Paeds MSK Flashcards
1
Q
What is Osteogenesis imperfecta
A
Genetic condition (auto dom) that results in brittle bones that are prone to fractures
affect formation of collage
2
Q
what type of collagen is affected in MC form of Osteogenesis imperfecta
A
type 1 collage
3
Q
how does Osteogenesis imperfecta present
A
- presents in childhood
- fractures following minor trauma
- blue sclera
- deafness secondary to otosclerosis
- dental imperfections are common
4
Q
how is Osteogenesis imperfecta managed medically
A
Bisphosphates to increase bone density
Vitamin D supplementation to prevent deficiency
5
Q
What do blood results for Osteogenesis imperfecta show
A
adjusted calcium, phosphate, parathyroid hormone and ALP results are usually normal in osteogenesis imperfecta
6
Q
How is Osteogenesis imperfecta managed by MDT team
A
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
7
Q
what is collagen
A
protein that is essential is maintaining the structure and function of bone, as well as skin, tendons and other connective tissues.
8
Q
What is rickets
A
defective bone mineralisation causing “soft” and deformed bones
9
Q
what causes rickets
A
deficiency in vitamin D or calcium
10
Q
what are nutritional sources of Vit D
A
eggs, oily fish or fortified cereals
11
Q
what are nutritional sources of calcium
A
diary
leafy greens
nuts
12
Q
what is hereditary hypophosphataemic rickets
A
commonly x linked
genetic defects that result in low phosphate in the blood
13
Q
what is Vitamin D
A
hormone (not technically a vitamin) created from cholesterol by the skin in response to UV radiation
14
Q
what can lead to vitamin D deficiency.
A
- Reduced sun exposure without vitamin D supplementation
- Malabsorption disorders - IBD
- CKD - kidney metabolize vit D to active form
15
Q
Vit D is essential for absorption of what in intestine and kidneys
A
calcium and phosphate
16
Q
what does low calcium and phosphate lead to
A
result in defective bone mineralisation
17
Q
how does low calcium affect the parathyroid glad
A
causes a secondary hyperparathyroidism by secreting parathyroid hormone
18
Q
what does parathyroid hormone stimulates
A
increased reabsorption of calcium from the bones
19
Q
how does vit D def present
A
Rickets, osteomalacia
20
Q
what are bone deformities associated with Rickets
A
- 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
- kyphoscoliosis
- Harrison’s sulcus
21
Q
what investigation establishes a diagnosis of vit D def
A
Serum 25-hydroxyvitamin D less than 25 nmol/L
22
Q
How does rickets present on XRay
A
osteopenia (more radiolucent bones).
23
Q
what investigations can be ordered for rickets and what would they show
A
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
24
Q
what addition investigations can be ordered to establish pathology in rickets
A
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
25
how is rickets managed
1st line = ergocalciferol -> vit D replacement
calcium lactate or calcium carbonat -> hypocalcaemia
ToUC
diet advice
sunlight exposure
26
what is the dose of ergocalciferol for children 6months to 12 years
6,000 IU per day for 8 – 12 weeks.
27
who si at higher risk of vit D def breastfed or formula babies
Breastfed babies
formula feed is fortified with vitamin D.
28
what is Transient synovitis
caused by temporary (transient) irritation and inflammation in the synovial membrane of the joint (synovitis).
29
what age is Transient synovitis common in
3 – 8 years
30
what is often associated with Transient synovitis
recent viral upper respiratory tract infection.
31
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
32
how is Transient synovitis managed
symptomatic
33
what is Transient synovitis prognosis
significant improvement in symptoms after 24 – 48 hours.
Symptoms fully resolve within 1 – 2 weeks
34
when can a child with suggestive of transient synovitis be managed in primary care
if the limp is present for less than 48 hours and they are otherwise well
35
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
if not --> urgent assessment in secondary care
36
what is septic arthritis
infection inside a joint
37
what age is septic arthritis mc
under 4 years
38
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
39
what is kocher rules for septic arthritis
- Fever higher than 38.6.
- ESR > 40 millimeters per hour (mm/hour)
- WCC > 12,000 cells/mm3
40
what is MC causative organisms of septic arthritis
Staphylococcus aureus
41
what are DDx for septic arthritis
Transient sinovitis
Perthes disease
Slipped upper femoral epiphysis
Juvenile idiopathic arthritis
42
How is septic arthritis investigated
* joint aspiration: for culture.
* raised WBC
* raised inflammatory markers
* blood cultures
43
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)
44
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
45
what is Osteomyelitis
inflammation/infection of a bone and bone marrow, usually caused by bacterial infection.
46
what is Haematogenous osteomyelitis
when a pathogen is carried through the blood and seeded in the bone
MC most common form in children
47
where does Osteomyelitis typically occur
metaphysis of the long bones
48
what is MC causative organism of Osteomyelitis
staphylococcus aureus.
except in patients with sickle-cell anaemia where Salmonella species predominate
49
what is chronic Osteomyelitis
deep seated, slow growing infection with slowly developing symptoms.
50
what is acute Osteomyelitis
presents more quickly with an acutely unwell child
51
what are RF for Osteomyelitis
Open bone fracture
Orthopaedic surgery
Immunocompromised
Sickle cell anaemia
HIV
Tuberculosis
52
how does Osteomyelitis present
Refusing to use the limb or weight bear
Pain
Swelling
Tenderness
Fever
53
how is Osteomyelitis investigated
1st = XRay
GS = MRI
Blood test = raised CRP and ESR and WBC
Blood culture
BM aspiration and Bone Biopsy with histology and culture
54
how is Osteomyelitis managed
Surgical debridement of the infected bone and tissues
Antibiotic therapy = 6 weeks of *flucloxacillin*, possibly with rifampicin or fusidic acid added for the first 2 weeks
clindamycin if allergic
55
How does Osteomyelitis present on XRay
Can be normal
- Periosteal reaction (changes to the surface of the bone)
- Localised osteopenia (thinning of the bone)
- Destruction of areas of the bone
56
what is Perthes Disease
disruption of blood flow to the femoral head, causing avascular necrosis of the bone
57
where does Perthes Disease affect
the epiphysis of the femur, which is the bone distal to the growth plate (physis)
58
what age does perthes disease mc occur in
4 – 8 years
5x more common in boys
59
what are possible causes of perthes disease
genetics vascular anomalies
60
what are 4 stages of perthes disease
Catterall staging
* Stage 1 - Clinical and histological features only
* Stage 2 - Sclerosis with or without cystic changes
* Stage 3 Loss of structural integrity of the femoral head
* Stage 4 Loss of acetabular integrity
61
what is the main complication of perthes disease
* osteoarthritis from deformed femoral head
* premature fusion of the growth plates
62
how does perthes disease present
slow onset
Pain in the hip or groin - 90% unilateral
Limp
Restricted hip movements
There may be referred pain to the knee
no history of trauma, if trauma think SUFE
63
What is 1st line investigation for Perthes
XRay - can be normal
64
what are other investigations for perthes disease
Technetium bone scan
MRI Scan
Blood test - usually normal
65
what can be seen on MRI for perthes
avascular necrosis
joint effusion
marrow oedema
66
what can be seen on XRay for perthes
* early changes include widening of joint space
* later changes include decreased femoral head size/flattening
67
how is perthes managed
initial = conservative to maintain healthy position and alignment of joint, reduce damage and deformity
Bed rest
Traction
Crutches
Analgesia
Physiotherapy is used to retain the range of movement
Regular xrays are used to assess healing.
68
what is the aim of sugery in perthes
aim is to improve the alignment and function of the femoral head and hip.
generally >6yrs
69
what is Slipped upper femoral epiphysis
Displacement of the femoral head epiphysis postero-inferiorly
70
what is mc age for SUFE
typically age group is 10-15 years
More common in obese children and boys
71
what is RF for SUFE
obesity
72
what are symptoms of SUFE
Hip, groin, thigh or knee pain
loss of internal rotation of the leg in flexion
Painful limp
bilateral 20% of cases
73
what is the typical SUFE patient
adolescent, obese male
undergoing a growth spurt.
may be a history of minor trauma
74
How does SUFE present on examination
limited movement of the hip
* prefer to keep the hip in external rotation.
* restricted internal rotation.
75
What is the 1st line investigation for SUFE
XRay
AP and lateral (typically frog-leg) views are diagnostic
76
what are other investigations that can used for SUFE diagnosis
Blood tests = normal --> used to exclude other causes of joint pain
Technetium bone scan
MRI scan
CT scan
77
How is SUFE managed
Surgery
internal fixation: typically a single cannulated screw placed in the centre of the epiphysis
78
What is Osgood-Schlatters Disease
inflammation of the patellar ligament at the tibial tuberosity
common cause of anterior knee pain
78
what are complications of SUFE
osteoarthritis
avascular necrosis of the femoral head
chondrolysis
leg length discrepancy
79
when does Osgood-Schlatters Disease typically occur
10 – 15 years
80
what is the pathophysiology of Osgood-Schlatters Disease
microtrauma causing inflammation and subsequent avulsion fracture of the secondary ossification centre
81
how does Osgood-Schlatters Disease present
Visible or palpable hard and tender lump at the tibial tuberosity
Pain in the anterior aspect of the knee
The pain is exacerbated by physical activity, kneeling and on extension of the knee
82
how is osgood-schlatter disease investiagted
mostly clinical
Xray = elevation or fragmentation of the tibial tubercle
83
how is Osgood-Schlatters Disease managed
Reduction in physical activity
Ice
NSAIDS (ibuprofen) for symptomatic relief
stretching and physiotherapy can be used to strengthen the joint
84
what is a complication of Osgood-Schlatters Disease
full avulsion fracture, where the tibial tuberosity is separated from the rest of the tibia
85
What is Developmental Dysplasia of the Hip
a structural abnormality in the hips caused by abnormal development of the fetal bones during pregnancy
leads to instability in the hips and a tendency or potential for subluxation or dislocation.
86
What can untreated Developmental Dysplasia of the Hip lead to in adulthood
* weakness
* recurrent subluxation or dislocation
* an abnormal gait
* early degenerative changes.
87
when is DDH usually picked up
newborn and infant physical examination (NIPE)
again at the six-week baby check
88
What are RF for DHH
* First degree family history
* Breech presentation from 36 weeks onwards
* Breech presentation at birth if 28 weeks onwards
* Multiple pregnancy
* female sex: 6 times greater risk
* firstborn children
* oligohydramnios
* birth weight > 5 kg
89
What is looked for in DDH newborn and infant physical examination (NIPE)
symmetry in the hips, leg length, skin folds and hip movements
90
what findings may suggest DDH
* Different leg lengths
* Restriction in abduction (bilateral or one side)
* Difference in the knee level when the hips are flexed
* Clunking of the hips on special tests
91
what two special tests are used to check for DDH
Ortolani test
Barlow test
92
What is Barlow test
test for dislocatable hip
adduct the hip and bring the thigh towards the midline then apply posterior pressure on the knee
93
what is Ortolani test
Flex the knees to 90°.
then abduct the legs by pushing the thighs outwards
should pop hip back in
94
what is positive sign in Ortolani
distinctive ‘clunk’ which can be heard and felt as the femoral head relocates anteriorly into the acetabulum.
95
what is investigation of choice in suspected DDH
ultrasound if <4.5 months
if > 4.5 months then x-ray
96
How is DDH managed <6 months
<6 months = Pavlik harness
keeps the baby’s hips flexed and abducted.
97
How is DDH managed >6 months
Surgery
After surgery a hip spica cast to immobilises the hip
98
what is Juvenile idiopathic arthritis
condition affecting children and adolescents where autoimmune inflammation occurs in the joints
99
when is JIA diagnosed
arthritis without any other cause, lasting more than 6 weeks in a patient under the age of 16.
100
what are key features of JIA
joint pain and swelling:
usually medium sized joints e.g. knees, ankles, elbows
limp
101
what are subtypes of Juvenile idiopathic arthritis
Systemic JIA
Polyarticular JIA
Oligoarticular JIA
Enthesitis related arthritis
Juvenile psoriatic arthritis
102
what is Systemic JIA
systemic illness
idiopathic inflammatory
103
what are features of Systemic JIA
* pyrexia
* salmon-pink rash
* lymphadenopathy
* arthritis
* uveitis
* anorexia and weight loss
104
What should blood show for systemic JIA
Antinuclear antibodies and rheumatoid factors are typically negative
raised inflammatory markers, with raised CRP, ESR, platelets and serum ferritin.
105
how does macrophage activation syndrome (MAS) present
acutely unwell child with disseminated intravascular coagulation (DIC), anaemia, thrombocytopenia, bleeding and a non-blanching rash.
106
what is a key complication of systemic JIA
macrophage activation syndrome (MAS)
107
what is a key investigation finding of macrophage activation syndrome (MAS)
low ESR
108
what are infective DDx for fevers for more than 5 days
Kawasaki disease, Still’s disease, rheumatic fever and leukaemia.
109
what is Polyarticular JIA
idiopathic inflammatory arthritis in 5 joints or more
tends to be symmetrical
small and large joints
110
how are Polyarticular JIA and systemic JIa differentiated
Polyarticular = Systemic symptoms are mild
111
what would bloods for rheumatoid factor show for Polyarticular JIA
Most children are negative for rheumatoid factor and are described as “seronegative”.
seropositive - older children and disease more similar to adults
112
what is Oligoarticular JIA
aka Pauciarticular
60% of cases of JIA
It involves 4 joints or less
MC knee or ankle
not rlly associated with systemic symptoms
MC girls < 6yr
113
what is a classic associated feature of Oligoarticular JIA
anterior uveitis
114
how does Oligoarticular JIA present on bloods
- Inflammatory makers will be normal or mildly elevated.
- Antinuclear antibodies are often positive
- Rheumatoid factor is usually negative.
115
what is Enthesitis-Related Arthritis
male >6yrs
paediatric version of the seronegative spondyloarthropathy
Patients have inflammatory arthritis in the joints as well as enthesitis.
116
what are seronegative spondyloarthropathy conditions
* ankylosing spondylitis
* psoriatic arthritis
* reactive arthriti
* inflammatory bowel disease-related arthritis.
117
what is enthesis
point at which the tendon of a muscle inserts into a bone
118
what can cause Enthesitis
can be caused by traumatic stress, such as through repetitive strain during sporting activities, or can be caused by an autoimmune inflammatory process.
119
how can Enthesitis be investigated
MRI
120
what gene is associated with enthesitis-related arthritis
HLA B27 gene
human leukocyte antige
121
what associated conditions should a patient with enthesitis-related arthritis be checked for
psoriasis (psoriatic plaques and nail pitting)
inflammatory bowel disease (intermitted diarrhoea and rectal bleeding).
122
how does enthesitis-related arthritis present on examination
tender to localised palpation of the entheses.
123
where are entheses located in body
Interphalangeal joints in the hand
Wrist
Over the greater trochanter on the lateral aspect of the hip
Quadriceps insertion at the anterior superior iliac spine
Quadriceps and patella tendon insertion around the patella
Base of achilles, at the calcaneus
Metatarsal heads on the base of the foot
124
what is Juvenile Psoriatic Arthritis
seronegative inflammatory arthritis associated with psoriasis
symmetrical polyarthritis affecting the small joints
or an asymmetrical arthritis affecting the large joints in the lower limb
125
what are signs or symptoms of Juvenile Psoriatic Arthritis
* Plaques of psoriasis on the skin
* Pitting of the nails
* Onycholysis, separation of the nail from the nail bed
* Dactylitis, inflammation of the full finger
* Enthesitis, inflammation of the entheses, which are the points of insertion of tendons into bone
126
how is JIA managed
- NSAIDs, such as ibuprofen
- Steroids, either oral, intramuscular or intra-artricular in oligoarthritis
- Disease modifying anti-rheumatic drugs (DMARDs)
- Biologic therapy
127
name Disease modifying anti-rheumatic drugs
methotrexate, sulfasalazine and leflunomide
128
name Biologic therapy such as tumour necrosis factor inhibitors
etanercept, infliximab and adalimumab
129
what is Torticollis
a painful neck.
130
what causes Torticollis
minor local musculoskeletal irritation causing pain and spasms in neck muscles.
131
what meniscus is affected in Discoid meniscus
lateral
132
how does Discoid meniscus present
vague pain in the knee
“click” on the lateral side of the knee
133
What are features of Growing pains
never present at the start of the day after the child has woken
no limp
no limitation of physical activity
systemically well
normal physical examination
motor milestones normal
symptoms are often intermittent and worse after a day of vigorous activity
