Why does urine turn red in porphyria?
The terms porphyrin and porphyria are derived from the Greek word porphyrus, meaning purple. Urine from porphyria patients may be dark or reddish in color due to the presence of excess porphyrins and related substances, and may darken further after exposure to light.
What is Hepatoerythropoietic porphyria?
Hepatoerythropoietic porphyria (HEP) is an extremely rare genetic disorder characterized by deficiency of the enzyme, uroporphyrinogen decarboxylase. This deficiency is caused by mutations of both copies of a person’s UROD gene, which means that the disorder is inherited as an autosomal recessive trait.
What are Coproporphyrins?
Coproporphyrin I is a porphyrin metabolite arising from heme synthesis. Porphyrins are natural chemicals in the body that help form many important substances in the body. One of these is hemoglobin, the protein in red blood cells that carries oxygen in the blood.
Why was King George’s pee purple?
In recent years, though, it has become fashionable among historians to put his “madness” down to the physical, genetic blood disorder called porphyria. Its symptoms include aches and pains, as well as blue urine.
What is erythropoietic Protoporphyria?
Erythropoietic protoporphyria (EPP) is a rare inherited metabolic disorder caused by a deficiency of the enzyme ferrochelatase (FECH), which results from changes (mutations) in the FECH gene.
What is Coproporphyrin III?
Coproporphyrin III is a tetrapyrrole dead-end product from the spontaneous oxidation of the methylene bridges of coproporphynogen, arising from heme synthesis and secreted in feces and urine. Increased levels of coproporphyrins can indicate congenital erythropoietic porphyria or sideroblastic anaemia.
What is the definition of insanity?
The definition of insanity is doing the same thing over and over again and expecting a different result. These words are usually credited to the acclaimed genius Albert Einstein. What do you think?
What is the difference between insanity and flexibility?
A universal characteristic of insanity is inflexibly doing the same thing over and over while hoping for different results. Flexibility in the face of changing circumstances, by contrast, is a hallmark of mental health. By 1990 the saying was being attributed to Einstein.
What is the link between repetition and insanity?
The linkage between insanity and repetition has a long history. The controversial book “Degeneration” by Max Nordau was published in German in 1892 and translated into English by 1895. Nordau examined the works of a variety of artists and savagely attacked those that contained repetition which he believed evinced a mental defect in the creator.
What did Einstein say about insanity?
An oft-quoted bon mot (frequently attributed to Albert Einstein, Benjamin Franklin, or a number of other people who probably never said it) is that insanity may be defined as “doing the same thing over and over and expecting different results.” While the job of lexicographers might be easier if they were allowed to use witty sayings instead of