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Paeds > Multi-system disorders > Flashcards

Multi-system disorders Flashcards

1
Q

What is HSP? Figures?

A

Henoch-Schönlein Purpura (HSP)
Acute IgA mediated vasculitis of skin, GI, kidneys, joints and sometimes CNS

20/10,000 children per year
More boys than girls
90% present before aged 10

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2
Q

Prodrome of HSP?

A

Headaches, anorexia and fever

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3
Q

Describe the rash in HSP

A

Especially involving the legs and buttocks

Erythematous macular/urticarial-> blanching papules-> palpable purpura

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4
Q

What other symptoms/signs are there in HSP?

A
  • Abdominal pain and vomiting (35-85%)
  • Joint pain and swelling (60-84%), especially involving the knees and ankles
  • Subcutaneous oedema (20-50%)
  • Scrotal oedema (2-35%)
  • Bloody stools (intussuseption)
  • Kidneys: glomerular lesions/capillary proliferation/necrosis/nephrotic syndrome
  • Less commonly intracranial haemorrhage/orbital haematomas
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5
Q

How do you diagnose HSP?

A

By excluding other causes.
• Anti-nuclear antibodies and rheumatoid factor to rule out other autoimmune conditions
• Urinalysis and U&Es for renal function
• D-dimer may be raised
• Immune complexes
• ITP (low platelets)
• Aplastic anaemia (low red, white blood cells and platelets)

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6
Q

Treatment of HSP

A
  • Hydration
  • Monitoring for renal complications (eg hypertension, urine dipstick for proteinuria)
  • Treating minor symptoms of arthritis, oedema, fever, or malaise
  • Discontinuance of any drugs suspected of playing a causative role
  • Analgesics: Acetaminophen, Ibuprofen, Naproxen
  • Steroids if nephrotic syndrome/severe abdo pain/oedema/neuro involvement
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7
Q

What is kawasaki’s?

A

Acute febrile vasculitic syndrome of early childhood, occurs after a variety of infections
Can lead to death from coronary artery aneurysm and MI

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8
Q

Symptoms of Kawasaki’s? (11)

A
  • Prolonged fever (5days) +
  • Irritability and miserable
  • Nonexudative bilateral conjunctivitis (90%)
  • Anterior uveitis (70%)
  • Perianal erythema (70%)
  • Sterile pyuria
  • Erythema and oedema on the hands and feet; the latter impedes ambulation
  • Strawberry tongue and lip fissures
  • Hepatic, renal, and GI dysfunction
  • Myocarditis and pericarditis
  • Lymphadenopathy (75%); generally, a single, enlarged, nonsuppurative cervical node measuring approximately 1.5 cm
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9
Q

How is Kawasaki’s diagnosed?

A
  • Elevated erythrocyte sedimentation rate (ESR), CRP, Alpha1-antitrypsin, platelets
  • Rule out viral and strep/staph infections
  • Urine proteins, meprin A and filamin C
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10
Q

Treatment of Kawasaki’s?

A
  • IV Immunglobulins
  • Aspirin
  • Steroids, infliximab, methotrexate, anticoagulants
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11
Q

What is Lyme’s disease?

A

Multisystem illness usually caused by infection with the spirochete Borrelia burgdorferi
Transmitted to humans via tick bites, from infected ticks of the genus Ixodes.

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12
Q

Signs and symptoms of Lyme’s

A
  • Erythema migrans (EM) - Rash
  • Fever
  • Myalgias
  • Malaise
  • Arthralgia
  • Headache
  • Tender local adenopathy (local, not diffuse)
  • Carditis (heart block)
  • Meningism (aseptic meningitis)
  • Cranioneuropathy (Bell’s palsy)
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13
Q

What does erythema migrans look like i Lyme’s?

A

Target lesion

annular homogenous erythema (59%), central erythema (30%), central clearing (9%), or central purpura

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14
Q

Diagnosis of Lyme’s

A

Clinical (from rash)
1: enzyme immunoassay (EIA) or immunofluorescence assay (IFA) - Total Lyme titer or IgG and IgM titers
2: Western blot testing
LP and ECG needed to investigate meningism/carditis

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15
Q

Treatment of Lyme’s

A
  • Doxycycline, amoxicillin, or cefuroxime axeti if early
  • (No Doxy in children)
  • If neuro symptoms: IV penicillin, ceftriaxone, or cefotaxime; oral doxycycline
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16
Q

Why is Lyme’s common in Southampton?

A

New forest deer are a reservoir

17
Q

What is rheumatic fever?

A

Acute rheumatic fever (ARF) is an autoimmune inflammatory process that develops as a sequela of group A streptococcal pharyngeal infection. Due to (a) strep toxin (b) abnormal immune response.

18
Q

Signs and symptoms of rheumatic fever?

A
  • Sore throat in preceding weeks
  • Polyarthritis
  • Carditis (esp younger children) -> congestive heart failure
  • Erythema marginatum
  • Chroea (esp in children, usually resolves)
  • Subcutaneous nodules (non pus producing)
19
Q

Is rheumatic fever common?

A

Hardly ever seen acutely in Western Europe, only as heart disease in the older generation. This decline due to improved socioeconomic conditions & decline in prevalence of the classically described rheumatogenic strains of group A strep

20
Q

What is JIA?

A

Juvenile Idiopathic Arthritis

21
Q

Signs and symptoms of JIA

A
  • Arthritis present for at least 6 weeks before diagnosis (mandatory for diagnosis of JIA)
  • Either insidious or abrupt disease onset, often with morning stiffness or gelling phenomenon and arthralgia during the day
  • Complaints of joint pain or abnormal joint use
  • History of school absences or limited ability to participate in physical education classes
  • Spiking fevers occurring once or twice each day at about the same time of day
  • Evanescent rash on the trunk and extremities
  • Psoriasis or more subtle dermatologic manifestations
22
Q

Describe the 6 subtypes of JIA

A
  • Systemic-onset juvenile idiopathic arthritis (2 weeks daily fever, rash? Lymph nodes?)
  • Oligoarticular juvenile idiopathic arthritis (destructive)
  • Polyarticular juvenile idiopathic arthritis (uveitis)
  • Psoriatic arthritis (dactylitis/nail pitting/psoriasis in family)
  • Enthesitis-related arthritis (inflam at tendon insertion, sacroiliac pain, HLA B27, heel pain)
  • Undifferentiated arthritis
23
Q

Diagnosis of JIA

A

Diagnosis is by history and examination. Tests could include ANA, RF, ESR, CRP, X-ray, HLA-B27

24
Q

If only one joint is affected in JIA, what do you need to exclude?

A
  • Soft tissue swelling/joint space narrowing
  • Bony erosions/osteopenia/osteoporosis
  • Growth disturbances/epiphyseal compression fracture
  • Joint subluxation/synovial cysts
  • Exclude: osteomyelitis, septic arthritis, trauma, IBD, malignancy, CT disease, sickle cell
25
Q

Treatment of JIA

A 
NSAIDs
Methotrexate
Sulfasalazine is an alternative but it should be avoided in systemic-onset JIA
Intra-articular steroids
•	Psychosocial and academic counselling
•	Nutrition, physical therapy and OT
26
Q

What is measles?

A

One of the most contagious infectious diseases. Viral infection is transmitted via respiratory droplets, which can remain active and contagious, either airborne or on surfaces, for up to 2 hours.
Notifiable disease
Late sequelae can cause developmental delay

27
Q

How is measles infection avoided?

A

Immunisation programme

MMR is a live vaccine. Infection confers life-long immunity. Given at age 12-13 months and 3yrs 4 months.

28
Q

Risk factors for measles infection

A
  • Children with immunodeficiency due to HIV or AIDS, leukaemia, alkylating agents, or corticosteroid therapy, regardless of immunisation status
  • Travel to areas where measles is endemic or contact with travellers to endemic areas
  • Infants who lose passive antibody before the age of routine immunisation
29
Q

Risk factors for severe measles illness

A
  • Malnutrition
  • Underlying immunodeficiency
  • Pregnancy
  • Vitamin A deficiency
30
Q

Signs and symptoms of measles

A
  • Prodromal high fever
  • Coryza, cough, conjunctivitis (3 C’s)
  • Pathognomonic enanthem (spots inside the mouth that look like a tiny grains of white sand surrounded by a red ring)
  • Blanching erythematous maculopapular rash develops on the third to seventh day. (exanthema)
  • Associated symptoms include photophobia, periorbital oedema, and myalgia
31
Q

Complications of measles infection

A

• Otitis media, interstitial pneumonitis, exacerbation of TB, transient loss of hypersensitivity reaction to tuberculin skin test, encephalomyelitis, diarrhoea, sinusitis, stomatitis, subclinical hepatitis, lymphadenitis, and keratitis, which can lead to blindness

32
Q

Differential diagnoses in child presenting with measles-like symptoms

A
  • Kawasaki disease
  • Dengue
  • Serum sickness
  • Syphilis
  • SLE
  • Toxic shock syndrome
33
Q

Management of measles

A 
Notifiable disease
IgM/viral culture/PCR
Maintain hydrations
Vit A supplementation
IgG
34
Q

Prognosis of measles

A

Croup, encephalitis, and pneumonia are the most common causes of death associated with measles. Measles encephalitis, a rare but serious complication, has a 10% mortality.

35
Q

3 common signs of congenital rubella

A

Cataracts
Congenital heart disease
Deafness

36
Q

Which trimester is rubella infection most dangerous?

A

1st trimester

Paeds (14 decks)

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