Background: The LMNA gene, one of 6 autosomal disease genes implicated in familial dilated cardiomyopathy, encodes lamins A and C, alternatively spliced nuclear envelope proteins. Mutations in lamin ...A/C cause 4 diseases: Emery-Dreifuss muscular dystrophy, limb girdle muscular dystrophy type 1B, Dunnigan-type familial partial lipodystrophy, and dilated cardiomyopathy. Methods and Results: Two 4-generation white families with autosomal dominant familial dilated cardiomyopathy and conduction system disease were found to have novel mutations in the rod segment of lamin A/C. In family A a missense mutation (nucleotide G607A, amino acid E203K) was identified in 14 adult subjects; disease was manifest as progressive conduction disease in the fourth and fifth decades. Death was caused by heart failure. In family B a nonsense mutation (nucleotide C673T, amino acid R225X) was identified in 10 adult subjects; disease was also manifest as progressive conduction disease but with earlier onset (third and fourth decades), ventricular dysrhythmias, left ventricular enlargement, and systolic dysfunction. Death was caused by heart failure and sudden cardiac death. Skeletal muscle disease was not observed in either family. Conclusions: Novel rod segment mutations in lamin A/C cause variable conduction system disease and dilated cardiomyopathy without skeletal myopathy.
With the evolution of surgical and anesthetic techniques, liver transplantation has become "routine," allowing for modifications of practice to decrease perioperative complications and costs. There ...is debate over the necessity for intensive care unit admission for patients with satisfactory preoperative status and a smooth intraoperative course. Postoperative care is made easier when the liver graft performs optimally. Assessment of graft function, vigilance for complications after the major surgical insult, and optimization of multiple systems affected by liver disease are essential aspects of postoperative care. The intensivist plays a vital role in an integrated multidisciplinary transplant team.
To explore the effect of various adverse hospital events on short- and long-term outcomes in a cohort of acutely ill hospitalized patients.
In a secondary analysis of a retrospective cohort of ...acutely ill hospitalized patients with sepsis, shock, or pneumonia or undergoing high-risk surgery who were at risk for or had developed acute respiratory distress syndrome between 2001 and 2010, the effects of potentially preventable hospital exposures and adverse events (AEs) on in-hospital and intensive care unit (ICU) mortality, length of stay, and long-term survival were analyzed. Adverse effects chosen for inclusion were inadequate empiric antimicrobial coverage, hospital-acquired aspiration, medical or surgical misadventure, inappropriate blood product transfusion, and injurious tidal volume while on mechanical ventilation.
In 828 patients analyzed, the distribution of 0, 1, 2, and 3 or more cumulative AEs was 521 (63%), 126 (15%), 135 (16%), and 46 (6%) patients, respectively. The adjusted odds ratios (95% CI) for in-hospital mortality in patients who had 1, 2, and 3 or more AEs were 0.9 (0.5-1.7), 0.9 (0.5-1.6), and 1.4 (0.6-3.3), respectively. One AE increased the length of stay, difference between means (95% CI), in the hospital by 8.7 (3.8-13.7) days and in the ICU by 2.4 (0.6-4.2) days.
Potentially preventable hospital exposure to AEs is associated with prolonged ICU and hospital lengths of stay. Implementation of effective patient safety interventions is of utmost priority in acute care hospitals.
The critical care community has been using severity and organ failure assessment tools for over 2 decades. The major adult severity assessment models are Acute Physiology and Chronic Health ...Evaluation, Simplified Acute Physiology Score, and Mortality Probability Model. All three recent versions of these models perform well in predicting hospital mortality. Sequential Organ Failure Assessment score is the most used tool for assessment of multiple organ failure. These tools have been used extensively in clinical research involving critically ill patients and for benchmarking and the measurement of performance improvement. Their roles as clinical decision support tools at the bedside await future studies because of their unknown or poor performance at the individual patient level.
We have compared the recovery characteristics of four different techniques for maintenance of anaesthesia in 99 day-case patients admitted for oral surgery. All patients received propofol for ...induction of anaesthesia followed by halothane, enflurane, isoflurane or propofol infusion for maintenance of anaesthesia. Each patient was subjected to a battery of psychometric tests which included Spielberger state, trait, mood stress and mood arousal questionnaires, Maddox-Wing test and five-choice serial reaction time. All tests were performed before operation and at 0.5, 1, 2, 4, 24 and 48 h after operation. Performance in the reaction time test decreased significantly in the immediate postoperative period, returning almost to preoperative values by 4 h. However, only those patients who received enflurane or propofol had returned to their performance level before surgery by 4 h, although all four groups had achieved this target by 24 h. There was a further improvement in performance at 48 h. Anxiety and stress were high before surgery and decreased rapidly in the postoperative period. The Maddox-Wing test demonstrated a significant impairment in performance in the first 1 h after surgery, which returned to normal by discharge at 4 h. There were no significant differences between the four groups in these latter tests. (Br. J. Anaesth. 1994; 72: 559–566)
Background There are few comparisons among the most recent versions of the major adult ICU prognostic systems (APACHE Acute Physiology and Chronic Health Evaluation IV, Simplified Acute Physiology ...Score SAPS 3, Mortality Probability Model MPM0 III). Only MPM0 III includes resuscitation status as a predictor. Methods We assessed the discrimination, calibration, and overall performance of the models in 2,596 patients in three ICUs at our tertiary referral center in 2006. For APACHE and SAPS, the analyses were repeated with and without inclusion of resuscitation status as a predictor variable. Results Of the 2,596 patients studied, 283 (10.9%) died before hospital discharge. The areas under the curve (95% CI) of the models for prediction of hospital mortality were 0.868 (0.854-0.880), 0.861 (0.847-0.874), 0.801 (0.785-0.816), and 0.721 (0.704-0.738) for APACHE III, APACHE IV, SAPS 3, and MPM0 III, respectively. The Hosmer-Lemeshow statistics for the models were 33.7, 31.0, 36.6, and 21.8 for APACHE III, APACHE IV, SAPS 3, and MPM0 III, respectively. Each of the Hosmer-Lemeshow statistics generated P values < .05, indicating poor calibration. Brier scores for the models were 0.0771, 0.0749, 0.0890, and 0.0932, respectively. There were no significant differences between the discriminative ability or the calibration of APACHE or SAPS with and without “do not resuscitate” status. Conclusions APACHE III and IV had similar discriminatory capability and both were better than SAPS 3, which was better than MPM0 III. The calibrations of the models studied were poor. Overall, models with more predictor variables performed better than those with fewer. The addition of resuscitation status did not improve APACHE III or IV or SAPS 3 prediction.
We investigated body size and survival by race/ethnicity in 11,351 breast cancer patients diagnosed from 1993 to 2007 with follow-up through 2009 by using data from questionnaires and the California ...Cancer Registry. We calculated hazard ratios and 95% confidence intervals from multivariable Cox proportional hazard model–estimated associations of body size (body mass index (BMI) (weight (kg)/height (m)2) and waist-hip ratio (WHR)) with breast cancer–specific and all-cause mortality. Among 2,744 ascertained deaths, 1,445 were related to breast cancer. Being underweight (BMI <18.5) was associated with increased risk of breast cancer mortality compared with being normal weight in non-Latina whites (hazard ratio (HR) = 1.91, 95% confidence interval (CI): 1.14, 3.20), whereas morbid obesity (BMI ≥40) was suggestive of increased risk (HR = 1.43, 95% CI: 0.84, 2.43). In Latinas, only the morbidly obese were at high risk of death (HR = 2.26, 95% CI: 1.23, 4.15). No BMI–mortality associations were apparent in African Americans and Asian Americans. High WHR (quartile 4 vs. quartile 1) was associated with breast cancer mortality in Asian Americans (HR = 2.21, 95% CI: 1.21, 4.03; P for trend = 0.01), whereas no associations were found in African Americans, Latinas, or non-Latina whites. For all-cause mortality, even stronger BMI and WHR associations were observed. The impact of obesity and body fat distribution on breast cancer patients' risk of death may vary across racial/ethnic groups.
Objective To establish the frequency of intensive care unit (ICU) admission after esophagectomy and to determine the associated outcomes. Design Retrospective cohort study. Setting Tertiary referral ...center. Participants Four hundred thirty-two patients who underwent esophagectomy between January 2000 and June 2004. Interventions None Measurements and Main Results Data relating to demographics, patient co-morbidities, perioperative management, complications, and Acute Physiology and Chronic Health Evaluation (APACHE) III variables were abstracted. Statistical analyses were performed to compare survivors with non-survivors and ICU patients with non-ICU patients. Of 432 patients included in the study, 123 (28.5%) were admitted to the ICU. Arrhythmias, new infiltrates on chest radiograph, and documented aspiration were common reasons for ICU admission. Patients admitted to ICU were of high acuity (mean APACHE III score 54.5, mean prediction of ICU death 6.4%). Of 352 patients originally not sent to the ICU, 43 (12.2%) were subsequently admitted to the ICU, often for aspiration. Overall in-hospital mortality was 3.7% (16 of 432 patients). Fifteen of the 123 ICU patients (12.2%) did not survive to hospital discharge. Conclusions A significant minority of patients will require ICU admission after esophagectomy, often for aspiration pneumonitis and arrhythmias. Despite high severity of illness scores, the perioperative mortality rate for patients after esophagectomy at a high-volume center is low.
A correlation of the catalytic properties of three supported Pt catalysts with their physical properties, chemical composition, and adsorption properties is presented. The catalytic properties of ...0.75 wt% Pt/silica, 0.89 wt% Pt/alumina, and 0.48 wt% Pt/molybdena are reported for the hydrogenolysis of cyclopropane and for the hydrogenations of ethene, 1,3-butadiene, and 2-butyne. The corresponding catalytic properties of the standard reference catalyst EUROPT-1, a 6.3 wt% Pt/silica, are reported for comparison. Pt/silica and EUROPT-1 each contained fully reduced Pt and were chloride free; their activities for structure-insensitive 1,3-butadiene and 2-butyne hydrogenations were in proportion to their respective Pt dispersions and product selectivities were identical. This intercatalyst comparison could not be extended to ethene hydrogenation because reaction over Pt/silica showed a kinetic discontinuity which is described in detail. Pt/alumina contained Ptδ+which had the effect of reducing activity in each hydrogenation reaction and of altering product selectivity in 1,3-butadiene hydrogenation. A condition was found under which this support effect was reversed. Cyclopropane hydrogenolysis over all four catalysts obeyed the Bond–Newham rate equation, and the rate coefficient varied with Pt particle size indicating the reaction to be structure sensitive. Pt/molybdena, the only catalyst having a wide range of Pt particle size, behaved in contrasting and predictable ways depending upon the structure-sensitive or-insensitive nature of the reaction under study.
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