代謝救急 - nagano-picu.jp

1 PICU consult PICU not doing well 500 1 first line second line CBC, BGA anion gap , , , , , , , 3- , , CK, , MRI , , , , , , , , -20 C , 3-10ml, -20 C , -20 C Guthrie 4 , -20 C 1ml, DNA , 4 C , 6-10mg/kg/min=10g/kg/day 60-100kcal/kg/day 15-20g/kg/day 2g/kg/day CV 10% First line glu, NH3, lac, keton AG HCO3 / / Na THAM.

2 Tris (hydroxymethyl) aminomethane Na 200 g/dL 120 mol/L 25-30mmHg N- key critical sample

3 C ACTH, cortisol, fT4, TSH, GH, IGF-1 20%Tz 1ml/kg bolus GIR 6-8mg/kg/min 4-6mmol/L B1 / L/P , 3- / 3-OHB/AA 1-2g/kg/day VPA , , , , , 1 10kg ; F Vit. B1 ; Vit. C ; Vit. H ; Vit. E ; CoQ ; 100mg 1g 5mg 100mg 50mg 300mg / 2-3 / 2-3 / 2-3 / 2-3 / 2-3 / 2-3 1 mmol/L = 9 mg/dL 1 mg/dL = mmol/L 1 mol/L = g/dL 1 g/dL = mol/L 1 mmol/L = 18 mg/dL 10 mg/dL = mmol/L ; 23 , ; 2006;69:1574-8 ; , 2006 Chinnery P, et al.

4 Treatment for mitochondrial disorders. Cochrane Database Syst Rev 2006; 97: 292-6 Cagnon L, Braissant O. Hyperammonemia-induced toxicity for the developing central nervous system. Brain Res Rev 2007; 56:183-97