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Porphyria / p ɔːr ˈ f ɪr i ə / is a group of disorders in which substances called porphyrins build up in the body, adversely affecting the skin or nervous system. [1] The types that affect the nervous system are also known as acute porphyria, as symptoms are rapid in onset and short in duration. [1]
Acute intermittent porphyria (AIP) is a rare metabolic disorder affecting the production of heme resulting from a deficiency of the enzyme porphobilinogen deaminase. It is the most common of the acute porphyrias .
Psychomotor agitation is a symptom in various disorders and health conditions. It is characterized by unintentional and purposeless motions and restlessness, often but not always accompanied by emotional distress and is always an indicative for admission.
Emil Kraepelin (1856–1926). The Kraepelinian dichotomy is the division of the major endogenous psychoses into the disease concepts of dementia praecox, which was reformulated as schizophrenia by Eugen Bleuler by 1908, [1] [2] and manic-depressive psychosis, which has now been reconceived as bipolar disorder. [3]
He termed this description as Lethal Catatonia. Other reasons for the manifestation of Bell's Mania, points at the use of stimulant drugs in excessive amounts and also psychiatric diseases like depression or schizophrenia. [12] [36] In 1985, Bell's Mania was first mentioned in a definitive manner using the term Excited Delirium (EXD).
Porphyria cutanea tarda is the most common subtype of porphyria. [1] The disease is named because it is a porphyria that often presents with skin manifestations later in life. The disorder results from low levels of the enzyme responsible for the fifth step in heme production. Heme is a vital molecule for all of the body's organs.
Variegate porphyria is inherited in an autosomal dominant pattern, which means the defective gene is located on an autosome, and inheriting one copy of the defective gene from an affected parent is sufficient to cause the disorder. More severe cases result from inheriting two copies of the defective gene.
Erythropoietic protoporphyria (or commonly called EPP) is a form of porphyria, which varies in severity and can be very painful.It arises from a deficiency in the enzyme ferrochelatase, leading to abnormally high levels of protoporphyrin in the red blood cells (erythrocytes), plasma, skin, and liver. [2]
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