Kawasaki disease

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KAWASAKI DISEASE

• Are we missing it

Dr. Krishna Kumar M.D., D.C.H., MRCP,
MRCPCH Paediatrician, Ranchi
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Tomisaku Kawasaki '67 Mucocut.
Lymph N. Syndrome Subsequent reports from all
over world Commonest cause of Acquired Heart
Disease in Children below 5 Yrs. in
developed world Indian Scenario
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Case - 1

• 1½ yrs. old boy
• unexplained fever 8 days
• trunkal rash, Red lips
• Platelet count - 4.5 lacs
• Rapid response to I.V.I.G.
• No follow up

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Case - 2

• 2 yrs. old girl
• high grade fever 5 days
• Conjunctival Congestion
• Bil. Cervical Lymph node
• Polymorphous rash
• Oedema hands feet

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Day 7
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28-28.04.064
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Case - 3

• 2½ yrs. old Male
• Fever for 20 days -
• had exanthema at outset, with conjunctivits, red
  lips cervical adenopathy
• Clue Platelet count 6 lacs
• - periungual desquamation

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Why this disease

• No known aetiology
• A vasculitis - affects medium sized arteries, esp
  coronaries
• Heals by fibrosis --gt Aneurysm / stenosis
• --------gt Coronary thrombosis.
• Incidence 20-25 in untreated cases of KD

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Diagnosis mainly Clinical AHA 2006
guidelines.

• Unexplained fever of at least 5 days, plus
• at least 4 of 5 Criteria.

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• 1. Polymorphous rash - (80)
• 2. Conjuntival Congestion - (85)
• 3. Changes in lips oral cavity- (90)
• 4. Cervical Lymphadenopathy - (70)
• Occ. solitary
• 5. Changes in extremities - (70)

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• Other important associations -
• - extreme irritability
• - Erythema and induration at BCG vaccination
  site.
• Incomplete KD - equally likely to cause coronary
  aneurysms

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• Phases of Illness -
• Acute 0 - 10 days
• stage of vasculitis - aneurysms
  rare
• IVIG most useful
• Subacute 10-28 days
• Stage of desquamation
• thrombocytosis
• Coronary Aneurysms - (20-25)
• Risk of sudden death
• Convalecent 4 wks - 6/8 weeks- Beau's lines.

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Duration (weeks)
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• Natural History of Coronary Changes -
• - Coronary A. dialatations, small aneurysms
  regress within 1-2 yrs.
• - Large aneurysms (gt 8 mm) fail to regress
• ----gt thrombosis / MI / Hge - sudden death.
  

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• Investigations -
• No diagnostic Lab tests -
• Acute phase reactants
• Platelet Count
• 2 D-Echo-cardiography

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• Treatment - aims at reducing inflammn
• I.V.I.G. 2 G / Kg.
• Reduces risk of CAA by 90
• Aspirin - Anti-inflam. dose - 2 wks.
• Antithrombotic dose - as required.
• Steroids reported to CAA incidence
• Follow up with serial 2 D echo - 2 wks, 6 wks,
• 12 wks. Further echo's as required.

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• TAKE HOME MESSAGES
• Not all fevers with rash is viral -
• KD needs prompt diagnosis which is primarily
  clinical -
• Early treatment within 10 days of onset with
  I.V.I.G. markedly reduces coronary
  complications.
• Thrombocytosis with the typical skin changes is
  pathognomonic of this condition but comes late.

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THANK YOU !