From: High blood pressure, a red flag for the neonatal manifestation of urea cycle disorders
Initial blood gases | Symptoms# | NH4+ | ||||||||||||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
No | Sex | Deficiency of | pH | Base excess | Lactate | CO2 | SBP | DBP | MAD | Onset | At obstetric unit | On admission at NICU | First test result | On admission at NICU | Peak NH4+ | Time until NH4+ < 150 μmol/l |
mmol/l | mmHg | Day of life | (μmol/l) | hours | ||||||||||||
1° | F | CPS1 | 7.42 | −2.3 | 6.0 | 76.7 | 86 | 52 | 69 | 3 | 7 | 1, 3, 6 | 4000 | 4420 | 4420 | 35 |
2 | M | CPS1 | 7.36 | −9.1 | 5.7 | 26.1 | 74 | 50 | 58 | 3 | n.d. | 1, 4 | 673 | 835 | 1100 | 21 |
3° | M | OTC | 7.52 | 2.9 | 2.6 | 29.6 | 84 | 53 | 63 | 9 | 4, 8 | 1, 4, 8 | 385 | 136 | 385 | 9 |
4° | M | OTC | 7.47 | −1.5 | 4.0 | 30.0 | 109 | 61 | 72 | 5 | 2, 4 | 2, 4 | 240 | 676 | 676 | 28 |
5 | M | OTC | 7.46 | −2.4 | 5.2 | 26.0 | 71 | 56 | 81 | 2 | 4 | 1, 4 | 286 | 3363 | 4050 | 28 |
6 | M | OTC | 7.17 | −4.1 | 3.9 | n.d. | 80 | 55 | 63 | 3 | 4, 5, 9 | 1, 4, 6, | 1000 | 1951 | 2213 | 30 |
7° | M | OTC | 7.36 | 1.4 | 2.5 | 50.5 | 110 | 84 | 98 | 4 | 1 | 1, 3 | 1494 | 3516 | 3516 | 34 |
8 | M | OTC | 7.48 | −4.1 | 2.9 | 21.5 | n.d. | n.d. | n.d. | 3 | 1 | 1, 6 | 750 | 1750 | 1750 | 34 |
9° | M | ASS1 | 7.55 | 0 | 3.7 | 25.8 | 121 | 83 | 94 | 4 | 1, 2 | 1, 4 | 1078 | 590 | 1078 | 12 |
10° | F | ASS1 | 7.39 | −3.8 | 7.2 | 33.2 | 95 | 64 | 74 | 3 | 1 | 1, 3 | 232 | 1430 | 1430 | 35 |
11° | M | ASS1 | 7.55 | −5.5 | 5.2 | 19.7 | 95 | 65 | 78 | 4 | 2,4 | 1, 4 | 735 | 1104 | 1104 | 14 |
12° | F | ASS1 | 7.55 | −2.3 | 4.9 | 20.4 | 111 | 70 | 91 | 4 | 2, 4, 5 | 1, 4, 6 | 484 | 1091 | 1091 | 33 |
13° | M | ASS1 | 7.48 | −3.4 | 2.7 | 24.3 | 92 | 61 | 71 | 3 | 1, 6 | 1, 6 | 235 | 1376 | 1645 | 24 |
14° | F | ASS1 | 7.45 | 0.4 | 3.5 | 34.7 | 110 | 88 | 95 | 4 | 1 | 1 | 1399 | 1734 | > 2000 | 15 |
15*†° | M | ASS1 | 7.34 | −7.6 | 5.9 | 32.0 | 85 | 61 | 71 | 7 | −/−* | 4 | 402 | n.d. | 402 | 4 |
16° | F | ASL | 7.36 | −4.9 | 5.8 | 35.0 | 110 | 84 | 89 | 3 | 1, 2, 3 | 1, 3, 6 | 1256 | 1082 | 1256 | 24 |
17° | M | ASL | 7.52 | 2.4 | n.d. | 29.5 | 108 | 88 | 94 | 6 | 1, 2, 4 | 1, 3, 5 | 1065 | 1100 | 1100 | 41 |