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Atypical Kawasaki disease with peripheral gangrene and myocardial infarction: therapeutic implications
M von Planta1, M Fasnacht, C Holm
1Division of Immunology/Haematology, University Children's Hospital Zürich, Switzerland.
Insights
Atypical Kawasaki disease (KD) with peripheral gangrene in infants can be treated with prostaglandin E1 (PGE1). Careful monitoring is crucial to prevent cardiac complications during this treatment.
Area of Science:
- Pediatric Cardiology
- Rheumatology
- Vascular Medicine
Background:
- Kawasaki disease (KD) is a leading cause of acquired heart disease in children.
- Peripheral gangrene is a rare but severe complication in infants under 7 months with KD.
- Current treatments focus on reducing inflammation, vasospasm, and thrombosis.
Observation:
- A 2-month-old girl presented with atypical KD, peripheral gangrene, and myocardial infarction.
- This patient had incomplete KD and peripheral ischemia, a challenging clinical scenario.
- The patient received prostaglandin E1 (PGE1) therapy.
Findings:
- PGE1 therapy appeared successful in restoring perfusion to the extremities.
- No significant long-term sequelae were observed in the peripheral vasculature.
- A potential link between PGE1 and myocardial infarction development was noted due to blood flow shunting.
Implications:
- PGE1 may be a viable treatment for atypical KD with peripheral gangrene in infants.
- Vigilant cardiac monitoring is essential during PGE1 treatment to detect myocardial infarction.
- This case highlights the complex interplay between KD complications and therapeutic interventions.
Abstract:
We describe a 2-month-old girl with atypical Kawasaki disease (KD) complicated by peripheral gangrene and myocardial infarction. Peripheral ischaemia leading to gangrene is a rare but serious complication of KD in infants younger than 7 months of age. Treatment has been targeted at reducing arterial inflammation, arteriospasm and thrombosis. We report the first patient with incomplete KD and peripheral ischaemia in whom therapy with prostaglandin E1 (PGE1) as vasodilating and antithrombotic agent appeared successful, restoring hand and foot perfusion without significant long-term sequelae. However, PGE1 could have supported development of myocardial infarction by shunting blood away from ischaemic areas distal to a giant coronary artery aneurysm with beginning thrombosis. CONCLUSION. Atypical KD with peripheral gangrene appears to react favourably to treatment with PGE1, but needs careful monitoring to detect early signs of cardiac ischaemia.