Causes of cryptopyrroluria

Where does cryptopyrroluria come from? What are the causes of this metabolic disorder?

Acquired and inherited form

Until recently, pyrroluria was regarded as generally genetic and familial. According to Kamsteeg, cryptopyrrole is produced as a waste product of the heme metabolism by a “minor inheritance error” of two enzymes (uroporphyrinogen-III-cosynthase, uroporphyrinogen decarboxylase) involved in the synthesis of the heme, leading to an increased formation of coproporphyrinogen I.

However, he mentions also an acquired form of KPU in acute poisoning by heavy metals (lead, mercury).

Recent research by Kuklinkski revealed a positive correlation between pathologically elevated pyrrole values, a disturbed mitochondrial function, and resulting nitrosative stress by increased nitric oxide (NO) formation in the organism.

Kuklinski explains the observed relationship by inhibiting iron-containing enzymes of the body, which are particularly sensitive to increased NO concentrations. These include, among others, mitochondria enzymes and the hepatic enzyme 7-alpha-hydroxylase, whose inhibition disrupts bile acid formation from cholesterol and leads to the increased occurrence of immature pyrrole rings in the urine.

Once acquired, this form of mitochondropathy can be inherited according to the known rules.

External factors influence the metabolic disorder

Some external factors may lead a latent pyrroluria to be acute or lead to the strengthening or the frequent occurrence of symptoms. Among other things, the impairment of the cytochrome P450 enzyme system plays a role, which is of crucial importance for the detoxification function of the liver.


  • Stress of any kind, including physical, e.g. through infections, injuries, surgeries, sunlight
  • Medication and other chemical stress such as birth control pill, hormone replacement therapies, psychopharmaceuticals, toxins of the living space, dietary supplements, heavy metals – Kamsteeg advises generally to be careful with porphyrinogenic drugs and additional copper loads (e.g., from the spiral)
  • Alcohol, drugs
  • Malnutrition (e.g. at intolerances), which leads to bowel dysfunctions and dysbacteria. In this way harmful substances arise in the intestine and apply a negatively affect for the immune system.

Heavy metals and KPU

KPU and, for example, amalgam stress are mutually reinforcing and there is an above-average number of KPU disturbances in the group of people who are particularly amalgamated and vice versa. This is related to the limited ability to detoxify and the zinc deficiency of the KPU sufferer, which causes the mercury to easily accumulate in the body (zinc is an antagonist of mercury). Mercury poisoning, in turn, creates stress and strengthens existing vitamin and mineral deficiencies. A clarification and, if necessary, a parallel treatment of the metabolic disorder and heavy metal loading are therefore recommended.