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Table 1 Effect of low dose nitisinone on alkaptonuria-related variables

From: Preventive use of nitisinone in alkaptonuria

  N patients Duration Dose Δ uHGA (%) Mean pTyrosine on treatment (µmol/l)
Suwannarat et al. [15] 9 3–4 mo 1.05 mg bid − 94 755 ± 167
603 ± 114*
Introne et al. [8] 40 4 mo–3 yrs 2 mg od − 95 670–826
Sloboda et al. [16] 3 3–5 yrs 0.2 mg od − 90 305–416
Ranganath et al. [14] 8 4 wks 1 mg od − 89.4 653 (450–806)
8 2 mg od − 94.9 715 (506–865)
8 4 mg od − 98.3 803 (657–1115)
8 8 mg od − 99 813 (523–927)
  1. *After initiation of protein restriction
  2. Bid, twice daily; mo, months; N, number; od, once daily; uHGA, urinary homogentisic acid excretion