Intro Week Lectures 2 Flashcards
When during pregnancy is fetal growth the fastest?
Peaks in 2nd trimester
How fast should children grow normally?
Growth velocity 5-7cm/yr
Falls slowly to <5cm/yr pre-pubertally
mid-childhood spurt at 6-8 yrs
First sign of puberty in girls v boys?
Breast change in girls/testicular growth in boys (4 mls size)
Sign of completion of puberty in girls v boys?
menarche in girls/ 10 mls size testes in boys
What tools can you use to assess growth?
Leicester height measure (stadiometer), weighing scales, head circumference
Growth charts
Bone age
Prader orchidometer
Short stature with normal growth velocity suggests what?
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
Causes of short stature with slow velocity?
Chronic disease - e.g. Coeliac
GH deficiency
Skeletal dysplasia
Syndromes e.g Turner, Down, Prader-Willi,
Endocrine e.g hypothyroidism, hypopituitarism, Cushings
Child abuse
How may isolated growth hormone deficiency present?
midline defects e.g. cleft lip and cleft palate
undescended testes
Questions to ask patient / parents with suspected Turner’s?
Lymphoedema of hands/feet in neonates
Congenital Heart Disease
Recurrent ear infections
Learning difficulties– coordination, numerical
Delayed puberty – primary amenorrhoea
How can Turner’s syndrome be managed?
Growth Hormone – supraphysiological doses
Pubertal hormones
Educational help
Hearing aid/grommets
Cardiac monitoring
Osteoporosis prevention
What conditions are screened for in newborn screening?
Phenylketonuria (PKU)
Congenital Hypothyroidism
Sickle Cell Disease (SCD)
Cystic fibrosis (CF)
Medium chain Acyl- CoA-dehydrogenase deficiency (MCADD)
Causes of tall stature in children?
Familial
Early / precocious puberty
Hyperthyroidism, GH secreting adenomas
Marfan’s, Klinefelters
How does Marfan’s syndrome present?
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
Define hypersensitivity
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
Define allergy
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
Define atopy
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.
Define anaphylaxis
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.
How may anaphylaxis present in children?
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
How do allergies develop?
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.
Outline the process of mast cell activation in IgE mediated allergy
antigen binds to specific IgE
IgE releases mediators such as histamine, tryptase and prostaglandins
Describe onset and resolution of sxs in IgE mediated versus non-IgE mediated allergy
IgE : comes on within 2 hours, usually resolves within 12 hours
non-IgE: comes on hours or days post ingestion, may continue for days
Describe sxs in IgE mediated allergy
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
Describe sxs in non-IgE mediated allergy
Often non-specific symptoms.
These can include diarrhoea, vomiting, colic/pain, blood in the stool, gastrooesophageal reflux and food refusal or aversion.
Give some examples of IgE-mediated clinical phenotypes
Acute urticaria and angioedema, anaphylaxis, oral allergy syndrome
Give some examples of non-IgE-mediated clinical phenotypes
Food protein induced proctocolitis, food protein induced enteropathy, allergic dysmotility
How might an IgE mediated reaction appear in the skin?
pruritus
erythema
acute urticaria
acute angioedema
How might a non-IgE mediated reaction appear in the skin?
pruritus
erythema
atopic eczema
What questions could you ask a parent to determine if their child has asthma or a viral wheeze?
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?
What questions would you ask the parent of a child with eczema?
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?
What should you ask in the family history component of an allergy history?
Does anyone in the family have: Asthma, Hay fever, Eczema, Food allergies
Any pets at home?
How can allergies be diagnosed?
specific IgE immunoassay
skin prick
Gold standard for food allergy is DBPCFC (Double blind placebo controlled food challenge)
How can allergies be managed?
allergen avoidance
disease specific tx e.g. non-sedating antihistamines for urticaria, emollients/steroids for eczema
How would you explain to a patient how to use their EpiPen?
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
What are the two main brands of adrenaline auto-injector?
EpiPen
Jext- good for older children / teenagers as links with an app
What online resource can be helpful for education and mx of children with allergy?
itchy sneezy wheezy - educational website
What is the acute mx of anaphylaxis?
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
What is involved in the long term risk reduction of anaphylaxis?
Risk assessment
Emergency preparedness:
Allergen identification, Emergency Action Plan, Adrenaline Auto-injector prescription
Allergen avoidance
Immunomodulation
Dose of adrenaline to give in kids in anaphylaxis?
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
What effects risk of anaphylaxis?
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?
What to ask in a history of a food allergy
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
What is the underlying pathology in inguinal hernias and hydrocoeles in infants?
patent processes vaginalis
fully patent = inguinal hernia
partially patent = hydrocoele ( enough space for peritoneal fluid to escape but not for bowel to pass through)
Parents bring in child to ED with sudden appearance of fluctuant testicular swelling that transilluminates following recent viral illness =
hydrocoele
during viral illness production of peritoneal fluid increases so may cause hydrocele to become more obvious
What is the gubernaculum?
embryological structure that guides the descent of the testes - becomes the tunica vaginalis (double layer that surrounds the testes)
What type of inguinal hernia is more common in kids?
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
What should be done if a paediatrician assesses that a child’s testicle is not situated within the scrotum after 3 months?
straight to examination under anaesthetic and diagnostic laparoscopy
USS is unhelpful as the testicle will be very small and difficult to see
Why is it important that testes are surgically returned to the scrotum if undescended ?
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
What does the blue dot sign on an infant’s testis suggest?
torted hyatid of morgagni (Mullerian remnant)
Give some causes of bilious vomiting in neonates
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
What is the definition of diabetes?
Fasting plasma glucose > 7.0 mmol/l
2 hour post prandial plasma glucose > 11.0 mmol/l during an OGTT
How should you investigate a child who presents to the GP with suspected T1DM?
Children and young people with suspected T1DM
should be offered immediate (same day) referral to multidisciplinary paediatric diabetes team - risk of deterioration to DKA
Ix in paediatric diabetes?
BMI
HbA1c
OGTT
Fasting Insulin
Fasting C peptide
Autoantibodies
What is DKA?
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)
Suspect DKA even if the blood glucose is normal in a child or young person with known diabetes and any of the following:
nausea or vomiting
abdominal pain
hyperventilation
dehydration
reduced level of consciousness
When a child or young person with suspected or known DKA arrives at hospital, measure their:
capillary blood glucose
capillary blood ketones (beta‑hydroxybutyrate) or urine ketones
capillary or venous pH and bicarbonate
Give some of the main differences between T1DM and T2DM
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
How do you calculate fluid requirements in children?
for 24 hours:
First 10 kg - 100 ml/kg
Second 10 kg - 50 ml/kg
Subsequent 10 kg – 20 ml/kg
What are the fluid requirements of neonates born at term?
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
Why do preterm babies have a high fluid requirement ?
very fragile skin = large fluid losses
require more fluid replacement than term babies and incubators often contain humidified air
What fractures should raise suspicion of physical abuse?
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
Give some differentials for unexplained bruising
NAI
Congenital melanocytic naevi
Thrombocytopenia
Haemophilia
Henoch-Schonlein purpura (HSP)
What is a skeletal survey used for?
Children under 2 with suspected physical abuse
Used to detect occult bone injury
Ideally within 72 hours, provides imaging of the whole skeleton
Why do babies have a large amount of brown fat?
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
