Morning Report 7/13/09

 Acute febrile vasculitic syndrome of early childhood  Affecting all blood vessels in the body but mostly medium and small vessels with a preferential involvement of the coronary arteries.  Exact etiology unknown but thought to be infectious in nature  Immune response thought to be oligoclonal or antigen driven

 Race: Japanese > Blacks, Polynesians, Filipinos > Whites  Gender: Male:Female~ 3:2  Age:  90-95% <10years old  Peak incident 18-24months

 Presence of 5 or more days of fever + 4 or more of the 5 principle clinical features

 Arthritis/arthralgia  Irritability  Diarrhea, Vomiting, Abdominal Pain  Hepatomegally, Jaundice  Pleural Effusions, infiltrates  Stiff Neck secondary to aseptic meningitis

 Children with unexplained fever for more than 5 days associated with 2-3 of the principle clinical features  More common in young infants  May be supported by laboratory evidence of systemic inflammation

 Viral Infections (Measles, adenovirus, enterovirus, EBV)  Scarlet Fever  Staphylococcal scalded skin syndrome  Bacterial cervical lymphadenitis  Rocky Mountain Spotted Fever  Leptospirosis

 Moderate to high WBC count with left shift  Anemia  Elevated ESR, CRP  Thrombocytosis  Mild-Moderate elevation in transaminases  Sterile Pyuria

 Toxic Shock Syndrome  Drug Hypersensitivity  Steven-Johnson syndrome  Juvenile idiopathic arthritis  Juvenile Polyarteritis Nodosa  Mercury hypersensitivity reaction

 Echocardiogram is critical for the evaluation of all patients suspected of having KD.  Baseline echo during acute stage to r/o coronary artery aneurysms and evidence of myocarditis, valvulitis, or pericardial effusion  Echo should be repeated in 2 nd -3 rd week of illness and again 1 month after (or once all lab values normalize)  Prior to treatment 20-25% of patients had Cardiac involvement with mortality rate 0.1-2%  With IVIG risk reduced to 5%

 Standard therapy is IVIG with Asprin  During the acute phase of illness  IVIG (2gm/kg) and  Asprin mg/kg /day  Continue high dose asprin until day 14 of illness if still afebrile  Continue asprin 3-5mg/kg/day until no evidence of coronary changes by 6-8 weeks

 ~10% fail to respond to initial IVIG therapy (persistence of fever after 36hrs)  Retreatment with IVIG at same dose recommended

 3 rd dose IVIG  Pulse Steroids (Methylprednisolone mg/kg for 2-3 hours qday x3days)  Infliximab (monoclonal ab against tumor necrosis factor)  Cyclophosphamide  Methotrexate

 MI caused by thrombotis occlusion of abnormal coronary artery Is principle cause of death  Usually occurs within first year  Children at high risk need frequent ECHO evaluations  Small solitary aneurysms-long term asprin therapy  Giant aneurysms or multiple complex aneurysms-long term antiplatelet therapy and anticoagulation  Primary surgical management is coronary artery bypass graft