Social cognition dysfunction in adolescents with 22q11.2 deletion syndrome (velo-cardio-facial syndrome): relationship with executive functioning and social competence/functioning.

BACKGROUND: Social difficulties are often noted among people with intellectual disabilities. Children and adults with 22q.11.2 deletion syndrome (22q11DS) often have poorer social competence as well as poorer performance on measures of executive and social-cognitive skills compared with typically developing young people. However, the relationship between social functioning and more basic processes of social cognition and executive functioning are not well understood in 22q11DS. The present study examined the relationship between social-cognitive measures of emotion attribution and theory of mind with executive functioning and their contribution to social competence in 22q11DS. METHOD: The present cross-sectional study measured social cognition and executive performance of 24 adolescents with 22q11DS compared with 27 age-matched typically developing controls. Social cognition was tested using the emotion attribution task (EAT) and a picture sequencing task (PST), which tested mentalising (false-belief), sequencing, cause and effect, and inhibition. Executive functioning was assessed using computerised versions of the Tower of London task and working memory measures of spatial and non-spatial ability. Social competence was also assessed using the parent-reported Strengths and Difficulties Questionnaire. RESULTS: Adolescents with 22q11DS showed impaired false-belief, emotion attribution and executive functioning compared with typically developing control participants. Poorer performance was reported on all story types in the PST, although, patterns of errors and response times across story types were similar in both groups. General sequencing ability was the strongest predictor of false-belief, and performance on the false-belief task predicted emotion attribution accuracy. Intellectual functioning, rather than theory of mind or executive functioning, predicted social competence in 22q11DS. CONCLUSIONS: Performance on social-cognitive tasks of theory of mind indicate evidence of a general underlying dysfunction in 22q11DS that includes executive ability to understand cause and effect, to logically reason about social scenarios and also to inhibit responses to salient, but misleading cues. However, general intellectual ability is closely related to actual social competence suggesting that a generalised intellectual deficit coupled with more specific executive impairments may best explain poor social cognition in 22q11DS.

A Pilot Study of BRAIN BOOTCAMP, a Low-Intensity Intervention on Diet, Exercise, Cognitive Activity, and Social Interaction to Improve Older Adults' Dementia Risk Scores.

BACKGROUND: Little is known about the impact of short, low-intensity multidomain dementia risk reduction interventions in older adults. OBJECTIVES: To examine the effectiveness and feasibility of a low-intensity multidomain lifestyle intervention on dementia risk and dementia literacy in Australian older adults. DESIGN: Single-group pre-post design. SETTING: Community-dwelling. PARTICIPANTS: A total of 853 older Australians (Mean age=73.3 years, SD=6.1) recruited from the community. INTERVENTION: A 3-month dementia risk reduction program, BRAIN BOOTCAMP, including education, personalised risk information, physical cues for healthier choices and goal setting and planning to target four modifiable risk factors of diet, exercise, cognitive activity and social interaction in older adults. MEASUREMENTS: The 'LIfestyle for BRAin health' (LIBRA) index was used to assess participants' modifiable dementia risk based on 12 factors, with higher scores indicating greater risk. Dementia literacy was measured using a modified questionnaire derived from Dutch and British surveys, encompassing knowledge, risk reduction, and awareness aspects. Paired t-tests were used to compare dementia risk scores and dementia literacy before and after the program. Multivariate regressions were performed to identify sociodemographic and psychological factors associated with change in the LIBRA index. RESULTS: Program attrition was high (58.3%). Participants who completed the program had decreased dementia risk scores (Cohen's d=0.59, p<0.001), increased dementia literacy and awareness (Cohen's d=0.64, p<0.001) and increased motivation to change lifestyle behaviors (Cohen's d=0.25-0.52, p<0.016). Participants with higher motivational beliefs had greater dementia risk reduction. CONCLUSIONS: Improving older adults' motivation and knowledge may help modify lifestyle behaviors to reduce dementia risk. However, program attrition remains a challenge, suggesting the need for strategies to enhance participant engagement and retention in such interventions.

Development of a screening tool for prediction of children at risk for lead exposure in a midwestern clinical setting.

OBJECTIVE: Universal screening for childhood lead poisoning is becoming quite common, with many states having legislation requiring screening. We set out to determine whether a questionnaire could be used to identify children at risk for exposure to lead to determine whether selective screening of those at risk was possible. METHODS: Parents of 370 children 12 to 36 months of age having well-child examinations completed a questionnaire and their children were screened by a fingerstick capillary blood lead test at two clinics. RESULTS: Of patients from clinic A, 5.4% had lead levels > or = 10 micrograms/dL compared with 16.8% of those from clinic B (P < .001). This difference between clinics could not be explained by the demographic characteristics of the patients or by differences in their potential exposures to lead. We evaluated the five questions suggested by Centers for Disease Control and Prevention for anticipatory guidance for their ability to identify children with elevated blood lead levels. In clinic A, this instrument had a sensitivity of 76.9% and a negative predictive value of 96.5%. In clinic B, it had a sensitivity of 63.6% and a negative predictive value of 81.4%. Based on an assessment of significant items from a large questionnaire, we determined five questions that were the best predictors of risk. On the basis of this risk assessment, 100% of the children from clinic A with elevated lead levels and 90.9% of the children from clinic B with elevated lead levels were classified as being at "high risk." Had this risk assessment been used as an initial screen in this sample, 40% of the patients from clinic A and 37% of the patients from clinic B would not have been screened with a blood lead test, because they were classified as being at "low risk." CONCLUSIONS: Results of this study suggest that there is great variability in the prevalence of elevated lead levels and potential risks between clinics within a fairly homogeneous community; however, selective screening with a community-specific questionnaire may be feasible if the prevalence is low and the risks to the population are known.

Late results after femoral artery embolectomy.

A review of 100 femoral artery embolectomies performed on 88 consecutive patients during a 10-year period was conducted to establish immediate and long-term results. There were 48 (55%) male and 40 (45%) female patients. Ages ranged from 33 to 97 years (mean, 73 years). Local anesthesia was used in 84 (84%) cases. Fifteen (17%) patients died within 30 days of the procedure. Major limb amputation was required within 30 days of operation in 16 (16%) patients. Mortality rate in the group of patients needing early amputation was 50% compared with 11% for those who underwent successful embolectomy procedures (p less than 0.001). Current follow-up was established for all patients. Five-year and 10-year survival rates for the entire group were 40% and 35%, respectively. The most frequent causes of late death after femoral artery embolectomy were arteriosclerotic heart disease (26%), cerebrovascular accident (26%), and advanced carcinoma (21%). All 39 remaining survivors were located 8 to 126 months (mean, 48 months) after discharge to complete a questionnaire designed to assess quality of life and symptoms of vascular insufficiency. Thirty-five (90%) survivors were fully ambulatory and 27 (70%) lived in their own homes. Although early survival is decreased after femoral embolectomy, long-term survivors can be expected to live independently with excellent limb salvage and function.

Subclinical hormone secretion by incidentally discovered adrenal masses.

OBJECTIVE: To determine the frequency of subclinical hormone secretion in incidentally discovered adrenal masses. DESIGN: We reviewed the radiologic reports of 1779 consecutive computed tomographic scans of the chest, abdomen, and pelvis. SETTING: Regional referral medical center. PATIENTS: Eighty-nine patients with abnormalities of one or both adrenal glands were identified. Patients with nonadrenal gland malignant neoplasms, primary aldosteronism, adrenal hemorrhage, and death or severe illness were not investigated. The final study group consisted of 26 patients with incidentally discovered adrenal masses. MAIN OUTCOME MEASURES: Aldosterone secretion was assessed by measuring plasma renin activity and the plasma aldosterone concentration in patients with unexplained hypokalemia. We evaluated cortisol secretion by performing a 1-mg overnight dexamethasone suppression test and by measuring the corticotropin concentration at 8 AM by a sensitive method. In patients with low corticotropin values, we also measured the 24-hour urinary excretion of free cortisol and 17-ketosteroids and assessed diurnal variation by measuring plasma cortisol concentrations at 8 AM and 4 PM. Adrenal medullary function was studied by measuring urinary free catecholamines. RESULTS: One patient had unrecognized primary aldosteronism, two patients had elevated free catecholamine excretion, and three patients (12%) had subclinical Cushing's syndrome. CONCLUSION: Based on our observations and a review of the literature, we conclude that subclinical hormone secretion, especially cortisol secretion, is more common in patients with incidentally discovered adrenal masses than previously appreciated. Surgeons and anesthesiologists must be alert to the possibility that adrenal insufficiency or a hypertensive crisis may develop in the perioperative period in patients with incidentally discovered adrenal masses.

Late results of peripheral vascular surgery in patients 80 years of age and older.

During a seven-year period, 114 patients 80 years of age and older underwent 119 peripheral arterial procedures. There were 26 elective aortic reconstructions, nine ruptured abdominal aortic aneurysm (AAA) repairs, 33 femoropopliteal bypasses, 13 femorotibial bypasses, 21 femoral embolectomies, and 17 miscellaneous procedures. Early mortality, morbidity, and Goldman cardiac risk factors were determined by chart review. All 48 survivors returned for current noninvasive vascular examination and life-style assessment. Perioperative mortality after elective AAA resection was 4.3%, vs 78% after ruptured AAA. Perioperative mortality after infrainguinal bypass was nil. Five-year survivals after elective aortic and infrainguinal reconstructions were 54% and 30%, respectively. Of 18 patients studied 19 to 68 months after infrainguinal bypass, limb salvage was achieved in 83% and graft patency in 76%. Thirty-one long-term survivors (65%) were living at home. Only seven patients (15%) were confined to a wheelchair or were bedridden, and 28 (58%) were fully ambulatory. Peripheral arterial reconstruction in patients 80 years of age and older can be performed safely with excellent long-term survival and quality of life.

Long-term outcome after hospitalization for small-bowel obstruction.

OBJECTIVE: To determine the recurrence rate of small-bowel obstruction and differences in recurrence rates stratified by cause of obstruction and method of treatment. DESIGN: Retrospective chart review with average follow-up of 53 months (range, 0 to 129 months). SETTING: Combined community hospital/clinic tertiary referral center. PATIENTS: 309 consecutive patients with documented mechanical small-bowel obstruction hospitalized from 1981 to 1986. MAIN OUTCOME MEASURES: Recurrence rates by the actuarial life-table method and comparisons made by the Wilcoxon and log-rank tests. RESULTS: Recurrent obstruction developed in 34% of all patients by 4 years and in 42% by 10 years. Recurrence rates were 29% and 53% in the patients who did and did not undergo surgery (P = .002). The recurrence rate in patients with surgery was 56% for malignant neoplasms, 28% for adhesions, and 0% for hernia. Recurrence rates were 50% and 40% for patients with and without prior multiple obstructions (P = .7). CONCLUSIONS: The long-term risk of recurrent small-bowel obstruction is high. The risk is lessened by operation but not eliminated. The risk of recurrence increases with longer duration of follow-up, but most recurrences occur within 4 years. Multiple prior obstructions did not increase the risk of future obstruction.

Wound complications after infrainguinal bypass. Classification, predisposing factors, and management.

We studied 135 patients during a three-year period to determine the incidence and treatment of wound complications after infrainguinal bypass. The site of distal anastomosis was the popliteal artery in 113 patients, tibial artery in 20 patients, and sequential bypass in two patients. Autogenous vein was used for 79 grafts (59%), polytetrafluoroethylene for 53 grafts (39%), and a composite for three grafts (2%). Perioperative antibiotics were administered to 130 patients (96%). Wound complications were separated into four categories: class 1, erythema or seroma without tissue breakdown; class 2, ischemic necrosis along an incision without infection; class 3, wound breakdown with infection; and class 4, open wound with exposed graft. Ninety incisions (67%) healed without incident. There were 21 class 1 (15%), 17 class 2 (13%), four class 3 (3%), and three class 4 (2%) complications. Serious wound problems were more frequent after bypasses with polytetrafluoroethylene grafts compared with vein grafts. Three prosthetic graft infections resulted in two amputations. Eight other factors were not predictive of wound breakdown. Wound complications after infrainguinal bypass are frequent. Management should be selective, based on the degree of severity.

Treatment and outcome of right colon cancers adherent to adjacent organs or the abdominal wall.

Fifty-four (4%) of 1284 patients treated for adenocarcinoma of the colon and rectum during a 10-year period ending in 1989 underwent potentially curative resection of right colon lesions found during surgery to be adherent to adjacent organs, abdominal wall, or retroperitoneum. Final pathologic staging was as follows: modified Dukes' class B1 (n = 2), B2 (n = 24), C1 (n = 1), and C2 (n = 27). Thirteen (24%) patients had postoperative complications, including two (3.7%) with sepsis. One patient died after surgery (mortality, 1.9%). Survival rates at 1, 3, and 5 years were 74%, 52%, and 37%, respectively. Only one (11%) of nine patients with pancreatic or duodenal adherence treated with limited resection was free of disease during follow-up. Adjuvant radiation therapy and chemotherapy did not improve survival. Histologic depth of tumor penetration could not be predicted by intraoperative assessment, and therefore radical resection is recommended whenever possible.

Perioperative stroke risk in 173 consecutive patients with a past history of stroke.

During an 8-year period ending in 1988, 173 consecutive patients with a history of previous cerebrovascular accident underwent general anesthesia for surgery. Five patients (2.9%) had documented postoperative cerebrovascular accidents from 3 to 21 days (mean, 12.2 days) after surgery. The risk of postoperative cerebrovascular accident did not correlate with age, sex, history of multiple cerebrovascular accidents, poststroke transient ischemic attacks, American Society for Anesthesia physical status, aspirin use, coronary artery disease, peripheral vascular disease, intraoperative blood pressure, time since previous cerebrovascular accident, or cause of previous cerebrovascular accident. Postoperative stroke was more common in patients given preoperative heparin sodium. We conclude that the risk of perioperative stroke is low (2.9%) but not easily predicted and that the risk continues beyond the first week of convalescence. Unlike myocardial infarction, cerebral reinfarction risk does not seem to depend on time since previous infarct.

Gunshot wounds: 10-year experience of a rural, referral trauma center.

The 10-year experience of a Level II trauma center with 122 gunshot wounds referred from a large rural area was analyzed to illustrate differences from the experience of urban centers. Most frequent causes of injury were attempted suicide in 38 (31%) patients, hunting mishaps in 32 (26%), unintentional accidents in 29 (24%), and intentional assault in 18 (15%). Of weapons specified, rifles were documented in 48 (39%) instances, shotguns in 25 (21%), and handguns in 24 (20%). Body regions injured were the trunk in 47 (39%) patients, head in 35 (29%), lower extremity in 31 (25%), and upper extremity in 29 (24%). Twenty-five patients (20%) died as a result of their injuries. The cause of death was brain injury in 18 (72%), exsanguination from truncal wounds in 5 (20%), myocardial infarction in 1 (4%), and multiple organ failure in 1 (4%). We conclude that the distributions of cause and type of gunshot wounds are unique in a rural setting. These differences have profound consequences in designing effective prevention programs for our area and support the design of more efficient trauma systems for rural North America.

Routine invasive hemodynamic monitoring does not increase risk of aortic graft infection.

OBJECTIVE: To determine whether invasive hemodynamic monitoring resulted in an increased rate of aortic graft infection. DESIGN: Retrospective review with establishment of late follow-up. SETTING: Two referral teaching community medical centers. PATIENTS: Three hundred thirty consecutive patients who underwent elective aortoiliac (174), aortofemoral (91), or aortic tube (65) prosthetic graft placements during a 6-year period at the two institutions. All patients had percutaneous pulmonary artery catheter and radial artery cannula placement before surgery. Monitoring catheters were routinely removed within 72 hours. OUTCOME MEASURES: Early and late complications of catheter placement, perioperative morbidity and mortality, and late graft-related complications. RESULTS: Eighteen (5%) patients died in the perioperative period. Less than 2-year follow-up was possible in 67 patients. The remaining 245 patients were observed from 2 to 9 years (mean, 4.4 years). Graft infection was not identified in any patient. CONCLUSION: Routine invasive hemodynamic monitoring was not associated with an increase in the rate of prosthetic aortic graft infection.

Fine-needle aspiration biopsy of thyroid nodules. A cost-effective diagnostic plan.

Fine-needle aspiration biopsy is a safe and accurate method for diagnosing thyroid nodules. Personnel who have experience with the aspiration technique and with the cytologic classification of specimens are crucial to accurate results. One important advantage of using fine-needle biopsy to initially screen patients with nodules is that it can save substantially on healthcare resources.

Surgical procedures in centenarians.

Sixteen patients 100 years of age and older underwent surgical procedures at a single institution during the 11-year period ending December 1991. There were 11 (69%) females and five males. Patient ages ranged from 100 to 104 (mean, 101.1 years). Procedures included six ophthalmologic operations, three permanent pacemaker implantations, three compression hip screw fixations, two leg amputations, one hemiglossectomy, and one cystostomy. There was one (6%) perioperative death. Long-term follow-up was established for each patient. One-year survival rate in these 16 centenarians was 69%. We conclude that selected patients 100 years old and older can survive certain surgical procedures with acceptable perioperative and long-term results.

Trends in the management of splenic trauma.

During the 20-year period ending December 1987, 179 consecutive splenic trauma patients were treated at a single institution. Procedures included splenectomy in 121 (67%) patients, splenectomy with autotransplantation in 7 (4%), splenorrhaphy in 23 (13%), laparotomy alone in 7 (4%), and nonoperative management in 21 (12%). Before 1976, all patients were treated by splenectomy. Since 1980, 18 (22%) were treated nonoperatively, 26 (33%) by splenic salvage techniques, and 36 (45%) by splenectomy. We conclude that nonoperative therapy and splenic salvage techniques are being employed with increasing frequency. Selective application of splenorrhaphy for injuries with a realistic expectation of success has resulted in no late procedures for hemorrhage. In the presence of severe splenic or associated injuries, splenectomy remains the procedure of choice.

Hunting-related injuries.

One hundred four patients treated for injuries incurred while hunting were prospectively studied during two consecutive fall seasons. A questionnaire was completed at initial evaluation and hospital records were subsequently reviewed. One hundred (98%) patients were male. Patient ages ranged from 10 to 78 years (median = 32 years). Mechanism of injury included knife or arrow penetrations (25), firearm wounds (12), falls (17), overexertion (5), and misadventures with hazards (40). Soft tissue, maxillofacial and orthopedic injuries predominated. Four patients experienced cardiac events. Hypothermia was noted in three, and animal-related injuries occurred in five. Eighteen (17%) patients were hospitalized. Serious injury was evident in 34 (33%). There were no deaths. Outpatient follow-up was necessary in 90%. Mishaps most frequently occurred because of overexcitement, unfamiliarity with equipment, or carelessness. Alcohol and drug use were only rarely identified. Almost one half of patients were injured during the 9-day gun deer hunting season. A wide variety of injuries occur during hunting activities. While many are minor, serious morbidity with potential long-term disability may result. Costs in time and money may be substantial. Simple measures could prevent many hunting-related mishaps.

Delayed diagnosis of flail chest.

A retrospective review of 99 consecutive patients with flail chest after trauma was conducted to determine the incidence and significance of delayed presentation. Patients ranged in age from 7 to 88 yr (mean 50.3). Hospitalization ranged from 1 to 129 days (mean 23). Mean Injury Severity Score was 30. Sixty (61%) patients were managed with mechanical ventilation. Pulmonary complications developed in 48 (48%) patients and 14 (14%) patients died. The flail chest injury was documented on initial examination of 77 (78%) patients. Delayed presentation occurred in 22 (22%) patients from 1 to 10 days after injury. Reasons for delayed diagnosis included intubation and mechanical ventilation before complete physical examination, development of pulmonary complications with the attendant increased work of breathing, and physician error. The time of presentation was not associated with patient age, sex, severity of injury, need for mechanical ventilation, duration of ventilation, incidence of pulmonary morbidity, or mortality.

Fine needle aspiration biopsy of thyroid nodules.

Between April 1982 and December 1988, we performed 611 fine needle aspiration (FNA) biopsies in 502 patients with solitary thyroid nodules. The biopsy results were classified as follows: unsatisfactory, 19%; benign, 53%; suspicious, 23%; and malignant, 5%. Malignant lesions were found at surgery in 24 of 25 patients with malignant cytology and 31 of 68 patients with suspicious cytology. Autonomous nodules, obviating the need for surgery, were detected in 5 of 36 (14%) patients with cytology suspicious for follicular neoplasm. We conclude that FNA biopsy of the thyroid nodule is a safe and accurate diagnostic test. Thyroid scintigraphy remains useful in patients with cytologic results suspicious for follicular neoplasm. A cost-effective strategy for managing thyroid nodules is presented.

Death and disability from agricultural injuries in Wisconsin: a 12-year experience with 739 patients.

During a 12-year period, 739 patients were admitted to a referral trauma center as the result of injuries incurred while farming. There were 608 (82%) male patients and 131 female patients. Ages ranged from 1 to 89 years including 160 patients (22%) less than 16 years old and 78 patients (11%) more than 65 years old. The injury mechanism was a farm animal in 225 (30%), farm machinery in 168 (23%), a tractor in 120 (16%), a fall in 77 (10%), a power take-off in 47 (7%), a cornpicker in 42 (6%), and miscellaneous in 60 (8%). There were 16 (2%) deaths attributable to the agricultural accident. Tractors were involved in eight of these deaths, falls in four, power take-offs in three, and farm machinery in one. Furthermore, 159 (22%) patients were left with significant permanent disability including orthopedic problems in 131 patients, neurologic deficits in 22, and pulmonary disability in 6. Agricultural trauma is frequent and diverse with unique injury mechanisms. Life-threatening injuries are often seen and permanent disability is common. Effective injury prevention must focus on farmer education, additional mandatory safety features on agricultural equipment, and appropriate design of rural trauma systems.

Prospective analysis of rural interhospital transfer of injured patients to a referral trauma center.

A 1-year prospective review of 78 multiply injured patients initially treated at local community hospitals and subsequently transported more than 25 miles to a referral trauma center was completed. Injury mechanisms were blunt in 74 (95%) patients and penetrating in four. Patient ages ranged from 6 to 88 years (mean, 33 years). Trauma Scores ranged from 6 to 16 (mean, 13.9) and Injury Severity Scores ranged from 4 to 54 (mean, 21.5). The quality of care during initial stabilization and transport was evaluated by ATLS guidelines for airway management, treatment of shock, spine and fracture immobilization, neurologic evaluation, secondary assessment, and chart documentation. Most frequent departures from these standards involved failure to place a nasogastric tube before transport (72%), failure to document neurologic status (47%), inadequate cervical spine immobilization (32%), inadequate intravenous access (29%), inadequate oxygen delivery (28%), and incomplete or absent records (22%). Life-threatening deficiencies were identified in four (5%) patients and serious deficiencies in 62 (80%). This study demonstrates the need for further education of rural physicians about priorities in trauma management. Increased emphasis on stabilization and transport should be added to ATLS training courses. Established transport protocols between institutions would enhance the quality of care and engender improved interhospital communication. The implementation of trauma systems designed specifically for rural areas must be supported.