Table 3. Plasma nitisinone levels (μmol/L).

Patient Number	Sample 1	Sample 2
2	2.07	2.08
101	1.73	1.55
103	2.18	2.91
106	2.10	2.23
107	2.70	3.02
110	2.93	2.53
112	2.57	2.50
113	2.43	1.97
118	1.41	1.34
120	1.62	1.72
122	2.70	2.30
123	2.14	1.47
126	2.15	1.99
128	3.57	3.05
129	1.32	<0.07
132	2.21	2.52
135	2.03	1.87
136	2.51	2.74
137	<0.07	1.60
124	<0.07
102	<0.07

Plasma nitisinone concentrations were measured on two separate admissions in 19 nitisinone-treated patients and in 2 untreated control patients (#124 and #102). Patient #129 had the second sample measured when removed from nitisinone; patient #137 had the first sample assayed prior to starting nitisinone.