Porphyria variegata (or South-African porphyria) is not a rare disease in Europe. Its transmission, in a Walloon family, is described. The pedigree extends over 7 generations and comprises 184 individuals. It was possible to carry out biological studies on 100 family members. The results of the analyses are presented and discussed. The levels of fecal porphyrines, the presence of cutaneous lesions and the genealogical relationships permitted the establishment of a coherent picture. Three other families suffering from the same disease are briefly reported. A systematic search for carriers of the gene is indispensable in porphyrias. All identified carriers must be warned of the risks they run and must be supplied with a list of medicaments to be avoided. The prevention of cutaneous lesions by beta-carotene is envisaged.

Adult↗

PubMed1977

New perspectives for laboratories of clinical toxicology.

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Binding Sites↗

PubMed1977

[The bronze-baby-syndrome: a complication of phototherapy (author's transl)].

A case of intense grey-brown discoloration of the skin, serum and urine in a premature twin is reported when phototherapy was used to reduce hyperbilirubinemia. All features made the diagnosis of bronze-baby-syndrome most probable. We were able to detect an increased level of coproporphyrin in serum. The patient did not show any signs of a kernicterus. Possible speculations about the pathogenetic and prognostic value of elevated levels of coproporphyrins in newborn infants under treatment with phototherapy are discussed. In these cases we must be aware of possible neurotoxic effects, since increased concentrations of bilirubin breakdown products may not be excreted as well through the normal biliary passage as normally.

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PubMed1977

[Total mercury and methylmercury in canned fish of Turkish origin].

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Animals↗

PubMedDate not supplied

Death through injection of barbiturates.

The authors report two cases of lethal intoxication due to barbiturates in two male individuals, respectively 24 and 35 years old. They stress the comparatively rare mode of administration of such drugs in the absence of another party, i.e. the parenteral way.

Adult↗

PubMedDate not supplied