Paediatrics Flashcards

Question 1

Q

What is the medical term for croup?

Answer

A

Laryngotracheobronchitis

Question 2

Q

What is the typical presentation and clinical course of croup?

Answer

A

1 - 2 days of coryza.On the 2nd or 3rd night / early morning, awake with a barking cough.Stridor might develop after that.Barking cough / stridor only lasts 2-3 days.Viral symptoms last 7 days

Question 3

Q

What is the typical microorganism which causes croup?

Answer

A

Parainfluenza virus

Question 4

Q

What is the management of croup?

Answer

A

Basics - minimal handling. Supplemental oxygen or respiratory support if required in severe cases.2. Place and person - admit to hospital if worried about increased WOB or hypoxia.3. Investigations and definitive diagnosis - clinical diagnosis4. Management - oral prednisolone (1mg/kg), oral dexamethasone (0.15 - 0.6 mg/kg), IV dexamethasone (0.2mg/kg), nebulised adrenaline (1:1000), ETT5. Long term

Question 5

Q

What are the two main types of croup?

Answer

A

Acute viral croup or recurrent spasmodic croup

Question 6

Q

What are the symptoms of epiglottis?

Answer

A

The four DsDysphagiaDyphoniaDroolingDyspnoea

Question 7

Q

What is the most common microorganism which causes epiglottis?

Answer

A

H. influenzae B

Question 8

Q

What is the most common microorganism which causes bacterial tracheitis?

Answer

A

Staphylococcus aureus (now more common than epilglottitis due to Hib vaccine)

Question 9

Q

what is the dosage of ipatropium bromide given in moderate / severe acute exacerbations of asthma?

Answer

A

6 yo give 8 puffs with salbutamol burst therapy (every 20 minutes for one hour)

Question 10

Q

What are the causes of cyanotic congenital heart disease?

Answer

A

12345TTruncus arteriosusTransposition of the Great ArteriesTricuspid / Pulmonary AtresiaTetralogy of FallotTotal anomolous pulmonary venous return

Question 11

Q

What are the two classes of acyanotic congenital heart disease?

Answer

A

Obstructive and L-R shunt

Question 12

Q

What are the causes of obstructive heart disease?

Answer

A

Hypoplastic L heart syndromeASCoarctation of the aortaInterruption of the aortic arch

Question 13

Q

As a general rule, which group of congenital heart defects are ALWAYS duct dependent?How is this treated in the early stages?

Answer

A

Obstructive congenital heart defects (hypoplastic L heart syndrome, AS, interruption of the aortic arch and aortic coarctation).They are treated by PGE1 in the short term, to keep the duct open

Question 14

Q

As a general rule, which group of congenital heart defects are USUALLY duct dependent?Is it harmful to treat with PGE1, while still in the diagnostic process?

Answer

A

R–>L shunts (1T2T3T4T5T) but this time you depend on the duct being open for blood to flow into the PULMONARY circulation

Question 15

Q

How and when do obstructive congenital heart defects present?

Answer

A

A shocked neonate. Often on D2 of life when PDA closes.Present as a dusky / grey coloured neonate with weak / absent pulses and a low BP, with a metabolic acidosis.

Question 16

Q

What are the defects of tetralogy of fallot?

Answer

A

PULMONARY STENOSIS2. RVH3. VSD4. Overriding aortic arch

Question 17

Q

When does tetralogy of fallot tend to present and how?

Answer

A

6-12 months of life.”Tet spells” or “hypoxic spells” - cyanosis or LOC / going floppy on feeding, crying or exertion

Question 18

Q

What is the surgical correction of transposition of the great arteries?

Answer

A

balloon atrial septotomy

Question 19

Q

What is the surgical correction of hypoplastic L heart syndrome?

Answer

A

Norwood operation

Question 20

Q

Which two congenital heart defects are associated with DiGeorge syndrome? Which chromosomal abnormality is this and what are the other associated features?

Answer

A

TOF and transpos are both associated with microdeletion of the long arm of chromosome 22.The other associated features are ‘CATCH 22’Cardiac (trasnpos, TOF)Abnormal (long) faciesThymic hypoplasiaCleft palateHypocalcaemia / hypoparathyroidism

Question 21

Q

With which CHD is Turner’s syndrome associated?

Answer

A

Aortic coarctation

Question 22

Q

Compare the timing of onset of transposition of the great arteries and TOF?

Answer

A

Transpos always presents as cyanosis in the newborn period. TOF presents 6-12 months of life as cyanosis.

Question 23

Q

What is the most common cause of ACQUIRED heart disease in paediatrics?

Answer

A

Kawasaki disease

Question 24

Q

What are the diagnostic features of Kawasaki disease?

Answer

A

Fever > 5 days PLUS at least 4/5 other diagnostic features.NB: can have ATYPICAL Kawasaki disease when there is 5 days (usually >38 degrees)Lymphadenopthy (Csp > 1.5cm)Atypical Kawasaki Disease (usually with evidence of coronary Artery Aneurysm)Mouth (strawberry lips and tongue)Eyes (bilateral non-purulent exudate) and Extermities (erythematous, oedematous, desquamation)Rash (polymorphous rash)

Question 25

Q

What is the treatment of Kawasaki disease

Answer

A

Intravenous immunoglobulin (2 g/kg over 10 hours; preferably within the first 10 days of the illness.)Aspirin 3 - 5 mg/kg once a day for at least 6 to 8 weeks

Question 26

Q

What should be organised before a patient treated for Kawasaki disease is discharged?

Answer

A

Follow-up echocardiogram in 6-8 weeks

Question 27

Q

Kawasaki disease is primarily a clincial diagnosis, but what Ix would you perform (and what might you expect to see)?

Answer

A

Bedside testsECG - check for ischaemia / infarctionUrine dipstick - negativeLaboratory TestsFBE - neutrophilia, anaemia, thrombocytosisESR/CRP - markedly raisedLFTs - ALT raisedImagingEchocardiogram

Question 28

Q

What are the common and less common presentations of HSP?

Answer

A

Common- purpuric rash over legs and buttocks- migrating arthlagia and swelling- abdominal painLess common- HSP nephritis (HT, proteinuria, haematuria)- intussuception- malaena and haematemesis- scrotal swelling

Question 29

Q

Describe the management of HSP

Answer

A

Basics- analgaesia for joint pain (paracetamol not NSAIDs)- analgaesia for abdo pain (steroids have evidence)Place & Person- Referral to outpatient paeditrician for ongoing observationDefinitive management- Education & supportive carePrevention- continue to review for 6 months (including BP and urine dipstick)- steroids are NOT indicated for nephritis prophylaxis

Question 30

Q

What are the DDx for brusing in children?

Answer

A

‘SHIELD’Sepsis (meningoccoal)HSP / HUS / HaemophiliaITPEvents (Trauma or non-accidental injury)LeukaemiaDIC (usually in the setting of severe illness)

Question 31

Q

How do you diagnose ITP?

Answer

A

An isolated thrombocytpoaenia (platelet count of

Question 32

Q

What is the management of ITP?

Answer

A

Basics- stop any active bleedingPlace and Person- refer to paediatric haematologyInvestigations and confirm diagnosis- FBE and blood film to confirm isolated thrombocytopaenia- BMAT if unsure / need to rule out leuakaemiaConservative Management- avoid contact sports / high-risk activities- monitor menstural bleeding if post-pubertal female- avoid NSAIDs and 5-ASA- ongoing follow up and FBEDefinitive Management’- steroids (low or high dose)- IVIg

Question 33

Q

What is the most dangerous complication of ITP?

Answer

A

Intra-cranial haemorrhageAlthough the risk if

Question 34

Q

What infections are transmitted through the birth canal at delivery?

Answer

A

GBS

E. Coli

Listeria

Hep B

HIV

Question 35

Q

What congenital infections can be transmitted transplacentally?

Answer

A

TORCHSHPV

Toxoplasmosis

Other

Rubella

CMV

HSV

Syphillus (treponema pallidum)

Hep B

Parvovirus B19

Varicella zoster

Question 36

Q

What do the placentally transmitted congenital infections cause?

Question 37

Q

What is the management of placentally transmitted congenital infections?

Answer

A

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.

Question 38

Q

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

Answer

A

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)

Question 39

Q

What are the effects of toxoplasmosis infection on a foetus?

Answer

A

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

Question 40

Q

What effect does congenitally acquired Rubella have on a foetus?

Answer

A

Outcome depends on gestation age at which it was transmitted.

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

Question 41

Q

What effect does congenitally acquired CMV have on the foetus?

Answer

A

Periventricular calcifications

Symmetrical IUGR with microcephaly

Developmental delay

Hepatospleenomegaly

Anemia

Sensorineural hearing loss

Visual problems

Question 42

Q

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

Answer

A

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

Neurosyphillis

Bone problems

Maculopapular rash

Question 43

Q

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

Answer

A

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

Question 44

Q

How do you approach the causes of neonatal sepsis?

Answer

A

EARLY
Vertical transmission –> GBS, E coli, Listeria
Risk factors

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

Question 45

Q

What is the management of neonatal sepsis?

Question 46

Q

What tests would you do at the first antenatal assessment?

Answer

A

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)

Question 47

Q

What are the diagnostic criteria for encopresis?

Answer

A

- 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

Question 48

Q

What are the potential complications of GORD in infants?

Answer

A

oesophagitis
failure to thrive
aspiration

Question 49

Q

What is colic?

Answer

A

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.

Question 50

Q

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

Answer

A

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

Question 51

Q

What is the management of GORD in infants?

Answer

A

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:

Question 52

Q

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

Answer

A

Most common - benign

Tiredness
Hunger - this is more likely if a mother reports:
1. inadequate milk supply
2. poor weight gain
3. baby has frequent feeds (ie,

Also consider

GORD
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
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

Question 53

Q

What is the classic triad of intssusception?

Answer

A

colicky abdominal pain, currant jelly stool, palpable abdominal mass

Question 54

Q

Describe the management of suspected intussusception

Question 55

Q

What are the Ix for suspected intussception?

Question 56

Q

What are the risk factors for NEC?

Answer

A

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

Question 57

Q

What is the relationship between gestational age and weight and NEC

Answer

A

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.

Question 58

Q

What is the management of NEC

Answer

A

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

Question 59

Q

What are the buzz words for pyloric stenosis?

Answer

A

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)

Question 60

Q

When does pyloric stenosis generally present?

Answer

A

Usually between 2-6 weeks of life

Question 61

Q

What is the clinical picture o pyloric stenosis?

Answer

A

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”)

Question 62

Q

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

Answer

A

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

Question 63

Q

What are the Ix for suspected pyloric stenosis?

Answer

A

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)

Question 64

Q

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

Answer

A

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.

Answer 61

A

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).

Answer 62

A

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

Answer 63

A

Malrotation + volvulus until proven otherwise!

Answer 64

A

History

Presents day 1-3 of life (60–80% of all cases of malrotation present in the first month of life, mostly in the first week.)
Bilious vomiting (malrotation until proven otherwise!)
No dirty nappies once there is volvulus
Rectal bleeding (a late symptom/sign)
Abdominal pain

Examination

Abdominal distension (late sign)
Peritonism (late sign)

Answer 65

A

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

Barium swallow - corkscrew sign

USS - whirlpool sign

Answer 66

A

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

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

Answer 67

A

Gastro
FAMINE HIP GAIT
Neurological
Increased ICP
Infectious
Gastroenteritis
Meningitis
Pertussis (post tussive cough)
Endocrinological
DKA
Errors of Inborn Metabolism
Psychogenic / Behavioural
Overfeeding in infants
Bulaemia in adolescents
Pregnancy in adolescents

Answer 68

A

FAMINEHIPGAIT

Answer 69

A

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

Answer 70

A

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 acidosis
- correction of fluid loss, hypothermia and glucose will correct acidosis
Correct Hypothermia
- monitor temperature and have overhead heater
Sepsis
- if at risk administer IV Abx after cultures taken
Surgical correction if required- according to cause

Answer 71

A

Prematurity issues
Asphyxia
Meconium aspiration syndrome
Hypoglycaemia, hypocalcaemia
Hypothermia
Polycythaemia
Impaired immune function
BM suppression
NEC
- Mechanism = hypoxic gut as foetus shunts blood to brain
Neurodevelopmental
- Cerebral palsy
- Learning and behavioural issues
Foetal origins of adult dz
- DM, HT, obesity, CAD, hypercholesterolaemia

Answer 72

A

Plus also ask about sleep

Answer 73

A

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?

Answer 74

A

The 5 Hs

Height (growth retardation)
Heart (arrythmia, QT, bradycardia, hypotension)
Happy Bones (irreversible osteoporosis)
Hormones (osteporosis, hypothyroid, delayed puberty/amenorrheoa)
Heads (poor concentration, slowed mentation)

Answer 75

A

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

Answer 76

A

Macrosomia >4500g

Low Birthweight (LBW) Very Low Birth Weight (VLBW)

Extremely LBW (ELBW)

Answer 77

A

Infancy - 25cm/yr
Childhood - 10cm/yr

Puberty - 5cm/yr

Answer 78

A

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.

Answer 79

A

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)

Answer 80

A

Relief by sleep

Dizziness/pallor

Abdo pain

Nausea and vomiting

Prodrome

Characteristic location of pain

Answer 81

A

Rhinovirus

Adenovirus

Influenza and parainfluenza

RSV

Enterovirus

EBV

Streptococci

Answer 82

A

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

Answer 83

A

Bacterial infection

Answer 84

A

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!

Answer 85

A

MR SARS EEK!

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

Answer 86

A

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

Answer 87

A

Rash from strep throat.

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

Answer 88

A

Hand, foot and mouth

Chickenpox

Herpes simplex

(Remember: vesicular just means blistering)

Answer 89

A

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

Answer 90

A

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!

Answer 91

A

> 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

Answer 92

A

Viral lower respiratory tract infection

Up to 18 months

Answer 93

A

RSV

Human metapneumovirus

Adenovirus –> bad because causes bronchiolitic obliterans!

Parainfluenza

Influenza

Answer 94

A

Exposure to cigarette smoke
Maternal smoking during pregnancy
Prematurity

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

Answer 95

A

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)

Answer 96

A

Basics

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

Person and Place

Admit to paeds ward if patient requires any of the above, or if concerned

Investigations and Diagnosis

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

Definitive Management

Supportive management only.

Usually resolves within a week.

Long-Term Management

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

Ensure immunizations are up to date

Answer 97

A

Bronchiolitis

Asthma

Inhaled foreign body

Pneumonia

Pertussis

Bronchiomalacia/tracheomalacia

Cardiac failure

CF or other suppurative lung dz

Recurrent aspiration

Answer 98

A

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

Answer 99

A

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

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

Answer 100

A

Parainfluenza

Influenza

Adenovirus

Answer 101

A

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

Answer 102

A

None, dont distress the kid!

Answer 103

A

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.

Answer 104

A

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

Answer 105

A

Transmission: Direct contact with droplet discharges

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

Answer 106

A

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

Answer 107

A

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

Answer 108

A

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

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

Answer 109

A

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

Answer 110

A

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

Answer 111

A

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

Answer 112

A

Rrecurrent wheezing episodes which are responsive to salbutamol.

Answer 113

A

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

Answer 114

A

Very safe, almost not clinically significant long term side effects

Hoarseness and pharyngeal candidiasis are not common

particular dry-powder formulas with pH

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

Answer 115

A

autosomal recessive

Answer 116

A

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

Answer 117

A

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

Answer 118

A

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

Answer 119

A

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

Answer 120

A

Gastroschisis

Urgent surgical repair

Answer 121

A

Phimosis (the foreskin cannot be fully retracted over the glans penis), normal up until puberty

Answer 122

A

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

Answer 123

A

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)

Answer 124

A

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

Answer 125

A

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.

Answer 126

A

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

Answer 127

A

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

Answer 128

A

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

Answer 129

A

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

Answer 130

A

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

Answer 131

A

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

Answer 132

A

duct dependent

In the first week of life

When the ductus closes

Answer 133

A

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

Large VSD – Quiet pansystolic murmur, heave

Answer 134

A

VSD in both

Answer 135

A

Small –> conservative management and monitoring

Large –> surgical repair

Answer 136

A

Secundem (most common)

Primium (AVSD)

Answer 137

A

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)

Answer 138

A

OH CRAP THAT BABY IS BLUE!

DONT SWEAR IN FRONT OF THE BABY!

OH, I SAID, CRIP

*C**yanotic Heart Disease (1,2,3,4,5 Ts)
*R**espiratory disease (Meconium aspiration syndrome, pulmonary hypoplasia)
*I**nfection (GBS)
*P**ersistent pulmonary HTN of the newborn (PPHN)

Answer 139

A

Pulmonary stenosis
Right ventricular hypertrophy
Overriding aorta
VSD

Pentology –> ASD

Answer 140

A

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’

Answer 141

A

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.

Answer 142

A

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

Answer 143

A

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

Answer 144

A

Prostaglandins

Atrial baloon septostomy

Arterial switch is definitive surgical correction at 6-9 months

Answer 145

A

Right ventricular hypertrophy

Coarse ES murmur on ULSE

Answer 146

A

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.

Answer 147

A

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

Answer 148

A

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

Answer 149

A

Small VSD

Usually no treatment required, spontaneous closure

Answer 150

A

Large VSD

Diuretics, captopril

Extra calories

Surgery at 3-6 months

Answer 151

A

Contact senior help
Arrange urgent echocardiography (and potentially an ECG and CXR in the meantime)
Give O2
Use frusemide or spironolactone to relieve vascular congestion
Use captopril to reduce after load
High nutrient feeds may be required
Consider inotropes for low-output failure

If neonate consider prostaglandins

Answer 152

A

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

Answer 153

A

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

Answer 154

A

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

Answer 155

A

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

Answer 156

A

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

Answer 157

A

ALL until proven otherwise

Answer 158

A

Intracranial haemorrhage - 1% of ITP cases

Answer 159

A

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)

Answer 160

A

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.

Answer 161

A

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.

Answer 162

A

FBE

Blood film

Ferritin

Reticulocyte count

Answer 163

A

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

Answer 164

A

2-5 years

Answer 165

A

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.

Answer 166

A

Acute is ITP less than 12 months
This is the common type of childhood (2-5 y/o)

Chronic ITP lasts >12 months
Occurs in children >7 y/o and adults
Women affected more often

Recurrent ITP exists but is rare.

Answer 167

A

Platelets consistently under 10

Answer 168

A

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

Answer 169

A

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.

Answer 170

A

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

Answer 171

A

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.

Answer 172

A

AML (less common than ALL)

Answer 173

A

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.

Answer 174

A

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.

Answer 175

A

Strangulation/NAI

Vomiting

Coughing

+ other causes of petechiae (SHIELD)

Answer 176

A

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.

Answer 177

A

· Oral – paracetemol, ibuprofen, oxycodone

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

· IV – morphine, ketamine

· IM – (rare) ketamine, morphine

· Local anaesthetic

Answer 178

A

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

Answer 179

A

Tracheo-oesophageal fistula

Answer 180

A

pyloric stenosis (if age 2-7wks)

duodenal atresia

Answer 181

A

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

Answer 182

A

Refusal to weight bear in a child is a red flag!

Septic arthritis

Osteomyelitis

Malignancy

Answer 183

A

Refusal to weight bear

Fever (>38.5)

Raised WBC

ESR >40

CRP >20

Answer 184

A

6 weeks, it gets better after that

Answer 185

A

Bring them back if:

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

Answer 186

A

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

Answer 187

A

Meningitis

Pneumonia

UTI

Sepsis

Answer 188

A

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.

Answer 189

A

6 months to 6 years

Answer 190

A

3 in 100 babies

Answer 191

A

Signs and symptoms of infection (often URTI, otitis media or UTI).
Description of a generalized, tonic-clonic seizure (simple) or a focal seizure (complex). Normal neurological exam post-ictally.
- *NB if eyes deviate to one side this is a sign of a focal seizure

Answer 192

A

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.”

Answer 193

A

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%.

Answer 194

A

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

Answer 195

A

No, not at all

Answer 196

A

Teething will not cause fever > 38.5oC

Answer 197

A

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

Answer 198

A

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
*

Answer 199

A

Birth incidence is about 1 in 1000 live births.

Overall incidence rises after maternal age of 35 years.

Answer 200

A

50% have congenital heart defects

particularly AVSD

Answer 201

A

Medical

Growth
- Plot growth on Trisomy 21 specific growth charts
- Prevent obesity
Cardiac
- ECHO at birth
- Annual follow up
GIT
- Coeliac screening
Endocrine
- Annual TSH (at increased risk of hypothyroidism)
Haeamtological
- Annual FBE (at increased risk of polycythaemia)
Hearing
- Hearing test as new born and at six months of age
Opthalmology
- Eye examination every 3 years
MSK
- Education RE: atlanto-axial instability, avoid trampoline and high risk activities, discuss positioning of the spine during anasthaesia/surgery
- Low threshold for imaging. Routine spinal xray is not recommended.

Non-Medical

?Early intervention
?Funding / Carer’s Pension
?Educational and Vocational Training

Answer 202

A

CVS = 1% above the background risk

Amniocentesis = 0.5%

Answer 203

A

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.

Answer 204

A

Discuss with registrar/consultant
Full sepsis work-up: FBE/film, blood culture, urine culture (SPA), LP ± CXR
Admit for empirical antibiotics

Answer 205

A

Discuss with registrar/consultant
Full sepsis workup:
- FBE/film, blood culture, urine culture (SPA) ± CXR (only if respiratory symptoms or signs) ± LP
Discharge home with review within 12 hours if the child is:
- Previously healthy
- Looks well
- WCC 5,000 - 15,000
- Urine microscopy clear
- CXR (if taken) clear
- CSF (if taken) negative
If the child is unwell or above criteria are not all satisfied, admit to hospital for observation +/- empiric i.v. antibiotics

Answer 206

A

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

Answer 207

A

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

Answer 208

A

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

Answer 209

A

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.

Answer 210

A

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

Answer 211

A

Intussusception

Answer 212

A

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

Answer 213

A

Fluid resuscitation
Analgesia
Air enema
Definitive surgery if necessary

Answer 214

A

Like a sock folding in on itself

Answer 215

A

99% in first day

Rest in second day

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

Answer 216

A

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

Answer 217

A

Suction rectal biopsy

Answer 218

A

1/25

1/2500

Answer 219

A

Cardiac defects

Down syndrome

Answer 220

A

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

Answer 221

A

20% chance

Answer 222

A

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

Answer 223

A

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

Answer 224

A

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%

Answer 225

A

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

Answer 226

A

In non-dehydrated children, consider using 2/3 maintenance doses in unwell children, especially those with pneumonia or meningitis.

Answer 227

A

Cryptosporidium
Giardia Lamblia

Answer 228

A

dysentery w blood, pus, pain and tenemus

Answer 229

A

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

Answer 230

A

Post-gastroenteritis lactase deficiency

Mx - Lactose free diet for a few weeks

Answer 231

A

Coeliac disease!

Answer 232

A

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

Answer 233

A

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

Answer 234

A

1/25 boys born

Answer 235

A

Parental anxiety

Answer 236

A

What worries you the most?

Answer 237

A

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

Roxithromycin PO for 10 days

Answer 238

A

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.

Answer 239

A

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

Answer 240

A

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.

Answer 241

A

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.

Answer 242

A

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.

Answer 243

A

The 9 S’s of development

Neonate

6 weeks

3 months

6 months

9 months

12 months

18 months

3 years

5 years

Answer 244

A

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

Answer 245

A

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.

Answer 246

A

ABCDEFGHI

A - Appendicitis
B – Balls (testicular torsion)
C – Chest infection
D – Diverticulum (Meckel’s) + DKA
E – E.Coli (UTI)
F – Faecal or urinary retention
G - Gastroenteritis
H – Hernias/HSP
I – Intussusception + intestinal malrotation

Answer 247

A

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.

Answer 248

A

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.

Answer 249

A

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

Answer 250

A

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.

Answer 251

A

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?

Answer 252

A

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

Answer 253

A

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.

Answer 254

A

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.

Answer 255

A

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.

Answer 256

A

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)

Answer 257

A

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.

Answer 258

A

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

Answer 259

A

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

Answer 260

A

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.

Answer 261

A

There are three problems:

Protrusion of the bowel through the processus vaginalis can obstruct it’s blood supply causing necrosis/peritonitis/sepsis
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
obviously there is the risk of bowel obstruction

Answer 262

A

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.

Answer 263

A

4 years old, topical steroid.

Answer 264

A

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.

Answer 265

A

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

Answer 266

A

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).

Answer 267

A

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

Answer 268

A

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

Answer 269

A

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)

Answer 270

A

pulmonary stenosis
RVH
VSD
over-arching aorta
(5. ASD)

Answer 271

A

When the child is >3 months of age

Presents as failure to thrive, reduced exercise tolerance, clubbing of fingers and tet spells.

They are not cyanosed at birth - may be cyanosed after 6-12 weeks

Answer 272

A

The classic story is severe, painful cyanosis on exertion/stress (feeding or crying).

Older children will classically squat in order to increase systemic resistance and reduce R–>L shunting

Answer 273

A

Basics

Comfort the child in a position with their hips and knees flexed to increase systemic resistance and thereby R–>L shunt.

Oxygen

IV fluids

B blockers

IM morphine

Place and Person

Refer to paediatric cardiac surgery

Ix and confirm diagnosis

With echocardiogram

Definitive management

Surgical

Long Term

Ongoing paediatric review

Answer 274

A

Deletion of the long arm of choromosome 22

What else is related? Think CATCH 22

Cardiac (TOD, TGA)

Abnormal facies

Thymic hypolasia (T cell deficiency)

Cleft palate

Hypocalcaemia/hypoparathyroidism

Answer 275

A

small VSD: loud pansystolic murmur

large VSD: softer pansystolic murmur + loud P2 (due to pulmonary hypertension)

Answer 276

A

GBS

E. Coli

Listeria

Hep B

HIV

Answer 277

A

TORCHSHPV

Toxoplasmosis

Other

Rubella

CMV

HSV

Syphillus (treponema pallidum)

Hep B

Parvovirus B19

Varicella zoster

Answer 278

A

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.

Answer 279

A

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)

Answer 280

A

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

Answer 281

A

Outcome depends on gestation age at which it was transmitted.

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

Answer 282

A

Periventricular calcifications

Symmetrical IUGR with microcephaly

Developmental delay

Hepatospleenomegaly

Anemia

Sensorineural hearing loss

Visual problems

Answer 283

A

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

Neurosyphillis

Bone problems

Maculopapular rash

Answer 284

A

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

Answer 285

A

EARLY
Vertical transmission –> GBS, E coli, Listeria
Risk factors

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

Answer 286

A

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)

Answer 287

A

- 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

Answer 288

A

oesophagitis
failure to thrive
aspiration

Answer 289

A

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.

Answer 290

A

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

Answer 291

A

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:

Answer 292

A

Most common - benign

Tiredness
Hunger - this is more likely if a mother reports:
1. inadequate milk supply
2. poor weight gain
3. baby has frequent feeds (ie,

Also consider

GORD
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
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

Answer 293

A

colicky abdominal pain, currant jelly stool, palpable abdominal mass

Answer 294

A

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

Answer 295

A

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.

Answer 296

A

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

Answer 297

A

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)

Answer 298

A

Usually between 2-6 weeks of life

Answer 299

A

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”)

Answer 300

A

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

Answer 301

A

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)

Answer 302

A

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.

Answer 303

A

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).

Answer 304

A

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

Answer 305

A

Malrotation + volvulus until proven otherwise!

Answer 306

A

History

Presents day 1-3 of life (60–80% of all cases of malrotation present in the first month of life, mostly in the first week.)
Bilious vomiting (malrotation until proven otherwise!)
No dirty nappies once there is volvulus
Rectal bleeding (a late symptom/sign)
Abdominal pain

Examination

Abdominal distension (late sign)
Peritonism (late sign)

Answer 307

A

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

Barium swallow - corkscrew sign

USS - whirlpool sign

Answer 308

A

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

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

Answer 309

A

Gastro
FAMINE HIP GAIT
Neurological
Increased ICP
Infectious
Gastroenteritis
Meningitis
Pertussis (post tussive cough)
Endocrinological
DKA
Errors of Inborn Metabolism
Psychogenic / Behavioural
Overfeeding in infants
Bulaemia in adolescents
Pregnancy in adolescents

Answer 310

A

FAMINEHIPGAIT

Answer 311

A

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

Answer 312

A

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

Answer 313

A

Prematurity issues
Asphyxia
Meconium aspiration syndrome
Hypoglycaemia, hypocalcaemia
Hypothermia
Polycythaemia
Impaired immune function
BM suppression
NEC
- Mechanism = hypoxic gut as foetus shunts blood to brain
Neurodevelopmental
- Cerebral palsy
- Learning and behavioural issues
Foetal origins of adult dz
- DM, HT, obesity, CAD, hypercholesterolaemia

Answer 314

A

Plus also ask about sleep

Answer 315

A

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?

Answer 316

A

The 5 Hs

Height (growth retardation)
Heart (arrythmia, QT, bradycardia, hypotension)
Happy Bones (irreversible osteoporosis)
Hormones (osteporosis, hypothyroid, delayed puberty/amenorrheoa)
Heads (poor concentration, slowed mentation)

Answer 317

A

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

Answer 318

A

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.

Answer 319

A

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)

Answer 320

A

Relief by sleep

Dizziness/pallor

Abdo pain

Nausea and vomiting

Prodrome

Characteristic location of pain

Answer 321

A

Rhinovirus

Adenovirus

Influenza and parainfluenza

RSV

Enterovirus

EBV

Streptococci

Answer 322

A

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

Answer 323

A

Bacterial infection

Answer 324

A

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!

Answer 325

A

MR SARS EEK!

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

Answer 326

A

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

Answer 327

A

Rash from strep throat.

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

Answer 328

A

Hand, foot and mouth

Chickenpox

Herpes simplex

(Remember: vesicular just means blistering)

Answer 329

A

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

Answer 330

A

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!

Answer 331

A

> 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

Answer 332

A

Viral lower respiratory tract infection

Up to 18 months

Answer 333

A

RSV

Picornovirus

Human metapneumovirus

Adenovirus –> bad because causes bronchiolitic obliterans!

Parainfluenza

Influenza

Answer 334

A

Exposure to cigarette smoke
Maternal smoking during pregnancy
Prematurity

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

Answer 335

A

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)

Answer 336

A

Basics

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

Person and Place

Admit to paeds ward if patient requires any of the above, or if concerned

Investigations and Diagnosis

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

Definitive Management

Supportive management only.

Usually resolves within a week.

Long-Term Management

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

Ensure immunizations are up to date

Answer 337

A

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

Answer 338

A

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

Answer 339

A

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

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

Answer 340

A

Parainfluenza

Influenza

Adenovirus

Answer 341

A

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

Answer 342

A

None, dont distress the kid!

Answer 343

A

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.

Answer 344

A

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

Answer 345

A

Transmission: Direct contact with droplet discharges

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

Answer 346

A

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

Answer 347

A

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

Answer 348

A

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

Answer 349

A

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

Answer 350

A

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

Answer 351

A

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

Answer 352

A

Rrecurrent wheezing episodes which are responsive to salbutamol.

Answer 353

A

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

Answer 354

A

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

Answer 355

A

autosomal recessive

Answer 356

A

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

Answer 357

A

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

Answer 358

A

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

Answer 359

A

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

Answer 360

A

Gastroschisis

Urgent surgical repair

Answer 361

A

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.

Answer 362

A

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.

Answer 363

A

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)

Answer 364

A

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

Answer 365

A

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.

Answer 366

A

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

Answer 367

A

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

Answer 368

A

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

Answer 369

A

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

Answer 370

A

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

Answer 371

A

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

Answer 372

A

duct dependent

In the first week of life

When the ductus closes

Answer 373

A

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

Large VSD – Quiet pansystolic murmur, heave

Answer 374

A

VSD in both

Answer 375

A

Small –> conservative management and monitoring

Large –> surgical repair

Answer 376

A

Secundem (most common)

Primium (AVSD)

Answer 377

A

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)

Answer 378

A

OH CRAP THAT BABY IS BLUE!

DONT SWEAR IN FRONT OF THE BABY!

OH, I SAID, CRIP

*C**yanotic Heart Disease (1,2,3,4,5 Ts)
*R**espiratory disease (Meconium aspiration syndrome, pulmonary hypoplasia)
*I**nfection (GBS)
*P**ersistent pulmonary HTN of the newborn (PPHN)

Answer 379

A

Pulmonary stenosis
Right ventricular hypertrophy
Overriding aorta
VSD

Pentology –> ASD

Answer 380

A

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’

Answer 381

A

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.

Answer 382

A

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

Answer 383

A

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

Answer 384

A

Prostaglandins

Atrial baloon septostomy

Arterial switch is definitive surgical correction at 6-9 months

Answer 385

A

Right ventricular hypertrophy

Coarse ES murmur on ULSE

Answer 386

A

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.

Answer 387

A

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

Answer 388

A

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

Answer 389

A

Small VSD

Usually no treatment required, spontaneous closure

Answer 390

A

Large VSD

Diuretics, captopril

Extra calories

Surgery at 3-6 months

Answer 391

A

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

Answer 392

A

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

Answer 393

A

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

Answer 394

A

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

Answer 395

A

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

Answer 396

A

ALL until proven otherwise

Answer 397

A

Intracranial haemorrhage - 1% of ITP cases

Answer 398

A

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)

Answer 399

A

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.

Answer 400

A

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.

Answer 401

A

FBE

Blood film

Ferritin

Reticulocyte count

Answer 402

A

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

Answer 403

A

2-5 years

Answer 404

A

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.

Answer 405

A

Platelets consistently under 10

Answer 406

A

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

Answer 407

A

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.

Answer 408

A

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

Answer 409

A

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.

Answer 410

A

AML (less common than ALL)

Answer 411

A

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.

Answer 412

A

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.

Answer 413

A

Strangulation/NAI

Vomiting

Coughing

+ other causes of petechiae (SHIELD)

Answer 414

A

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.

Answer 415

A

· Oral – paracetemol, ibuprofen, oxycodone

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

· IV – morphine, ketamine

· IM – (rare) ketamine, morphine

· Local anaesthetic

Answer 416

A

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

Answer 417

A

Tracheo-oesophageal fistula

Answer 418

A

pyloric stenosis (if age 2-7wks)

duodenal atresia

Answer 419

A

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

Answer 420

A

Refusal to weight bear

Fever (>38.5)

Raised WBC

ESR >40

CRP >20

Answer 421

A

6 weeks, it gets better after that

Answer 422

A

Bring them back if:

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

Answer 423

A

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

Answer 424

A

Meningitis

Pneumonia

UTI

Sepsis

Answer 425

A

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.

Answer 426

A

6 months to 6 years

Answer 427

A

3 in 100 babies

Answer 428

A

Signs and symptoms of infection (often URTI, otitis media or UTI).
Description of a generalized, tonic-clonic seizure (simple) or a focal seizure (complex). Normal neurological exam post-ictally.
- *NB if eyes deviate to one side this is a sign of a focal seizure

Answer 429

A

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.”

Answer 430

A

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%.

Answer 431

A

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

Answer 432

A

No, not at all

Answer 433

A

Teething will not cause fever > 38.5oC

Answer 434

A

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

Answer 435

A

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
*

Answer 436

A

Birth incidence is about 1 in 1000 live births.

Overall incidence rises after maternal age of 35 years.

Answer 437

A

50% have congenital heart defects

particularly AVSD

Answer 438

A

Medical

Growth
- Plot growth on Trisomy 21 specific growth charts
- Prevent obesity
Cardiac
- ECHO at birth
- Annual follow up
GIT
- Coeliac screening
Endocrine
- Annual TSH (at increased risk of hypothyroidism)
Haeamtological
- Annual FBE (at increased risk of polycythaemia)
Hearing
- Hearing test as new born and at six months of age
Opthalmology
- Eye examination every 3 years
MSK
- Education RE: atlanto-axial instability, avoid trampoline and high risk activities, discuss positioning of the spine during anasthaesia/surgery
- Low threshold for imaging. Routine spinal xray is not recommended.

Non-Medical

?Early intervention
?Funding / Carer’s Pension
?Educational and Vocational Training

Answer 439

A

CVS = 1% above the background risk

Amniocentesis = 0.5%

Answer 440

A

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.

Answer 441

A

Discuss with registrar/consultant
Full sepsis work-up: FBE/film, blood culture, urine culture (SPA), LP ± CXR
Admit for empirical antibiotics

Answer 442

A

Discuss with registrar/consultant
Full sepsis workup:
- FBE/film, blood culture, urine culture (SPA) ± CXR (only if respiratory symptoms or signs) ± LP
Discharge home with review within 12 hours if the child is:
- Previously healthy
- Looks well
- WCC 5,000 - 15,000
- Urine microscopy clear
- CXR (if taken) clear
- CSF (if taken) negative
If the child is unwell or above criteria are not all satisfied, admit to hospital for observation +/- empiric i.v. antibiotics

Answer 443

A

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

Answer 444

A

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

Answer 445

A

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

Answer 446

A

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.

Answer 447

A

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

Answer 448

A

Intussusception

Answer 449

A

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

Answer 450

A

Fluid resuscitation
Analgesia
Air enema
Definitive surgery if necessary

Answer 451

A

Like a sock folding in on itself

Answer 452

A

99% in first day

Rest in second day

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

Answer 453

A

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

Answer 454

A

Suction rectal biopsy

Answer 455

A

1/25

1/2500

Answer 456

A

Cardiac defects

Down syndrome

Answer 457

A

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

Answer 458

A

20% chance

Answer 459

A

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

Answer 460

A

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

Answer 461

A

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%

Answer 462

A

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

Answer 463

A

In non-dehydrated children, consider using 2/3 maintenance doses in unwell children, especially those with pneumonia or meningitis.

Answer 464

A

Cryptosporidium
Giardia Lamblia

Answer 465

A

dysentery w blood, pus, pain and tenemus

Answer 466

A

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

Answer 467

A

Post-gastroenteritis lactase deficiency

Mx - Lactose free diet for a few weeks

Answer 468

A

Coeliac disease!

Answer 469

A

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

Answer 470

A

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

Answer 471

A

1/25 boys born

Answer 472

A

SpO2

Oral intake

Parental anxiety

Answer 473

A

What worries you the most?

Answer 474

A

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

Roxithromycin PO for 10 days

Answer 475

A

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.

Answer 476

A

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

Answer 477

A

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.

Answer 478

A

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.

Answer 479

A

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.

Answer 480

A

The 9 S’s of development

Neonate

6 weeks

3 months

6 months

9 months

12 months

18 months

3 years

5 years

Answer 481

A

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

Answer 482

A

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.

Answer 483

A

ABCDEFGHI

A - Appendicitis
B – Balls (testicular torsion)
C – Chest infection
D – Diverticulum (Meckel’s) + DKA
E – E.Coli (UTI)
F – Faecal or urinary retention
G - Gastroenteritis
H – Hernias/HSP
I – Intussusception + intestinal malrotation

Answer 484

A

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.

Answer 485

A

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.

Answer 486

A

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

Answer 487

A

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.

Answer 488

A

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?

Answer 489

A

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

Answer 490

A

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.

Answer 491

A

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.

Answer 492

A

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.

Answer 493

A

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)

Answer 494

A

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.

Answer 495

A

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

Answer 496

A

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

Answer 497

A

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.

Answer 498

A

There are three problems:

Protrusion of the bowel through the processus vaginalis can obstruct it’s blood supply causing necrosis/peritonitis/sepsis
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
obviously there is the risk of bowel obstruction

Answer 499

A

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.

Answer 500

A

4 years old, topical steroid.

Answer 501

A

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.

Answer 502

A

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

Answer 503

A

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).

Answer 504

A

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

Answer 505

A

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

Answer 506

A

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)

Answer 507

A

(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)

Answer 508

A

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

Answer 509

A

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

Answer 510

A

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

Answer 511

A

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

Answer 512

A

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

Answer 513

A

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

Answer 514

A

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

Answer 515

A

Enterocolitis

Decompression washout enemas, IV ABx and intensive care

Answer 516

A

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

Answer 517

A

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)

Answer 518

A

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.

Answer 519

A

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

Answer 520

A

Staphylococcus aureus

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

Answer 521

A

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

Answer 522

A

Acute

Gastro

Chronic

Coeliac

Lactose intolerence (temporary after gastro or permanent)

Pancreatic insufficiency from CF

Answer 523

A

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

Answer 524

A

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.

Answer 525

A

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

Answer 526

A

80% of preterm infants

60% of term infants

Answer 527

A

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

Answer 528

A

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

Answer 529

A

Slera = 50

Body = 100 (progresses cephalocaudally)

Answer 530

A

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

Answer 531

A

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

Answer 532

A

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

Answer 533

A

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

Answer 534

A

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

Answer 535

A

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

Answer 536

A

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’

Answer 537

A

Determine the time course of the jaundice
1. early, high, accelerating, late
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
Ask about symptoms of kernicterus
1. irritability
2. decreased appetite
3. seizures
4. drowsiness
5. arching back
Screen for kernicterus risk factors
1. prematurity
2. low albumin
3. Sepsis, particularly meningitis
4. Asphyxia, acidosis, hypoxia and hypothermia

Answer 538

A

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.

Answer 539

A

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

Answer 540

A

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

Answer 541

A

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

Answer 542

A

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)

Answer 543

A

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

Answer 544

A

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)

Answer 545

A

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!)

Answer 546

A

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

Answer 547

A

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

Answer 548

A

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.

Answer 549

A

Basics
Admit, ABC support and monitoring

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

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

Follow up

Answer 550

A

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.

Answer 551

A

Infants with a suggestive clinical examination

OR

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

Answer 552

A

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

Answer 553

A

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)

Answer 554

A

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.

Answer 555

A

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

Answer 556

A

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

Answer 557

A

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

Answer 558

A

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

Answer 559

A

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+)

Answer 560

A

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.

Answer 561

A

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!

Answer 562

A

Oligo = no

Poly = can be, particularly if RF+

Answer 563

A

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.

Answer 564

A

>6yo boys

10% progress to AnkSpond

Answer 565

A

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

Answer 566

A

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

Answer 567

A

Jumping up and down leading to severe pain

Answer 568

A

Boot shaped heart

Tetralogy of Fallot

Answer 569

A

Damage from intubation

Presents with chronic fixed stridor

Classically an ex-preterm infant who had long term ventilation

May require endoscopic ablation to resolve

Answer 570

A

Palivizumab monoclonal antibody against RSV

Super expensive, only for very high risk babies

Give as monthly injections throughout RSV season

Answer 571

A

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

Answer 572

A

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.

Answer 573

A

Long term mineralocorticoid and glucocorticoid replacement.

Answer 574

A

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?

Answer 575

A

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.

Answer 576

A

Good. Most grow out of it.

Answer 577

A

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

Answer 578

A

18 months

Answer 579

A

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%

Answer 580

A

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

Answer 581

A

Tachycardia,

Tachypnoea

Metabolic acidosis.

Lactate commonly high.

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

Answer 582

A

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.

Answer 583

A

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

Answer 584

A

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.

Answer 585

A

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

Answer 586

A

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.

Answer 587

A

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.

Answer 588

A

Foetal alcohol syndrome

Most distintive features are thin upper lip and indistinct philtrum

Answer 589

A

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.

Answer 590

A

Nitric oxide (NO)

Answer 591

A

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

Answer 592

A

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

Answer 593

A

15mg / kg

So a 30kg 6 year old would get 450mg

Answer 594

A

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).

Answer 595

A

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.

Answer 596

A

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

Answer 597

A

Think of:

Reduced calories in
Reduced absorption
Increased calories out
Increased caloric requirement
Inability to use calories

Reduced calories in
- Recurrent vomiting / possets
- Poor sucking reflex
- Cleft palate
- Underlying organic pathology –> tiring at the breast
Reduced absorption
- Coeliac disease
- Milk protein allergy
- Pancreatic insufficiency (eg. CF)
Increased calories out
- Diarrhoea
Increased caloric requirement
- Anything!
- Congenital heart disease
- Sepsis
- Hyperthyroidism
Inability to use calories
- Inborn errors of metabolism

Answer 598

A

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.

Answer 599

A

FRANGRANT PPP THHD

F - Feeding difficulty

R - Respiratory distress syndrome (RDS)

A - Anaemia

G - GORD

R - Retinopathy of prematurity

A - Apneoa of prematurity

N - NEC

T - Transient tachypneoa of the newborn

P - Patent PDA

P - Physiological jaundice

P - Periventricular leukomalacia

T - Thermoregulation problems

H - Hypoglycaemia

H - Haemorrhage (intraventricular)

D - Developmental problems (ID, cerebral palsy)

Answer 600

A

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

Answer 601

A

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

Answer 602

A

4j per kg

Answer 603

A

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

Answer 604

A

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.

Answer 605

A

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.

Answer 606

A

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

Answer 607

A

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

Answer 608

A

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

Answer 609

A

non-independent walking

non-independent feeding

epilepsy

blindness

hearing problems

Later then have learning difficulties and behavioural problems

Answer 610

A

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).

Answer 611

A

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

Answer 612

A

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.

Answer 613

A

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.

Answer 614

A

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.

Answer 615

A

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.

Answer 616

A

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

Answer 617

A

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

Answer 618

A

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

Answer 619

A

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

Answer 620

A

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

Answer 621

A

Ethosuximide is used commonly to treat absence seizures. Ethosuximide is ineffective in controlling tonic-clonic seizures, so is not used in children with juvenile absence epilepsy. 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. Intellectual development isusually normal.

Answer 622

A

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,

Answer 623

A

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.

Answer 624

A

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.

Answer 625

A

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.

Answer 626

A

Anterior fontanelle closes at 18-24 months

Posterior fontanelle closes at 3 months

Answer 627

A

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.

Answer 628

A

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.

Answer 629

A

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.

Answer 630

A

Ankyloglossia

May require surgery if severe

Answer 631

A

Pierre Robin Sequence

microganthia and cleft palate can cause severe respiratory problems

Answer 632

A

Around 18 months

Answer 633

A

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

Answer 634

A

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

Answer 635

A

Spastic cerebral palsy: - most common type (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

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

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

Mixed

Answer 636

A

1 in 500 Australian babies is diagnosed with cerebral palsy.

Answer 637

A

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).

Answer 638

A

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

Answer 639

A

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.

Answer 640

A

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

Answer 641

A

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

Answer 642

A

What were you doing at the time the headache first commenced?
What had you been doing beforehand?
What was the first thing you noticed and how long did it take from when you first noticed the symptom until it reached its maximum severity?

Headache Differentials QUESTION CLUSTERS

MIGRAINE
- Did you see any funny smells, flashing lights, a funny sensations leading up to the headache, some people say it’s like an aura?
- Did you vomit?
- Does the light hurt your eyes?
CLUSTER HEADACHE
- Did you get very bad pain in your eye?
- Or have you had any tearing up in one eye?
- Or was there watery discharge from your nose?
- Do you have any sinus symptoms?
TENSION HEADACHE
- Is it recurrent? How regularly do you get it?
- Do they get worse over the day?
- How are things going generally? Is work okay? Is any thing stressful going on in your life?
HAEMORHAGE (Sub-Arachnoid)
- Have you had any trauma?
- Did it feel like a thunderclap when it started?
- Does your neck feel stiff?
- Is it at the back of the head?
- Have you noticed any weakness in your body? Numbness? Have you had any speech or vision changes?
STROKE
- Have you felt any weakness anywhere in your arms leg or face?
- Have you had any funny sensations anywhere in arms legs or face?
- Has your vision been affected?
- Has your speech become slurred?
- Did you lose any control of your bladder?
MENINGITIS
- Have you felt any neck stiffness?
- Any fevers? Sweats? Shakes?
- Are you sensitive to light?
TEMPORAL ARTERITIS
- When you chew food does it hurt?
- Have you lost vision in one eye?
- Is your scalp tender, say when you comb your hair?
MALIGNANCY
- Fever, sweats, shakes
- Loss of weight, loss of appetite, fatigue,
- Nausea & vomiting
- Prominent headache behind your eye
- Weakness
- Better on leaning back, worse leaning forward
REBOUND HEADACHE
- Are you taking any medication to manage the pain?
- What kind and how much?

Answer 643

A

Anatomy

Extradural space. Between the dura mater and the skull. (That is, both layers of dura are squashed against brain by pooling blood).

Classically transection of the anterior division of the middle meningeal artery running behind the pterion.

Clinical vignette

Traumatic injury to side of head in kids or young adult. Fluctuating conscious state.

Symptoms come on within hours of injury (arterial bleed –> fast!)

Management

Surgical evacuation

Answer 644

A

Anatomy

In the potential space between the dura and the subarachnoid. That is, both layers of dura are squashed against skull, the arachnoid is pushed against brain.

Transection of the cerebral veins (as they enter the sinuses).

Presentation

Older people, usually after acceleration-deceleration injury (imagine the brain shakes around in the head) eg. after fall. This is because in the older population the brain shrinks, stretching the veins which bridge the subdural space.

This occurs in alcoholics too for the same reasons.

Symptoms come on more slowly, within weeks to months. Think of the little old lady who has a fall and presents with focal neurology 3 weeks later.

Management

Surgical evacuation

Answer 645

A

Anatomy

Subarachnoid space. Between the arachnoid and pia mater, mixed in with the CSF.

Classically an aneurysm of the Circle of Willis.

Presentation

Often sponteneous.
1-6% of genpop have a subdural aneurysm. 1% of aneurysms bleed.
Classically women under 60 with lots of vascular risk factors.
“Thunderclap headache”
- The worst headache the patient has ever experienced.
- The pt will be able to pinpoint the second the headache started à “I was reaching for the remote.”
- May also get signs of meningism, as the pia and arachnoid mater are irritated (positive Kernig’s and Brudzinski’s sign, nuchal rigidity and photophobia).
- They also can get a ‘sympathetic surge’.

Management

Calcium channel blockers

Surgery (coil/clip)

Answer 646

A

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

Answer 647

A

PATIOS

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

Answer 648

A

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

Answer 649

A

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?

Answer 650

A

Cystic fibrosis transmembrane conductance regulator (CFTR) ΔF508

Chromosome 7

Answer 651

A

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

Answer 652

A

6 puffs every 20 minutes x 3 if

12 puffs every 20 minutes x 3 if > 6 yo

Answer 653

A

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

Answer 654

A

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.

Answer 655

A

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.

Answer 656

A

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?

Answer 657

A

SHIRTFRONT LAD AJ

Sepsis
Hypoglycaemia (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

Answer 658

A

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)

Answer 659

A

MR PANCREAS

Meconium Ileus / Distal Intestinal Obstruction

Recurrent infections

Pancreatic failure (exocrine and endocrine)

Allergic Bronchopulmonary Aspergilliosis

Nasal polyps and sinusutus

Cirrhosis of the liver

Restricted growth

Emotions

Airway leaks (males)

Sterility in males

Answer 660

A

Levels of immunoreactive tripsin are tested for on the Gutherie Card (with the blood collected via heel prick test).

Answer 661

A

CFTR gene mutations screened for on Gutherie Card

(most common is delta F507)

+/-

Sweat Test

Answer 662

A

1:2500

autosomal recessive

Delta F507 (Long arm of Chromosome 7)

Answer 663

A

Congenital hypothyroidism

CF

Metabolic disorders eg. PKU

Answer 664

A

Concerning Airway Wall

Constrction*
Asthma
Compression*
Mediastinal mass
Congenial heart disease eg. causing L-R shunt and causing engorgment of the pulmonary vasculature

Structure

Bronchomalacia
Tracheomalacia
Bronchopulmonary Dysplasia

Concerning Airway Lumen

Pus / Infection

Bronchiolitis
Viral Induced Wheeze / Asthma
Pneumonia
Petussis
Croup (even though an URTI)
Gunk*
CF
Other*
FB

Answer 665

A

With Fever

Abcess (peritonsillar - qunsiy, or retrophrayngeal)
Big tonsils / bacterial tonsillitis
Croup
Diptheria
Epiglottitis

Without Fever

Floppy airways (tracheomalacia, laryngomalacia)
Gagging on a foreign body
Hypersensitivity / haemangioma

Answer 666

A

Infrequent Intermittent

Frequent Intermittent

Persistent

Mild
Moderate
Severe

Answer 667

A

Attacks are >6 weeks apart

No interval symptoms (morning cough / wheeze, night cough / wheeze, exercise tolerance, days at school missed)

Answer 668

A

Attacks are

No interval symptoms (morning cough / wheeze, night cough / wheeze, exercise tolerance, number of days missed at school)

Answer 669

A

Attacks

Mild = interval symptoms are less than 1 x per week

Moderate = 1 x per week

Severe = >1 x per week

Answer 670

A

Morning cough / wheeze?

Night cough / wheeze?

Reduced exercise tolerance? (Able to keep up with kids in sport / at school)

Missed days at school because of asthma?

Answer 671

A

Q - how often do you have attacks?

Q - when you do have attacks, do you have to

miss school
go to ED
be admitted to the ward
be admitted to ICU
take prednisolone (how many times have you used prednisolone in the last 12 months)

A - what triggers your asthma?

A - which medications allieviate it? Have you had to take prednisolone in the last 12 months? How often do you have to use your reliever?

Answer 672

A

SABA prn

+preventor (ICS or chromone or LTRA or combination)

Answer 673

A

SABA prn

Symptoms controller: ICS + or minus LTRA / cromone

LABA

oral corticosteroid

Answer 674

A

Primary Features

WOB
Neurological Status

Secondary Features

SPO2
HR
Ability to talk

Answer 675

A

Increased RR
Cyanosis
Grunting
Nasal flare
Intercostal / Subcostal recession
Head bobbing

Answer 676

A

OASIS

Oxygen

And (Amyophilline / MGSO4 if severe, in ICU)

Salbutamol

Ipatropium Bromide

Steroids

Answer 677

A

Basics

make comfortable and reassure
paracetamol for comfort if febrile due to viral induced
assess hydration status: oral, NG or IV fluids if dehydrated (but avoid IV, procedure will make breathing more difficult)

Place and Person

Contact Paeds resp team, likely to be monitored in ED

Ix and Confirm Diagnosis

SpO2

Definitive Management

O - no oxygen unless SpO2 is

A

Salbutamol - 6 puffs if 6

I

Steroids - 1mg/kg of prednisolone for 3 days

Plan / Follow Up

Medication review

Education RE asthma delivery devices

Answer 678

A

Basics

Make the child comfortable
Paracetamol for comfort if febrile (viral-induced exacerbation)
Assess hydration status - encourage oral fluids, consider NGT or IV fluids if necessary, but try to avoid procedures as this may further upset patient

Place and Person

Contact paediatric team

Ix and confirm Dx

SpO2

Definitive Management

Oxygen via NP
A
Salbutamol - burst therapy:
- Six puffs every 20 minutes x 3 if
- 12 puffs every 20 mins x 3 if >6yo
Iprtropium bromide - not in moderate asthma in victoria
Steroids: 1mg/kg for 5 days

Follow Up

Medication review
Ensure correct use of asthma delivery devices

Answer 679

A

6 puffs of salbutamol every 20 minutes x 3 if

12 puffs of salbutamol every 20 minutes x 3 if >6yo

In moderate or severe exacerbations of asthma

Answer 680

A

in severe exacerbations of asthma

4 puffs every 20 mins if

8 puffs every 20 minutes if >6 years old (with salbutamol)

Answer 681

A

Basics

Comfort child (but the child is usually flat)
Paracetamol for comfort if febrile
Oral, NG or IV fluids if dehydrated

Place and Person

Contact paeds resp / paeds ICU
Contact PIPER if rurally

Investigate and confirm Dx

SpO2
ABG (if doesn’t further upset child)

Definitive management:

Oxygen - via HFNP or CPAP or consider intubation
Salbtamol Burst therapy - MDI or nebulised
Ipretropium bromide
- 4 puffs every 20 minutes if
- 8 puffs every 20 minutes if >6yo, x 3
Steroids - IV methylprednisolone
MgSO4 or amiophilline in ICU

Follow Up

Medication review
Ensure correct understanding and usage of asthma delivery devices