Table 1.

Biochemical findings in patients with LP.

Patient no.
Acute phaseNonacute phaseReference value
13451*12
Urine
 PBG, μmol/day39186662140<8.8
 ALA, μmol/day1540228854167122175<38
 ALA/PBG4012.713295.84.4<4.2
 URO,2 nmol/day9172423183694<36
 COP I, nmol/day8499262099321238<92
 COP III, nmol/day25150997591603936510375<275
Plasma
 Peak at 404/∼635 nm++++++
Erythrocytes
 ALAD, % of normal13102557060940 ± 1803
 Rate reactivation410117203.76.22 ± 0.5
  • 1 After treatment with mercapto succinate.

  • 2 URO, uroporphyrin; RBC, red blood cells.

  • 3 Normal enzyme activity expressed as μmol PBG · L RBC−1 · h−1.

  • 4 Multiple increase in ALAD activity in the presence of Zn2+ and cysteine.