Congenital adrenal hyperplasia. I: Gender-related behavior and attitudes in female patients and sisters.

Thirty-five female patients with congenital adrenal hyperplasia (CAH) were compared to a group of 16 healthy sisters in regard to gender-related behavioral patterns, present attitudes, and plans for the future. A semi-structured interview with the subjects, ages 11 to 41 yr, and their mothers concentrated on four to five age stages. Results of retrospective data from single items as well as from several related composite scales ("interests and behavior," "appearance," "overall scores") revealed significant group differences: Both in mother-assessment and self-assessment, CAH patients showed a "more masculine" orientation than their sisters, but this was far from consistent across all age stages, especially for single items. Unexpectedly, the gender-behavior differences between CAH patients and sisters did not hold for certain items and scales of "social behavior" (e.g., assertiveness, dominance, acceptance in peer groups) and, in contrast to some of the existing literature, also not for "high-energy expenditure." With regard to expectations for the future, CAH patients had less of a "wish to have their own children" and a higher preference for "having a career versus staying at home." Age, socioeconomic status, intelligence, and presence or absence of a sister as possibly intervening psychosocial/demographic factors could not explain the group differences in behavior. Degree of genital masculinization (Prader stages) or "onset and quality" of therapy as measures of pre- and postnatal androgenization, respectively, could also not account for the degree of the "more masculine" orientation in the CAH group. Nevertheless, the overall results are compatible with earlier findings on the masculinizing effects of prenatal androgens on behavior in humans and point to a time period after sexual differentiation of the genitalia and before birth as the most likely one for the effects of prenatal hormones on behavioral masculinization in humans.

Congenital adrenal hyperplasia. II: Gender-related behavior and attitudes in female salt-wasting and simple-virilizing patients.

The salt-wasting (SW) and simple-virilizing (SV) forms of congenital adrenal hyperplasia (CAH) are characterized by distinct prenatal hormonal milieus. To test whether these hormonal milieus differentially influence the development of a "more masculine" behavioral pattern in female CAH patients (Dittmann et al., 1990), SW patients (N = 13) were compared both to SV patients (N = 20) and healthy sisters of both groups (N = 16). The data are based on semi-structured interviews in which subjects (11-41 yr) and mothers were asked about aspects of "Gender-related interests and behavior," "Level of activity," "Social behavior," (reflecting e.g., assertiveness, dominance, and acceptance by peer groups) and "Appearance"; these areas of interest were represented by composite scales. On most scales, and by both mother-assessment and self-assessment, SW patients differed significantly from both SV patients and sisters in having a "more masculine" orientation. SW patients also showed a higher "Level of activity." These SW group results probably account for much of the CAH/sister differences reported in the companion article (Dittmann et al., 1990). In contrast, SV patients differed from the sister sample on only a few scales. There were no significant differences between SV and SW subjects in the degree of virilization of the external genitalia (indicating no group difference in prenatal androgenization). SW patients were treated "earlier" and "better" after birth (indicating less postnatal androgenization). However, these medical conditions, as well as several psychosocial/demographic variables, could not explain the group behavioral differences. These results do not support a primarily psychosocial explanation of behavioral development in CAH patients, especially those with the SW condition; they rather suggest differential organizational effects of two different hormonal environments (SV vs. SW) during critical periods of prenatal CNS development.

[Risk of chickenpox during pregnancy and the neonatal period]. / Risiko von Windpocken in der Schwangerschaft und Neonatalperiode.

A newborn fell ill from congenital chickenpox eight days after delivery, a chickenpox rash having been noted in the mother two days before delivery. Administration of zoster-hyperimmune serum to the mother at the onset of the rash and to the child immediately after birth had no demonstrable effect on the course of the disease. Autopsy, the baby having died on the eleventh day post-partum, revealed focal necrotizing pneumonia of all the lobes. Varicella-zoster virus was isolated from lung material. On the basis of present knowledge the risk of varicella infection in the fetus and neonate is 1-7 maternal cases per 10 000 pregnancies. Transplacental passage of the virus can occur at any time during pregnancy. A congenital varicella syndrome is very rare in the first two thirds of pregnancy, but bland infection of the fetus is also likely. Illness of the mother at the end of pregnancy leads to clinically manifest varicella in about a quarter of neonates. The death-rate of congenital varicella is up to 30% if the mother falls ill between the fourth day before and the second day after delivery. Neonatal varicella acquired post-partum takes a relatively mild course. The most important prophylactic measure, in addition to the administration of zoster-hyperimmune serum to the mother within three days of incubation, is to delay delivery past the critical period.