Plant

Question 1

What is the classic triad of Henoch–Schönlein purpura?

Answer 1

Purpura
Joint pain/ Arthritis
Abdominal Pain

Question 2

What is HSP?

Answer 2

Henoch–Schönlein purpura.
It is a systemic Vasculitis caused by deposition of immune complexes containing IgA
Most common in children under 10 (90%) under 6 (60%)

Question 3

At what age does the anterior fontanelle usually close?

Answer 3

18-24 months of age

Question 4

What is most common cause of gastroenteritis in children? (UK)

Answer 4

Rotavirus
Most will have by 5 years and develop immunity
Next most common is adenovirus

Question 5

What is Intussusception?

Answer 5

Invagination if the proxinal bowel into a dustal segment- usually ileum into caecum through the ileocecal valve

Question 6

What is common presentation of Intussusception?

Answer 6

3 months- 2yrs
RED CURRENT JELLY stool containing blood and mucus
Paroxysnal colicky pain when child draws legs upward

Question 7

What are kernig and bardzinski’s signs?

Answer 7

Kernig +ve when thigh is flexed at the hip + knee is at 90• angle. Extension of knee from this postition is painful
Burdzinski +ve when forced flexion of the neck elicits a reflex flexion of the hip and knee and abduction of the leg

Question 8

What are +ve kernig and burdzinski’s signs suggeative of?

Answer 8

Meningitis
Subarachnoid haemorrhage
Possibly encephalitis

Question 9

What is pre-hospital setting treatment for meningitis?

Answer 9

IM benzylpenicillin

Question 10

What is the hospital management of meningitis?

Answer 10

Lumbar puncture to confirm diagnosis
IV ceftriaxone ASAP!
Dexamthasone as adjunct to antibiotic (reduce complications)

Question 11

What test are run on CSF sample in suspected meningitis?

Answer 11

PCR for virology, pneumococcus, meningitis, glucose, protein, culture and sensitivity , microscopy and gram stain

Question 12

Children at high risk of sepsis are those with:

Answer 12

Congenital heart disease
Significant burn injury
Sickle cell
Neonates
Asplenia
Chronic steroid dependency

Question 13

What is the treatment for moderate/severe croup (aka laryngotrachealbronchitis)

Answer 13

Nebulised adrenaline
Oral dexamethasone 0.15mg/kg as a single dose
Paracetamol

Question 14

Mutation in what gene causes Cystic Fibrosis and what type of mutation is it?

Answer 14

The CFTR gene
Most common serious autosomal recessive condition in the European Caucasian population (approximately 1/2500 live births)

Question 15

What is the function of the CFTR Gene?

Answer 15

Codes for the CFTR protein essential to sodium transport channels across epithelial cell membranes. Mutations cause thickening of mucus predisposing to the blockage of pancreatic ducts, vas deferens and recurrent respiratory and sinus infections.

Question 16

What are neonates tested for when screening for CF?

Answer 16

Immunoreactive Trypsinogen (IRT), this is released from the pancreas, usually in low levels but is usually high in CF- not reliable after 2 weeks of life

Question 17

How is CF managed?

Answer 17

regular (at least twice daily) chest physiotherapy and postural drainage. Parents are usually taught to do this.
Deep breathing exercises are also useful
high calorie diet, including high fat intake*
vitamin supplementation
pancreatic enzyme supplements taken with every meals
heart and lung transplant

Question 18

What is hirschprungs disease?

Answer 18

Absence of ganglion cells from myenteric and submucosal plexuses results in neonate not passing meconium
Occurs in 1/5000 births
Full thickness rectal biopsy for diagnosis

Question 19

What is the treatment for hirschprungs disease?

Answer 19

Initially rectal washout procedure

Then an anorectal pull through procedure

Question 20

What is a meconium

Answer 20

Meconium is the earliest stool of a mammalian infant. Unlike later feces, meconium is composed of materials ingested during the time the infant spends in the uterus: intestinal epithelial cells, lanugo, mucus, amniotic fluid, bile, and water.
Dark green sludge!

Question 21

What is Roseola infantum?

Answer 21

A common disease of infancy caused by the human herpes virus 6 (HHV6). It has an incubation period of 5-15 days and typically affects children aged 6 months to 2 years. Treatment supportive

Question 22

What are the symptoms of roseola infantum?

Answer 22

high fever: lasting a few days, followed by a
maculopapular rash
febrile convulsions occur in around 10-15%
diarrhoea and cough are also commonly seen

Question 23

Define Cerebral Palsy

Answer 23

A disorder of movement and posture due to a non-progressive lesion of the motor pathways in the developing brain. It affects 2 in 1,000 live births and is the most common cause of major motor impairment

Question 24

Projectile vomiting after every feed that is not settling is suspicious of what in neonates?

Answer 24

Pyloric stenosis

Question 25

What is pyloric stenosis?

Answer 25

Hypertrophy of the circular pyloric muscles leading to stenosis of the pylorus. Incidence of 4 per 1,000 live births, 4 times more common in males, 10-15% of infants have a positive family history,first-borns are more commonly affected

Question 26

What is hand foot and mouth disease?

Answer 26

A self-limiting condition affecting children characterized by vesicles on the palms and soles of the feet, oral ulcers and mild symptomatic unwellness. It is caused by the intestinal viruses of the Picornaviridae family (most commonly coxsackie A16 and enterovirus 71). It is very contagious and typically occurs in outbreaks at nursery

Question 27

``` What childhood syndrome has the following features? Micrognathia Low-set ears Rocker bottom feet Overlapping of fingers ```

Answer 27

Edward’s syndrome , trisomy 18

Question 28

``` What childhood syndrome has the following features? Microcephalic, small eyes Cleft lip/palate Polydactyly Scalp lesions ```

Answer 28

Patau syndrome, trisomy 13

Question 29

``` What childhood syndrome has the following characteristics? Learning difficulties Macrocephaly Long face Large ears Macro-orchidism ```

Answer 29

Fragile X syndrome

Question 30

``` What childhood syndrome has the following features? Webbed neck Pectus excavatum Short stature Pulmonary stenosis ```

Answer 30

Noonan syndrome

Question 31

What childhood syndrome has the following features? Micrognathia Posterior displacement of the tongue (may result in upper airway obstruction) Cleft palate

Answer 31

Pierre-Robin syndrome or Treacher-Collins syndrome (the autosomal dominant version)

Question 32

What childhood syndrome has the following features? Hypotonia Hypogonadism Obesity

Answer 32

Prader-Willi syndrome

Question 33

``` What childhood syndrome has the following features? Short stature Learning difficulties Friendly, extrovert personality Transient neonatal hypercalcaemia Supravalvular aortic stenosis ```

Answer 33

William’s syndrome

Question 34

What are the types of acute lymphoblastic leukaemia ?

Answer 34

common ALL (75%), CD10 present, pre-B phenotype T-cell ALL (20%) B-cell ALL (5%)

Question 35

What is inheritance of haemophilia A?

Answer 35

X linked recessive

Question 36

What are the signs, in the baby, of maternal alcohol abuse during pregnancy.

Answer 36

Presentation: Intra Uterine Growth Restriction, microcephaly, midfacial hypoplasia, micrognathia, smooth philtrum, microphthalmia, short palpebral fissures, thin upper lip, irritability, ADHD.

Question 37

Define micrognathia

Answer 37

The jaw is undersized

Question 38

Define dermoid cyst

Answer 38

Dermoid cysts occur at sites of embryonic fusion and may contain multiple cell types. They occur most often in children.

Question 39

When can a child go back to school after gastroenteritis infection

Answer 39

48hours after symptoms subside

Question 40

What is the triad of haemolytic uraemic syndrome?

Answer 40

Haemolytic anaemia Thrombocytopenia AKI

Question 41

How to reduce likelihood of sudden infant death syndrome?

Answer 41

Lie baby flat on back, don’t share bed with baby, don’t bundle the baby up too much with too many quilts/ blankets and breastfeed

Question 42

What two dangerous types of infection can haemophilus influenza B cause?

Answer 42

Epiglotitis | Meningitis

Question 43

What are the components of fallot's tetralogy

Answer 43

Ventricular septal defect, pulmonary artery stenosis, right ventricular hypertrophy, overriding aorta

Question 44

How much should I feed my baby?

Answer 44

If under a month old 150mL/kg/day | If over a month old 100mL/kg/day

Question 45

What is the inheritance of Marfan syndrome?

Answer 45

Autosomal dominant (though 25% of cases are spontaneous mutations of FBN1 gene)

Question 46

What are the features of Marfan syndrome?

Answer 46

Connective tissue disorder-Dislocated lens (ectopia lentis), Aorta weakness leading to aneurysm and aortic dissection also atrial valve regurgitation, mitral valve prolapse, Tall, slender, elongated fingers and toes (arachnodactyly)

Question 47

What is Hyper obstructive cardiomyopathy ?

Answer 47

``` Autosomal dominant condition characterized by thickening of Diastolic dysfunction Mitral regurgitation (due to the Venturi effect) Arrhythmia End-stage HOCM results in systolic dysfunction, or “burnt out HOCM” ```

Question 48

What is the autism triad of impairments?

Answer 48

Deficits in flexible thinking regarding interests, routines, perspectives and rules Deficits in understanding how to behave and interact with other people Deficits in ability to communicate effectively with other people

Question 49

Treatment for GORD in children?

Answer 49

Sit baby up for 30mins after eating. Gaviscon Ranitidine

Question 50

What is shoulder distocia

Answer 50

Shoulder of baby’s gets stuck on the pubic symphysis. Can cause bruising, broken clavicles and nerve damage.

Question 51

Define anorexia nervosa

Answer 51

BMI less than 17.5 or weight less than 85 % of predicted based on height

Question 52

What are the three categories of reasons a child might fail to thrive?

Answer 52

Inadequate calorie intake Inadequate calorie absorption Excessive calorie requirement

Question 53

What is choanal atresia?

Answer 53

Congenital disorder where posterior nasal airway is occluded by soft tissue or bone. Associated with other congenital conditions, cyanosis is worse during feeding

Question 54

What is the classic presentation of androgen insensitivity?

Answer 54

groin swellings, 'primary amenorrhoea' and no public hair