Reciprocal relations across time between basic values and value-expressive behaviors: A longitudinal study among children

Tracing children’s values and value-expressive behavior over a sixth-month period, we examined stability and change of values and behavior and the reciprocal relations between them. Three hundred and ten sixth-grade students in Italy completed value and value-expressive behavior questionnaires three times in three-month intervals during the scholastic year. We assessed Schwartz's (1992) higher-order values of conservation, openness to change, self-enhancement, and self-transcendence, as well as their respective expressive behaviors. Reciprocal relations over time between values and behaviors were examined using a cross-lagged longitudinal design. Results showed that values and behaviors had reciprocal longitudinal effects on one another, after the stability of the variables was taken into account (i.e., values predicted change in behaviors, but also behaviors predicted change in values). Our findings also revealed that: (1) values were more stable over time than behaviors, and (2) the longitudinal effect of values on behaviors tended to be stronger than the longitudinal effect of behaviors on values. Findings are discussed in light of the recent developmental literature on value change.

Values in middle childhood: Social and genetic contributions

Theories of value development often identify adolescence as the period for value formation, and cultural and familial factors as the sources for value priorities. However, recent research suggests that value priorities can be observed as early as in middle childhood, and several studies, including one on preadolescents (Knafo & Spinath, 2011), have suggested a genetic contribution to individual differences in values. In the current study, 174 pairs of monozygotic and dizygotic 7-year old Israeli twins completed the Picture-Based Value Survey for Children (PBVS–C; Döring et al., 2010). We replicated basic patterns of relations between value priorities and variables of socialisation – gender, religiosity, and socioeconomic status– that have been found in studies with adults. Most important, values of Self-transcendence, Self-enhancement, and Conservation, were found to be significantly affected by genetic factors (29%, 47% and 31% respectively), as well as non-shared environment (71%, 53% and 69% respectively). Openness to change values, in contrast, were found to be unaffected by genetic factors at this age and were influenced by shared (19%) and non-shared (81%) environment. These findings support the recent view that values are formed at earlier ages than had been assumed previously, and they further our understanding of the genetic and environmental factors involved in value formation at young ages.

Detailed phenotypic and genotypic characterization of bietti crystalline dystrophy

OBJECTIVE: To provide a detailed phenotype/genotype characterization of Bietti crystalline dystrophy (BCD). DESIGN: Observational case series. PARTICIPANTS: Twenty patients from 17 families recruited from a multiethnic British population. METHODS: Patients underwent color fundus photography, near-infrared (NIR) imaging, fundus autofluorescence (FAF) imaging, spectral domain optical coherence tomography (SD-OCT), and electroretinogram (ERG) assessment. The gene CYP4V2 was sequenced. MAIN OUTCOME MEASURES: Clinical, imaging, electrophysiologic, and molecular genetics findings. RESULTS: Patients ranged in age from 19 to 72 years (median, 40 years), with a visual acuity of 6/5 to perception of light (median, 6/12). There was wide intrafamilial and interfamilial variability in clinical severity. The FAF imaging showed well-defined areas of retinal pigment epithelium (RPE) loss that corresponded on SD-OCT to well-demarcated areas of outer retinal atrophy. Retinal crystals were not evident on FAF imaging and were best visualized with NIR imaging. Spectral domain OCT showed them to be principally located on or in the RPE/Bruch's membrane complex. Disappearance of the crystals, revealed by serial recording, was associated with severe disruption and thinning of the RPE/Bruch's membrane complex. Cases with extensive RPE degeneration (N = 5) had ERGs consistent with generalized rod and cone dysfunction, but those with more focal RPE atrophy showed amplitude reduction without delay (N = 3), consistent with restricted loss of function, or that was normal (N = 2). Likely disease-causing variants were identified in 34 chromosomes from 17 families. Seven were novel, including p.Met66Arg, found in all 11 patients from 8 families of South Asian descent. This mutation appears to be associated with earlier onset (median age, 30 years) compared with other substitutions (median age, 41 years). Deletions of exon 7 were associated with more severe disease. CONCLUSIONS: The phenotype is highly variable. Several novel variants are reported, including a highly prevalent substitution in patients of South Asian descent that is associated with earlier-onset disease. Autofluorescence showed sharply demarcated areas of RPE loss that coincided with abrupt edges of outer retinal atrophy on SD-OCT; crystals were generally situated on or in the RPE/Bruch's complex but could disappear over time with associated RPE disruption. These results support a role for the RPE in disease pathogenesis.