Fresno VA Medical Center

BACKGROUND: Non-Q-wave myocardial infarction is usually managed according to an "invasive" strategy (i.e., one of routine coronary angiography followed by myocardial revascularization). METHODS: We randomly assigned 920 patients to either "invasive" management (462 patients) or "conservative" management, defined as medical therapy and noninvasive testing, with subsequent invasive management if indicated by the development of spontaneous or inducible ischemia (458 patients), within 72 hours of the onset of a non-Q-wave infarction. Death or nonfatal infarction made up the combined primary end point. RESULTS: During an average follow-up of 23 months, 152 events (80 deaths and 72 nonfatal infarctions) occurred in 138 patients who had been randomly assigned to the invasive strategy, and 139 events (59 deaths and 80 nonfatal infarctions) in 123 patients assigned to the conservative strategy (P=0.35). Patients assigned to the invasive strategy had worse clinical outcomes during the first year of follow-up. The number of patients with one of the components of the primary end point (death or nonfatal myocardial infarction) and the number who died were significantly higher in the invasive-strategy group at hospital discharge (36 vs. 15 patients, P=0.004, for the primary end point; 21 vs. 6, P=0.007, for death), at one month (48 vs. 26, P=0.012; 23 vs. 9, P=0.021), and at one year (111 vs. 85, P=0.05; 58 vs. 36, P= 0.025). Overall mortality during follow-up did not differ significantly between patients assigned to the conservative-strategy group and those assigned to the invasive-strategy group (hazard ratio, 0.72; 95 percent confidence interval, 0.51 to 1.01). CONCLUSIONS: Most patients with non-Q-wave myocardial infarction do not benefit from routine, early invasive management consisting of coronary angiography and revascularization. A conservative, ischemia-guided initial approach is both safe and effective.

The role of myocardial oxygen demand in the genesis of silent myocardial ischemia was evaluated by measuring the heart rate and blood pressure changes preceding the silent ischemic events during daily life in 25 men with proven coronary artery disease. Simultaneous 24-48-hour ambulatory electrocardiographic and blood pressure monitoring were performed during unrestricted daily activities. Of the 92 transient ischemic events recorded during monitoring, 85 (92%) were silent. Sixty-one percent of the silent events were preceded by an increase in the heart rate of 5 beats/min or more. Seventy-three percent of the silent ischemic events showed an average increase of 10 mm Hg in systolic blood pressure within 6 minutes preceding the onset of ST segment depression. The silent ischemic events showed a circadian pattern with a high density (34% of total events) between 6:00 AM and noon. The increase in heart rate and blood pressure paralleled the increase in silent ischemic events during these hours. These results showing significant (p less than 0.001 for both) increases in heart rate and blood pressure preceding a majority of silent ischemic events suggest that increase in myocardial oxygen demand plays a significant role in the genesis of silent ischemia. This pathophysiological mechanism has important therapeutic implications.

CONTEXT: Internal medicine residency training is demanding and residents can experience a wide variety of professional and personal difficulties. A problem resident is defined by the American Board of Internal Medicine as "a trainee who demonstrates a significant enough problem that requires intervention by someone of authority." Data are sparse regarding identification and management of such residents. OBJECTIVE: To gain more understanding of the prevalence, identification, management, and prevention of problem residents within US internal medicine residency programs. DESIGN, SETTING, AND PARTICIPANTS: Mailed survey of all 404 internal medicine residency program directors in the United States in October 1999, of whom 298 (74%) responded. MAIN OUTCOME MEASURES: Prevalence of problem residents; type of problems encountered; factors associated with identification and management of problem residents. RESULTS: The mean point prevalence of problem residents during academic year 1998-1999 was 6.9% (SD, 5.7%; range, 0%-39%), and 94% of programs had problem residents. The most frequently reported difficulties of problem residents were insufficient medical knowledge (48%), poor clinical judgment (44%), and inefficient use of time (44%). Stressors and depression were the most frequently identified underlying problems (42% and 24%, respectively). The most frequent processes by which problem residents were discovered included direct observation (82%) and critical incidents (59%). Chief residents and attending physicians most frequently identified problem residents (84% and 76%, respectively); problem residents rarely identified themselves (2%). Many program directors believed that residents who are from an underrepresented minority, are international medical graduates, or are older than 35 years are at increased risk of being identified as a problem resident (P<.05). Program directors believed that frequent feedback sessions (65%) and an assigned mentor for structured supervision (53%) were the most helpful interventions. CONCLUSION: Nearly all internal medicine residency programs in this sample had problem residents, whose presenting characteristics and underlying issues were diverse and complex. JAMA. 2000;284:1099-1104

Acute myocardial infarction (AMI) and sudden cardiac death (SCD) are among the most serious and catastrophic of acute cardiac disorders, accounting for hundreds of thousands of deaths each year worldwide. Although the incidence of AMI has been decreasing in the US according to the American Heart Association, heart disease is still the leading cause of mortality in adults. In most cases of AMI and in a majority of cases of SCD, the underlying pathology is acute intraluminal coronary thrombus formation within an epicardial coronary artery leading to total or near-total acute coronary occlusion. This article summarizes our current understanding of the pathophysiology of these acute coronary syndromes and briefly discusses new approaches currently being researched in an attempt to define and ultimately reduce their incidence.

OBJECTIVE: The objective was to assess anxiety and burnout levels, home life changes, and measures to relieve stress of U.S. academic emergency medicine (EM) physicians during the COVID-19 pandemic acceleration phase. METHODS: We sent a cross-sectional e-mail survey to all EM physicians at seven academic emergency departments. The survey incorporated items from validated stress scales and assessed perceptions and key elements in the following domains: numbers of suspected COVID-19 patients, availability of diagnostic testing, levels of home and workplace anxiety, severity of work burnout, identification of stressors, changes in home behaviors, and measures to decrease provider anxiety. RESULTS: A total of 426 (56.7%) EM physicians responded. On a scale of 1 to 7 (1 = not at all, 4 = somewhat, and 7 = extremely), the median (interquartile range) reported effect of the pandemic on both work and home stress levels was 5 (4-6). Reported levels of emotional exhaustion/burnout increased from a prepandemic median (IQR) of 3 (2-4) to since the pandemic started a median of 4 (3-6), with a difference in medians of 1.8 (95% confidence interval = 1.7 to 1.9). Most physicians (90.8%) reported changing their behavior toward family and friends, especially by decreasing signs of affection (76.8%). The most commonly cited measures cited to alleviate stress/anxiety were increasing personal protective equipment (PPE) availability, offering rapid COVID-19 testing at physician discretion, providing clearer communication about COVID-19 protocol changes, and assuring that physicians can take leave for care of family and self. CONCLUSIONS: During the acceleration phase, the COVID-19 pandemic has induced substantial workplace and home anxiety in academic EM physicians, and their exposure during work has had a major impact on their home lives. Measures cited to decrease stress include enhanced availability of PPE, rapid turnaround testing at provider discretion, and clear communication about COVID-19 protocol changes.

BACKGROUND: Computed tomography of the head (HCT) is an integral part of the diagnosis and management of the patient with head injury, but the utility of repeated HCT performed solely for routine follow-up in the patient with blunt head trauma has not been defined. In the absence of clinical indications, routinely repeated HCT, even in patients with significant brain injury, does not contribute to patient care. METHODS: Trauma registry records at a Level I trauma center from July 1, 1997, to June 30, 2002, were reviewed. Patients with severe blunt head injury (Abbreviated Injury Scale score > or = 3) admitted to the intensive care unit and who had a repeat HCT scan obtained for scheduled follow-up were included. Those patients with initial craniotomy, repeat HCT more than 72 hours after the initial HCT, or repeat HCT ordered for clinical indications were excluded. Data included were age, mechanism of injury, time to initial (HCT1) and repeat HCT (HCT2), indications for HCT2, and HCT findings. Additional data included Glasgow Coma Scale (GCS) score (admission and at HCT2); Injury Severity Score; occurrence of hypotension, coagulopathy, or elevated intracranial pressure (ICP); interventions made; and patient outcome. RESULTS: Entry criteria were met in 462 patients. Most were injured in motor vehicle crashes; the average age was 36 years and the mean initial GCS score was 9. The mean time to HCT1 was 1.3 hours and the mean time to HCT2 was 22.6 hours. HCT2 showed worsening in 85 patients (18.4%), and 16 patients had interventions in response to HCT2 (repeat HCT in 8, ICP monitoring or drainage in 6, and craniotomy in 2). No patient undergoing routine repeat HCT without other clinical findings required intervention. All patients with worsening HCT findings requiring intervention had coagulopathy, hypotension, ICP elevation, or marked decrease in GCS score. CONCLUSION: In the absence of clinical indicators or risk factors, repeat HCT after blunt head injury does not alter patient management and is unnecessary.

Emergency medicine (EM) program directors have expressed a desire for more evaluative data to be included in application materials. This is consistent with frustrations expressed by program directors of multiple specialties, but mostly by those in specialties with more competitive matches. Some of the concerns about traditional narrative letters of recommendation included lack of uniform information, lack of relative value given for interval grading, and a perception of ambiguity with regard to terminology. The Council of Emergency Medicine Residency Directors established a task force in 1995 that created a standardized letter of recommendation form. This form, to be completed by EM faculty, requests that objective, comparative, and narrative information be reported regarding the residency applicant.

BACKGROUND: The mechanism for clearing the cervical spine in patients with altered mental status remains controversial. Recommendations have ranged from removal of the cervical collar after 24 hours in patients with normal radiographs, to indefinite immobilization in a cervical collar, and recently cervical flexion-extension examinations using dynamic fluoroscopy. The purpose of this study was to evaluate the efficacy and safety of dynamic fluoroscopy flexion-extension examinations in identifying ligamentous cervical spine injury and clearing the cervical spine in patients with altered mental status after trauma. METHODS: Patients with a Glasgow Coma Scale score < 13 for greater than 48 hours after admission and normal cervical spine radiographs were candidates for fluoroscopic evaluation. The protocol required visualization of the entire cervical spine, through T1, through full extension and flexion under the direct supervision of a radiologist. Oblique fluoroscopic views were obtained, as necessary, to visualize the cervicothoracic junction. Demographic data, fluoroscopy time, total time per study, true and false positives and negatives, and complications were recorded. RESULTS: From July 1992 through December 1999, fluoroscopic examinations were performed on 301 patients. There were 297 true-negative examinations, 2 true-positive examinations (stable injuries), 1 false-negative examination, and 1 false-positive examination. The incidence of ligamentous injury identified by fluoroscopy in this study was 2 of 301 (0.7%). Unstable cervical spine ligamentous injuries were identified in only 0.02% of all trauma patients. One patient developed quadriplegia when fluoroscopic evaluation was performed after two protocol violations. CONCLUSION: Unstable cervical spine ligamentous injury without fracture is a rare occurrence. The cervical spine may be cleared after a normal cervical spine series (plain radiograph and computed tomographic scan) as recommended in the 1998 Eastern Association for the Surgery of Trauma guidelines. If dynamic fluoroscopy is to be used, adherence to the protocol, including review of the cervical spine radiographs before fluoroscopy and visualization of the entire cervical spine, C1-T1, is mandatory to ensure patient safety.

OBJECTIVE: To compare the prehospital time intervals from patient contact and medication administration to clinical response for intranasal (IN) versus intravenous (IV) naloxone in patients with suspected narcotic overdose. METHODS: This was a retrospective review of emergency medical services (EMS) and hospital records, before and after implementation of a protocol for administration of intranasal naloxone by the Central California EMS Agency. We included patients with suspected narcotic overdose treated in the prehospital setting over 17 months, between March 2003 and July 2004. Paramedics documented dose, route of administration, and positive response times using an electronic record. Clinical response was defined as an increase in respiratory rate (breaths/min) or Glasgow Coma Scale score of at least 6. Main outcome variables included time from medication to clinical response and time from patient contact to clinical response. Secondary variables included numbers of doses administered and rescue doses given by an alternate route. Between-group comparisons were accomplished using t-tests and chi-square tests as appropriate. RESULTS: One hundred fifty-four patients met the inclusion criteria, including 104 treated with IV and 50 treated with IN naloxone. Clinical response was noted in 33 (66%) and 58 (56%) of the IN and IV groups, respectively (p = 0.3). The mean time between naloxone administration and clinical response was longer for the IN group (12.9 vs. 8.1 min, p = 0.02). However, the mean times from patient contact to clinical response were not significantly different between the IN and IV groups (20.3 vs. 20.7 min, p = 0.9). More patients in the IN group received two doses of naloxone (34% vs. 18%, p = 0.05), and three patients in the IN group received a subsequent dose of IV or IM naloxone. CONCLUSIONS: The time from dose administration to clinical response for naloxone was longer for the IN route, but the overall time from patient contact to response was the same for the IV and IN routes. Given the difficulty and potential hazards in obtaining IV access in many patients with narcotic overdose, IN naloxone appears to be a useful and potentially safer alternative.

BACKGROUND: Hip fractures result in both health and cost burdens from a public health perspective and have a major impact on the health care system in the USA. The purpose was to examine whether there were systematic differences in hip fracture incidence and 30-, 90-, and 365-day mortality after hip fracture in the California population as a function of age, gender, and race/ethnicity from 2000-2011. METHODS: This was a population-based study from 2000 to 2011 using data from the California Office of Statewide Health and Planning and Development (OSHPD, N = 317,677), California State Death Statistical Master File records (N = 224,899), and the US Census 2000 and 2010. There were a total of 317,677 hospital admissions for hip fractures over the 12-year span and 24,899 deaths following hip fractures. All participants without linkage (substituted for social security) numbers were excluded from mortality rate calculations. Variation in incidence and mortality rates across time, gender, race/ethnicity, and age were assessed using Poisson regression models. Odds ratio and 95 % confidence intervals are provided. RESULTS: The incidence rate of hip fractures decreased between 2000 and 2011 (odds ratio (OR) = 0.98, 95 % confidence interval (CI) 0.98, 0.98). Mortality rates also decreased over time. There were gender, race/ethnicity, and age group differences in both incidence and mortality rates. CONCLUSIONS: Males were half as likely to sustain a hip fracture, but their mortality within a year of the procedure is almost twice the rate than women. As age increased, the prevalence of hip fracture increased dramatically, but mortality did not increase as steeply. Caucasians were more likely to sustain a hip fracture and to die within 1 year after a hip fracture. The disparities in subpopulations will allow for targeted population interventions and opportunities for further research.

Silent myocardial ischemia has been shown to occur far more frequently than anginal episodes in patients with coronary artery disease. Both an increase in myocardial oxygen demand and abnormalities of coronary vasomotor tone appear to play a significant role in the genesis of silent ischemia. Recent data show that in excess of 40% of patients with stable angina have frequent episodes of silent ischemia. The presence of silent ischemia predicts an increased risk of coronary events and cardiac death. Based on these data, it has been proposed that anti-ischemic therapy should be directed toward control of total ischemic burden. Although several recent studies have demonstrated efficacy of various antianginal drugs in reducing the number and duration of silent ischemic episodes, none has demonstrated beneficial effect on the associated adverse prognosis.

BACKGROUND: Domestic violence (DV) has received increased recognition as a significant mechanism of injury. To improve awareness about DV at our institution, an educational program was presented to the departments of surgery and emergency medicine. Pre and posttests were given and improvement in knowledge was demonstrated. In addition, a screening question for DV was added to the trauma history and physical (H & P) form. This study was done to determine the long-term efficacy of these efforts in increasing recognition of DV and referral to social services in patients admitted to the trauma service. Recognition of DV and appropriate referral should be increased after education and change in H & P form. METHODS: All patients admitted to the trauma service at a Level I trauma center over a 10 month period with the mechanism of injury "assault" were reviewed. DV was determined to be present, likely, unknown, or absent based on information from the prehospital report and medical records. The DV screen question was reviewed for use and accuracy. RESULTS: During the study period, 1,550 patients were admitted to the trauma service, with assault listed as the mechanism of injury for 217 (14%). DV was confirmed or likely in 27 patients (12.4% of the assaults). Of patients with confirmed or likely DV, only 7 received appropriate referrals, with 2 generated by the nursing staff. Of the confirmed and likely DV patients, 17 (63%) were sent home without investigation of safety and only 21% of all assault victims had any social services evaluation (usually to investigate funding or placement). The DV screen was used in only 12 patients. Reasons given for failure to complete the DV screen on the H & P included examiner discomfort in asking the question, and an environment judged to be inappropriate (resuscitation area in the emergency department). CONCLUSION: DV is unrecognized and underreported. Efforts to improve recognition and reporting of DV events need to be ongoing. Screening for DV is not effectively done as part of the initial evaluation. Assessment for DV may be more appropriate as part of the tertiary survey.

The utility of base deficit (BD) as a marker of shock and as an indicator of resuscitation requirements has been recognized in the trauma population. Base deficit in thermally injured patients has not been closely examined. The purpose of this study was to evaluate the usefulness of initial BD related to other resuscitation parameters in thermally injured patients. Burn center records over a 2-year period were reviewed; patients who survived at least 24 hours and had initial arterial blood gases were included. Parkland estimated fluid requirements underestimated actual volume requirements, but Parkland-calculated fluid requirements were related (p < 0.01) to actual volume requirements. BD had a better correlation to actual volume requirements, and a BD of -6 or less correlated with larger burn size (23% +/- 2% vs 47% +/- 9% total body surface area), and markedly increased mortality rate (9% vs 72%, p < 0.001).

OBJECTIVES: To determine the prevalence of diabetes and awareness, treatment and control of cardiovascular risk factors in population-based participants in India. METHODS: A study was conducted in 11 cities in different regions of India using cluster sampling. Participants were evaluated for demographic, biophysical, and biochemical risk factors. 6198 participants were recruited, and in 5359 participants (86.4%, men 55%), details of diabetes (known or fasting glucose >126 mg/dL), hypertension (known or blood pressure >140/>90 mm Hg), hypercholesterolemia (cholesterol >200 mg/dL), low high-density lipoprotein (HDL) cholesterol (men <40, women <50 mg/dL), hypertriglyceridemia (>150 mg/dL), and smoking/tobacco use were available. Details of awareness, treatment, and control of hypertension and hypercholesterolemia were also obtained. RESULTS: The age-adjusted prevalence (%) of diabetes was 15.7 (95% CI 14.8 to 16.6; men 16.7, women 14.4) and that of impaired fasting glucose was 17.8 (16.8 to 18.7; men 17.7, women 18.0). In participants with diabetes, 27.6% were undiagnosed, drug treatment was in 54.1% and control (fasting glucose ≤130 mg/dL) in 39.6%. Among participants with diabetes versus those without, prevalence of hypertension was 73.1 (67.2 to 75.0) vs 26.5 (25.2 to 27.8), hypercholesterolemia 41.4 (38.3 to 44.5) vs 14.7 (13.7 to 15.7), hypertriglyceridemia 71.0 (68.1 to 73.8) vs 30.2 (28.8 to 31.5), low HDL cholesterol 78.5 (75.9 to 80.1) vs 37.1 (35.7 to 38.5), and smoking/smokeless tobacco use in 26.6 (23.8 to 29.4) vs 14.4 (13.4 to 15.4; p<0.001). Awareness, treatment, and control, respectively, of hypertension were 79.9%, 48.7%, and 40.7% and those of hypercholesterolemia were 61.0%, 19.1%, and 45.9%, respectively. CONCLUSIONS: In the urban Indian middle class, more than a quarter of patients with diabetes are undiagnosed and the status of control is low. Cardiovascular risk factors-hypertension, hypercholesterolemia, low HDL cholesterol, hypertriglyceridemia, and smoking/smokeless tobacco use-are highly prevalent. There is low awareness, treatment, and control of hypertension and hypercholesterolemia in patients with diabetes.

INTRODUCTION: Patients with head injuries frequently have abnormal coagulation studies. Monitoring intracranial pressure (ICP) in head injured patients is common practice, but no best practice guidelines exist for coagulation parameters for ICP monitor placement. PURPOSE: To test the hypothesis that hemorrhagic complication rates from ICP monitor placement are low and that the use of FFP to correct coagulation parameters to "normal" is not indicated. METHODS: Retrospective review of all patients admitted to a Level I trauma center over a 3 year period, who underwent fiberoptic intraparenchymal ICP monitoring was undertaken. Inclusion criteria were coagulation studies (prothrombin time (PT), partial thromboplastin time (PTT), international normalized ratio (INR), platelet count) before ICP monitor placement and head CT scans to assess for hemorrhage before and after monitor placement. Data collected included age, Glasgow coma score (GCS), head region abbreviated injury score (H_AIS), time to ICP monitor placement, complications and outcomes. RESULTS: From 8/1/00 through 7/31/03, 5163 trauma patients were admitted, and 157 met inclusion criteria. Patients were stratified by INR, at the time of ICP placement as normal (0.8-1.2, 103 patients), borderline (1.3-1.6, 42 patients) and increased (>/=1.7, 12 patients). There was no difference between the groups in age, gender or H_AIS. Twenty two patients had component therapy to correct coagulopathy before ICP insertion, but 10 had INRs in the borderline group and 12 remained with INRs >/=1.7. Eleven patients had platelet counts 50,000-100,000 at ICP monitor placement, despite platelet transfusions. Time from admission to ICP monitor placement was significantly longer in patients who received component therapy (19.2 +/- 19.7 hours versus 8.8 +/- 13.9 hours, p < 0.002). Three patients had clinically insignificant, petechial hemorrhages (1.9%); one in each group, with INRs of 1.2, 1.3, and 2.5, respectively. CONCLUSIONS: In patients with INR </=1.6, hemorrhagic complications after ICP monitor placement were infrequent. The use of FFP to "normalize" INR below this threshold is not supported by this data and delays monitor placement.

BACKGROUND: Antibiotics are routinely used for diverticulitis irrespective of severity. Current practice guidelines favor against the use of antibiotics for acute uncomplicated diverticulitis. OBJECTIVE: We performed a systematic review and meta-analysis to examine the role of antibiotic use in an episode of uncomplicated diverticulitis. DATA SOURCES: PubMed/Medline, Embase, Scopus, and Cochrane were used. STUDY SELECTION: Eligible studies included those with patients with uncomplicated diverticulitis receiving any antibiotics compared with patients not receiving any antibiotics (or observed alone). MAIN OUTCOME MEASURES: Pooled odds rate of total complications, treatment failure, recurrent diverticulitis, readmission rate, sigmoid resection, mortality rate, and length of stay were measured. RESULTS: Of 1050 citations reviewed, 7 studies were eligible for the analysis. There were total of 2241 patients: 895 received antibiotics (mean age = 59.1 y; 38% men) and 1346 did not receive antibiotics (mean age = 59.4 y; 37% men). Antibiotics were later added in 2.7% patients who initially were observed off antibiotics. Length of hospital stay was not significantly different among either group (no antibiotics = 3.1 d vs antibiotics = 4.5 d; p = 0.20). Pooled rate of recurrent diverticulitis was not significantly different among both groups (pooled OR = 1.27 (95%, CI 0.90-1.79); p = 0.18). Rate of total complications (pooled OR = 1.99 (95% CI, 0.66-6.01); p = 0.22), treatment failure (pooled OR = 0.68 (95% CI, 0.42-1.09); p = 0.11), readmissions (pooled OR = 0.75 (95% CI, 0.44-1.30); p = 0.31). and patients who required sigmoid resection (pooled OR = 3.37 (95% CI, 0.65-17.34); p = 0.15) were not significantly different among patients who received antibiotics and those who did not. Mortality rates were 4 of 1310 (no-antibiotic group) versus 4 of 863 (antibiotic group). LIMITATIONS: Only 2 randomized controlled studies were available and there was high heterogeneity in existing data. CONCLUSIONS: This meta-analysis of current literature shows that patients with uncomplicated diverticulitis can be monitored off antibiotics.

We present the case of an intraforaminal iliosacral screw placed percutaneously with aid of C-arm using inlet, outlet, and lateral views of the pelvis. The iliosacral screw was placed above the S1 foramen on the outlet view, into the middle of S1 via the ala on the inlet view, and below the cortical shadow of the ala on the lateral view. The patient was neurologically intact postoperatively, but began to complain of severe radicular pain in the S1 distribution down to the foot within 1 week postsurgery. There was mild weakness of plantar flexion. Postoperative computed tomography scan showed that the iliosacral screw was within the S1 foramen. Because of the tangential nature of the S1 foramen, slight posterior placement of the screw into the S1 body and not into the promontory resulted in violation of the foramen despite it being above the cortical shadow on the outlet view.

Degos' disease, a rare multisystem vasculopathy of unknown etiology, only occasionally involves the nervous system. We report the Mayo Clinic experience of the neurologic features of Degos' disease in a series of 15 patients. All 15 patients had the typical skin lesions of Degos' disease, confirmed by skin biopsy. Ten patients developed neurologic manifestations including fatal hemorrhagic or ischemic strokes (n=5), disabling polyradiculoneuropathy (n=1), and nonspecific neurologic symptoms without objective findings (n=4). Results of laboratory tests varied but none were pathognomonic of the disease. Long-term follow-up revealed death in six patients; nine patients were nearly asymptomatic. Immunosuppressive and antiplatelet agents were not of benefit. CNS infarcts and hemorrhages with intravascular thrombi, but without evidence of vasculitis, were characterized features at autopsy.

Comparative genomic hybridization (CGH) arrays have significantly changed the approach to identifying genetic alterations causing intellectual disability and congenital anomalies. Several studies have described the microduplication of Xp22.31, involving the STS gene. In such reports characteristic features and pathogenicity of Xp22.31 duplications remains a subject of debate. Here we present a series of nine previously unreported individuals with Xp22.31 duplications, found through microarray analysis in the course of genetic workup for developmental delay, associated with a combination of talipes anomalies, seizures and/or feeding difficulties. The size of the Xp22.31 duplications ranged from 294 kb to 1.6 Mb. We show a comparison of the breakpoints, inheritance and clinical phenotype, and a review of the literature. This clinically detailed series of Xp22.31 duplication patients provides evidence that the Xp22.31 duplication contributes to a common phenotype.

Spiegel's ( 20 ) distinction between studies of relations and studies of transactions are reviewed and applied to the tendency of current research to assess transactional theory using methods that are focused on individual elements. Several of the complexities of transactional research are reviewed with a call for the utilization of methods that are more commensurate with the level of theory under test.