Psychological evaluation of treated females with virilizing congenital adrenal hyperplasia.

The psychological development of females with congenital adrenal hyperplasia (CAH) has been previously studied by Money, et al, who found that psychological development of sex identity was consistent with sex assignment despite virilizing adrenal hormones and abnormal external genitalia requiring surgical correction. In this study, using a variety of psychological tests, we assessed the sex-dimorphic behavior, body image, cognitive functioning, and sex-role identity of nine patients ranging in age from 13 to 21, all treated with glucocorticoids and surgical correction. Four of the nine showed moderate virilization despite treatment. Psychological measures included the Wechsler Intelligence Scale for Children-Revised (WISC-R), the Bem Sex-Role Inventory, the Draw-A-Person (DAP) and an interview with patient and family. Results indicate that patients fall within the normal expectable range for this developmental period in visual-spatial and verbal cognitive functioning, in sex-role identity, and in social interpersonal early behaviors. In two areas of functioning these patients demonstrated some variance from the norms, specifically in sexual identity and early activity levels. This suggests that sexual identity and physical activity are most prone to hormonal and psychological impact but that cognition and sex-role identity are not affected. Future studies of this sample will look at personality dimensions such as ego functioning, defense and affect to consider the impact of body image concerns and conflicts.

Relationships in families of children and adolescents with sickle cell disease.

This study represents the findings from interviews and assessments of children and adolescents with sickle cell disease and their parents regarding the quality of family relations, the degree to which sickle cell disease has impacted on these relations, and the variables which contribute to these relations. With 70 families as respondents, the data reveal a wide variance in quality of relations, but a broad base of positive relations. Parent report tends to be more positive than child report, both in terms of quality of relations and the impact of sickle cell disease on relations. Social support and knowledge about the disease are significant contributors to positive relations, while socioeconomic status, family structure, and illness severity are not predictive of quality of relations. With age and gender as covariates, results indicate that the families of girls tended to have more positive relations. The data suggest approaches to family and community support in order to help families maintain and build relations in the face of the stresses which sickle cell disease imposes.

Psychological findings in early treated cases of female pseudohermaphroditism caused by virilizing congenital adrenal hyperplasia.

Nine female adolescents with female pseudohermaphroditism resulting from virilizing congenital adrenocortical hyperplasia (CAH) were studied in terms of gender identity, sex-role behavior, psychological adjustment, and psychosexual development. A group of adolescents with chronic illness was used as a control. The Draw-A-Person, the Bem Sex-Role Inventory, Rorschach, TAT, and a questionnaire reviewing peer and romantic activities were administered to both groups. The two groups were comparable on measures of general personality adjustment, with the CAH girls showing a trend toward greater bodily concerns. Sex-role identity for both groups was near the adolescent girl norms for both Femininity and Masculinity, with virilized CAH girls showing slightly higher Androgyny scores. Significant differences were found on gender identity as measured by greater differentiation of the drawn male figure as well as a trend toward drawing the male figure first. The CAH females also showed consistent patterns of psychosocial delay in dating and sexual relations as compared to the control group. Gender identity in this group appears to be mediated by body image. The resulting ambivalence may be evidence of feelings of incompetence, leading to resistance to social interactions and goals involving intimacy and nurturance.

Relation between severity of chronic illness and adjustment in children and adolescents with sickle cell disease.

Tested the relationship between illness severity and adjustment in 70 sickle cell patients, aged 8-16. Illness severity was measured by frequency of hospitalization and emergency room visits, frequency and intensity of pain crises, and duration of illness. Adjustment variables were IQ, self-esteem, social and personal adjustment, behavioral problems, school performance, and peer relations. Medical variables-associated illness patterns and type of hemoglobinopathy-were also considered. Sex and age were covariates. The hypothesis that illness severity would affect adjustment was generally not supported. Other factors such as SES, family structure, or support systems may affect adjustment and suppress the hypothesized relationship. The need for a more global intervention approach than using illness severity to measure need for psychotherapeutic assistance is suggested.

Cognitive mediators of ego functioning in adolescence.

It has often been assumed that a relationship exists between higher levels of cognitive functioning, particularly formal operations, and mature ego functioning in adolescence. This research examined the relationships between ego functioning and two domains of operational thinking: social interpersonal reasoning and physical-mathematical reasoning in 139 high school seniors. Subjects were given two measures of physical-mathematical reasoning, two measures of interpersonal reasoning, and the Sentence Completion Test of ego functioning, as well as a measure of verbal intelligence. Results indicated significant differences between males and females in patterns of correlations as well as in patterns of relationships in a causal analysis. Ego functioning was predicted by interpersonal reasoning for females and by physical-mathematical reasoning and verbal intelligence for males.

Functional outcomes of children with sickle-cell disease affected by stroke.

The nature and degree of functional recovery after stroke in children with sickle-cell disease (SCD) has not been extensively investigated. The purpose of this study was to evaluate retrospectively the functional status of 14 SCD children who had had strokes and to compare them with age-matched and gender-matched SCD children who had not had strokes. By doing so, we would be able to quantify the eventual physical and cognitive functional outcomes of survivors of stroke secondary to SCD and assess the impact of stroke on these patients. These children (five boys and nine girls) with SCD and stroke(s) were 11.6 +/- 4.3 years of age (range five to 18 years). They experienced one to three strokes at a mean age of 6.1 +/- 5.2 years (range one to 17 years). A series of tests were administered to these subjects to evaluate physical and psychosocial functions. These tests were performed at least one year after the latest stroke. This study showed that all of the SCD-stroke children were physically independent. Only a few had impairments of hand functions and mild difficulties in self-care activities. However, most of these children demonstrated intellectual deficits ranging from borderline to moderate mental retardation, reduced language functions ranging from low normal to retarded range, and problems in adjustment. Intelligence quotient of the children with SCD-stroke(s) was significantly lower than those of age-matched and gender-matched nonstroke SCD children, suggesting that stroke caused an adverse effect on the cognitive functioning of these children. The results indicate that in the SCD-stroke children psychosocial deficits outweighed physical disabilities.(ABSTRACT TRUNCATED AT 250 WORDS)