5-AMINOLAEVULINATE - urine
Specimen: Random urine.
Method: Spectrophotometry.
Reference Interval:
|
<3.8 mmol/mol creatinine
|
|
<40 mmol/L
|
Application: Diagnosis of acute neurological
porphyrias
during an acute episode, however urine porphobilinogen is the preferred test, except
for plumboporphyria. Limited value in the diagnosis of lead poisoning; the test is
less sensitive than blood lead and may be negative in organic lead poisoning (leaded
petrol).
Interpretation: Increased levels are found during acute attacks of acute intermittent
porphyria, hereditary coproporphyria, porphyria variegata and plumboporphyria. In
inorganic lead poisoning, porphobilinogen synthetase is inhibited, resulting in increased
levels of 5-aminolaevulinate. However, the test does not reliably detect lead exposure
in the distant past as, with time, lead is sequestered in bone. The test is also
unreliable in patients with very recent exposure to organic lead, eg petrol sniffing.
Increased levels are found in patients with type 1 tyrosinaemia.
Reference: Thunell S et al. J Clin Chem Clin Biochem 1987; 25: 5-14.