10 research outputs found
Expressive language in children with Kabuki syndrome
No full textSince the description of Kabuki syndrome in 1981 over 300 cases from a variety of countries have been reported. Only a limited number of these reports, however, provided data on speech language development. Ghent University Hospital (J. Van Borsel, T. Defloor) and the Department of Clinical Genetics, Academic Hospital Maastricht (C.T.R.M. Schrander-Stumpel, L.M.G. Curfs) are directing research on the specific nature of communicative development in persons with Kabuki syndrome. The aim of the present study was to delineate the language difficulties in the syndrome. The subjects were six Dutch-speaking children (three male, three female), with chronological age ranging from 4.4 to 10.6 years (mean = 8.1). Spontaneous speech samples were collected, subjected to a consensus orthographic transcription, and analyzed by means of TOAST [Moerman-Coetsier and Van Besien, 1987], a Dutch standardized diagnostic instrument to investigate different aspects of spontaneous language production. In all the children, expressive language abilities were impaired. Poor morphosyntactic abilities were consistently demonstrated. Lexical and pragmatic difficulties were also present, whereas phonological development was less often affected
Spraak bij het Prader-Willi syndroom
No full textDiss. doct. sociale gezondheidswetenschappen: logopedie en audiologi
Kabuki syndrome: Clinical data in 20 patients, literature review, and further guidelines for preventive management
No full textAerodynamic and acoustic characteristics of voice in Prader-Willi syndrome.
No full textAbstract: Prader-Willi syndrome (PWS) is a multisystem disorder caused by DNA abnormalities involving chromosome 15. Major characteristics are infant hypotonia, hypogonadism, mental retardation, a short stature, atypical facial appearance, and the onset of obesity due to insatiable hunger in early childhood. Also. speech and language abnormalities have been reported including voice disorders. These have seldom been studied in detail, however. This paper reports the results of an acoustic and aerodynamic investigation of the voice in 22 individuals with PWS. Two age groups were distinguished, a group of children [chronoiogical age (CA)6 years, 7 months through 11 years, 7 months; total intelligence quotient (TIQ) 40-88] and a group of adolescents and adults (CA 17 years, 1 month through 29 years, 5 months; TIQ 41-94). Both aerodynamic and acoustic parameters were obtained and compared with normative data from the Belgian Study Group on Voice Disorders. It was found that voice difficulties do commonly occur in individuals with PWS including impairment of frequency levels, voice quality, and poor aerodynamic capabilities
A Formal Analysis of SCTP: Attack Synthesis and Patch Verification
No full textSCTP is a transport protocol offering features such as multi-homing,
multi-streaming, and message-oriented delivery. Its two main implementations
were subjected to conformance tests using the PacketDrill tool. Conformance
testing is not exhaustive and a recent vulnerability (CVE-2021-3772) showed
SCTP is not immune to attacks. Changes addressing the vulnerability were
implemented, but the question remains whether other flaws might persist in the
protocol design.
We study the security of the SCTP design, taking a rigorous approach rooted
in formal methods. We create a formal Promela model of SCTP, and define 10
properties capturing the essential protocol functionality based on its RFC
specification and consultation with the lead RFC author. Then we show using the
Spin model checker that our model satisfies these properties. We define 4
attacker models - Off-Path, where the attacker is an outsider that can spoof
the port and IP of a peer; Evil-Server, where the attacker is a malicious peer;
Replay, where an attacker can capture and replay, but not modify, packets; and
On-Path, where the attacker controls the channel between peers. We modify an
attack synthesis tool designed for transport protocols, Korg, to support our
SCTP model and four attacker models.
We synthesize 14 unique attacks using the attacker models - including the CVE
vulnerability in the Off-Path attacker model, 4 attacks in the Evil-Server
attacker model, an opportunistic ABORT attack in the Replay attacker model, and
eight connection manipulation attacks in the On-Path attacker model. We show
that the proposed patch eliminates the vulnerability and does not introduce new
ones according to our model and protocol properties. Finally, we identify and
analyze an ambiguity in the RFC, which we show can be interpreted insecurely.
We propose an erratum and show that it eliminates the ambiguity
Heel Pressure Ulcers: a study of wound healing
No full textBackground
Heels are a common location for pressure ulcers (PUs); they may be physiologically different to other PU sites and their healing is poorly understood.
Aim
To summarise the effects of support surfaces and identify prognostic factors in healing heel PUs.
Objectives
1. Review the effects of support surfaces for heel PU healing
2. Identify factors which independently impact on heel PU healing
3. Describe the characteristics of patients, current management practices and progress of heel PUs
Methods
Systematic review of the evidence of effectiveness for pressure relieving devices in the treatment of heel PUs.
Prospective cohort study of patients with heel PUs ≥ Grade 2 in an acute hospital and followed for 18 months or up till healed.
Results
Systematic review identified 467 potentially relevant articles, retrieved 70 for screening and included 1in a narrative synthesis. No recommendations for practice could be made.
Cohort study recruited 140 people with 183 heel ulcers. 77 (42%) ulcers healed, 88 (48%) did not heal due to death, 5 (3%) were on limbs subsequently amputated, 11 (6%) were unhealed at 18 months, 2 (1%) were lost to follow-up. Cox proportional hazards models identified 12 significant (p≤0.2) variables affecting time to healing in the univariate analysis. Eight entered the multivariate model: 2 reached significance (p≤0.1): severe (cf superficial) ulcers and the presence of (cf the absence) peripheral vascular disease (PVD) reduced the chance of healing.
Ulcer area did not change in a uniform manner. Treatments e.g. support surfaces and dressings were inconsistently used. Many patients experienced non-pressure ulcer related infections during the study.
Conclusion
It is not known if support surfaces aid heel PU healing. The severity of the PU and the presence of PVD are independent prognostic factors for healing. Further work is needed to explore prognostic factors which change over time
Evaluation of Evidence-Based Practices in Online Learning: A Meta-Analysis and Review of Online Learning Studies
No full textA systematic search of the research literature from 1996 through July 2008 identified more than a thousand empirical studies of online learning. Analysts screened these studies to find those that (a) contrasted an online to a face-to-face condition, (b) measured student learning outcomes, (c) used a rigorous research design, and (d) provided adequate information to calculate an effect size. As a result of this screening, 51 independent effects were identified that could be subjected to meta-analysis. The meta-analysis found that, on average, students in online learning conditions performed better than those receiving face-to-face instruction. The difference between student outcomes for online and face-to-face classes—measured as the difference between treatment and control means, divided by the pooled standard deviation—was larger in those studies contrasting conditions that blended elements of online and face-to-face instruction with conditions taught entirely face-to-face. Analysts noted that these blended conditions often included additional learning time and instructional elements not received by students in control conditions. This finding suggests that the positive effects associated with blended learning should not be attributed to the media, per se. An unexpected finding was the small number of rigorous published studies contrasting online and face-to-face learning conditions for K–12 students. In light of this small corpus, caution is required in generalizing to the K–12 population because the results are derived for the most part from studies in other settings (e.g., medical training, higher education)
Why do clinicians place indwelling urinary catheters with patients in acute medical care?
No full textBackground: Indwelling urinary catheters (IUCs) placed for short-term use in hospital frequently become long-term catheters, increasing the potential for infections, trauma and other complications. Current research has focused on the prompt removal of IUCs in place, with no published review of interventions to reduce the initial placement. Furthermore, little is known about why clinicians place IUCs in acute medical care. Without this knowledge, the effectiveness of strategies aimed at reducing IUC use is likely to be sub-optimal.Aim: To understand why clinicians decide to place IUCs in acute medical care. Methods: (1) A systematic review of interventions to minimise the initial placement of urinary catheters in acute care. (2) A qualitative study in the A&E department and acute medical wards of a 1200+ bed hospital. Clinicians who made the decision to place an IUC were asked to participate in a retrospective think aloud interview describing how they came to the decision, later participating in a semi-structured interview to discuss their wider experiences of making the decision to place an IUC. A purposive sample and thematic analysis were used. Results: (1) Eight (six uncontrolled) studies met the inclusion criteria for the systematic review, using a variety of interventions including clinician education and introduction of guidelines to reduce IUC use. Although seven demonstrated a reduction in the initial use of IUCs post-intervention (relative risk 0.19 – 0.86), the impact of individual interventions was unclear. Notably, each study provided a list of reasons considered to provide justifications for IUC use, with substantial variation between the lists. (2) 30 retrospective think aloud interviews and 20 semi-structured interviews were undertaken. Clinicians were influenced by cues taken from three groups; individual beliefs (e.g. on the clinical indication or IUC-associated risks), patient factors (e.g. age or gender) and organisational factors (e.g. resources or policy). Many spectrums of belief were found (e.g. varying opinions on using IUCs to protect skin from urinary incontinence). Conclusions: This work establishes that understanding of interventions to reduce the initial placement of IUCs is poor and there is a lack of agreement on when the benefits of IUC use outweigh the risks. Clinical reasoning in this area is frequently inconsistent and IUC placement decisions vary widely, indicating that there is considerable scope for a reduction in use
