Paediatrics 1

Question 1

Neonate NEWBORN BLS

5 steps

Answer 1

1. Dry baby and maintain temperature
2. Assess tone, respiratory rate, heart rate
3. If gasping or not breathing give 5 inflation breaths*
4. Reassess (chest movements)
5. If the heart rate is not improving and <60bpm start compressions and ventilation breaths at a rate of 3:1

*Inflation breaths are different from ventilation breaths. The aim is to sustain pressure to open the lungs.

Question 2

8-year-old boy presented with:
- progressive gait disturbance and falls, unsteady gait and toe walking at the age of 4 years.
- Tremors in the hands were noted sometime prior to this visit.
- Gait was wide-based and unsteady
- pes cavus
- mild scoliosis
- absence of cardiac murmur.

diagnosis and genetic pattern?

Answer 2

Friedreich’s ataxia is autosomal recessive

Question 3

Perthes disease- Avascular necrosis of head femoral head

More common in boys or girls?

Caterall Staging
Stage 1
Stage 2
Stage 3
Stage 4

Answer 3

5 times more common in boys

Caterall Staging

Stage 1 Clinical and histological features only
Stage 2 Sclerosis with or without cystic changes and preservation of the articular surface
Stage 3 Loss of structural integrity of the femoral head
Stage 4 Loss of acetabular integrity

Question 4

Perthes Disease Management
1. Supportive:
2. < 6yo
3. Older
4.

Answer 4

1. To keep the femoral head within the acetabulum: cast, braces
2. If less than 6 years: observation
3. Older: surgical management with moderate results
   4 .Operate on severe deformities

Question 5

ITP Management

Usually…

BUT
1
2
3

Answer 5

• usually, no treatment is required. Advice about preventing trauma for bleeding

BUT if platelets <10 * 109/L) or there is significant bleeding:
- oral/IV corticosteroid
- IV immunoglobulins
- platelet transfusions can be used in an emergency (e.g. active bleeding) but are only a temporary measure as they are soon destroyed by the circulating antibodies

Question 6

Going back to school rules:
- Rubella
- Scarlet fever
- Hand foot and mouth
- Whooping cough
- Measles
- Chickenpox
- Mumps
- Infectious gastroenteritis
- Impetigo

Answer 6

• Rubella- 5 days from rash onset
• Scarlet fever- 24 hours from when abx started
• Hand foot and mouth- none

Whooping cough- 2 days after commencing abx (or 21 days from onset of symp if no abx)

• Measles- 4 days from rash onset
• Chickenpox- until all lesions have crusted over
• Mumps- 5 days from swollen glands
• Infectious gastroenteritis- 48 hours from when symptoms have settled
• Impetigo- Until lesions are crusted and healed, or 48 hours after commencing antibiotic treatment

Question 7

Fragile X syn.

genetic pattern

features 6

Answer 7

X-linked dominant

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

Question 8

Is conjugated or unconjugated bilirubin elevated in biliary atresia?

Answer 8

Conjugated bilirubin is elevated in biliary atresia

Unconjugated -> conjugated in the liver

Question 9

Before starting anti-TNF med, what investigation would you do and why?

Answer 9

CXR- to rule out latent TB which can be reactivated

Question 10

Seborrhoeic dermatitis in children

aka

Site? (4)
Describe the rash basically

Management:
1.
2.
3. If severe/ persistent

Answer 10

aka- ‘Cradle cap’

site- scalp, nappy area, face, limb flexures

erythematous rash with coarse yellow scales

Management
1. Reassurance that it doesn’t affect the baby, usually resolves within a few weeks
2. Massage a topical emollient onto the scalp to loosen scales, brush gently with a soft brush and wash off with shampoo.
3. If severe/persistent a topical imidazole cream may be tried

Question 11

Knee pain children and young adult STEMS

1. Chondromalacia patellae -
2. Osgood-Schlatter disease
   (tibial apophysitis)
3. Osteochondritis dissecans
4. Patellar subluxation
5. Patellar tendonitis

Answer 11

Chondromalacia patellae - teenage girls, anterior knee pain on walking up + down stairs, rising from prolonged sitting

2. Osgood-Schlatter- sporty teenagers, Pain, tenderness + swelling over the tibial tubercle, Pain on bending down
3. Osteochondritis dissecans- Pain after exercise
   Intermittent swelling and locking
4. Patellar subluxation- Medial knee pain due to lateral subluxation of the patella
   Knee may give way
5. Patellar tendonitis- athletic teenage boys, Chronic anterior knee pain that worsens after running
   Tender below the patella on examination

Question 12

Risk factors for noonatal hypoglycaemia

Answer 12

1. preterm birth (< 37 weeks)
2. maternal diabetes mellitus
3. IUGR
4. hypothermia
5. neonatal sepsis
6. inborn errors of metabolism
7. nesidioblastosis
8. Beckwith-Wiedemann syndrome

Question 13

Managment of threadworm

Answer 13

Single dose of oral Mebendazole and for the entire household + hygeine advice

Question 14

Down syndromes are at in crease of what with regards to contact sport STEM!

Answer 14

atlanto-axial instability- If participate in specific sports, they may carry an increased risk of neck dislocation (e.g. trampolining, gymnastics, boxing, diving, rugby and horse riding)

Question 15

Pataus is what chromsome?

Features? (4)

Answer 15

13

Microcephalic, small eyes
Cleft lip/palate
Polydactyly
Scalp lesions

Question 16

Diet advice for children with ADHD

Answer 16

Normal balanced

Question 17

If its a young teen with hip pain, how can you differenciate between Perthes and SUFE? (2)

Answer 17

SUFE-
Age 10-15yo
key examination finding being the loss of internal rotation of the leg in flexion.

Perthes
3-10yo
NO LIMIT IN MOVEMENT

Question 18

Congenital heart defect

Cyanotic presenting in the first days of life =

Cyanotic presenting in the first 1-2 months =

Answer 18

Days = Transposition of great vessels

Months = TOF

Question 19

What vaccines do you recive between the ages of 12-13 and then 13-18yo?

Answer 19

12-13yo = HPV vaccination
13-18 years = ‘3-in-1 teenage booster’ (tetanus, diphtheria and polio)
Men ACWY

Question 20

When should you routinely assess APGAR score?

Answer 20

1 and 5 minutes of age

Question 21

Whooping cough going back to school rules?

Also what abx is used?

Answer 21

48 hours following commencement of antibiotics (or 21 days from onset of symp if no abx)

clarithromycin

Question 22

Briefly explain the pathology and what you’d see on a hormonal panel in these conditions causing delayed puberty in females?

Turners-

Consitutional delay of puberty

Congenital adrenal hyperplasia

Functional hypothalamic amenorrhoea

Premature ovarian insufficiency

Answer 22

Turners- Due to ‘streak’ ovaries- Ovaries dont produce oestrogen but increase in LH and FSH. Also low anti-mullarian hormone (low reserve)

Consitutional delay of puberty- Normal hormonal panel, LH and FSH will not be elvated in pre-pubertal girls

Congenital adrenal hyperplasia-

Functional hypothalamic amenorrhoea- usually a result of stress, eating disorder or intense exercise resulting in depression of GnRH. Might expect to see a low BMI, which causes a low level of LH and FSH to be released into the bloodstream

Premature ovarian insufficiency- ovaries entering a menopausal state early, usually before a patient’s 40s. The oestrogen level decreases resulting in a high LH and FSH level

Question 23

What is hypospadias and what condition is assoc. with it?

3 management points for hypospadias

Answer 23

hypospadias- congenital abnormality of the penis. Urethra comes out on the ventral side.

Assoc. with Cryptorchidism (undestended testes) 1/10 with hypospadias have cryptorchidism therefore examine groin.

Management:
- Make sure they pass urine in first 24 hours
- Don’t get circumcised because foreskin can be used in surgery
- Surgery at 12 months

Question 24

Major risk factors for SIDS ( sudden infant death syndrome) are: (5)

Answer 24

• prone sleeping
• parental smoking
• bed sharing
• hyperthermia and head covering
• prematurity

Question 25

What is alpha thalassaemia? What chromo? There are two seperate alpha-globulin genes located on each chromosome.... Severity depends on the number of alpha globulin alleles affected: 1 or 2 alpha globulin alleles affected = If are 3 alpha globulin alleles are affected = If all 4 alpha globulin alleles are affected (i.e. homozygote) =

Answer 25

- Due to a deficiency of alpha chains in haemoglobin chromosome 16 1 or 2 alpha globulin alleles affected = hypochromic and microcytic, but Hb levels would be typically normal 3 = hypochromic microcytic anaemia with splenomegaly. This is known as Hb H disease All 4 = death in utero (hydrops fetalis, Bart's hydrops)

Question 26

Kochers criteria for septic arthritis

Answer 26

1. Non-weight bearing - 1 point 2. Fever >38.5ºC - 1 point 3. WCC >12 * 109/L - 1 point 4. ESR >40mm/hr 5.

Question 27

Receiving the MMR as a child later in life. When do you give second dose? Max response =

Answer 27

Max response = 3 months between doses to maximise the response rate 1 month is considered adequate if the child is > 10 years of age. In an urgent situation (e.g. an outbreak at the child's school) then a shorter period of 1 month can be used in younger children.

Question 28

If you have a VSD what are you at an increase of getting later in life?

Answer 28

endocarditis

Question 29

Management steps for cows milk protein allergy If formula fed 1. First-line = 2. If breast fed 1. 2. 3. When breast feeding stops-

Answer 29

If formula fed 1. extensive hydrolysed formula (eHF) milk is the first-line with mod symp 2. Amino acid-based formula (AAF) in infants with severe CMPA or if no response to eHF 10% of infants are also intolerant to soya milk If breast fed 1. continue breastfeeding 2. eliminate cow's milk protein from maternal diet. Can have calcium supplements for mum 3. When breast feeding stops- eHF milk when breastfeeding stops, until 12 months of age and at least for 6 months

Question 30

ADHD med methylphenidate side effect 5 but 1 main 1

Answer 30

stunted growth insomnia, weight loss, anxiety, nausea and pain.

Question 31

What is the number one cause of painless massive GI bleeding requiring a transfusion in children between the ages of 1 and 2 years.

Answer 31

Meckels diverticulum results from the failure of the vitelline duct to obliterate during the fifth week of fetal development

Question 32

Mitochondrial diseases are inherited following 3 principles:

Answer 32

- Affected males cannot pass it onto their children - Affected females will pass it onto all of their children - All individuals who inherit the affected mitochondria, will have the disease

Question 33

Complete androgen insensitivity- - classic presentation (STEM 2) what is it?

Answer 33

- primary amenorrhoea - You get bilateral inguinal hernias which are actually undescended testes - Small breast bud development and spare public hair due to aromatase androgens phenotypically female genitalia in a genetic male, caused by failure of androgen masculinisation of genitalia in-utero

Question 34

Route and how many times do you give Vit K to a neonate? why is it given? Who is at risk of this? (2)

Answer 34

Once- off dose IM injection can also be given orally but IM is preferred if compliance is an issue with parents Why? Given to prevent hemorrhagic disease - breast fed babaies - mums on anti-epileptics

Question 35

What exam findings would you ding with neonate Congenital diaphragmatic hernia (2 and 1 STEM)

Answer 35

- dyspnoea and tachypnoea at birth STEM - classical concave abdominal appearance - Reduced breath sound bilaterally

Question 36

Genetic inheritance pattern of Sickle cell?

Answer 36

Autosomal recessive

Question 37

DDH Indication for 2 week hip USS Indication for 6 week USS

Answer 37

2 weeks- abnormal birth check with ortolini and barlow 6 weeks - Breech at birth - Breech at 36 weeks - 1st degree relatives with Hx of Hip problems - Twin/Multiple pregnancy

Question 38

Management of thread worm a rogue fact which is very frustrating...

Answer 38

One off Mebendazole for patient and all family members + hygeine Cannot be given to < 6 months!)

Question 39

Febrile convulsions Simple criteria (4) Complex criteria (3) Febrile status

Answer 39

Simple criteria (4) - < 15 minutes - generalised - don't reoccur in 24 hours - complete recovery within 1 hour Complex criteria (3) - 15- 30 mins - focal seizure - may repeat within 24hrs/ same illness Febrile status - > 30 mins

Question 40

Orofacial cleft lip causes what maternal drugs in preg? (2) Maternal factor in preg - Infection in preg syndromic disorders (3)

Answer 40

what maternal drugs in preg? - Benzos - Anti-epileptics (phenytoin) Maternal factor in preg - smoking Infection in preg - rubella syndromic disorders - **18- edwards - 13 pataus - 15 - Prader-Willi syndrome?

Question 41

Genetic pattern of inheritance Haemophilia A?

Answer 41

X-linked recessive Therefore only mother to son passing

Question 42

Features which may be present in a sexually abused child (8)

Answer 42

- pregnancy - sexually transmitted infections, recurrent UTIs - sexually precocious behaviour - anal fissure, bruising - reflex anal dilatation - enuresis and encopresis -behavioural problems, self-harm - recurrent symptoms e.g. headaches, abdominal pain

Question 43

undescended unilateral testicle what surgery is done? Bilateral undescedended testes management?

Answer 43

Reassure and Referral if at 3 months of age hasn't descended child should be referred to a paediatric surgeon and seen before 6 months of age Orchidopexy- this should occur at around 1yo BILATERAL- Review by senior paed within 24 hours as the child may need urgent endocrine or genetic investigation