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Pragmatic language impairment and social deficits in Williams syndrome: a comparison with Down's syndrome and specific language impairment.

BACKGROUND: The social communication strength of individuals with Williams syndrome described by some researchers contrasts with the picture of social difficulties painted by others. AIMS: To study the pragmatic aspects of language, social relationships and unusual interests in a group of children and adults with Williams syndrome, and to compare them with a group of children and adults with Down's syndrome, children with specific language impairment, and a group of typically developing children. METHODS & PROCEDURES: Parents or teachers completed the Children's Communication Checklist or a modified version of it with wording appropriate for adults. Study groups consisted of 19 children and young adults with Williams syndrome, 24 with Down's syndrome, 17 children with specific language impairment and 32 typically developing children. OUTCOMES & RESULTS: Checklist ratings for the group with Williams syndrome indicated significant levels of pragmatic language impairment, and difficulties with social relationships. Together with evidence of unusual or restricted interests, the results suggested deficits across all three domains covered by the checklist. CONCLUSIONS: Despite earlier reports that emphasize a strong social interest and empathy, this study suggests that individuals with Williams syndrome have pragmatic language impairments, poor social relationships and restricted interests. Far from representing the polar opposite of autism, as suggested by some researchers, Williams syndrome would seem to share many of the characteristics of autistic disorder.

Adolescent↗

Evaluation of simple criteria to predict successful weaning from mechanical ventilation in intensive care patients.

BACKGROUND: There is increasing evidence that weaning protocols improve outcome from mechanical ventilation, but it is unclear how best to implement such protocols in large intensive care units. We evaluated a checklist of simple bedside criteria to determine whether it could be used reliably to predict successful discontinuation of mechanical ventilation. METHODS: We carried out a prospective observational cohort study in a 12-bedded general intensive care unit (ICU). We developed a checklist of metabolic, cardiorespiratory and neurological criteria that suggested that patients should start the weaning process. We performed daily assessments throughout ICU stay and recorded whether the criteria were met. Ultimate ventilator independence was used as the reference standard. RESULTS: We studied 325 sequential admissions to the ICU. Data were available for 98% of patients; 97% of admissions were mechanically ventilated on admission to ICU. Overall, 205 of the 308 ventilated patients (67%) achieved ventilator independence during ICU admission; the other patients died or were transferred ventilated to other ICUs. Eighty-three per cent of the patients who achieved ventilator independence met the set criteria. Fulfilling the criteria was a moderately strong predictor of ultimate ventilator independence: specificity 89%, positive predictive value 94%, positive likelihood ratio (LR) 7.6. When we analysed data by the day from admission on which patients were examined, the test was a strong predictor of subsequent ventilator independence when criteria were met by day 1 (LR 11.1) or day 2 (LR 6.9), but weaker when met by more than/equal to 4 days (LR <3). Patients who met criteria after more than/equal to 4 days often had prolonged weaning and a high incidence of re-intubation. Patients who achieved ventilator independence without fulfilling the criteria (n=35) had a short duration of mechanical ventilation (median 2 days, interquartile range 1-3 days). The most frequent reason for failing criteria before ventilator independence was a Pa(O(2))/FI(O(2)) ratio less than 24 kPa (49% of cases). CONCLUSIONS: A simple checklist can assist nurse assessment of suitability for weaning and could be used as a trigger to commence a weaning protocol. The day on which criteria are met is a useful way of stratifying patients for likely patterns of weaning.

Adolescent↗

The BCPT symptom scales: a measure of physical symptoms for women diagnosed with or at risk for breast cancer.

BACKGROUND: Documentation of concurrent and late side effects of medical interventions to prevent and treat breast cancer is important in research and clinical practice. We used the Breast Cancer Prevention Trial (BCPT) Symptom Checklist to develop an instrument (BCPT Symptom Scales) that could be used to assess side effects and to examine correlates of the derived symptom dimensions among patient populations. METHODS: Exploratory and confirmatory factor analyses were conducted using data from the 42-item BCPT Symptom Checklist completed by four distinct patient populations (N = 2208) who had previously been diagnosed with breast cancer or were at risk for the disease. We examined associations among the resulting BCPT Symptom Scales and demographic and cancer-related variables and a widely used measure of health-related quality of life. RESULTS: Exploratory and confirmatory factor analyses revealed eight factors corresponding to physical symptoms associated with cancer treatment, chemoprevention, menopause, and normal aging: hot flashes, nausea, bladder control, vaginal problems, musculoskeletal pain, cognitive problems, weight problems, and arm problems. On the derived BCPT Symptom Scales, women reported somewhat higher mean scores on scales for hot flashes, pain, and weight problems than on scales for the other symptoms. Demographic and cancer-related variables accounted for up to 15% of the interindividual variance in how women responded to the symptom scales. The most consistent predictors of reporting greater symptoms included lower education level and previous receipt of chemotherapy. CONCLUSIONS: Meaningful symptom dimensions, identified across four samples of women, were associated with demographic and breast cancer-related variables. The BCPT Symptom Scales offer a valuable refinement of the original BCPT Symptom Checklist to assess side effects associated with the treatment and prevention of breast cancer.

Adult↗

Reporting, appraising, and integrating data on genotype prevalence and gene-disease associations.

The recent completion of the first draft of the human genome sequence and advances in technologies for genomic analysis are generating tremendous opportunities for epidemiologic studies to evaluate the role of genetic variants in human disease. Many methodological issues apply to the investigation of variation in the frequency of allelic variants of human genes, of the possibility that these influence disease risk, and of assessment of the magnitude of the associated risk. Based on a Human Genome Epidemiology workshop, a checklist for reporting and appraising studies of genotype prevalence and studies of gene-disease associations was developed. This focuses on selection of study subjects, analytic validity of genotyping, population stratification, and statistical issues. Use of the checklist should facilitate the integration of evidence from these studies. The relation between the checklist and grading schemes that have been proposed for the evaluation of observational studies is discussed. Although the limitations of grading schemes are recognized, a robust approach is proposed. Other issues in the synthesis of evidence that are particularly relevant to studies of genotype prevalence and gene-disease association are discussed, notably identification of studies, publication bias, criteria for causal inference, and the appropriateness of quantitative synthesis.

Case-Control Studies↗

Bruxism in children: effect on sleep architecture and daytime cognitive performance and behavior.

STUDY OBJECTIVES: Sleep bruxism is an involuntary mandibular movement with tooth grinding during sleep. The prevalence of sleep bruxism in children is high and may lead to frequent arousals with altered daytime functioning. We investigated the sleep architecture, the incidence of gastroesophageal reflux, and the daytime cognitive behavioral functioning in a group of children with sleep bruxism. DESIGN-PATIENTS: This prospective pilot study included 10 children. Polysomnographic data with pH-probe analysis was compared with 10 age- and sex-matched controls. Each patient completed a dental evaluation, a nighttime polysomnogram, and cognitive behavioral tests (Kaufman Brief Intelligence Test and Achenbach Child Behavior Checklist). RESULTS: Eight of 10 children had clinically significant bruxism and the 2 remaining patients had recent teeth exfoliation. There was no difference on sleep architecture between patients and controls, except for a higher arousal index for the bruxism group (36.7 vs 20.7, p < .007). Sleep bruxism occurred more frequently in stage 2 and rapid eye movement sleep, with arousals in 66% of the cases. There was no relationship of bruxism to gastroesophageal reflux or intelligence. However, 40% of the patients had elevated scores on the Achenbach Child Behavior Checklist, indicating significant attention and behavior problems, and there were moderate correlations between the arousal index and several of the behavior-problem scales from the Achenbach Child Behavior Checklist (0.5 to 0.6). CONCLUSIONS: The data suggest that children with bruxism have a higher arousal index, which may be associated with an increased incidence of attention-behavior problems. Future studies investigating pediatric sleep bruxism will need to focus on behavior issues that may be prevalent in this population.

Adolescent↗

Assessment of basic physical examination skills of internal medicine residents.

BACKGROUND: Internal medicine faculty at the Mayo Clinic designed a clinical evaluation exercise that separates assessment of physical examination skills from that of medical interviewing and reasoning skills. This report summarizes the first year's experience with assessment of basic physical examination skills. METHOD: A core faculty of five general internists and three internist subspecialists designed a 45-item general examination checklist (e.g., measure blood pressure, examine mouth, palpate liver, drape to ensure privacy). In addition, the core faculty generated a menu of 27 focused examination skills (e.g., examine for carpal tunnel syndrome) from which the faculty examiner would select five items for the resident to perform. Each checklist item was scored 0, 1, or 2 for a maximum possible score of 100. The core faculty selected a criterion-based scoring reference and established a passing score of 90 based on practice examinations with residents and faculty. The core faculty made an instructional videotape of a model examination that was available to all residents. In 1991-92, prior to examination, the checklist was distributed to all first-year categorical (43), preliminary (25), and newly appointed second-year residents (eight). RESULTS: Of the 76 residents examined, 11 (14%) failed and 65 (86%) passed. All failing scores were 86 or lower. The absence of scores 87, 88, and 89 suggested that faculty upgraded borderline performances. All 11 residents who initially failed retook the examination and passed. The five most commonly missed items were (1) inspect the skin, (2) complete examination in logical sequence, (3) palpate aorta, (4) auscultate anterior breath sounds, and (5) palpate axillary and inguinal nodes. Other important observed errors were failure to measure vital signs, confusion of liver and spleen, failure to use bell on stethoscope, and inadequate breast examination. Twenty-eight residents completed an optional feedback form. Reviews were mixed but generally favorable. CONCLUSION: Assessment of the basic physical examination skills of the internal medicine residents was useful, and such skills were able to be assessed separately from physical diagnosis skills and interviewing skills. Direct observation of basic physical examination skills revealed important deficiencies, which provided opportunity for remediation.

Adult↗

An innovative CME program in cardiology for primary care practitioners.

The authors describe an innovative continuing medical education (CME) program they developed to improve the ability of community practitioners to manage common cardiology problems. The program includes an objective structured clinical examination (OSCE) with nine standardized patient (SP) stations. The SPs are trained to use checklists to assess the examinees' clinical skills, and to enter the checklist data directly into computers located within each of the examining rooms. Checklists cover the participants' knowledge of cardiology, and their interviewing, physical-examination, and counseling skills. The computer immediately generates detailed individual reports, which include a skills report, reflecting performance of core skills across all nine stations with group means for comparison, and an omissions report, listing items missed in each case for each participant and, again, comparing the results with group means. Participants review and discuss their performances and then discuss identified learning issues with a cardiologist. The cardiologist also reviews state-of-the-art diagnosis and treatment for the cases presented in the OSCE. Participants have reported having a high regard for the program and have indicated that the program has left them better prepared to deal with clinical cardiology issues.

Attitude of Health Personnel↗

The reliability of six faculty members in identifying important OSCE items.

PURPOSE: To measure the agreement among faculty members about the importance of items on a checklist used to grade an objective structured clinical examination (OSCE) station. METHODS: Six faculty members rated the importance of 47 items for an OSCE station in which students took the history of a patient with sore throat and hoarseness. RESULTS: Of the 47 items, the raters independently identified 15 items as very important. The reliability of each individual rater was fair (averaged value of alpha = .63.) The reliability of the mean rating of the six raters was high (alpha = .91). CONCLUSIONS: The results strongly suggest that when a group of faculty members cooperatively identifies the important items to be included in an OSCE checklist, the reliability of the checklist is superior to one created by a single author.

Algorithms↗

Evaluating procedural skills competence: inter-rater reliability of expert and non-expert observers.

PURPOSE: To examine the inter-rater reliability of expert and non-expert observers when they used objective structured checklists to evaluate candidates' performances on three simulated medical procedures. METHOD: Simulations and structured checklists were developed for three medical procedures: endotracheal intubation, application of a forearm cast, and suturing a simple skin laceration. Groups comprised of two expert and two non-expert observers scored the performances of 101 procedures by 38 medical trainees and practitioners of varying skill levels. Inter-rater reliability was assessed using Pearson correlation coefficients. RESULTS: Inter-rater reliability was good for expert/expert, expert/non-expert, and non-expert/non-expert pairings in all three skills simulations. CONCLUSION: Both expert and non-expert observers demonstrated good inter-rater reliability when using structured checklists to assess procedural skills. Further study is required to determine whether this conclusion may be extrapolated to other study groups or procedures.

Clinical Competence↗

Assessing the generalizability of OSCE measures across content domains.

PURPOSE: To assess the degree to which OSCE measures generalize across multiple administrations to the same students. METHODS: Students' scores from three OSCEs at one institution were correlated to determine the generalizability of the scoring systems across course domains. RESULTS: Analysis revealed that while checklist scores showed quite low correlations across examinations from different domains (ranging from 0.14 to 0.25), global process scores showed quite reasonable correlations (ranging from 0.30 to 0.44), with the correlations for global scores being significantly higher than those for checklist scores in all three comparisons. CONCLUSION: These data would seem to confirm the intuitions about each of these measures: the checklist scores are highly content-specific, while the global scores are evaluating a more broadly based set of skills. Implications for the use of these scales are discussed.

Clinical Clerkship↗

Structured assessment of minor surgical skills (SAMSS) for family medicine residents.

PURPOSE: To develop a valid and reliable examination to assess the technical proficiency of family medicine residents' performance of minor surgical office procedures. METHOD: A multi-station OSCE-style examination using bench-model simulations of minor surgical procedures was developed. Participants were a randomly selected group of 33 family medicine residents (PGY-1 = 16, PGY-2 = 17) and 14 senior surgical residents who functioned as a validation group. Examiners were qualified surgeons and family physicians who used both checklists and global rating scales to score the participants' performances. RESULTS: When family medicine residents were evaluated by family physicians, interstation reliabilities were .29 for checklists and .42 for global ratings. When family medicine residents were evaluated by surgeons, the reliabilities were .53 for checklists and .75 for global ratings. Interrater reliability, measured as a correlation for total examination scores, was .97. Mean scores on the examination were 60%, 64%, and 87% for PGY-1 family medicine, PGY-2 family medicine, and surgery residents, respectively. The difference in scores between family medicine and surgery residents was significant (p < .001), providing evidence of construct validity. CONCLUSION: A new examination developed for assessing family medicine residents' skills with minor surgical office procedures is reliable and has evidence for construct validity. The examination has low reliability when family physicians serve as examiners, but moderate reliability when surgeons are the evaluators.

Ambulatory Surgical Procedures↗

Development of an office-based curriculum of common pediatric primary care skills for residents.

OBJECTIVE: Basic primary care skills-such as injections and hearing screening-are commonly absent from residency curricula, yet competence in these skills is required by residency accrediting organizations. To meet this need at our program, an office-based curriculum of common pediatric primary care skills was developed and piloted in a resident continuity practice. DESCRIPTION: Based on a needs assessment, an eight-unit curriculum was developed to teach residents basic ambulatory primary care skills. The program was designed as a skills supplement to existing primary care curricula and includes hearing screening, vision screening, developmental screening, injections, venipuncture, urinalysis, in-office rapid testing, and analysis of skin scrapings. Each unit lasted one month, using a "skill of the month" format during continuity clinic. During the month, residents received instruction and demonstration, practiced the skill, and tested to document competence. A pilot of the unit "Intramuscular, Subcutaneous, and Intradermal Injections," was conducted at the Pediatric Primary Care Center of Cincinnati Children's Hospital with 26 pediatrics residents. Fourteen residents participated in the pilot prior to testing and a comparison group of 12 was tested without the pilot experience. The pilot occurred over two weeks. The first week, a 20-minute training session was held at the beginning of continuity clinic to discuss a procedure checklist for injections and allow residents to practice with a mannequin. Throughout the remainder of the continuity clinic during the pilot, residents administered injections to their patients, following the procedure checklist and under supervision by medical assistants. At the conclusion of the second week, residents were evaluated with a written test and a practicum. The 12 residents in the control group were tested identically. DISCUSSION: This pilot demonstrated that it is feasible to teach primary care skills to residents in the office setting. In our pilot, the test group performed 61% better on a written test and 64% better on a practical test when compared with the control group (p <.0001). Residents who participated in the pilot felt the methods used were appropriate and effective and that the skills taught were important. Additionally, they found the pilot did not interfere with the operation of the continuity clinic. The procedure checklist proved to be an effective and simple method of instructing a psychomotor skill. Conducting the educational sessions at the beginning of clinic was difficult due to interruptions and tardiness. While other methods, such as noon conferences, may also be effective, instruction in the actual clinical setting appeared to better demonstrate the importance, practicality, and relevance of the skill. The residents were more enthusiastic during this office-based curriculum than a typical resident conference. We conclude that this model is an effective and practical method to teach primary care skills in a clinical setting. Our success with the pilot unit has been encouraging, and we plan to develop and test the remaining units of the curriculum.

Clinical Competence↗

Domestic violence: increasing knowledge and improving skills with a four-hour workshop using standardized patients.

PURPOSE: Domestic violence (DV) is common, yet physicians feel unprepared to address it. Educational interventions may improve the care provided to DV victims, yet the effectiveness of interventions is often unproven. METHOD: Written questions and DV-specific standardized patient (SP) checklist items from an end-of-clerkship and fourth-year comprehensive multispecialty (the Clinical Performance Examination or CPX) examinations of medical students participating in a DV workshop using SPs was compared with nonparticipants. RESULTS: DV workshop participants' and nonparticipants' written question mean scores were 93.2% and 85.8%, respectively, p =.02. End-of-clerkship SP examination DV-specific checklist scores for participants and nonparticipants was 76.3% and 60.0%, respectively, p =.002. Workshop participants scored 44.1% on the CPX DV-specific checklist items versus 35.6% for the nonparticipants, p =.01. CONCLUSION: A DV workshop improved knowledge and skills assessed four and an average of 27 weeks later.

Clinical Clerkship↗

The relationship between the National Board of Medical Examiners' prototype of the Step 2 clinical skills exam and interns' performance.

PURPOSE: To examine the relationship between graduates' performances on a prototype of the National Board of Medical Examiners' Step 2 CS and other undergraduate measures with their residency directors' ratings of their performances as interns. METHOD: Data were collected for the 2001 and 2002 graduates from the study institution. Checklist and interpersonal scores from the prototype Step 2 CS, along with United States Medical Licensing Examination (USMLE) Step 1 and 2 scores and undergraduate grade-point average (GPA), were correlated with residency directors' ratings (average score for six competencies, quartile ranking, and isolated interpersonal communication competency score). Stepwise linear regression was used to identify the best outcome predictors. RESULTS: Quartile ranking was more highly correlated with GPA than Step 2 CS prototype interpersonal score, USMLE Step 2 score, USMLE Step 1 score, and Step 2 CS prototype checklist score. The average score on the residency director's survey was more highly correlated with GPA than USMLE Step 2 score, USMLE Step 1 score, Step 2 CS prototype interpersonal score, and Step 2 CS prototype checklist score. The best predictors for both quartile ranking and average competency score were GPA and Step 2 CS prototype interpersonal score (R(2) = 0.26 and 0.28). CONCLUSION: Both scores from the Step 2 CS prototype significantly correlated with the interns' quartile ranking and average competency score. Only GPA and Step 2 CS prototype interpersonal score accounted for most of the variance of performance in the regression model.

Clinical Competence↗

Objectively evaluating student case presentations.

Evaluation and documentation of student performance is of major importance to a radiology elective coordinator. To better assess a variety of aspects of competence and learning, an oral case presentation was included in the final elective evaluation. This article describes the 31-item checklist designed to assess the level of student achievement of the objectives for the oral presentation. In addition to developing this standard evaluation instrument, methodologies were used to increase the validity and reliability of the oral presentation; the examiners were briefed in advance, the checklist included cognitive and noncognitive measures, and a simple grading score was used for each item. Students who received the checklist and written course objectives at the beginning of the elective earned better grades than those students who did not receive the materials. Student evaluations reveal that the oral case presentation as presently organized is felt to be of value.

Educational Measurement↗

The Johns Hopkins Depression Scale: normative data and validation in child psychiatry patients.

With a newly devised 38-item symptom checklist based upon DSM-III criteria for major affective disorder, normative data were obtained on 1,004 school children, aged 5 to 13 years. The items on the checklist were scored on a severity scale of 0 to 4 with a maximum score of 152. The average raw scores in this population was 31.2, SD +/- 2.4. Of 57 inpatients, 27 met DSM-III criteria for a major affective disorder and obtained mean raw scores of 66.8, SD +/- 13.4. The 30 nondepressed inpatients had scores of 36.4, SD +/- 5.3. The differences in the raw scores between the depressed inpatients and normal controls, and depressed and nondepressed inpatients were found to be statistically significant (p less than 0.01). The new checklist can serve as a potential screening instrument, is not time consuming, and has been standardized on a large normal control population for age and sex.

Adolescent↗

Sherlock Holmes and child psychopathology assessment approaches: the case of the false-positive.

OBJECTIVE: To explore the relative value of various methods of assessing childhood psychopathology, the authors compared 4 groups of children: those who met criteria for one or more DSM diagnoses and scored high on parent symptom checklists, those who met psychopathology criteria on either one of these two assessment approaches alone, and those who met no psychopathology assessment criterion. METHOD: Parents of 201 children completed the Child Behavior Checklist (CBCL), after which children and parents were administered the Diagnostic Interview Schedule for Children (version 2.1). Children and parents also completed other survey measures and symptom report inventories. The 4 groups of children were compared against "external validators" to examine the merits of "false-positive" and "false-negative" cases. RESULTS: True-positive cases (those that met DSM criteria and scored high on the CBCL) differed significantly from the true-negative cases on most external validators. "False-positive" and "false-negative" cases had intermediate levels of most risk factors and external validators. "False-positive" cases were not normal per se because they scored significantly above the true-negative group on a number of risk factors and external validators. A similar but less marked pattern was noted for "false-negatives." CONCLUSIONS: Findings call into question whether cases with high symptom checklist scores despite no formal diagnoses should be considered "false-positive." Pending the availability of robust markers for mental illness, researchers and clinicians must resist the tendency to reify diagnostic categories or to engage in arcane debates about the superiority of one assessment approach over another.

Adaptation, Psychological↗

Social competence and behavioral problems of pediatric survivors of burns.

Sixty children (35 boys, 25 girls) with burns were surveyed at least 1 year after burn injury to assess the behavior problems and difficulties with competency that they were having. The Child Behavior Checklist, the Youth Self Report, and the Teacher Report Form developed by Achenbach were administered to obtain standardized measures of behavior and competence. The mean values for these scales were in the normal range for each age and sex. However, the Child Behavior Checklist revealed a statistically significant (p < 0.05) increase in problems and decrease in competency for most age groups and both sexes when compared with the reference population. In contrast, the Teacher Report Form and the Youth Self Report revealed very few differences from the reference population. The burn size (15% to 20% total body surface area, 35% to 50% total body surface area, and > 70% total body surface area) did not account for any of the group differences. Further studies involving detailed clinical interviews and other standardized measures are needed to understand the discrepancies between the Child Behavior Checklist and the other scales.

Adolescent↗