Intro Week Lectures 2

Question 1

When during pregnancy is fetal growth the fastest?

Answer 1

Peaks in 2nd trimester

Question 2

How fast should children grow normally?

Answer 2

Growth velocity 5-7cm/yr

Falls slowly to <5cm/yr pre-pubertally

mid-childhood spurt at 6-8 yrs

Question 3

First sign of puberty in girls v boys?

Answer 3

Breast change in girls/testicular growth in boys (4 mls size)

Question 4

Sign of completion of puberty in girls v boys?

Answer 4

menarche in girls/ 10 mls size testes in boys

Question 5

What tools can you use to assess growth?

Answer 5

Leicester height measure (stadiometer), weighing scales, head circumference
Growth charts
Bone age
Prader orchidometer

Question 6

Short stature with normal growth velocity suggests what?

Answer 6

constitutional delay

Often associated with medical conditions e.g. asthma
Delayed bone age
Later onset of puberty
Often familial, will eventually achieve genetic height potential

Question 7

Causes of short stature with slow velocity?

Answer 7

Chronic disease - e.g. Coeliac
GH deficiency
Skeletal dysplasia
Syndromes e.g Turner, Down, Prader-Willi,
Endocrine e.g hypothyroidism, hypopituitarism, Cushings
Child abuse

Question 8

How may isolated growth hormone deficiency present?

Answer 8

midline defects e.g. cleft lip and cleft palate
undescended testes

Question 9

Questions to ask patient / parents with suspected Turner’s?

Answer 9

Lymphoedema of hands/feet in neonates
Congenital Heart Disease
Recurrent ear infections
Learning difficulties– coordination, numerical
Delayed puberty – primary amenorrhoea

Question 10

How can Turner’s syndrome be managed?

Answer 10

Growth Hormone – supraphysiological doses
Pubertal hormones
Educational help
Hearing aid/grommets
Cardiac monitoring
Osteoporosis prevention

Question 11

What conditions are screened for in newborn screening?

Answer 11

Phenylketonuria (PKU)

Congenital Hypothyroidism

Sickle Cell Disease (SCD)

Cystic fibrosis (CF)

Medium chain Acyl- CoA-dehydrogenase deficiency (MCADD)

Question 12

Causes of tall stature in children?

Answer 12

Familial
Early / precocious puberty
Hyperthyroidism, GH secreting adenomas
Marfan’s, Klinefelters

Question 13

How does Marfan’s syndrome present?

Answer 13

tall stature with arm span to height ratio > 1.05
high-arched palate
pes planus
arachnodactyly
pectus excavatum
lungs: repeated pneumothoraces
eyes: upwards lens dislocation

Question 14

Define hypersensitivity

Answer 14

Objectively reproducible symptoms or signs following exposure to a defined stimulus (e.g. food, drug, pollen) at a dose which is tolerated by normal people

Question 15

Define allergy

Answer 15

A hypersensitivity reaction initiated by specific immunological mechanisms, leading to disease. This can be IgE mediated or non-IgE mediated.

Allergy is not a disease, but a mechanism leading to a disease

Question 16

Define atopy

Answer 16

A personal and/or familial tendency to produce IgE antibodies in response to ordinary exposures to potential allergens, usually proteins.

Strongly associated with asthma, rhinitis and conjunctivitis, eczema and food allergy.

Question 17

Define anaphylaxis

Answer 17

Severe, potentially life-threatening generalised or systemic hypersensitivity reaction which is characterised by being rapid in onset, effecting airway, breathing or circulatory problems, and is usually associated with skin and mucosal changes.

Question 18

How may anaphylaxis present in children?

Answer 18

Signs that are difficult to interpret: behavioural changes, fussing, irritable
hoarseness (after crying), drooling
regurgitation (common after feeds)
drowsiness, somnolence (after feeds)

Signs obvious but non-specific: rapid onset coughing, choking, stridor
sudden, profuse vomiting
rapid onset unresponsiveness

Question 19

How do allergies develop?

Answer 19

Allergic diseases occur when individuals make an abnormal immune response to harmless environmental stimuli, usually proteins

The developing immune system must be ‘sensitised’ to an allergen before an allergic immune response develops.

Sensitisation may be ‘occult’ e.g. sensitisation to egg in from exposure to trace quantities in maternal breast milk.

Question 20

Outline the process of mast cell activation in IgE mediated allergy

Answer 20

antigen binds to specific IgE

IgE releases mediators such as histamine, tryptase and prostaglandins

Question 21

Describe onset and resolution of sxs in IgE mediated versus non-IgE mediated allergy

Answer 21

IgE : comes on within 2 hours, usually resolves within 12 hours

non-IgE: comes on hours or days post ingestion, may continue for days

Question 22

Describe sxs in IgE mediated allergy

Answer 22

Gastrointestinal such as vomiting, pain and diarrhoea

Cutaneous such as urticaria, angiodema, pruritis

Respiratory such as acute rhinoconjunctivitis, wheezing, coughing, stridor

Cardiovascular such as collapse due to hypotension

Question 23

Describe sxs in non-IgE mediated allergy

Answer 23

Often non-specific symptoms.

These can include diarrhoea, vomiting, colic/pain, blood in the stool, gastrooesophageal reflux and food refusal or aversion.

Question 24

Give some examples of IgE-mediated clinical phenotypes

Answer 24

Acute urticaria and angioedema, anaphylaxis, oral allergy syndrome

Question 25

Give some examples of non-IgE-mediated clinical phenotypes

Answer 25

Food protein induced proctocolitis, food protein induced enteropathy, allergic dysmotility

Question 26

How might an IgE mediated reaction appear in the skin?

Answer 26

pruritus erythema acute urticaria acute angioedema

Question 27

How might a non-IgE mediated reaction appear in the skin?

Answer 27

pruritus erythema atopic eczema

Question 28

What questions could you ask a parent to determine if their child has asthma or a viral wheeze?

Answer 28

Does your child have a diagnosis of asthma? Is your child ever wheezy? When do they become wheezy? What triggers it? Do they get symptoms with exercise, stress, with colds? Do they cough at night? Do they respond to salbutamol? How do you give the salbutamol?

Question 29

What questions would you ask the parent of a child with eczema?

Answer 29

When did the eczema start? What happened around the time of weaning? Severity? When did the eczema get worse? How much moisturiser / steroid do you use?

Question 30

What should you ask in the family history component of an allergy history?

Answer 30

Does anyone in the family have: Asthma, Hay fever, Eczema, Food allergies Any pets at home?

Question 31

How can allergies be diagnosed?

Answer 31

specific IgE immunoassay skin prick Gold standard for food allergy is DBPCFC (Double blind placebo controlled food challenge)

Question 32

How can allergies be managed?

Answer 32

allergen avoidance disease specific tx e.g. non-sedating antihistamines for urticaria, emollients/steroids for eczema

Question 33

How would you explain to a patient how to use their EpiPen?

Answer 33

lie down remove blue safety cap - 'blue to the sky, orange to the thigh' hold pen with fist - don't put thumb over the top as if wrong way round will stab finger jab the EpiPen firmly into your outer thigh at a right angle until you hear a click. Hold firmly for 3 seconds, before removing and safely discarding massage the injection site for 10 seconds elevate your legs if the first injection does not work, do a second injection after 5-15 minutes call 999

Question 34

What are the two main brands of adrenaline auto-injector?

Answer 34

EpiPen Jext- good for older children / teenagers as links with an app

Question 35

What online resource can be helpful for education and mx of children with allergy?

Answer 35

itchy sneezy wheezy - educational website

Question 36

What is the acute mx of anaphylaxis?

Answer 36

Assessment: ABCDE First-line treatment IM adrenaline Second- and third-line treatment Removal of the trigger and call for help Posture Oxygen Fluids Other drugs e.g. β2-agonists, glucocorticoids

Question 37

What is involved in the long term risk reduction of anaphylaxis?

Answer 37

Risk assessment Emergency preparedness: Allergen identification, Emergency Action Plan, Adrenaline Auto-injector prescription Allergen avoidance Immunomodulation

Question 38

Dose of adrenaline to give in kids in anaphylaxis?

Answer 38

0-6 years or up to 30kg : 0.15 mg 6-12 years or 30-50 kg : 0.30 mg > 12 years or above 50kg : 0.50 mg

Question 39

What effects risk of anaphylaxis?

Answer 39

Age: infants, adolescents, elderly Concomitant disease: asthma and other respiratory diseases, cardiovascular disease, psychiatric illness Concomitant medication e.g. beta-blockers, ACE inhibitors Cofactors that amplify anaphylaxis: stress, infection exercise Allergen itself - prevalent?

Question 40

What to ask in a history of a food allergy

Answer 40

food and amount eaten, timing of symptoms nature of symptoms (each system) details around event (e.g. cold, exercise) associated allergies (i.e. asthma, eczema) concomitant diseases

Question 41

What is the underlying pathology in inguinal hernias and hydrocoeles in infants?

Answer 41

patent processes vaginalis fully patent = inguinal hernia partially patent = hydrocoele ( enough space for peritoneal fluid to escape but not for bowel to pass through)

Question 42

Parents bring in child to ED with sudden appearance of fluctuant testicular swelling that transilluminates following recent viral illness =

Answer 42

hydrocoele during viral illness production of peritoneal fluid increases so may cause hydrocele to become more obvious

Question 43

What is the gubernaculum?

Answer 43

embryological structure that guides the descent of the testes - becomes the tunica vaginalis (double layer that surrounds the testes)

Question 44

What type of inguinal hernia is more common in kids?

Answer 44

indirect - passes through the deep and then superficial inguinal ring can cause obstruction!!! - look for hernia in young child with bowel obstruction of unknown cause

Question 45

What should be done if a paediatrician assesses that a child’s testicle is not situated within the scrotum after 3 months?

Answer 45

straight to examination under anaesthetic and diagnostic laparoscopy USS is unhelpful as the testicle will be very small and difficult to see

Question 46

Why is it important that testes are surgically returned to the scrotum if undescended ?

Answer 46

reduced risk of testicular cancer allows to self examine for testicular tumour as risk is still higher than general population ideal temperature for hormone production and maintaining fertility reduced risk of trauma

Question 47

What does the blue dot sign on an infant’s testis suggest?

Answer 47

torted hyatid of morgagni (Mullerian remnant)

Question 48

Give some causes of bilious vomiting in neonates

Answer 48

duodenal atresia jejunal atresia malrotation with volvulus meconium ileus necrotising enterocolitis NOT pyloric stenosis - in PS obstruction is above the 2nd segment of the duodenum where bile enters the GI tract so vomitus will not be bilious

Question 49

What is the definition of diabetes?

Answer 49

Fasting plasma glucose > 7.0 mmol/l 2 hour post prandial plasma glucose > 11.0 mmol/l during an OGTT

Question 50

How should you investigate a child who presents to the GP with suspected T1DM?

Answer 50

Children and young people with suspected T1DM should be offered immediate (same day) referral to multidisciplinary paediatric diabetes team - risk of deterioration to DKA

Question 51

Ix in paediatric diabetes?

Answer 51

BMI HbA1c OGTT Fasting Insulin Fasting C peptide Autoantibodies

Question 52

What is DKA?

Answer 52

diabetic ketoacidosis - caused by uncontrolled lipolysis which results in an excess of free fatty acids that are converted to ketone bodies hyperglycaemia (plasma glucose more than 11 mmol/litre) and acidosis (indicated by blood pH below 7.3 or plasma bicarbonate below 15 mmol/litre) and ketonaemia (indicated by blood ketones > 3 mmol/litre) or ketonuria (++ and above on the standard strip marking scale)

Question 53

Suspect DKA even if the blood glucose is normal in a child or young person with known diabetes and any of the following:

Answer 53

nausea or vomiting abdominal pain hyperventilation dehydration reduced level of consciousness

Question 54

When a child or young person with suspected or known DKA arrives at hospital, measure their:

Answer 54

capillary blood glucose capillary blood ketones (beta‑hydroxybutyrate) or urine ketones capillary or venous pH and bicarbonate

Question 55

Give some of the main differences between T1DM and T2DM

Answer 55

Deficiency of insulin v insulin resistance Absent C peptide v detectable C peptide Markers of autoimmunity v none Sudden v gradual onset Ketoacidosis common v rare Not obese v obese

Question 56

How do you calculate fluid requirements in children?

Answer 56

for 24 hours: First 10 kg - 100 ml/kg Second 10 kg - 50 ml/kg Subsequent 10 kg – 20 ml/kg

Question 57

What are the fluid requirements of neonates born at term?

Answer 57

Day 1 – 50 ml/kg/day Day 2 – 75 ml/kg/day Day 3 – 100 ml/kg/day Day 4 – 120 ml/kg/day Day 5 onwards - 150 ml/kg/day

Question 58

Why do preterm babies have a high fluid requirement ?

Answer 58

very fragile skin = large fluid losses require more fluid replacement than term babies and incubators often contain humidified air

Question 59

What fractures should raise suspicion of physical abuse?

Answer 59

Metaphyseal fractures - bucket handle or corner fractures Rib fractures - commonly posterior Skull fracture - usually non parietal and associated with subdural haemorrhage Scapular fractures Sternal fractures

Question 60

Give some differentials for unexplained bruising

Answer 60

NAI Congenital melanocytic naevi Thrombocytopenia Haemophilia Henoch-Schonlein purpura (HSP)

Question 61

What is a skeletal survey used for?

Answer 61

Children under 2 with suspected physical abuse Used to detect occult bone injury Ideally within 72 hours, provides imaging of the whole skeleton

Question 62

Why do babies have a large amount of brown fat?

Answer 62

carries a high water volume to prevent them from becoming dehydrated colostrum is calorie rich but does not contain enough fluids significant WL in babies suggests dehydration