Matches in SemOpenAlex for { <https://semopenalex.org/work/W2897584530> ?p ?o ?g. }
- W2897584530 endingPage "686" @default.
- W2897584530 startingPage "673" @default.
- W2897584530 abstract "Porphyrias are disorders caused by defects in the biosynthetic pathway of heme. Their manifestations can be divided into three distinct syndromes, each attributable to the accumulation of three distinct classes of molecules. The acute neurovisceral syndrome is caused by the accumulation of the neurotoxic porphyrin precursors, delta aminolevulinic acid, and porphobilinogen; the syndrome of immediate painful photosensitivity is caused by the lipid-soluble protoporphyrin IX and, the syndrome of delayed blistering photosensitivity, caused by the water-soluble porphyrins, uroporphyrin, and coproporphyrin. Porphyrias can manifest with one, or with a combination, of these syndromes, depending on whether one or more types of molecules are being accumulated. Iron plays a significant role in some of these conditions, as evidenced by improvements in both clinical manifestations and laboratory parameters, following iron depletion in porphyria cutanea tarda, or iron administration in some cases of X-linked erythropoietic protoporphyria. While the pathophysiology of a specific type of porphyrias, the protoporphyrias, appears to favor the administration of zinc, results so far have been conflicting, necessitating further studies in order to assess its potential benefit. The pathways involved in each disease, as well as insights into their pathobiological processes are presented, with an emphasis on the development of photosensitivity reactions." @default.
- W2897584530 created "2018-10-26" @default.
- W2897584530 creator A5009484606 @default.
- W2897584530 creator A5014889026 @default.
- W2897584530 creator A5036079806 @default.
- W2897584530 creator A5077907936 @default.
- W2897584530 date "2018-10-23" @default.
- W2897584530 modified "2023-09-27" @default.
- W2897584530 title "Porphyrias and photosensitivity: pathophysiology for the clinician" @default.
- W2897584530 cites W1522917098 @default.
- W2897584530 cites W1548130696 @default.
- W2897584530 cites W1556837744 @default.
- W2897584530 cites W157954461 @default.
- W2897584530 cites W1676842176 @default.
- W2897584530 cites W170873858 @default.
- W2897584530 cites W1709418723 @default.
- W2897584530 cites W1714222561 @default.
- W2897584530 cites W1843848517 @default.
- W2897584530 cites W1965180761 @default.
- W2897584530 cites W1965474638 @default.
- W2897584530 cites W1967594483 @default.
- W2897584530 cites W1967839501 @default.
- W2897584530 cites W1974125090 @default.
- W2897584530 cites W1974135984 @default.
- W2897584530 cites W1976436613 @default.
- W2897584530 cites W1977104952 @default.
- W2897584530 cites W1977870833 @default.
- W2897584530 cites W1983912885 @default.
- W2897584530 cites W1984867950 @default.
- W2897584530 cites W1986983408 @default.
- W2897584530 cites W1991759638 @default.
- W2897584530 cites W1994999960 @default.
- W2897584530 cites W1997494859 @default.
- W2897584530 cites W1998100430 @default.
- W2897584530 cites W2001932807 @default.
- W2897584530 cites W2004180297 @default.
- W2897584530 cites W2009581025 @default.
- W2897584530 cites W2022780552 @default.
- W2897584530 cites W2023070199 @default.
- W2897584530 cites W2027173801 @default.
- W2897584530 cites W2030028187 @default.
- W2897584530 cites W2036370236 @default.
- W2897584530 cites W2037539395 @default.
- W2897584530 cites W2039686970 @default.
- W2897584530 cites W2044487970 @default.
- W2897584530 cites W2045586767 @default.
- W2897584530 cites W2052210161 @default.
- W2897584530 cites W2061655936 @default.
- W2897584530 cites W2068183958 @default.
- W2897584530 cites W2069536180 @default.
- W2897584530 cites W2070116787 @default.
- W2897584530 cites W2071866838 @default.
- W2897584530 cites W2076425914 @default.
- W2897584530 cites W2078608203 @default.
- W2897584530 cites W2088575848 @default.
- W2897584530 cites W2093804948 @default.
- W2897584530 cites W2096035745 @default.
- W2897584530 cites W2097694649 @default.
- W2897584530 cites W2100073287 @default.
- W2897584530 cites W2101143075 @default.
- W2897584530 cites W2107857612 @default.
- W2897584530 cites W2111482157 @default.
- W2897584530 cites W2114852835 @default.
- W2897584530 cites W2129881842 @default.
- W2897584530 cites W2142545562 @default.
- W2897584530 cites W2143603770 @default.
- W2897584530 cites W2145613121 @default.
- W2897584530 cites W2167982364 @default.
- W2897584530 cites W2169059885 @default.
- W2897584530 cites W2180660046 @default.
- W2897584530 cites W2293458435 @default.
- W2897584530 cites W2344483344 @default.
- W2897584530 cites W2403788404 @default.
- W2897584530 cites W2414248059 @default.
- W2897584530 cites W2507658028 @default.
- W2897584530 cites W2530814891 @default.
- W2897584530 cites W2562225798 @default.
- W2897584530 cites W2587614559 @default.
- W2897584530 cites W2593022947 @default.
- W2897584530 cites W2619151721 @default.
- W2897584530 cites W2751374343 @default.
- W2897584530 cites W2753442568 @default.
- W2897584530 cites W2766944858 @default.
- W2897584530 cites W2790744750 @default.
- W2897584530 cites W3082816727 @default.
- W2897584530 cites W3156803523 @default.
- W2897584530 cites W3189899230 @default.
- W2897584530 cites W4232732219 @default.
- W2897584530 cites W4239844158 @default.
- W2897584530 cites W4253599714 @default.
- W2897584530 cites W4322699750 @default.
- W2897584530 cites W933215294 @default.
- W2897584530 doi "https://doi.org/10.1080/00325481.2018.1533380" @default.
- W2897584530 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/30296862" @default.
- W2897584530 hasPublicationYear "2018" @default.
- W2897584530 type Work @default.
- W2897584530 sameAs 2897584530 @default.
- W2897584530 citedByCount "6" @default.