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Partial trisomy 2p.

A 4-year-and-10-month-old girl was referred to the Child Development Center, Rhode Island Hospital, Providence, Rhode Island, USA, for evaluation because of mental retardation. She was found to have short stature, congenital heart disease and dysmorphic facial features. A chromosome analysis revealed an unbalanced translocation with trisomy of the distal part of the short arm of chromosome 2 (2p21----2pter). A balanced reciprocal translocation was identified in the maternal karyotype 46,X,t(X;2)(q28;p21). The phenotype of partial trisomy 2p is discussed.

Child, Preschool↗

Prevalence and features of intermittent explosive disorder in a clinical setting.

OBJECTIVE: To determine the lifetime and current prevalence, along with other characteristics such as age at onset, patterns of comorbidity, and interest in treatment, of DSM-IV intermittent explosive disorder (IED) in an outpatient psychiatric sample. METHOD: 1300 individuals presenting for outpatient psychiatric treatment at Rhode Island Hospital, Providence, R.I., underwent structured diagnostic assessment for Axis I and II disorders. The diagnosis of IED was made according to DSM-IV criteria. RESULTS: 6.3% (SE, +/- 0.7%) of patients met criteria for lifetime DSM-IV IED, and 3.1% +/- 0.5% of patients met criteria for current DSM-IV IED. While DSM-IV IED was the current principal diagnosis in only 0.6% +/- 0.2% of patients, most patients with current DSM-IV IED (80%) were interested in treatment for their intermittent aggressive behavior. Only lifetime alcohol/drug disorder was more frequent in DSM-IV IED compared with non-IED patients. Age at onset for DSM-IV IED peaked in the teen years, was earlier for men than women, and occurred earlier than all comorbid disorders, with the exception of phobic anxiety disorders, suggesting that IED cannot be attributed to most comorbid conditions. CONCLUSIONS: DSM-IV IED in psychiatric samples is far more common than previously thought. DSM-IV IED develops early in life, especially in male patients, and its development may be independent of most other disorders.

Adult↗

Trillion virion delay: time from testing positive for HIV to presentation for primary care.

BACKGROUND: Human immunodeficiency virus (HIV)-infected individuals' initial presentation to medical care frequently occurs at a point of advanced immunosuppression. OBJECTIVES: To investigate the time between HIV testing and presentation to primary care. Also to examine factors associated with delayed presentation. METHODS: One hundred eighty-nine consecutive outpatients without prior primary care for HIV infection were assessed at 2 urban hospitals: Boston City Hospital, Boston, Mass, and Rhode Island Hospital, Providence. Sociodemographics, alcohol and drug use, social support, sexual beliefs and practices, and HIV testing issues were examined in bivariate and multivariate analyses for association with delay in presentation to primary care after positive test results for HIV. RESULTS: Of 189 patients, 74 (39%) delayed seeking primary care for more than 1 year, 61 (32%) delayed for more than 2 years, and 35 (18%) for more than 5 years after an initial positive HIV serologic evaluation. The median CD4+ cell count of subjects was 0.28 x 10(9)/L (range, 0.001-1.71 x 10(9)/L). In multiple linear regression analysis the following characteristics were found to be associated with delayed presentation to primary care after HIV testing: history of injection drug use (P<.001); not having a living mother (P=.01); not having a spouse or partner (P=.08); not being aware of HIV risk before testing (P<.001); and being notified of HIV status by mail or telephone (P=.002). An interaction effect between sex and screening for alcohol abuse was significant (P=.03) and suggested longer delays for men with positive screening test results (CAGE [an alcoholism screening questionnaire containing 4 structured questions], 2+) compared with men without positive screening test results or women. CONCLUSIONS: Patients with positive HIV test results often delay for more than a year before establishing primary medical care. Information readily available at the time of HIV testing concerning substance abuse, social support, and awareness of personal HIV risk status is useful in identifying patients who are at high risk of not linking with primary care. Patients who were notified of their HIV status by mail or telephone delayed considerably longer than those notified in person. Efforts to ensure primary care linkage at the time of notification of positive HIV serostatus are necessary to maximize benefits for both individual and public health and should be an explicit task of posttest counseling.

Alcohol Drinking↗

Reassessment of parathyroid hormone monitoring during parathyroidectomy for primary hyperparathyroidism after 2 preoperative localization studies.

HYPOTHESIS: For patients with primary hyperparathyroidism and patients with 2 localization studies showing the same single location of parathyroid disease, use of intraoperative parathyroid hormone (IOPTH) measurement does not significantly increase the success of minimally invasive parathyroidectomy. DESIGN: Retrospective cohort study. SETTING: Experience of 2 academic centers over 5 years (at Brigham and Women's Hospital, Boston, Mass) and almost 4 years (at Rhode Island Hospital, Providence). PATIENTS: A total of 569 patients with primary hyperparathyroidism who underwent technetium Tc 99m sestamibi (MIBI) parathyroid imaging and neck ultrasonography (US). MAIN OUTCOME MEASURES: Incidence of correct prediction of location and extent of disease. RESULTS: In 322 patients (57%), MIBI and US imaging identified the same single site of disease. In 319 (99%) of these 322 patients, surgical exploration confirmed a parathyroid adenoma at that site, and the IOPTH levels normalized on removal. In 3 (1%) of the 322 patients, IOPTH measurement identified unsuspected additional disease. In 3 (1%) of the remaining 319 patients, IOPTH-guided removal of a single adenoma failed to correct hypercalcemia. Therefore, the failure rate of surgery in patients with positive MIBI and positive US imaging was 1% with IOPTH measurement and 2% without IOPTH measurement (P = .50). In 201 (35%) of the 569 patients, only 1 of the 2 studies recognized an abnormality or the studies disagreed on location. In these cases, either MIBI imaging or US imaging (if MIBI imaging was negative) failed to predict the correct site or extent of disease in 76 (38%) of the 201 patients (P<.001 vs concordant studies). CONCLUSIONS: In primary hyperparathyroidism, concordant preoperative localization with MIBI and US imaging is highly accurate. Use of IOPTH measurement in these cases adds only marginal benefit. When only 1 of the 2 studies identifies disease or the studies conflict, however, IOPTH measurement remains essential during minimally invasive parathyroidectomy.

Chi-Square Distribution↗

K-ras-2 topographic genotyping of pancreatic adenocarcinoma.

OBJECTIVE: To determine the frequency distribution of K-ras-2 point mutation genotypes in pancreatic adenocarcinoma and to evaluate the effectiveness of K-ras-2 genotyping as a means to predict localized disease and potential long-term survival. DESIGN: Topographic genotyping from archival formalin-fixed, paraffin-embedded large- and biopsy-sized tissue specimens as well as cytologic fluid using polymerase chain reaction products and direct sequencing together with clinicopathologic and statistical analysis. SETTING: Tertiary care medical center with molecular diagnostics pathology laboratory. PATIENTS: Patients treated between 1988 and 1993 at Rhode Island Hospital, Providence, yielding 55 primary and 56 metastatic specimens of pancreatic adenocarcinoma. RESULTS: Each primary pancreatic adenocarcinoma was found to contain one of eight specific genotypes that was maintained in all metastatic deposits of that individual tumor. Primary adenocarcinomas confined to the pancreatic bed at diagnosis were predominantly of a normal genotype (56% [14/25]). Pancreatic adenocarcinomas progressing to distant hematogenous metastasis were almost exclusively mutated (88% [7/8]; P < .005). Patients undergoing pancreatic resection (Whipple's operation) and having a normal K-ras-2-genotype (58% [11/19]) had a significantly longer survival (21.3 months) than similar patients with mutated tumors (8.2 months). CONCLUSIONS: The findings support the feasibility of K-ras-2 topographic genotyping to identify potentially indolent disease and suggest a potentially useful role in the preoperative evaluation of pancreatic adenocarcinoma.

Adenocarcinoma↗

Effects of motivational interviewing on smoking cessation in adolescents with psychiatric disorders.

OBJECTIVE: To test the hypothesis that among adolescent smokers hospitalised for psychiatric and substance use disorders, motivational interviewing (MI) would lead to more and longer quit attempts, reduced smoking, and more abstinence from smoking over a 12 month follow up. DESIGN: Randomised control trial of MI versus brief advice (BA) for smoking cessation, with pre- and post-intervention assessment of self efficacy and intentions to change, and smoking outcome variables assessed at one, three, six, nine, and 12 month follow ups. SETTING: A private, university affiliated psychiatric hospital in Providence, Rhode Island, USA. PATIENTS OR OTHER PARTICIPANTS: Consecutive sample (n = 191) of 13-17 year olds, admitted for psychiatric hospitalisation, who smoked at least one cigarette per week for the past four weeks, had access to a telephone, and did not meet DSM-IV criteria for current psychotic disorder. INTERVENTIONS: MI versus BA. MI consisted of two, 45 minute individual sessions, while BA consisted of 5-10 minutes of advice and information on how to quit smoking. Eligible participants in both conditions were offered an eight week regimen of transdermal nicotine patch upon hospital discharge. MAIN OUTCOME MEASURES: Point prevalence abstinence, quit attempts, changes in smoking rate and longest quit attempt. Proximal outcomes included intent to change smoking behaviour (upon hospital discharge), and self efficacy for smoking cessation. RESULTS: MI did not lead to better smoking outcomes compared to BA. MI was more effective than BA for increasing self efficacy regarding ability to quit smoking. A significant interaction of treatment with baseline intention to quit smoking was also found. MI was more effective than BA for adolescents with little or no intention to change their smoking, but was actually less effective for adolescents with pre-existing intention to cut down or quit smoking. However, the effects on these variables were relatively modest and only moderately related to outcome. Adolescents with comorbid substance use disorders smoked more during follow up while those with anxiety disorders smoked less and were more likely to be abstinent. CONCLUSIONS: The positive effect of MI on self efficacy for quitting and the increase in intention to change in those with initially low levels of intentions suggest the benefits of such an intervention. However, the effects on these variables were relatively modest and only moderately related to outcome. The lack of overall effect of MI on smoking cessation outcomes suggests the need to further enhance and intensify this type of treatment approach for adolescent smokers with psychiatric comorbidity.

Adolescent↗

An outcome monitoring system for psychiatric inpatient care.

As the national impetus for examining treatment outcomes increases, the demand for cost-effective systems for psychiatric outcome monitoring grows. This article describes one local effort to develop a monitoring system at Butler Hospital in Providence, Rhode Island. The system uses a combination of face-to-face and computer interviews of a random sample of recently admitted inpatients, with follow-up interviews one month postdischarge and one year following admission. The patient self-report data and interviewer assessment data generated by these interviews are used to quantify patient symptoms and social functioning. With a large enough sample, these findings can be used to identify areas where patient care may be improved. The article also discusses the process and problems involved in implementing the monitoring system and presents illustrative preliminary findings.

Hospital Bed Capacity, 100 to 299↗

Fetal heart rate patterns and sudden infant death syndrome.

OBJECTIVE: To determine differences in electronic fetal monitoring patterns between infants who died of sudden infant death syndrome and controls. DESIGN: Case-control study (N = 127). SETTING: A tertiary-level women's hospital in Providence, Rhode Island. PARTICIPANTS: Infants born between 1990 and 1998 who subsequently died of sudden infant death syndrome and controls. Demographic and clinical data included medical maternal charts and fetal monitoring records. RESULTS: Compared with controls (n = 98), the mothers whose infants subsequently died of sudden infant death syndrome (n = 29) had lower birthweight babies (sudden infant death syndrome 2,840 vs. controls 3,385 g; p < .01), were younger (22 vs. 28 years; p < .01), were more likely to receive Medicaid health insurance (odds ratio 4.6; confidence interval 1.9-11.2), were more likely to be unmarried (odds ratio 5.2; confidence interval 2.1-12.8), had less intention to breastfeed (26% vs. 57%), and were more likely to smoke (odds ratio 4.6; confidence interval 9-11.2). MAIN OUTCOME MEASURES: There were no statistical differences in fetal heart rate variability or sleep/wake cycles detected between groups. CONCLUSION: Statistical differences were found in demographic characteristics between sudden infant death syndrome mother-infant couples and their controls. However, no differences were detected in the intrapartum electronic fetal monitoring records, specifically in variability and sleep/wake cycles.

Adult↗

First detection of group C rotavirus in fecal specimens of children with diarrhea in the United States.

Group C rotaviruses cause sporadic cases and outbreaks of acute diarrhea in children and adults in many countries but have never been identified from patients in the United States. Fecal specimens from children with diarrhea who were hospitalized in Providence, Rhode Island, were screened for group C rotaviruses if rotavirus was detected by electron microscopy but the specimens were negative for group A rotavirus by ELISA. Of 16 specimens examined, 3 were positive for group C rotavirus by ELISA using reagents specific to the Cowden strain of porcine group C rotavirus and all 16 were positive using a more sensitive assay: reverse transcriptase-polymerase chain reaction. Group C rotavirus infections occurred primarily among infants in winter in 4 of the 5 years examined and were acquired both in community and nosocomial settings. Future clinical and epidemiologic studies with group C rotavirus will require development of assays that are more sensitive and simpler to perform.

Animals↗

The development of a medical ethics curriculum in a General Internal Medicine Residency Program.

Since 1984, a three-year curriculum in medical ethics has operated at Rhode Island Hospital as part of the General Internal Medicine Residency Program at the Rhode Island Hospital and Brown University. The residency program was founded in 1980 to develop a model training program for internal medicine residents with a primary care focus. The three objective of the curriculum are (1) to enable residents to recognize the ethical implications of both inpatient and outpatient clinical cases, (2) to teach residents to recognize ethics issues and alternatives in order to arrive at a well-rounded clinical strategy, and (3) to help them learn to communicate sensitively with patients and others about these ethics issues and proposed management plans. The six major topics covered by the curriculum (for example, informed consent) are co-taught in a seminar format by one or more of the three members of the multidisciplinary ethics faculty (a philosopher, an internist, and a communications specialist) and experienced physicians. The authors describe the program, the roles of the various faculty members, the program's focus on case discussions, the receptivity of the residents and faculty to the program, the use and training of a multidisciplinary faculty, efforts aimed at evaluation, and future directions of the program.

Bioethical Issues↗

A morphometric study of the external ear: age- and sex-related differences.

The human ear is a defining feature of the face. Its subtle structures convey signs of age and sex that are unmistakable yet not easily defined. With analysis of normative cross-sectional data, this study explored anatomic and aesthetic differences in the ear between men and women, as well as changes in ear morphology with age. A total of 123 volunteers were randomly selected for this study. The cohort consisted of 89 women ages 19 to 65 years (median age, 42 years) and 34 men ages 18 to 61 years (median age, 35 years). The average total ear height across the entire cohort for both left and right ears was 6.30 cm, average lobular height was 1.88 cm, and average lobular width was 1.96 cm. As expected based on head size, significant sex-related differences were noted in the distance from the lateral palpebral commissure to both the helical root and insertion of the lobule. Measured distances in both vectors were approximately 4.6 percent longer in men than in women. Similarly, the height of the pinna was significantly larger in men than in women by approximately 6.5 percent. The average height and width of the lobule, however, were nearly identical in men and women. Analysis of age-related data showed a significant difference in the total ear height between the subpopulations; however, this difference was not significant after the lobular height was subtracted from total ear height, suggesting that the lobule was the only ear structure that changed significantly with age. In addition, lobular width decreased significantly with age. This study establishes normative data for ear morphology and clearly demonstrates the changes in earlobe morphology that occur with advancing age.

Adolescent↗

Gender differences in the behavioral manifestations of Alzheimer's disease.

OBJECTIVE: To examine the relationship between gender and specific types of behavior problems that occur in patients with Alzheimer's disease. DESIGN: This was an observational study using the Dementia Behavior Disturbance Scale to quantify and define behavioral problems encountered by caregivers. Multiple regressions were used to control for the possible influence of dementia severity as measured by the Mini-Mental State Examination and the duration of dementia. SETTING: Patients were sampled from the outpatient dementia clinics of Roger Williams Hospital and Miriam Hospital in Providence, Rhode Island. PARTICIPANTS: A total of 125 patients with probable Alzheimer's disease, defined by NINCDS-ADRDA diagnostic criteria, were included in the study. There were 75 women and 50 men. MAIN OUTCOME MEASURES: Caregivers rated the presence and frequency of 28 different behavior problems from the Dementia Behavior Disturbance Scale. Domains of behavior disturbance were then defined by a factor analysis of the data. RESULTS: Male and female groups were comparable for the demographic variables of age, education, and duration of dementia, as well as severity of depression, degree of cognitive impairment and overall severity of behavior disturbance. Among the six behavior factors that were defined, two were significantly related to gender. One factor, which included apathy and vegetative signs, was related to male gender; a second factor, which included reclusiveness and emotional lability, was related to female gender. CONCLUSIONS: Although overall severity of behavior disturbance in Alzheimer's disease may be related primarily to severity in dementia, significant differences in the types of behaviors manifested exist between males and females with the disease.

Aged↗

Teaching clinical medical ethics: a model programme for primary care residency.

Few residency training programmes explicitly require substantive exposure to issues in medical ethics and fewer still have a formal curriculum in this area. Traditional undergraduate medical ethics courses teach preclinical students to identify ethical issues and analyse them at a theoretical level. Residency training, however, is the ideal time to establish the critical behavioural link which makes ethics truly useful in clinical medicine. The General Internal Medicine Residency Training Program at Rhode Island Hospital has developed an integrated, three-year curriculum with the goals of helping residents to perceive ethical issues in clinical practice, to utilise basic philosophical principles in resolving ethical dilemmas and to communicate these issues clearly and sensitively to patients. The curriculum has been well received by residents and has had a hospital-wide impact. We believe that training residents in medical ethics and communication skills is an effective approach to developing physicians' humane qualities.

Ambulatory Care↗

Streptococcus viridans infections in midgestation.

During a 14-year period Streptococcus viridans was isolated at autopsy from 18 neonates or stillborn fetuses delivered in midgestation (18-28 weeks) at Women & Infants' Hospital in Providence, Rhode Island. Evidence of swallowed and/or aspirated, infected amniotic fluid by the fetus was found in 16 of 18 cases along with chorioamnionitis and funisitis. Of the 18 cases, maternal infection was documented in 6 cases; delivery was preceded by spontaneous rupture of membranes in 9 cases and vaginal bleeding in 7 cases. Poor maternal obstetric history was indicated by only 12 living children from 42 total pregnancies in the 12 multigravidas in the study. It is concluded that S. viridans may have a significant role in the pathogenesis of second trimester amniotic fluid infections with abortions and fetal and neonatal sepsis.

Chorioamnionitis↗

House officer responses to impaired physicians.

Although physician impairment may have substantial personal and clinical consequences, it is commonly held that physicians fail to act in the case of impaired colleagues. To learn about initial responses when confronted with impaired house officers and attending physicians, we administered five case scenarios to all 76 internal medicine house officers at a large, urban, teaching hospital. House officer responses to an alcohol-impaired physician differed depending on whether that physician was an attending physician or a house officer. Seventy-two percent of house officers would tell the chief resident about the alcohol-impaired attending physician, whereas 96% would confront the alcohol-impaired house officer personally. The most common response to the incompetent attending physician (67%) and the incompetent house officer (49%) was to tell the chief resident; only 25% would confront an incompetent house officer. In contrast, 71% would confront a depressed house officer.

Attitude of Health Personnel↗

Effects of maternal transport on admission patterns at a tertiary care center.

Major changes have occurred in the delivery of perinatal care during the last two decades. Documentation of perinatal regionalization statistics at Women and Infants Hospital of Rhode Island in Providence support the increasing proportion of low birth weight infants in the region being born at the tertiary care center, the decreasing number of neonatal transports in concert with an increasing number of maternal transports, and the increasing proportion of neonatal transports with birth weights greater than or equal to 2500 gm. Patterns of modern perinatal care are materially changing the delivery of health care at tertiary care facilities.

Birth Weight↗