Uroporphyrin III

Optimal Result: 0.7 - 7.4 mcg/g creat.

Urinalysis

From Healthmatters.io Team

Lab Results Explained and Tracked

What is the Porphyrins test?

Porphyrins are a group of compounds defined by their chemical structure. These compounds are by-products of heme synthesis and are normally present at low levels in blood and other body fluids. Porphyrin tests measure porphyrins and their precursors in urine, blood, and/or stool.

Heme is an iron-containing pigment that is a component of hemoglobin and a number of other proteins. It consists of an organic portion (protoporphyrin) bound to an iron atom. The synthesis of heme is a step-by-step process that requires the sequential action of eight different enzymes. If there is a deficiency in one of these enzymes, the process is impeded and intermediate porphyrins such as uroporphyrin, coproporphyrin, and protoporphyrin build up in the body’s fluids and tissues. The precursors that accumulate depend on which enzyme is deficient, and they can exert toxic effects.

Function of Porphyrin tests:

Porphyrin tests are used to help diagnose and monitor a group of disorders called porphyrias. There are seven types of porphyria, and each one is associated with a different enzyme deficiency. Most porphyrias are inherited, the result of a gene change (variant). Porphyrias may be classified according to the signs and symptoms of the disease as neurological, cutaneous, or both.

The porphyrias that cause neurological symptoms present with acute attacks lasting days or weeks. Signs and symptoms during the attack include abdominal pain, constipation, confusion, hallucinations, and/or seizures.

There are four neurologic porphyrias:

- acute intermittent porphyria (AIP),

- variegate porphyria (VP),

- hereditary coproporphyria (HCP),

- and the very rare ALA dehydratase deficiency porphyria (ADP).

Some cases of VP and HCP may also have skin-related symptoms.

The cutaneous porphyrias are associated with photosensitivity that causes redness, swelling, a burning sensation, blistering, skin thickening, hyperpigmentation, and/or scarring.

There are three cutaneous porphyrias:

- porphyria cutanea tarda (PCT),

- erythropoietic protoporphyria (EPP),

- and congenital erythropoietic porphyria (CEP).

Experts have not yet reached agreement on whether all porphyrias are inherited. Porphyria cutanea tarda (PCT) may result from genetic or environmental factors such as exposure to certain chemicals or significant liver damage. This type of PCT is termed “acquired” or “sporadic.” In people with acquired PCT, the disorder usually develops after age 30 and onset in childhood is rare.

To diagnose porphyrias, laboratories measure porphyrins and their precursors in urine, blood, and/or stool. Testing may include measurement of one or more of the following:

- Porphobilinogen (PBG), a porphyrin precursor, in urine

- Delta-aminolevulinic acid (ALA), another porphyrin precursor, in urine

- Porphyrins (uroporphyrin, coproporphyrin, and protoporphyrin) in urine, blood, or stool

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What does it mean if your Uroporphyrin III result is too high?

The following table summarizes the patterns of results that are typical for each type of porphyria:

**Pattern of the Different Porphyrias**
Disease	Uro	Hepta	Hexa	Penta	Copro I	Copro III
+ = increased; ++ = strongly increased; n = normal; v = varies.
Values from: Doss M. Porphyrinstoffwechsel. In: Greiling H, Gressner AM (Hrsg). Lehrbuch der Klinischen Chemie und Pathobiochemie. Dritte Auflage. Stuttgart, Germany: Schattauer Verlag.
Values marked with * and ** respectively, are derived from:
Hindmarsh JT, Oliveras L, Greenway DC. Biochemical differentiation of the porphyrias. Clin Biochem.* 1999 Nov; 32(8):609-619.
*Elder GH, Smith SG, Smyth SJ. Laboratory investigation of the porphyrias. Ann Clin Biochem.* 1990 Sep; 27(Pt 5):395-412.
Porphyria cutanea tarda (PCT)	++	++	+	+	+*	+*
Acute intermittent porphyria (AIP)	++	+	+	++	+*	++*
Porphyria variegata (VP)	++	+	+	++	+*	++*
Hereditary coproporphyria (CP)	+	+	+	++	n*	++*
Protoporphyria	v	n	n	v	+*	v
Morbus Gunther	++	+	+	+	++**
Porphobilinogen synthase deficiency	+	+	+	++		++

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Uroporphyrin III

All Your Lab Results. One Simple Dashboard.

What does it mean if your Uroporphyrin III result is too high?

All Your Lab Results. One Simple Dashboard.

Import, Track, and Share Your Lab Results Easily

Import, Track, and Share Your Lab Results

Article Review & Sources

Bring All Your Lab Results Together — In One Place

Gather Your Lab History — and Finally Make Sense of It

Finally, Your Lab Results Organized and Clear

Advanced Plan

Unlimited Account

Pro Monthly

What's included in a Healthmatters membership

Import Lab Results from Any Source

See Your Health Timeline

Understand What Your Results Mean

Enter Your Lab Reports Yourself — Always Free Enter Your Lab Reports — Always Free

Visualize Your Results

Export Your Complete Lab History in a Single File Export Your Complete Lab History

Comprehensive Data Entry Service for Your Reports Data Entry Service for Your Reports

Securely Share With Anyone You Trust

Let Your Lab Results Tell the Full Story

Once your results are in one place, see the bigger picture — track trends over time, compare data side by side, export your full history, and share securely with anyone you trust.

Bring all your results together to compare, track progress, export your history, and share securely.

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All Your Lab Results.
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