Table 1.

Patient lab values at initial presentation (I), peak of illness (P), and most recent (R). Age at initial, peak, and most recent lab values provided in parentheses.

	Phase	Bicarbonate	Lactate	Ammonia	CK	AST	ALT	PT/INR	PTT	Conjugated bilirubin
Ref. range		18–29 mmol/L	0.5–1.6 mmol/L	9–33 umol/L	60–305 U/L	20–64 U/L Patients 1–4 35–140 U/L Patients 5–6	12–42 U/L Patients 1–4 6–50 U/L Patients 5–6	10.9–13.4s / 0.8–1.2	22–36s	0–0.3 mg/dL
Patient 1	I (3m)	11	12.7	11	60	151	87	16/1.32	34.7	0.2
	P (3m)		18.5	38	1383 (15m)	376	165	21.4/1.86	46.6	0.1
	R (41m)		3.0	<9	682	98	40	12.6/1.04	27.9	0.6
Patient 2	I (2m)	24	2.5	33		91 (1m)	48 (1m)	15.8/1.2	38	1.5 (1m)
	P (6m)		9.7	83	142 (10m)	1322	918	16.2/1.6	38 (2m)	1.5 (1m)
	R (10m)		4.8	14	142	161	121	14.3/1.2	33.3	0.8 (10m)
Patient 3	I (2m)	3.6	10.7	<9	711	190	324	19.3/1.79	60.6	1.3
	P (3m)		19.3 (9w)	393	4099	2615	503	43/4.26	192.4	20.9
	R (3y)		1.4 (19m)	17 (13m)		67	67	12.6/1.1	33	0
Patient 4	I (2w)	22 (3w)	10.8	51		79	64	16.5/1.3 (3w)	40.6 (3w)	<0.1
	P (3m)		24.8	393	158 (2m)	367	65 (5d)	38.4/3.77	199.2	0.7
	R (N/A)
Patient 5	I (<1w)	7	14.8	165	221	89	24	15.1 / 1.2 (1m)	32.0 (1m)	0.0
	P (2m)		>19.9	344 (<1w)		1228	309	62.1 / 7.2	53.5	8.3
	R (N/A)
Patient 6	I (<1w)	16	5.7	17		64	24	15.7 / 1.2	35.7	0.0
	P (3m)		41.9	90 (2m)	73 (2m)	540	347	19.7 / 1.6	34.8 (2m)	8.5
	R (15m)		5.5			117	72	15.4 / 1.2		0.0

m: months, w: weeks. Reference ranges for PT/INR and PTT are approximate, as normal reference values vary in infancy.