Williams syndrome (WS) is characterized by apparent relative strengths in language, facial processing and social cognition but by profound impairment in spatial cognition, planning and problem solving. Following recent research which suggests that individuals with WS may be less linguistically able than was once thought, in this paper we begin to investigate why and how they may give the impression of linguistic proficiency despite poor standardized test results. This case study of Brendan, a 12-year-old boy with WS, who presents with a considerable lack of linguistic ability, suggests that impressions of linguistic competence may to some extent be the result of conversational strategies which enable him to compensate for various cognitive and linguistic deficits with a considerable degree of success. These conversational strengths are not predicted by his standardized language test results, and provide compelling support for the use of approaches such as Conversation Analysis in the assessment of individuals with communication impairments.
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http://dx.doi.org/10.1080/02699200500266547 | DOI Listing |
J Intellect Dev Disabil
March 2024
Department of Psychology and Human Development, IOE UCL's Faculty of Education and Society, London, UK.
The home literacy environment (HLE) has rarely been examined for individuals with neurodevelopmental disorders, including individuals with Williams syndrome and Down syndrome. The current study surveyed carers of individuals with Down syndrome (n = 48) and Williams syndrome (n = 18) in the United Kingdom (UK). The study reports that individuals with Down syndrome were rated higher in general reading skills and writing, while the Williams syndrome group scored higher for speaking.
View Article and Find Full Text PDFJ Infect Dis
January 2025
Department of Anesthesiology, Critical Care, and Pain Medicine, Boston Children's Hospital, Boston, Massachusetts, USA.
Background: Pediatric respiratory syncytial virus (RSV)-related acute lower respiratory tract infection (LRTI) commonly requires hospitalization. The Clinical Progression Scale Pediatrics (CPS-Ped) measures level of respiratory support and degree of hypoxia across a range of disease severity, but it has not been applied in infants hospitalized with severe RSV-LRTI.
Methods: We analyzed data from a prospective surveillance registry of infants hospitalized for RSV-related complications across 39 U.
JAMA Pediatr
January 2025
Department of Cardiology, Harvard Medical School and Boston Children's Hospital, Boston, Massachusetts.
Importance: Multisystem inflammatory syndrome in children (MIS-C) is a life-threatening complication of COVID-19 infection. Data on midterm outcomes are limited.
Objective: To characterize the frequency and time course of cardiac dysfunction (left ventricular ejection fraction [LVEF] <55%), coronary artery aneurysms (z score ≥2.
Pediatr Crit Care Med
January 2025
Paediatric Intensive Care Unit, Great Ormond Street Hospital for Children NHS Foundation Trust and NIHR Biomedical Research Centre, London, United Kingdom.
Objectives: A conservative oxygenation strategy is recommended in adult and pediatric guidelines for the management of acute respiratory distress syndrome to reduce iatrogenic lung damage. In the recently reported Oxy-PICU trial, targeting peripheral oxygen saturations (Spo2) between 88% and 92% was associated with a shorter duration of organ support and greater survival, compared with Spo2 greater than 94%, in mechanically ventilated children following unplanned admission to PICU. We investigated whether this benefit was greater in those who had severely impaired oxygenation at randomization.
View Article and Find Full Text PDFDig Dis Sci
January 2025
School of Nursing, University of Washington, Seattle, WA, USA.
Background: Whether pathophysiological factors differ between males and females with irritable bowel syndrome-diarrhea (IBS-D) remains to be tested. To better understand potential sex differences, males with IBS-D were compared to naturally cycling females and to females with IBS-D taking hormonal contraception on plasma levels of cytokines and gut microbiome characteristics.
Methods: Males and females with Rome III IBS-D completed questionnaires and kept a daily symptom diary for 28 days.
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