Paediatrics Flashcards

1
Q

What is osteogensis imperfecta? What are bones like with this condition?

A

defect in maturation and organization of T1 collagen

Bones are thin with thin cortices and osteopenic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

osteogensis imperfecta is an autosomal - condtion.

A

dominant

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

osteogensis imperfecta is characterised by which 3 signs?

A

Children with osteogensis imperfecta shop at BHS:
Blue sclera
Hearing loss
Short stature

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Management of OI?

A

No cure
COnservative: splintage, traction or surgical stabilisation
Maintain health
Vitamin D supplements
PAMIDRONATE - type of biophosphate to prevent loss bone mass

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What is skeletal dysplasia?

A

Short stature

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What is achondroplasia?

A

disproportionately short limbs
prominent forehead
widened nose

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What are the features associated with Marfan’s syndrome?

A
High arched palate
Scoliosis
Pectus excavatum
eye Problems 
aortic aneurysm
cardiac valve incompetance
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What is marfan’s syndrome?

A

mutation fibrillin gene

tall with disproportionately long limbs and ligamentous laxity

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What is Ehlers-Danlos syndrome?

A

Abnormal elastin and collagen

Hypermobility, vascular fragility, easy bruised, joint instability, scoliosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

MSK manifestation down syndrome?

A

short
ligamentous laxity
recurrent dislocation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Muscular dystrophies are rare and only affect boys

A

T

usually x linked recessive

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What is Duchenne muscular dystrophy?

A

Defect in dystrophin gene involving calcium transport

muscle weakness

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

How is Duchenne muscular dystophy diagnosis confirmed?

A

raised serum phosphokinase

abnormalities muscle biopsy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Duchenne muscular dystophy is obvious at birth

A

F

muscle weakness may only be noticied when child walks with difficulty

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Duchenne muscular dystophy can cause cardiac and resp failure

A

T

death typical early 20s

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What is the cause of cerebral palsy?

A

Insult to brain before or after birth

genes, brain malformation, early pregnancy, intrauterine infection

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Incidence of brachial plexus injury is around 2/100

A

F

2/1000

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

brachial plexus injury commonly arises in large babies

A

T

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

What is Klumpke’s palsy?

A

lower brachial plexus injry caused by forceful adduction

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Consequences of klumpke’s palsy?

A

paralysis of intrinsic hand muscles - fingers typically flexed WAITER’S TIP POSTURE
Horner’s syndrome (drooping eyelid and bloodshot eyes)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

At birth children have - knees

A

varus

bow legs

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

At 14 months the legs become..

A

straight

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Children develop - knees at age 3 which gradually become less extreme at age 7/9

A

valgus (knock knees)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Lower limb variations are abnormal in children if:

A

greater than 2 yrs old
pain
unilateral

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

What is femoral neck anteversion? What does it cause?

A

femoral neck slightly pointing forwards

in toeing

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

femoral neck anteversion is a surgical emergency

A

F

part of normal anatomy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

At birth all feet are flat

A

T

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

What is mobile flat footedness?

A

Only present on weight bearing

flattened medial arch forms with dorsiflexion of great toe

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

rigid type flat foot is normal and requires no intervention

A

F

suggests underlying bony abnormality

30
Q

Curly toes will correct without intervention

A

T

31
Q

What is the ortolani manouvere?

A

For dislocted hip:

abduction and anteromedially directed force causes reduction

32
Q

What is the Barlow manouvere?

A

To dislocate the hip: adduction and posterior force

33
Q

What is DDH?

A

developmental dysplasia of hip

dislocation or subluxation of femoral head during perinatal period

34
Q

RIsk factors for DDH?

A

Born in BREACH position (bottom first)
FH
THESE BABIES HAVE ROUTINE ULTRASOUND

35
Q

X rays can be used to diagnose DDH

A

F

femoral head unossified until 4-6 months

36
Q

How is DDH treated in patients <2yrs old

A
PAVLIK HARNESS (high success rate!)
maintaining reduction via abduction and comfortable flexion
used for 6 wks full time + 6 wks part time
37
Q

Why is early diagnosis key in DDH?

A

over 2 yrs old NEVER NORMAL

open reduction needed

38
Q

What is the commonest cause of hip pain in childhood?

A

Transient synovitis of hip

39
Q

Transient synovitis commonly occurs shortly after an upper resp tract infection

A

T

40
Q

How does transient synovitis commonly present?

A

Limp/ relucatance to weight bear
Restricted range motion
low grade fever

41
Q

Treatment transient synovitis?

A

NSAIDs and rest
self limiting
if pain not resolved within few weeks check another cause pain

42
Q

What is Perthes disease?

A

Idiopathic osteochondritis of femoral head

43
Q

Perthes disease pathology?

A

Femoral head transiently loses its blood supply

Resulting in necrosis, sclerosis, fragmentation, reossification and remodelling

44
Q

Perthes: Affected children present with pain and a limp, most cases are unilateral

A

T

45
Q

What is the FIRST CLINICAL SIGN of perthes disease?

A

Loss of internal rotation

46
Q

Phenotype of perthes disease patient?

A

Small hyperactive boy

47
Q

Treatment perthes disease?

A

Attempt influence shape femoral head
activity modification
bracing

48
Q

What is SUFE?

A

slipped upper femoral epiphyses

femoral head slips inferiorly in relation to femoral neck

49
Q

What is the pathology in SUFE?

A
growth plate (physis) not strong enough to support body weight
femoral epiphyses slips due to strain
50
Q

Typical phenotype SUFE?

A

black overweight adolescent boy

Hani’el is a sufi

51
Q

How does SUFE present?

A

Limp
Pain midgroin
CAN PRESENT PURELY WITH KNEE PAIN

52
Q

Why can sufe present with only knee pain?

A

Obturator nerve supplies femur and knee

53
Q

What is the PREDOMINANT clinical sign in sUFE?

A

Loss of internal rotation of the hip

54
Q

Adolescent body weight increase and sporting activities can commonly lead to what?

A

knee extensor mechanism pain

55
Q

Patellar tendonitis is self-limiting

A

T

56
Q

What is the aetiology of anterior knee pain (patellofemoraldysfunction)

A

muscle imbalance
ligamentous laitiy
genu valgum, wide hips, femoral neck anteversion
ADOLESCENT GIRLS

57
Q

anterior knee pain (patellofemoraldysfunction) is self limiting

A

T

58
Q

younger patients have a lower chance of healing a meniscal tear

A

F

higher chance

59
Q

What is the deformity in talipes equinovarus?

A

aka clubfoot
Ankle plantar flexion (equinous)
Supination of forefoot
Varus alignment of forefoot

60
Q

girls are twice as likely to get clubfoot

A

F

boys are twice as likely to get clubfoot

61
Q

What is the ponseti technique?

A

Plaster cast to correct talipes equinovarus deformity

62
Q

talipes equinovarus can result in fixed deformity if presentation is delayed

A

T

63
Q

80% of children with a ponseti plaster require tenotomy of achilles tendon

A

T

64
Q

Back pain in children and adolescents is not a red flag sign

A

F

IT TOTALLY IS

65
Q

In patients presenting with SUFE approximately 1/3 of cases are bilateral

A

T

66
Q

If a flat foot is described as rigid,what is a possible cause of the flat foot?

A

tarsal coalition -where the bones of the midfoot have an abnormal bony or cartilaginous connection

67
Q

If a flat foot is described as dynamic, present when the foot is weightbearing only, a type of mobile/flexible flat foot) what is a possible cause of the flat foot?

A

ligamentous laxity

68
Q

Patellar dislocations, are fairly common and the patient will usually be able to tell you that their knee cap moved round to the outside of their knee. They spontaneously relocate when the knee is straightened.

A

T

69
Q

Developmental dysplasia of the hip affects more girls than boys

A

T

70
Q

The patellar ‘always’ dislocates in which direction?

A

laterally

71
Q

20% of patients who suffer a patella dislocation will sustain a second one

A

T