Acid mucopolysaccharides

• On day 1, urinate into the toilet upon arising in the morning.
• Collect all subsequent urine (in a special container) for the next 24-hours.
• On day 2, urinate into the container in the morning upon arising.
• Cap the container. Keep it in the refrigerator or a cool place during the collection period. Label the container with your name, the date, the time of completion, and return it as instructed. Deliver it to the laboratory or your health care provider as soon as possible upon completion.

How to Prepare:
No special preparation is needed. Drink a normal volume of fluids, and consume a normal diet.

How it Feels:
The test involves only normal urination, and there is no discomfort.

Risks:
There are no risks (except for the chance of inaccurate results if all of the urine is not collected during the 24-hour period).

Why this Test is Performed:
This test is performed to detect a rare disorder of excess acid mucopolysaccharides accumulation (especially dermatan sulfate and heparan sulfate) in the tissues. The severe form of this disorder can lead to Hurler's syndrome. Typically this test is only ordered for infants where there is a family history of mucopolysaccharidosis .

Normal Values:
Normal acid mucopolysaccharide values vary with age, and are commonly reported as a ratio of glucuronic acid (mg) over creatinine (g).

• age 2: 8 to 30
• age 4: 7 to 27
• age 6: 6 to 24
• age 8: 4 to 22
• age 10: 2 to 18
• age 12: 0 to 15
• age 14: 0 to 12

Note: mg = milligrams; g = grams

Abnormal Results:
Abnormally high levels of acid mucopolysaccharides reliably indicates mucopolysaccharidosis , and further detailed studies can identify the enzyme which is defective.

Cost:
Information not available.

Special Considerations:

• if the test was performed on an infant, remove all remaining adhesive from the collection device with warm soap and water.