Paeds 3 - Allergy/Neonates/Development etc Flashcards
what is impetigo?
highly infectious skin infection - common on face in infants and young children, esp in eczema etc.
caused by staph aureus - rarely can be caused by strep infection
what are the clinical features of impetigo?
erythematous macules develop into honey-crusted lesions.
how is impetigo treated?
topical antibiotics if only mild e.g. mupirocin
if more severe - fluclozacillin.
nasal carriage can cause reinfection - nasal cream containing chlorhexidine and neomycin can be used to eradicate.
what is infantile seborrhoeic eczema and how is it treated?
cradle cap - scaly, non-itchy rash on scalp, might spread.
treatment - emollients and mild topical steroids.
what is atopy?
predisposition to atopic disorders - eczema, asthma and hay fever.
genetic, confers abnormal immune response to environmental allergens. often a lot of FHx.
what are the clinical features of eczema?
dry, red, itchy rash - usually on extensor surfaces and face in infants and young children, and the flexures (inner elbow, back of knees) in older kids.
how is eczema managed?
need to hydrate skin and minimise inflammation.
- general measures - cotton clothing, non-biologic detergents, no parental smoking, pets etc
- emollients - 2-3 times a day, replace soap with aqueous cream.
- mild topical steroid - 1% hydrocortisone ointment/cream applied to affected areas twice daily - highly effective to get under control, then focus on emollients.
- new class - topical immunomodulators e.g. tacrolimus cream
what is Stevens-Johnson syndrome?
widespread erythema multiforme (target lesions ± macules/papules/bullae), often painful. often a drug reaction or infection (herpes simplex, mycoplasma, EBV).
supportive management, pain relief, may need ICU.
what is allergic rhinitis?
hay-fever! also dust, pet allergies. 20% of children will have it.
IgE mediated inflammation from allergen exposure to nasal mucosa - release of inflammatory mediatory from mast cells.
what are the symptoms of allergic rhinitis?
sneezing, pruritus, nasal discharge (bilateral), itchy red eyes.
how do you manage a patient with allergic rhinitis?
allergen avoidance.
antihistamines e.g. loratidine, cetirizine.
intranasal corticosteroid sprays e.g. fluticasone.
describe the pathology behind anaphylaxis
severe allergic reaction with respiratory difficulty (wheeze/obstruction) and/or cardiovascular symptoms (shock, hypotension, dizziness).
prior exposure to antigen-sensitising mast cells and basophils –> systemic release of inflammatory mediators –> capillary leak, mucosal oedema, smooth muscle contraction.
mediated by IgE.
common allergens - food (peanuts), insect venom (rare), latex, drugs (penicillin, vaccines - rare)
what are the symptoms/signs of anaphylaxis?
resp - cough, stridor, hoarseness, facial swelling, wheeze
cario - faint, dizziness, syncope, pallor, tachycardia
may also see uritcaria, GI signs etc - these aren’t as life threatening.
describe the management of an anaphylactic reaction
IM adrenaline 150/300/500 micrograms depending on age.
follow with IV or oral steroids and antihistamine - helps prevent protracted reaction.
what is urticaria?
transient, itchy, erythematous rash - raised welts (hives).
induced by mast cell degranulation - histamines etc released causing vasodilation and increased capillary permeability.
mostly due to viral infections, might be an allergy thing.
what is angioedema?
swelling of lips and eyes that sometimes accompanies utricaria
if lips are involve - emergency, tongue may get involved and then there’s a risk of respiratory obstruction.
how do you manage urticaria/angioedema?
urticaria - should resolve spontaneously, give antihistamine if itchy, avoid precipitating factors.
angioedema - go to hospital, ABCDE.
what is Henoch-Schonlein purpura?
multisystem vasculitis of small blood vessels.
follow UTRI or exposure to drug or allergen.
think it’s immune-mediated - IgA.
what are the clinical features of HSP?
skin - purpuric rash of legs and buttocks
GI - colicky abdo pain ± gross/occult bleeding
joints - pain and swelling of large joints
kidneys - glomerulonephritis - microscopic haematuria
how is HSP diagnosed and managed?
clinical diagnosis.
Rx - symptomatic/supportive. recover in 4-6 weeks.
what is achondroplasia?
autosomal dominant disorder - short limbs, large head, abnormal neurology.
gene on chromosome 4, fibroblast growth receptor gene (FGFR3).
what is Fragile X syndrome?
X linked disorder typically involving severe learning impairment, more common in males, associated with autism.
physical features - dysmorphic facial appearance (large forehead, long face, large prominent ears), macrocephaly, macro-orchidism.
briefly explain the genetics of Downs syndrome?
trisomy 21 - extra chromosome can come from non-dysjunction (the chromosome 21 pair fails to separate at meiosis, so one gamete has two copies), translocation or mosaicism (occurs during mitosis after formation of zygote, so some cells might be normal, may have milder phenotype).
risk increases with maternal age.
what are the clinical features of Down syndrome?
dysmorphic facial features - round face, epicanthis folds, flat nasal bridge, protruding tongue, small ears, Brushfield spots on iris.
other dysmorphic features - single palmar creases, flat occiput, incurved little fingers, sandal toe gap, small stature.
structural defects - cardiac defects, duodenal atresia.
neurology - hypotonia, developmental delay, mean IQ = 50
how is Down syndrome diagnosed and managed?
nuchal translucency on antenatal US.
chromosome analysis, FISH is faster.
support and education for parents.
what are the genetics in Turner syndrome?
only 1 normal X chromosome.
affects 1 in 2500 live born females.
might have 45, XO
or deletion of short arm of one X chromosome
some have mosaicism.
what are the clinical features of Turner syndrome?
hallmarks - short stature + primary amenorrhoea.
normal intelligence, specific learning difficulties.
dysmorphic features - neck webbing, widely spaced nipples (shield chest), wide carrying angle, short stature.
structural/functional - gonadal dysgenesis, congenital heart disease, renal anomalies
how is Turner’s syndrome identified/diagnosed?
prenatally on USS.
at birth - presence of puffy hands and feet.
in childhood - short stature
in adolescence - primary amenorrhoea.
diagnosis confirmed by peripheral blood karyotype.
how is Turner’s syndrome managed?
GH therapy improves final height.
oestrogen therapy at c.11 years to promote secondary sex characteristics.
towards end of puberty, progestogen added to maintain uterine health
what is Klinefelter’s syndrome?
47, XXY - males born with two X chromosomes.
primary features - infertility, small testicles.
lack of hair/general secondary sex characteristics, very tall and slim, gynaecomastia, learning disability.
what is Edwards syndrome?
trisomy 18 (full, mosaic, or partial).
usually picked up in antenatal screening - nuchal translucency thickness/hypolasia of nasal bone.
risk rises with maternal age.
facial dysmorphisms - Low-set and malformed ears, Micrognathia (small jaw), Prominent occiput, Small facial features, Microcephaly, Cleft lip and palate
other dysmorphic features - overriding fourth/fifth finger., rocker bottom feet, thumb aplasia.
congenital heart defects, GI/kidney abnormalities.
what is Patau’s syndrome?
trisomy 13.
often incompatible with life.
severe brain and CNS abnormalities.
mean life expectancy is 2.5 days.
what is Angelman’s syndrome?
genetic disorder featuring happy demeanour, easily provoked laughter, developmental delay from 6 months. often epileptic.
Chromosome 15
what is Prader Willi syndrome?
result of genetic imprinting.
hypotonia and developmental delay in infancy. obesity, LD and behavioural issues in childhood. narrow face, almond shaped eyes. hypogonadism. cryptorchidism.
chromosome 15
describe the normal gross motor development you’d see in a newborn baby?
limbs flexed, symmetrical posture.
marked head lag on pulling up.
at what age would you expect a baby to raise their head to 45 degrees whilst prone (look up whilst lying on front)?
6-8 weeks
at what age would you expect a baby to sit without support?
6-8 months.
with rounded back at 6 months.
with straight back at 8 months.
at what age would you expect a baby to start crawling?
8-9 months.
at what age would you expect a baby to start furniture cruising?
10 months.
at what age would you expect a baby to start walking (unsteadily)?
12 months.
at what age would you expect a child to start walking steadily?
15 months.
at what age would you expect a child to start running?
2 years.
at what age would you expect a child to start jumping / hopping / be able to do heel to toe?
3 / 4 / 5 years
at what age would you expect a child to be able to follow a moving object/face by turning their head?
6 weeks
at what age would you expect a child to be able to reach out for toys?
4 months
at what age would you expect a child to use a palmar grasp?
4-6 months
at what age would you expect a child to be able to transfer toys from one hand to another?
7 months
at what age would you expect a child to have a mature pincer grip?
10 months
at what age would you expect a child to be able to make marks with a crayon?
16-18 months
at what age would you expect a child to be able to build towers of: 3, 6, 8 blocks, a bridge, a set of steps?
3 blocks - 18 months
6 blocks - 2 years
8 blocks - 2.5 years
bridge - 3 years
steps - 4 years
at what age would you expect a child to be able to draw a: line, circle, cross, square, triangle/6 part person?
line - 2.5 years
circle - 3 years
cross - 3.5 years
square - 4 years
triangle/6 part person - 5 years
at what age would you expect a child to startle to loud noise?
newborn
at what age would you expect a child to vocalise alone/when spoken to, coos, laughs?
3-4 months
at what age would you expect a child to turn to soft sounds out of sight?
7 months
at what age would you expect a child to say dada/mama?
7-10 months
at 7 months - just saying them to make sounds
10 months - saying them to mean the parents
at what age would you expect a child to be able to say 2 or 3 words, other than mama/dada?
12 months
at what age would you expect a child to know 6-10 words, and show you 2 parts of the body?
18 months
at what age would you expect a child to use 2+ words to make simple phrases e.g. give me teddy, want more
18 months
at what age would you expect a child to talk constantly in 3-4 word sentences?
2.5-3 years
at what age would you expect a child to smile?
6 weeks