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Finals > Paeds: Rheum > Flashcards

Paeds: Rheum Flashcards

1
Q

Henoch-Schonlein Purpura

what is it

A

an IgA vasculitis that presents with a purpuric rash affecting the lower limbs and buttocks in children.

Inflammation occurs in the affected organs due to IgA deposits in the blood vessels.

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2
Q

Henoch-Schonlein Purpura

What are the 4 classic features

A
  1. purpura
  2. joint pain
  3. abdo pain
  4. renal involvement
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3
Q

Henoch-Schonlein Purpura

what age is it most common in

A

children <10y

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4
Q

Henoch-Schonlein Purpura

what is the condition often triggered by

A

an upper airway infection or gastroenteritis

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5
Q

Henoch-Schonlein Purpura

what is the rash caused by

A

inflammation and leaking of blood from small blood vessels under the skin, forming purpura

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6
Q

Henoch-Schonlein Purpura

what is purpura

A

red-purple lumps under the skin containing blood

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7
Q

Henoch-Schonlein Purpura

which joints are mostly affected

A

knees and ankles

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8
Q

Henoch-Schonlein Purpura

GI: in severe causes, HSP can lead to?

A
  • GI haemorrhage
  • intussusception
  • bowel infarction
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9
Q

Henoch-Schonlein Purpura

what can HSP nephritis lead to

A

microscopic or macroscopic haematuria and proteinuria

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10
Q

Henoch-Schonlein Purpura

Ddx for a non-blanching rash

A
  • meningococcal septicaemia
  • leukaemia
  • ITP
  • haemolytic uraemic syndrome
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11
Q

Henoch-Schonlein Purpura

EULAR/PRINTO/PRES criteria for dx

A

palpable purpura (not petichiae) + at least one of:

  • Diffuse abdo pain
  • Arthritis or arthralgia
  • IgA deposits on histology (biopsy)
  • Proteinuria or haematuria
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12
Q

Henoch-Schonlein Purpura

mnx

A

supportive: analgesia, rest, hydration

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13
Q

Henoch-Schonlein Purpura

monitoring

A
  • Urine dipstick monitoring for renal involvement

- Blood pressure

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14
Q

Henoch-Schonlein Purpura

prognosis

A
  • abdo pain: few days
  • kidney: 4-6w
  • 1/3 have recurrence within 6m
  • small proportion develop end stage renal failure
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15
Q

Juvenile Idiopathic Arthritis

what is it

A

autoimmune inflammation in the joints

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16
Q

Juvenile Idiopathic Arthritis

dx criteria

A

arthritis without any other cause

lasting >6 w

U16

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17
Q

Juvenile Idiopathic Arthritis

key presenting features

A

joint pain, swelling and stiffness.

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18
Q

Juvenile Idiopathic Arthritis

what are 5 key subtypes of JIA

A
  1. Systemic JIA
  2. Polyarticular JIA
  3. Oligoarticular JIA
  4. Enthesitis related arthritis
  5. Juvenile psoriatic arthritis
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19
Q

Juvenile Idiopathic Arthritis

what is Systemic JIA aka

A

Still’s disease

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20
Q

Juvenile Idiopathic Arthritis

what is Systemic JIA

A

a systemic illness that can occur throughout childhood in boys and girls.

idiopathic inflammatory condition

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21
Q

Juvenile Idiopathic Arthritis

Systemic JIA: typical features

A
  • Subtle salmon-pink rash
  • High swinging fevers
  • Enlarged lymph nodes
  • Weight loss
  • Joint inflammation and pain
  • Splenomegaly
  • Muscle pain
  • Pleuritis and pericarditis
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22
Q

Juvenile Idiopathic Arthritis

Systemic JIA: lab findings

A

-ve ANA + RF

raised CRP, ESR, platelets + ferritin

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23
Q

Juvenile Idiopathic Arthritis

Systemic JIA: key complication

A

macrophage activation syndrome (MAS)

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24
Q

Juvenile Idiopathic Arthritis

Systemic JIA: what is macrophage activation syndrome (MAS)

A

a severe activation of the immune system with a massive inflammatory response.

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25
Q

Juvenile Idiopathic Arthritis

Systemic JIA: how does someone with macrophage activation syndrome present

A
  • acutely unwell child
  • DIC
  • anaemia
  • thrombocytopenia
  • bleeding
  • non-blanching rash

life threatening

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26
Q

Juvenile Idiopathic Arthritis

Systemic JIA: key inx finding with someone with MAS

A

low ESR

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27
Q

for children that have fevers >5d, what are the key non-infective differentials

A
  • Kawasaki disease
  • Still’s disease
  • rheumatic fever
  • leukaemia
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28
Q

Juvenile Idiopathic Arthritis

Polyarticular JIA: what is it

A

involves idiopathic inflammatory arthritis in 5 joints or more.

equivalent of RA in adults

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29
Q

Juvenile Idiopathic Arthritis

Polyarticular JIA: arthritic presentation

A
  • symmetrical

- small joints of the hands and feet, as well as the large joints such as the hips and knees

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30
Q

Juvenile Idiopathic Arthritis

Polyarticular JIA: are there systemic sx

A

minimal

  • mild fever
  • anaemia
  • reduced growth
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31
Q

Juvenile Idiopathic Arthritis

Polyarticular JIA: lab findings

A

most are RF -ve (seronegative)

Seropositive patients tend to be older children and adolescents

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32
Q

Juvenile Idiopathic Arthritis

Oligoarticular JIA: aka

A

pauciarticular JIA

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33
Q

Juvenile Idiopathic Arthritis

Oligoarticular JIA: how many joints does it involve and which ones

A

4 joints or less

typically only affecting a single joint (monoarthritis)

knee or ankle

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34
Q

Juvenile Idiopathic Arthritis

Oligoarticular JIA: who does it occur most frequently in

A

girls <6y

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35
Q

Juvenile Idiopathic Arthritis

Oligoarticular JIA: classic associated feature

A

anterior uveitis

refer to opthalmologist for mnx and follow up of uveitis

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36
Q

Juvenile Idiopathic Arthritis

Oligoarticular JIA: lab findings

A
  • mildly or normal inflamm markers
  • +ve ANA
  • RF -ve
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37
Q

Juvenile Idiopathic Arthritis

Enthesitis-Related Arthritis: who is it more common in

A

boys >6y

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38
Q

Juvenile Idiopathic Arthritis

Enthesitis-Related Arthritis: if this was an adult condition, what is it equivalent to

A

the seronegative spondyloarthropathy group of conditions:

  • ankylosing spondylitis
  • psoriatic arthritis
  • reactive arthritis
  • IBD-related arthritis
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39
Q

Juvenile Idiopathic Arthritis

Enthesitis-Related Arthritis: what is enthesis

A

the point at which the tendon of a muscle inserts into a bone

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40
Q

Juvenile Idiopathic Arthritis

Enthesitis-Related Arthritis: which gene do majority of patients have

A

HLA B27 gene

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41
Q

Juvenile Idiopathic Arthritis

Enthesitis-Related Arthritis: what other conditions may they have

A
  • psoriasis

- anterior uveitis

42
Q

Juvenile Idiopathic Arthritis

Enthesitis-Related Arthritis: why is it worth palpating key areas

A

to elicit tenderness of the entheses

43
Q

Juvenile Idiopathic Arthritis

Enthesitis-Related Arthritis: where is it common on the body

A
  • 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
44
Q

Juvenile Idiopathic Arthritis

Juvenile Psoriatic Arthritis: what is it

A

seronegative inflammatory arthritis associated with psoriasis

45
Q

Juvenile Idiopathic Arthritis

Juvenile Psoriatic Arthritis: pattern of joint involvement

A

varies:
- symmetrical polyarthritis affecting the small joints similar to rheumatoid

  • asymmetrical arthritis affecting the large joints in the lower limb
46
Q

Juvenile Idiopathic Arthritis

Juvenile Psoriatic Arthritis: signs on examination

A
  • Plaques of psoriasis
  • nail pitting
  • Onycholysis
  • Dactylitis
  • Enthesitis
47
Q

Juvenile Idiopathic Arthritis

mnx

A
  • NSAIDs
  • PO, IM or intra-articular steroids
  • DMARDs
  • Biologics: tumour necrosis factor inhibitors etanercept, infliximab and adalimumab
48
Q

Ehlers-Danlos Syndrome

what is it

A

umbrella term for a group of genetic conditions that cause defects in collagen,

resulting in hypermobility of the patient’s joints and abnormalities in connective tissue

49
Q

Ehlers-Danlos Syndrome

name 4 types

A
  1. Hypermobile
  2. Classical
  3. Vascular
  4. Kyphoscoliotic
50
Q

Ehlers-Danlos Syndrome

which type is most common in exams and clinical cases

A

Hypermobile

51
Q

Ehlers-Danlos Syndrome

key features of hypermobile EDS

A
  • most common
  • joint hypermobility
  • soft + stretchy skin
  • no gene identified
  • no mode of inheritance
52
Q

Ehlers-Danlos Syndrome

key features of Classical EDS

A
  • stretchy skin that feels smooth and velvety
  • severe joint hypermobility
  • joint pain
  • abnormal wound healing
  • lumps over pressure points e.g. elbow
53
Q

Ehlers-Danlos Syndrome

Classical EDS: what are they prone to

A
  • hernias
  • prolapses
  • mitral regurgitation
  • aortic root dilatation
54
Q

Ehlers-Danlos Syndrome

Classical EDS: inheritance pattern

A

autosomal dominant

55
Q

Ehlers-Danlos Syndrome

what is the most dangerous type

A

Vascular Ehlers-Danlos syndrome

56
Q

Ehlers-Danlos Syndrome

Vascular EDS: what is it

A

blood vessels are particularly fragile as a result of defective collagen

skin, internal organs and arteries are fragile and prone to rupturing.

57
Q

Ehlers-Danlos Syndrome

Vascular EDS: presentation

A

thin, translucent skin that you can almost see through.

58
Q

Ehlers-Danlos Syndrome

Vascular EDS: inheritance pattern

A

autosomal dominant

59
Q

Ehlers-Danlos Syndrome

Kyphoscoliotic EDS: presentation

A
  • hypotonia as neonate + infant
  • followed by kyphoscoliosis as they grow
  • significant joint hypermobility
  • tall + slim
60
Q

Ehlers-Danlos Syndrome

Kyphoscoliotic EDS: risk of what

A

rupture in the medium sized arteries.

61
Q

Ehlers-Danlos Syndrome

Kyphoscoliotic EDS: inheritance pattern

A

autosomal dominant

62
Q

Ehlers-Danlos Syndrome

presentation

A
  • Hypermobility in joints
  • Joint pain after exercise or inactivity
  • Joint dislocations
  • Soft stretchy skin
  • Easy bruising
  • Poor healing of wounds
  • Bleeding
  • Headaches
  • Autonomic dysfunction: dizziness, syncope
  • GOR
  • Abdo pain
  • IBS
  • Menorrhagia and dysmenorrhea
  • Premature rupture of membranes in pregnancy
  • Urinary incontinence
  • Pelvic organ prolapse
  • Temporomandibular joint dysfunction
  • Myopia and other
63
Q

Ehlers-Danlos Syndrome

what is used to assess the extent of hypermobility and support the diagnosis of hypermobility syndrome

A

Beighton Score

64
Q

Ehlers-Danlos Syndrome

what is the Beighton Score

A

1 point for each side of body (max 9):

  • Palms flat on floor with straight legs (score 1)
  • Elbows hyperextend
  • Knees hyperextend
  • Thumb can bend to touch the forearm
  • Little finger hyperextends past 90 degrees
65
Q

Ehlers-Danlos Syndrome

mnx

A
  • follow up for complications
  • physio
  • occupational therapy
  • good posture
  • moderate intensity of activity
  • psychology
66
Q

Ehlers-Danlos Syndrome

what syndrome can occur as a result of autonomic dysfunction

A

Postural Orthostatic Tachycardia Syndrome (POTS)

67
Q

Ehlers-Danlos Syndrome

what is POTS

A

Postural Orthostatic Tachycardia Syndrome

inappropriate tachycardia on sitting or standing up,

resulting presyncope, syncope, headaches, disorientation, nausea and tremor.

68
Q

Kawasaki Disease

aka

A

mucocutaneous lymph node syndrome

69
Q

Kawasaki Disease

what is it

A

a systemic, medium-sized vessel vasculitis

70
Q

Kawasaki Disease

who does it typically effect

A

children >5y

more common in Japanese and Korean children

B>G

71
Q

Kawasaki Disease

key complication

A

coronary artery aneurysm

72
Q

Kawasaki Disease

clinical features

A
  • fever>39 for >5d
  • widespread erythematous maculopapular rash
  • desquamation on palms and soles
  • strawberry tongue
  • cervical lymphadenopathy
  • bilateral conjunctivitis
  • cracked lips
73
Q

Kawasaki Disease

inx and results

A

FBC: anaemia, leukocytosis, thrombocytosis

LFTs: hypoalbuminaemia + elevated liver enzymes

raised ESR

raised WBC on urinalysis

echocardiogram: coronary artery pathology

74
Q

Kawasaki Disease

what are the 3 phases

A
  1. acute phase
  2. subacute phase
  3. convalescent stage
75
Q

Kawasaki Disease

what happens in the acute phase

A

The child is most unwell with the fever, rash and lymphadenopathy. This lasts 1 – 2 weeks.

76
Q

Kawasaki Disease

what happens in the subacute phase

A

desquamation and arthralgia occur and there is a risk of coronary artery aneurysms forming. This lasts 2 – 4 weeks.

77
Q

Kawasaki Disease

what happens in the convalescent stage

A

The remaining symptoms settle, the blood tests slowly return to normal and the coronary aneurysms may regress. This last 2 – 4 weeks.

78
Q

Kawasaki Disease

mnx

A
  • High dose aspirin to reduce the risk of thrombosis

- IV immunoglobulins to reduce the risk of coronary artery aneurysms

79
Q

Kawasaki Disease

why is aspirin usually avoided in children

A

risk of Reye’s syndrome

80
Q

Rheumatic Fever

what is it

A

an autoimmune condition triggered by streptococcus bacteria.

a multi-system disorder that affects the joints, heart, skin and nervous system

81
Q

Rheumatic Fever

what is it caused by

A

antibodies created against the streptococcus bacteria that also target tissues in the body. e,g, myocardium

82
Q

Rheumatic Fever

what is the causative organism

A

group A beta-haemolytic streptococcal, typically streptococcus pyogenes causing tonsillitis.

83
Q

Rheumatic Fever

what type hypersensitivity reaction is it

A

type 2: the immune system begins attacking cells throughout the body.

84
Q

Rheumatic Fever

how long after the initial infection does the type 2 hypersensitivity reaction occur

A

2-4w after initial infection

85
Q

Rheumatic Fever

presentation (6)

A
  1. fever
  2. joint pain
  3. rash
  4. SOB
  5. Chorea
  6. Nodules
86
Q

Rheumatic Fever

what is migratory arthritis

A

different joints become inflamed and improve at different times

giving the appearance that the arthritis is moving from one joint to the next.

87
Q

Rheumatic Fever

how is the heart involved

A

carditis w/ pericarditis, myocarditis + endocarditis leads to:

  • tachy/bradycardia
  • murmurs from mitral valve disease
  • pericardial rub
  • heart failure
88
Q

Rheumatic Fever

what are the 2 key skin findings

A

Subcutaneous nodules

Erythema marginatum rash

89
Q

Rheumatic Fever

what is erythema marginatum

A

a rash which involves pink rings of varying sizes affecting the torso and proximal limbs.

90
Q

Rheumatic Fever

what is the key nervous system sx

A

chorea: irregular, uncontrolled and rapid movements of the limbs

aka Sydenham chorea and historically as St Vitus’ Dance.

91
Q

Rheumatic Fever

inx that can help support dx

A
  • throat swab
  • ASO antibody titres
  • Echo, ECG + CXR can assess the heart involvement
  • Jones Criteria
92
Q

Rheumatic Fever

dx

A

Jones Criteria: recent strep infection + 2 major criteria

or 1 major + 2 minor

93
Q

Rheumatic Fever

Jones Criteria: major criteria

A

JONES

  • Joint arthritis
  • Organ inflammation: e.g. carditis
  • Nodules
  • Erythema marginatum rash
  • Sydenham chorea
94
Q

Rheumatic Fever

Jones Criteria: minor criteria

A

FEAR

  • Fever
  • ECG changes (prolonged PR interval) without carditis
  • Arthralgia without arthritis
  • Raised CRP + ESR
95
Q

Rheumatic Fever

what are ASO antibody titres

A

Anti-streptococcal antibodies are antibodies against streptococcus.

they indicate a recent streptococcus infection

96
Q

Rheumatic Fever

describe the ASO antibody titre levelss after an acute strep infection

A

Rise over 2 – 4 weeks

Peak around 3 – 6 weeks

Gradually Rheumatic Feverfalls over 3 – 12 months

97
Q

Rheumatic Fever

why are ASO levels repeated after 2w

A

Confirm a negative test

Assess whether levels are rising or falling

98
Q

Rheumatic Fever

what mnx helps prevent the development of rheumatic fever.

A

trx of strep infection

tonsillitis: phenoxymethylpenicillin (penicillin V) for 10 days

99
Q

Rheumatic Fever

mnx

A
  • NSAIDs
  • aspirin + steroids: carditis
  • prophylactic penicillin continued into adulthood
  • monitoring complications
100
Q

Rheumatic Fever

complications (3)

A
  • recurrence
  • mitral stenosis
  • chronic HF

Finals (47 decks)

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