Behavioural profile and maternal stress in Greek young children with Williams syndrome

Abstract

The consistency of the socio-emotional characteristics of children with WS across cultures and developmental stages implies a strong influence of the genetic phenotype. However, Greek mothers avoided to characterize these behaviours as pathological. Implications of these findings for clinical practice are also discussed.

Behavioral Profiles of Children With Williams Syndrome From Spain and the United States: Cross-Cultural Similarities and Differences

“…The proportion of individuals with elevated T-scores for Total Problems was 30% or higher in every study except the one with the youngest participants (Papaeliou et al, 2012) – which also was the only one of the non-US studies that used country-specific norms – and the level was higher than expected even in that study. The proportion of participants with elevated T-scores for the Internalizing and Externalizing Problems higher-order factors was higher than expected in nine of the 10 studies.…”

“…All of these studies were cross-sectional. Studies are ordered by the approximate mean chronological age (CA) of the sample, which ranged from 5.13 years for Papaeliou et al (2012) and 6.13 years for Fidler et al (2000) to 18.16 years for Porter et al (2009) and 28.23 years for Fu (2012; Fu et al, 2015). Note that although the last three studies in the table included adults in their samples, the oldest age for which the CBCL is normed is 18 years.…”

“…Basically, the proportion of participants with elevated T-scores (≥65) for a particular scale was considered “typical” if it was less than 15% (approximately twice as high as the proportion for the norming sample), “high” if it was between 15% and 29% (approximately three times as high as the proportion for the norming sample), “very high” if it was between 30% and 49%, and “extremely high” if it was 50% or higher. The T-scores reported by Papaeliou et al (2012) were based on the Greek norms; the T-scores for the remaining studies were based on the US norms.…”

“…The 11 published studies of the CBCL include six based on United States (US) samples (Dilts, Morris, & Leonard, 1990; Fidler, Hodapp, & Dykens, 2000; Fu, 2012 – also reported in Fu, Lincoln, Bellugi, & Searcy, 2015; Greer, Brown, Pai, Choudry, & Klein, 1997; Klein-Tasman, Lira, Li-Barber, Gallo, & Brei, 2015; Pagon, Bennett, LaVeck, Stewart, & Johnson, 1987), and one study each from Australia (Porter, Dodd, & Cairns, 2009); Brazil (Teixeira, Monteiro, Velloso, Kim, & Carreiro, 2010), Germany (Sarimski, 1997), Greece (Papaeliou et al, 2012), and Spain (Pérez-García, Granero, Gallastegui, Pérez-Jurado, & Brun-Gasca, 2011). Most of these studies used the CBCL 4–18 (Achenbach, 1991), which included eight empirically-derived problem (“syndrome”) scales based on factor analysis of parental responses to individual items.…”

“…Most of these studies used the CBCL 4–18 (Achenbach, 1991), which included eight empirically-derived problem (“syndrome”) scales based on factor analysis of parental responses to individual items. The more-recent CBCL 6–18 (Achenbach & Rescorla, 2001) and/or CBCL 1½-5 (Achenbach & Rescorla, 2000), which provide both empirically-based scales and DSM-oriented scales, were used by Porter et al (2009), Teixeira et al (2010), Papaeliou et al (2012), and Klein-Tasman et al (2015).…”