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G264 Is it Kawasaki? To treat or not?
  1. S Deepak,
  2. K Warrior
  1. Paediatric Rheumatology, Nottingham University Hospitals NHS Trust, Nottingham, UK

Abstract

Aim To describe rare and severe complication of Kawasaki’s disease (KD) in a child who was not treated with Immunoglobulins (IVIG).

Methods Case report.

Results A previously fit and well 4-year-old boy presented with fever, red tongue, rash and cervical adenopathy. He was reviewed in the local hospital and was diagnosed with viral infection. He continued to be febrile and was diagnosed with KD after two weeks, when the skin of his palms and soles started peeling. He was managed conservatively and not given IVIG as the fever had settled. His echocardiogram showed aneurysm of left coronary artery and was commenced on aspirin. At the 3-month follow up with the cardiology team, he was noted to be hypertensive, requiring multiple anti-hypertensives, and was referred to the Paediatric Nephrology team.

On assessment, he appeared pale and lethargic and with raised inflammatory markers (ESR 110mm/hr) and nephrotic range proteinuria. His Doppler ultrasound scan showed bilateral renal artery aneurysms. In conjunction with Paediatric Rheumatology team, he was treated with two doses of IVIG with good clinical and biochemical response (ESR 14). His catheter angiogram showed widespread aneurysms affecting all the medium sized abdominal vessels (Figure 1). Because of the extensive involvement, he was treated with methylprednisolone and pulses of IV cyclophosphamide as per the polyarteritis nodosa protocol. He then developed an acute ischaemic cardiac event (Figure 2) and acute kidney injury requiring ventilation and haemodialysis in PICU. He was transferred to quaternary centre for cardiac care, where his coronaries were stented and immunosuppression switched to Infliximab on completion of the Cyclophosphamide regime. He has residual cardiac and renal impairment.

Conclusions This case highlights the importance of expeditious diagnosis and timely treatment to achieve optimal clinical outcome in KD, which is the commonest cause of acquired heart disease in the west. American Heart Association statement supports the diagnosis of KD even in the absence of full criteria when coronary abnormalities are present. Early referral to tertiary services needs to be considered in an atypical KD with low threshold for treatment with IVIG.

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