31 KB144 KB31 KB200520252005Krnjak, LjubaSkitek, Milanštevilka:2letnik:566 stranistr. 125-130ISSN:0014-8229COBISSID:1790065URN:URN:NBN:SI:doc-XMUDSCPWslSlovensko farmacevtsko društvoFarmacevtski vestniklaboratorijska diagnostikaporfirijaporfiriniPomembnost porfirinov v laboratorijski diagnostiki|Porphyrins are cyclic tetrapyrroles which can be considered as derivatives of the parent tetrapyrrole. They are synthesized in all mammalian cells. They excrete of the health human body in small quantities through excrement and urinous. In humans, there are three major porphyrinsČ uroporphyrin, coproporphyrin and protopotphyrin. Porphyrias are hereditary or acquired disorders of heme biosynthesis. For practical reasons, they are classified into erythropoietic or hepatic forms according to the primary site of enzyme deficiency. For the clinical manifestation of hepatic porphyrias secondary factors are usually of importance, e.g. drugs, hormones, nutrition, alcohol, certain halogenated hydrocarbon compounds, hepatic lesions. Acute hepatic porphyrias have to give rise to medical emergencies and require intensive care treatmentPorfirini so ciklični tetrapiroli, derivati osnovnega tetrapirol porfina. So vmesni presnovni produkti v biosintezi hema. Sintentizirajo se v vseh celicah sesalcev. Pri zdravem človeku se v manjših količinah izločajo iz organizma z blatom in urinom. Poznamo tri glavne vrste porfirinov: uroporfirin, koproporfirin in protoporfirin. Bolezni, ki se pojavljajo v zvezi z njimi, so dedne ali pridobljene motnje v biosintezi hema. Za boljše razumevanje jih razdelimo v eritropoetične ali hepatične oblike, glede na primarni položaj pomanjkanja encima. Za klinični dokaz hepatičnih porfirij so ponavadi pomembni sekundarni dejavniki kot: zdravila, hormoni, prehrana, alkohol, določene halogenske hidrokarbonske mešanice in poškodbe jeter. Pri akutnih hepatičnih porfirijah je nujno takojšnje diagnosticiranje in zdravljenjeTEXTznanstveno časopisjejournalsSlovenian National E-content AggregatorNational and University Library of SloveniaSlovensko farmacevtsko društvo