porphyrias/альбумины

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Intravenous heme-albumin in acute intermittent porphyria: evidence for repletion of hepatic hemoproteins and regulatory heme pools.

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The purpose of this study was to assess effects of heme administered intravenously, complexed to human serum albumin, on activities of the hepatic hemoproteins, cytochrome(s) P-450, and tryptophan pyrrolase, and on the size of the heme pool that regulates activity of 5-aminolevulinate synthase.

Depletion of histidine and tryptophan serum levels in porphyria cutanea tarda and congenital erythropoietic porphyria patients after irradiation with visible light.

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We have studied the photosensitized oxidation, via singlet oxygen production, of histidine (His) and tryptophan (Trp) in the serum of porphyria patients and in the serum of healthy volunteers before or after addition of hematoporphyrin. It was observed that free plasma His and Trp are good probes of

Acute intermittent porphyria, an important and rare differential diagnosis of acute abdomen: case report and literature review.

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Porphyrias are metabolic disorders related to heme biosynthesis pathway enzyme dysfunctions. The heme pathway is fundamental for the formation of a number of molecules, and such defects cause noxious precursors (porphyrins) to build up. Porphyrias are heterogeneously manifested by symptoms that can

A tightly bound protein-porphyrin complex isolated from the plasma of a patient with variegate porphyria.

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Free acid porphyrins were isolated from plasma of a patient with variegate porphyria. Part of the total porphyrin content--which included protoporphyrin IX, harderoporphyrin and uroporphyrin in a molar ratio of 1.2:1:0.5 and traces of pentacarboxylic porphyrin--was extractable with ethyl

Liver Transplantation because of Acute Liver Failure due to Heme Arginate Overdose in a Patient with Acute Intermittent Porphyria.

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In acute attacks of acute intermittent porphyria, the mainstay of treatment is glucose and heme arginate administration. We present the case of a 58-year-old patient with acute liver failure requiring urgent liver transplantation after erroneous 6-fold overdose of heme arginate during an acute

[Treatment of acute intermittent porphyria with a new protein-bound lyophilized hematin].

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Hematin (iron-III-protoporphyrin 9) has been used for several years in the treatment of acute attacks of inducible porphyrias. Three patients were treated with a new, albumin-bound lyophilized hematin preparation (manufactured by Behringwerke). In all patients it achieved a reduction in the urine of

Acute intermittent porphyria: vector optimization for gene therapy.

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BACKGROUND Acute intermittent porphyria (AIP) is an autosomal dominant disorder caused by the half-normal activity of hydroxymethylbilane synthase (HMB-synthase). Affected individuals can experience episodic, life-threatening, acute neurological attacks that are precipitated by various drugs,

[Zinc and porphyria cutanea tarda].

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In 30 patients affected by Porphyria Cutanea Tarda we determined: plasmatic, erythrocytary and urinary zinc (by atomic absorption spectrophotometry); total porphyrins in plasma and in urines, coproporphyrins and protoporphyrins in erythrocytes (by spectrophotometric methods); haptoglobin, hemopexin,

The role of pentachlorophenol in causing mitochondrial derangement in hexachlorobenzene induced experimental porphyria.

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Hexachlorobenzene feeding to rats for 60 days to induce experimental porphyria resulted in partial and constant uncoupling of oxidative phosphorylation of liver mitochondria from the early phase (i.e. 20 days) of treatment. Direct experimental evidence has been presented that this uncoupling is

[Treatment of porphyria cutanea tarda using plasma exchange].

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Results in the treatment of 5 patients with porphyria cutanea tarda by plasma exchange are presented. The procedure was applied every second or third day, 2-4 procedures in total. Saline solution, 5% or 12% albumin were used as plasma substitutes. The treatment lead to rapid fall in urine porphyrin

Correlation between levels of free and protein-bound plasma porphyrin and urinary porphyrins in porphyria cutanea tarda.

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Porphyria cutanea tarda (PCT) is a disorder of porphyrin metabolism that leads to massive overproduction and excretion of uroporphyrin. Most plasma porphyrins are bound to albumin and hemopexin. The aim of this work was to analyze the relationship between the concentrations of serum albumin and

Protein binding of salicylate in cutaneous hepatic porphyria.

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(1) Plasma protein binding of salicylate was studied in 14 patients with cutaneous hepatic porphyria (CHP) and 11 normal subjects using ultrafiltration with centrifugation (membrane cones) and continuous ultrafiltrations. (2) Albumin and haemoglobin levels were significantly reduced in patients with

Iron and porphyria cutanea tarda.

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In order to evaluate the pathogenetic role of iron in Porphyria cutanea tarda (PCT), the metabolism of iron was studied in 440 patient with PCT and associated chronic liver disease (CLD) and in 91 nonporphyric CLD patients (used as a control group). The parameters considered were the following:

Hemodialysis: a therapeutic option for severe attacks of acute intermittent porphyria in developing countries.

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Acute intermittent prophyria (AIP) is an autosomal dominant disease that results from a defect in the enzyme porphobilinogen deaminase. Acute intermittent porphyria is the most common of hepatic porphyrias and can tax the therapeutic capabilities of the physician to the limit. Motor weakness is a

Structural and functional properties of rat liver mitochondria in hexachlorobenzene induced experimental porphyria.

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A possible link between changes in iron and porphyrin content in liver mitochondria, from rats treated with either hexachlorobenzene, iron, or hexachlorobenzene plus iron, as a function of treatment time and their structural-functional properties, has been investigated. Normal oxidative

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