Paediatric - MSK Flashcards
what is osteoporosis in adults?
a disease characterised by low bone mass and micro architectural deterioration of bone tissue, leading to enhanced bone fragility and a consequent increase in fracture risk
osteoporosis definition in children
1 or more vertebral crush fractures (unless in high speed traffic accident or falling from a height of >3 metres)
or
reduced size adjusted bone density
what is the most common form of bone fragility in children?
osteogenesis imperfecta
symptoms/signs of osteogenesis imperfect
bone fragility, fracture and deformity (constantly breaking and fixing - will not always fix straight/bowing)
:could present as little as 2 weeks old with broken leg with no trauma history
strong positive family history of fractures following trivial trauma
bone pain (high turnover)
impaired mobility
poor growth - the more affected the shorter you are (adults (like grandparents) may be shrinking)
deafness, hernias, valvular prolapse
wormian bones - of the skull on x ray in type 3
sillence classification of Osteogenesis imperfecta
type 1 - mild
type 2 - lethal
type 3 - severe
type 4 - everything else (Moderate)
how are children with osteogenesis imperfecta managed?
MDT
physician - bone targeted drugs - complications, pain, associated medical problems
surgeon - long bones, spine (rods in to straighten) skull base, hearing, teeth
therapists, nurses:
muscle strength, mobility (aids), social and education, home adaptation, school adaptation
what drugs are given in OI?
and what do they do?
bisphosphonate therapy
increased bone mass
also
reduction in fracture frequency increased vertebral height suppressed bone markets reduced pain increased overall mobility no adverse effect on growth
causes of rickets
lack of sunlight
poor nutrition
what does vitamin D do?
increase calcium absorption from the gut
If calcium is low it:
Reduces calcium loss from kidneys
increases calcium release from bone
roles in immune function/tolerance
maternal vitamin D influences bone size and mass in childhood
what level of vitamin D is classed as deficient in the UK?
25
what can maternal vitamin D deficiency do?
low vit D stores in the newborn
if they are exclusively breastfed can lead to (especially in prolonged unsupplemented breast feeding)
severe and life threatening
- cardiomyopathy
- hypocalcaemia
- convulsions
severe
- clinically apparent rickets- metaphysical swelling
- bony deformity
moderate
- radiological or biochem appearances
what are the presentations of symptomatic vit D deficiency
bowed legs
rickets presentations
rickets is due to under mineralised bones
clinically: metaphyseal swellings bowing deformities slowing of linear growth motor delay hypotonia fractures resp distress if extreme - multiple rib fractures leading to flail chest
radiologically - cupping, splaying and flaying of the metaphyses
pathologically: failure to mineralise new bone
what is the main biochemical result in rickets?
low fasting phosphate
also
raised serum alkaline phosphatase
if vit D deficient: raised PTH, low 25 OH-D
how to treat vitamin D/hypocalcaemia?
replace!
oral vit D and calcium
unless they are fitting from hypocalcaemia you give it via IV - BUT must do this slowly
but if you worry about compliance by the family not giving them the vit D supplement
- give stoss therapy - massive amount of it D all in one go, which won’t lead to adverse calcium reaction
if concerned about NAI in a child presenting with a fracture, what other things might you look for on examination?
Bruises- particular site of bruises for NAI eg. middle of cheek (where soft)
mouth- frenulum burst eg. if punched face,
bruising middle of back, face,
eye injury- subconjunctival haemorrhages eg. from shaking.
Patterned bruising eg. grab marks, belt mark, bruising on genitalia.
what tests might you do if you suspected NAI?
tests:
• Skeletal survey= do arms and legs and everywhere to see if any fractures if think NAI
• CT head if <2 and think its NAI- subdurals
how is osteogenesis imperfecta normally inherited?
autosomal dominant
what is the defect in osteogenesis imperfecta?
defects in t1 collagen gene - makes bone more brittle
what is a classic presentation of systemic onset JIA in children?
- Fever, high swinging, early evening temp for >2 week
- Rash – pink, maculopapular rash associated with the fever
- MSK pain
- Generalised lymphadenopathy
- Hepatosplenomegaly
- Normally ESR/CRP raised, raised neutrophils and platelets?
what drugs can be used to manage JIA?
- NSAIDS/paracetamol acutely
- Later:
- Corticosteroids and immunosuppressants (DMARDS) (methotrexate) often used for systemic disease
- Monoclonal abx may be used – biologics
what professionals would be involved in JIA cases?
Paediatrician, Physio, paed rhuematology, nurses, OPTHALMOLOGIST BECAUSE OF UVEITIS, and the GP as they might prescribe the treatment long term
school, maybe social service if difficulty in getting to appointments,
What is rickets?
a disease of children caused by vitamin D deficiency, characterized by imperfect calcification, softening, and distortion of the bones typically resulting in bow legs.
