Paeds MSK Flashcards
What is Osteogenesis imperfecta
Genetic condition (auto dom) that results in brittle bones that are prone to fractures
affect formation of collage
what type of collagen is affected in MC form of Osteogenesis imperfecta
type 1 collage
how does Osteogenesis imperfecta present
Hypermobility
Blue / grey sclera (the “whites” of the eyes)
Triangular face
Short stature
Deafness from early adulthood
Dental problems, particularly with formation of teeth
Bone deformities, such as bowed legs and scoliosis
Joint and bone pain
how is Osteogenesis imperfecta managed medically
Bisphosphates to increase bone density
Vitamin D supplementation to prevent deficiency
What do blood results for Osteogenesis imperfecta show
adjusted calcium, phosphate, parathyroid hormone and ALP results are usually normal in osteogenesis imperfecta
How is Osteogenesis imperfecta managed by MDT team
Physiotherapy and occupational therapy to maximise strength and function
Paediatricians for medial treatment and follow up
Orthopaedic surgeons to manage fractures
Specialist nurses for advice and support
Social workers for social and financial support
what is collagen
protein that is essential is maintaining the structure and function of bone, as well as skin, tendons and other connective tissues.
What is rickets
defective bone mineralisation causing “soft” and deformed bones
what causes rickets
deficiency in vitamin D or calcium
what are nutritional sources of Vit D
eggs, oily fish or fortified cereals
what are nutritional sources of calcium
diary
leafy greens
nuts
what is hereditary hypophosphataemic rickets
commonly x linked
genetic defects that result in low phosphate in the blood
what is Vitamin D
hormone (not technically a vitamin) created from cholesterol by the skin in response to UV radiation
what can lead to vitamin D deficiency.
- Reduced sun exposure without vitamin D supplementation
- Malabsorption disorders - IBD
- CKD - kidney metabolize vit D to active form
Vit D is essential for absorption of what in intestine and kidneys
calcium and phosphate
what does low calcium and phosphate lead to
result in defective bone mineralisation
how does low calcium affect the parathyroid glad
causes a secondary hyperparathyroidism by secreting parathyroid hormone
what does parathyroid hormone stimulates
increased reabsorption of calcium from the bones
how does vit D def present
what are bone deformities associated with Rickets
- Bowing of the legs, where the legs curve outwards
- Knock knees, where the legs curve inwards
- Rachitic rosary, where the ends of the ribs expand at the costochondral junctions, causing lumps along the chest
- Craniotabes, which is a soft skull, with delayed closure of the sutures and frontal bossing
- Delayed teeth with under-development of the enamel
what investigation establishes a diagnosis of vit D def
Serum 25-hydroxyvitamin D less than 25 nmol/L
How does rickets present on XRay
osteopenia (more radiolucent bones).
what investigations can be ordered for rickets and what would they show
Serum calcium may be low
Serum phosphate may be low
Serum alkaline phosphatase may be high
Parathyroid hormone may be high
Serum 25-hydroxyvitamin D
what addition investigations can be ordered to establish pathology in rickets
Full blood count and ferritin, for iron deficiency anaemia
Inflammatory markers such as ESR and CRP, for inflammatory conditions
Kidney function tests, for kidney disease
Liver function tests, for liver pathology
Thyroid function tests, for hypothyroidism
Malabsorption screen such as anti-TTG antibodies, for coeliac disease
Autoimmune and rheumatoid tests, for inflammatory autoimmune conditions
how is rickets managed
1st line = ergocalciferol -> vit D replacement
calcium lactate or calcium carbonat -> hypocalcaemia
ToUC
diet advice
sunlight exposure
what is the does of ergocalciferol for children 6months to 12 years
6,000 IU per day for 8 – 12 weeks.
who si at higher risk of vit D def breastfed or formula babies
Breastfed babies
formula feed is fortified with vitamin D.
what is Transient synovitis
caused by temporary (transient) irritation and inflammation in the synovial membrane of the joint (synovitis).
what age is Transient synovitis common in
3 – 10 years
what is often associated with Transient synovitis
recent viral upper respiratory tract infection.
how does Transient synovitis present
within a few weeks of a viral illness
Limp
Refusal to weight bear
Groin or hip pain
Mild low grade temperature
otherwise well
how is Transient synovitis managed
symptomatic
what is Transient synovitis prognosis
significant improvement in symptoms after 24 – 48 hours.
Symptoms fully resolve within 1 – 2 weeks
when can a child with Transient synovitis be managed in primary care
aged 3 – 9 years with symptoms suggestive of transient synovitis may be managed in primary care if the limp is present for less than 48 hours and they are otherwise well,
if children with Transient synovitis worsen what is management
followed up at 48 hours and 1 week to ensure symptoms are improving and then fully resolve
what is septic arthritis
infection inside a joint
what age is septic arthritis mc
under 4 years
how does septic arthritis present
Hot, red, swollen and painful joint
Refusing to weight bear
Stiffness and reduced range of motion
Systemic symptoms such as fever, lethargy and sepsis
what is kocher rules for septic arthritis
- Fever higher than 38.6.
- ESR > 40 millimeters per hour (mm/hour)
- WCC > 12,000 cells/mm3
what is MC causative organisms of septic arthritis
Staphylococcus aureus
what are DDx for septic arthritis
Transient sinovitis
Perthes disease
Slipped upper femoral epiphysis
Juvenile idiopathic arthritis
How is septic arthritis investigated
joint aspiration for synovial fluid analysis, gram staining and culture.
what are other possible causative organisms of septic arthritis
Neisseria gonorrhoea (gonococcus) in sexually active teenagers
Group A streptococcus (Streptococcus pyogenes)
Haemophilus influenza
Escherichia coli (E. coli)
how is septic arthritis managed
Empirical IV antibiotics
1st line = Flucloxacillin (often first-line)
Clindamycin (penicillin allergy)
Vancomycin (if MRSA is suspected)
Abx for 3-6 weeks
surgical drainage and washout of joint
what is Osteomyelitis
inflammation in a bone and bone marrow, usually caused by bacterial infection.
what is Haematogenous osteomyelitis
when a pathogen is carried through the blood and seeded in the bone
MC mode of infection
where does Osteomyelitis typically occur
metaphysis of the long bones
what is MC causative organism of Osteomyelitis
staphylococcus aureus.
what is chronic Osteomyelitis
deep seated, slow growing infection with slowly developing symptoms.
what is acute Osteomyelitis
presents more quickly with an acutely unwell child
what are RF for Osteomyelitis
Open bone fracture
Orthopaedic surgery
Immunocompromised
Sickle cell anaemia
HIV
Tuberculosis
how does Osteomyelitis present
Refusing to use the limb or weight bear
Pain
Swelling
Tenderness
Fever
What is Barlow test
test for dislocatable hip
adducting the hip (bringing the thigh towards the midline) whilst applying light pressure on the knee with your thumb, directing the force posteriorly
what is Ortolani test
Flex the hips and knees of a supine infant to 90°.
place anterior pressure on the greater trochanters, gently and smoothly abduct the infant’s legs using your thumbs.
