Martin Seip

INSECTICIDES IN HUMAN BREAST MILK

Abstract. Fifty samples of human breast milk were analysed by gas chromatography and thin layer chromatography for DDT (dichlordiphenyltrichloraethan), hexachlorobenzol, benezene‐hexachlorides, dieldrin, aldrin and heptachlorepoxide. The three first substances were found in all samples in amounts varying from quite small up to eleven times the WHO recommended maximum for cow milk. The other substances were found in fewer milk samples, however, in some of these samples they were found in relatively high amounts. In Norway, only DDT has been investigated earlier (4), and compared to that study, there has been no significant change in the mean concentration of DDT in human breast milk during the last five years. The insecticide content was highest in colostrum and decreased with increasing duration of lactation. Considerable fluctuations in the content of insecticides were recorded in repeated milk samples collected from the same woman a few days apart. The percentage of samples with insecticide content higher than that permitted for cows milk was greater in May/June (79%) than in early April (54%). The significance of these findings is discussed.

CONGENITAL ERYTHROPOIETIC PORPHYRIA WITH A HITHERTO UNDESCRIBED PORPHYRIN PATTERN

One proven and one probable case of CEP in second cousins from Northern Norway are reported. The porphyrin pattern in Case 1, who has been studied extensively, in certain respects differs from that reported earlier in CEP, indicating that the disease may be genetically heterogeneous. The most impressive finding is the extraordinary high amounts of 7 carboxylic porphyrin in urine. Isomer analysis has shown this porphyrin to belong to the isomer III series. The presence of increased amounts of protoporphyrin in plasma and feces is also at variance with the picture seen in classical CEP.

A Comparison of Hemoglobin and Erythrocyte Values in the First‐horn and the Second‐born Twin, and in First, Second and Third Triplet during the Neonatal Period

The last‐born twin or triplet usually has a higher hemoglobin and erythrocyte content of the peripheral blood during the first two weeks of life than twin no. 1 (triplets nos. 1 and 2). This is assumed to be due to an extra “blood transfusion” to the last‐born twin or triplet from the placenta and umbilical vessels after birth, before the clamping of the cord, caused by the more complete contraction of the uterus when the last fetus is also delivered. The last‐born twin or triplet therefore is likely to have a somewhat greater iron reserve at birth.

CONGENITAL ERYTHROPOIETIC PORPHYRIA: The Effect of Light Shielding

The effect of various types of measures taken for light shielding against daylight in a recently detected case of CEP, has been followed for more than a year. It has been found that the celluloid film, “Para‐Sol”, which has a good translucency for wavelengths above 550 nm, but is not translucent to wavelengths below 510 nm, not only removes the troublesome skin lesions seen in this disease, but also. brings about an almost complete compensation of the anemia, disappearance of the splenomegaly and marked changes in the blood and urinary porphyrin patterns. In blood the erythrocyte porphyrins change from predominantly proto‐porphyrin with trace amounts of copro‐ and not detectable uroporphyrin, to high amounts of both proto‐, copro‐ and uroporphyrins. The plasma porphyrins remain essentially unchanged. The urinary pattern changes from predominantly free porphyrins to predominantly porphyrinogens.

Dietary treatment of cystinosis.

The amino acid pattern in the urine and serum was investigated in two cases of cystinosis before and on treatment with a diet low in methionine and cystine. The cerebrospinal fluid and the liver were studied two months after dietary treatment was started. The urinary amino acids in both cases were increased, similarly as in the Fanconi syndrome. Some of the amino acids occurred in elevated amounts in serum. The aminoaciduria, therefore, must be extrarenal as well as renal in origin. The treatment with the methionine and cystine poor diet did not produce a great change in the concentration of the urinary and serum amino acids, but the amino acids showed a slight tendency towards normalization. The cerebrospinal fluid and the liver showed high levels of cystine.

THE EFFECT OF VARIOUS THERAPEUTIC TRIALS ON THE PORPHYRIN EXCRETION IN A CASE OF CONGENITAL ERYTHROPOIETIC PORPHYRIA

ABSTRACT: Eriksen, L. and Seip, M. (Institute of Physiology, University of Oslo, and Department of Pediatrics, University Hospital, Oslo, Norway). The effect of various therapeutic trials on the porphyrin excretion in a case of congenital erythropoietic porphyria. Acta Paediatr Scand 64:287, 1975.–A patient with a biochemically “new” type of congenital erythropoietic porphyria has been studied under various therapeutic trials. Splenectomy had no demonstrable effect on porphyrin excretion or clinical picture. Vitamin E caused a moderate fall in porphyrin excretion, however, there was no significant improvement in light tolerance and tendency to hemolysis. β‐carotene reduced skin photosensitivity appreciably, while total porphyrin excretion remained unchanged and the tendency to develop hemolytic anemia showed only slight improvement. Red cell transfusion caused a rapid, dramatic fall in porphyrin excretion (in 4–5 days) and a transient increase in light tolerance, while the distribution of the different porphyrins excreted remained unchanged. These observations indicate that all or nearly all the abnormal porphyrins excreted are of erythropoietic origin, and that the overwhelming part of the porphyrins originate from an abnormal population of shortlived red cells. Findings on fluorescence microscopy of blood and bone marrow support this view. Meticulous protection against light of the shorter wavelengths caused a similar rise in hemoglobin level as produced by red cell transfusion, however, in this instance the total excretion of porphyrins did not fall. It is suggested that the inhibitory effect of transfusion on erythropoiesis (and thereby porphyrin excretion) might be due partly to a depression of erythropoietin formation, partly to the presence of an erythropoiesis inhibiting factor (chalone) in the transfused red cells.

Hair amino acids in cystinosis, homocystinuria, Fölling's disease and tyrosinosis.

The amino acid content of the hair was determined in patients with cystinosis, homocystin‐uria, phenylketonuria and tyrosinosis, and in two normal controls. No significant increases of cystine, homocystine, methionine, phenylalanine or tyrosine were found. Analysis of hair amino acids therefore does not seem to give any information about the diagnosis in these disorders.