as crying >3 hours/day for >3 days/week.  This is often referred to as "colic". However, many babies present with lesser amounts of crying, as the parents perceive it as excessive.

17

What symptoms would make you worry about GORD in a baby with GOR?

  • Pronounced irritability with arching
  • Refusal to feed / failure to thrive
  • Feeding and behavioural problems
  • Weight loss or crossing percentiles
  • Haematemesis
  • Chronic cough/wheeze
  • apnoeas

18

What is the management of GORD in infants?

Non-invasive management

  • Prone position after feeding (supervised)
  • Milk thickening agents (eg. rice cereal) reduce the number of episoes of vomiting but not the total time of oesophageal acidity
  • Don't encourage parents to change formulas
  • Never change a breastfed child to formula

Definitive management

PPIs relieve symptoms and assist mucosal healing

Omeprazole

Under 10kg: 5mg daily
10-20kg: 10mg daily
Over 20kg: 20 mg daily

Surgical

Failure of medical therapy or recurrent respiratory symptoms (aspiration) may necessitate Nissen fundoplication:

19

What are the most causes of "colic"/excess crying in an infant?

Most common - benign

  1. Tiredness

  2. Hunger - this is more likely if a mother reports:

    1. inadequate milk supply 

    2. poor weight gain

    3. baby has frequent feeds (ie, < 3 hourly)

Also consider

  1. GORD
  2. Cows milk intolerance
    1. Check if vomiting, blood/mucus in diarrhoea, poor weight gain, family history in first degree relative, signs of atopy (eczema / wheezing), significant feeding problems
  3. Lactose intolerance (rare)

If Acute onset consider:

  • UTI
  • Otitis media
  • Raised intracranial pressure
  • Hair tourniquet of fingers / toes
  • Corneal foreign body / abrasion
  • Incarcerated inguinal hernia

20

What is the classic triad of intssusception?

colicky abdominal pain, currant jelly stool, palpable abdominal mass

21

Describe the management of suspected intussusception

22

What are the Ix for suspected intussception?

23

What are the risk factors for NEC?

Prematurity and low birth weight
Feeding (especially enteral/cow’s milk/early formula)
Infection/ sepsis
Bowel ischemia
Hypotension and congenital heart disease
Hypoxia/respiratory distress/ birth asphyxia

24

What is the relationship between gestational age and weight and NEC

The incidence of NEC is inversely proportional to birth weight. In general, the age of onset is inversely proportional to gestation; therefore smaller babies present later.


90% of babies with NEC are preterm.

25

What is the management of NEC

Basics

NBM (gut rest)
NGT (drainage)
Insert IV line for fluids and to take sample for FBE & blood cultures

Place and Person

Refer to paeds gastroenterology / surgery

Investigate and confirm diagnosis

AXR is gold standard

Rationalise ABx once cultures back

Definitive management

Empirical antibiotic therapy:

Metronidazole
Ampicillin
Gentamycin

Only in stage 3B disease:

Surgery to resect affected section with stoma diversion and abscess drainage if needed 

26

What are the buzz words for pyloric stenosis?

Projectile, non-bilious vomiting
First born, Caucasian male with a positive family history
Hypochloraemic, hypokalaemic metabolic alkalosis (also get hyponatraemia too, but not included in the buzz word)

27

When does pyloric stenosis generally present?

Usually between 2-6 weeks of life

28

What is the clinical picture o pyloric stenosis?

A sudden onset of vomiting between 2 and 6 weeks of life.

Before the onset of vomiting, these infants feed well and are thriving.
The infant then loses weight (or inadequately gains weight) and becomes dehydrated

Projectile, non bilious vomiting. May contain altered blood.
“Hungry vomiters” – vomit immediately after feeding, and then are hungry again
May be constipated (not absolute)

Examination

Palpable, mobile pyloric tumour in the midline of the epigastrium between the rectus abdominal muscles OR the right rectus and the liver edge (“olive”)

29

What do you assess to determine the hydration status of a baby?

Behaviour/demeanour

HR/BP

Cap refill (check centrally)

Sinking of the anterior fontanelle and eyes

Tear production/Urine output

Skin turgour

Mucus membranes

Weight loss

 

30

What are the Ix for suspected pyloric stenosis?

Usually a clinical diagnosis

Bedside

ABG --> hypochloremic, hyponatremic, hypokalaemic, metabolic alkalosis 

BGL

Bloods and urine

FBE

UEC --> hydration

Coags

Imaging

Ultrasound

If very unsure you can do a barium meal (not usually needed)

 

31

What is the relevance of metabolic derangement in the surgical management of pyloric stenosis?

It is particularly important to fully correct serum bicarbonate before theatre because of the risk of hypoventilation/ apnoeas post-operatively in the setting of metabolic alkalosis.

32

What is the management of pyloric stenosis?

Medical (not surgical) emergency

Basics

IV access and fluid resusc if required and as directed by UEC/ABG results

NBM

NG tube if continuing to vomit

Place and person

Paediatric gastroenterology, admit

Definitive

Pyloromyotomy (Ramstedt operation).

33

What fluid and what rate would you use for bolus IV therapy in a child?


–10-20ml/kg of normal (0.9%) saline, which may be repeated
 

34

How do you calculate maintenance fluid requirements in a child?

35

Baby has bilious vomiting, what is the diagnosis?

Malrotation + volvulus until proven otherwise!

36

What is the clinical presentation of malrotation and volvulus?

History

žPresents day 1-3 of life (60–80% of all cases of malrotation present in the first month of life, mostly in the first week.)
žBilious vomiting (malrotation until proven otherwise!)
žNo dirty nappies once there is volvulus
žRectal bleeding (a late symptom/sign)
žAbdominal pain

Examination

žAbdominal distension (late sign)
žPeritonism (late sign)

37

What imaging would you order for suspected malrotation/volvulus?

What would you expect to see?

AXR - non-specific, gas in bowel, can get "double bubble sign"

Barium swallow - corkscrew sign

USS - whirlpool sign

38

What is the management of malrotation/volvulus?

Basics

NBM

NGT – check aspirates for bile

Insert IV and commence fluids

Place and person

Admit and refer to paediatric surgeon

Ix

ABG

Septic screen

Blood group and hold

Definitive management

žUrgent surgical correction with follow up
žLadd procedure: untwist counter-clockwise, divide Ladd’s band, widen mesentery attachment, appendicectomy, and replace the bowel in the right location.
žWhere there is necrotic bowel second laparotomy performed in 24-48hrs later to see if viable gut can be saved – resection 

39

What are your differentials for vomiting in a paediatric case?


Gastro
FAMINE HIP GAIT
žNeurological
Increased ICP
žInfectious
Gastroenteritis
Meningitis
Pertussis (post tussive cough)
žEndocrinological
DKA
Errors of Inborn Metabolism
žPsychogenic / Behavioural
Overfeeding in infants
Bulaemia in adolescents
Pregnancy in adolescents
 

40

What are the GIT causes of vomiting in a paeds case?

FAMINEHIPGAIT

41

What can you do for supportive therapy in a child with a respiratory infection?


Oxygen therapy if hypoxic
Assisted ventilation if increased work of breathing
NG feeding if poor feeding
NG or IV fluids if dehydrated
 

42

What are the causes of stridor in a child?

ABCDEFGH

43

What is the generic management of a bowel obstruction in a child, let's say a 7yo girl?

Basics

DRSABCD

NBM

Place and person
™™Transport to surgical unit via NETS
Ix

ECG, ABG, BGL

FBE, UEC, LFTs, LDH, Coags, Group and hold

AXR, CT, USS

Definitive

  • Drip and suck-
    • –NBM and NG tube suction - relieves any respiratory distress from abdominal distension (may also be caused by vomit aspiration)
    • –Rapid IVF resuscitation with boluses of 10mL/kg given at 15 minute intervals if shocked otherwise give maintenance and mL by mL match for NGT losses. 
      • –Glucose- monitor BSLs and glucose solution should be given with IVF (glucose stores low, no absorption --> anaerobic metabolism)
  • –Correct Hypothermia
    • monitor temperature and have overhead heater
  • Correct acidosis

    correction of fluid loss, hypothermia and glucose will correct acidosis

  • Sepsis
    • if at risk administer IV Abx after cultures taken
  • ™Surgical correction if required- according to cause

44

What are the internationally recognised warning signs of autism in an infant?

45

What is the acronym for interviewing a teenager?

Plus also ask about sleep

46

What are the screening questions for eating disorder?

SCOFF

Do you make yourself Sick because you feel uncomfortably full?

Do you worry you have lost Control over how much you eat?

Have you recently lost more than One stone (6.35kg) in a three month period?

Do you believe yourself to be Fat when others say you are thin?

Would you say Food dominates your life? 

 

Plus to screen for bulimia nervosa:

Are you satisfied with your eating pattern?

Do you ever eat in secret? 

47

What are the complications of eating disorders in paeds?

The 5 Hs

  1. Height (growth retardation)

  2. Heart (arrythmia, QT, bradycardia, hypotension)

  3. Happy Bones (irreversible osteoporosis)

  4. Hormones (osteporosis, hypothyroid, delayed puberty/amenorrheoa)

  5. Heads (poor concentration, slowed mentation)

48

What are the red flags for abuse in an injured child?


History provided not consistent with injury
Delay in presentation
Claims that injury caused by young sibling
Denial that it is an injury
Multiple injuries of different types and ages
Injuries not consistent with developmental age
Child brought in by person other than parent
Shaken baby syndrome --> subdural haematoma + retinal haemorrhage

49

What are the normal nap patterns for babies?

50

How do you take a sleep history from a child?

51

What are the general considerations in approaching epilepsy in a child

Unless it is catastrophic epilepsy, early treatment does not change long term outcome

Be hesitant to make a diagnosis because it will change a child's life forever

Epilepsy will declare itself by progression or resolution. 

52

What is the most important feature of headache in determining aetiology and how would you interpret it?

Time course

Red is raised ICP

Green is migraine

Blue is muscular

Severity

Best to ask what they did during the headache to determine severity (confined to dark room = severe)

 

53

Apart from headache what are the symptoms of migraine in a child?

Relief by sleep

Dizziness/pallor

Abdo pain

Nausea and vomiting

Prodrome

Characteristic location of pain

54

What are the most common causes of URTIs?

Rhinovirus

Adenovirus

Influenza and parainfluenza

RSV

Enterovirus

EBV

Streptococci

55

What differentials should you always consider for a febrile child?

 

  • ear, nose, or throat infection
  • acute respiratory infection
  • gastroenteritis
  • urinary tract infection
  • meningitis
  • osteomyelitis / septic arthritis

56

Baby/child with a CRP > 80, what do you think?

Bacterial infection

57

What do you need to consider about the utility of urine dipstick in a child?

Urine dipstick not useful in babies under 1 because they pee as soon as urine created which means not enough time for bacteria/WCC to accumulate

 

Can cause false negative!

58

What are the differentials for fever + blotchy/spotty rash in a child?

MR SARS EEK!

 

  • Measles
  • Rubella
  • Systemic JCA (Still’s disease)
  • Allergy
  • Roseola infantum (HHV6)
  • Scarlet fever
  • Erythema infectiosum (Parvovirus B19)
  • Erythema multiforme
  • Kawasaki disease

59

What are the complications of Kawasaki disease?

Mild, diffuse dilatation of coronary arteries usually begins on day 10 from fever onset. If left untreated, 25% of these patients progress to true aneurysms, with 1% becoming ‘giant aneurysms’ (>8 mm internal diameter). These giant aneurysms have the worst prognosis, with risk of acute thrombosis and long-term risk of myocardial ischaemia

  • Carditis during the febrile phase
    • myocarditis with ST-T changes (25%)
    • pericardial effusions (20-40%)
    • valvular dysfunction (1-2%)
    • cardiac failure (~5%)
  • Coronary vessel abnormalities (occur in 20% of cases if untreated and
  • aneurysm formation
    • may lead to fatalities from
      • thrombosis,
      • rupture or
      • ischemia-related dysrhythmia 

60

What is Scarlet fever?

Rash from strep throat.

Tx with phenoxymethylpenicillin (PO) or benzathine penicillin (IM) to prevent post strep GN or acute rheumatic fever

61

What are the differentials for fever + vesicular rash in a child?

Hand, foot and mouth

Chickenpox

Herpes simplex

 

(Remember: vesicular just means blistering)

62

What are the signs/symptoms of meningitis in a child?

Fever

Stiff neck*/Kernig's sign

Bulging fontanelle

Irritable

Drowsiness

Poor feeding/vomiting

Palpable non-blanching purpuric rash => meningococcal meningitis

Convulsions/coma/shock --> consider meningitis

Stiff neck is not relevant in infants

63

What are the different patterns of CSF results in a child with viral/bacterial/TB meningitis?

64

What are the acute and long term complications of meningitis?

65

What is the relationship between immune development and prematurity?


Transplacental transfer of IgG happens in the third trimester. Very premature babies can miss out on this process rendering them very vulnerable to infection


NB: The process is actually active and means that the baby ends up with a higher concentration of IgG than their mum!
 

66

What would be considered febrile in a child younger than 3 months?

What would you do with a child 2.5 months old with T38.2 degrees

> 38.0 degrees

INVESTIGATE IT!

FBE

blood culture

CRP

Urine dipstick/Urine MCS

CXR only if respiratory signs are present

stool culture, if diarrhoea is present

67

What is bronchiolitis and what age kids does it affect?

Viral lower respiratory tract infection

Up to 18 months

68

What pathogens cause bronchiolitis?

RSV

Picornovirus

Human metapneumovirus

Adenovirus --> bad because causes bronchiolitic obliterans!

Parainfluenza

Influenza

 

69

What are the risk factors for bronchiolitis?

Exposure to cigarette smoke
Maternal smoking during pregnancy
Prematurity

Chronic cardiorespiratory diseases (including congenital heart disease and cystic fibrosis)
Immunodeficiency

70

What investigations are required for bronchiolitis?

None, clinical diagnosis

However, nasopharyngeal aspirate is usually done because we like to know which viruses are doing the rounds in the community and to rule out pertussis (which is life threatening to infants)

71

What is the management of bronchiolitis?

Basics

Oxygen therapy if hypoxic​

Person and Place

 

Criteria for admission:

  • <50% normal oral intake
  • Sats <93%
  • Parental anxiety
  • Not yet day 2/3 and expected to worsen

Investigations and Diagnosis

Clinical diagnosis, NPA if required to isolate virus or exclude pertussis

Definitive Management

  • NG feeds
  • IV fluids
  • O2 support
    • Low or high flow nasal prongs
    • CPAP
    • Intubation

Long-Term Management

Usually resolves after a week

RSV is a highly infectious virus, ensure good hand hygiene to prevent spread.

Ensure immunizations are up to date

72

What are the differentials for wheeze in a young child?

Bronchiolitis / bronchitis

Asthma / viral induced wheeze

Viral pneumonitis

Croup (can cause wheeze)

Pneumonia

Pertussis

Bronchiomalacia/tracheomalacia

Cardiac failure

CF or other suppurative lung dz

Recurrent aspiration

Inhaled foreign body

73

What ages does croup typically occur?

6 months to 12 years of age, with a peak incidence at age 2

74

What is the significance of hypoxia in croup?

If you're thinking about hypoxia the croup is life threatening.

Adequate SPO2 should not be reassuring – low oxygen saturations is a BAD sign.

75

What pathogens cause croup?

Parainfluenza

Influenza

Adenovirus

76

What is the clinical presentation/natural history of croup?

  • Begins with coryzal symptoms for 1-2 days
  • Fever is generally low grade (38-39) – no signs of toxicity
  • Barking cough – usually begins at night or early hours of morning.  Peaks at day 2 and 3 of disease
    • It takes two or three days before the sub-glottic area narrows enough to cause the barking cough
    • It happens first at night, usually, because we have reduced upper airway tone when we sleep
  • Inspiratory stridor is heard as the disease progresses
  • The viral illness typically lasts 7-10 days, but the typical croupy cough only lasts 2-3 nights
  • May have associated widespread wheeze, increased work of breathing and hypoxia – child may become distressed and restless, cyanosis is a late and ominous sign
  • Recurrent spasmodic croup
    • ‘Upper airway hyperresponsiveness’
    • No viral prodrome
    • Children are well when they go to bed, and wake up in the early hours of the morning with a barking cough and stridor
    • Fever is unusual in this form of croup
    • Children often have asthma or atopy, or a FHx of asthma and atopy

77

What are the investigations for croup?

None, dont distress the kid!

78

How do you differentiate croup from epiglottitis?

79

What is the management of croup?

Basics

Minimal handling
Oxygen is not usually required unless severe obstruction
IV access should be deferred

Place and person

Grade 1 – manage at home

Grade 2 – admit to hospital via ED

Grade 3 – admit to ICU

 

If having to administer oxygen, contact anesthetics!

If having to administer a second dose of adrenaline, contact anaethetics!

Investigate and confirm diagnosis

Clinical diagnosis

Non-invasive management

Keep child relaxed

Definitive management

Mild to moderate

Oral prednisolone 1mg/kg
OR Oral dexamethasone 0.15mg/kg – 0.6mg/kg

Severe

  • Oxygen
  • Nebulised adrenaline 1:1000 solution
    • Works by vasoconstriction
    • Beware of possible rebound effect after 2-3 hours – must observe
    • This is more symptomatic treatment to buy you time
  • Dexamethasone IV 0.2mg/kg
  • Follow with oral steroids
  • ETT if going into respiratory failure

Long term

Consider transfer when no improvement following nebulized adrenaline

Discharge requirements

Four hours post nebulized adrenaline
Half hour post oral steroid
Stridor free at rest

Recurrent-Spasmodic Croup

Usually don’t require treatment as attacks are very short-lasting. But can treat the same as acute viral croup. Some evidence to suggest that ICS may reduce frequency of attacks, but children will most likely grow out of this.

80

Who does pertussis affect?

Most common age group in community is adolescents and young adults, but not dangerous for this group except for being vector

 Under 12 months deadly

Under 6 months VERY deadly

81

Discuss the transmission of pertussis?

Transmission: Direct contact with droplet discharges

Highly contagious – spreads to 80-90% of susceptible individuals coming in contact with disease

82

Describe the natural history of pertussis?

Catarrhal stage (7-14 days)

  • Anorexia
  • Rhinnorrhoea
  • Conjunctivitis/lacrimation
  • Dry cough

Paroxysmal stage (about 4 weeks)

  • Paroxysms of severe coughing with inspiratory ‘whoop’
  • Vomiting (after coughing)
  • Coughing mainly at night
  • Lymphocytosis

Convalescent stage

  • Chronic cough which may last for weeks 

83

What tests do you use for pertussis?

FBE --> lymphocytosis

CXR to exclude pneumonia

Culture and/or PCR of nasopharygeal aspirate (within 1 week from onset of cough). Both of these tests have 70% sensitivity

84

What is the management of pertussis?

Basics

ABC

Place and person

If younger than 6 months – hospitalization

School exclusion until at least 5 days of antibiotic use

Investigate and confirm diagnosis

Nasopharygeal aspirate

FBE, IgA serology

CXR – to exclude pneumonia

Non-invasive management

Good ventilation

Avoid emotional excitement or distress

Avoid overfeeding during paroxysmal stage

Definitive management

  • Pertussis infection (Group B disease) requires written notification within five days of diagnosis.
  • Antibiotics minimize transmission but do not effect course of disease
  • Azithromycin 500 mg 5 days OR
  • Clarithromycin 7 days

Prophylaxis

Same treatment as above for household and other close contacts if commenced within 3 weeks of onset of cough in the patient

Prevention

  • Acellular pertussis is part of the immunization schedule
  • Initial protection
    • 2, 4 and 6months
  • Booster (dTpa)
    • 4 years
    • 10-15 years
    • Parents or household contacts of newborns 

85

What are the complications of pertussis?

Pneumonia
Atelectasis
Pneumothorax
Seizure
Asphyxia
Apnoea/bradycardia
Rib fracture
Encephalopathy
Otitis media 

86

What is the treatment for infrequent episodic asthma?

SABA PRN only, NO ROLE FOR PREVENTER IT IS NOT EFFICACIOUS

87

What is the stepwise pharmacological management of childhood asthma?


—Step 1: RELIEVER
—SABA


—Step 2: PREVENTOR
—Low dose ICS (200 – 400mcg / day)
—OR LTRA
—OR inhaled cromone


—Step 3: ADD OR INCREASE STEROID
—Increase ICS (Double: 400 – 800mcg / day)
—LTRA / inhaled cromone + low dose ICS


—Step 4: ALL PREVENTORS AND HIGHEST ICS DOSE
—LTRA / cromone + ICS (800mcg)


—Step 5: ADD SYMPTOM CONTROLLER / ORAL STEROIDS
—LABA (not in children  

88

What is the correct definition of asthma?

Rrecurrent wheezing episodes which are responsive to salbutamol.

89

Is asthma more prevalent in males or females?

Boys generally have smaller airways than girls and tend to suffer more from asthma. However, after adolescence the prevalence is higher in females.

90

What are the side effects of inhaled corticosteroids in children?

Very safe, almost not clinically significant long term side effects

Hoarseness and pharyngeal candidiasis are not common

Treatment beginning before puberty is associated with a small (mean approximately 1 cm) reduction in adult height

91

What is the genetic inheritance of cystic fibrosis?

autosomal recessive

92

What are the clinical manifestations of cystic fibrosis?

93

What are the complications of cystic fibrosis?

MR PANCREAS

M - Meconium ileus (at birth) and distal intestinal    obstruction (children and adults)

R - Recurrent infections

P - Pancreatic failure (exocrine and endocrine)

A - Allergic bronchopulmonary aspergillosis

N - nasal polyps and recurrent sinusitis

C - Cirrhosis of hepatobiliary system

E - Emotions (teenagers often depressed) and electrolyte disturbances (low Na+)

A - Airway leaks (pneumothorax)

S - Sterility in males

94

What is the management of cystic fibrosis?

Basics

NA

Place and person

Referral to

  • Respiratory physician
  • Chest physiotherapy
  • Dietitian

Investigate and confirm diagnosis

Sweat test and genotyping

Non-invasive management

  • Chest physiotherapy
  • Monitoring and optimizing nutrition
    • High intake of dietary fat
    • Pancreatic enzyme supplements
    • Fat soluble Vitamin supplements  

Definitive management

  • For respiratory infections
    • S. aureus infection
      • Di/flucloxacillin
    • S. aureus and Haemophilus influenzae infection
      • Augmentum or trimethoprim and sulfamethoxazole
    • Initial pseudomonal infection
      • Ciprofloxacin PLUS
      • Tobramycin by nebulizer OR
      • Colistimethate sodium by nebulizer
    • Chronic pseudomonal exacerbation
      • Ciprofloxacin PLUS
      • Tobramycin by nebulizer OR
      • Colistimethate sodium by nebulizer OR
      • Gentamicin by nebulizer

Long term

  • Respiratory
    • Daily chest physiotherapy
    • Aerosolised mucolytics
      • Dornase alfa
      • Nebulised hypertonic saline
  • Inhaled bronchodilators
  • Inhaled corticosteroids

Chronic pseudomonal infection

Azithromycin orally 3 times weekly  

GORD

PPI

95

What is this and what is the treatment?

Exomphalos is a type of abdominal wall defect. It occurs when a child’s abdomen does not develop fully while in the womb. 

Tx = surgical correction in first 2 days of life

96

This baby has just been born, what is the diagnosis and management?

Hydrocele. It will self resolve. But you must make sure to rule out testicular torsion.

97

What is this? What is the management?

Gastroschisis

Urgent surgical repair

98

What is this? Is it always abnormal?

Phimosis (the foreskin cannot be fully retracted over the glans penis). Most kids born with non-retractile foreskin. Normal up until puberty. If not loosening by age 4 prescribe steroid cream to promote loosening of the skin.

99

What is this? What is the management?

Paraphimosis (the foreskin becomes trapped behind the glans penis, and cannot be reduced).

 

Management:

Apply local or general anaesthetic, compress the oedema (may have to apply pressure for >5 mins) to drain the fluid. If that fails contact paeds surg to puncture the skin with a needle and aspiration fluid. Then replace the foreskin.

 

 

100

What are the causes of cyanotic heart disease in infants?

1,2,3,4,5 T’s
1 vessels (Truncus arteriosus)
2 vessels swap (Transposition of the Great Arteries)
3 = Tricuspid atresia
4 = Tetralogy of Fallot
5 words (Total Anomalous Pulmonary Venous Return)

101

What are the causes of acyanotic heart disease in kids?

102

You diagnose a child with a septal defect (either atrial or ventricular), what is the physiology of the condition and what is your initial management?

Septal defects cause L --> R shunts

 

ACEi, diuretics to reduce preload and strain on right heart plus feeding to increase baby's weight ready for surgical correction

103

How is a patent ductus arteriosis usually picked up?

 

What is the problem with a patent ductus arteriosis?

Identified if infant experiences heart failure, or later in life because doctor hears machinery murmur or notices wide pulse pressure (caused by the run-off of blood flow from the aorta to the pulmonary artery during diastole).

What is the problem?

Blood flows from the high pressure aorta into the low pressure pulmonary artery, overloading pulmonary circulation and causing pulmonary hypertension and heart failure (dyspeneoa, FTT, recurrent infections). If not picked up until adulthood pulmonary hypertension can be a significant issue.

Children with a small PDA are often asymptomatic, but may develop Infective Endocarditis.

104

You hear a machinery murmur in a 3 month old, what investigations would you do?

Bedside tests

ECG – normal with small PDAs. 

Left ventricular hypertrophy with larger PDAs

Bloods and urine

Nil

Imaging

CXR – full pulmonary artery silhouette and increased pulmonary vascularity.  Cardiomegaly may be present in a larger shunt.

 

Echocardiography – visualise the PDA to confirm diagnosis

Special tests

Nil

105

What is the management of patent ductus arteriosis?

Premature infants

  • IV indomethacin in patients with very low birth weight
  • IV indomethacin or ibuprofen of premature infants of larger weight
  • Treatment can be given before evidence of a PDA becomes evident
  • Prophylactic indomethacin might increase ductal closure and decrease the incidence of intraventicular haemorrhage

Term infants

  • In asymptomatic or symptomatic patients, percutaneous catheter closure is the first-line treatment in order to treat heart failure and prevent elevated pulmonary pressures or endarteritis.
  • Infants who are symptomatic and too small to undergo percutaneous device closure need ACEi/diuretic treatment and feeding until they are large enough for surgical management

106

Aortic coarctation


When does it present?

How does it present?

What is the management?

When does it present?
 If severe: acute circulatory collapse at 2 days of life after being previously well (duct closure). This is the usual scenario.
If mild: Later in life, often discovered when pt is being investigated for refractory hypertension. The later the abnormality is discovered the harder the HTN is to treat.
How does it present?
Sick baby

Hypertension

Unequal BP between arms

Absent femoral pulses

Severe metabolic acidosis

May have an ejection systolic murmur between the shoulder blades

MGMT?
ABC, prostaglandins, urgent surgery
 

107

What is the management of hypoplastic left heart syndrome?


The Norwood operation, which connects the aorta and the returning pulmonary veins to the R heart – a one chamber heart!

 

Usually receives follow up operations at 3 months and 6 years

108

How does blood flow typically work in infants with interruption of the aortic arch?

What is the management of this condition?

In the immediate newborn period blood flows through the 'Ductus' into the Descending Aorta and hence reaches the lower part of the circulation.

Fortunately these babies usually have a VSD, meaning that the blood that flows through the pulmonary artery, though the ductus and into the descending aorta is at least partly oxygenated.

As the ductus closes after birth blood pressure in the lower circulation becomes inadequate and severe symptoms develop.

Most affected infants develop severe symptoms (difficulty breathing and impaired kidney function) in the first week of life and need urgent surgery

 

Mx = ABC, prostaglandins, surgery in days

109

What is the clinical presentation of hypoplastic left heart syndrome?

Presents early in life (usually D2 when duct closes) as the “shocked neonate” – dusky colour, weak or absent pulses, low BP, reduced UO, metabolic acidosis

110

Obstructive heart diseases in neonates are usually XXX which means they often present XXXXX when XXXX happens.....

duct dependent

In the first week of life

When the ductus closes

111

Describe the murmur of VSD

Small VSD – high pitched pan-systolic murmur, associated with a thrill

 

Large VSD – Quiet pansystolic murmur, heave

112

What is the most common heart defect?

 

What is the most common heart defect in babies with downs syndrome?

VSD in both

113

What is the management of VSD?

Small --> conservative management and monitoring

 

Large --> surgical repair

114

What are the forms of ASD and which form is most common?

Secundem (most common)

 

Primium (AVSD)

115

What is the management of ASDs?

Most affected children are free of any major symptoms, but the risk of heart failure developing later in life makes closure desirable, unless the defect is very small. These defects may be repaired surgically or by using an expanding plug ("Device"), which can be inserted through a heart catheter without an operation (Device Closure)

116

What are the causes of cyanosis in the neonate?

OH CRAP THAT BABY IS BLUE!

DONT SWEAR IN FRONT OF THE BABY!

OH, I SAID, CRIP

Cyanotic Heart Disease (1,2,3,4,5 Ts)
Respiratory disease (Meconium aspiration syndrome, pulmonary hypoplasia)

Infection (GBS)
Persistent pulmonary HTN of the newborn (PPHN)

117

What is the workup (exam and investigations) of a cyanosed neonate?

118

What are the abnormalities in Tetralogy/Pentology of Fallot


Pulmonary stenosis
Right ventricular hypertrophy
Overriding aorta
VSD

Pentology --> ASD

119

What is the classical presentation of Tetralogy of Fallot?

Murmur, cyanosis or congestive heart failure, typically develops over the first 6-12 weeks of life

If mild disease not picked up until later childhood. Presentation at this stage is characterised by FFT, clubbing of the fingers, reduced exercise tolerance and ‘Tet spells’
 

120

What is the management of a Tet spell?


Comfort the child
Rest younger infants against the parents shoulders with knees tucked in
Encourage older children to squat
O2, IV fluids

Beta-blockade and ICU support may be required
IM morphine can reduce pain and venous return

Phenylephrine is last line*

 

*Used to increase systemic venous resistance by direct simulation of peripheral alpha-adrenergic receptors.

The goal is to increase the systemic vascular resistance above the resistance of the pulmonary outflow tract so that blood in the right ventricle preferentially flows through the pulmonary circulation, rather than across the ventricular septal defect and into the systemic circulation.

121

What are the complications of Tetralogy of Fallot?


Heart failure is rare. But cyanosis --> polycythemia --> thromboembolism can occur. Infective endocarditis and cerebral abscess too!
 

122

What genetic abnormality is associated with TOF? What are the other features of this genetic condition?

CATCH 22q11 (Di George Syndrome)

Tetralogy of Fallot is famously associated with the deletion of the long arm chromosome 22
Cardiac anomalies (tetra, transpos)
Abnormal facies
Thymic hypolasia (T cell deficiency)
Cleft palate
Hypocalcaemia/hypoparathyroidism
 

123

What is the management of transposition of the great arteries?

Prostaglandins

Atrial baloon septostomy

Arterial switch is definitive surgical correction at 6-9 months

124

What ECG findings are there in TOF?

What murmur is heard in TOF?

Right ventricular hypertrophy

 

Coarse ES murmur on ULSE

125

What do you know about pulmonary atresia?

  • This defect is a form of Tetralogy of Fallot
  • Blood cannot enter the pulmonary circulation via the pulmonary valve.
  • Blood entering the right ventricle moves into the left ventricle via an ASD/VSD or foramen ovale, is pumped via the left ventricle and then shunts back to the pulmonary circulation via the PDA.
  • Survival depends on the ductus remaining open in the early days of life
  • Cyanotic heart disease
  • Surgical correction with a shunt is required immediately, with complete repair performed after age 1.
     

126

What do you know about truncus arteriosis?

A single ventricular outflow tract straddles a large VSD.

It has an abnormal valve.

Presents in newborn period with mild cyanosis and congestive heart failure.
The abnormal valve is often stenosed (systolic murmur) and may be regurgitant (diastolic murmur).

Urgent surgery

127

You examine a 2 year old and find:

Fixed splitting of S2

May have murmur at the upper left sternal edge

What is the diagnosis and management?

ASD

If symptomatic or causing R ventricular dilation, catheter closure at 3-5 years

Partial AVSD needs surgery at 3 years

Complete AVSD: 3-5 months

128

You examine a 2 month old and find:

Loud, pansystolic murmur with a thrill (in a patient with no symptoms)

What is the management?

Small VSD

Usually no treatment required, spontaneous closure

129

You examine a 2 month old and find:

Softer pansystolic murmur with a loud P2. R sided heave and heart failure. Cardiomegaly on CXR

What is the diagnosis, what is the manamagement?

Large VSD

Diuretics, captopril

Extra calories

Surgery at 3-6 months

130

Explain the splitting of the second heart sound

  • Normally, the pulmonary valve closes a little after the aortic (A2, P2).
  • During inspiration, venous return in increased at the R ventricle takes a little longer to empty.
  • Blood pools in the lower pressures of the pulmonary circuit, reducing return to the left heart, which now finishes emptying a little sooner.
  • Therefore, the split normally widens on inspiration
  • During expiration, the right heart is given the chance to catch up a little and the split is narrowed
     

131

What is the management of VSDs?

  • Stabilise with diuretics, ACEIs and high caloric diet
  • Treat if:
    • Heart failure or failure to thrive on medical management
    • Pulmonary hypertension
    • Aortic valve prolapse
    • Infective endocarditis
  • Surgical and trans-catheter options exist

132

What would you find on investigations of a VSD?

 

  • Small VSD may have no abnormal findings
  • Larger VSDs may demonstrate cardiomegaly and pulmonary plethora of CXR
  • Biventricular hypertrophy can be shown on ECG
  • Diagnose with echo
     

133

What is the relationship between large VSDs and the aortic valve?


High flow VSDs can suck in the nearby aortic leaflet by the Venturi effect and cause aortic regurgitation
 

134

What are the differentials for a shocked neonate?

What are the differentials for a cyanosed neonate?

What are the differentials for infants with heart failure?

135

What do you check on a paediatric cardio exam?

136

Which congenital heart defects increase the risk of infective endocarditis?

Essentially all congenital heart abnormalities increase your risk, except for secundum ASDs

137

8 year old with pallor and bone pain, what is your spot diagnosis?

ALL until proven otherwise

138

What is the main risk you worry about in immune thrombocytopaenic purpura and how common is it?

Intracranial haemorrhage - 1% of ITP cases

139

What are the features of anaemia in a child?

  • Pallor – conjunctival, palmar crease
  • Fatigue, listlessness
  • Poor growth
  • Flow murmur
  • Air hunger (severe)
  • Hypotension and cardiac failure (severe)
  • Iron deficiency can present with poor attention span, irritability and pica (eating dirt, clay, ice etc)

140

What are the causes of iron deficiency in a child?

Malabsorption and occult blood loss (although rare) should be considered, but usually after failure of dietary measures.

Polyps, haemangiomas, Meckel’s diverticula and hereditary telangectiasia are rare causes of GI blood loss. 

141

What are the differentials for normocytic anaemia in a child?

142

What are the differentials for microcytic anaemia in a child?

143

What is the haematological aspect of parvovirus B19?

 One of the most common and serious complications of parvovirus B19 infection is transient aplastic crises in patients with chronic haemolytic anaemia such as sickle cell disease and hereditary spherocytosis. Pure red cell aplasia may also develop with a persistent infection of parvovirus B19 in immunocompromised individuals.

144

A five year old presents with pallor, lethargy, difficulty concentrating worsening over the past few months. What investigations will you order?

FBE

  Blood film

 Ferritin

  Reticulocyte count

145

You diagnose an 8 year old girl with Fe deficiency anaemia, what is your management advice to the parents?

146

What is the presentation of factor dysfunction vs platelet dysfunction?

147

A 6 year old child presents with petechiae, what are your differentials?

SHIELD

148

A 5 year old girl has an isolated raised APTT, what are the possible causes?

A raised APTT in the absence of anticoagulant therapy suggests a deficiency in a factor or an inhibitor.

An inhibitor is usually an antibody made against a factor, such as lupus anticoagulant or antibodies formed by haemophilia patients receiving recombinant factors

 

149

What is the peak incidence of immune thrombocytopaenic purpura in children?

2-5 years

150

What is the classical presentation of ITP?

What do you find on examination?

What do you find on investigations?

Presentation
Clinical manifestations of platelet disorder develop acutely – mucosal and skin petechiae, epistaxis, rectal bleeding, haematuria, bleeding gums

Examination

Normal examination – no pallor, no lymphadenopathy or hepato-/spleno- megaly (exclude leukaemia)

​Investigations

FBE --> thrombocytopaenia, blood film shows isolated low platelets with no other findings

A bone marrow aspirate is an invasive procedure with some morbidity in children who bruise easily, and is only necessary if the diagnosis is uncertain. It is rarely necessary in uncomplicated ITP.

 


ITP is a diagnosis of exclusion, but an FBE and blood film may be sufficient for diagnosis if Hx and Ex consistent.
It can be thought of as isolated thrombocytopenia with no other suggestive findings on Hx, Ex, FBE or blood film. 

 

151

In ITP what level of platelets makes you worry?

Platelets consistently under 10

152

How long does ITP typically take to resolve in kids with conservative or active treatment?

Untreated 75% in 6 weeks, 90% will resovle within 6 months

With standard dose steroids 1 week

With high dose steroids 2-4 days

With IVIG 1-3 days

153

What are the stepwise treatment options for ITP? What advice do you give the parents?

 

  • Safety precautions are key
    • Allow for generous supervision, especially of young children
    • Avoid contact sports or any moderate-impact activity
    • Regular follow up
    • Parents must receive thorough education
    • Avoid NSAIDs and 5-ASA

 

Splenectomy is a last line option. Often very effective but vaccinations and life-long antibiotic prophylaxis required. 
 

154

What is the typical presentation of haemophilia?

Neonates

  • Often picked up on screening due to FMHx
  • Cord blood can be used for genetics and coagulation studies
  • Intracranial haemorrhage, severe birth bruising and excessive bleeding after circumcision can suggest the disese


Older children

  • Haemarthroses tend to occur in walking children


 

155

What is the management of haemophilia?

 

  • Mild-moderate haemophilia A can be treated with DDAVP after appropriate challenge test (>5y/o)
  • Not adequate for major bleeding
  • Tranexamic acid can reduce mucosal bleeds and epistaxis
  • Severe disease can be treated with 25-40 units/kg of recombinant factor concentrates three times a week. 

156

What are the clinical features of leukaemia?

157

Auer rods = ?

AML (less common than ALL)

158

What is the initial work up of a child with suspected leukaemia?

 

  • CXR is used to investigate the possibility of mediastinal masses (occurs in 50% of T-ALL).
  • Lumbar punctures are mandatory to detect occult CNS infiltration. They are often delayed until treatment has begun to reduce the risk of seeding into CNS. Also because often thrombocytopenic 
     

Many more tests are done. This is the realm of a paediatric oncologist. Tumour lysis bloods, liver and renal function, lactate dehydrogenase and coagulation studies all form part of the initial work-up at baseline.

159

What is the cure rate of ALL?

What are the general principles of chemotherapy approaches to ALL?

What steps are taken to reduce the chances of tumour lysis syndrome?

It is commonly quoted that cure rates (in children) are 80%. The current rate is probably higher, especially in low risk disease.

Remission Induction

  • Aims to eliminate 99% of the tumour cell population. This should achieve normal haematopoiesis and functional status.
  • Does the ‘heavy lifting’ --> remaining treatment is about total eradication, relapse prevention and cure
  • High risk of tumour lysis in early chemo. 
    • the breakdown products of dying cells causes hyperkalaemia, hyperphosphataemia (which causes hypocalcaemia), high blood uric acid and high urine uric acid and consequent acute uric acid nephropathy and acute kidney failure.

    • Tumour lysis prevention:

      • bicarb to alkylise urine and prevent uric acid crystal formation in kidney

      • allopurinol

      • fluid diuresis

      • monitor all electrolytes

CNS protection

  • Most chemo penetrates the BBB poorly
  • Intrathecal and IV methotrexate can obviate the need for cranial irradiation (CI). CI is still used for overt CNS disease or high risk patients.

160

A child presents with petechiae on their lips, eyes and gums but nowhere else, what are your differentials?

Strangulation/NAI

Vomiting

Coughing

+ other causes of petechiae (SHIELD)

161

Why do kids with ITP and really low platelets not bleed as much as you’d think?

 Immature megakaryocytes in BM churning out platelets. The platelets are young/healthy/sticky. Vs kids with cancer with same low platelet count who will bleed because unhealthy platelets.

162

What are analgesic options for kids?

·      Oral – paracetemol, ibuprofen, oxycodone

·      Intranasal – fentanyl (device that makes a mist out of it)

·      IV – morphine, ketamine

·      IM – (rare) ketamine, morphine

·      Local anaesthetic

163

What things do you assess when examining for dehydration in a child?

  • Wet nappies
  • Tears
  • Fontanelles
  • Skin turgor
  • Mucus membranes
  • Cap refill
  • HR/BP
  • Weight
  • Vomits/diarrheoa
  • Alertness

164

Newborn with cough, cyanotic episodes with feeding, respiratory distress, recurrent pneumonia, what is the diagnosis?

Tracheo-oesophageal fistula

165

What are the differentials for projectile vomiting?

pyloric stenosis (if age 2-7wks)

duodenal atresia 

166

What is a SUFE?

How does it present, how is it diagnosed and what is the treatment?

A 'Slipped Upper Femoral Epiphysis' (also called a 'SUFE') is when the femoral epiphyseal plate  is weak and the ball head of the femur slips downward and backward. The exact cause of this condition is not known. 

A 'SUFE' is not usually associated with an injury.  The symptoms often develop slowly - over several months - and may seem like a pulled muscle in the hip, thigh or knee.  It is important to get an early diagnosis and treatment before the slip gets worse and children may need to have the unaffected side treated as well to prevent future slipping.

Clasically happens to obese teenage boys, can be bilateral

True hip pathology causes GROIN pain!

 

Signs and symptoms

Groin, hip, thigh or knee pain. (Some people only have knee pain, even though the condition affects the hip)
Limp
Limited movement of the hip joint
There may be slight shortening of the affected leg.

Diagnosis

Diagnosis is usually made by physical examination of hip movement and an x-ray.

Attached X ray shows how in the abnormal side the line does not cut off the femoral head like it should 

Management

Surgical correction with screw

167

What features differentiate septic arthritis from post viral synovitis?

Refusal to weight bear

Fever (>38.5)

Raised WBC

ESR >40

CRP >20

168

At what age does crying peak?

6 weeks, it gets better after that

169

A child in brought in to the ED for some totally benign condition (like an URTI). You need to give the parents advice on what situations should prompt them to bring the child back in to ED, what do you tell them.

Bring them back if:

  1. They become dehydrated (because of vomiting/diarrheoa) with poor oral intake and no wet nappies
  2. Become drowsy or not responsive to interactions
  3. Puffing very hard or finding it difficult to breathe
  4. Uncontrollably irritable, not able to be soothed

170

You are trying to find a localising source of a young child's fever. They have a history of vomiting, what does that tell you?

Nothing, vomiting is not a localising sign in a kid. Could be anything from UTI to meningitis.

171

A child presents to ED with a fever, you don't think it's serious. What conditions do you need to rule out before sending them home?

Meningitis

Pneumonia

UTI

Sepsis

172

What are the diagnostic criteria for febrile convulsions?

What additional criteria apply to simple vs complex febrile convulsions?

Febrile convulsions / seizures are seizures in which

  • Fever >38 degrees

  • No evidence of CNS infection

  • No history of afebrile seizures

  • No history of neurological deficits or developmental delay

Simple febrile seizures are

  • generalized tonic and/or clonic seizures
  • Short in duration
  • Do not recur within the same febrile illness

Complex febrile seizures are

  • focal features at onset or during the seizure
  • duration of more than 15 minutes
  • Recurrence within the same febrile illness
  • Incomplete recovery within 1 hour.

173

At what age do febrile convulsions typically occur?

6 months to 6 years

174

How common are febrile convulsions?

3 in 100 babies

175

What investigations should you order for a febrile convulsion?

Bedside tests

  • BGL – this is the only investigation you should do if the child is >6 months and the source of the fever can be identified
  • Urine dipstick and MC&S

Bloods and urine

  • FBE
  • CRP
  • Blood culture
  • UEC / CMP
    • check for electrolyte derangement.
    • Feeding babies water bottles causes hyponatraemic seizures

Imaging

CXR if respiratory symptoms

Special tests

LP – low threshold if unknown source of fever.

From RCH “consider LP if the child is less than 12 months and not up to date with immunisations, if they are clinically unwell, or if they are already on oral antibiotics that may mask meningitis.”

176

What can you tell the parents of a child who has just had a febrile convulsion about his/her prognosis?

  • Recurrence rate depends on the age of the child;
    • the younger the child at the time of the initial convulsion, the greater the risk a further febrile convulsion
      • (1 year old 50%; 2 years old 30%).
  • Risk of future afebrile convulsions (epilepsy) is increased by:
    • family history of epilepsy,
    • any neurodevelopmental problem,
    • atypical febrile convulsions (prolonged or focal).
    • With none of these risk factors risk of recurrence is similar to the rest of the population (1%). With 1 risk factor, risk is 2%, with >1 risk factor, 10%. 

177

The parents of a child who has just had a febrile seizure ask about what they can do to prevent it happening again, what do you tell them?

  • Fever control:
    • minimal clothing, nappy alone or light outer layer depending on ambient temperature
  • Tepid sponging, baths and fans are ineffective at lowering core temperature and are NOT recommended
  • Paracetamol has not been shown to reduce the risk of further febrile convulsions. It may be used for pain/discomfort associated with febrile illnesses (e.g otitis media),it should not be solely used to control fever
  • If the seizure recurs, roll him onto his side and allow him to fit, Seizure should not last longer than 3-5 minutes. Any longer and an ambulance should be called.
  • If Henry was to have recurrent or prolonged febrile seizures, prophylactic oral diazepam or rectal diazepam (rescue therapy) may be prescribed 

178

In hospital, how long would you allow a febrile convulsion to continue before commencing active management with benzos?

5-10mins

179

Is the response (or lack of response) of a child's fever to paracetemol significant?

No, not at all

180

Does teething cause fever?

Teething will not cause fever > 38.5oC
 

181

A parent brings a febrile child to you, what do you do on history and exam?

History

  • Headache (if child can talk)
  • Photophobia (if child can talk)
  • Cough
  • Coryza
  • Diarrheoa
  • Vomiting
  • Abdominal pain
  • Joint symptoms
  • Dysuria/foul smelling urine
  • Travel history
  • Sick contacts
  • Immunisations

Exam:

  • Vitals, general appearance, conscious state
  • Neck stiffness
  • ENT examination
  • Work of breathing
  • Abdominal exam
  • Skin rash
  • Joint swelling

182

What are the clinical indications for antibiotics in children with painful otitis media 

  • Sick child with fever
  • Vomiting
  • Red-yellow bulging TM
  • Discharge
  • Loss of TM landmarks
  • Persistent fever and pain after 3 days of conservative approach
  • Bilateral AOM

183

How common is Down's syndrome?

What increases the risk?

Birth incidence is about 1 in 1000 live births.

Overall incidence rises after maternal age of 35 years.

184

How common are cardiac conditions in babies with Down's syndrome?

50% have congenital heart defects

particularly AVSD

185

What is the risk of miscarriage associated with CVS vs amniocentesis?

CVS = 1% above the background risk

Amniocentesis = 0.5%

186

When in pregnancy do you perform CVS vs amniocentesis?

187

What are the advantages of CVS?

What are the advantages of amniocentesis?

188

This is a 3 year old child. What is the diagnosis?

What is the management?

What is the prognosis?

Diagnosis = herpes simplex gingivostomatitis

Management = Pain relief (codeine may be required as it is very sore). Antivirals inneffective

Prognosis =

Child may have a fever and become irritable, a day or two later ulcers develop on the lips and gums. These are painful and often lead to drooling and a refusal to eat or drink, dehydration can occur. The pain normally subsides after 3-4 days, the blisters usually take from 10 -14 days to go away. The blisters never leave scars.

189

A 4 month old child presents with a fever but no clear focus of infection. What is your management?

190

What are the different types of nephrotic syndrome in kids?

Idiopathic Nephrotic Syndrome (90% of presenting children)

  • Minimal change disease
  • Focal segmental glomerulosclerosis
  • Also called steroid-sensitive nephrotic syndrome as 80+90% of cases will respond to initial steroid therapy

Non-idiopathic Nephrotic Syndrome (rare)

  • Secondary
    • SLE
    • Henoch Schonlein Purpura
    • Membranoproliferative GN
    • Membranous nephropathy
    • Congenital nephrotic syndrome 

191

What is the prognosis of minimal change disease/focal segmental glomerulosclerosis?

  • 80% of children with Minimal change will relapse
  • Steroid-responsive patients have little risk of chronic renal failure
  • Patients with FSGS may initially respond to steroids but later develop resistance
  • Many children with FSGS progress to end-stage kidney failure
  • Recurrence of FSGS occurs in 30% of children who undergo renal transplantation 

192

What do you know about membranoproliferative glomerular nephropathy in kids?

What do you know about membranous glomerular nephropathy in kids?

Membranoproliferative glomerulonephritis

Characterised by hypocomplementemia (low C3 and C4) with signs of glomerular renal disease
5 to 15% of children with primary nephrotic syndrome
Typically persistent
Has a high likelihood of progression to renal failure over time

Membranous nephropathy

Less than 5% of children with primary NS
Seen most commonly in adolescents and children with systemic infections

Hep B, syphilis, malaria and toxoplasmosis

Or on specific medications

Gold, penicillamine 

193

What are the clinical features and management of testicular torsion?

Usually neonates or teenagers

Sudden onset testicular pain and swelling; occasionally nausea, vomiting.

Note: pain may be in the iliac fossa, may be associated with sports activity

Discolouration of scrotum; exquisitely tender and swollen testis, riding high. Cremasteric reflex is usually absent.

 

Management: Early surgical consultation is vital, as delay in scrotal exploration and detorsion of a torted testis will result in testicular infarction within 8-12 hours. Keep the child fasted.

194

What is the management of pulled elbow?

  • Perform a reduction manoeuvre (see below)
  • expect distress and pain
  • a click may be felt over the radial head
  • review after ten minutes
  • if reduction fails, consult with senior medical staff

https://youtu.be/-0ROu4hCXwQ

195

Colicky abdo pain, kid goes white when pain comes on, bilious vomiting

What is the diagnosis?

Intussusception 

196

At what age does intussusception typically occur? What typically causes it?


Primary – ages 3 months – 2 year olds

Ileocaecal location

Idiopathic
Secondary – ages 4 years – 12 year olds

Ileoileul location

Mesenteric lymph nodes

HSP --> pathological lead point of submucosal haemorrhage

Meckels diverticula

Haemophilia --> submucosal haemorrhage
 

197

What is the management of intussusception?

  1. Fluid resuscitation
  2. Analgesia
  3. Air enema
  4. Definitive surgery if necessary

198

What is the best way to describe intussusception to parents?

Like a sock folding in on itself

199

When is meconium normally passed?`

99% in first day

Rest in second day

If not passed on 3rd day it's a sign of abnormality

200

How do you differentiate between meconium ileus and hirschprungs?

In hirschprungs on rectal exam there is explosive discharge of flatus and faeces
 

201

What is the formal investigation to confirm hirschbrungs?

Suction rectal biopsy

202

A mother with a child with hirschprungs disease asks what the chances of her next baby having the condition, what do you say?

3-5%

203

What is the carrier rate for the cystic fibrosis gene in the Caucasian population?

What is the incidence in the genpop?

(This is frequently examined)

1/25

1/2500

204

What conditions are associated with duodenal atresia?

Cardiac defects

Down syndrome

205

How would you show an OSCE examiner that you had pyloric stenosis as a DDx in an OSCE?

Ask if either parent had pyloric stenosis when they were born.

206

The parents of a child with pyloric stenosis ask you what the chances of their next baby having the condition are, what do you say?

20% chance

207

What do you tell the parents of a child who has just had a Ramstedt procedure?

The vomiting may continue for 3 - 5 days, that is normal. 

208

What is the most likely cause of vomiting in neonates of different ages in Monash OSCEs?

That is, in Monash exams what causes vomiting:

Immediately after birth

First few days after birth

3-4 weeks after birth


Immediately after birth = duodenal atresia
3 days after birth = malrotation with volvulus
First few days of life with failure to pass stools = MI or Hirschsprungs
3-4 weeks after birth = pyloric stenosis
 

209

What are the different RCH levels of dehydration and their associated clinical presentations?

Mild dehydration = No signs
Moderate dehydration = 4-6% depleted - Signs of dehydration
Severe dehydration is >= 7% depleted - Shock

For the purposes of calculation of fluid requirements use:

Mild - 3%

Moderate - 5%

Severe - 10%
 

210

A 6kg infant is moderately shocked, what IV fluids do you prescribe?

First: For shock give 10-20ml boluses of normal saline until signs of shock improve.

Second: Replace deficit, so for a 6kg kid if they're moderately shocked that approx 5%, so their deficit is approx 300ml, give 300ml normal saline

Third: Give maintenance fluids using normal saline + 5% glucose. Using the 4,2,1 rule for a 6 kg infant that is 24ml/hr of normal saline + 5% glucose.

Monitor electrolytes daily and consider adding 20mmol K

211

Child with acute onset diarrheoa + abdo pain that has now been going for 16 days, what is the diagnosis?


Cryptosporidium
Giardia Lamblia
 

212

What are the features of diarhoea caused by salmonella species and shigella?

dysentery w blood, pus, pain and tenemus

213

What investigations do you order for a kid with acute onset diarrheoa?

Bedside

None

Bloods and urine

UECs and glucose a minimum if commencing any IV rehydration

Cultures
Faecal culture and MCS if significant abdominal pain, blood or mucus, or septic child
Faecal ova, cysts and parasites – if considering parasitic causes
C difficile toxin if recent use of antibiotics
 

214

A child had an an episode of diarrheoa, abdo pain and fever that came on suddenly and lasted for 3 days. It's now two weeks later and they're feeling better, but still having watery diarrheoa. What is the diagnosis?

What's the management?


Post-gastroenteritis lactase deficiency

Mx - Lactose free diet for a few weeks
 

215

2 year old boy recently arrived from Iraq has a 1 year history of diarrhoea with significant weight loss. On examination he has a distended abdomen and wasted buttocks.

Coeliac disease!

216

James, an 18 month old child has chronic diarrheoa and a distended abdomen. You suspect coeliac disease, what investigations will you order?

Bedside

NA

Bloods

  • (not anti TTG or IgA like normal as low sensitivity under 2 years)
  • Anti Gliadin
  • HLA testing
  • Screen for T1DM and thyroid disease
  • FBE
    • May show microcytic or macrocytic anaemia from iron or folate deficiency
  • U&E
    • may reveal reduced concentrations of calcium, magnesium, total protein, albumin or vitamin D

Imaging

None

Special

Gastroscopy and small bowel biopsy

217

What is the management of coeliac disease?


Exclusion of gluten (barley, rye, wheat, and oats)
Dietary fortification – iron, folate, calcium and vitamin D
 

218

How common are undescended testes?

1/25 boys born

219

What are the (informal) criteria for admission for a child with bronchiolitis?

 

SpO2 <93%

Oral intake <50% of normal

Parental anxiety

Or, if these criteria are not quite met but the condition is likely to worsen before it gets better (remember the worst symptoms are on day 2-4) then consider admission

220

What question should you ask every parent who brings their child to you for review?

What worries you the most?

221

What is the clinical picture of a mycoplasma pneumonia in kids?

What antiobiotic do you use?

Subacute onset, prominent cough, +/- headache, +/- sore throat

Roxithromycin PO for 10 days

222

A child presents with non-specific symptoms and a fever of 38.6, you give them paracetemol in the ED. Do you care if the paracetemol brings the fever down? Do you care if the paracetemol changes anything else?

The response of fever to paracetemol is not significant, and is not an indicate of illness severity.

However, if the child was tachycardic and the paracetemol does not bring their HR down, you would consider myocarditis. If it was myocarditis admit them for monitoring and supportive care.

223

What conditions cause bilious vomiting in a child?

  • malrotation and volvulus
  • duodenal atresia (if obstruction distal to Ampulla of Vater 80% of cases)
  • jejunoileal atresia
  • meconium ileus
  • necrotising enterocolitis

224

When does meconium usually pass?

The first stool is passed within 24 hours of birth in 99 percent of healthy full-term infants and within 48 hours in all healthy full-term infants. Failure of a full-term newborn to pass meconium within the first 24 hours should raise a suspicion of intestinal obstruction.

225

What is this?

What is the condition associated with?

How is it diagnosed?

What is the management?

Exophalos (omphalocoele) - large defect at the umbilicus with herniation of bowel and liver into a sac covered by fused amniotic membrane and peritoneum. 

Associations

Prematurity/IUGR

30% will have CVS defects

Also associated with trisomy 13 and 18, renal abnormalities and other syndromes. 

Diagnosis

Usually diagnosed on the 12 and 20 week scans.

Aim is to then monitor them and deliver them in a tertiary centre.

Management:

  • Glad wrap
  • NG decompression
  • IV Ab and fluids
  • Catheter to monitor fluid output
  • Heater
  • BSL
  • Look for associated malformations
  • Delayed operation (as often unstable initially). 
    • appendix is removed and the goal is to get it all inside the abdomen. This is not always possible and thus a 'silo reduction' may be used. 

 

226

What is this?

What is it associated with?

How is it diagnosed?

What is the management?

Gastroschisis - defect immediately to the right of the umbilicus through which bowel (and sometimes gonads) herniate. There is no covering sac and the eviscerated small and large bowel is thickened and densely matted with exudate as the result of amniotic peritonitis before birth. 

Associations

Prematurity/IUGR

Related to maternal drug use. 

Diagnosis

Usually diagnosed on the 12 and 20 week scans.

Aim is to then monitor them and deliver them in a tertiary centre.

Management:

  • Glad wrap
  • NG decompression
  • IV Ab and fluids
  • Catheter to monitor fluid output
  • Heater
  • BSL
  • Look for associated malformations
  • Immediate operation
    • appendix is removed and the goal is to get it all inside the abdomen. This is not always possible and thus a 'silo reduction' may be used. 

227

What key developmental milestones do you know?

The 9 S's of development

Neonate

6 weeks

3 months

6 months

9 months

12 months

18 months

3 years

5 years

228

What are the causes of petechiae in a child?

SHIELD + vWD

vWD less likely to be acute diagnosis. The story is "abnormally prolonged bleeding when prompted (like teeth brushing)" versus SHIELD which is acute onset petechiae

229

Why do you need to do a challenge test before prescribing a child desmopressin?

Because remember that desmopressin/DDAVP is just synthetic ADH. ADH causes aquaporins to be plugged in to the distal collecting tubule allowing for resorbtion of water which can cause dilutional hyponatremia. The challenge test it to make sure the kid doesn't get hyponatraemic.

230

What do you know about post gastro lactase deficiency?

  • Following infectious diarrhoea, infants may have temporary lactose intolerance.
  • This sequela of acute gastroenteritis used to be prominent in infants
  • This may be due in part to early oral feeding which aids mucosal recovery.
  • Clinical features of sugar intolerance include:
    • Persistently fluid stool
    • Excessive flatus
    • Excoriation of the buttocks
    • Typically, these infants appear well.
  •  

    Breast feeding should continue unless there are persistent symptoms with buttock excoriation and failure to gain adequate weight. Formula-fed infants should be placed on a lactose-free formula for 3–4 weeks. 

    231

    What is "toddler's diarrheoa", what is the management?

    A clinical syndrome characterised by chronic diarrhoea often with undigested food in the stools of a child who is otherwise well, gaining weight and growing satisfactorily. Stools may contain mucus and are passed 3–6 times a day; they are often looser towards the end of the day.

    Onset is usually between 8 and 20 months of age.

    Management

    Reassurance and explanation. No specific drug or dietary therapy has been shown to be of value in toddler diarrhoea. 

    232

    A child presents with fever and malaise for 2 days. What are your systems review questions?

    • Headache/neck stiffness/photophobia/Rash
    • Cough, coryza, sore throat
    • Dysuria/frequency
    • Vomiting/diarrheoa/abdominal pain
    • Joint or elsewhere pain?
    • Contacts
    • Travel
    • Immunisations
    • Intake/vomiting/urine production

    233

    How many hours after the onset of symptoms does irreversible ischaemic injury occur to a torted testicle?

    6 hours

    234

    What are your differential diagnosis for an acute scrotum?

    What are the distinguishing feartures on history and exam of each of these conditions that will help you differentially diagnose each of them?

    • Tumour
      • painless
    • Henoch Schonlein purpura
      • purpuric rash on testicle
    • Varicocele
      • bag of worms, left side, teenager
    • Hydrocele
      • transilluminates, soft, can feel testis
    • Incarcerated inguinal hernia
    • Epididymoorchitis
      • fever, nausea, vomiting, urinary symptoms
    • Torsion of the appendix testis (hydatid of Morgagni)
      • More gradual onset. Focal tenderness at upper pole of testis; "blue dot" sign - necrotic appendix seen through scrotal skin.
    • Torsion
      • Sudden onset testicular pain and swelling; occasionally nausea, vomiting. Discolouration of scrotum; exquisitely tender and swollen testis, riding high. Cremasteric reflex is usually absent.

    • Idiopathic scrotal oedema
      • It usually affects boys between 5-9 years of age.

        There is insidious onset of redness and swelling of perineum or inguinal region (groin), extending to scrotum. It may be itchy but is not often painful.

    235

    An 8 year old presents with an acute scrotum, what will you do on examination?

    • observation of the patients gait and resting position
    • natural position of the testis in the scrotum while standing
    • presence or absence of cremasteric reflex (this is absent in torsion)
    • palpation of lower abdomen, inguinal canal and cord
    • palpation of scrotum and contents, compare with unaffected hemiscrotum
    • transillumination
    • Is the swelling reducible?

    236

    What are the causes of chronic diarrheoa in a child?

    Without failure to thrive

    • Infectious
      • Bacterial - campylobacter, salmonella
      • Antibiotic induced - C difficile colitis
      • Parasitic - Giardia lamblia
    • Post-infectious - secondary lactase deficiency
    • Toddler's diarrhoea
    • Lactase deficiency (lactose intolerance)
    • Irritable bowel syndrome - a diagnosis of exclusion in an older child/adolescent

    Chronic diarrhoea with failure to thrive

    • GI causes
      • Coeliac disease
      • Milk protein allergy - cow's milk, soya protein, etc.
      • Inflammatory bowel disease
    • Pancreatic insufficiency --> Cystic fibrosis
    • Others
      • Thyrotoxicosis
      • Food allergy

    237

    What is the paediatric clinical picture of coeliac disease?

    The classical presentation is of a profound malabsorptive syndrome at 6-24 months of age after the introduction of wheat-containing weaning foods (e.g. steatorrhoea, vomiting in some cases).

    • FTT with poor appetite
    • Steatorrhoea
    • Anorexia, nausea and vomiting, abdominal pain
    • Abdominal distention
    • Buttock wasting
    • General irritability, apathy and fatigue - consider Fe deficiency

    Presentation is highly variable, and may be less acute later in childhood. 

    238

    What causes constipation in kids?

    What is the management?

    Most commonly functional constipation (99%) - due to lack of fibre, poor fluid intake, behavioural things (especially around toilet training), and changes in diet (esp. when changing to cow's milk).

    Management

    Clean out + maintain with stool softeners and adequate fibre. Address any underlying problems. This may need to be done multiple times. 

    239

    What is the management of gastro in a child?

    Basics

    • Keep breastfeeding
    • Regular small volume flood intake
    • Ondansetron

    Place and person

    Admit the following:

    • Infants with moderate-severe dehydration
    • Diagnosis in doubt
    • Those who are high risk of dehydration (keep in hospital for 4-6 hours):
      • Infants
      • Infants
      • High frequency diarrhoea (8 per 24 hours)
      • High frequency vomiting (>4 per 24 hours)

    Investigations

    • Mostly not required.
    • UECs and glucose - if severe dehydration and needs resuscitation.
    • If there is significant abdominal pain, or blood or mucus in the stools, or if the child appears septic
      • Faecal culture
      • Faecal leukocytes or blood
      • Faecal ova, cysts and parasites - when considering parastic infections
    • C difficile toxin (faecal) - if recent use of antibiotics 

    Definitive

    Oral, NG or IV rehydration

    There is no role for antibiotics in bacterial gastroenteritis. 

    Long term

    Early feeding as soon as rehydrated reduces stool output and aids gut recovery. Anti-diarrhoeals and maxalon are not recommended.
     

    240

    At what age does foreskin typically become retractible?

     

    What is the treatment of true phimosis?

    The proportion of boys with retractile foreskins is: 40% at 1 year, 90% at 4 years and 99% at 15 years.

    Management of true phimosis

    • 0.05% betamethasone cream should be used twice daily for 2 to 4 weeks.
      • Gently retract foreskin without causing any discomfort and apply a thick layer of cream to the tightest part of the foreskin.
    • Circumcision (if significant phimosis and steroid creams fail)

    241

    What is smegma?

    What is Balanitis?

    Smegma = white/yellow lumps are called smegma and are made up of the cells that once attached the foreskin to the head of the penis. Smegma is normal and will eventually disappear. No treatment is needed.

     

     Balanitis: severe inflammation of the glans penis +/- foreskin is often due to infection and is usually termed balanitis. Tx is Soaking in a warm bath with the foreskin retracted plus topical steroid.

     

     

    242

    What pathogens typically cause encephalitis in a child?

    • Enterovirus
    • HSV
    • Other herpes viruses (EBV, CMV, HHV6, VZV)
    • Arboviruses.
    • Less commonly, encephalitis can be caused by bacteria, fungi or parasites. 

    243

    What pathogens typically cause meningitis in kids below 2 months of age?

    What about kids above 2months?

    What empirical drugs do you use in each of these age groups?

    Below 2 months of age

     

    • Group B streptococcus
    • E. coli and other Gram-negative organisms
    • Listeria monocytogenes  
    • Plus the below....

    Use cefotaxime and ben pen

    Above 2 months of age

    • Streptococcus pneumoniae 
    • Neisseria meningitidis 
    • Haemophilus influenzae type B (in unimmunised children)
      • As a result of immunisations Hib meningitis is now rare and there has been a reduction in the incidence of pneumococcal meningitis. 

    Use ceftriaxone and dexamethasone

    244

    A mother has been told her child might have autism and wants to know if her child with have a low IQ, what do you say?

    25% of people with autism will have normal/high IQ

    75% will have some degree of intellectual disability

    The child's level of ability is often uneven, with areas of strength and weakness. 

    245

    The mother of an 8month old boy with an inguinoscrotal hernia asks whether surgical intervention is really necessary and why do you need to repair the patent processus vaginalis, what do you tell her?

    There are three problems:

    1. Protrusion of the bowel through the processus vaginalis can obstruct it's blood supply causing necrosis/peritonitis/sepsis
    2. Obstruction of the processus vaginalis can put pressure on and ultimately obstruct the blood supply to the testicles and the spermatic cord, resulting in death of the testis and/or reduced fertility
    3. obviously there is the risk of bowel obstruction

    246

    What is this? What is the management?

    Ballooning of the foreskin during urination due to physiological phimosis.

     

    In itself is not a concern, no need for management, will resolve as phimosis resolves. 

    247

    A 2 year old child has non-retractible foreskin, you advice the parents that this is normal, not to retract the foreskin for cleaning, and that it will resolve itself. But, you say, if it has not resolved by a certain age you can prescribe them something. At what age would you intervene and what would you prescribe.

    4 years old, topical steroid.

    248

    How do you distinguish between an inguinoscrotal hernia and a hydrocele?

    • Hernia is reducable (unless strangulated, in which case it will be extremely painful)
      • ​Hydrocele is not reducable, it would take hours for you to squeeze the fluid back up the narrow patent processus vaginalisis
    • Hydrocele transilluminates, hernia does not
    • You can get above a hydrocele but not a hernia
    • Hydrocele doesn’t really ever have to be fixed, but you do for cosmesis after age 2. 

     

    249

    What is the clinical picture of NEC?

    What do you see on Xray?

    • Pre-term baby, typically in the 2nd-3rd week of life
    • Infant stops tolerating feeds --> vomiting (bilious sometimes)
    • Abdominal distention
    • Rapidly becomes shocked
    • Other features:
      • Bloody stools
      • Pneumatosis intestinalis - gas cysts in the bowel wall; diagnostic for NEC
      • Diminished bowel sounds
      • Signs of bowel perforation - sepsis, shock, peritonitis, DIC 

    250

    Oesophageal atresia is usually associated with what other anatomical abnormality?

    How do you confirm the diagnosis?

    Tracheo-oesophageal fistula in 85% of cases

    Diagnosis is confirmed by passing a large firm catheter through the mouth and finding that it cannot be passed more than about 10cm from the gums. A plain XRAY of the torso will show gas in the bowel (if there is distal TOF). 

    251

    What is a SUFE?

    What are the different ways to classify the condition?

    Slipped upper femoral epiphysis (SUFE) is the most common hip disorder in the adolescent age group.  It occurs when weakness in the proximal femoral growth plate allows displacement of the capital femoral epiphysis.  The metaphysis displaces anteriorly and superiorly, leading to the slipped state. 

    There are a number of ways that SUFE can be classified

    Stable vs unstable

    The patient is able to weight bear on the affected leg (stable) versus unable to weight bear even with crutches (unstable).

    Acute, chronic or acute on chronic

    Acute = Sudden onset of severe symptoms and inability to weight bear

    Chronic = Gradual onset and progression of symptoms for more than 3 weeks, without sudden exacerbation. This is the most common presentation (85% of patients with SUFE)

    Acute on chronic = Sudden exacerbation of symptoms due to acute displacement of a chronically slipped epiphysis

    Radiographical degree of displacement

    Type I (0 to 33% displacement)
    Type II (34 to 50% displacement)
    Type III (>50% displacement)

    The likelihood of complications increases with displacement 

     

    252

    A 12yo obese boy comes in with a sore hip, you think it's a SUFE, what examination could you perform to confirm this?

    A very reliable sign of chronic SUFE is detection of obligatory external rotation during flexion of the hip.  As the hip is flexed on the affected side, the thigh will automatically rotate and abduct

    253

    What is the full management of a SUFE?

    Basics

    Nil by mouth

    Depending on severity, pain management according to WHO pain management ladder.

    Place and person

    Urgent orthopaedic referral

    Investigate and confirm diagnosis

    AP and frog lateral pelvis x-rays of both hips

    Non-invasive management

    The patient needs to be kept non-weight bearing

    Definitive management

    Immediate surgical screw fixation across the physis (within 24 hours)

    254

    Name the different types of JIA in order of frequency.

    What age group and gender does each type tend to affect?

    (1 - 50%) Oligoarticular JIA (peak age 2-3, rare after age 10, F>M)

    (2 - 40%) Polyarticular JIA (dual peaks at 2-5 then 10-14 years, F>M)

    (3 - 10%) Less common types like systemic JIA, psoriatic and enthesitis (HLAB27+) (anyone less than 17, M=F)

    255

    What are the diagnostic criteria for oligo, poly, systemic JIA and enthesitis?

    JIA diagnosed if age 6 weeks of symptoms

    Systemic arthritis

    • 1 or more joints
    • Preceded by at least 2 weeks of fever
    • Signs/symptoms include one or more of:
      • evanescent rash,
      • generalised lymphadenopathy,
      • hepato/splenomegaly,
      • serositis

    Oligoarthritis

    • Arthritis affecting 1-4 joints during the first 6 months
    • Persistent oligoarthritis affects up to 4 joints throughout the course of the disease

    Polyarthritis

    • 5 or more joints during the first 6 months
    • RF+ disease is more aggressive than RF-

    Psoriatic arthritis

    • Diagnosed if there is arthritis and psoriasis, or
    • arthritis plus 2 or more of:
      • dactylitis,
      • nail pitting,
      • onycholysis,
      • fam hx of psoriasis

    Enthesitis-related arthritis

    • Diagnosed if there is arthritis and/or enthesitis with 2 or more of:
      • hx of sacroiliac joint tenderness
      • HLA B27 antigen,
      • onset of arthritis in a male over 6 years of age
      • acute anterior uveitis
      • Fhx of ankylosing spondylitis, enthesitis-related arthritis, sacroiliitis with inflammatory bowel disease, Reiter’s syndrome, or acute anterior uveitis in a first-degree relative

    256

    What is the (generic) management of JIA?

    Basics

    Rule out septic arthritis

    Place and person

    Refer to specialist paediatric rheumatology multidisciplinary team

    Ix and confirm diagnosis

    • FBE (anaemia, thrombocytosis, WCC)
    • ESR/CRP
    • RhF, anti-CCP
    • ANA (for anterior uveitis risk)
    • HLA B27 genotyping if suspect enthesitis
    • Fe studies (reactive ferritin)
    • Xray

    Non-invasive management

    Physiotherapy and occupational therapy
    Swimming and cycling encouraged, weight-bearing activities for those at greater risk of low bone mineral density

    Definitive management

    Varies depending on type of JIA, sounds very specialized.

    • Pain relief with NSAIDs or corticosteroid injections
    • Methotrexate, sulfasalazine, steroids
    • Biologics (anti-TNFa) increasingly used

     

     

    257

    What is the prognosis of JIA

    • 70% of patients will have minimal or no joint disease by age 15
    • Oligoarticular disease has the best prognosis, with most patients achieving remission
    • RF+ve patients and those with systemic arthritis have a worse prognosis, with more continuing to have active disease after several years

    258

    What complication do you worry about in systemic JIA?

    MAS – Macrophage activation syndrome

    • Life-threatening emergency
    • Usually occurs early in the onset of systemic JIA
    • high fever, hepatosplenomegaly, lymphadenopathy, pancytopenia, liver dysfunction, disseminated intravascular coagulation, hypofibrinogenemia, hyperferritinemia, and hypertriglyceridemia
    • Reduced ESR
    • Tx with high dose steroids and cyclosporin

    259

    A baby presents with malrotation/volvulus, when should the corrective surgery be done?

     Now. You have 6 hours before bowel becomes ischaemic and patient dies 

    260

    How is oesophageal atresia diagnosed?

    • Wide-calibre feeding tube is passed and checked by X-ray to see if it reaches the stomach
      • AXR shows gasless abdomen --> absent gastric bubble
    • If not diagnosed at birth, presents clinically with:
      • Persistent salivation and drooling from the mouth after birth
      • ​Infant will cough and choke when fed, and have cyanotic episodes

    261

    If you discover a congenital abnormality in a newborn what do you do?

    Check for others, they tend to co-occur....

    VACTERL

    V - Vertebral anomalies
    A - Anal atresia
    C - Cardiovascular anomalies
    T - Tracheoesophageal fistula
    E - Esophageal atresia
    R - Renal (Kidney) and/or radial anomalies
    L - Limb defects

    262

    What are the differences betweeen omphalocele and gastroschisis?

    263

    What is the most dangerous manifestation of Hirschprungs? How is it managed?

    Enterocolitis 

    Decompression washout enemas, IV ABx and intensive care

    264

    Describe the surgical management of hirschprungs and the complications of this approach

    • Surgery too hard in neonate, use colonic washout enemas until baby is 5-6kgs

    • Pull-through procedure

      • Involves resection of distal bowel to transition point (established via contrast enema X-ray), and mobilisation of proximal bowel to allow pull-through to anal anastomosis

    • Complications

      • Prone to dehydration as large bowel is responsible for absorption of sodium, potassium and fluid
        Increased frequency of bowel movements due to increased fluidity of stools

      • Often incontinent

      • Technically difficult procedure due to risk of damage to continence muscles, possibility of anastomotic breakdown, stricture

      • Prone to Hirschsprung’s Enterocolitis even years after corrective procedure

        • Requires IV antibiotics every time 

    265

    What are the causes of a limp/non-weight bearing in a child?

    HAI! DONT TAP SAM

    H - HSP

    A - Appendicitis

    I - Inguinoscrotal pathology/testicular torsion

    D- DDH (age 1-4)

    O- Osteomyelitis/septic arthritis

    N- NAI

    T- Toddler’s fracture (age 1-4)

     

    T- Transient synovitis

    A- Arthritis/JIA

    P- Perthes disease (age 4-10)

     

    S- SUFE (aged >10)

    A- Articular or non-articular stress fracture

    M- Malignancy (ALL)

    266

    What is the management of transient synovitis of the hip?

    Basics

    Bed rest is important for children with transient synovitis.
    Analgesia; NSAID (eg ibuprofen) +/- paracetamol

    Place and person
    Review with local doctor within 3 days.

    Ix

    None

    Definitive

    Analgesia

    Long term/follow up
    Return to hospital if febrile, unwell or getting worse
    Patients with symptoms for greater than 4 weeks can be referred to rheumatology clinic.

    267

    A child presents to ED due to a painful hip. They are not weight bearing. Describe in full what you would do on examination.

    • General appearance, temp
    • Gait - running may exaggerate a limp
    • Neurological examination - look for ataxia, weakness
    • Generalised lymphadenopathy (viral infection / haematological cause)
    • Excessive bruising or bruising in unusual places (NAI, haematological)
    • Abdomen, scrotum and inguinal area (masses)
    • Bony tenderness
    • All joints
      • knee pain can be referred from the hip, and thigh pain can be referred from the spine
      • Include sacro-iliac joints and spine in joint assessment - look for pain on flexion and/or midline tenderness which may be present in discitis
      • Exaggerated lordosis (discitis)
    • Hip abduction and internal rotation are often the most restricted movements in hip pathology 

    268

    What organisms typically cause osteomyelitis?

    Staphylococcus aureus

    but can be caused by group A b-haemolytic streptococci and Haemophilus influenzae. 

    269

    What do you know about Perthe's disease?

    A self-limiting disease of the femoral head comprising of necrosis, collapse, repair, and re-modelling that presents in the first decade of life and is more commonly seen in boys. 

    Presentation

    • An occasional limp in the early phase
    • Knee pain
    • Worsening pain and limping as the disease progresses
    • Pain in the knee, thigh or groin on movement or when putting weight through the leg
    • Decreased movement
    • Uneven leg length – the affected leg may become shorter 

    Investigation - Xray +/- MRI

    Management

    If the hip joint is only mildly affected, no immediate treatment may be required. If there is pain or stiffness, they will need to rest to relieve the hip joint from weight-bearing movements (no high-impact activities, like jumping or running, until the joint has recovered.) Low impact activities like swimming and gentle cycling are recommended. Ortho referral for consideration of surgical repair

    270

    What are the causes of diarrheoa in a child?

    Acute

    Gastro

    Chronic

    Coeliac

    Lactose intolerence (temporary after gastro or permanent)

    Pancreatic insufficiency from CF

    271

    Parents of a 6month old baby present to you worried about a lump under the babies umbilicis which appears when he cries or becomes irritable. What do you tell them?

    It's an umbilical hernia.

    Common condition and > 95% will resolve spontaneously by 2-3 years. 
    In infants, hernia may become large and increase in size in the first 6 months.

    No treatment needed

    Refer to the OPD of General Surgery if still present after 2 years or if there is parental anxiety due to large size in infancy

    272

    Parents bring in a 9 month old boy and report than he gets a lump next to his penis when he cries, it is not there now. What investigations should you order.

    None. This is an indirect inguinoscrotal hernia protruding through a patent processus vaginalis. If the duct was smaller it would just cause a hydrocele. There are no tests indicated. Ultrasound can not see anything.

    273

    A father brings his 7yo daughter to you and explains that she has been weak, tired and pale. You suspect anaemia, what:

    • history questions do you ask?
    • examination do you perform?
    • Investigations do you order?

    History

    • Microcytic anaemia -- Iron deficiency
      • Intake
        • diet, meat, chicken, veges
      • Absorbtion
        • Screen for malabsorbtion
          • diarheoa, steatorheoa, poor growth, abdo pain
          • Family history or coeliac
      • Loss
        • blood loss
    • Microcytic anaemia -- thalassaemia
      • family history of thalassaemia
    • Normocytic anaemia -- aplastic anaemia
      • Weight loss, night sweats, predisposition to infection, bruising/petechiae
      • Slapped cheek picture or rash (transient erythroblastopenia of Parvovirus)
    • Macrocytic anaemia -- B12 and folate
      • adequate intake of folate and B12?

    Exam

    • Vitals
    • Growth charts
    • Pallor palmar creases/conjunctiva
    • Jaundice
    • Flow murmur?
    • Hepatosplenomegaly
    • Lymphadenopathy

    Investigations

    • FBE
    • Blood flm
    • Reticulocyte count
    • Fe studies

     

    274

    How common is jaundice in neonates?

    80% of preterm infants

    60% of term infants

    275

    You order a fractionated serum bilirubin for a jaundiced baby.  At what % of conjugated billirubin would you call the situation a "conjugated bilirubin - hepatic/post hepatic cause"

    If >15% of total bilirubin is conjugated or absolute conjugated bili of 15umol, that’s when to consider a hepatic/post-hepatic causes

    276

    What are the four different types of pathological jaundice in a neonate?

    1. Too early (
    2. Too high (More than 320)
    3. Rising too quickly (Increasing at a rate of greater than 85 micromol per day)
    4. Too late (more than 2 weeks)

    277

    At what level can you see jaundice in the sclera/body?

    Slera = 50

    Body = 100 (progresses cephalocaudally)

    278

    What are the causes of jaundice "too early"

    Before 24 hours

    • Haemolysis due to blood group incompatibility
      • Rhesus incompatibility
      • ABO incompatibility
      • Minor blood group incompatibility
    • Haemolysis due to congenital infection/sepsis
    • Haemolysis due to RBC abnormality
      • RBC enzyme deficiency --> G6PD
      • RBC membrane defect --> hereditory spherocytosis

    279

    Who is it at high risk of kernicterus?

    • Premature infants
    • Low albumin
    • Sepsis (esp meningitis)
    • Unconjugated bilirubin >350 micromol/L
    • Asphyxia, acidosis, hypoxia and hypothermia
       

    280

    You have been asked to get consent from Lily's parents for phototherapy because she is mildly jaundiced. What do you say?

    Introduction

    Explanation (before, during and after)

    • Phototherapy transforms your baby’s bilirubin into a less harmful chemical
    • Your baby is placed in an incubator, they are undressed and wear eye protection
    • Because they can dehydrate, you will need to feed your baby every 3-4 hours
    • This therapy will normally last 1-2 days until the bilirubin levels are rechecked are low enough to be safe

    Benefits

    Safe way to reduce the level of bilriubin and protect the brain

    Risks

    Overheating, water loss, diarrhea, rash, (theoretical) retinal damage

    Alternatives

    A bili-blanket is a pad that applies light directly to the skin. Can allow your baby to stay in the cot

    281

    What are the causes of late jaundice?

    Depends whether it is conjugated or not…

    Unconjugated

    • G6PD
    • Hypothyroidism
    • Breast mild jaundice
    • Sepsis

    Conjugated

    • Hepatitis due to TORCH infections
    • Alpha1 antitrypisin
    • Cholestasis due to biliary atresia or choledocal cyst

    282

    What are the acute signs and long term side effects of kernicterus?

    Acute signs

     

    • Lethargy/drowsiness
    • Poor feeding
    • Irritability
    • Temperature instability
    • Arching of the head, neck and back (opisthotonos)
    • Seizures

    Long term

    • Death
    • Permanent brain damage including:
    • Athetoid cerebral palsy
    • Deafness
    • Mental retardation

    283

    What are the differentials for haematochezia in a child?

    • Inflammation
      • Crohn’s, ulcerative colitis
    • Infection
      • C diff, giardia, enamoeba, shigella, other causes of gastro
    • Meckel’s
    • Milk
      • Cow’s milk protein intolerance is a common cause of rectal bleeding
      • Trial a non-milk (and non-soy) diet
      • Often resolves with age
         

    284

    Why do we think breast feeding can cause jaundice? What is the management of breast milk jaundice?


    Breast milk is high calories of low volumes --> more enterohepatic circulation
    Don’t stop breast feeding!
    In fact, important to keep feeding to rehydrate and to interrupt enterohepatic circulation / ‘get the gut moving’
     

    285

    How would you approach history taking for a jaundiced neonate?

    1. Determine the time course of the jaundice
      1. early, high, accelerating, late
    2. Ask after the underlying cause
      1. Blood groups/anti-D
      2. PPROM/GBS/infections
      3. Family history of blood disorders
      4. Instrumental delivery/cephalhaematoma
      5. Dirty nappes/bowel obstruction
    3. Ask about symptoms of kernicterus
      1. irritability
      2. decreased appetite
      3. seizures
      4. drowsiness
      5. arching back
    4. Screen for kernicterus risk factors
      1. prematurity
      2. low albumin
      3. Sepsis, particularly meningitis
      4. Asphyxia, acidosis, hypoxia and hypothermia

    286

    What is G6PD deficiency?

    an X-linked recessive genetic condition that predisposes to hemolysis and resultant jaundice in response to a number of triggers, such as certain foods, illness, or medication.

    There is no treatment.

    287

    What investigations would you order for a jaundiced neonate?

    Frickety Fuck Bloody Mum Seems To Give Crappy Antibodies - Liver Ultrasound

    FBE/Retics/film

    Fractionated SBR

    Bloood group and hold

    Mothers blood type

    Septic screen

    Thyroid function tests

    G6PD deficiency

    Coombs Direct antibody test (detects IgG attached to RBCs suggesting autoimmune haemolysis)

    Alpha1 anti-trypsin

    LFTs

    Ultrasound

    288

    A neonate is jaundiced, what is your management?

    Basics

    Ensure adequate caloric intake / glucose (breast, formula)

    Ensure kept at warm temperature (incubator)

    Place and person

    Contact Paeds

    Admit to SCN / NICU

    Investigate and confirm the diagnosis

    Frickety Fuck Bloody Mum Seems To Give Crap Antibodies – Liver Ultrasound

    • FBE/Retics/film
    • Fractionated SBR
    • Bloood group and hold
    • Mothers blood type
    • Septic screen
    • Thyroid function tests
    • G6PD deficiency
    • Coombs Direct antibody test (detects IgG attached to RBCs suggesting autoimmune haemolysis)
    • Alpha1 anti-trypsin
    • LFTs
    • Ultrasound

    Definitive management

    • Treat cause if possible
    • Phototherapy
    • IVIG if immune mediated haemolysis
    • Exchange transfusion if anaemic
    • Low threshold for empirical ABx

     

     

    289

    What are the causes of "too high" jaundice?

    • All the causes of early jaundice
    • Cephalhaematoma
    • Polycythemia (were they IUGR?)
    • Poor feeding
    • Bowel obstruction causing increased enterohepatic circulation

    290

    How can anaemia present in a child?

    • Pallor – conjunctival, palmar crease
    • Fatigue, listlessness
    • Poor growth
    • Flow murmur
    • Air hunger (severe)
    • Hypotension and cardiac failure (severe)
    • Iron deficiency can present with poor attention span, irritability and pica (eating dirt, clay, ice etc)

    291

    What are the minimum investigations for suspected haemolytic anaemia in a child?

    • FBE
    • Blood film (e.g. spherocytosis)
    • Bilirubin
    • Reticulocytes
    • Direct Coombs test
    • G6PD assay

    292

    Andrew is a 7 year old boy from Hong Kong. A few days ago he developed a cough and a sore throat.
    He presents with pallor and mild jaundice. On examination he has splenomegaly. His mother mentioned that this has happened before.
     

    G6PD deficiency

    Common in Chinese, Mediterranean and African people

    X-linked defect in the enzyme that protects cells from oxidative damage

    Triggered by infections, fava beans and drugs (sulfurs, antimalarials) 
     

    293

    Martha is fed up. She is 25 years old. An upper abdominal U/S has confirmed yet another gallstone, despite her cholecystectomy last year. She has mild splemegaly.

    On history, she tells you that she was jaundiced at birth and, now that you mention it, she has occasionally gone yellow during periods of illness.

    Her blood film shows:
     

    Hereditary Spherocytosis

    • Fairly common
    • Usually associated with neonatal jaundice.
    • These RBCs have a membrane defect, that causes their funny shape and predisposed them to destruction in periods of illness. May present with jaundice, anaemia and splenomegaly throughout life (even in adult life with recurrent cholelithiasis!)
       

    294

    What are the causes of abdominal pain in kids?

    MIDGUT MISHAP

    Migraines - abdominal
    Intussception
    DKA
    Gastroenteritis
    UTI
    Testiicular Torsion

    Malignancy (Wilm’s, Leukaemia, Neuroblastoma)
    Inguinal hernia - stranulated
    Stuck poo (constipation)
    HSP
    Appendix + mimickers (mesenteric adenopathy / Meckel’s diverticulum)
    Pregnancy / ectopics / gynae

    295

    You diagnose transient synovitis of the hip. The parents ask:

    "In a sentence, what is it?"

    What age kids does it typically affect?

    How long till it will be better?

    It's the common cold of the hip

    It affects kids age 3-10

    It should improve within 3 days

    It should be resolved within 2 weeks

    296

    What is the management of transient synovitis of the hip?

    • Home care should involve complete rest and pain relief
      • Time off school until better
      • Avoidance of sport and strenuous activity until completely better
      • Paracetemol
        • NSAIDs should be used with caution in asthmatics
        • Aspirin should be avoided in children
    • Importantly, tell them to come back if:
      • High fevers
      • Swelling
      • Redness
      • Severe or worsening pain
      • Pain that does not improve after 3 days or significantly resolve in a week. 

    As a side note the concern with aspirin in kids under 16 is Reyes Syndrome. It is a very rare condition of acute deadly noninflammatory encephalopathy and fatty degenerative liver failure. 

    297

    What is the management of septic arthritis?

    Basics

    ABC

    Antiemesis

    Analagesia

    NBM

    Place and person

    Admit and refer to ortho for washout, abscess draining

    Investigation

    Blood – FBE, ESR, cultures
    Imaging – plain film
    Special – joint aspiration, bone scan (best test)

    Definitive Management
    Urgent aspiration +/- arthrotomy and washout
    Flucloxacillin IV

    Follow up
     

    298

    What are the risk factors for DDH?

    Breech presentation (10x)
    Female (4x)
    Oligiohydraminos (4x)
    Macrosomia (>4kg) (2x)
    1st born baby (2x)
    FMHx

    Essentially, anything that causes the foetus to be squished can cause hip development issues. 

    299

    Who should receive USS screening for DDH?

    Infants with a suggestive clinical examination

    OR

    risk factors (breech birth, oligohydraminos, macrosomia and FMHx) 

    300

    What investigations do you use for suspected DDH?

    Before 4-6 weeks, the hip is largely cartilaginous --> No test is useful

    Formal investigation is often deferred until after 6 weeks

    Before 4-6 months, ossification of the upper femur has not occurred
    --> Ultrasonography is the best test.

    After 4-6 months, there is sufficient ossification to enable interpretable radiography
    --> Plain film becomes the best test
     

    301

    A concerned mother brings her 7 month old to you because her sister told her that she thinks the baby may have DDH, how would you check if the sister was right?

    After 3 months, the Barlow and Ortolani tests are less useful
    More meaningful signs include:

    • Asymmetrical hip abduction
    • Uneven leg length
    • Asymmetrical skin creases (weak sign)
    • High greater trochanter (dislocated)
    • Gait disturbance and chronic limp (older children)
       

    302

    What is a toddler's fracture?

    What is the management?

    What is the prognosis?

    A radiologically subtle spiral fracture of the distal tibia that occurs in toddlers (9 months to 3 years). The periosteum remains intact and the bone is stable.  These fractures occur as a result of a twisting injury.

    Suspect when:

    • Unexpected pain or duration of limp after a relatively minor injury
    • Twisting one leg causes no pain, twisting the affected leg is very painful
    • Tender tibia on palpation

    Management

    • Above-knee plaster can give pain relief, but if the pain is minimal treat conservatively.
    • May continue to limp for 6 weeks.



     

    303

    What would you do for a kid with a Perthes disease?

    What is the prognosis?

    Orthopaedics referral

    Normal recovery expected in 18-36 months

    Femoro-acetabular contact is key to normal growth

    Treatment depends on severity:

    Symptomatic --> slings/braces --> broomstick plaster --> surgery

    304

    How do you detect a SUFE on xray?

    Notice that a line along the lateral edge of the femoral neck does not cross the epiphysis on the affected side. 

    305

    An obese 12yo boy presents with a painful left hip to ED. What do you do?

    This is a SUFE

    Order a plan x ray


    No weight bearing until definitive management

    Urgent orthopaedic review

    In situ pinning of the epiphysis to the femoral neck
     

    306

    What are the features of an inflammed joint?

    • Pain
    • Warmth
    • Swelling
    • Erythema --> usually suggests an infectious pathology!
    • Stiffness, worse in morning
    • Loss of function (reduced range of movement)
       

    307

    What are the forms of JIA?

    • Oligoarthritis
      • 4 or fewer joints affected in the first 6 months of the disease
      • Broken down into normal and extended
    • Polyarthritis
      • 5 or more joints affected in the first 6 months of the disease
      • RH - and RF +
        • RF + is basically just RA, bad prognosis
    • Systemic Arthritis
      • Formerly called ‘Still’s disease’
      • Systemic symptoms like fever, rashes, lymphadenopathy and hepatosplenomegaly predominant the early illess
    • Beyond the joint
      • psoriatic
      • enthesitis (HLA B27+)

    308

    This is the knee of a 14yo boy who has just had a growth spurt. He has come in because his knee is very painful.

    What's the diagnosis?
    What's the treatment?

    What's the prognosis?

    • Osgood-Schlatter syndrome is a painful knee condition that affects adolescents.
    • It often occurs during a growth spurt and is associated with physical activity. Symptoms include pain and local swelling in the front of one or both knees.
    • It is thought that the quadricepts tendon becomes tight and causes inflammation and micro-fractures in the tibial tuberosity.
    • Diagnosis
      • Clinical but get an Xray
    • Management
      • Physio referral for excercises
      • Rest
      • NSAID analgesia
    • Prognosis
      • The condition usually completely resolves within 12 months but the prominent tibial tuberosity will always be there. 

    309

    Sally, a 6yo girl has severe extended oligoarthritis of her right wrist and elbow. What effect may this have on her growth?

    Chronic inflammation can lead to hyperaemia of the affected joint and the local epiphysis. This can lead to abnormally increased growth and lengthening of the affected bone!

    310

    Is oligoarticular JIA erosive?

    Is polyarticular JIA erosive?

    Oligo = no

    Poly = can be, particularly if RF+

    311

    What are the clinical features of the presentation of Still's disease?

    • High spiking (>39) fever, with resolution between spikes. May occur 1-2x daily.
    • 95% have a salmon pink rash on the limbs or trunk that forms during the fever spike. Rash may be urticarial.
    • The rash may be triggered by warm baths or skin irritation (Koebner phenomenon)
    • Lymphadenopathy, hepatosplenomegaly often occur
    • Pericarditis and pleuritis are common
    • There may be severe anaemia.
    • Joint disease may manifest months to years after systemic presentation. 
       

    312

    Who (gender/age) does enthesitis related arthritis typically affect in chldhood?

    What % of these patients go on to have AnkSpond?

    >6yo boys

    10% progress to AnkSpond

    313

    In which of the JIA conditions is RF positive?

    Only a small proportion of polyarticular JIA. Associated with poor prognosis.

    314

    What is the significance of a positive ANA in JIA?

    What types of JIA is ANA typically positive in?

    What is the management of ANA positive JIA?

    Increases risk of anterior uveitis

    Commonly positive in oligo and polyarticular JIA


    Anterior uveitis is often asymptomatic, opthal review 2-6 monthly is needed
    If untreated, can lead to cataract, adhesions in the anterior chamber and glaucoma.

    Treat with topical steroids, dilators (mydriatics) and maybe MTX 
     

    315

    What bedside test is thought to have 100% sensitivity for appendicitis?

    Jumping up and down leading to severe pain

    316

    This is an xray of a neonate, what is the diagnosis?

    Boot shaped heart

    Tetralogy of Fallot

    317

    What causes subglottic stenosis?

    Who does this typically affect?

    Damage from intubation

    Presents with chronic fixed stridor

    Classically an ex-preterm infant who had long term ventilation

    May require endoscopic ablation to resolve

    318

    A baby was born at 32 weeks and has tracheomalacia. If he gets bronchiolitis it could be deadly. What do you do?

    Palivizumab monoclonal antibody against RSV

    Super expensive, only for very high risk babies

    Give as monthly injections throughout RSV season

    319

    What are the two groups that get intussuception?

    Around 9 months old - no pathological lead point - respond well to air enema

     

    Over 3 years - pathological lead point (Meckels, polyp, lymphoma, TB, HSP) - often require surgery

    320

    At what age should you start screening a child with T1DM for micro and macrovascular complications?

    Age 10

    321

    What is the inheritence pattern of congenital adrenal hyperplasia?

    What is the pathophysiology?

    What are the two forms of the condition?

    What is the clinical presentation?

    Inheritance pattern = autosomal recessive

    Pathophysiology = Enzyme defect leading to impaired production of glucocorticoids and mineralocorticoids by the adrenal glands. >95% of cases are caused by a 21-hydroxylase deficiency. Reduced negative feedback to the hypothalamus and pituatary leads to increased ACTH secretion and adrenal stimulation. The adrenal gland can only respond with hyperplasia and increased sex steroid synthesis.

    Forms =  simple virilising and salt-losing

    Clinical presentation =  in females either form presents with ambiguous genitalia. If this is missed they may present with  salt losing adrenal crisis in the following 1-2 weeks. Missing this can lead to death. In males the virilising form leads to signs of puberty as a toddler.

    322

    In one sentence, what is the management of Congenital adrenal hyperplasia?

    Long term mineralocorticoid and glucocorticoid replacement.

    323

    A 5 month old child presents with a UTI. What is the likely organism? What is the management?

    E. Coli.

    IV cephalosporin

    Why? In a child this young they cant present with dysuria so they present late when septic. In an older child trimethoprim would be fine?

    324

    A child comes to ED having had a febrile seizure. Mum asks you what the chance of it happening again are. What do you say?

    30-40%

    325

    Mum brings her 2 yo to you because he's  been febrile with an URTI for 3.5 days. She is worried because he's had a febrile seizure in the past. What do you tell her about the chance of him having a seizure?

    If he's had one there's a 30-40% chance he'll have another one at some point. But they generally occur early in the course of a febrile illness. At day 3.5 it's less likely to occur now.

    326

    A 6 month old girl is brought to you because her parents are worried that she has "bouts of spasms", where she grimaces and throws her arms out. She is unresponsive during these brief attacks but then recovers. What is going on? What do you know about this condition?

    • Infantile spasms / West syndrome
      • catastrophic childhood epilepsy
    • Seizures start between 4 - 12 months
    • Urgent neuro referral
    • 1/20 mortality
    • Poor prognosis with developmental delay and long term serious epilepsy

    327

    At what age would you worry if a child hadn't started walking?

    18 months

    328

    What is short gut syndrome?

    What causes it?

    How is it managed?

    What is the prognosis?

    What is it?

    Malabsorbtion due to severe disease or surgical removal of a significant proportion of the small intestine.

    Causes

    Although SBS may rarely be a congenital condition, most patients with SBS have undergone bowel resection for one of the following:

    • A congenital anomaly such as an omphalocele, gastroschisis, or intestinal atresia
    • Necrotizing enterocolitis associated with prematurity
    • Intestinal ischemia from malrotation and volvulus
    • Crohn disease (infrequent with current treatment)

    Management

    Usually dependent on TPN

    Prognosis

    Fairly catastrophic

    In newborn infants, the 4-year survival rate on parenteral nutrition is approximately 70%.

    In newborn infants with less than 10% of expected intestinal length, 5 year survival is approximately 20%

    329

    In a setence, what is duplex kidney and what complications can it cause?

    The inferior ureter can insert low down in the bladder leading to VU reflex and hydronephrosis

    330

    What are the features of salbutamol toxicity?

    Tachycardia,

    Tachypnoea

    Metabolic acidosis.

    Lactate commonly high.

    Controversial: can cause VQ mismatch and worsen symptoms in very high doses

    331

    A nurse brings you an ABG on a child having a moderately severe asthma attack. She is worried because it shows metabolic acidosis, hyperglycaemia, hypokalaemia and a raised lactate. What do you think?

    The lactate is high because of the large amounts of salbutamol the child would have received.

    The glucose is likely high because the kid has probably been given prednisolone.

    Metabolic acidosis and hypokalaemia are a sign of salbutamol toxicity. You would worry about this (stop salbutamol) but not so much the other stuff.

    332

    What is HSP?

    What age/gender groups does it affect?

    What is the aetiology?

    What complications/other symptoms can it cause?

     

    Henoch-Schonlein purpura (HSP) is the most common vasculitis of childhood and affects the small vessels. HSP is characterised by the classic tetrad of rash, abdominal pain, arthritis/arthralgia, and glomerulonephritis. 

    Age groups

    Between the ages of 3 and 15 years. 

    Males twice as commonly as females

    Aetiology

    The underlying cause of HSP remains unknown. It is an immune-mediated vasculitis, with a variety of infectious and chemical triggers having been proposed as a cause. Many cases of HSP occur after a URTI, especially streptococcal infections

    Complications/other symptoms

    • Nephritic syndrome and long term renal damage
    • Subcuteneous oedema
    • Intussusception
    • Pancreatitis
    • Sponteneous bowel perforation
    • bloody stools
    • Haematemesis
    • Painful scotal swelling

    333

    What is the significance of abdo pain in HSP?

    Uncomplicated abdominal pain often resolves spontaneously within 72 hours. However serious abdominal complications may occur including intussusception, bloody stools, haematemesis, spontaneous bowel perforation, and pancreatitis.

    334

    What investigations would you order for suspected HSP?

    Bedside

    None

    Bloods and urine

    Urinalysis and urine microscopy (to quanitify haematuria)

    FBE

    UEC (elevated creatinine indicated renal Cx)

    Blood culture

    Imaging

    USS of scrotum of abdo if suspect intusscusception, testicular torsion or other

    Special

    Can biopsy lesions in legs if really need to

    335

    What is the management of HSP?

    Basics

    Analgesia

    Place and person

    Any purpuric rash should be reviewed by at least a Reg

    Consider admission if renal complications, suspicion of abdominal complications or severe pain

    Investigate and confirm diagnosis

    Urinalysis and urine microscopy

    FBE/UEC

    Blood cultures

    Definitive

    • Document the child's blood pressure
    • Consider a surgical consult if abdominal features are prominent. Testicular torsion can be hard to differentiate from the pain of vasculitic testicular pain.
    • The use of prednisolone has not been shown to make clinically important improvements in the rate of long-term renal complications.  It has been shown to reduce the duration of abdominal and joint pain and may reduce the risk of abdominal complications. It may be considered for use in patients with more than mild joint or abdominal pain. Consider prednisolone 1mg/kg while symptoms persist.

    336

    You see a kid in ED who you diagnose with HSP. You tell the parents that it will most likely self-resolve. What else do you need to tell them?

    Come back if...

     if your child has increasing stomach pains or swelling, blood in the stools or urine, or if you are worried for any other reason.

    What you need to do now....?

    BP and urinalysis with GP monthly for 6 months to ensure that if a kidney problem does arise it is picked up early.

    337

    What can you tell about this little girl?

    Foetal alcohol syndrome

    Most distintive features are thin upper lip and indistinct philtrum

    338

    What is persistent pulmonary hypertension of the neonate?

    What causes it?

    What is it?

    Delayed relaxation of the pulmonary vascular bed at birth means that the baby maintains it's foetal circulation (right to left shunt across the ductus arteriosus and foramen ovale ).

    What causes it?

    PPHN is seen in association with many neonatal respiratory diseases including:

    • lung hypoplasia (eg diaphragmatic hernia)
    • meconium aspiration syndrome
    • pneumonia
    • surfactant deficiency

    The syndrome may also be seen in the absence of triggering diseases.

    339

    What is the main drug used for PPHN?

    Nitric oxide (NO) 

    340

    This baby has just been born, he is on vigorous and is not crying. Should you stimulate him?

    No, meconium stained liquor suggests he could have mec in his oropharynx. Suction his oropharynx first before stimulating. Intubate immediately.

    341

    Babies usually lose weight in the first few days after birth. How much weight loss would make you worry though?

    If they lose 10% or more of their birth weight you would worry.

    342

    What is the dosage of paracetemol in paeds?

    15mg / kg

     

    So a 30kg 6 year old would get 450mg

    343

    What is a posterior urethral valve?

    How common is it?

    Why is it a problem?

     

    What is it?

    An abnormal leaflet of tissue exists in the urethra, causing an obstruction to urine flow. It is a developmental anomaly, present before birth and only affects boys.

    How common?

    PUV is rare, occurring in 1 in 4000-6000 boys

    Why is it a problem?

    Urethral blockage near the bladder makes it hard for the bladder to expel urine, so the bladder has to push harder to try to empty. This increases pressure in the urinary tract. The pressure may push the urine back through the ureters to the kidney and cause the ureters, kidneys and bladder to dilate (expand). 

     

    344

    What are the investigations, management and prognosis of posterior urethral valves?

    Investigations...

    • UEC
    • Ultrasound
    • Micturating cystourethogram (MCU) 
      • This is the definitive test for PUV. It is performed with a catheter placed through the urethra into the bladder. Contrast material is injected through the catheter to outline the bladder. An X-ray is taken which will show the shape of the bladder, whether there is any back flow up the ureters (reflux). Xrays are taken to identify the contrast outlining the urethra and any internal obstruction (valve). 
    • Cystoscopy 
      • This is both a confirmatory and therapeutic intervention. It is a surgical procedure, performed under anaesthesia. A small telescope is placed inside the urethra to identify the obstruction. Treatment of the valves can be performed at the same time. Other tests may be needed to check the function of the kidneys, the drainage, and to monitor treatment

    Management

    Catheter insertion then ablation via cystoscopy

    Prognosis

    About one third will develop kidney failure at some stage, requiring dialysis or renal transplant. 

    About one third of patients will have a degree of renal impairment, managed with medication and diet. 

    About one third of patients will have normal kidney function, but may have ongoing issues with urinary tract drainage, infection and bladder function.

    345

    What is the mortality rate of NEC?

    20-40% 

    346

    How much weight should babies gain per week in the first 3 months of life?

    An infant should gain 180g/week within the first three months of life 

    347

    What are the causes of failure to thrive?

    • PATIOS
    • Psychosocial
    • Absorption
      • CF
      • Coeliac
      • Milk allergy
    • Thyroid disease
    • Intake
      • Milk/formula supply
      • Sucking
      • GORD
    • Output --> diarrheoa
    • Sick

    348

    What do you know about innocent mumurs in childhood?

    An innocent murmur is a murmur arising from turbulent blood flow through a normal heart, for reasons which may be unclear.

    The 5 ‘S’s of an innocent murmur are:

    • innoSent
    • aSymptomatic
    • Soft
    • Systolic
    • left Sternal edge

    Innocent murmurs are very common and may be heard in around 50% of normal school aged children and approximately 80% of children in early infancy

    An important DDx from an innocent murmur is an atrial septal defect (ASD). Therefore must carefully listen for wide or fixed splitting of S2 before concluding that it is an innocent murmur.

    Innocent murmurs are made louder, and hence more easily detected, during an illness with a fever (as the heart works harder and this increases the turbulence in blood flow). If you hear the mumur during an illness wait until they've recovered then review it again.

    349

    What is intraventricular haemorrhage of prematurity?

    How does it present?

    How is it diagnosed?

    What are the different grades?

    What is the management?

    Immature capillary bed lining the ventricles / vascular fragility of the germinal matrix. Occurs within 48 hours of birth.

    Clinical presentation

    • Often asymptomatic
    • Tense fontanelles
    • Floppy / limp
    • Seizure activity
    • Decerebrate posture

    How is it diagnosed?

    U/S between 5 and 7 days of life. Repeat at 28 days of life

    Grades

    Grade I - bleeding occurs just in the germinal matrix
    Grade II - bleeding also occurs inside the ventricles, but they are not enlarged
    Grade III - ventricles are enlarged by the accumulated blood
    Grade IV - bleeding extends into the brain tissue around the ventricles

    Grades I and II are most common, and often there are no further complications. Grades III and IV are the most serious and may result in long-term brain injury to the infant. After a grade III or IV IVH, blood clots may form which can block the flow of cerebrospinal fluid, leading to hydrocephalus and then cerebral palsy, epilepsy, hearing/visual problems.

    Management

    Grade 1 and 2 have no treatment.

    Grade 3 and 4 --> VP shunt + follow up to monitor development

    350

    What is respiratory distress syndrome? 

    What is the clinical picture?

    How can you prevent it?

    How do you manage it?

     

     

    What is it?

    • Also known as hyaline membrane disease or surfactant deficiency syndrome
    • Due to immaturity of the type 2 pneumocytes --> surfactant deficiency --> alveolar collapse --> stiff lungs --> increased WOB --> decreased tidal volume --> hypoxaemia --> respiratory acidosis
    • Lung surfactant is only adequate by 30-32 weeks gestation
      • RDS affects 50% of neonates with GA
      • RDS affects almost 100% of those born
    • Causes:

      • Prematurity

      • Perinatal asphyxia (hypoxia inhibits surfactant production)

      • Maternal diabetes (delays surfactant synthesis)

    • RDS affects about 1% of newborn infants and, along with NEC, is the leading cause of death in preterm infants

    Clinical picture:

    Signs of increased WOB (increased RR, nasal flaring, use of accessory muscles, grunting)
    Hypoxaemia, cyanosis

    Whether treated or not, the clinical course for the acute disease lasts about 2 to 3 days. During the first day the patient worsens and requires more support. During the second day the baby may be remarkably stable on adequate support and resolution is noted during the third day, heralded by a prompt diuresis. 

    Prevention

    Antenatal betamethasone is used in the prevention of RDS, given to a mother expecting a premature baby (up to 24 hours before birth, should be given two courses. Unlikely to give beneficial effect if given >2 weeks prior to birth)

    Mx

    • Oxygen
    • Nasal CPAP
    • Surfactant administration (can be given down ETT!)
    • Mechanical ventilation (severe)
    • Non respiratory
      • Attention to thermoregulation can reduce the severity of the condition
      • Antibiotics commence penicillin and gentamicin therapy after initial investigations.

      • Minimal handling

      • No feeding until resolved

    351

    What is the dose of energy used for defibrillation of children?

    4j per kg

    352

    What is apnoea of prematurity?

    Why does it happen/what are the different types?

     

    Defined no respiratory effort for greater than 20 seconds. No respiratory effort for shorter periods of time may also be classified as apnoea if accompanied by cyanosis or bradycardia

    Why does it happen/what are the types?

    Central apnoea: (40%) Caused by decreased central nervous system stimuli to respiratory muscles. Both the respiratory effort and airflow cease simultaneously. (Absence of chest wall movement and airflow).


    Obstructive apnoea: (10%) Caused by pharyngeal instability / collapse, neck flexion or nasal obstruction.  Absence of airflow in presence of inspiratory efforts. (Presence of chest wall movement but no airflow).


    Mixed apnoea: (50%) Has a mixed aetiology. Central apnoea is either preceded (usually) or followed by obstructed respiratory effort

    NB: Short episodes of apnoea are usually central whereas prolonged ones are often mixed

     

    353

    Apneoa of prematurity

     

    When does it happen?

    What is the management?

    What is the prognosis?

    When does it happen?

    Onset is from days 2-7 of life.  Apnoea beginning immediately after birth suggests another cause.

    Management

    • Monitoring in SCN/NICU
    • Positioning:
      • Ensure the neonate's head and neck are positioned correctly (head and neck in neutral position) to maintain a patent airway.
    • Tactile stimulation:
      • Gentle rubbing of soles of feet or chest wall is usually all that is required for episodes that are mild and intermittent. 
    • Clear airway:
      • Suction mouth and nostrils.
    • Methyxanthines
      • caffeine or theophyllline
        • Improve diaphragm contraction and stimulates the respiratory centres
    • Nasal CPAP (reducing obstruction; reducing work of breathing)
    • Mechanical ventilation (if severe)

    Prognosis

    Since AOP is fundamentally a problem of the immaturity of the physiological systems of the premature infant, it is a self-limited condition that will resolve when these systems mature. It is unusual for an infant to continue to have significant problems with AOP beyond 42 weeks post-conceptual age.

    354

    What is the ductus venosis? What does it do?

    What is the clinical significance?

    In the fetus, the ductus venosus shunts a portion of the left umbilical vein blood flow directly to the inferior vena cava. Thus, it allows oxygenated blood from the placenta to bypass the liver. Compared to the 50% shunting of umbilical blood through the ductus venosus found in animal experiments, the degree of shunting in the human fetus under physiological conditions is considerably less, 30% at 20 weeks, which decreases to 18% at 32 weeks, suggesting a higher priority of the fetal liver than previously realized.

    Clinical significance

    You use the ductus venosis for venous access in resusc of the neonate.

    355

    How often is basic neonatal resusc required?

    How often is advanced neonatal resusc required?

    10%

    1%

    356

    Describe the APGAR scoring criteria.

    357

    What do you do with APGAR scoring if the 5min APGAR is 6?

    If less than 7 you score it every 5minute until it gets to 7

    358

    What is the nature of the relationship between the risk of cerebral palsy and prematurity?

    The risk is much greater in preterm babies, but if you pick a random kid with CP 60% of them will have been born at term

    359

    What is the most significant risk factor for cerebral palsy?

    HIE is the most common identifiable risk factor, but it contributes to less than a quarter of cases

    360

    What are the hallmark symptoms of cerebral palsy?

    non-independent walking

    non-independent feeding

    epilepsy

    blindness

    hearing problems

    Later then have learning difficulties and behavioural problems

    361

    What is the sensitivity of antenatal ultrasounds to congenital cardiac pathology?

    Varies from 60-95% depending on the skill of the clinician.

    Make sure where you send them have a Certificate of Obstetric and Gynaecological Ultrasound (COGU).

    362

    Why do paediatric surgeons see a lot of intussusception in winter?

    Viral illnesses which occur in winter cause swelling of Peyer's patches in the bowel which serve as pathological lead points for intussusception

    363

    What is Turner's syndrome?

    What are some of the key features

    XO females

    • short stature
    • infertility 
    • congenital heart defects
    • spatial awareness issues – problems with tasks such as maths but otherwise normal IQ
    • Amenorrhoea
    • Hearing problems
    • no breays
    • extra skin (‘webbing’) on the neck
    • puffy hands and feet
    • low hairline.

    364

    Spot diagnosis

    Turner's syndrome XO females

    • short stature
    • infertility 
    • congenital heart defects
    • spatial awareness issues – problems with tasks such as maths but otherwise normal IQ
    • Amenorrhoea
    • Hearing problems
    • no breays
    • extra skin (‘webbing’) on the neck
    • puffy hands and feet
    • low hairline.

    365

    What is Eisenmenger syndrome?

    In what cardiac conditions can it occur?

    Eisenmenger's syndrome is defined as the process in which a left to right shunt caused by a congenital heart defect in the fetal heart causes increased flow through the pulmonary vasculature, causing pulmonary hypertension, which in turn causes increased pressures in the right side of the heart and reversal of the shunt into a right-to-left shunt.

    Eisenmenger syndrome is a cyanotic heart defect characterized by a long-standing intracardiac shunt (caused by ventricular septal defect, atrial septal defect, or less commonly, patent ductus arteriosus) that eventually reverses to a right-to-left shunt. This syndrome is less frequent today because of medical screening withechocardiography early in life.

     

    Prognosis is poor if early intervention doesn't happen. After pulmonary hypertension has developed, a heart–lung transplant or a lung transplant with repair of the heart is a possible option.

    366

    A baby is born with imperforate anus as well as tetrology of Fallot. What does this make you think?

    The VACTERL association (also VATER association) refers to the non-random co-occurrence of birth defects 

    Vertebral anomalies, 

    Anal atresia 

    Cardiac defects 

    Tracheoesophageal fistula

     Esophageal atresia

    Renal & Radial anomalies

    Limb defects.

    The reason it is called an association, rather than a syndrome is that while the complications are not pathogenically related they tend to occur together more frequently than expected by chance. In general, the etiology of "associations" are not defined.

    367

    A baby is born with an umbilical hernia. You are able to easily reduce it, but of course coughing/crying pops it out again. What do you tell the parents?

    • It's very common
    • Very likely will self resolve
    • However, it will probably get bigger when the baby starts crawling as gravity takes it's course
    • If it hasn't resolved by age 2 get a surgical consult to consider repair
    • Tell parents warning signs
      • can't reduce
      • skin changes
      • pain (when able to talk)
      • constipation/vomiting late signs

    368

    How many puffs of salbutamol should you give a kid having an asthma attack?

    How often?

    When can you discharge?

    For kids under 6 = 6 puffs

    For kids over 6 = 12 puffs

    Give every 20mins as required, then stretch. Can discharge when stretched to 3 hourly

    369

    A child has come in to ED severely dehydrated and shocked. How do you calculate their IV fluids now and for the next 24 hours?

    • 10-20ml/kg boluses of normal saline until not shocked
    • Maintenance fluids of normal saline + 5% glucose according to 4,2,1 rule
    • Slowly correct deficit 
      • ignore bolus fluid volumes given
      • If severe shock assume 10% dehydrated
      • Give 10% of bodyweight in fluids
        • Give first half of this over the first 6 hours
        • Give the second half of this over the second 18 hours
        • You can do this more slowly if needs be

    370

    What is the most common presentation of worms in kids?

    How do you confirm the diagnosis?

    How are the worms spread?

    What is the management?

    What is the prognosis?

    Presentation

    Itching around the anus (pruritus ani). The itchiness tends to be worse at night and can cause trouble sleeping. The itching is caused by an inflammatory reaction to the adult worm and eggs on the skin.

    Diagnosis

    Sticky tape the kid's bum at night, examine the tape for worms/eggs.

    How are they spread?

    Through swallowing the eggs. When someone with pinworm scratches their anus, the eggs get lodged beneath their fingernails, and are then deposited on clothing and furniture. Uninfested individuals can come in contact with pinworm eggs and ingest them unknowingly. Infested individuals continue to ingest the eggs (auto-infection).

    Other family members are at particular risk of pinworm infestation.

    Management

    • Pyrantel (Combantrin) or mebendazole (Banworm).
      • Very safe medications
      • Treat the entire household
      • Shower the night before taking and the morning after. Wash all sheets in very hot water and vacuum the bedroom.
    • Have the child wash his/her hands with warm soapy water for at least 10 seconds after going to the toilet.

    Prognosis

    Reinfection is very common

    It's a mild disease though so doens't really matter

    371

    At what ages do absence seizures typically occur?

    4 to 14. It’s also possible for older teens and adults to have absence seizures, but it’s less likely.

    372

    What is the drug management of absence seizures?

    Either ethosuximide or sodium valproate

     

    Ethosuximide is used commonly to treat absence seizures. It is better tolerated than sodium valproate but has the disadvnatage of not controlling tonic-clonic seizures.

    Treatment is usually for 2 years only in typical childhood absence epilepsy, with an expectation of seizure remission, and through puberty in juvenile absence epilepsy, with later appearance of tonic-clonic seizures being a risk. 

    373

    What is a breath holding attack?

    • Attacks usually commence in the first or second year of life.
    • Crucial to the diagnosis is recognition that attacks are precipitated by either physical trauma, such as a knock or a fall, or emotional trauma such as fright, anger or frustration, the precipitants not always being significant and noticed.
    • Attacks usually commence with crying, but this may be brief or absent. Apnoea then occurs with cyanosis or pallor, and may then either terminate without apparent loss of consciousness, or progress with the child becoming unconscious, limp and sometimes briefly stiffening or jerking in response to the cerebral hypoxia. Incontinence may occur with such convulsive features.
    • Recovery is usually rapid, although some children are drowsy and lethargic after an attack with convulsive features. Attacks usually cease by the third or fourth year of life.
    • They are not a cause of death, epilepsy, intellectual disability or cerebral damage in later life.
    • The pathophysiology of breath holding attacks is not well understood, 

    374

    What is this?

    What is the significance?

    Salmon patch

    Also known as nevus simplex or "angel kisses", these are a common capillary malformations that are present at birth. Eyelid spots generally fade over several months. Lesions on the glabella may take several years to resolve, and occasionally the outlines can be seen into adulthood, especially when the face is flushed.

    375

    This is a newborn, what is the diagnosis?

    What is the prognosis?

    Milia are keratin filled epithethial cysts which occur in up to 40% of newborns. Spontaneous exfoliation and resolution is expected within a few weeks. Parents will occasionally mistake these lesion for neonatal acne, but milia are present at birth and have no inflammatory component.

    376

    When performing a newborn examination you feel a ridge over the suture sites adjacent to the anterior fontanelle. What is the diagnosis? What's the significance/prognosis of this diagnosis?

    Craniosynostosis

    One or more of the fibrous sutures in an infant skull prematurely fuses by turning into bone (ossification), thereby changing the growth pattern of the skull. Because the skull cannot expand perpendicular to the fused suture, it compensates by growing more in the direction parallel to the closed sutures.

     

    Sometimes the resulting growth pattern provides the necessary space for the growing brain, but results in an abnormal head shape and abnormal facial features. In cases in which the compensation does not effectively provide enough space for the growing brain, craniosynostosis results in increased intracranial pressure leading possibly to visual impairment, sleeping impairment, eating difficulties, or an impairment of mental development combined with a significant reduction in IQ.

    377

    Can you visualise and name the newborn fontanelles and sutures?

    When do they close?

    Anterior fontanelle closes at 18-24 months

    Posterior fontanelle closes at 3 months

    378

    In what layers do cephalhaematomas occur?

    Where on the head do they normally occur?

    What do they look/feel like on examination?

    What causes them?

    What is the management?

    Layers

    Under periosteum of skull bone but superficial to skull

    Where on the head

    Usually parietal bones, because this is where forceps attach

    What do they look/feel like?

    Margins are firm w/ a soft fluctant centre. They do not cross suture lines (help in by periosteum).

    What causes them?

    The usual causes of a cephalohematoma are a prolonged second stage of labor or instrumental delivery, particularly ventouse.

    Management

    Phototherapy may be necessary if blood accumulation is significant leading to jaundice. Rarely anaemia can develop needing blood transfusion. Do not aspirate to remove accumulated blood because of the risk of infection and abscess formation. The presence of a bleeding disorder should be considered but is rare. Skull radiography or CT scanning is also used if concomitant depressed skull fracture is a possibility. It may take weeks and months to resolve and disappear completely.

    379

    What are the differences between caput succedaneum and cephalhaematoma?

    380

    What is this? What layers are involved?

    What does it look/feel like on examination?

    What is the management?

    Caput succedaneum is a neonatal condition involving a serosanguinous, subcutaneous, extraperiosteal fluid collection with poorly defined margins caused by the pressure of the presenting part of the scalp against the dilating cervix (tourniquet effect of the cervix) during delivery.

    It involves bleeding below the scalp and above the periosteum.

    Features on examination

    Caput succedaneum presents as a scalp swelling that extends across the midline and over suture lines and is associated with head molding.

    Management

    Caput succedaneum does not usually cause complications and usually resolves over the first few days.

    381

    What is a subgaleal haematoma? What are the features on clinical exam?

    What causes it?

    What is the management?

    What are the complications?

    What is it?

    Subgaleal hemorrhage or hematoma is bleeding in the potential space between the skull periosteum and the scalp galea aponeurosis.

    It presents as a fluctuant boggy mass developing over the scalp (especially over the occiput) with superficial skin bruising. The swelling develops gradually 12–72 hours after delivery, although it may be noted immediately after delivery in severe cases. The hematoma spreads across the whole calvaria as its growth is insidious and may not be recognized for hours. If enough blood accumulates a visible fluid wave may be seen.
    Cause

    The majority (90%) result from applying a vacuum to the head at delivery (Ventouse assisted delivery). 

    Management

    Management consists of vigilant observation over days to detect progression.

    Complications

    The subgaleal space is capable of holding up to 50% of a newborn baby's blood and can therefore result in acute shock and death. Fluid bolus may be required if blood loss is significant and patient becomes tachycardic. Transfusion and phototherapy may be necessary. Investigation for coagulopathy may be indicated.

     

    382

    Spot diagnosis?

    Management?

    Ankyloglossia

    May require surgery if severe

    383

    Spot diagnosis?

    Why is this diagnosis important?

    Pierre Robin Sequence

    microganthia and cleft palate can cause severe respiratory problems

    384

    At what age is cerebral palsy usually diagnosed?

    Around 18 months

    385

    What are some of the indicators that an infant may have cerebral palsy?

    • Can't hold their head up
    • Feels floppy when picked up
    • Unusual posturing
    • Stiff muscles
    • Delayed developmental milestones
      • rolling
      • sitting
      • standing
      • walking

    386

    Cerebral palsy

    Define dystonia, chorea, athetosis and ataxia

    Dystonia: characterized by involuntary, sustained contractions

    Chorea: rapid, involuntary, jerky and fragmented motions

    Athetosis: slower, constantly changing, writhing or contorting movements 

    Ataxia: loss of muscular coordination with abnormal force and rhythm and impairment of accuracy

    387

    Name and describe the four types of cerebral palsy

    Spastic cerebral palsy: - Motor cortex lesion (86%)

    • stiffness or tightness of muscles on movement
      • Monoplegia – single limb involvement
      • Hemiplegia: involvement of an ipsilateral upper and lower extremity
      • Diplegia: predominant involvement of both lower extremities, greater than upper limbs
      • Quadriplegia: involvement of all limbs and trunk.

    Dyskinetic - Basal ganglia lesion

    • involuntary, recurring and occasionally stereotyped movements with a varying muscle tone.
    • Dystonia: characterized by involuntary, sustained contractions
    • Chorea: rapid, involuntary, jerky and fragmented motions
    • Athetosis: slower, constantly changing, writhing or contorting movements

    Ataxic - Cerebellar lesion

    Loss of muscular coordination with abnormal force and rhythm and impairment of accuracy

    Mixed 

    388

    What is the most common cause of cerebral palsy where the condition is acquired after 1 month of age?

    Stroke

    389

    What is the incidence of cerebral palsy?

    1 in 500 Australian babies is diagnosed with cerebral palsy.

    390

    What % of people with cerebral palsy have an intellectual disability?

    50%

    391

    What are the risk factors for cerebral palsy?

    • Antenatal (70-80% of cases)
      • premature birth (less than 37 weeks)

      • low birth weight (small for gestational age)

      • maternal thrombophilia

      • placental abruption

      • TORCH infections

    • Perinatal
      • RH or A-B-O blood type incompatibility between mother and baby

      • Asphyxia during birth (contributes to less than 10% of cases)

    • Postnatal
      • hyperbilirubinaemia,
      • neonatal sepsis,
      • respiratory distress,
      • early-onset meningitis,
      • intraventricular haemorrhage,
      • head injuries prior to 3 years (including child abuse and shaken baby syndrome).

    392

    List the childhood seizure disorders?

    393

    Childhood absence epilepsy. What is the?

    Age of onset

    Aetiology

    Primary seizure activity

    Associated with other seizure types

    Treatment

    Prognosis

    4 – 9 years

    Idiopathic but strong genetic component

    Absence seizures (can be up to 100s per day)

    It is rare for children to have other seizure types at first, but about 40% of children with childhood absence epilepsy develop tonic-clonic seizures as well.

    Ethosuximide and sodium valproate are equally effective, but ethosuximide is better tolerated. However, unlike sodium valproate, ethosuximide does not protect against associated generalised tonic-clonic seizures.

    Usually resolves two to five years after the seizures begin or when the child is a teenager

    394

    Juvenile absence epilepsy. What is the:

    Age of onset

    Aetiology

    Primary seizure activity

    Associated with other seizure types

    Treatment

    Prognosis

    10 – 15 years

    Idiopathic but strong genetic component

    Absence seizures (less frequent that childhood absence epilepsy)

    80% of children with juvenile absence epilepsy also have tonic-clonic seizures. These may begin later than the absence seizures.  Some also have myoclonic seizures and absence status epilepticus.

    Ethosuximide and sodium valproate are equally effective, but ethosuximide is better tolerated. However, unlike sodium valproate, ethosuximide does not protect against associated generalised tonic-clonic seizures.

    We don’t have a lot of data. The seizures usually respond well to anti-epileptic drugs, but children with this syndrome may need to take medication to control seizures all their lives.

    395

    Juvenile myoclonic epilepsy. What is the:

    Condition

    Age of onset

    Primary seizure activity

    Associated with other seizure types

    Treatment

    Prognosis

    15-19

    Myoclonic jerks that occur on awakening from sleep either in the morning or from a nap. They are typically described as shock-like, irregular and arrhythmic movements of both arms. Sometimes these movements are restricted only to the fingers making the patient or individual look clumsy or prone to dropping things.

    They ALL have tonic clonic seizures

    One third have absence seizures

    sodium valproate; however, avoidance of sleep deprivation and excessive alcohol may also be necessary to achieve complete freedom from seizures. 

    Although full remission is unlikely and most patients require lifelong treatment, the prognosis for seizure control is very good. Most patients remain neurologically normal

    396

    Benign childhood epilepsy with centrotemporal spikes. What is the:

    Age of onset

    Primary seizure activity

    Associated with other seizure types

    Treatment

    Prognosis

    2 - 13

    Seizures usually occur during sleep and begin in the face or mouth, producing a typical glugging sound. Speech arrest is common. The partial (focal) seizure may become secondarily generalised.

    The EEG is typical and shows epileptiform discharges in the centrotemporal region.

    Carbamazepine, sodium valproate and sulthiame are the drugs of choice. Treatment is not always indicated. 

    most children are seizure-free by early adolescence

    397

    When trying to determine if a child has appendicitis, what one question should you ask to help rule out an important differential?

    Has the child had an URTI or other illness recently -- is this mesenteric adenitis!?

    398

    What are the most common causes of failure to thrive?

    PATIOS

    • Psychosocial
    • Absorption
      • CF
      • Coeliac
      • Milk allergy
    • Thyroid disease
    • Intake
      • Milk/formula supply
      • Sucking
      • GORD
    • Output --> diarrheoa
    • Sick

    399

    What history questions would you ask the parents of a baby with failure to thrive?

    400

    What should a baby be eating? How much?

    When do you introduce solids?

    Breastfeeding every 2 to 3 hours for 15mins per breast

    May be less on formula.

    Around 60ml per time but massive variation. Around 800ml per day

     

    Start to introduce solids at 6 months

    401

    What questions do you ask in antenatal and birth histories?

    • Antenatal
      • Normal scans?
      • Complications during pregnancy? High blood pressure or sugars?
      • Blood type and anti D
      • GBS status
    • Birth
      • Gestational age
      • NVB or LUSCS
        • Instrumental (forceps/vacuum(
      • PROM?
      • Did baby need resus? Prolonged hospital stay?
      • Heel prick test done?
      • Prolonged jaundice?
         

    402

    What gene mutation accounts for 95% of cystic fibrosis?

    What chromsome is it on?

     Cystic fibrosis transmembrane conductance regulator (CFTR) ΔF508  

    Chromosome 7

    403

    What is this?

    What are the other symptoms?

    Who gets this condition?

    What is the management?

    What are the complications?

    Measles

    What are the symptoms?

    • High fever
    • Irritability
    • Exudative conjunctivitis
    • Rhinorrhea
    • Otitis media
    • Exanthem = mobilloform, blotchy and raised
    • Enanthem = Koplik’s spots

    Who gets it?

    In developed countries --> unimmunised

    In developing countries --> Malnourished and Vit A deficient

    Management

    Report, isolate, supportive

    Complications

    Complications can include otitis media, pneumonia and encephalitis. Measels specific IgM or IgG

    404

    What is burst therapy for asthma?

    6 puffs every 20 minutes x 3 if <6 yo

    12 puffs every 20 minutes x 3 if > 6 yo

    405

    What are the systems questions for asthma?

    FIT CAMPSITES

     

    F - Frequency and severity of attacks

    I - Interval symptoms?

    T - Technique using inhaler

    C - Compliance with preventer

    A - Atopy - personal or family history

    M - Medications, past and current

    P - Pets in the house

    S - School missed

    I - ICU or hospital admissions

    T - Triggers

    E - Exercise tolerance

    S - Smokers in the house

    406

    What are the potential complications if an undescended testis is not corrected by orchidopexy?

    • Testicular torsion.
    • Inguinal hernias through the patent processus vaginalis
    • Lower fertility - the body temperature in the abdomen is higher than in the scrotum. This can affect sperm production in the testes.
    • Risk of testicular cancer. The risk is usually less than 1 in 100.
    • Poor self esteem.

    407

    What is the correct term for undescended testis?

    What are the two types?

    Cryptorchidism, pronounced kript-ork-id-iz-im

     

    Congenital

    • In premature babies, the testes may not have dropped down yet (usually happens in the eight month of pregnancy).
    • Some hormone and genetic disorders can cause undescended testes
    • Usually idiopathic

    Acquired - boys who develop undescended testes after birth.

    • This can happen between 1-10 years of age.
    • The testes are in the scrotum at birth
    • As the boy grows the cord attached to the testes (spermatic cord), fails to grow at the same rate.  It ends up too short and pulls the testes back up into the groin.

    408

    What is the management of congenital Cryptorchidism?

    409

    What is the management of acquired Cryptorchidism?

    410

    How can you ask a developmental history in a child under 3?

    Is he walking?

    If no --> How does it get around?

    If yes --> does he walk steadily? Does he run? Does he jump?

    Is he talking?

    If no --> does he babble?

    If yes --> how many words? What does he say?

    Does he point to things to show you?

    If no --> does he wave bye bye?

    How does he hold small objects?

    Does he draw?

    411

    A mother has a baby born at 30 weeks. She asks you what the complications of prematurity are and what you will do about them. What do you say?

    SHIRTFRONT LAD AJ


    Sepsis
    Hypoglycaemia (<2.6)
    Intraventricular haemorrhage
    Respiratory distress syndrome/Meconium ileus syndrome
    Transient tachypneoa of the newborn
    Feeding difficulties
    Retinopathy of prematurity
    Osteopaenia of prematurity
    NEC
    Temperature instability


    Leukomalacia (periventricular)
    Anaemia / Fe deficiency
    Developmental problems (ID, cerebral palsy)


    Apneoa of prematurity
    Jaundice

    412

    What are the causes of IUGR

    Foetal

    Chromosomal disorders

    Congenital anomalies

    Multiple gestation

    TORCH infection

    Placental

    Uteroplacental insufficiency

    Abnormal implantation

    Placental abruption

    Maternal

    Chronic illness

    Pre-eclampsia

    Early or advanced age

    Malnutrition

    Substance abuse (e.g. cigarettes, alcohol, narcotics, cocaine)

    Medications (e.g. warfarin, anticonvulsants)

    413

    What are the three different forms of spina bifida?

    414

    A foetus is diagnosed with spina bifida on antenatal ultrasound. The parents ask you what caused the condition, what do you say?

    The aetiology of NTDs is still debated. Polygenic inheritance and environmental and teratogenic factors have been implicated.

    It has been demonstrated unequivocally that vitamin supplementation with folic acid reduces the incidence of recurrence in high-risk populations. Dietary factors may therefore play a major part in low-risk populations.

    415

    What do you know about Trisomy 18?

    • Edwards syndrome occurs in around one in 6,000 live births, and around 80% of those affected are female.
    • The majority of fetuses with the syndrome die before birth.
    • The incidence increases as the mother's age increases.
    • The syndrome has a very low rate of survival, resulting from heart abnormalities, kidney malformations, and other internal organ disorders.

    416

    What do you know about CMV infection?

    It's a herpes virus. Almost all adults are infected.

     

    In healthy people, CMV infection causes nothing more than a flu-like illness that lasts a few days. In certain people, however, including transplant patients and pregnant women, the effects can be much more serious.

     

    Symptoms may vary depending on which organ is affected, but generally a person with CMV will experience lethargy, a high temperature and a drop in white blood cell levels. For most people, a CMV infection poses no real health threat. The symptoms are generally mild and flu-like, although some may feel unwell for a few weeks, rather than a few days. Some people experience no symptoms.

     

    Antiviral medications may be used in pregnant or immunocompromised people.

    417

    A midwife asks you to come and review a baby who has a prominent sacral dimple, she is concerned about spina bifida. What questions do you ask to determine how likely this may be?

    • How low down the back is it? (the higher the more likely it is to be pathological)
    • Is it hairy?
    • Does it have any skin tags?
    • How deep is it?

    418

    What is the most common form of stridor in infancy?

    Laryngomalacia

    419

    Laryngomalacia

    What is the presentation, management and prognosis?

    Stridor in infancy, abnormal voice

    Close monitoring, 85% chance will resolve by age 2 as cartilage hardens.

    Laryngomalacia becomes symptomatic after the first few months of life( 2-3 months), and the stridor may get louder over the first year, as the child moves air more vigorously. Most of the cases resolve spontaneously and less than 15% of the cases will need surgical intervention.

    420

    A child presents with petechiae. What questions do you ask to rule in/our your differentials?

    SHIELD

    Sepsis (meningococcal)

    conscious state?
    fever?
    headache, photobia or neck stiffness?
     

    Henoch-Schonlein Purpura/HUS

    haematuria or bloody diarrhea
    arthritis
    abdominal pain
    vomits

     

    Event (non-accidental injury or accident)

    Was the event witnessed

    Any other injuries

    Did they present straight away?

     

    ITP

    Recent URTI?

     

    Leukaemia

    pale, SOB or tired

    fevers, nightsweats, weight loss
    severe, persistent, unusual or recurrent infections
    lumps or bumps

     

    DIC

    seriously unwell

    421

    You diagnose a child with ITP and advise conservative management, the parents have researched the condition on google and ask, "Wouldn’t steroids help? Why can’t we use them?”. What do you say?

     

    Steroids can accelerate the recovery of platelets, BUT:

    • most children with recover spontaneously
    • Side effects are common and include (name at least 3)
      • Gastric irritation
      • Behaviour change irritability and sleep disturbance
      • Transient diabetes and weight gain
      • Immunosuppression
      • Moon face and buffalo hump fat distribution
      • Growth delay
      • Bone weakness

    422

    You diagnose 7 year old Annie with ITP. She has a platelet count of 35. You decide to manage her conservatively. What do you tell her mum?

    • Annie should avoid contact sports or anything that could induce bleeding
    • No aspirin or ibuprofen
    • Annie should be supervised if you feel she cannot look after herself
    • Level of activity should increase as platelets rise

    423

    What is this?

    What is the management?

    Naevus simplex

    • Aka “salmon patch” or “stork mark”
    • Pale pink to bright-red capillary vascular malformations
    • Indistinct borders that blanch and become more prominent with crying and straining
    • Commonly affects the forehead, glabella, upper eyelids and nape
    • Prevalence 20-60%
    • Most spontaneously disappear between age 1-3
    • Thought to be related to immaturity of the vasculature
    • Treatment usually  not required

    424

    What is this?

    What is the management?

    Naevus flammeus - Aka “port wine stain”

    • Capillary vascular malformations
    • Unilateral, homogenous, red-to-violaceous macules involving the skin and sometimes mucosa.
    • Darkens and thickens over time
    • Incidence 0.3%
    • Can occur anywhere but many involve the face and follow the distribution of the trigeminal nerve
    • Largely a cosmetic concern but may be a marker for ophthalmologic or neurologic structural abnormalities
    • Further investigation is warranted.
    • Sturge-Weber syndrome = facial naevus flammeus + an ipsilateral vascular malformation of the leptomeninges and eye.
      • Location of the naevus helps identify newborns at risk of underlying abnormalities.
        • Parents of patients without V1 involvement may be reassured and informed about vascular laser as an option for treatment.

    425

    What is this?

    What is the management?

    • Infantile haemangioma - Aka “strawberry naevus”
    • Benign vascular tumours
    • Occur in 5-10% of newborns. More common in premature infants
    • Often unapparent at birth, they appear in the first few weeks of life, proliferate rapidly for several months, and then gradually fade over several years.
    • Segmental haemangiomas have a linear or geographic distribution and are more likely to have associated structural abnormalities
    • Those that are large or occupy the airway, eyes and vital organs can cause serious or life threatening complications. Prompt specialist referral is required.
    • ​Prognosis
      • Resolution is considered complete in 76% of children by age 7.
      • 50% will have minor residual changes such as telangiectasia or wrinkling.
    • Treatment goals – to prevent serious complications and permanent disfigurement.
      • 1st line therapy = systemic prednisolone during the proliferative phase.
      • 2nd line therapy = propanalol
      • Laser therapy can sometimes also be an option

    426

    What is this?

    What is the management?

    Café au lait macules

    • Usually noted in infancy and childhood.
    • Solitary CALMs are common in up to 3% of healthy infants and 25% of children.
    • Multiple CALMS are rare in healthy children.
    • Majority of children with 6 or more CALMs will eventually be diagnosed with neurofibromatosis-1 (NF-1) potentially affecting multiple organ systems.
    • Children with 3 or more CALMs should be monitored for other features of NF-1
    • CALMs may also be seen in multiple other rare syndromes

    427

    How can you remember the viral exanthems of childhood?

    First         = Measles (because it is the first named virus in the MMR vaccine)
    Second    = Scarlett fever, caused by Streptococcus (S,S,S)
    Third        = Rubella aka ‘3 day measles’ (the third named virus in the MMR vaccine
    Fourth      = doesn’t exist
    Fifth          = Parvovirus B19
    Sixth         = Roseola

     

    428

    What are the features of Scarlett fever?

    Due to group A beta-haemolytic streptococci. An abrupt onset of fever, headache, vomiting, malaise and sore throat. Show a mobilliform exanthem and a “strawberry tongue” (bright red oral mucosa and palatal petechiae).

    429

    This photo is of a newborn. What is the diagnosis and management?

    Erythema toxicum neonatorum

    • Most common rash in infants, seen in up to 50% of newborns
    • Begins as blotchy macular erythema
    • Progresses to pustular/papular rash over trunk, face & extremities
    • Usually appears on 2nd/3rd day of life, can appear as late as 2-3 weeks
    • Distinguished from infection by lack of tenderness, warmth, or induration
    • Cause unknown
    • Self-resolving, typically by 5-7 days after appearance, usually by 2 weeks age
    • Requires no treatment

    430

    What is the diagnosis?

    What are the 2 forms of this condition?

    What is the causative organism?

    What is the management?

    Diagnosis and forms

    Impetigo is a highly contagious superficial, contagious, blistering infection of the skin also called “school sores” because it most often affects children.

    It has 2 forms: non-bullous and bullous. Bullae are fluid-filled lesions of >0.5 cm in diameter.

    Causative organism

    Impetigo in patients from affluent communities is most commonly caused by Staphylococcus aureus, and less commonly by Streptococcus pyogenes. In patients from socioeconomically disadvantaged areas, such as remote Indigenous communities in central and northern Australia, S. pyogenes is often the primary pathogen.

    Management

    • In non-remote community settings suspect S. aureus as the pathogen.
      • For localised skin sores, use:
        • mupirocin 2% ointment or cream
      • ​For multiple skin sores or recurrent infection, use:
        • di/flucloxacillin 500 mg
    • In remote community settings in central and northern Australia suspect S. pyogenes as the pathogen.
      • benzathine penicillin 900 mg

    431

    A junior medical student asks you what the differences are between a caput, cephalhaematoma and subgaleal haemorrhage. What do you say?

    432

    What is this condition?

    How common is it and what causes it?

    What is the management?

    Congenital talipes equinovarus (club foot)

    1 in 1000 births, congenital

    Management

    Basics

    Prenatal education, reassurance!

    Place and person

    Refer to ortho

    Treatment should commence in the first week of life.

    Definitive management

    • Ponseti technique
      • 4-6 weeks of toe to groin plaster casts
      • Changed weekly with gentle manipulation of the foot
    • Following casting, most cases (80%) require an Achilles tenotomy to correct residual deformity.  This can be done under local anaesthetic by an orthopedic surgeon in the clinic. 
    • Boots and bar abduction brace
      • Worn 23 hours a day for 3 months and then 14–16 hours, while asleep, until the child's fourth birthday.
    • Rarely, more aggressive surgical procedures need to be done – posterior medial release.  This is more common in patients with comorbidities such as cerebral palsy.

    Long term

    The child is monitored every 3 to 6 months until 4 years of age.  The child is then brought into the clinic every 1 to 2 years until reaching skeletal maturity. 

    433

    What is the incidence of down syndrome?

    1 in 1000 live births

     

    434

    Which viruses cause the most severe cases of bronchiolitis?

    RSV and influenza

    435

    Describe the management of acute asthma in a child, including what drugs you would use.

    436

    What drugs can you use for ADHD?

    What % of kids will respond to the first drug you prescribe for ADHD? What % will respond to the 2nd or 3rd drug you try?

    First line

    Methylphenidate (Ritalin - short acting, Concerta - long acting)

    Dexamphetamine

    Second line

    Atomoxetine

    Vyvanse (lisdexamfetamine)

    Response rates

    75% will respond to first drug, 95% will respond to 2nd or 3rd

     

     

    437

    What questions would you ask to determine if a child has ADD/ADHD?

    Firstly, they need to have inattention, hyperactivity and/or impulsivity for more than 6 months

    Two sets of DSM criteria, ADD/innattention set and ADHD/hyperactivity set

    ADD/innatention set - 6 or more of ADHD DSM DX

    • Absent-minded- forgetful in daily activities
    • Devil is in the details – overlooks details, inaccurate work
    • Hopeless at finding things – bit of forcing for the mnemonic
    • Derailable – starts things but gets sidetracked
    • Daily activities forgetfulness (chores, errands, appointments)
    • Sustained attention
    • Mind elsewhere when spoken to directly
    • Disorganised (sequential tasks, time maagement etc.)
    • Xternal stimuli is distracting

    ADHD/hyperactivity set - 6 or more of PPQRRSSTT

    • Pre-emptive conversation – doesn’t wait for appropriate turn in convos
    • Pushy – butts into conversations, doesn’t ask for permission, intrude into activities
    • Quiet play impossible
    • Restless – can’t stay still for long periods
    • Runs and climbs inappropriately
    • Squirms and fidgets
    • Seat leaving inappropriately
    • Taking turns is difficult
    • Talks excessively

     

    438

    A parent brings their 2.5 year old to see you because her childcare workers have said that she has speech delay. She says words but does not say 2 word sentences. What things do you consider when assessing the child?

    • Is this global delay? Check gross/fine motor, social and other domains of development on the Denver chart
    • Is this an expressive, receptive or mixed picture? Receptive MUCH more concerning.
    • Is there a hearing issue?
    • Is this autism? Use an autism screening questionnaire to check
    • Is there a family history of delayed speech - highly heritable phenomenon

    439

    A parent brings their 7 year old to see you because his teacher thinks he has a learning disorder. You know that this is a complex condition with many different causes. How do you organise your thoughts as to what might be going on?

    440

    An ex-35 weeker is now term and ready for discharge when you notice a moderate sized, reducable inguinal hernia. You refer the baby to the surgeons. How quickly would you recommend that the repair is carried out?

    1-2 weeks

    441

    New parents ask you whether they should fork out the extra cash for more expensive formula brands/types or if it's all the same. What do you say?

    If you can afford it go the more expensive ones. There's no hard evidence but theoretically it helps brain function.

    442

    A 5 week old is brought in to ED due to persistent vomiting for the past 3 days. She has been slightly more irritable than normal but otherwise fine. Physical examination and vital signs are unremarkable. Bloods show very mild metabolic acidosis, abdo ultrasound is clear. What is the most likely diagnosis?

    UTI.

    Urinary sepsis is a very common cause of this presentation and (annoyingly) the kids are often afebrile.

    443

    A term baby born by NVD is now at day 3 of life. He has had no complications and looks well. His mum is desperate to take him home to show the family. But just as they are about to leave bub vomits up a decent amount of blood. 

    What diagnosis do you expect?

    How can you confirm it?

    Is the baby safe to go home?

    Swallowed blood from cracked nipples OR from the delivery

    "Apt test" to determine if the blood is foetal or maternal

    If maternal blood safe to ignore and discharge the bub as planned

    444

    What are the differences between cows milk protein allergy and lactose intolerance?

    445

    A married couple fall pregnant with their first child who is found antenatally to have trisomy 21. They are fundamentalist Christians and are thus opposed to termination. When the child is born they ask what MEDICAL management the child will require. What do you tell them?

    COMETH said the lord, you say.

     

    C – Coeliac screening

    O – obesity, prevention of

    M – MSK*

    E – eye exam and echo at birth and every 3 years

    T – TSH annually (high risk hypothyroidism)

    H – hearing test as newborn every 6 months till age 4

     

    *Education regarding atlanto-axial instability: avoid trampoline and high risk activities. Discuss positioning of the spine during anasthaesia/surgery. Low threshold for imaging although routine spinal xray is not recommended.

    446

    What colour is bile stained vomit in a neonate?

    Green

    447

    At what age would the presence/persistence of the newborn primitive reflexes make you worried about neurological damage/disease?

    6 months

    448

    A 2 yo boy presents to you with some PR bleeding. He is well, running around the ED playing with his trucks. Physical exam is normal except for a mild tachycardia. What is the diagnosis?

    Meckel's diverticula

    449

    A child presents with a moderate-severe asthma attack. The consultant instructs that they should be prescribed the standard course of prednisolone for such situations. What you prescribe?

    2mg/kg for Day 1

    1mg/kg for Day 2 and 3

    450

    How do you calculate the size of a baby's fontanels?

    What is a normal size?

     

    Size is calculated by the average of the anteroposterior and transverse dimensions

    On the first day of an infant's life, the normal ANTERIOR fontanel ranges from 0.6 cm to 3.6 cm, with a mean of 2.1 cm.

    At birth, the average size of the POSTERIOR fontanel is 0.5 cm

     

    451

    What are the most common causes of a large fontanel?

    The most common causes of a large anterior fontanel or delayed fontanel closure are:

    • achondroplasia (a form of dwarism)
    • hypothyroidism,
    • Down syndrome,
    • increased intracranial pressure, and
    • rickets. 

    452

    What are the symptoms and signs of heart failure in an infant/young child?

    • Symptoms
      • Breathless during feeding
      • Excess sweating from sympathomimetic activation
      • Cold hands and feet
      • Recurrent chest infections from L->R shunt causing pulmonary congestion
      • FTT
    • Signs
      • Murmur
      • Hepatomegaly
      • Tachycardia and tachypneoa
      • Dependent oedema (need to press down for a while to appreciate it)
      • clubbing

    453

    A child presents to ED with oedema. The consultant you're reporting to asks you if you think it is of renal or cardiac origin. How would you tell the difference?

    • Renal
      • Hypo-osmotic oedema so affects areas with interstitial tissues such as face and groin
      • Worse in morning, gets better over the course of the day
    • Cardiac
      • Orthostatic oedema so affects dependent areas
      • Worse in the evening and after exertion
      • Typically resolves overnight

    454

    What is the difference between membranous and muscular VSDs?

    Different systems for describing the location of ventricular septal defects are used. Some are located in the lower portion of the septum called the muscular septum. Defects in this location are called muscular ventricular septal defects. Perimembranous ventricular septal defects (also called membranous VSD'S) are located in the membranous septum, a relatively small portion of the septum located near the heart valves.

    455

    An eager midwife pages you because she auscultated the heart of a baby born this morning and she thinks that it has a VSD murmur. What do you say?

    VSD murmurs aren't audible on D1 of life. The reason babies have a six week check is to look for these kinds of mumurs.

     

    At day 1 there is no pressure differential between the L and R heart. This means that there is no flow between the two, which means that there is no murmur.

    456

    A baby is born with a VSD, but unfortunately the mumur is not picked up at the 6 week checkup. How might the problem be identified later?

    Murmur may be heard at future assessments

    Infection (chest infections from increased pulmonary circulation, cerebral abscess or IE)

    Decreased exercise tolerance

    Failure to thrive

    Other signs/symptoms of heart failure

    457

    You hear fixed splitting in the second heart sound of an infant. What does this make you think?

     

    What symptoms may the child have?

     

    What should you do?

    Is there an ASD? In infancy this could just be a patent formen ovale that could self resolve. But ASDs typically don't self resolve.

     

    ASDs are typically less symptomatic than VSDs and are usually just picked up on clinical exam. They can however get chest infections.

     

    Do an echo to see if it's PFO or ASD. If it's still there at 1 year refer for surgical repair to avoid long term pulmonary hypertension.

    458

    What do you know about [Na] in DKA?

    Dilutional hyponatremia occurs because of shift of water from the intracellular to the extracellular compartment secondary to hyperglycemia and increased plasma osmolality.

    To "correct" sodium concentration, use the following formula:
    Corrected (i.e. actual) Na = measured Na + 0.3 (glucose - 5.5) mmol/l
    i.e. 3 mmol/l of sodium to be added for every 10 mmol/l of glucose above 5.5 mmol/l.

    If Na is > 160 mmol/l, discuss with senior doctor.

    If sodium does not rise as the glucose falls during treatment or if hyponatraemia develops, it usually indicates overzealous volume correction and insufficient electrolyte replacement. This may place the patient at risk of cerebral oedema.
     

    459

    In broad terms, what insulin regimes do you recommend for kids with T1DM?

    460

    A child presents with a sore testicle. You suspect it is epididimoorchitis. What are the two causes of this condition you must now search for?

    UTI or STI

    461

    A very experienced midwife calls you in the middle of the night to say a 20hour old baby looks sick. The baby had PROM and it's mum's GBS status is unknown. What antibiotics do you prescribe?

    Presumed neonatal sepsis ABx are BenPen and Gentamycin

    462

    You ask a patient with asthma how often they use their salbutamol. How often is acceptable before you would consider boosting their preventer therapy?

    >2 times per week is an indication for upping the preventer therapy

    463

    What are the features of mycoplasma pneumonia in a child?

    • Bilateral CXR changes, can mimic a viral picture
    • >5 days of cough
    • Not THAT sick, but has systemic symptoms

    464

    What are the features of a staph aureus pneumonia in children?

    • Very sick, unwell child
    • CXR shows abscess, empyema and/or effusion
    • CXR shows gas blebs

    465

    Describe the RCH flowchart management of pneumonia in children? What antibiotics do you use?

    Generally speaking BenPen IV (or amoxycillin PO)  

    But if suspect mycoplasma roxithromycin for 10 days

    If suspect Staph Fluclox plus Gentamycin

    If under 3 months add Gent

    466

    You are treating a febrile 7yo girl as an inpatient. She has increased WOB and you're trying to work out why. You suspect pneumonia but the CXR seems normal. What do you think?

    Pneumonia severe enough to cause increased WOB will be visible on CXR.

    Thus in this case you can essentially rule out pneumonia.

    467

    A 4 year old boy is brought in by his parents because they are concerned he may have an austistic spectrum disorder. List as many questions as you can that you would ask them to screen the child for autistic behaviours/traits.

    SOCIAL INTERACTION DIFFICULTIES (with same age peer)     

    1. Poor eye contact, or staring from unusual angle          

    2. Ignores when called, pervasive ignoring, not turning head to voice  

    3. Excessive fear of noises (vacuum cleaner); covers ears frequently  

    4. In his/her own world (aloof)     

    5. Lack of curiosity about the environment          

    6. Facial expressions don't fit situations    

    7. Inappropriate crying or laughing          

    8. Temper tantrums, overreacting when not getting his/her way         

    9. Ignores pain (bumps head accidentally without reacting)     

    10. Doesn't like to be touched or held (body, head)       

    11. Hates crowds, difficulties in restaurants and supermarkets

    12. Inappropriately anxious, scared          

    13. Inappropriate emotional response (not reaching to be picked up)

    14. Abnormal joy expression when seeing parents         

    15. Lack of ability to imitate

     

    SPEECH AND LANGUAGE DELAY

     1. Loss of acquired speech 

    2. Produces unusual noises or infantile squeals  

    3. Voice louder than required        

    4. Frequent gibberish or jargon     

    5. Difficulty understanding basic things ("just can't get it")      

    6. Pulls parents around when wants something  

    7. Difficulty expressing needs or desires, using gestures

    8. No spontaneous initiation of speech and communication       

     9. Repeats heard words, parts of words or TV commercials  

    10. Repetitive language (same word or phrase over and over) 

    11. Can't sustain conversation       

    12. Monotonous speech, wrong pausing   

    13. Speaks same to kids, adults, objects (can't differentiate)     

    14. Uses language inappropriately (wrong words or phrases)  

     

    ABNORMAL SYMBOLIC OR IMAGINARY PLAY

    1. Hand or finer flapping; self stimulation

    2. Head banging       

    3. Self mutilation, inflicting pain or injury 

    4. Toe walking, clumsy body posture         

    5. Arranging toys in rows    

    6. Smelling, banging, licking or other inappropriate use of toys 

    7. Interest in toy parts, such as car wheels           

    8. Obsessed with objects or topics (trains, weather, numbers, dates)   

    9. Spinning objects, self, or fascination with spinning objects    

    10. Restricted interest, (watching the same video over and over)        

    11. Difficulty stopping repetitive "boring" activity or conversation       

    12. Attachment to unusual objects, (sticks, stones, strings, or hair)       

     13. Stubborn about rituals and routines; resists to change       

    14. Restricted taste by consistency, shape or form (refuses solids)      

     15. Savant ability, restricted skill superior to age group (reads early, memorizes books)

    468

    At what age would you consider puberty "too early"/precocious puberty?

     before 8 years in girls and 9 years in boys

    469

    What causes precocious puberty in girls and boys?

    Idiopathic: most common cause in females (90%) but rare in males (less than 10%)

    Causes in males are above our paygrade

    470

    Aside from treating the cause (where one is found), what is the mainstay of medical management for precocious puberty?

    gonadotrophin-releasing hormone (GnRH) agonist

    Continuous exposure to GnRH suppresses puberty, as it is only pulsatile exposure that triggers pubertal progression. GnRH agonists are the only effective treatment modality for GDPP.

    Drug is goserelin

    471

    What are the two complications of precocious puberty that you worry about?

    Psychological problems and short stature

     

    Sex hormones directly stimulate the growth plate, resulting in the growth spurt. There is also an increase in GH secretion. Oestrogen, either from the ovary or aromatised from testicular testosterone, is the factor that mediates the increased GH response during puberty.

    The premature secretion of gonadal steroids in children results in the paradox of tall stature during childhood, due to an accelerated rate of linear growth, with eventual short stature as an adult, due to early fusion of the epiphyseal growth plates.

    472

    You are paged by a midwife because a 4 hour old baby is in respiratory distress. As you run to the SCN you think of all the causes of respiratory distress in the newborn. What are they?

    https://app.box.com/s/7rd57a3q4f8a6odrcgwtk23n9rbum73t

     

    473

    What do you know about the significance of the cremaster reflex when assessing a child with an acute scrotum?

    In the literature the presence of the cremaster reflex is 100% reassuring for testicular torsion. That is, the ABSENCE of a cremaster reflex is 100% sensitive for torsion so if the cremaster reflex is present you should feel reassured.

     

    In real life, you will probably send them for surgical exploration anyway.

    474

    A child presents with both vomiting and diarrheoa, what are your differentials?

    GASTRO

    Gastro

    Appendicitis

    Sepsis

    uTi

    Raging HUS

    Obstruction of the bowel (early)

    475

    Your intern calls you because she has just heard a murmur in a baby who is 20 hours old. What are the causes of a murmur on day 1 of life?

    (On day 1 there is no pressure differential between the L and R circulations, therefore there is no flow through any defects and the only things that can cause a murmur are valvular)

    ·      Hyperdynamic flow murmur (anaemia, thyrotoxicosis)

    ·      Left outflow obstruction (coarctation, AS)

    ·      Mitral valve pathology

    ·      Tricuspid valve pathology

    ·      Right outflow obstruction

    476

    What are the causes of an acute scrotum?

    ‘ITCH HIS BITS’

    I - Inguinal hernia*

    T - Torsion – of testis, of testicular appendage*

    C - Cancer

    H - Henoch-scholein purpura

     

    H - Hydrocele*

    I - Infection – orchitis, epididymitis

    S - Spermatocele*

     

    B - Blood (haematocele)/trauma

    I - Idiopathic scrotal odema*              

    T - Trauma                                            

    S - Swollen veins (varicocele)  

    *affects neonates

    477

    What are the risk factors for testicular torsion?

     

    • žOccurs in boys of all ages but is most common in young adolescents
    • Hx of previously self-limiting episodes
    • Anatomical variation – bell clapper testes
      • If torsion is confirmed, always fix both testes because these variants may be bilateral
    • Undescended testes
       

    478

    A child presents late with suspected testicular torsion. You suspect that the testis is no longer viable, should you still operate?

    Yes

    Surgery should always be performed, even if non-viability is likely because partial torsion or intermittent torsion may present similarly but have longer viability times (that is, the testis may have untangled for a bit and got some blood flow --> it could still be alive!).

    The second reason is to prevent the formation of anti-sperm antibodies (theoretical risk). This is because the testes are a site of immune privelige but damage to them may allow the immune system to see them, forming antibodies which could attack the surviving testis.

    479

    A boy has presented late with an torted testis. You need to perform an orchidectomy. What risks do you inform the parents of?

    Acute complications

    • Bleeding
    • Infections
    • Anaesthesia
    • Illioinguinal nerve damage causing allodynia

    ž
    žLate complications

    • A single testis is usually sufficient to maintain normal sexual function and hormone levels. Some men will have reduced fertility. 

    480

    What is a spermatocele?

    How does it present?

    What is the management?

    An epididymal cyst filled with sperm
    ž
    žNon-tender and transilluminates – the only other scrotal mass that does this beside hydrocele!
    ž
    žUnlike hydrocele, the testis can be felt separately
    ž
    žTreat only if symptomatic
     

    481

    What is the maximum age by which intervention should ideally be in place if a pre-lingually deaf child is to acquire language in a manner as close as possible to a hearing child, in terms of both speed of development and completeness of development?

     

    6 months

    Studies have shown that if congenital hearing loss is identified, diagnosed and appropriate intervention begun by the age of 6 months, a child's spoken language development will progress in the same way as that of a normal hearing child.

    The intervention will consist of hearing aid fitting initially to allow all available sound to be delivered to the child's developing auditory system.

    For children with a severe-profound hearing loss, for whom hearing aids are insufficient, cochlear implantation should be considered and, where appropriate, carried out as early as is practicably possible.

    482

    A fully immunized 2-year-old presents to the emergency room with several days of low-grade fever, barking cough, and noisy breathing. Over the past few hours he has developed a fever of 40°C and looks toxic. He has inspiratory and expiratory stridor. The family has not noticed drooling, and he seems to be drinking without pain. 

     

    What is the diagnosis and management?

    Diagnosis

    Bacterial tracheitis

    • Rare but serious
    • Can complicate viral croup
    • Suspect if similar features to croup (URTI symptoms, barking cough, stridor) PLUS:
    • Acute temperature elevation
    • Acute increase in respiratory distress
    • Very sick (“toxic”) looking child

    Management:

    • Minimal examination/investigations
    • Secure the airway (first priority) – e.g. endotracheal intubation
    • Empirical IV antibiotics 
       

    483

    What is the diagnosis?

    A 4-year-old boy is brought to clinic by his mother who considers he has weak legs and is finding climbing the stairs difficult. He walked independently at 20 months and has received speech therapy for a mild speech delay. On examination his lower limb reflexes are normal, his muscles are well developed distally although weak proximally.

    Duchenne Muscular Dystrophy occurs in boys and presents with delayed walking that is, 18 months. Speech delay, cardiomyopathy, scoliosis and calf pseudohypertrophy are also features.

    484

    What % of congenital cardiac defects present in infancy?

    50%

    485

    Are most babies with cyanotic congenital heart disease are small for gestational age?

    No

    Growth failure is masked by fluid retention and reduced urine volume which leads to inappropriate weight gain.

    486

    A baby is diagnosed with pulmonary stenosis. What is this associated with?

    Noonan's syndrome (chromosome 12q24.1 defect)

    Congenital rubella

    Connective tissue disorders

    Williams syndrome

    487

    What is the most important single factor in predicing the outcomes of a premmie baby?

    Birthweight

    488

    What is the most common cause of neonatal heart block?

    Maternal SLE

    489

    What is the most common cause of bleeding in utero?

    NAIT - neonatal alloimmune thrombocytopenia

    This is the equivalent of Rh disease but for platelets

    Thrombocytopaenia caused by transplacental passage of maternal platelet-specific alloantibody and rarely human leukocyte antigen (HLA) allo-antibodies  (which are expressed by platelets) to fetuses whose platelets express the corresponding antigens.

    490

    A 5 year old boy has weight loss, diarrheoa and abdo pain. What new test could you do to diagnose inflammatory bowel disease like UC or Crohns?

    What is the sensitivity and specificity of this test?

    Faecal calprotectin is a substance that is released into the intestines in excess when there is any inflammation there. Its presence can mean a person has an inflammatory bowel disease such as Crohn's disease or ulcerative colitis. These conditions can cause very similar symptoms to irritable bowel syndrome.

    Sensitivity is 95% to 100% at cut-off of 50 µg/g

    Specificities ranges from 44-93%.

    491

    What what ways do the different types of headaches present differently in kids to adults?

    Tension headache is still the band-like headache 
    Migraine is across the forehead rather than unilateral like it is in adults
    If a child complains of point tenderness (particularly at the pterion) it is likely a MSK issue, most commonly in the knee, as the child is tensing all muscles on the affected side. Ask the child to walk, and if they aren't swinging the arm on the affected side it is almost a certain diagnosis

    492

    At what gestational age are newborns most at risk of respiratory distress syndrome?

    <32 weeks

    493

    What is the management of respiratory distress syndrome?

    Basics

    Prevent preterm birth

    • Maternal cervical cerclage
    • Bed rest
    • Treatment of infections
    • Administration of tocolytic medications
      • Prevention of neonatal cold stress, birth asphyxia and hypovolaemia

    Minimal handling is important

    Airway management – place the unwell infant in the lateral or prone posture

    Oxygen through incubator or headbox

    Place and person

    Preterm infants should be in neonatal ICU

    Investigate and confirm diagnosis

    ABG, SpO2, CXR

    Non-invasive management

    Temperature control – hypo and hyperthermia may worsen symptoms

    Definitive management

    • Commence penicillin and gentamicin therapy after initial investigations (it is very difficult to differentiate between RDS and sepsis!)
    • Antenatal corticosteroid therapy
      • Given to pregnant women at risk for preterm labour
    • Intratracheal administration surfactant therapy
      • Can be administered repeatedly
    • Withold feeding
      • Enteral feeding cannot commence until the RR is less than 60, due to the risk of aspiration
    • Intubation and intermittent positive pressure ventilation
      • Indications
        • Cyanosis despite maximal oxygen therapy
        • Severe recurrent apnoea
        • Respiratory failure (pCO2>70 and pH <7.2
      • Some hospitals will use CPAP first

    Long term

    Preterm babies will recover within days

    494

    When can premature babies start breastfeeding?

    Extremely premature babies need parenteral nutrition, while less premature need nasogastric until sucking-swallowing coordination developed (@ 36-37 weeks).

    Usually add pentavite and Fe supplementation to feed

    495

    When do you vaccinate premature babies?

    Hep B vaccine given a week after birth, unless before 32 weeks (in which case give at 32 weeks)

    Future vaccines should not be adjusted for prematurity and given according to chronological age

    496

    What kinds of fractures set of immediate alarm bells for you for child abuse?

    Any fracture in a child <18 months

    Metaphyseal fractures (more often than not related to abuse)

    497

    The ED registrar has called you to review a child because they have some suspicious bruises and they are concerned there could be child abuse/neglect. What questions will you ask the parents when you interview them?

    CHILD WORD

     

    C – Caregivers (who are all the people who look after the child)

    H – Hospital presentations previously?

    I – Injuries previously?

    L – Little brothers and sisters?  (Relevant to their safety as well)

    D – Drug and alcohol use?

     

    W – Wanted? Was the child planned?

     – Crying, is the child often miserable? (can be cause OR effect of abuse)

    R – Resources, financial and social. How is the family going?

    D – Depression? Post natal or otherwise poor mental health?

     

    Plus a normal paeds history including antenatal, birth, developmental and growth histories.

    498

    You are paediatric endocrinology and the consultant asks you what you know about congenital adrenal hyperplasia. What do you say?

    • Autosomal recessive condition causing partial or total defect 
of enzymes required for cortisol and aldosterone production
    • Occurs in 1/15,000 live births and is the most common cause of ambiguous genitalia 

    • The salt-wasting form of CAH is a medical emergency - babies can die of vomiting, dehydration and shock at 2-4 wks of age.

     

    Etiology

    • 21-hydroxylase deficiency causes 95% of CAH cases; this causes decreased cortisol and aldosterone with shunting toward overproduction of androgens 

    • Cortisol deficiency leads to elevated ACTH, which causes adrenal hyperplasia 


     

    Clinical presentation

    Depends on the specific deficiency and the cause - may present with shock and hyperkalemia if not suspected. Can be divided into:

     

    Salt-Wasting 21-Hydroxylase Deficiency (2/3 of cases)

    Infants present with shock, FTT, low Na+, high K+, low Cl, low glucose, adrenal insufficiency, high ACTH, hyperpigmentation of genitals and areola and postnatal virilization 


     

    Late-Onset 21-Hydroxylase Deficiency

    Allelic variant of classic 21-hydroxylase deficiency- mild enzymatic defect 


    Girls present with amenorrhea 


    Boys present with precocious puberty with early adrenarche, dehydration 


     

    Simple Virilizing 21-Hydroxylase Deficiency

    Virilization in girls but not boys 


     

    11-Hydroxylase Deficiency

    Sexual ambiguity in females


    May have insidious onset; may present with hirsutism, occasionally hypertension 


     

    17-Hydroxylase Deficiency

    Sexual ambiguity in males, hypertension

     

    Investigations

    • Low Na+, high K+, low cortisol, high ACTH if both glucocorticoid and mineralocorticoid deficiency
    • increased serum 17-OH-progesterone (substrate for 21-hydroxylase) 

    • increased testosterone, DHEAS


     

    Treatment

    • glucocorticoid replacement to lower ACTH

    • in salt-wasting type mineralocorticoids given as well

    • spironolactone is used in late-onset 21-hydroxylase deficiency as anti-androgen
    • surgery to correct ambiguous genitalia 


     

    499

    At what gestational ages would you order a triple test or a quad test?

    What is involved in each test at and what gestational ages?

    What can each of these tests screen for?

    No ultrasound in quad test, just four serum biomarkers.

    The quad test is free in Victoria, but less sensitive with a 5% false positive rate.

    500

    At what gestational ages can you order CVS versus amniocentesis?

    501

    What are the risks to both mum and bubs of twin pregnancies?

    Risks to fetuses

    • PROM and Pre-term birth (PTB)
      • PTB is the biggest cause of multi fetal M&M
      • PTB at 24-32 weeks affects:
        • 1% of singletons
        • 5% DC twins
        • 10% MC twins
    • Cord Prolapse
      • Polyhydramnios, PPROM and malpresentation can lead to cord prolapse
    • Antepartum and post partum haemorrhage
    • Congenital malformation
    • Twin-twin transfusion syndrome (TTTS)
      • 10-15% of all monochorionic gestations
      • If untreated, severe early-onset TTTS is associated with a perinatal mortality rate of over 90%.

    Maternal complications

    • Hyperemesis gravidarum
    • Anemia (greater haemodilution)
      • Iron and folic acid supplementation recommended
    • Gestational Diabetes 
    • Antepartum and post partum hemorrhage
      • Larger placental surface area, uterine over distension,
    • Higher rates of operative birth
    • Postnatal depression
    • Maternal mortality – Two fold higher in twin pregnancies.

    502

    You are called to neonatal code blue. A baby has just been born with no respirations or muscle tone. Describe your actions.

    • Call for senior help/call code
    • Don PPE/gloves
    • History
      • Gestational age
      • mec stained liquour
      • mothers GBS status
      • maternal fever
      • complications in labour?
    • Stimulate the baby for 30 secs, check HR and RR with stethoscope
    • If inadequate resps commence IPPV on room air whilst attaching Spo2 and telemetry
      • PIP (30 cmH2O for a term infant or 20 - 25 cmH2O for a preterm infant), PEEP 5cm
    • If no response after 30 seconds and HR
    • At this stage consider intubation, adrenaline and 10ml/kg normal saline through umblical artery
    • Titrate oxygen to time of life

    503

    What are the most common cancers of childhood and at what ages do they typically occur?

    504

    What do you know about Wilm's tumours?

    • Most common in preschool aged children
    • Rapidly growing retroperitoneal tumour
    • Often asymptomatic mass
    • May rupture, bleed and cause pain (20%)
    • Haematuria and HTN occur in some patients. Can cause an aquired vWD.
    • Surgery and chemotherapy achieve 90% cure rates
    • Metastasize very late

    505

    What do you know about neuroblastoma?

    • Most commonly arises from the adrenal glands
    • Variable biology: may spontaneously regress or be chemo-refratory
    • 75% metastatic disease at diagnosis
    • May infiltrate abdomen, thorax and spine. Lymph nodes and BM are common metastatic sites
    • May have interesting paraneoplastic effects:
      • Opsomyoclonus (dancing eyes, dancing feet syndrome)
      • Excessive catecholamines: flushing, tachycardia, HTN
      • VIP secretion – severe refractory diarrhea with FTT and low K+
    • Diagnosis
      • Urinary cathecholamines are raised in 90%
      • Biopsy for tissue diagnosis and risk stratification
      • CT/MRI
      • BMAT
      • MIBG (radioisotope) scan can show areas of cathecholamine synthesis
    • Treatment
      • Surgery for low risk disease. More intensive chemo-/radio- therapy for worse disease.

    506

    What are the diagnostic criteria for Kawasaki disease?

    507

    At what point in gestation is hyperglycaemia most dangerous? Why is this?

    The first trimester

    Fetuses don't make their own insulin until 14 weeks so hyperglycaemia before this is teratogenic

    508

    A 15-year-old boy presents to a physiotherapist complaining of tenderness near the base of his thumb. He recalls falling during cross country running about 1 week ago.

     

    A - Fractured radius

    Metaphyseal fractures have a peak incidence during the adolescent growth spurt (girls aged 11-12 years, boys 12-13 years) due to weakening through the metaphysis with rapid growth.

    509

    What is a buckle's fracture?

    Failure of a child's bone in compression results in a "buckle" injury, also known as a "torus" injury. These most commonly occur in the distal metaphysis, where porosity is greatest.

    510

    What type of fracture does a FOOSH cause in a kid under 10 versus an adult?

    In an adult a FOOSH = radial fracture. In a kid FOOSH = suprcondylar fracture

    511

    What type of childhood cancer is associated with familial adenomatosis polyposis?

    Hepatoblastomaa

    512

    Buzz word diagnosis...?

    Ultrasound shows small baby + choroid plexus cysts + ‘rocker bottom feet’ = what syndrome?

    [hint: usually hasn’t had screening test]

    Edward’s Syndrome (Trisomy 18)

    The majority of fetuses with the syndrome die before birth.

    513

    Ultrasound shows foetus has a cystic hygroma = what syndrome?

    Turner’s Syndrome

    45XO

     

    Cystic hygromas are benign and can happen to anyone, but most common in context of Turner's syndrome

    514

    Buzzword diagnosis:

    Ultrasound shows frontal bossing with head and AC on the 50th centile but all four limbs below the 3rd centile

    Achondroplasia

    A common cause of dwarfism. It occurs as a sporadic mutation in approximately 80% of cases (associated with advanced paternal age) or it may be inherited as an autosomal dominant genetic disorder.

    515

    A child presents with recurrent infections in the setting of neutropenia, as well as short stature and mucusy faeces.

    What is the diagnosis?

    Shwachman-Diamond syndrome --> a rare autosomal recessive disorder

    After cystic fibrosis (CF), it is the second most common cause of exocrine pancreatic insufficiency in children.

    Recurrent infection is common due to neutropenia and pancytopaenia. 

    Growth retardation occurs in more than 50% of cases.

    516

    What is Alagille syndrome and what is it associated with?

    Rare genetic condition that causes the bile ducts to be narrow, malformed, and reduced in number (bile duct paucity). This causes jaundice, hepatosplenomegaly.

    Also associated with Tetralogy of Fallot, skeletan and eye deformities and a characteristic facial appearance

     

    517

    What is Beckwith Wiedemann Syndrome and what is it associated with?

    An overgrowth disorder usually present at birth, characterized by an increased risk of childhood cancer and certain congenital features.

    Common features used to define BWS are:
    - macroglossia (large tongue),
    - macrosomia (above average birth weight and length),
    - midline abdominal wall defects (omphalocele/exomphalos, - umbilical hernia, diastasis recti),
    - ear creases or ear pits, and
    - neonatal hypoglycemia (low blood sugar after birth).

     

    Associated with increased risk of cancer, specifically Wilms' tumor (nephroblastoma), pancreatoblastoma and hepatoblastoma

    518

    What is ataxia telangectasia and what is it associated with?

    A rare, neurodegenerative, inherited disease causing severe disability. 

    A-T affects many parts of the body:

    • It impairs certain areas of the brain including the cerebellum, causing difficulty with movement and coordination.
    • It weakens the immune system, causing a predisposition to infection.
    • It prevents repair of broken DNA, increasing the risk of cancer.

    It is associated with leukaemias and lymphomas

    519

    What is Peutz-Jeghers syndrome and what is it associated with?

    Peutz Jeghers syndrome, also known as hereditary intestinal polyposis syndrome, is an autosomal dominant genetic disease characterized by the development of:

    1. benign hamartomatous polyps in the gastrointestinal tract and
    2. hyperpigmented macules on the lips and oral mucosa 

    Associated with cancer!

    While the hamartomatous polyps themselves only have a small malignant potential, patients with the syndrome have an increased risk of developing carcinomas of the pancreas, liver, lungs, breast, ovaries, uterus, testicles and other organs.

    520

    What is the classic clinical picture of Roseola?

    What are the complications?

    Tends to occur in younger (6-36 months) children with high fevers preceding a sudden rash that begins on the trunk.

    The rash can last from a few hours to 3 days

    Essentially no worrying complications, conservative management. Can cause febrile convulsions (like any febrile illness) or otitis media.

     

    521

    What are the symptoms of Rubella?

    Rash / exanthem – not raised (c.f. measles), often starts on the face in children then spreads down the neck, trunk and extremities

    Enanthem – Forchheimer’s spots

    Lymphadenopathy is common

    Constitutional symptoms: conjunctivitis, arthralgia, malaise, fever, coyza

    522

    A woman insists on drinking alcohol in pregnancy, you have been asked to educate her about fetal alcohol syndrome. What complications/features of FAS do you inform her about?

    1. IUGR
    2. Facial dysmorphisms (see photo)
    3. Global cognitive or intellectual deficits representing multiple domains of deficit
    4. Social problems

    523

    What is this?

    Epidermolysis bullosa (EB) is a group of inherited connective tissue diseases that cause blisters in the skin and mucosal membranes. It is a result of a defect in anchoring between the epidermis and dermis, resulting in friction and skin fragility. Its severity ranges from mild to lethal.

    524

    What is this?

    Aplasia cutis (sometimes called ‘aplasia cutis congenita’) is a condition where a newborn child is missing skin from certain areas.

    525

    What is this?

    Acrodermatitis enteropathica is a rare genetic disorder characterised by diarrhoea, an inflammatory rash around the mouth and/or anus, and hair loss.

    Acrodermatitis enteropathica is due to malabsorption of zinc through the intestinal cells.

    It is autosomal recessive

    526

    What is this?

    Linear epidermal nevus/Lines of Blaschko

    Epidermal naevi are due to an overgrowth of the epidermis. Lesions are present at birth (50%) or develop during childhood (mostly in the first year of life). The abnormality arises from a defect in the ectoderm. This is the outer layer of the embryo that gives rise to epidermis and neural tissue.

    527

    What is this?

    Incontinentia pigmenti --> an dominant X-linked disease

    ASSOIATED WITH TEETH, EYE AND CNS PROBLEMS

    528

    What is this?

    Cutis marmorata telangiectatica congenita (CMTC) is a rare, deep purple, marble- or net-like birthmark. It is mostly cosmetic, and while it is present at birth, it fades considerably over a child's first year.

    529

    What is this?

    Ash lead macules --> associated with tuberous sclerosis.

    Note the hypopigmented macules, in contrast to vitiligo where there is NO pigmentation

    530

    What is this?

    Congenital melanocytic naevus

    proliferations of benign melanocytes (pigment cells) that are present at birth or develop shortly after birth.

     

    The risk of melanoma is mainly related to the size of the congenital melanocytic naevus. Small and medium sized congenital melanocytic naevi have a very small risk, well under 1%. Melanoma is more likely to develop in giant congenital naevi (lifetime estimates are 5-10%), particularly in lesions that lie across the spine or where there are multiple satellite lesions.

    531

    What is this?

    What is the management?

    Umbilical granuloma

    Common condition resulting from low-grade infection of umbilical stump. Presents soon after cord separation as red, friable granulation tissue in region of umbilicus.

    The key to treatment is early application of silver nitrate three times daily applied directly onto granulation tissue. Protect surrounding skin with Vaseline.

    532

    What is this? What is the management?

    A sebaceous naevus is an uncommon type of birthmark. Present at birth, it is most often found on the scalp, but sebaceous naevi may also arise on the face, neck or forehead. It consists of overgrown epidermis (upper layers of the skin), sebaceous glands, hair follicles, apocrine glands and connective tissue. It is a type of epidermal naevus and is classified as a benign hair follicle tumour. A sebaceous naevus is also called an organoid naevus because it may include components of the entire skin.

    Monitor and refer to derm. Probably no Tx required.

    533

    This is a 5 day old baby. What is the diagnosis and suggested management?

    Congenital dermoid cyst

    Common and easily resectable

    534

    What is this?

    What is the prognosis and management?

    Haemangioma --> occurs in 10% of babies, come up in first few weeks of life.

    It grows grows rapidly in the first 6 months of life and then slows down. It looks like a strawberry, or if they arise deeper in the skin they can look like a blueish lump. 

    The vast majority regress completely by age 3, but about 10% may take until age 9-10 to regress.

    They can ulcerate and become secondarily infected or bleed.

    Because infantile haemangiomas are likely to improve or regress completely with time, there is no need for specific treatment in most cases. Treatment should be considered in the following circumstances.

    • Very large and unsightly lesions
    • Ulcerating haemangiomas (up to 5-25% of lesions)
    • Lesions that impair vision, hearing, breathing or feeding
    • If they fail to resolve by school age

    535

    What is this? What is it associated with?

    Port Wine stain over the ophthalmic and maxillary branches of trigeminal nerve

    Associated with Sturge-Weber syndrome --> a rare disorder that is present at birth. A child with this condition will have a port-wine stain birthmark (usually on the face) and may have nervous system problems.

    Worry about epilepsy, paralysis and ID

    Most cases of Sturge-Weber are not life-threatening. The patient's quality of life depends on how well the symptoms (such as seizures) can be prevented or treated.

    Patients will need to visit an ophthalmologist at least once a year to treat glaucoma. They also will need to see a neurologist to treat seizures and other nervous system symptoms.

    536

    You are treating a 7yo child in ED for severe asthma. The nurse assisting you offers to insert an IV line for IV steroids, what do you tell her?

    There is no evidence that IV steroids are any more effective than PO steroids for asthma exacerbations.

    Their role is just for kids who can’t tolerate PO meds (like if they’re vomiting).

    537

    When is bronchiolitis most severe?

    Bronchiolitis is always worst on day 3, counting day 1 as the first day of respiratory distress (not day 1 of the coryza)

    538

    What is this USS showing?

    The keyhole sign is an ultrasonograhic sign seen in boys with posterior urethral valves. It refers to the appearance of posterior urethra which is dilated, and associated thick walled distended bladder which on ultrasound may resemble a key hole.

    539

    What are the causes of recurrent UTI in children?

    • Idiopathic
    • Vesicoureteric reflux with or without radiographically evident structural cause
    • Renal tract abnormality, eg: posterior urethral valves
    • Poor hygiene
    • Neurogenic bladder --> incomplete emptying
    • Voiding dysfunction/constipation

    540

    What investigations would you order for a child with a UTI?

    For infants and children aged 6 months and older with first-time UTI that responds to treatment, routine ultrasound is not recommended unless the infant or child has atypical UTI

     

    • Children <6 months should have a renal ultrasound within 6 weeks of diagnosis. It should be performed during the illness if the UTI is atypical or not responding to antibiotics within 48 hours.
    • Older children do not require and ultrasound post first UTI, but should have a renal ultrasound for recurrent UTI. 

    If indicated, a DMSA scan 4–6 months following the acute infection should be used to detect renal parenchymal defects. This is a nuclear med scan that looks for a renal scar that may have been caused by the UTI.

    541

    Hydrocephalus

    What are the two types/causes in paeds?

    What is the key sign in a newborn?

    What clinical sign would suggest that ICP is increasing?

    What is the mainstay of treatment?

     

    Types/causes

    Congenital hydrocephalus – is present from birth and is associated with other birth defects such as spina bifida and Dandy-Walker syndrome.

    Acquired hydrocephalus – can be triggered by tumours, infection or bleeding within the brain that blocks the movement or absorption of CSF.

    Key signs

    of hydrocephalus in a newborn --> increasing head circumference

    Sundowning (downward deviation of the eyes) occurs later on in infancy, as fontanelles close its easier for ICP to increase causing neuro Sx. 

    Treatment

    Mainstay of treatment is therapeutic LP and/or VP shunt

    542

    What are the three forms of neurofibromatosis?

    How are they inherited?

    What is the classical presentation of each?

    NF1 – most common neurocutaneous syndrome

    Autosomal dominant – chromosome 17, loss of functional NF1 gene, which is a tumour suppressor gene

    • Lots of cutaneous neurofibromas
    • cafe au lait spots (>6)
    • Axillary freckling
    • Lisch nodule in iris

    NF2

    Autosomal dominant – chromosome 22

    Bilateral acoustic neuromas (think: NF2 for 2 ears!)

    Schwannomatosis
    Rare (1 in 40,000)
    Similar to NF1/2 but get multiple schwannomas rather than neurofibromas

    543

    Tuberous Sclerosis

    What is it?

    How is it diagnosed?

    What are the buzz words for clinical presentation?

    What are the complications?

    What is the management?

    What is it?

    • Autosomal dominant condition, 66% of cases due to de novo mutations
    • Occurs in 1 in 5,000 - 10,000 births
    • Disorder is primarily caused by development of benign hamartomas affecting brain, skin, heart, kidneys, eyes, lungs and heart

    How is it diagnosed?

    • Diagnosis is made clinically according to criteria (SLE/DSM style)

    Buzz words/presentation

    • Ash leaf spots (hypopigmented)
    • Periungal fibromas
    • Shagreen patches
    • Facial angiofibromas
    • Forehead fibrous plaques

     

    Complications

    • Epilepsy
      • Infantile spasms are the most common presenting seizure

    • Intellectual disability
    • Autism
    • Risk of invasive malignancy

    Management

    Symptomatic tx of seizures and ID

    MRI every 1-3 years for monitoring for new brain lesions

    Surgical treatment of brain lesions only undertaken if they are causing intractable epilepsy, hydrocephalus, haemorrhage, focal neurologic deficits etc.

    544

    Friedreich ataxia

    What is it and how is it inherited?

    What is the effect of Friedreich's ataxia on cognition?

     

    What are the symptoms and prognosis?

    What is it?

    Friedreich's ataxia is an autosomal recessive inherited disease that causes progressive damage to the nervous system. It manifests in initial symptoms of poor coordination such as gait disturbance; it can also lead to scoliosis, heart disease and diabetes, but does not affect cognitive function.

    The disease progresses until a wheelchair is required for mobility. Its incidence in the general population is roughly 1 in 50,000.

    Symptoms

    • Worsening ataxia
    • Distal leg wasting
    • Absent lower limb reflexes (preserved extensor plantar response)
    • Pes cavus (high arch foot)
    • Dysarthria
    • Impairment of proprioception, vibration sense
    • Often optic atrophy
    • Associated with evolving kyphoscoliosis and cardiomyopathy that can cause cardiorespiratory compromise and death at 40-50 years.
    • No effect on cognition

    545

    Tay-Sachs disease

    What is it?

    What is the effect on an infant/child?

    What are the buzz words?

    What is it?

    Tay-Sachs disease (TSD) is an inherited (genetic) condition common in some Ashkenazi Jews and French-Canadians. However, it can affect people of any nationality. A mutated gene stops the body from producing an enzyme needed for proper brain functioning. This leads to paralysis and death, usually before the age of five.

    Symptoms/effect

    Symptoms first appear at around six months of age in a previously healthy baby. Over a short period of time, the baby stops moving and smiling, becomes paralysed and eventually dies. Most children with TSD die before their fifth birthday. There is no cure.

    Buzz words

    Cherry red spot

    Hypotonia

    Developmental regression in infancy

    Death 2-5 years

     

    546

    Gaucher disease

    What is it?

    What are the key features?

    An autosomal recessive disease in which glucocerebroside accumulates in cells and certain organs.

    The disorder is characterized by:

    • splenomegaly
    • bone marrow suppression

    • neurological degeneration

    • seizures

    Most common in Ashkenazi Jews

    547

    Niemann-Pick disease

    What is it?

    How does it typically first present and what are the clinical features?

    What is the prognosis?

    What is it?

    Niemann-Pick Disease is one of a group of lysosomal storage diseases that affect metabolism and that are caused by genetic mutations. 

    Presentation/features

    3-4 months feeding difficulties

    FTT

    Hepatosplenomegaly

    Developmental delay

    Hypotonia

    Deterioration of hearing and vision

    Cherry red spot in 50%,

    Prognosis

    Death by 4 years.

    548

    Spinal muscular atrophy

    What is it?

    What are the key clinical features/buzz words?

    What is the prognosis

    What is it?

    Autosomal recessive condition --> degeneration of the anterior horn cells

    Second most common neuromuscular disease after Duchenne’s.

    There are four types, type 1 being the worst and most common.

    Key clinical features

    Severe onset in the first months of life

    Severe hypotonia – “frog-like posture”

    Characteristic “bright eyes” – reflects normal intellect

    Areflexia

    Weakness worse in legs than arms

    Prognosis

    Usually fatal by 2 yo from respiratory failure

    549

    Charcot-Marie Tooth disease

    What is it?

    What are the clinical features/buzz words?

    What is it?

    A genetically and clinically heterogeneous group of inherited disorders of the peripheral nervous system characterised by progressive loss of muscle tissue and touch sensation across various parts of the body.

    What are the clinical features?

    Symptoms of CMT usually begin in early childhood or early adulthood, but can begin earlier. Usually, the initial symptom is foot drop early in the course of the disease. This can also cause hammer toe, where the toes are always curled. Wasting of muscle tissue of the lower parts of the legs may give rise to a "stork leg" or "inverted champagne bottle" appearance.

    Loss of touch sensation in the feet, ankles and legs, as well as in the hands, wrists and arms occur with various types of the disease.

    High arched feet (pes cavus) or flat arched feet (pes planus) are classically associated with the disorder.

    550

    What pathogen causes hand, food and mouth disease?

    coxsackie virus A

    551

    Describe the progression of the Measles rash....

    The measles rash starts as spots, which then begin to blend together. The rash begins around the ears and on the forehead at the hairline. Over three days, it spreads sequentially to cover the face, neck, trunk, arms, buttocks, and legs.

    552

    What are the potential complications of Varicella (chickenpox)?

    Chickenpox is generally a benign and self limiting disease but may be associated with complications including:

    • bacterial superinfection (particularly group A beta haemolytic streptococcus and Staph aureus),
    • pneumonia,
    • encephalitis,
    • cerebellitis,
    • hepatitis,
    • arthritis and
    • Reye syndrome.

    553

    A child has been diagnosed with chickenpox. His mother asks, "is he contagious?" What do you say?

    The patient is infectious from one to two days before the onset of the rash until the lesions have fully crusted over.

    Children must be excluded from school until fully recovered (all lesions crusted over) or at least one week after the eruption first appears.

    554

    What is the manaegement of varicella in a child?

    In immunocompetent children no specific therapy is indicated.

    Symptomatic treatment consists of Calamine lotion, cool compresses, possibly oral  antihistamines at night to improve sleep. Keeping the skin cool may reduce the number of lesions. Scratching increases the risk of secondary bacterial infection - cut the child's nails short at the first sign of the disease.

    Avoid aspirin --> Reyes syndrome

    Aciclovir is indicated in children with impaired immunity

    555

    Eosinophillic oesophagitis

    What is it? What conditions is it associated with?

    What is the classic presentation?

    What is the management?

    Can you visualise what it looks like on endoscopy?

    What is it ?

    Eosinophilic oesophagitis is a recently recognised panoesophagitis in children, with diagnosis based on histological evidence of at least 15 eosinophils per high power field on oesophageal biopsies obtained at gastroscopy. 

    It is closely associated with food allergy (including IgE and non-IgE mediated) and other atopic conditions such as eczema, allergic rhinitis, asthma or family history of atopy.

    Classic presentation

    • Eosinophilic oesophagitis can present at any age with nonspecific gastrointestinal symptoms, including regurgitation, vomiting, food refusal or dysphagia.
    • A classic infancy EO presentation includes irritability, feeding refusal and failure to thrive, which often overlaps with GORD presentation.
    • On the other hand, food bolus impaction is the most common EO presentation in school aged children and adolescents. This presents as dysphagia, or a sensation of choking/food getting stuck (often with meat)

    Management

    Management of suspected EO requires referral to a gastroentologist for diagnosis by endoscopy. Treatment usually consists of a trial of food allergen elimination (ie. empirically or based on allergy testing – with referral to an allergist) or swallowed inhaled corticosteroids.

    556

    A child presents with constipation. What physical examinations must you perform?

    Height and weight – failure to thrive

    Abdomen - palpable faeces

    Spine – deep sacral cleft or tuft of hair

    Neurology - assessment of lower limbs.

    Anal area – visually examine for fissures. Internal examination not required.

    557

    What is the mainstay treatment of idiopathic constipation of childhood?

    Mainstay of Tx of are osmotic laxatives --> osmolax.

    Very safe. Other option is lactulose with is less good.

    558

    What is a normal birth LENGTH for a newborn?

    50cm

    559

    What is a normal HEAD CIRCUMFERENCE at birth?

    35cm

    560

    What is a ballbark normal feed volume for a newborn?

    150ml/kg

    561

    What is a normal weight gain for a newborn? What about for premature babies?

    30g per day.

    For premmies 15-20g/day

    562

    What is this?

    What would you say to parents of a child with this condition to explain what it is?

    What is the management?

    Plagiocephaly

    is the most common craniofacial problem today.

    Explain to parents

    It is common for a newborn baby to have an unusually shaped head. This can be caused by the position of the baby in the uterus during pregnancy, or can happen during birth. Your baby's head should go back to a normal shape within about six weeks after birth. Sometimes a baby's head does not return to a normal shape and the baby may have developed a flattened spot at the back or side of the head. This condition is known as deformational plagiocephaly.

    Plagiocephaly does not affect the development of a baby's brain, but if not treated it may change their physical appearance by causing uneven growth of their face and head.

    Management

    Many children with deformational plagiocephaly do not need any treatment at all, because the condition can improve naturally as the child grows and begins to sit up. For children where treatment is necessary, it is important to see a specialist (plastic surgeon) between four and eight months of age. This is because the greatest amount of correction will occur before 12 months of age.

    Where needed, treatment may involve helmets or correctional positioning.

    563

    What are the MOST COMMON causes of delayed fontanelle closure?

    Vit D deficiency and hypothyroidism

    564

    How far apart should the eyes be set in an infant?

    Eyes should be one eye length apart

    565

    What colour is bile stained vomit?

    Green

    566

    A boy presents with an inguinal hernia.

    Based on their current age, how quickly do they need surgery?

    • Under 6 weeks -> 2 days
    • Under 6 months -> 2 weeks
    • Under 6 years -> 2 months

    567

    What cardiac anomalies are associated with fetal alcohol syndrome?

    ASD, VSD, TOF

    568

    What cardiac anomaly is associated with Turners?

    AS and coarctation

    569

    What cardiac anomalies are associated with Noonan syndrome?

    HOCM, ASD, pulmonary stenosis

    570

    What are the various classic presentations of Acute Lymphoblastic Leukaemia?

    Most common in 2-4 year olds

    Clinical presentation usually related to marrow infiltration or occasionally mass effect, certain subtypes more likely to manifest in certain ways, e.g:

    BPrecursor ALL: extensive infiltration of marrow and lymphoid organs (bone pain, bleeding, bruising, neutropenia, lymphadenopathy and hepato- splenomegaly)

    Mature-B ALL: extramedullary masses in abdomen or head/neck , CNS involvement more common than in other types

    T-cell ALL: bulky mediastinal mass,

    571

    What types of Lymphoma occur in children, and at what ages?

    How do they present?

    NHL more common in children

    NHL is on a continuum with some types of ALL

    T-cell malignancies can present as either an ALL or NHL, and mediastinal mass is a common feature to both

    Mature B-cell types tend to present more as NHL, with masses in the abdomen, and head/neck region

     

    HL more common in adolescent and younger adults

    B symptoms are less common

    Often a neck / upper body mass, although can occur any

    Very good overall survival rates in adolescence, even with disseminated disease

    Ann Arbour staging system

    572

    Describe the Ann Arbour staging criteria...

    573

    Describe the structure of the heart (and the pattern of blood flow) in tricuspid atresia...

    Tricuspid atresia --> Absence of tricuspid valve AND of functioning RV.

    Systemic venous return is therefore shunted from the RA through the FO/ASD into the LA where it mixes with pulmonary venous return, resulting in semi-oxygenated blood output to the Aorta.

    If a VSD is present there will be a small hypoplastic RV and blood will enter the pulmonary arteries through this. (This is pictured)

    If there is no VSD, the RV will be completely hypoplastic, pulmonary atresia will be present, and the pulmonary arterial circulation will be completely dependant on shunting of blood from the aorta to the pulmonary circulation via the DA.

    574

    What is the classic presentation and management of SVT in children?

    Presentation:

    • Paroxysmal episodes of palpitations
    • Rapid heart rate = poor cardiac output = hypotension which often causes:
      • Chest pain
      • Fatigue
      • Lightheadedness
      • (neonates with these symptoms may just be irritable, poor feeding, drowsy etc – difficult to Dx)
    • If SVT is prolonged or left undiagnosed for a long period it may cause CCF

    Mx:

    Confirm with Holter monitor

    Acutely:

    • Assess for haemodynamic compromise, if yes:
      • Chemical cardioversion: adenosine, verapamil
      • DC cardioversion

    Long term:

    • Beta-blocker (propranolol - infants, atenolol –older) or Flecainide
    • If refractory to medical therapy then consider RF Ablation, relatively dangerous procedure though given most SVT relatively harmless.

    575

    576

    What are the two defining features of Jervell and Lange-Nielsen syndrome

    Profound hearing loss from birth plus

     long QT syndrome

    577

    What is the buzz word for imaging findings of TOF?

    boot shaped heart

    578

    The parents of an 18month year old child bring her to you to review her feet, which they think look abnormal. What do you tell them is the problem? When should they worry?

    This is called flat feet (pes planus

    It is very common from age 1 to 2

    As long as the arch comes back when they're when on tiptoes it's totally fine

    Orthotics do little, no management required

    When to worry?

    Most children develop an arch in standing by around age six. However, about one in five children never develop an arch and that CAN indicate Achilles tendon contracture or inflammatory arthtropathy --> should be referred.

     

     

    579

    A 2 year old's parents bring him for review because they think he has "bow legs" (pictured). What do you say?

    This is called Bow legs (genu varum) 
    Self resolves by age 3

    Just make 100% sure it's not rickets

    580

    The parents of a 4 year old bring their child to you concerned about her "knock knees". What do you say? What management is required.

    Knock knees are common in children between the ages of three and five.

    In most children the legs gradually straighten with growth, and are usually in a normal position by the time they are eight years old.

    Because bow legs and knock knees usually correct naturally, special shoes, braces or exercises are not recommended.

    If you are concerned about the shape of your child’s legs, it may be helpful to take a photograph of your child every six months to show your doctor. Photograph your child standing with their knees facing forward.

    581

    Blue sclera = ??

    Osteogenesis imperfecta = fragile bones

    582

    A 15 year old boy who is training to be a professional football player presents with persistent knee pain localised pain over femoral condyles. What is the diagnosis and management?

    Osteochondritis dissecans

    (separation of bone and cartilage from medial femoral condyle following avascular necrosis)

    If complete separation of articular fragment loose body formation will give symptoms of knee locking or giving way

    Spontaneous healing is usual unless there is an unstable fragment, and treatment revolves around rest and immobilisation for up to a year

    583

    A 13 year old girl has pain when standing up from sitting, as well as when walking up stairs. She has no other symptoms. What is the diagnosis and management?

    Chondromalacia patellae = softening of patella articular cartilage

    often affects adolescent females

    Rx with rest and PT for quad strengthening

    584

    What are the most common/problematic congenital malformations associated with Turner's syndrome?

    Coarctation of the Aorta

    Horseshoe kidney

    Primary amenorrheoa/ovarian failure

    Increased risk of autoimmune disorders

     

    585

    hockey-stick palmar crease = ???

    Fetal alcohol syndrome

    586

    What is the genetic defect in Williams Syndrome?

    (Mainly sporadic) deletion in chromosome 7

    (7q11.23)

    587

    What are the main features of Williams Syndrome?

    • “Elfin” facies (a.k.a. “pixie-like”)
    • Cognitive profile consisting of impaired cognition and development accompanied by a friendly, social personality
    • Love for and skill at music
    • hyperacusis – extreme sensitivity to sound, which may include being startled by ordinary noise levels

    • Supravalvular aortic and/or pulmonary stenosis
    • Hypertension
    • Short stature
    • Endocrine abnormalities including hypercalcemia, diabetes mellitus, and subclinical hypothyroidism
    • Genitourinary abnormalities 
    • MANY MANY MANY others

    588

    Diagnosis?

    Prognosis?

    Edwards syndrome --> Trisomy 18

    <10% live beyond 1 year