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Biomedical subjects

J G Koppe

Publications and source records attributed to J G Koppe.

At least 73 records · Page 4Linked to original sources

Sex-linked ichthyosis and placental sulphatase C deficiency.

Ichthyosis was diagnosed in six boys born from pregnancies in which placental steroid sulphatase deficiency was present. In four cases the diagnosis of sex-linked ichthyosis was certain, in the other two the ichthyosis was probably of the sex-linked type. No arylsulphatase C activity could be demonstrated histochemically either in the epidermis of these boys, or in the epidermis of adults with sex-linked ichthyosis, whilst it was present in normal controls. In cultured skin fibroblasts steroid-sulphatase activity was extremely low. We conclude that deficiency of steroid sulphatase C or arylsulphatase C or a third functionally linked enzyme is an important factor in the pathogenesis of sex-linked ichthyosis.

Adult↗

Baka, a new platelet-specific antigen involved in neonatal allo-immune thrombocytopenia.

A family is described in which the mother developed platelet-specific antibodies, not directed against the antigens of the Zw(PlA) or the Ko system. The antibodies were only detectable in the immumofluorescence test and the radioactive antiglobulin test on platelets, and proved to be mainly IgG1 antibodies, although weak IgM antibodies of the same specificity were also detected. By cross-absorption studies, investigation of the family and a small-scale population study, it appeared that the antibodies were directed against a new antigen which we have called Baka. The frequency of the Baka phenotype in the Dutch population was 90.76%, the calculated genotype frequency 0.696. The Baka phenotype was neither sex-linked nor, so far as evaluable, closely linked to other platelet, red-cell, granulocyte, or HLA groups. The first child of this mother died of neonatal thrombocytopenia, the second child was unaffected. During the second pregnancy, the titre of the antibodies did not rise. The platelets of this child were found not to carry the Baka antigen.

Antibody Formation↗

The influence of betamethasone and orciprenaline on the incidence of respiratory distress syndrome in the newborn after preterm labour.

In a randomized double-blind trial on antenatal corticosteroid treatment for the prevention of respiratory distress syndrome (RDS) a corticosteroid related beneficial effect was found. Possibly of more significance was the finding that the children born within 12 hours of their mother's admission to hospital showed a higher incidence of RDS than those born between 12 hours and one week after admission even in the placebo and untreated groups. Bèta-adrenergic drugs seemed to exert no other influence on the occurrence of RDS than can be explained by the delay of delivery. Prolonged ruptured membranes appeared to decrease the incidence of RDS to the same extent as other symptoms of threatened preterm labour.

Betamethasone↗

Effects of glucocorticoids during pregnancy on the outcome of the children directly after birth and in the long run.

We studied 17 mothers and the outcome of their 34 pregnancies. The purpose of this retrospective study was to determine possible adverse effects on growth of prolonged use of glucocorticoids, which have been recommended for short-term use in the prevention of respiratory distress syndrome (Liggins). There was no effect on duration of pregnancy; no congenital abnormalities were seen in this small group of children when corticoids were used during the whole pregnancy. There was a slight decrease in birth weight and placenta weight and, when known, also in length in almost every case. However, after long-term follow-up all of these children except 1 showed a catch-up growth and were slightly above normal. Their neurological and mental development was also normal except in 1 child who was born severely asphyxiated. We conclude from these results that there appears to be no adverse effect on later growth in the use of glucocorticoids during pregnancy. Therefore, it is unlikely that the short-term use of glucocorticoids as recommended by Liggins would have any influence on later growth.

Birth Weight↗