Porphyria Educational Services

PORPHYRIA EDUCATIONAL SERVICES BULLETIN
Vol. 2 No. 1                                            January 2, 2000
FOCUS:  Delta-ALA Urine test.

HAPPY NEW YEAR EVERYONE !

The Delta-ALA urine  test is a test that measures the
amount of delta-ALA in urine. It is one of the mmore basic porphyria tests.

Delta-ALA means delta-aminolevulinic acid.  It is a basic component of the
heme biopathway.

A 24-hour urine sample collection of urine is needed.
An order for the test must be given by your physician.
The physician will instruct you, if necessary, to discontinue drugs
that will most likely compromise the results of the test.
Also check with your physician that the correct preservative
is being used in the collection jug.  There are several
porphyrin urine tests and each has its own specified preservative.

A few things to remember about the tests is that light, air,
and heat will effect the outcome of the test because these factors will
lower the porphyrin count in the collection.

On day 1, urinate into the toilet upon arising in the morning.

Collect all subsequent urine in the special plastic "hat" for the next
24-hours.

On day 2, urinate and pour all of the ujrine into the container in the
morning upon arising.

Cap the container.
Keep it in the refrigerator or a cool place [36 to 40 degrees F.]
during the entire collection period.
Label the container with your name, the date, the time of completion,
and return it as instructed by yopur physician.

Deliver it to the laboratory or your physician as soon as possible
upon completion of the test.  Be sure that it is properly stored in
refrigeration and not left on the counter in the light and heat.

Avoid exposure of the urine to direct light.  The best results for this
test happen when the urine is voided in complete darkness and
poure directly into the collection jug in the dark or very dim
indirect light.

This test is useful in detecting specific liver abnormalities and is a good
indicator test for patients suspected of having porphyria.

Delta-ALA is a chemical produced from amino acids in the liver.
It is the basic "building block" for the synthesis of porphyrins.
The most important function of porphyrins are as components
of heme.  Heme is the major building block of hemoglobin.
Oxygen binds to the iron in the heme molecules.

Various kinds of porphyrins exist with the same basic structure
 but with slightly different chemical "side-chains".
The major biochemical pathway is delta-ALA --> PBG --> uroporphyrin -->
coproporphyrin --> protoporphyrin --> heme.
Each step in the pathway requires a specific enzyme.
 If any of the enzymes is deficient, a type of porphyria results.

Normal values
1 to 7 mg per 24-hours

Before starting this test be sure that you are not taking any drugs which
may compromise the test results.

Drugs that may increase test measurements include penicillin,
barbiturates, oral contraceptives, and griseofulvin.

Increased abnormal  levels of urinary delta-ALA may indicate
many types of porphyria.
In addition increased levels may indicate lead poisoning.

Decreased levels may occur with chronic liver disease.