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Are Children With Kawasaki Disease and Prolonged Fever at Risk for Macrophage Activation Syndrome?
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Tables
- TABLE 1.
Changes in Laboratory Values During Course of Illness
Day At Presentation Onset MAS 48 Hours Later At Discharge 2 Months Later White blood count (4–10 109/L) 24.1 × 109/L (103/μl) 10.2 × 109/L (103/μl) 19.7 × 109/L (103/μl) 6.8 × 109/L (103/μl) 12.9 × 109/L (103/μl) Polys (2.00–7.50 109/L) 20.5 × 109/L (103/μl) 2.68 × 109/L (103/μl) 14.43 × 109/L (103/μl) 5.04 × 109/L (103/μl) 11.1× 109/L (103/μl) Lymph (1.5–7.0 109/L) 0.96 × 109/L (103/μl) 0.20 × 109/L (103/μl) 0.93 × 109/L (103/μl) 1.02 × 109/L (103/μl) 1.3 × 109/L (103/μl) Hemoglobin (120–160 g/L) 113 g/L (11.3 g/dL) 85 g/L (8.5 g/dL) 93 g/L (9.3 g/dL) 101 g/L (10.1 g/dL) 136 g/L (13.6 g/dL) PLT (150–400 109/L) 623 × 109/L (103/μl) 104 × 109/L (103/μl) 133 × 109/L (103/μl) 464 × 109/L (103/μl) 293 × 1109/L (103/μl) Erythrocyte sedimentation rate (1–10 mm/hour) 118 mm/hour 70 mm/hour 53 mm/hour 36 mm/hour 1 mm/hour AST (0–36 units/L) 105 units/L 2083 units/L 1339 units/L 96 units/L 26 units/L ALT (0–40 units/L) 12 units/L 903 units/L 951 units/L 79 units/L 31 units/L ALK Phos (180–475 units/L) 121 units/L 339 units/L 247 units/L 111 units/L 51 units/L Conj. bili (0–2 μmol/L) 0 μmol/L (0 mg/dL) 10 μmol/L (0.58 mg/dL) 1 μmol/L (0.06 mg/dL) 0 μmol/L (0 mg/dL) N/A Unconj. bili (0–17 μmol/L) 0 μmol/L (0 mg/dL) 0 μmol/L (0 mg/dL) 0 μmol/L (0 mg/dL) 1 μmol/L (0.06 mg/dL) N/A LDH (432–700 units/L) N/A 29691 units/L 5062 units/L N/A N/A Ferritin (22.0–400 μg/L) N/A 1156 μg/L 1256 μg/L N/A N/A Fibrinogin (1.60–4.00 g/L) N/A 0.47 g/L 0.76 g/L 4.41 g/L N/A D-Dimer (0–499 ng/L) N/A 5420 ng/L 1970 ng/L 2160 ng/L 429 ng/L Triglyceride (0.40–1.30 mmol/L) N/A 2.52 mmol/L (223 mg/dL) 2.80 mmol/L (248 mg/dL) N/A N/A Albumin (33–58 g/L) N/A 28 g/L (2.8 g/dL) 23 g/L (2.3 g/dL) N/A N/A Laboratory values with normal SI unit values in brackets are shown. N/A indicates not available; ALT, alanine aminotransferase; AST, aspartate aminotransferase; ALK phos, alkaline phosphatase; PLT, platelet; conj. bili, conjugated bilirubin; unconj. bili, unconjugated bilirubin.
- TABLE 2.
Comparison of Patients With KD Who Developed MAS
Patient 1 (Ohga et al9) 2 (Kaneko et al8) 3 (Al-Eid et al7) 4 (Present Case) Age 32 mos 12 mos 6 y 9 y Gender Male Female Male Male Days to MAS 21 24 20 23 Number of courses of IVIG 3 5 2 2 Corticosteroid None None 1 course of IVMP 2 courses of IVMP followed by oral therapy Laboratory evidence of MAS Cytopenia Yes Yes Yes Yes Hyperferritinemia Yes Yes Yes Yes Elevated LDH Yes N/A Yes Yes Elevated acyl-transferases N/A Yes Yes Yes Hypofibrinogenemia No N/A Yes Yes Hypertriglyceridemia N/A N/A Yes Yes Evidence of MAS by biopsy Bone marrow Bone marrow Liver Bone marrow IVMP indicates intravenous methylprednisolone; N/A, not available.
Are Children With Kawasaki Disease and Prolonged Fever at Risk for Macrophage Activation Syndrome?
