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Kardielle komplikationer ved Kawasakis syndrom - en opdatering Jesper Reimers,

Kardielle komplikationer ved Kawasakis syndrom - en opdatering Jesper Reimers, BørneUngeKlinikken, Rigshospitalet. Hospital Discharge Register, Denmark. Hospital Discharge Register, Sweden. Hospital Discharge Register, Finland. Incidence /100 000 <5y , Nordic countries. Sweden 10.4

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Kardielle komplikationer ved Kawasakis syndrom - en opdatering Jesper Reimers,

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  1. Kardielle komplikationer ved Kawasakis syndrom - en opdatering Jesper Reimers, BørneUngeKlinikken, Rigshospitalet

  2. Hospital Discharge Register, Denmark

  3. Hospital Discharge Register, Sweden

  4. Hospital Discharge Register, Finland

  5. Incidence /100 000 <5y, Nordic countries • Sweden 10.4 • Norway 7.2 • Denmark 11.7 • Iceland 12.8 • Finland 11.5 • (Japan i 2006: 185)

  6. Kawasaki sygdoms forløb

  7. Kardiovaskulære manifestationer • Myocardit • Tachycardi • Nedsatkontraktilitet • Pericardit • Gnidningsmislyd • Perikardieexudat • Koronaraffektion • Fusiformeaneurismer < 8 mm • Giant aneurismer > 8 mm • Periferarterielaffektion • Aneurismer

  8. Koronar aneurismer • Risiko op til 25% hosubehandlede • Hyppigsthospatienter < 1 år • Giant aneurismer • Op til 33% risiko for AMI, arrhythmi, død • Fuciformeaneurismer • Op til 50% forsvinder

  9. Ekko af koronararterier

  10. Koronar aneurismer

  11. Medicinsk behandling • Acetylsalisylsyre • (30-) 80-100 mg/kg pofordeltpå 4 dagligedoserindtilfeberenervæk • 3-5 mg/kg po x 1 dagligimindst 6 ugerellerindtilkoronaraneurismerervæk. • Immunoglobulin • 2 mg/kg iv fordelt over 1, 2 eller 5 dage • Corticosteroid • Po 2-3 mg/kg i 2 uger, 1.5 mg/kg i 2 uger • Iv 2 mg/kg imindst 5 dage, aftrappesoralt over 15 dage. • Anti-TNFalfa • 5-10 mg/kg iv

  12. Koronar aneurismer • Effektaf immunoglobulin behandling • reducererrisikoen for udviklingafaneurismer med 85 -95% • hosbørn < 1 år= 8% • hosbørn > 1 år = 2% • Givetpå 5 dagenmedførerlavererisiko • Givetefter 10 dagenmedførerhøjererisiko

  13. Medicinsk behandling • Acetylsalisylsyre • (30-) 80-100 mg/kg pofordeltpå 4 dagligedoserindtilfeberenervæk • 3-5 mg/kg po x 1 dagligimindst 6 ugerellerindtilkoronaraneurismerervæk. • Immunoglobulin • 2 mg/kg iv fordelt over 1, 2 eller 5 dage • Corticosteroid • Po 2-3 mg/kg i 2 uger, 1.5 mg/kg i 2 uger • Iv 2 mg/kg imindst 5 dage, aftrappesoralt over 15 dage. • Anti-TNFalfa • 5-10 mg/kg iv

  14. Effekt af immunoglubulin og prednisolone behandling • Newburger et al. Randomized trial of pulsed corticosteroid therapy for primary treatment of Kawasaki disease. N Engl J Med 2007:356;663-75 • Ingeneffektpåkoronaraneurismer • Inoue et al. A multicenter prospektive randomized trial of corticosteroids in primary therapy for Kawasaki disease. J Pediatr 2006:149;336-41 • Reducerethyppighedafkoronaraneurismerog “treatment failure”

  15. Severe Kawasaki Defineresved >= 5 points Points 2 2 2 2 1 1 1 • S-Na <= 133 mmol/l • Syg <= 4 dage • ASAT >= 100U/L • Neutrofile >= 80% • Blodplader <= 30 x 104/µl • CRP >= 100 mg/L • Alder <= 12 måneder Ca 25% afderespatienterhar severe Kawasaki

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