PMID: 7253546Jul 1, 1981

The "glucose effect" in acute hepatic porphyrias and in experimental porphyria

Klinische Wochenschrift
M Doss, F Verspohl

Abstract

The "glucose effect" was investigated in human acute hepatic porphyrias (acute intermittent porphyria, variegate porphyria, coproporphyria and porphobilinogen synthase defect porphyria) and in avian liver cells. 8 patients (7 women) with acute abdominal-neurological porphyria syndrome and 3 patients (2 women) in the remission phase were treated with high carbohydrate intake (approximately 500 g/24 h), mainly in form of intravenous glucose infusions. The biochemical response with a decrease of metabolites of porphyrin biosynthesis was highly significant, accompanied by clinical improvement in 10 courses of 9 patients. Two patients with delayed detection of the disease under the condition of Landry paralysis died after temporary clinical improvement due to ventricular arrythmias in one case and septicemia in the other. The importance of early diagnosis and therapy, and omission of drugs and alcohol cannot be overemphasized. Complementary studies show the "glucose effect" in drug -mediated induction of porphyrin synthesis in liver cells grown in culture: delta-Aminolevulinic acid synthase and protoporphyrin synthesis are repressed.

References

Dec 4, 1976·Lancet·G H ElderN Thomas
Sep 2, 1978·Lancet·J M LamonD P Tschudy
Sep 12, 1979·Biochemical and Biophysical Research Communications·G SrivastavaW H Elliott
Sep 1, 1977·Medicine·D M Becker, S Kramer
Dec 1, 1975·Annals of Internal Medicine·D P TschudyC R Magnussen
Apr 1, 1976·Metabolism: Clinical and Experimental·H L BonkowskyD P Tschudy
Oct 30, 1971·Lancet·M Doss
Feb 1, 1971·Proceedings of the National Academy of Sciences of the United States of America·D S FeldmanC J Watson
Aug 17, 1967·The New England Journal of Medicine·K B KnudsenF Lecocq
Dec 1, 1972·Scandinavian Journal of Clinical and Laboratory Investigation·B LundhE Cavallin-Stahl
Jun 15, 1972·The New England Journal of Medicine·U A MeyerH S MARVER
Sep 1, 1971·Acta Medica Scandinavica·A W Sorensen, T K With
Nov 1, 1971·Proceedings of the National Academy of Sciences of the United States of America·H L BonkowskyC J Watson
Jul 1, 1968·Metabolism: Clinical and Experimental·M G PERLROTHA G Redeker
Feb 15, 1968·The New England Journal of Medicine·A Kappas
Sep 8, 1966·Biochemical and Biophysical Research Communications·A D WaxmanD P Tschudy
Mar 1, 1967·Medicine·B F Felsher, A G Redeker
Jan 1, 1980·The International Journal of Biochemistry·M J BrennanS Kramer
Jan 1, 1980·The International Journal of Biochemistry·S G Smith, M A El-Far
Jan 1, 1980·The International Journal of Biochemistry·M R Moore
Apr 3, 1980·The New England Journal of Medicine·D A Brenner, J R Bloomer
Aug 15, 1980·Klinische Wochenschrift·C A PierachC J Watson
May 1, 1964·Metabolism: Clinical and Experimental·D P TschudyG HUNTER
Apr 1, 1965·Proceedings of the National Academy of Sciences of the United States of America·D P TschudyM RECHCIGL

Citations

Mar 19, 1999·Journal of Internal Medicine·C AnderssonF Lithner
Nov 6, 2014·Metabolites·Siddesh BesurHerbert L Bonkovsky
Sep 1, 1985·Journal of Clinical Chemistry and Clinical Biochemistry. Zeitschrift Für Klinische Chemie Und Klinische Biochemie·M DossF Verspohl
Nov 26, 2010·Der Internist·U StölzelM O Doss
Apr 24, 2010·Applied Spectroscopy·Grasiele FauazMaria Helena Bellini

Related Concepts

Acute Disease
Metazoa
Chick Embryo
Anhydrous Dextrose
Parenteral Infusion
Disorders of Porphyrin Metabolism
Porphyrins

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