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1

Stölzel, Ulrich, Thomas Stauch, and Ilja Kubisch. "Porphyrien." Der Internist 62, no. 9 (June 29, 2021): 937–51. http://dx.doi.org/10.1007/s00108-021-01066-1.

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ZusammenfassungPorphyrien werden durch Enzymdefekte der Hämbiosynthese hervorgerufen und anhand spezifischer biochemischer Muster von Porphyrinen und deren Vorläufern in Urin, Stuhl und Blut diagnostiziert. Das jeweilige Muster der akkumulierten Porphyrine, Vorläufer und Derivate ist verbunden mit der klinischen Ausprägung, die abdominale, neurologische, psychiatrische, endokrine, kardiovaskuläre Symptome, Leberschaden und/oder Lichtempfindlichkeit der Haut umfassen kann. Klinisch werden akute und nichtakute Porphyrien unterschieden. Bei symptomatischen (klinisch aktiven), akuten hepatischen Porphyrien – hierzu gehören akute intermittierende Porphyrie, Porphyria variegata, hereditäre Koproporphyrie und Doss-Porphyrie – kommt es aufgrund einer Regulationsstörung zur Kumulation der Porphyrinvorläufer 5‑Aminolävulinsäure und Porphobilinogen. Bei den nichtakuten Formen – u. a. Porphyria cutanea tarda, erythropoetische und X‑chromosomale Protoporphyrie sowie kongenitale erythropoetische Porphyrie – führen akkumulierte Porphyrine zu Lichtempfindlichkeit (Fotodermatose) und mitunter auch zu schweren Leberschäden. Zur Therapie der Porphyrien stehen sowohl bewährte und sichere als auch innovative Optionen zur Verfügung.
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2

Trewick, S. A. "Flightlessness and phylogeny amongst endemic rails (Aves: Rallidae) of the New Zealand region." Philosophical Transactions of the Royal Society of London. Series B: Biological Sciences 352, no. 1352 (April 29, 1997): 429–46. http://dx.doi.org/10.1098/rstb.1997.0031.

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The phylogenetic relationships of a number of flightless and volant rails have been investigated using mtDNA sequence data. The third domain of the small ribosomal subunit (12S) has been sequenced for 22 taxa, and part of the 5′ end of the cytochrome– b gene has been sequenced for 12 taxa. Additional sequences were obtained from outgroup taxa, two species of jacana, sarus crane, spur–winged plover and kagu. Extinct rails were investigated using DNA extracted from subfossil bones, and in cases where fresh material could not be obtained from other extant taxa, feathers and museum skins were used as sources of DNA. Phylogenetic trees produced from these data have topologies that are, in general, consistent with data from DNA–DNA hybridization studies and recent interpretations based on morphology. Gallinula chloropus (moorhen) groups basally with Fulica (coots), Amaurornis (= Megacrex ) ineptus falls within the Gallirallus/Rallus group, and Gallinula (= Porphyrula ) martinica is basal to Porphyrio (swamphens) and should probably be placed in that genus. Subspecies of Porphyrio porphyrio are paraphyletic with respect to Porphyrio mantelli (takahe). The Northern Hemisphere Rallus aquaticus is basal to the south–western Pacific Rallus (or Gallirallus ) group. The flightless Rallus philippensis dieffenbachii is close to Rallus modestus and distinct from the volant Rallus philippensis , and is evidently a seperate species. Porzana (crakes) appears to be more closely associated with Porphyrio than Rallus . Deep relationships among the rails remain poorly resolved. Rhynochetos jubatus (kagu) is closer to the cranes than the rails in this analysis. Genetic distances between flightless rails and their volant counterparts varied considerably with observed 12S sequence distances, ranging from 0.3 % ( Porphyrio porphyrio melanotus and P. mantelli mantelli ) to 7.6 % ( Rallus modestus and Rallus philippensis ). This may be taken as an indication of the rapidity with which flightlessness can evolve, and of the persistence of flightless taxa. Genetic data supported the notion that flightless taxa were independently derived, sometimes from similar colonizing ancestors. The morphology of flightless rails is apparently frequently dominated by evolutionary parallelism although similarity of external appearance is not an indication of the extent of genetic divergence. In some cases, taxa that are genetically close are morphologically distinct from one another (e.g. Rallus (philippensis) dieffenbachii and R. modestus ), whilst some morphologically similar taxa are evidently independently derived (e.g. Porphyio mantelli hochstetteri and P. m. mantelli ).
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3

Bertolero, Albert, Sofia Rivaes, François Mougeot, Inés S. Sánchez-Barbudo, Karl B. Andree, and Carles Ibáñez. "Sexing and Ageing the Purple Swamphen Porphyrio porphyrio porphyrio by Plumage and Biometry." Ardeola 63, no. 2 (September 1, 2016): 261. http://dx.doi.org/10.13157/arla.63.2.2016.ra3.

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4

Lopes, Ricardo Jorge, Juan Antonio Gomez, Alessandro Andreotti, and Maura Andreoni. "Purple Swamphen or Gallinule (Porphyrio porphyrio) and Humans." Society & Animals 24, no. 6 (December 1, 2016): 574–95. http://dx.doi.org/10.1163/15685306-12341432.

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Our knowledge of the historical use of nonhuman animal species in captivity and subsequent human-induced changes in their distribution is poor in comparison to contemporary case studies. Here we assess the hypothesis that, in the case of one waterbird species, the purple swamphen or gallinule (Porphyrio porphyrio), we have neglected the high probability that people transported these birds within the Mediterranean, from Roman to recent times. In ancient iconographies, literary sources, and more recent records there is ample evidence for the use of this species in captivity, captive-breeding, and for trade during several historical periods, especially within the Mediterranean region. All this evidence supports the hypothesis that released or escaped birds might have hybridized with other populations living in the wild. This case study stresses the importance of taking into account past human activity when interpreting contemporary distributional patterns of species.
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5

Hindmarsh, J. Thomas, Linda Oliveras, and Donald C. Greenway. "Plasma Porphyrins in the Porphyrias." Clinical Chemistry 45, no. 7 (July 1, 1999): 1070–76. http://dx.doi.org/10.1093/clinchem/45.7.1070.

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Abstract Background: As an aid in the diagnosis and management of porphyria we have developed a method to fractionate and quantify plasma porphyrins and have evaluated its use in various porphyrias. Methods: We used HPLC with fluorometric detection to measure plasma concentrations of uroporphyrin I and III, heptacarboxyl III, hexacarboxyl III, pentacarboxyl III, and coproporphyrin I and III. We studied 245 healthy subjects, 32 patients with classical porphyria cutanea tarda (PCT), 12 patients with PCT of renal failure, 13 patients with renal failure, 8 patients with pseudoporphyria of renal failure, 3 patients with acute intermittent porphyria, 5 patients with variegate porphyria, 5 patients with hereditary coproporphyria, and 4 patients with erythropoietic protoporphyria. Results: Between-run CVs were 5.4–13%. The recoveries of porphyrins added to plasma were 71–114% except for protoporphyrin, which could not be reliably measured with this technique. Plasma porphyrin patterns clearly identified PCT, and its clinical sensitivity equaled that of urine porphyrin fractionation. The patterns also allowed differentiation of PCT of renal failure from pseudoporphyria of renal failure. Conclusions: The assay of plasma porphyrins identifies patients with PCT and appears particularly useful for differentiating PCT of renal failure from pseudoporphyria of renal failure.
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6

Kaczynski, Jerzy, Göran Hansson, and Sven Wallerstedt. "Increased Porphyrins in Primary Liver Cancer Mainly Reflect a Parallel Liver Disease." Gastroenterology Research and Practice 2009 (2009): 1–6. http://dx.doi.org/10.1155/2009/402394.

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Hepatic porphyries have been associated with an increased risk of primary liver cancer (PLC), which on the other hand may cause an increased porphyrin production. To evaluate the role of an underlying liver disorder we analyzed porphyrins in patients with hepatocellular carcinoma (HCC)(n=65), cholangiocellular carcinoma(n=3), or suspected PLC, which turned out to be metastases(n=18)or a benign disorder(n=11). None of the patients had a family history of porphyry or clinical signs of porphyry. Increased aminolevulinic acid or porphyrin values were common not only in patients with PLC (43%) but also in metastatic (50%) and benign (64%) liver disorders. The corresponding proportion for HCC patients with liver cirrhosis (55%) was higher(P<.05)than in those without cirrhosis (17%). We conclude that symptomatic porphyries are unusual in PLC, whereas elevated urinary and/or faecal porphyrins are common, primarily reflecting a parallel liver disease and not the PLC.
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7

Kasparek, Max, C. Can Bilgin, and Aydin Akin. "The Purple Gallinule,Porphyrio porphyrio, in the Eastern Mediterranean." Zoology in the Middle East 3, no. 1 (January 1989): 19–30. http://dx.doi.org/10.1080/09397140.1989.10637570.

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8

Norman, FI, and L. Mumford. "Studies on the Purple Swamphen, Porphyrio porphyrio, in Victoria." Wildlife Research 12, no. 2 (1985): 263. http://dx.doi.org/10.1071/wr9850263.

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Details are presented of gonad parameters and reproductive status, body measurements, moult, and food taken by purple swamphens collected in the Gippsland Lakes area between July 1971 and July 1973. Information from other studies is also discussed. Plant material (76% estimated average volume) dominated gizzard contents, particularly when inorganic material (23%) was discounted, and animal remains (c. 1%) were found in only 51 of the 234 gizzards with food examined. Mostly semi-aquatic plants were eaten, predominantly from Gramineae (59%), Cyperaceae (17%) and Hydrocharitaceae (11%), and Phragrnites australzs was the main species identified. The proportion of roots, rhizomes and tubers in gizzards increased in autumn samples. Monthly variations in the grit content of gizzards were significant; males (which were larger than females) held more large-sized fragments than females, and there was a moderate negative correlation between hard seeds and grit. Ovarian follicles were generally largest between July and November and seminiferous tubule diameters in males increased from June and July to reach a maximum from September to November; sperm were present from July to November. There were no simple correlations between gonad parameters and local rainfall in the preceding 30 days. The presence of a bursa of Fabricius did not successfully exclude reproductively active birds, but male swamphens with cloaca1 papillae and no bursa had larger testes than those with a bursa but no papillae. Colour scores for bill and tarsus showed some seasonal variation in subsamples of males and females. Management of wetlands for this species must take account of its requirement for a relatively restricted range of food types.
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9

Puzikova, А. I., Е. А. Litvin, D. А. Kildyushkin, and А. Е. Druy. "Application of high-performance liquid chromatography in porphyrias diagnostics." Pediatric Hematology/Oncology and Immunopathology 20, no. 3 (October 8, 2021): 140–44. http://dx.doi.org/10.24287/1726-1708-2021-20-3-140-144.

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Porphyrias are distinguished by the stage of heme synthesis at which a failure occurs, leading to the accumulation of intermediate products – porphyrins. Due to the low specificity of clinical manifestations of porphyria and the latent course of the disease, their timely diagnosis is difficult. This article substantiates the effectiveness of high-performance liquid chromatography method in the determination of porphyrins. The method is suitable for porphyrin determination in urine, blood and feces of patients. Examples of its work are shown.
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10

Woolf, Jacqueline, Joanne T. Marsden, Timothy Degg, Sharon Whatley, Paul Reed, Nadia Brazil, M. Felicity Stewart, and Michael Badminton. "Best practice guidelines on first-line laboratory testing for porphyria." Annals of Clinical Biochemistry: International Journal of Laboratory Medicine 54, no. 2 (January 19, 2017): 188–98. http://dx.doi.org/10.1177/0004563216667965.

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The porphyrias are disorders of haem biosynthesis which present with acute neurovisceral attacks or disorders of sun-exposed skin. Acute attacks occur mainly in adults and comprise severe abdominal pain, nausea, vomiting, autonomic disturbance, central nervous system involvement and peripheral motor neuropathy. Cutaneous porphyrias can be acute or chronic presenting at various ages. Timely diagnosis depends on clinical suspicion leading to referral of appropriate samples for screening by reliable biochemical methods. All samples should be protected from light. Investigation for an acute attack: • Porphobilinogen (PBG) quantitation in a random urine sample collected during symptoms. Urine concentration must be assessed by measuring creatinine, and a repeat requested if urine creatinine <2 mmol/L. • Urgent porphobilinogen testing should be available within 24 h of sample receipt at the local laboratory. Urine porphyrin excretion (TUP) should subsequently be measured on this urine. • Urine porphobilinogen should be measured using a validated quantitative ion-exchange resin-based method or LC-MS. • Increased urine porphobilinogen excretion requires confirmatory testing and clinical advice from the National Acute Porphyria Service. • Identification of individual acute porphyrias requires analysis of urine, plasma and faecal porphyrins. Investigation for cutaneous porphyria: • An EDTA blood sample for plasma porphyrin fluorescence emission spectroscopy and random urine sample for TUP. • Whole blood for porphyrin analysis is essential to identify protoporphyria. • Faeces need only be collected, if first-line tests are positive or if clinical symptoms persist. Investigation for latent porphyria or family history: • Contact a specialist porphyria laboratory for advice. Clinical, family details are usually required.
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11

Hardin, Scott, Ellen Donlan, Marsha Ward, and Dave Eggeman. "Attempted eradication of Porphyrio porphyrio Linnaeus in the Florida Everglades." Management of Biological Invasions 2, no. 1 (2011): 47–55. http://dx.doi.org/10.3391/mbi.2011.2.1.05.

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12

Callaghan, Corey T., and Dale E. Gawlik. "Diet and Selectivity of Porphyrio porphyrio (Purple Swamphen) in Florida." Southeastern Naturalist 15, sp8 (December 2016): 1–14. http://dx.doi.org/10.1656/058.015.sp801.

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13

Gunn, M. R., Z. Champion, M. E. Casey, P. Teal, and P. J. Casey. "Testicular and spermatozoan parameters in the pukeko (Porphyrio porphyrio melanotus)." Animal Reproduction Science 109, no. 1-4 (December 2008): 330–42. http://dx.doi.org/10.1016/j.anireprosci.2007.11.002.

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14

Hindmarsh, J. T. "The porphyrias: recent advances." Clinical Chemistry 32, no. 7 (July 1, 1986): 1255–63. http://dx.doi.org/10.1093/clinchem/32.7.1255.

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Abstract Recent research has elucidated several of the hitherto poorly understood steps in heme synthesis. This review describes this metabolic pathway and pinpoints the enzymatic blockages in the various porphyrias. Recent advances in the understanding of the etiology of porphyria cutanea tarda are discussed, as are the abnormalities of porphyrin metabolism seen in chronic renal failure and in lead poisoning. An outline is given of the clinical and biochemical abnormalities seen in the porphyrias. Included is an algorithm to aid in the differential diagnosis of these diseases, and a brief review of the new analytical techniques available for the identification and quantification of porphyrins and their precursors in body fluids.
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15

Doss, D. Paramanantha Swami, N. Gopukumar, and K. Sripathi. "Breeding Biology of the Purple Swamphen (Porphyrio porphyrio) at Tirunelveli, South India." Wilson Journal of Ornithology 121, no. 4 (December 2009): 796–800. http://dx.doi.org/10.1676/08-101.1.

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16

Clapperton, B. Kay. "Individual recognition by voice in the pukeko,Porphyrio porphyrio melanotus(Aves: Rallidae)." New Zealand Journal of Zoology 14, no. 1 (January 1987): 11–18. http://dx.doi.org/10.1080/03014223.1987.10422677.

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17

Subrata, Sena Adi. "GENETIC DIVERSITY AND POPULATION STRUCTURE OF MANDAR BESAR (Porphyrio porphyrio) iNHABITING JAVA WETLANDS." KnE Life Sciences 2, no. 1 (September 20, 2015): 119. http://dx.doi.org/10.18502/kls.v2i1.129.

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<p>Despite the occurrence of a massive agricultural landscape change in Java, very few reliable studies evaluate its genetic consequence for impacted wildlife species. This study reported the genetic diversity of the Mandar besar (Porphyrio porphyrio) in the modified landscape of Java as a backdrop for such an evaluation. Aim of this study was to estimate genetic diversity and genetic structure of the species. I collected eighty-eight blood samples from five a-priory populations (Demak, Jombor, Kendal, Pagak, and Nawan) and genotyped them at ten selected microsatellite loci to estimate allelic richness (R) and expected heterozygosity (He), fixation index (FST) and genetic clusters (K). DNA was isolated using a commercial kit (Invitrogen) and amplified using touch-down polymerase-chain-reaction technique. Allele length was estimated using an automatic sequencer. Pre-analysis showed that all selected loci have null alleles frequency less than 0.2, successful amplification rate more than 80%, no significant linkage between loci and no deviation from HWE. Further analysis employing GDA and FSTAT showed that Demak population has richest genetic diversity in term of R, meanwhile Jombor was the poorest population. Analysis of population structure employing FSTAT and STRUCTURE showed that the population was likely structured into four subpopulations, as showed by FST (0.085; P&lt;0.05) and K=4 (ln probability -1725.2+3.92). This study might reveal that genetic diversity and population structure of Mandar besar in Java were still in common level as compared to other aquatic bird species.</p><p><br /><strong>Keywords </strong>:Swamphen, bird, richness, genetic, wetlands</p>
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18

Jamieson, Ian G. "Testing reproductive skew models in a communally breeding bird, the pukeko, Porphyrio porphyrio." Proceedings of the Royal Society of London. Series B: Biological Sciences 264, no. 1380 (March 22, 1997): 335–40. http://dx.doi.org/10.1098/rspb.1997.0048.

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19

Ricci, Andrea, Claudio Carmine Guida, Paola Manzini, Chiara Cuoghi, and Paolo Ventura. "Kidney Involvement in Acute Hepatic Porphyrias: Pathophysiology and Diagnostic Implications." Diagnostics 11, no. 12 (December 10, 2021): 2324. http://dx.doi.org/10.3390/diagnostics11122324.

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Porphyrias are a group of rare disorders originating from an enzyme dysfunction in the pathway of heme biosynthesis. Depending on the specific enzyme involved, porphyrias manifest under drastically different clinical pictures. The most dramatic presentation of the four congenital acute hepatic porphyrias (AHPs: acute intermittent porphyria—AIP, ALAD deficiency, hereditary coproporphyria—HCP, and porphyria variegata—VP) consists of potentially life-threatening neurovisceral attacks, for which givosiran, a novel and effective siRNA-based therapeutic, has recently been licensed. Nonetheless, the clinical manifestations of acute porphyrias are multifaceted and do not limit themselves to acute attacks. In particular, porphyria-associated kidney disease (PAKD) is a distinct, long-term degenerating condition with specific pathological and clinical features, for which a satisfactory treatment is not available yet. In PAKD, chronic tubule-interstitial damage has been most commonly reported, though other pathologic features (e.g., chronic fibrous intimal hyperplasia) are consistent findings. Given the relevant role of the kidney in porphyrin metabolism, the mechanisms possibly intervening in causing renal damage in AHPs are different: among others, δ-aminolevulinic acid (ALA)-induced oxidative damage on mitochondria, intracellular toxic aggregation of porphyrins in proximal tubular cells, and derangements in the delicate microcirculatory balances of the kidney might be implicated. The presence of a variant of the human peptide transporter 2 (PEPT2), with a greater affinity to its substrates (including ALA), might confer a greater susceptibility to kidney damage in patients with AHPs. Furthermore, a possible effect of givosiran in worsening kidney function has been observed. In sum, the diagnostic workup of AHPs should always include a baseline evaluation of renal function, and periodic monitoring of the progression of kidney disease in patients with AHPs is strongly recommended. This review outlines the role of the kidney in porphyrin metabolism, the available evidence in support of the current etiologic and pathogenetic hypotheses, and the known clinical features of renal involvement in acute hepatic porphyrias.
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20

Krivosheev, Alexander B., L. Ya Kupriyanova, and M. A. Kondratova. "DOUBLE PORPHYRIA: LITERATURE REVIEW AND ANALYSIS OF CLINICAL OBSERVATION." Russian Journal of Skin and Venereal Diseases 21, no. 2 (April 15, 2018): 120–24. http://dx.doi.org/10.18821/1560-9588-2018-21-2-120-124.

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A brief review of the literature on the problem of double porphyria and analysis of its own observation is presented. For more than 10 years patient B was observed for more than 10 years with a verified diagnosis of acute intermittent porphyria, which manifested with acute pain abdominal syndrome, neurological disorders in the form of peripheral polyneuropathy and hemiparesis of lower extremities, and hypertension syndrome was also noted. The observed clinical symptoms corresponded to an acute porphyrin crisis in the manifestation and / or relapse of acute intermittent porphyria. The diagnosis was confirmed by a quantitative determination of the excretory profile of porphyrin precursors (δ-aminocaproic acid, porphobilinogen) and porphyrin fractions (uroporphyrin, coproporphyrin). Their concentrations are significantly (especially porphyrin precursors) exceeding the control values, which is the cardinal diagnostic criterion of acute intermittent porphyria. Against the backdrop of persistent clinical and biochemical remission of acute intermittent porphyria, symptoms of photosensitization of the skin (blisters, erosion, pigment spots) on the dorsal surface of the hands began to appear in 4 years. Later, hypertrichosis was formed in the temporo-periorbital region. The constellation type of the excretory profile of porphyrins began to change. Against the backdrop of persistent increased excretion of porphyrin precursors (δ-aminolevulinic acid and porphobilinogen), a progressive increase in the excretion of the fraction of uroporphyrin was observed, which became dominant (up to 58% of the total content of porphyrins). Such a prolonged observation in the dynamics allowed us to state the appearance of a new variant of the porphyrin exchange disturbance, which, taking into account clinical symptoms, corresponded to another form of hepatic porphyria, namely, late cutaneous porphyria. The clinical and biochemical changes in the excretory profile of the parameters of porphyrin metabolism registered in the dynamics of observation may indicate the occurrence of a combined enzymatic defect characteristic of double porphyria. In our case, a manifestation of late cutaneous porphyria was noted against a background of compensated acute intermittent porphyria.
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21

Boev, Zlatozar. "Bird images in the mosaics of Late Antique basilicas in the town of Sandanski (Blagoevgrad Region, SW Bulgaria)." Historia naturalis bulgarica 41, no. 2 (January 3, 2020): 13–21. http://dx.doi.org/10.48027/hnb.41.02001.

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This paper presents and analyses for the first time all data on bird images of two Late Antique basilicas of the Roman period (5th-6th c. AD) in the town of Sandanski (SW Bulgaria). A total of eight species have been recognised on the floor mosaics of both basilicas: Anas platyrhynchos, Bucephala clangula, Dendrocygna viduata, Nettapus coromandelianus, Marmaronetta angustirostris, Porphyrio porphyrio, Psittacula krameri, and Sypheotides indicus. Exotic birds of Palaeotropic (Ethiopian) and Indomalayan (Oriental) distribution confirm once again the active ancient Roman contacts with East and Trans-Saharan Africa and the Hindustan Peninsula.
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22

Jamieson, Ian G., John L. Craig, and Edward O. Minot. "Incubation by young, nonbreeding birds: potential versus realization of behaviour." Canadian Journal of Zoology 65, no. 10 (October 1, 1987): 2567–70. http://dx.doi.org/10.1139/z87-386.

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Incubation behaviour is described in nonbreeders of two species of birds. Among communally breeding pukeko (Porphyrio porphyrio melanotus), some subadult members of a group incubate eggs but are not involved in courtship or mating. In one population of white-rumped swiftlet (Aerodramus spodiopygius), a single nestling is reared initially at each nest but that nestling incubates a second egg before fledging. The standard proximate and ultimate explanations given for parental incubation would be difficult to apply in these cases. Incubation of eggs might therefore be better understood by looking at the circumstances that cause birds to realize their potential for exhibiting incubation behaviour.
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23

Seymour, John. "Purple Swamphens Porphyrio porphyrio and Dusky Moorhens Gallinula tenebrosa climbing trees and feeding on lerps." Australian Field Ornithology 33 (2016): 16–19. http://dx.doi.org/10.20938/afo33016019.

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24

Sánchez-Lafuente, Alfonso M., Pedro Rey, Francisco Valera, and Joaquín Muñoz-Cobo. "Past and current distribution of the purple swamphen Porphyrio porphyrio L. in the Iberian Peninsula." Biological Conservation 61, no. 1 (1992): 23–30. http://dx.doi.org/10.1016/0006-3207(92)91204-6.

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25

Dawe, Robert. "An overview of the cutaneous porphyrias." F1000Research 6 (October 30, 2017): 1906. http://dx.doi.org/10.12688/f1000research.10101.1.

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This is an overview of the cutaneous porphyrias. It is a narrative review based on the published literature and my personal experience; it is not based on a formal systematic search of the literature. The cutaneous porphyrias are a diverse group of conditions due to inherited or acquired enzyme defects in the porphyrin–haem biosynthetic pathway. All the cutaneous porphyrias can have (either as a consequence of the porphyria or as part of the cause of the porphyria) involvement of other organs as well as the skin. The single commonest cutaneous porphyria in most parts of the world is acquired porphyria cutanea tarda, which is usually due to chronic liver disease and liver iron overload. The next most common cutaneous porphyria, erythropoietic protoporphyria, is an inherited disorder in which the accumulation of bile-excreted protoporphyrin can cause gallstones and, rarely, liver disease. Some of the porphyrias that cause blistering (usually bullae) and fragility (clinically and histologically identical to porphyria cutanea tarda) can also be associated with acute neurovisceral porphyria attacks, particularly variegate porphyria and hereditary coproporphyria. Management of porphyria cutanea tarda mainly consists of visible-light photoprotection measures while awaiting the effects of treating the underlying liver disease (if possible) and treatments to reduce serum iron and porphyrin levels. In erythropoietic protoporphyria, the underlying cause can be resolved only with a bone marrow transplant (which is rarely justifiable in this condition), so management consists particularly of visible-light photoprotection and, in some countries, narrowband ultraviolet B phototherapy. Afamelanotide is a promising and newly available treatment for erythropoietic protoporphyria and has been approved in Europe since 2014.
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Meyer, Douglas. "Azure Gallinule, Porphyrio flavirostris (Aves: Rallidae): first record for the state of Santa Catarina, Southern Brazil." Check List 11, no. 5 (September 9, 2015): 1732. http://dx.doi.org/10.15560/11.5.1732.

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27

Griffith, Harry S. "Hemin Prophylaxis for Patients with Frequently Recurring Acute Porphyric Attacks: Experience with Five Cases." Blood 106, no. 11 (November 16, 2005): 3545. http://dx.doi.org/10.1182/blood.v106.11.3545.3545.

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Abstract Background The acute porphyrias are a group of four clinically indistinguishable diseases characterized by sudden, acute episodes of neurovisceral symptoms that can be life-threatening. Attacks are precipitated by a genetic deficiency in one of the enzymes involved in heme biosynthesis, acting in concert with one or more endogenous or exogenous triggers. The resultant stimulation of hepatic heme results in overproduction of highly reactive heme intermediaries - porphyrins and porphyrin precursors - which accumulate in the liver, blood, and bone marrow. A small percentage of patients have attacks that recur every several weeks, even after all known triggers are eliminated. Although hemin prophylaxis has been recommended for such patients (Anderson K, et al. Recommendations for the Diagnosis and Treatment of the Acute Porphyrias. Annals of Internal Medicine. 2005 Mar; 142(6):439–50), no adequate and well-controlled clinical studies have been conducted. Methods We report on five patients, all diagnosed with acute porphyria on the basis of elevated levels of urinary aminolevulinic acid and porphobilinogen (4 with acute intermittent porphyria, 1 with hereditary coproporphyria). All had a history of recurring acute porphyric attacks and received hemin prophylaxis for at least one year in an out-patient setting and were previously treated for a minimum of one year in a hospital setting. The dosage and frequency of prophylactic hemin was based on each patient’s clinical picture, and ranged from a one-day course of 1–4mg/kg/day every 10 to 40 days to a four day-course of 1–4 mg/kg/day every 30 days. The outcome of prophylaxis hemin treatment was determined on the basis of frequency of severe attacks requiring hospitalization and both physician and patient assessment of the severity of symptoms. Results Five patients (3 female, 2 male), ranging in age from 25–61 years old suffered from frequently recurring acute porphyric attacks, with symptoms that included abdominal pain (5), psychological disturbances (2), peripheral motor neuropathy (2), and nausea (2). Two patients had a significant reduction in the frequency of their acute attacks and three reported a moderate decrease with prophylactic hemin treatment. All five patients experienced a marked improvement in the reduction of the severity of their attacks. Overall, the occurrence of severe acute attacks requiring hospitalization declined by 66%. Three of the five patients did not require hospitalization over the 12 month period of time. One patient experienced phlebitis during one course of hemin treatment. Conclusions Hemin prophylaxis can be an effective disease management strategy in acute porphyria patients with frequently recurring acute attacks.
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Pacheco, Carlos, and Peter K. McGregor. "Conservation of the purple gallinule (Porphyrio porphyrio L.) in Portugal: causes of decline, recovery and expansion." Biological Conservation 119, no. 1 (September 2004): 115–20. http://dx.doi.org/10.1016/j.biocon.2003.11.001.

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29

Egemen, Gamze, Mustafa Hayvalı, Zeynel Kılıç, A. Osman Solak, and Zafer Üstündağ. "Phosphorus-nitrogen compounds Part 17: The synthesis, spectral and electrochemical investigations of porphyrino-phosphazenes." Journal of Porphyrins and Phthalocyanines 14, no. 03 (March 2010): 227–34. http://dx.doi.org/10.1142/s1088424610001945.

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The reactions of unsymmetrical porphyrins (1 and 2) with Ni(OAc)2·4H2O in boiling DMF produce porphyrin complexes (3 and 4). From the reactions of free porphyrin ligands 1 and 2 with hexachlorocyclotriphosphazatriene, N3P3Cl6 , the new free porphyrino-phosphazene derivatives (5 and 6) are obtained. On the other hand, the reactions of N3P3Cl6 with porphyrin complexes (3 and 4) afford the new porphyrino-phosphazene complexes (7 and 8). In the literature there are a few examples of the porphyrino-phosphazene architectures. The structural investigations of all the compounds have been made by elemental analyses, MS, FTIR, 1H NMR, 31P NMR and UV-visible techniques. The cyclic voltammograms (CVs) are examined in acetonitrile (MeCN) containing 0.1 M tetrabutylammonium-tetrafluoroborate (TBATFB) to investigate the surface attachment properties at the glassy carbon electrode (GCE) and the influence of the presence of metal cations in the porphyrin ring.
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30

Carson, R. W., E. J. Dunnigan, T. D. DuBose, D. E. Goeger, and K. E. Anderson. "Removal of plasma porphyrins with high-flux hemodialysis in porphyria cutanea tarda associated with end-stage renal disease." Journal of the American Society of Nephrology 2, no. 9 (March 1992): 1445–50. http://dx.doi.org/10.1681/asn.v291445.

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Plasma porphyrin levels are markedly increased in patients with porphyria cutanea tarda (PCT) associated with end-stage renal disease. Conventional hemodialysis (CHD) with lower blood flow rates (less than 250 mL/min) and cuprophan or cellulose acetate membranes is ineffective in removing significant amounts of porphyrins in this condition. Changes in plasma porphyrin levels and porphyrin clearances during hemodialysis with higher blood flow rates and more-permeable, high-efficiency cellulose acetate and high-flux polysulfone dialyzers were evaluated in a chronic hemodialysis patient with PCT and markedly elevated plasma porphyrins. The polysulfone membrane achieved significantly better fractional porphyrin removal (P = 0.02) and porphyrin clearances (P less than 0.01) than did the high-efficiency cellulose acetate membrane. After conversion from maintenance CHD with a standard cellulose acetate dialyzer to a 4-wk period of high-flux hemodialysis (HFHD) with a polysulfone dialyzer, predialysis plasma porphyrins fell by 37%. After returning to CHD, plasma porphyrins returned to the higher prestudy levels. These observations suggest that HFHD with more permeable membranes and higher blood flow rates removes porphyrins more effectively than does CHD. HFHD may be a useful adjunct to other measures used in treating dialysis patients with PCT.
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31

Lambie, Deborah, Chris Florkowski, Chris Sies, Anthony Raizis, Wai-Kwan Siu, and Cindy Towns. "A case of hereditary coproporphyria with posterior reversible encephalopathy and novel coproporphyrinogen oxidase gene mutation c.863T>G (p.Leu288Trp)." Annals of Clinical Biochemistry: International Journal of Laboratory Medicine 55, no. 5 (April 27, 2018): 616–19. http://dx.doi.org/10.1177/0004563218774597.

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A 21-year-old female had recurrent presentations to the emergency department with myalgia, vomiting, abdominal pain and subsequently developed generalized seizures. She was volume depleted with a plasma sodium of 125 mmol/L (reference interval: 135–145) and she had fluctuating hypertension. Acute porphyria was suspected and confirmed with raised urine porphobilinogen/creatinine ratio of 12:4 μmol/mmoL (reference interval < 1:5) and she was treated with intravenous haem arginate. Urinary porphyrin/creatinine ratio was 673 nmol/mmoL (reference interval <35) and faecal porphyrins 2430 μmol/kg dry weight (reference interval: <200) were markedly elevated, with raised faecal CIII:CI ratio, consistent with acute coproporphyria. Diagnosis was confirmed by the demonstration of a novel missense variant in the coproporphyrinogen oxidase gene c.863T > G (p.Leu288Trp) predicted to be deleterious and which segregated with three other affected family members. Although CT head was normal, magnetic resonance imaging scan revealed symmetrical signal abnormalities and swelling in the parietal and occipital lobes consistent with posterior reversible encephalopathy. Over several days, her seizures ceased and sodium and blood pressure normalized. The aetiology of the acute porphyric attack was likely multifactorial with contributions from a recent viral illness and caloric deprivation. No drug precipitant was identified. We postulate that untreated hypertension played a key role in the development of posterior reversible encephalopathy. Early clinical suspicion and urine porphobilinogen testing are the key components in preventing morbidity and mortality in acute porphyrias.
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32

Subrata, S. A., and I. Storch. "A core set of microsatellite markers identified for use in population genetic studies of Purple Swamphen (Porphyrio porphyrio)." Animal Biodiversity and Conservation 35, no. 1 (June 2012): 23–26. http://dx.doi.org/10.32800/abc.2012.35.0023.

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Populations of Purple Swamphen have been declining in some parts of the world due to wetland degradation and poaching. Conservation efforts might benefit from population genetic studies using microsatellite markers. Here we describe the selection of microsatellite markers suitable for such studies. We tested the markers against the evidence of the null alleles and gametic disequilibrium. From fourteen candidate loci, ten displayed a null alleles frequency of less than 0.2 and had no significant linkage disequilibrium. The selected loci showed no deviation from HWE and had a mean polymorphic information content of 0.516. Based on the test, we suggest these loci are suitable for population genetic studies of the species.
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33

Ruggian, J. C., S. Fishbane, F. J. Demento, J. K. Maesaka, and G. L. Frei. "Porphyria cutanea tarda in a patient on chronic ambulatory peritoneal dialysis." Journal of the American Society of Nephrology 7, no. 3 (March 1996): 397–402. http://dx.doi.org/10.1681/asn.v73397.

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Porphyria cutanea tarda is a disorder of heme biosynthesis resulting from a defect or deficiency in the enzyme uroporphyrinogen decarboxylase. Heme precursors accumulate in the blood, urine, stool, and skin, where exposure to sunlight results in the clinical manifestations. Porphyria cutanea tarda has been described in adult hemodialysis patients. The pathogenesis of porphyria cutanea tarda in this population is thought to be related to the inability of hemodialysis to adequately clear porphyrin precursors, resulting in increased precursor serum levels, precursor skin deposition, and subsequent clinical manifestations. A proper diagnosis of porphyria cutanea tarda in hemodialysis patients requires fractionation of serum porphyrins. Normalization of the porphyrin profile and reversal of the dermal manifestations require the withdrawal of hepatotoxic agents and the reversal of hepatic iron overload. A case of porphyria cutanea tarda in an adult ESRD patient treated with continuous ambulatory peritoneal dialysis is described. In this patient, the disease was related to elevated serum levels of phenytoin, which had been administered for seizure disorder.
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34

Olson, Storrs L. "Birds, including extinct species, encountered by the Malaspina Expedition on Vava'u, Tonga, in 1793." Archives of Natural History 33, no. 1 (April 2006): 42–52. http://dx.doi.org/10.3366/anh.2006.33.1.42.

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Four drawings and a short manuscript reference from the Spanish Malaspina Expedition provide certain records of five species of birds on the island of Vava'u, Kingdom of Tonga, in 1793. Three of these are extant species known historically on Vava'u: Purple Swamphen (Porphyrio porphyrio), Purple-capped Fruit-Dove (Ptilinopus porphyraceus), and Blue-crowned Lorikeet (Vini australis). Also depicted is what is believed to be an extinct rail, probably flightless, of the genus Gallirallus, with possible relatives known from bones on ‘Eua, Tongatapu Group, and from an historical account on Tahiti. Another drawing appears to represent a male of the extinct parrot Eclectus infectus, recently described from bones from ‘Eua and from Lifuka and ‘Uiha in the Ha'apai Group. Thus, this parrot and an extinct rail appear to have survived on Vava'u as late as the end of the eighteenth century.
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35

Teplyuk, N. P., Ekaterina Yu Vertieva, D. V. Ignatyev, and I. S. Dzhavakhishvili. "Porphyria cutanea tarda concomitant with chronic hepatitis C." Russian Journal of Skin and Venereal Diseases 19, no. 1 (February 15, 2016): 17–20. http://dx.doi.org/10.18821/1560-9588-2016-19-1-17-20.

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Porphyrias form a group of rare metabolic diseases associated with disorders in the heme biosynthesis enzymes, leading to porphyrin accumulation in tissues. The disease is rare and is often diagnosed too late. The most incident is porphyria cutanea tarda. All patients with this condition should be tested for viral hepatites and hemochromatosis. A clinical case is presented: a patient with porphyria cutanea tarda associated with alcohol abuse and viral hepatitis C.
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36

Lockwood, W. H., V. Poulos, E. Rossi, and D. H. Curnow. "Rapid procedure for fecal porphyrin assay." Clinical Chemistry 31, no. 7 (July 1, 1985): 1163–67. http://dx.doi.org/10.1093/clinchem/31.7.1163.

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Abstract Hydrochloric acid extraction of feces in the presence of ether yields an extract suitable for spectrophotometric estimation of total porphyrin and for further separation by "high-performance" liquid chromatography (HPLC) or thin-layer chromatography. A total porphyrin reference interval of less than 200 nmol/g dry weight of feces was established from data on 106 normal subjects on an unrestricted diet. Total fecal porphyrin values in 11 porphyria cutanea tarda patients were considerably higher than given by the widely used Rimington method (respective means, 652 and 239 nmol/g dry weight). Our HPLC method for separation of porphyrin methyl esters on a silica column, with quantification by fluorescence, is described. HPLC separations performed on 23 porphyria cutanea tarda patients gave the following mean proportions of total fecal porphyrins: dicarboxylics 21%, coproporphyrin 9%, isocoproporphyrins 28%, pentacarboxylporphyrin 9%, hexacarboxylporphyrin 11%, heptacarboxylporphyrin 18%, and uroporphyrin 4%.
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37

Dogra, Ashu. "Role of therapeutic phlebotomy in management of case of porphyria cutanea tarda (PCT) admitted in tertiary care hospital Vadodara." Hematology & Transfusion International Journal 8, no. 1 (January 30, 2020): 4–6. http://dx.doi.org/10.15406/htij.2020.08.00212.

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Porphyria cutanea tarda is the most frequent type of Porphyria worldwide & presents with skin symptoms mainly. Porphyrias can affect peripheral, autonomic and central nervous system. In Porphyria conditions there is accumulation of heme precursors 5 Aminolevulinic acid, Porphobilinogen and porphyrins which are associated with characteristic clinical feature with acute neurovisceral attacks and skin lesions. This case report summarizes Case of PCT that was successfully managed with Therapeutic Phlebotomy.
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38

Johnson, P. M., S. L. Perkins, and S. W. Kennedy. "A high-speed liquid-chromatographic method for measuring urinary porphyrins." Clinical Chemistry 34, no. 1 (January 1, 1988): 103–5. http://dx.doi.org/10.1093/clinchem/34.1.103.

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Abstract We describe a rapid quantitative and qualitative "high-performance" liquid-chromatographic (HPLC) method for measuring porphyrins in urine. Direct injection of acidified, filtered urine onto a 3-micron (particle size) 3-cm-long reversed-phase column fully resolves uroporphyrin, hepta-, hexa-, and pentacarboxylic acid porphyrins, and coproporphyrin. Instrument response is linearly related to concentration over the range 25 to 300 nmol/L. The method provides data essential for the differential diagnosis of porphyric states, including porphyria variegata and porphyria cutanea tarda. This relatively inexpensive method requires a run time of only 8 min per sample, making it particularly suitable for routine use in the clinical chemistry laboratory.
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Perkins, S. L., and P. M. Johnson. "Loss of porphyrins from solution during analysis: effect of sample pH and matrix on porphyrin quantification in urine by "high-performance" liquid chromatography." Clinical Chemistry 35, no. 7 (July 1, 1989): 1508–12. http://dx.doi.org/10.1093/clinchem/35.7.1508.

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Abstract We report the effect of sample matrix and pH on quantification of porphyrins by HPLC with fluorimetric detection. For aqueous solutions of pH less than 2.5, HPLC peak heights of the porphyrins increased with decreasing pH, reaching a plateau at pH less than 1.0. This loss of porphyrins from solutions with pH greater than 1.0 appeared to be due to a combination of microprecipitation and aggregation effects. No such "pH effect" was observed for urine samples supplemented with mixed-porphyrin standards. Addition of trace amounts of albumin to aqueous solutions also decreased these pH-related losses. These findings suggest a porphyrin-protein interaction that prevents microprecipitation and aggregation processes. We conclude that standard solutions of porphyrins for HPLC analysis should be prepared in a urine matrix. If aqueous solutions are used, then the pH must be adjusted to less than 1.0. Urine samples from normal individuals require only adjustment of pH to less than 2 before analysis; however, porphyric urines requiring dilution should be prepared with porphyrin-free urine diluent.
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40

Schoenfeld, N., and R. Mamet. "Interference of ofloxacin with determination of urinary porphyrins." Clinical Chemistry 40, no. 3 (March 1, 1994): 417–19. http://dx.doi.org/10.1093/clinchem/40.3.417.

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Abstract The second-generation quinolone ofloxacin interferes with the screening test of porphyrins. We observed a 20-fold increase in the porphyrin concentration measured in urine of an ofloxacin-treated patient, compared with drug-free normal urine. Two other fluorinated 4-quinolones tested, norfloxacin and ciprofloxacin, had a less marked effect (a twofold increase), whereas the first-generation quinolone, nalidixic acid, did not affect the measured porphyrin concentration at all. The interference is probably due to the overlap in the emission fluorescence spectra of ofloxacin and urinary porphyrins at approximately 600 nm. To avoid a false-positive diagnosis of porphyria, we suggest using HPLC to separate ofloxacin (10-min retention time) from urinary porphyrins (which only start to elute at 12 min). Nonetheless, given a threefold increase in urinary porphyrins observed in the urine of an ofloxacin-treated patient, we also discuss a possible interference of the drug with the metabolism of porphyrins.
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41

Lai, C. K., C. W. Lam, and Y. W. Chan. "High-performance thin-layer chromatography of free porphyrins for diagnosis of porphyria." Clinical Chemistry 40, no. 11 (November 1, 1994): 2026–29. http://dx.doi.org/10.1093/clinchem/40.11.2026.

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Abstract In this simple high-performance thin-layer chromatographic technique for evaluating porphyrin excretion, porphyrins rapidly extracted from urine or feces are separated on reversed-phase octadecylsilyl (C18)-bonded silica thin-layer chromatography plates. We observed &gt; 12 distinct bands of different porphyrins by viewing the plates under long-wave ultraviolet light. Positive screening tests can readily be characterized by the relative fluorescence of various free porphyrins. The two patients presented in this paper are possibly the first two cases of porphyria cutanea tarda reported in Hong Kong Chinese.
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42

HUME, JULIAN PENDER. "Systematics, morphology and ecology of rails (Aves: Rallidae) of the Mascarene Islands, with one new species." Zootaxa 4626, no. 1 (July 3, 2019): 1–107. http://dx.doi.org/10.11646/zootaxa.4626.1.1.

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Five species in five genera of extinct endemic rails have been described from the Mascarene Islands of Mauritius, Réunion and Rodrigues: the Mauritian Red Rail or Poule Rouge Aphanapteryx bonasia; Mascarene Coot or Poule d’eau Fulica newtonii; which occurred on Mauritius and Réunion; Réunion Wood Rail Dryolimnas augusti; Réunion Gallinule or Oiseaux bleu ‘Porphyrio caerulescens’; and Rodrigues or Leguat’s Rail Erythromachus leguati. All are known from fossil remains and/or from contemporary accounts and illustrations. A sixth species of rail Dryolimnas sp. nov. is described herein from fossils from Mauritius, but was not unequivocally previously reported in the contemporary literature. This paper provides an analysis of the Rallidae of the Mascarene Islands based on existing and newly discovered fossil remains, and details historical reports and accounts. Comprehensive osteological descriptions and synonymies are also included. Their ecology and extinction chronologies are interpreted from historical ev­idence. The relationships of Aphanapteryx and Erythromachus are unresolved, having clearly been isolated for a considerable time; the middle Miocene is the earliest their ancestors could have arrived on the Mascarenes, but this may have happened more recently. Mascarene derivatives of Fulica, Porphyrio and Dryolimnas are of much more recent origin, and appear to have originated in Africa or Madagascar. All terrestrial rails on Mauritius, Réunion and Rodrigues, were probable victims of cat predation following their historic introduction to the islands, whereas over-hunting by humans was probably the primary cause of extinction of ‘Porphyrio caerulescens’ on Réunion. The only extant rail on the Mascarenes today, the Madagascar race of Eurasian Moorhen Gallinula chloropus pyrrhorrhoa, is a recent arrival, having colonised Mauritius and Réunion after the extinction of Fulica newtonii.
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43

Baytaeva, D. A., and S. S. Bessmel’tsev. "Porphyrin metabolism in secondary hepatic porphyria in patients with hereditary deficiency of glucose-6-phosphate dehydrogenase." Kazan medical journal 93, no. 3 (June 15, 2012): 451–55. http://dx.doi.org/10.17816/kmj1865.

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Aim. The study the porphyrin metabolism during the development of secondary hepatic porphyria in patients with glucose-6-phosphate dehydrogenase deficiency. Methods. Examined were 148 male patients aged 5-19 years (median 12 years) with impaired activity of glucose-6-phosphate dehydrogenase in combination with β-thalassemia and without it. Qualitative and quantitative methods of examining the activity of this enzyme were used in order to verify the diagnosis. Taking into account the varying degree of glucose-6-phosphate dehydrogenase deficiency, the indices of metabolism of the enzyme and of the porphyrins were correlated with the severity of anemia, functional liver capacities, with parameters reflecting iron content in blood serum, bone marrow, liver and urine. The markers of intoxication were also taken into account in the development of secondary hepatic porphyria and endotoxemia. Therapeutic plasmapheresis was used to correct the revealed disorders. Results. The influence of glucose-6-phosphate dehydrogenase deficiency on the metabolism of porphyrins and liver functional status has been shown, which leads to the development of anemia and endogenous intoxication. With the help of parameters, which characterize the porphyrin metabolism in patients, secondary hepatic porphyria was revealed. It was established that determination of the content of glucose-6-phosphate dehydrogenase and porphyrins makes it possible to detect disturbances in heme synthesis at an early stage and to evaluate the compensatory abilities of the liver. An important diagnostic feature for glucose-6-phosphate dehydrogenase deficiency, regardless of severity, is the impaired synthesis of the end products of metabolism of porphyrins - uro-, copro- and protoporphyrin. The effectiveness of therapeutic plasmapheresis for hemolysis, secondary hepatic porphyria and endogenous intoxication has been shown. Conclusion. Increased excretion of uro-and coproporphyrin with urine reflects the severity of endotoxemia, and is an alternative to markers of intoxication; high concentration of free protoporphyrin and low concentration of uro- and coproporphyrin in erythrocytes is an important diagnostic sign of impaired activity of glucose-6-phosphate dehydrogenase in patients.
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44

Rose, I. S., G. P. Young, D. J. St John, M. C. Deacon, D. Blake, and R. W. Henderson. "Effect of ingestion of hemoproteins on fecal excretion of hemes and porphyrins." Clinical Chemistry 35, no. 12 (December 1, 1989): 2290–96. http://dx.doi.org/10.1093/clinchem/35.12.2290.

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Abstract Stools from asymptomatic volunteers on diets containing red meat, whole blood, or high fiber were analyzed for their content of hemes and dicarboxylic (heme-derived) porphyrins by the "HemoQuant" assay, the "Hemoccult" test, and "high-performance" liquid chromatography (HPLC). In 49 subjects, ingestion of red meat increased HemoQuant-determined combined fecal heme plus dicarboxylic porphyrins by an average 375%; the contribution of heme-derived porphyrins to total fecal porphyrins increased from 37% to 78%. Of subjects on a red-meat diet, 27% passed stools with a porphyrin content suggestive of a porphyria, compared with only 4% on a red-meat-free diet. These increases were due largely to protoporphyrin and its derivatives pemptoporphyrin and deuteroporphyrin, all of which were present in feces as iron-free porphyrins and iron-ligated (heme) forms. Ingestion of blood had an effect similar to that of red meat, but ingestion of fiber had no effect. These effects of dietary and endogenous hemoproteins must be considered when such methods are used to test feces for occult blood or to test for excess fecal porphyrins as an indicator of a porphyria.
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45

Mouslim, Bara, Merzoug Seyf Eddine, Khelifa Rassim, Bouslama Zihad, and Houhamdi Moussa. "Aspects of the breeding ecology of the Purple Swamphen Porphyrio porphyrio in the wetland complex of Guerbes-Sanhadja, north-east Algeria." Ostrich 85, no. 2 (May 4, 2014): 185–91. http://dx.doi.org/10.2989/00306525.2014.971901.

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46

Moreno-Opo, Rubén, and Josep Piqué. "Reconciling the conservation of the purple swamphen (Porphyrio porphyrio) and its damage in Mediterranean rice fields through sustainable non-lethal techniques." PeerJ 6 (April 24, 2018): e4518. http://dx.doi.org/10.7717/peerj.4518.

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Resolving human–wildlife conflicts requires the assessment and implementation of appropriate technical measures that minimize negative impacts on socio-economic uses, including agriculture, and ensure the adequate protection of biological diversity. Rice paddies are widely distributed in the western Mediterranean region. Because of their high productivity, they can be a good habitat for waterbirds, including the purple swamphen Porphyrio porphyrio, particularly in areas where natural wetlands have been removed or reduced. As a result of its population growth, there have been increasing levels of damage caused by this species in rice fields due to stem-cutting and opening of bald patches in rice fields. With the aim of reducing damage, we evaluated the effectiveness of passive and active measures that would limit access to rice fields and deter/scare away purple swamphens in affected areas of the Ebro Delta (NE Spain). We selected the techniques according to the growth phase of rice and the activity of birds in the rice fields (perimeter fences and clearing vegetation around the rice plots during sprouting and growing phases, and falconry at maturation). There were positive results during the sprouting and growing phases thanks to fences and clearing vegetation, reducing the affected area by 37.8% between treatment and control plots. This would mean an economic savings of 18,550 €/year in compensation payments by regional administrations including the investment in implementing and maintaining passive protection measures. Active deterrence through falconry did not reduce the level of damage. The analysis of purple swamphen home range, activity centers (centroids), and the proportion of locations in and outside of rice fields showed no differences before and after dissuasive practices. These results were influenced by multiple concurrent factors including weather, the structural configuration of the rice plots and their location. In summary, we recommend the establishment of protection measures (perimeter fences + clearing vegetation around the rice plots) to reduce the level of damage.
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47

Samraoui, Farrah, Riad Nedjah, Ahmed H. Alfarhan, and Boudjéma Samraoui. "An overview of the Rallidae of Algeria with particular reference to the breeding ecology of the Purple Swamp-Hen Porphyrio porphyrio." Wetlands Ecology and Management 23, no. 3 (December 31, 2014): 505–17. http://dx.doi.org/10.1007/s11273-014-9404-0.

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48

Beukeveld, G. J., B. G. Wolthers, J. J. van Saene, T. H. de Haan, L. W. de Ruyter-Buitenhuis, and R. H. van Saene. "Patterns of porphyrin excretion in feces as determined by liquid chromatography; reference values and the effect of flora suppression." Clinical Chemistry 33, no. 12 (December 1, 1987): 2164–70. http://dx.doi.org/10.1093/clinchem/33.12.2164.

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Abstract While determining reference values for porphyrins in feces as measured by liquid chromatography, we observed strong fluctuations in fecal porphyrin contents. To explain these fluctuations, we selectively suppressed the intestinal flora of healthy persons. Suppression of aerobic flora had no effect on fecal porphyrin excretions, whereas suppression of anaerobic flora completely inhibited the transformation of protoporphyrin to pempto- and deuteroporphyrin for as long as five days after stopping medication. During this latter, the conversion to mesoporphyrin was clearly increased in one person and in others partly affected or decreased. During complete suppression of flora for prolonged periods, the production of proto- and coproporphyrins was decreased and deutero-, pempto-, and mesoporphyrins were absent. We conclude that the nature of fecal porphyrins is mostly affected by action of anaerobic bacteria, different kinds of bacteria having different effects. Some, like aerobic Gram-negative bacteria, have little or no effect on porphyrins; some cause production of mesoporphyrin; some promote a conversion to pempto- and deuteroporphyrin; and some mainly cause production of copro- and protoporphyrin. We give examples in which normal to slightly increased excretions of fecal porphyrin do not exclude a diagnosis of porphyria, and relatively high concentrations do not confirm one.
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49

Koukouzas, Nikolaos, Petros Koutsovitis, Pavlos Tyrologou, Christos Karkalis, and Apostolos Arvanitis. "Potential for Mineral Carbonation of CO2 in Pleistocene Basaltic Rocks in Volos Region (Central Greece)." Minerals 9, no. 10 (October 11, 2019): 627. http://dx.doi.org/10.3390/min9100627.

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Pleistocene alkaline basaltic lavas crop out in the region of Volos at the localities of Microthives and Porphyrio. Results from detailed petrographic study show porphyritic textures with varying porosity between 15% and 23%. Data from deep and shallow water samples were analysed and belong to the Ca-Mg-Na-HCO3-Cl and the Ca-Mg-HCO3 hydrochemical types. Irrigation wells have provided groundwater temperatures reaching up to ~30 °C. Water samples obtained from depths ranging between 170 and 250 m. The enhanced temperature of the groundwater is provided by a recent-inactive magmatic heating source. Comparable temperatures are also recorded in adjacent regions in which basalts of similar composition and age crop out. Estimations based on our findings indicate that basaltic rocks from the region of Volos have the appropriate physicochemical properties for the implementation of a financially feasible CO2 capture and storage scenario. Their silica-undersaturated alkaline composition, the abundance of Ca-bearing minerals, low alteration grade, and high porosity provide significant advantages for CO2 mineral carbonation. Preliminary calculations suggest that potential pilot projects at the Microthives and Porphyrio basaltic formations can store 64,800 and 21,600 tons of CO2, respectively.
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Bonkovsky, Herbert L. "Neurovisceral Porphyrias: What a Hematologist Needs to Know." Hematology 2005, no. 1 (January 1, 2005): 24–30. http://dx.doi.org/10.1182/asheducation-2005.1.24.

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Abstract The acute or inducible hepatic porphyrias comprise four inherited disorders of heme biosynthesis. They usually remain asymptomatic for most of the lifespan of individuals who inherit the specific enzyme deficiencies but may cause life-threatening attacks of neurovisceral symptoms. Failure to consider the diagnosis frequently delays effective treatment, and inappropriate diagnostic tests and/or mistaken interpretation of results may lead to misdiagnosis and inappropriate treatment. The four disorders are ALA dehydratase deficiency porphyria, acute intermittent porphyria, hereditary coproporphyria, and variegate porphyria. Other conditions that clinically and biochemically may mimic acute porphyria include lead poisoning and hereditary tyrosinemia type I. The diagnosis of one of these acute porphyric syndromes should be considered in many patients with otherwise unexplained abdominal pain, severe constipation, systemic arterial hypertension, or other characteristic symptoms. Critical to the rapid diagnosis of the three most common of these disorders is demonstration of markedly increased urinary porphobilinogen (PBG) in a single-void urine specimen. The treatment of choice for all but mild attacks of the acute porphyrias is intravenous hemin therapy, which should be started as soon as possible. Intravenous glucose alone is recommended only for mild attacks (no weakness or hyponatremia) or until hemin is available.
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