Parent insights into atypicalities of social approach behaviour in Williams syndrome.

BACKGROUND: Individuals with Williams syndrome have been reported to show high levels of social interest and a desire to interact with others irrespective of their familiarity. This high social motivation, when combined with reduced intellectual capacity and a profile of atypical social behaviour, is important in terms of social vulnerability of individuals with the disorder. Therefore, social approach to unfamiliar people and the role of this behaviour within the Williams syndrome (WS) social phenotype warrant further research to inform social skills' intervention design. METHODS: The current study used parent interviews (n = 21) to probe aspects of social behaviour and interactions with strangers, as well as the impact of such behaviour on the family. Using thematic analysis, it was possible to explore themes that emerged from the interviews, offering qualitatively rich insight into the variability of social approach behaviour in WS. RESULTS: Thematic analysis confirmed a significant desire to interact with strangers as well as a lack of awareness of appropriate social boundaries. However, parental reports about their child's social approach behaviour varied considerably. The within-syndrome variability of the sample was emphasised in parental reports of their child's personality characteristics (e.g. levels of impulsiveness), as well as the level of parental supervision employed. CONCLUSIONS: These in-depth parent insights can help target the needs of individuals with WS and emphasise that an individual approach to intervention will be essential because of the heterogeneity of the WS social profile.

Exploring the prevalence and phenomenology of repetitive behaviours and abnormal sensory processing in children with Williams Syndrome.

BACKGROUND: A small amount of research with individuals who have Williams Syndrome (WS) suggests that children with the condition may be vulnerable to sensory processing abnormalities and present with repetitive and restricted behaviours. METHODS: Parents of 21 children with WS aged 6-15 years completed a semi-structured interview designed to elicit the form, frequency, impact and developmental course of a range of sensory processing abnormalities and repetitive behaviours. RESULTS: Findings indicate that sensory processing difficulties are predominantly characterised by hypersensitivities, particularly in relation to vestibular, auditory, gustatory and proprioceptive functioning. Parents also reported the presence of a range of restricted and repetitive behaviours, which were often associated with their child's sensory symptoms. CONCLUSIONS: This study makes a significant contribution to our understanding of sensory functioning and repetitive behaviours in WS. It also highlights the need for a multidisciplinary assessment of the difficulties experienced by children with the disorder.

[Skin antisepsis in premature infants]. / Problemi di antisepsi nei neonati prematuri.

In some premature newborns, 7 months old and with less than 1 kg of body wheight, total parenteral nutrition is used for weeks, so that good antiseptics can cooperate to the prevention of nosocomial infections, associating the best biocide effects to the best topical tolerability. Details are reported on the biocide cutaneous properties of some chloro-derivates, as sodium hypochlorite and NaDCC, of ethyl and propyl alcohols, of chlorhexidine, of iodophors and also of triclosan and octenidine (even if these latter biocids are not normally available in Italy as cutaneous antiseptics).

The loading dose in continuous infusion extradural analgesia in obstetrics.

We have investigated the need for a loading dose of local anaesthetic before continuous extradural analgesia. After a standard test dose, patients received 0.08% bupivacaine 8 ml, isotonic saline 8 ml or nothing. A continuous infusion of 0.08% bupivacaine 20 ml h-1 was then commenced. The mean times to onset of satisfactory pain relief were similar in the first two groups, but prolonged significantly in the third. This group also required more interventions for inadequate analgesia, although not significantly so. Mean time to achieve highest level of block was significantly shorter in the first group.

Quality assurance of paediatric cardiac surgery: a prospective 6-year analysis.

OBJECTIVE: To audit effective quality assurance methods to monitor outcomes following paediatric cardiac surgery at a single institution. METHODS: All patients undergoing cardiac surgery from January 1996 to December 2001 were enrolled prospectively. Patients were stratified by complexity of surgical procedure into four groups, with Category 4 being the most complex procedure. Outcome measures included death, length of admission and morbidity from complications. RESULTS: A total of 1815 patients underwent 1973 surgical procedures. Of these, 1447 (73.3%) were cardiopulmonary bypass procedures, and 543 (27.5%) were more complex (Category 3 and 4) procedures. Median patient age was 3.5 years (range, 1 day-20 years) and patient weight 15.0 kg (range, 900 g to 90 kg). Sixty-six patients (3.6%) died during the study period. Of the procedures in 1996, 22.7% were classified as complex compared with 29.2% of procedures in 2001. The annual surgical mortality ranged from 1.9-4.7% (P = 0.20), and when mortality was adjusted for complexity of surgery, there was no significant yearly variation in the mortality rate (P = 0.57). Analysis of individual surgeon's results showed no significant difference in the mortality rate by complexity of surgery performed (P = 0.90). Mean ventilation times did not change significantly over time (P = 0.79). The yearly incidence of significant neurological complications ranged from 0.6% to 4.5% and the incidence of arrhythmias from 4.2% to 8.0%. No difference was detected between the years. CONCLUSIONS: Stratifying complexity of surgery proved valuable in monitoring surgical outcomes and detecting differences in performance over time as large subgroups were created for analysis.