A 60-year-old patient with a clinical diagnosis of schizophrenia underwent a magnetic resonance imaging (MRI) scan related to the evaluation of isolated seizures that emerged while medicated with clozapine. Unexpectedly, the MRI scan revealed evidence of asymmetric and enlarged cerebral ventricles that were interpreted as congenital in origin. The presence of both congenital lateral ventricular asymmetry and ventriculomegaly may interact to increase risk of schizophrenia. The history and clinical features, including cognitive testing, of the illustrative patient are presented.

Williams syndrome is a neurodevelopmental disorder that results from the deletion of ~25-30 genes spanning about 1.5 megabases in the q11.23 region of chromosome 7. Patients with this syndrome present with a combination of a distinctive elfin-like facial appearance; growth retardation; mild mental retardation; an inconsistent cognitive profile that includes visuospatial impairments with good facial discrimination and relatively preserved expressive language skills; and cardiovascular abnormalities. In addition, a striking behavioral feature of the syndrome is the high sociability and empathy that these patients show for others. The study of patients with “partial” deletions of the chromosome band 7q11.23, mutated genes in this region and knockout mice with deletions of specific genes in the homologous G1–G2 region of mouse chromosome 5 are clarifying some genotype/phenotype relationships. Futhermore, genes located in this region that are prominently expressed have been implicated in brain development and function.The neuropsychological profile of patients with Williams syndrome is heterogeneous, highlights important dissociations between cognitive functions and suggests that the behavioral dimensions of sociability, empathy, engageability, and talkativeness may be independent of, or not easily explained by, the cognitive deficits. Williams syndrome has enormous heuristic value because its pathological feature of heightened “sociability” can be a “deficit” symptom of major complex neuropsychiatrie disorders, such as schizophrenia and autism. Data consistent with a core inability of patients with Williams syndrome to inhibit social approach suggest that this disorder may afford an opportunity to study the biological basis of the “drive” toward socialization. From a research perspective, the syndrome lends itself to neurobiological studies of sociability as a dimension that varies independently of cognition (or at least many separable cognitive processes). Importantly, from a clinical perspective, the syndrome challenges us to administer strategic psychosocial interventions that take advantage of the opportunities that “pathological” sociability provide, while avoiding its threats. An illustrative example of an effective strategically planned psychosocial intervention for a patient with Williams syndrome is briefly presented.

Background: Various organizations and universities have developed competencies for health professionals and other emergency responders. Little effort has been devoted to the integration of these competencies across health specialties and professions. The American Medical Association Center for Public Health Preparedness and Disaster Response convened an expert working group (EWG) to review extant competencies and achieve consensus on an educational framework and competency set from which educators could devise learning objectives and curricula tailored to fit the needs of all health professionals in a disaster.

Methods: The EWG conducted a systematic review of peer-reviewed and non–peer reviewed published literature. In addition, after-action reports from Hurricane Katrina and relevant publications recommended by EWG members and other subject matter experts were reviewed for congruencies and gaps. Consensus was ensured through a 3-stage Delphi process.

Results: The EWG process developed a new educational framework for disaster medicine and public health preparedness based on consensus identification of 7 core learning domains, 19 core competencies, and 73 specific competencies targeted at 3 broad health personnel categories.

Conclusions: The competencies can be applied to a wide range of health professionals who are expected to perform at different levels (informed worker/student, practitioner, leader) according to experience, professional role, level of education, or job function. Although these competencies strongly reflect lessons learned following the health system response to Hurricane Katrina, it must be understood that preparedness is a process, and that these competencies must be reviewed continually and refined over time. (Disaster Med Public Health Preparedness. 2008;2:57–68)

Mass casualty triage is the process of prioritizing multiple victims when resources are not sufficient to treat everyone immediately. No national guideline for mass casualty triage exists in the United States. The lack of a national guideline has resulted in variability in triage processes, tags, and nomenclature. This variability has the potential to inject confusion and miscommunication into the disaster incident, particularly when multiple jurisdictions are involved. The Model Uniform Core Criteria for Mass Casualty Triage were developed to be a national guideline for mass casualty triage to ensure interoperability and standardization when responding to a mass casualty incident. The Core Criteria consist of 4 categories: general considerations, global sorting, lifesaving interventions, and individual assessment of triage category. The criteria within each of these categories were developed by a workgroup of experts representing national stakeholder organizations who used the best available science and, when necessary, consensus opinion. This article describes how the Model Uniform Core Criteria for Mass Casualty Triage were developed.

(Disaster Med Public Health Preparedness. 2011;5:129-137)

Public health and the emergency care community must work together to effectively achieve a state of community-wide disaster preparedness. The identification of model communities with good working relationships between their emergency care community and public health agencies may provide useful information on establishing and strengthening relationships in other communities. Seven model communities were identified: Boston, Massachusetts; Clark County, Nevada; Eau Claire, Wisconsin; Erie County, New York; Louisville, Kentucky; Livingston County, New York; and Monroe County, New York. This article describes these communities and provides a summary of common findings. Specifically, we recommend that communities foster respectful working relationships between agency leaders, hold regular face-to-face meetings, educate each other on their expertise and roles during a disaster, develop response plans together, work together on a day-to-day basis, identify and encourage a leader to facilitate these relationships, and share resources. (Disaster Med Public Health Preparedness. 2007;1:142–145)

Mass casualty triage is a critical skill. Although many systems exist to guide providers in making triage decisions, there is little scientific evidence available to demonstrate that any of the available systems have been validated. Furthermore, in the United States there is little consistency from one jurisdiction to the next in the application of mass casualty triage methodology. There are no nationally agreed upon categories or color designations. This review reports on a consensus committee process used to evaluate and compare commonly used triage systems, and to develop a proposed national mass casualty triage guideline. The proposed guideline, entitled SALT (sort, assess, life-saving interventions, treatment and/or transport) triage, was developed based on the best available science and consensus opinion. It incorporates aspects from all of the existing triage systems to create a single overarching guide for unifying the mass casualty triage process across the United States. (Disaster Med Public Health Preparedness. 2008;2(Suppl 1):S25–S34)

Young children readily acquire new words with consonants and syllable structures already used accurately (IN words). They have more difficulty acquiring new words with consonants or syllable structures never before produced or attempted (OUT words). In the present study, we examined children's acquisition of a third type of word, containing consonants the children had attempted in the past but never produced accurately (ATTEMPTED words). IN, OUT and ATTEMPTED words and their object referents were presented to 11 young children in a series of play sessions. The children's production and comprehension of the words were then assessed. No comprehension differences among the three types of words were observed. However, ATTEMPTED words as well as OUT words were less likely to be acquired in production than IN words. Some revisions in models of child phonology are proposed to accommodate these findings.

Specifically-language-impaired children and younger normal children matched for expressive language were presented with unfamiliar object names and referents across five experimental sessions. The objects differed in the degree to which they were associated with actions, and only certain of the object exemplars were named during presentation. Comprehension testing revealed that the specifically-language-impaired children acquired a greater number of object concepts presented in a no-action condition than the normal children. However, their extension of the names to new exemplars was more restricted and less differentiated. Several possible accounts of these findings are evaluated.

The influence of phonological selection and avoidance upon early lexical acquisition was examined within an experimental paradigm. During 10 bi-weekly experimental sessions, 12 children (1;0.21 to 1;3.15 at the outset) were presented with 16 contrived lexical concepts, each consisting of a nonsense word and four unfamiliar referents. For each child, eight words involved phonological characteristics which had been evidenced in production (in) and eight had characteristics which had not been evidenced in production or selection (out), in words were produced imitatively and non-imitatively in greater numbers and in earlier sessions than OUT words, providing evidence for the influence of selection and avoidance. The degree of phonetic accuracy of these two types of productions did not differ. These findings are discussed in terms of a proposal concerning early phonological representation and acquisition.