Treatment of a six-year-old boy with porphyria congenita (Günther) by small amounts of chloroquine was followed by a sharp but transient increase of the urinary excretion of porphyrins. Moreover, a nearly complete normalization of the previously observed rigidity of the erythrocytes occurred. With respect to the possibility that this elevated rigidity plays an important role for the typical hemolysis connected with this porphyria, the application of chloroquine could be of therapeutic value for this disease.

Child↗

PubMed1978

The biosynthesis of haem in congenital (erythropoietic) porphyria.

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5-Aminolevulinate Synthetase↗

PubMed1977

[Determination of porphyrins in red blood cells. Comparison of two methods (author's transl)].

Two methods for measuring porphyrin concentrations in red blood cells were compared, one by Piomelli in which porphyrin is extracted from the RBCs and then measured fluorometrically, the other in which the blood-porphyrin content is measured direct with the hematofluorometer ZnP 400. Sample size is the same for both. Results in 59 patients exposed to lead were evaluated: the correlation coefficient between the two tests was 0.99.

Environmental Exposure↗

PubMed1976

[Erythropoietic protoporphyria: porphyrin content of a gall-stone (author's transl)].

In the case of a 48 year old woman with characteristic signs of erythropoetic protoporphyria, a solitary gall-stone which had persisted over a period of 10 years was removed. Porphyrin-concentration of this gall-stone was found to be 22.6 mcg/g, which is almost twenty times the concentration of other cholestrol-stones. Besides protoporphyrin, porphyrins with a higher degree of carboxylation were discovered in four different samples by thin layer chromatography. It is postulated that the increased bile-concentration of protoporphyrin in the liver causes the cristallisation of cholestrol and the formation and growth of gall-stones. The biochemical finding of porphyrins with a higher degree of carboxylation indicates that the porphyrins of the gall-stone may not only come from hemolysed erythrocytes but possibly also from metabolites of disturbed hepatic porphyrin biosynthesis.

Cholelithiasis↗

PubMedDate not supplied

Association of ethylene-oxide-induced IgE antibodies with symptoms in dialysis patients.

IgE-type antibodies to human serum albumin/ethylene oxide conjugates were determined in 153 patients (140 unselected, 13 selected for presence of eosinophilia) on regular dialysis treatment by radioallergosorbent test (RAST) techniques. In 10.7% of unselected patients and 30.8% of selected patients, RAST values suggested ethylene oxide sensitisation. High RAST values were commonly associated with anaphylactoid reactions during dialysis and with chronic asthma. Ethylene oxide antibodies should be sought routinely in patients presenting with these symptoms and the necessity of ethylene oxide sterilisation of disposable dialysis equipment should be re-evaluated.

Adult↗