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Background Numerous studies have assessed the socio-cognitive profile in Williams syndrome (WS) and, independently, in 22q11.2 deletion syndrome (22q11.2DS). Yet, a cross-syndrome comparison of these abilities between individuals with these two syndromes with known social deficits has not been conducted. Methods Eighty-two children participated in four study groups: WS ( n = 18), 22q112.DS ( n = 24), age-matched individuals with idiopathic developmental disability (IDD; n = 20) and typically developing (TD) controls ( n = 20). Participants completed four socio-cognitive tests: facial emotion recognition, mental state attribution, differentiating real from apparent emotions and trait inference based on motives and actions-outcomes. Results The current findings demonstrate that children with WS were better in labelling happy faces compared with children with 22q11.2DS, partially reflecting their exaggerated social drive. In the false belief task, however, the WS and IDD groups performed poorly compared with the 22q11.2DS group, possibly due to their difficulty to interpret subtle social cues. When asked to identify the gap between real-negative vs. apparent-positive emotions, the 22q11.2DS group performed similarly to TD children but better than the WS group, possibly due to their anxious personality and their innate bias towards negatively valence cues. Finally, individuals with WS were more willing to become friends with a story character even when the character's motives were negative, reflecting their difficulty to avoid potentially harmful real-life situations. Conclusions Overall, our multi-facet socio-cognitive battery uncovered strengths and weaknesses in social cognition that are syndrome-specific, shared among the genetic syndromes, or common to the three clinical groups compared with healthy controls. Our findings underscore the need to devise age-specific and condition-specific assessment tools and intervention programs towards improving these children's socio-cognitive deficits. [ABSTRACT FROM AUTHOR]

Aim This study aimed to assess the progression-free and overall survival of patients with residual microscopic disease following neoadjuvant chemoradiotherapy and rectal resection for locally advanced rectal cancer. Method Two-hundred and thirty-four consecutive rectal cancer patients who had neoadjuvant chemoradiotherapy followed by radical resection (from May 2000 to April 2012) were divided according to pathological tumour response: residual microscopic disease ( MIC), complete response (pCR) and partial/no response (non- CR). Data on the neoadjuvant regime, treatment-to-surgery interval, final pathology, type of operation, operative time, postoperative complications, length of hospital stay, disease recurrence and mortality were compared between the groups. Results There were 13 (5.5%) MIC patients, 48 (20.5%) with pCR and 173 (73.9%) with non- CR group. The groups were demographically comparable. MIC patients had more retrieved lymph nodes compared with the non- CR and pCR patients (median 13 compared with 8 and 10, respectively, P = 0.0086). The 5-year overall survival rates were 93.4% for the pCR and MIC patients vs 82.1% for the non- CR patients ( P = 0.0324). The 5-year progression-free survival was 85.2% for the pCR and MIC patients vs 73.8% for the non- CR patients ( P = 0.086). Conclusion We have identified and assessed a new pathological subgroup of rectal cancer patients who had residual microscopic disease after neoadjuvant therapy. The survival analysis aligned them closely with pCR patients. [ABSTRACT FROM AUTHOR]

Background: Recent reports showed that children born with intrauterine growth restriction (IUGR) are at greater risk of experiencing verbal short-term memory span (STM) deficits that may impede their learning capacities at school. It is still unknown whether these deficits are modality dependent. Methods: This long-term, prospective design study examined modality-dependent verbal STM functions in children who were diagnosed at birth with IUGR ( n = 138) and a control group ( n = 64). Their STM skills were evaluated individually at 9 years of age with four conditions of the Visual–Aural Digit Span Test (VADS; Koppitz, 1981 ): auditory–oral, auditory–written, visuospatial–oral and visuospatial–written. Cognitive competence was evaluated with the short form of the Wechsler Intelligence Scales for Children – revised (WISC-R95; Wechsler, 1998 ). Results: We found IUGR-related specific auditory-oral STM deficits ( p < .036) in conjunction with two double dissociations: an auditory-visuospatial ( p < .014) and an input–output processing distinction ( p < .014). Cognitive competence had a significant effect on all four conditions; however, the effect of IUGR on the auditory-oral condition was not overridden by the effect of intelligence quotient (IQ). Conclusions: Intrauterine growth restriction affects global competence and inter-modality processing, as well as distinct auditory input processing related to verbal STM functions. The findings support a long-term relationship between prenatal aberrant head growth and auditory verbal STM deficits by the end of the first decade of life. Empirical, clinical and educational implications are presented. [ABSTRACT FROM AUTHOR]

This study examines long-term effects of antenatal management of intrauterine growth restriction (IUGR) on developmental outcome and on maternal coping using a prospective cross-sectional design. Sixty-nine families were evaluated using psychological testing and risk questionnaires. The effects of timing of diagnosis (prenatal/perinatal) and of pregnancy management [induction of labor (IL)/conservative management (CM)/none, i.e., diagnosed-at-birth (DaB)] on maternal stress were tested at 6 years' postbirth. In general, prenatal management protocols of IUGR were efficient in preventing major disabilities; however, 49% of the variance in maternal stress at 6 years' postbirth could be attributed to the child's presenting behavior and to pregnancy management of IUGR condition. Mothers who received CM treatment reported being more stressed by their child's poor emotional adjustment (ps < .01–.002) and distractibility (p < .029), and to have more difficulty in accepting them (p < .01). Prenatal psychological consultation to better handle stress for parents whose fetus is diagnosed with IUGR is recommended, particularly when pregnancy is managed conservatively and familial–educational resources are low. [ABSTRACT FROM AUTHOR]

Although research has demonstrated poor visual skills in premature infants, few studies assessed infants' gaze behaviors across several domains of functioning in a single study. Thirty premature and 30 full-term 3-month-old infants were tested in three social and nonsocial tasks of increasing complexity and their gaze behavior was micro-coded. In a one-trial version of the visual recognition paradigm, where novel stimuli were paired with familiar stimuli, preterm infants showed longer first looks to novel stimuli. In the behavior response paradigm, which presented infants with 17 stimuli of increasing complexity in a predetermined "on-off" sequence, premature infants tended to look away from toys more during presentation. Finally, during mother-infant face-to-face interaction, the most dynamic interpersonal context, preterm infants and their mothers displayed short, frequent episodes of gaze synchrony, and lag-sequential analysis indicated that both mother and infant broke moments of mutual gaze within 2 sec of its initiation. The propotion of look away during the behavior response paradigm was related to lower gaze synchrony and more gaze breaks during mother-infant interactions. Results are discussed in terms of the unique and adaptive gaze patterns typical of low-risk premature infants., (Copyright © International Society on Infant Studies (ISIS).)

Although feeding problems are common during infancy and are typically accompanied by relational difficulties, little research observed the mother-infant feeding relationship across the first year as an antecedent to the development of feeding difficulties. We followed 76 low-risk premature infants and their mothers from the transition to oral feeding in the neonatal period to the end of the first year. Prior to hospital discharge, microlevel patterns of maternal touch and gaze were coded during feeding and nonfeeding interactions, global patterns of maternal adaptation were assessed, and infants' neurobehavioral status was tested. Psychomotor development was evaluated at 4 months. At 1 year, feeding difficulties were determined on the basis of maternal interview and direct observations of feeding interactions. Mothers of infants who exhibited feeding difficulties at 1 year showed less affectionate touch and gaze during nonfeeding interactions and more gaze aversion and lower adaptability during feeding interactions already in the neonatal period. Infants with feeding difficulties demonstrated poorer psychomotor performance at 4 months. Feeding interactions of infants with feeding difficulties at 1 year were characterized by higher maternal intrusiveness, lower infant involvement, and greater infant withdrawal. Less maternal affectionate touch and lower maternal adaptation in the neonatal period, poor infant psychomotor skills, and higher maternal intrusiveness and lower infant involvement at 1 year predicted feeding difficulties. The findings underscore the role of the relational components across the first year in the development of feeding difficulties., (2009 International Society on Infant Studies.)