The effect of diet in variegate (South African genetic) porphyria

Perlroth, Mark G.; Tschudy, Donald P.; Ratner, Arnold C.; Spaur, William; Redeker, Allan G.

doi:10.1016/0026-0495(68)90015-2

Cited by 34 publications

(7 citation statements)

References 27 publications

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“…Fasting is known to be a causative factor in acute attacks of hepatic porphyrias in patients with latent porphyria [11][12][13][14]. A high glucose intake ameliorates the clinical and biochemical signs and symptoms of the acute attack in many patients [14][15][16].…”

Section: Discussionmentioning

confidence: 99%

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An in vitro model demonstrating the glucose effect and the influence of fasting on porphyrin metabolism

et al. 1982

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Section: Discussionmentioning

confidence: 99%

“…A similar phenomenon, first described in rats was termed the 'glucose effect' [2]. Subsequently, it was shown that other carbohydrates have also a marked effect on porphyrin metabolism in humans with hepatic porphyria both in the latent phase and during attacks [12][13][14][15][16][17].…”

Section: Discussionmentioning

confidence: 99%

An in vitro model demonstrating the glucose effect and the influence of fasting on porphyrin metabolism

et al. 1982

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“…Administration of carbohydrates to rats markedly inhibits the induction of ALA synthase. It was then demonstrated that Symptoms and biochemical fmdings in variegate porphyria were rapidly reversed by intravenous glucose application (5). The observations were explained implying the "glucose effect" on hepatic ALA synthase.…”

Section: Introductionmentioning

confidence: 98%

"Glucose Effect" and Rate Limiting Function of Uroporphyrinogen Synthase on Porphyrin Metabolism in Hepatocyte Culture: Relationship with Human Acute Hepatic Porphyrias

Doss¹,

Sixel-Dietrich²,

Verspohl³

1985

Clinical Chemistry and Laboratory Medicine

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Doss, Sixcl-Dietrich and Verspohl: "Glucose effect" in hepatocyte culture and in acute hepatic porphyrias 505 J. Clin. Chem. Clin. Biochem. Vol. 23, 1985, pp. 505-513 (Received January 26/May 8, 1985) Summary: The effect of glucose on drug-promoted induction of porphyrin synthesis was studied in chick embryo liver cell culture with and without the addition of exogenous -aminolaevulinic acid (ALA). Induction of ALA synthase was abolished by haemin or glucose. Less than 10% 0f porphobilinogen is converted into protoporphyrin. Protoporphyrin synthesis cannot be enhanced by high ALA concentrations. The conversion of exogenous ALA into porphyrins decreases with increasing concentrations of ALA from 0.1 to 2.0 mmol/1, whereas porphobilinogen accumulates, thus reflecting the rate limiting function of Uroporphyrinogen synthase, which is not influenced by glucose. This needle-eye-like function of Uroporphyrinogen synthase within the porphyrin biosynthetic chain explains the urinary increase of ALA and porphobilinogen in the acute phase of variegate and coproporphyria, similar to that in acute intermittent porphyria. i * The "glucose effect" was also investigated in vivo in humans in 32 courses of hereditary acute hepatic . v porphyrias (acute intermittent porphyria, variegate porphyria, coproporphyria and porphobilinogen synthase defect porphyria). Patients were treated with high carbohydrate intake (~500 g/24 h), mainly in the form of glucose infusions. There was a resulting coiisistent and highly significant decrease of porphyrin biosynthesis metabolites, accompanied by clinical improvement in most of the patients. "Glucose-Effeki" und limitierende Funktion der Uroporphyrinogen-Synthase auf den Porphyrinstoffwechsel in der Leberzellkultur in Bezug auf die akuten hepatischen Pörphyrien des MenschenZusammenfassung: Die Wirkung von Glucose auf die Arzneimittel-vermittelte Induktion der Porphyrinsynthese wurde in Hühnerembryo^Leberzellkulturen unter Bedingungen mit endogener und exogener 5-Aminolävulinsäüre (ALS) studiert. Die Induktion der ALS-Synthase konnte durch Hämin oder Glucose aufgehoben werden. Wenjger als 10% Porphobilinogen wird in Porphyrine konvertiert. Die Synthese von Protoporphyrin kann unter hohen ALS-Konzentrationen nicht gesteigert werden. Die Umwandlung exogener ALS in Porphyrine fällt mit steigender Konzentration der ALS von 0,1 bis 2,0 mmol/1, während Porphobilinogen akkumuliert: Hierin spiegelt sich die Umsatz-limitierende Funktion der Uroporphyrinogen-Synthase, die nicht durch Glucose beeinflußt wird". Die Nadelöhr-ähnliche Funktion der Uroporphyrinogen-Synthase innerhalb der Porphyrinbiosynthesekette erklärt den Anstieg von ALS und Porphobilinogen im Urin in der akuten Phase der Porphyria variegata und der Koproporphyrie, welcher demjenigen bei akuter intermittierender Porphyrie ähnlich ist.Der "Glucose-Effekt" in vitro wurde mit dem in vivo beim Menschen verglichen und bei 32 Verläufen hereditärer akuter hepatischer Porphyrieii untersucht (akute intermittierende Porphyrie, Porphyria varie...

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“…In fact, the strict gluten-free diet had a beneficial effect on the abdominal symptoms (confirming the diagnosis of coeliac disease), on the skin lesions of porphyria and the urinary levels of porphyrins as well. Diet has been shown to influence the course of variegate porphyria in that both carbohydrate restriction and low caloric intake may precipitate an acute crisis of the disease ('glucose effect') [10,12]. The beneficial effect of the gluten-free diet on porphyria can be explained, therefore, by the restoration of the intestinal absorption of food and the consequent normal caloric intake.…”

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