Suppl. Table 10. Emergency Treatment (First Day)

Suppl. Table 10. Emergency treatment (first day)

Dietary management / Pharmacotherapy
Disease / Patients
n / Patients with ET
n / Episodes
n / Carbohydrates
Mean; median [n]
(Q1;Q3)
[min;max] in %Recomm.* / Natural protein
Mean; median [n]
(Q1;Q3)
[min;max] in %WHO** / Total protein
Mean; median [n]
(Q1;Q3)
[min;max] in %WHO** / AAM
Mean; median [n]
(Q1;Q3)
[min;max] in g/kg/d / Calories
Mean; median [n]
(Q1;Q3)
[min;max] in %FAO*** / BZA (incl. bolus)
Mean; median [n]
(Q1;Q3)
[min;max] in mg/kg/d / CNT (incl. bolus)
Mean; median [n]
(Q1;Q3)
[min;max] in mg/kg/d / CBG
Mean; median [n]
(Q1;Q3)
[min;max] in mg/kg/d / ARG-HCl (incl. bolus)
Mean; median [n]
(Q1;Q3)
[min;max] in mg/kg/d
MMA-Cbl- / 117 / 26 / 54 / 147; 151 [49]
(120;156)n.r.
[56;311] / 27; 0 [51]
(0;44)
[0;140] / 61; 52 [51]
(0;93)
[0;237] / 0.3; 0.27 [49]
(0;0.41)
[0;1.05] / 75; 65 [49]
(54;87)
[17;157] / 265; 265 [2]
(222;---)
[222;308] / 179; 161 [50]
(96;249)
[8;683] / 8; 8 [1]
(8;8)
[8;8] / n.r.
MMA-Cbl+ / 47 / 4 / 6 / 92; 94 [4]
(74;109)
[73;109] / 13; 0 [3]
(0;---)
[0;39] / 29; 0 [3]
(0;---)
[0;87] / 0.17; 0 [3]
(0;---)
[0;0.5] / 38; 38 [1]
(38;38)
[38;38] / n.r. / 114; 100 [3]
(83;---)
[83;158] / n.r. / n.r.
PA / 144 / 25 / 111 / 170; 166 [108]
(112;207)
[52;357] / 50; 35 [104]
(27;76)
[0;141] / 92; 91 [105]
(64;122)
[0;219] / 0.4; 0.35 [102]
(0.22;0.57)
[0;1.3] / 96; 98 [109]
(69;117)
[10;198] / 191; 153 [21]
(102;256)
[4;500] / 127; 101 [108]
(98;145)
[9;471] / 117; 118 [3]
(115;---)
[115;119] / 139; 126 [60]
(98;201)
[40;217]
IVA / 52 / 8 / 11 / 178; 156 [9]
(133;230)
[114;311] / 35; 0 [8]
(0;91)
[0;132] / 61; 0 [8]
(0;133)
[0;220] / 0.29; 0 [8]
(0;0.75)
[0;1.3] / 105; 73 [9]
(56;148)
[49;257] / n.r. / 146; 143 [10]
(92;200)
[25;282] / n.r. / n.r.
GA1 / 176 / 29 / 89 / 180; 133 [61]
(128;156)
[85;2182] / 27; 0 [79]
(0;41)
[0;178] / 105; 107 [79]
(88;145)
[0;270] / 0.84; 0.9 [75]
(0.8;1.1)
[0;1.5] / 101; 93 [69]
(75;126)
[29;211] / n.r. / 150; 163 [75]
(102;190)
[2;248] / n.r. / 149; 149 [1]
(149;149)
[149;149]
Total / 452 / 92 / 271 / 167; 149 [231]
(118;180)
[52;2182] / 37; 28 [245]
(0;62)
[0;178] / 88; 92 [246]
(49;122)
[0;270] / 0.5; 0.5 [237]
(0.2;0.84)
[0;1.5] / 93; 88 [237]
(64;116)
[10;257] / 197; 222 [23]
(108;259)
[4;500] / 145; 113 [246]
(99;185)
[2;683] / 90; 116 [4]
(35;118)
[8;119] / 139; 127 [61]
(98;199)
[40;217]

*According to recommended carbohydrate management for emergency treatment of GA1 patients (Kölker et al. 2011). ---, quartile could not be calculated due to small group size or distribution; **according to WHO 2007; ***according to FAO (2001); for adults a physical activity level (PAL) of 1.76 according to table 5.1 in FAO (2001) was taken, which is equivalent to the mean PAL of an active or moderately active life style; this life style is in between the extreme lifestyles “sedentary or light activity lifestyle” and “vigorous and vigorously active lifestyle”; AAM, amino acid mixture; ARG-HCl, arginine hydrochloride; BZA, sodium benzoate; CNT, carnitine; ET, emergency treatment; n.r., not reported; Recomm., guideline recommendation.