Paeds: The Limping Child (2) Flashcards

1
Q

Features of Henoch-Schonlein-Purpura

A
  • palpable purpuric rash (with localized oedema) over buttocks and extensor surfaces of arms and legs
  • abdominal pain
  • polyarthritis
  • features of IgA nephropathy may occur e.g. haematuria, renal failure
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2
Q

Management of Henoch-Schonlein -Purpura

A
  • analgesia for arthralgia
  • treatment of nephropathy is generally supportive
  • there is inconsistent evidence for the use of steroids and immunosuppressants
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3
Q

Prognosis of Henoch-Schonlein purpura

A
  • usually excellent, HSP is a self-limiting condition, especially in children without renal involvement
  • around 1/3rd of patients have a relapse
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4
Q

How common malignancy is in paediatric rheumatology?

A
  • 1% of patients referred to paediatric rheumatology have underlying malignancy
  • Haematological disease, e.g. leukaemia and solid organ, e.g. neuroblastoma
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5
Q

What are primary bone tumours?

A

Primary bone tumour

  • Osteosarcoma
  • Osteoid osteoma (benign)
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6
Q

Osteoid osteoma

  • epidemiology
  • symptoms
  • location
  • x - ray findings
A

Osteoid osteoma → benign, bone-forming neoplasm

  • M>F = 2:1
  • Teens and 20s

Symptoms:

  • Severe nocturnal pain relieved by aspirin
  • Hot on bone scan

Location: lower limb, diaphyseal cortex

X-ray: lytic lesion with central nidus and sclerotic rim

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7
Q

Osteosarcoma

  • epidemiology
  • symptoms
  • location
  • management
A

Osteosacroma

  • 20% of all primary bone tumours
  • Incidence of 5 per 1,000,000
  • Peak age 15-30, commoner in males
  • Commonest primary bone tumour
  • May arise secondary to Paget’s or irradiation

Symptoms: pain, warm, bruit

Location: knee metaphysis

Management: limb preserving surgery may be possible and many patients will receive chemotherapy

Location

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8
Q

Callular changes in osteosarcoma

A

Mesenchymal cells with osteoblastic differentiation

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9
Q

X-ray changes in osteosarcoma

A

Periosteal Elevation:

  • Sunburst appearance
  • Codman’s triangle
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10
Q

Ewing’s sarcoma

  • epidemiology
  • presentation
  • location
  • management
A

Ewing’s sarcoma

Epidemiology

  • commoner in males
  • Incidence of 0.3 / 1, 000, 000
  • onset typically between 10 and 20 years of age
  • Histologically it is a small round tumour

Signs and Symptoms:

painful, warm, enlarging mass, fever, ↑ESR, anaemia, ↑WCC

Location: femoral diaphysis is commonest site

Management: Blood borne metastasis is common and chemotherapy is often combined with surgery

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11
Q

X-ray changes seen with Ewing’s sarcoma

A
  • Lytic tumour
  • Onion-skin periosteal reaction
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12
Q

(3) types of Benign Cartilaginous Neoplasms

A
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13
Q

Association of ALL and MSK

A
  • Bone pain and arthralgia in 20 - 40%
  • Suspect from history, exam, or blood count
  • Diagnosis by bone marrow aspirate
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14
Q

Neuroblastoma

  • epidemiology
A

Neuroblastoma

  • one of the top five causes of cancer in children
  • accounting for around 7-8% of childhood malignancies
  • Median age of onset is around 20 months
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15
Q

Pathophysiology of neruroblastoma

A

The tumour arises from neural crest tissue of the adrenal medulla (the most common site) and sympathetic nervous system

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16
Q

Features of neuroblastoma

A
  • abdominal mass
  • pallor, weight loss
  • bone pain, limp from secondary spread
  • hepatomegaly
  • paraplegia
  • proptosis
17
Q

Ix in neuroblastoma

A
  • raised urinary vanillylmandelic acid (VMA) and homovanillic acid (HVA) levels
  • calcification may be seen on abdominal x-ray
  • biopsy
18
Q

Treatment and prognosis in neuroblastoma

A

Treatment surgery, radiation, chemotherapy, stem cell transplantation Prognosis US five-year survival ~95% (< 1 year old), 68% (1–14 years old)

19
Q

Features that raise concerns of possible malignancy (in MSK) - red flags

A
  • Bone pain (night time)
  • Weight loss
  • Night sweats or fevers
  • Abnormal bloods
  • X-ray changes
20
Q

What’s Juvenile Idiopathic arthritis?

A

Juvenile idiopathic arthritis (JIA)

  • arthritis occurring in someone who is less than 16 years old
  • lasts for more than 6 weeks
21
Q

What’s pauciarticular juvenile idiopathic arthritis?

  • features
A

Pauciarticular JIA

  • if 4 or less joints are affected
  • it accounts for around 60% of cases of JIA

Features of pauciarticular JIA

  • joint pain and swelling: usually medium sized joints e.g. knees, ankles, elbows
  • limp
  • ANA may be positive in JIA - associated with anterior uveitis
22
Q

What’s systemic-onset juvenile idiopathic arthritis?

A

Systemic onset JIA is a type of JIA which is also known as Still’s disease

23
Q

Features of Systemic onset idiopathic juvenile arthritis

+ investigations

A

Features of systemic onset JIA aka Still’s disease:

  • pyrexia
  • salmon-pink rash
  • lymphadenopathy
  • arthritis
  • uveitis
  • anorexia and weight loss

Investigations

  • ANA may be positive, especially in oligoarticular JIA
  • rheumatoid factor is usually negative
24
Q

Classification of Juvenile Idiopathic Arthritis (JIA)

A

Classification into sub-types by clinical onset pattern in first 6 months

25
Q

Systemic arthritis features and criteria

A

Systemic arthritis
= arthritis and quotidian (high daily) fever PLUS

One of:

  • evanescent, pink rash
  • lymphadenopathy
  • serositis
  • hepatosplenomegaly

*Unwell - exclude sepsis & malignancy

26
Q

What’s Macrophage activation syndrome?

A
  • Life threatening complication of sJIA (systemic juvenille idiopathic arthritis)
  • Anaemia, leucopenia, thrombocytopaenia
  • Raised triglycerides
  • Falling ESR (low fibrinogen)
  • Raised liver enzymes
  • Raised ferritin (may be > 100,000)
  • Treat any underlying trigger PLUS high dose steroids / CyA
27
Q

Features of oligoarthritis

A
28
Q

Features of polyarthritis

A
29
Q

Diagnosis of Idiopathic Juvenile Osteoarthritis

A

Juvenile idiopathic arthritis

  • a group of diseases characterised by inflammation of joints that cannot be explained by another mechanism - i.e. exclusion of conditions such as infection / trauma / malignancy and others
  • onset pattern of arthritis
  • Polyarthritis onset appears different to oligoarthritis onset and subsequent polyarticular course (“extended oligoarthritis”)

*Although predominantly a clinical diagnosis investigation may be needed to exclude other causes, but this will be driven by the clinical scenario.

30
Q

Management of idiopathic juvenile osteoarthritis

A
  • MDT approach - paediatric rheumatology review via network

OPTHALMOLOGY REFERRAL ! Needed

  • Drugs: IA steroid induction then DMARD if recurrent. Methotrexate first line then biologics anti TNF poly/extended oligo or anti IL-6 systemics.
31
Q

How to clinically differentiate Juvenile idiopathic arthritis to another alternative diagnosis

A
  • other (than JIA) conditions can often be excluded by careful history e.g. fever/rashes / pallor / careful history of trauma mechanism (including NAI)

Alternative diagnosis:

  • Reactive arthritis → usually viral and self-limiting over a few days
  • Trauma → would need to be significant to cause persistent swelling
  • Septic arthritis → usually associated with fever and systemic upset. Immunosuppressed patients may behave differently
  • Malignancy → look for other signs, especially constant limb / joint pain / pallor / hepatosplenomegaly / lymphadenopathy
  • Consider IBD / congenital disease / coagulopathy, and look for signs
32
Q

Investigations for Juvenile Idiopathic Arthritis

A

Investigations:

For JIA - nil specific

  • Often ANA +
  • RF if poly disease
  • HLA B27 in boys (usually)
  • Non-specific inflammatory response
  • XRAY for bony change - rare early in disease course
  • US / MR scan for soft tissues