Invasive serogroup B Neisseria meningitidis in Quebec, Canada, 2003 to 2010: persistence of the ST-269 clone since it first emerged in 2003

In the era after the introduction of the meningococcal serogroup C conjugate vaccine, from 1 January 2003 to 31 December 2010, serogroup B meningococci were the major cause of invasive meningococcal disease in the province of Québec, Canada, being responsible for 72% of all meningococcal disease cases. Of the 334 invasive serogroup B Neisseria meningitidis strains analyzed, 53.9% belonged to the ST-269 clonal complex (CC). Since it first emerged in 2003, the percentage of invasive serogroup B isolates that belonged to the ST-269 CC had increased from 35% in 2003 to 76% in 2010. Among the 180 meningococci in the ST-269 CC, 91.7% belonged to a single ST (ST-269). The most common PorA genotypes identified in the ST-269 CC were (i) VR1 19-1, VR2 15-11, VR3 36 (84%) and (ii) VR1 18-7, VR2 9, VR3 35-1 (9%). Cases of invasive disease due to the ST-269 CC were commonly found in those aged 11 to 19 years (30.5%) and 20 to 40 years (25.5%). Meningococci of the ST-269 CC were uncommon in other Canadian provinces. In contrast to the ST-269 CC, invasive serogroup B meningococci that belonged to the ST-41/44 CC were much more diverse genetically. However, one ST (ST-571), which is uncommon in the United States, accounted for 35% of all cases due to this CC. The current finding suggests that the ST-269 clone may indeed represent an emerging hypervirulent clone of meningococci.     

Complications of frontofacial advancement

Purpose

The purpose of this study was to document the early and late complications associated with frontofacial distraction.

Method

A systematic review of the literature on complications in craniofacial surgery and a review of records of patients undergoing frontofacial distraction at Great Ormond Street Hospital for Children over a 10-year period were conducted.

Results

Complications arising from frontofacial advancement are common. Mortality rates varied between 0 and 4.5?%. There has been a general decline in reported mortality rates with time, and the most recent series report mortalities of less than 1?%. The incidence of significant blood loss (greater than one blood volume) in patients undergoing monobloc osteotomy varied between 5.3 and 9.1?%. CSF leaks following monobloc distraction are common (incidence, 2–20?%). Most of these leaks settle spontaneously. The incidence of frontal bone flap necrosis requiring debridement and a subsequent cranioplasty varied between 3 and 20?%.

Conclusions

The functional and aesthetic benefits of frontofacial distraction are well documented, but these advantages are associated with a significant complication rate. The potential benefits of frontofacial distraction need to be carefully weighed against the potential complications when deciding to recommend surgery.     

Come play with me: an argument to link autism spectrum disorders and anorexia nervosa through early childhood pretend play

This article builds on the argument of a link between behaviours observed in persons with autism spectrum disorders and persons with anorexia nervosa. In describing these behaviours, a link is made between deficits in social cognition, lack of flexible and creative thinking, theory of mind, and deficits in early pretend play ability. Early pretend play ability is a strong avenue to the development and strengthening of social cognition, problem solving, language, logical sequential thought, and understanding social situations. Currently, there is no literature on the pretend play ability of persons who develop anorexia nervosa. This article argues for research into this area which may potentially contribute to developments in new intervention strategies for these persons.     

Abnormal myelination in ring chromosome 18 syndrome

Partial deletion of genetic material from the long arm of chromosome 18 results in a syndrome with multisystemic involvement, including dysmorphic features, intellectual disability, cardiac malformations, endocrine abnormalities, immunodeficiency, musculoskeletal deformities, and variable neurologic manifestations. Hypomyelination has been reported in patients with chromosome 18q- and postulated to be secondary to deletion of the gene coding for myelin basic protein found at 18q23. Little however is reported on cerebral anomalies seen in patients with ring chromosome 18, an analogous syndrome but with expectedly more severe phenotype secondary to the combined deletions of genetic material from both the short (p-) and long arm (q-) of chromosome 18. We are reporting a case of a girl with ring chromosome 18 and deletions involving 18p11.32-18p11.21 and 18q21.31-18q23. The abnormalities observed on magnetic resonance imaging are discussed with a specific focus on the evolution and significance of associated white matter changes.     

K27M mutation in histone H3.3 defines clinically and biologically distinct subgroups of pediatric diffuse intrinsic pontine gliomas

Pediatric glioblastomas (GBM) including diffuse intrinsic pontine gliomas (DIPG) are devastating brain tumors with no effective therapy. Here, we investigated clinical and biological impacts of histone H3.3 mutations. Forty-two DIPGs were tested for H3.3 mutations. Wild-type versus mutated (K27M-H3.3) subgroups were compared for HIST1H3B, IDH, ATRX and TP53 mutations, copy number alterations and clinical outcome. K27M-H3.3 occurred in 71 %, TP53 mutations in 77 % and ATRX mutations in 9 % of DIPGs. ATRX mutations were more frequent in older children (p < 0.0001). No G34V/R-H3.3, IDH1/2 or H3.1 mutations were identified. K27M-H3.3 DIPGs showed specific copy number changes, including all gains/amplifications of PDGFRA and MYC/PVT1 loci. Notably, all long-term survivors were H3.3 wild type and this group of patients had better overall survival. K27M-H3.3 mutation defines clinically and biologically distinct subgroups and is prevalent in DIPG, which will impact future therapeutic trial design. K27M- and G34V-H3.3 have location-based incidence (brainstem/cortex) and potentially play distinct roles in pediatric GBM pathogenesis. K27M-H3.3 is universally associated with short survival in DIPG, while patients wild-type for H3.3 show improved survival. Based on prognostic and therapeutic implications, our findings argue for H3.3-mutation testing at diagnosis, which should be rapidly integrated into the clinical decision-making algorithm, particularly in atypical DIPG.     

A comparison of two prompting procedures for teaching basic skills to children with autism

We compared two prompting techniques that are commonly used to teach individuals with autism. In the “most-to-least” (MTL) prompting condition, the therapist initially delivered the most intrusive prompt necessary to achieve a correct response. Prompts were gradually faded across subsequent trials, while errors resulted in the provision of increasingly more intrusive prompts. Prompt fading occurred in the same manner for the “no-no-prompt” (NNP) condition; however, the therapist presented the initial instruction up to two times before delivering a prompt. Four children with autism participated. Rate of skill mastery, frequency of errors, and maintenance of skills under the two prompting conditions were compared via combined multielement and multiple baseline designs. Although the NNP method resulted in faster skill acquisition, MTL prompting was associated with fewer errors for all participants and better performance during 1- and 2-week maintenance probes for 3 of the 4 participants.     

New findings validate an ancient technique: How massage affects the biochemistry of inflammation

Massage is a manual soft tissue manipulation, and includes holding,causing movement,and/or applying pressure to the body defined by American Massage Therapy Association(AMTA).References to massage can be found in many ancient civilizations. The ancient Chinese medical book called Huangdi’s Internal Classic(Huang Di Nei Jing,2760 BC) recommended"use massage therapy for treatment of numbness caused by channels blockage."There is no legal scope of practice for massage