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Recent studies have provided much needed data on the probability of seizure remission among adults with chronic intractable epilepsy treated medically. Here we provide an extended follow-up to our earlier study in order to provide a more comprehensive picture of long-term prognosis in this patient population during medical treatment. The prevalence cohort was followed for two outcomes-complete seizure remission for ≥ 12 months and subsequent seizure relapse among those attaining a seizure remission. The study outcomes were estimated using Kaplan-Meier analysis. We found that the probability of attaining a ≥ 12 months of complete seizure freedom to be approximately 3-4% per year through 8 years of follow-up. By year 5 since the start of seizure remission, the cumulative probability of seizure relapse was 81%, although only half of the patients with seizure relapse went on to experience their previous seizure frequency., (Copyright © 2010 Elsevier B.V. All rights reserved.)

Posterior Reversible Encephalopathy Syndrome (PRES) is a syndrome comprising headache, altered mentation, and seizures, associated with neuroimaging findings characteristic of subcortical edema in the posterior regions. It is usually seen in patients treated with immunosuppressants, in renal failure, or with eclampsia. Recurrent episodes of PRES in the same patient are rarely observed. Although seizures are often seen in PRES, EEG findings are not well described and include generalized and focal slowing with epileptiform discharges; there are limited reports of Periodic Lateralized Epileptiform Discharges (PLEDs) occurring during PRES, and there are no reports of PRES associated with PLEDs with subsequent development of epilepsy. We report a patient we followed for one year with recurrent episodes of PRES associated with posteriorly dominant independent bilateral PLEDs who subsequently developed epilepsy. Patients with PRES and PLEDs should be treated aggressively with anti-hypertensive and anti-epileptic agents in order to avoid potential complications. Although PRES is typically thought of as a reversible syndrome, this case illustrates that PRES may have serious long term sequelae after the reversible syndrome has resolved. This case highlights the importance of aggressive management of PRES as well as the prevention of subsequent episodes of PRES as patients may develop permanent brain dysfunction., (Published by Elsevier Ltd.)

On 12 January 2010, a 7.0-magnitude earthquake devastated the island nation of Haiti, leading to the world's largest humanitarian effort in over 6 decades. The catastrophe caused massive destruction of homes and buildings and overwhelmed the Haitian health care system. The United States responded immediately with a massive relief effort, sending U.S. military forces and civilian volunteers to Haiti's aid and providing a tertiary care medical center aboard the USNS COMFORT hospital ship. The COMFORT offered sophisticated medical care to a geographically isolated population and helped to transfer resource-intensive patients from other treatment facilities. Working collaboratively with the surgical staff, ancillary services, and nursing staff, internists aboard the COMFORT were integral to supporting the mission of the hospital ship and provided high-level care to the casualties. This article provides the perspective of the U.S. Navy internists who participated in the initial response to the Haitian earthquake disaster onboard the COMFORT.

On January 12, 2010, a 7.0 Richter earthquake devastated Haiti and its public health infrastructure leading to a worldwide humanitarian effort. The United States sent forces to Haiti's assistance including the USNS Comfort, a tertiary care medical center on board a ship. Besides setting a transparent triage and medical regulating system, the leadership on the Comfort instituted a multidisciplinary Healthcare Ethics Committee to assist in delivering the highest level efficient care to the largest number of victims. Allocation of resources was based on time-honored ethics principles, the concept of mass casualty triage in the setting of resource constraints, and constructs developed by the host nation's Ministry of Health. In offering aid in austere circumstances, healthcare practitioners must not only adhere to the basic healthcare ethics principles but also practice respect for communities, cultures, and traditions, as well as demonstrate respect for the sovereignty of the host nation. The principles outlined herein should serve as guidance for future disaster relief missions. This work is in accordance with BUMEDINST 6010.25, Establishment of Healthcare Ethics Committees.

Objective: To describe a patient with an acute fulminant delirium and eventual spinal fluid block secondary to sarcoid meningitis., Design: Case report., Setting: Hospital and Neurology Clinic., Patient: A previously healthy, 24-year-old man., Interventions: Antimicrobials, corticosteroids, lumbar puncture, myelography, and lymph node biopsy., Main Outcome Measures: Cerebrospinal fluid, clinical status., Results: The patient improved after treatment with corticosteroids., Conclusion: Sarcoid meningitis may present with delirium and spinal block.

Improved recognition and availability of noninvasive testing of autonomic disorders has prompted a better understanding of disease mechanisms of some disease forms, especially potentially treatable immune-mediated autonomic neuropathies. Development is acute, subacute, or less commonly chronic. Autonomic involvement is common and an important cause of morbidity and mortality in Guillain-Barré syndrome. Acute autonomic neuropathy can affect parasympathetic, sympathetic, and enteric nerves or neurons and is associated with antibodies to ganglionic nicotinic acetylcholine receptors. These antibodies appear to be causative based on a rabbit immunization model and serum transfer studies from patients and animals. Other important immune autonomic disorders discussed include Lambert-Eaton myasthenic syndrome, some forms of orthostatic intolerance, chronic autonomic neuropathy, and Sjögren syndrome. Paraneoplastic autonomic disorders are clinically indistinguishable and associated with various overlapping antibody associations, including anti-Hu (ANNA-1), ganglionic acetylcholine receptors, CRMP-5, and PCA-2.

Objective: To describe the coping strategies used by the spouses of cardiac patients who are pursuing heart transplant and to determine whether coping strategies predict depression in these spouses., Method: This is a cross-sectional design with 28 spouses (86% female) of cardiac patients who were being evaluated for heart transplant at a large medical center. Coping styles were measured using the COPE Inventory (Carver CS, Scheier MF, Weintraub JK, J Pers Soc Psychol 1989: 56: 267). Depression was assessed with the Center for Epidemiological Studies Depression Scale (Radloff LS, Appl Psychol Meas 1977: 1: 385)., Results: Spouses reported using adaptive coping strategies more than the less adaptive strategies. Increased levels of depression were positively correlated with 'behavioral disengagement' and negatively correlated with 'planning' coping strategies., Conclusions: Most studies find that maladaptive coping styles are associated with psychological distress. Consistent with these findings, we found that behavioral disengagement was associated with increased levels of depression. However, in the present study the more adaptive coping strategy of planning was associated with lower levels of psychological distress. These findings suggest that in the spouses of heart transplant candidates, both maladaptive and adaptive coping strategies may serve as markers of the presence or absence of emotional distress and thus may help in identifying spouses who may benefit from psychological, social work, or nursing interventions.

There are few data available regarding the safety or efficacy of thrombolysis of acute ischemic stroke secondary to myxoma. We present a patient treated with intravenous recombinant tissue plasminogen activator who developed hemorrhage remote from the location of ischemic stroke. Intra-arterial local thrombolysis may be a preferable alternative because of greater risk of hemorrhage from occult tumor emboli or microaneurysms in these patients.

The Fontan circulation or one of its many modifications is a common surgical procedure implemented in children with congenital heart defects to improve pulmonary circulation. Two modifications are the atriopulmonary connection (AP) and the modified total cavopulmonary connection (TCPC). Successful AP and TCPC operations performed in lambs show respiration has a major influence on systemic and pulmonary hemodynamics. Instantaneous pressure and flow waveforms are obtained before and after the procedure and energies are calculated. Hemodynamic changes in relevant vessels are also acquired. Time delays due to respiration will be calculated and transfer functions computed. The purpose of this study is to examine these waveforms and energy gains/losses under varying respiration parameters in order to aid in determining an optimal surgical protocol as well as assist in the development an accurate computer simulation model.

Objectives: We reviewed our experience with lung transplant for cystic fibrosis (CF) over a 10-year period to identify factors influencing long-term survival., Methods: One hundred and twenty-three patients with CF have undergone 131 lung transplant procedures at our institution; 114 have had bilateral sequential lung transplants (DLTX) and nine have had bilateral lower lobe transplants from living donors. Three patients had retransplant for acute graft failure, and five had late retransplant for bronchiolitis obliterans syndrome (BOS). Kaplan-Meier survival was calculated for the entire cohort and for subsets at higher risk of death to determine factors predicting a better outcome., Results: Actuarial survival for the entire group of DLTX CF patients was 81% at 1 year, 59% at 5 years, and 38% at 10 years. Lobar transplant was associated with a poorer survival (37.5% at 1 and 5 years). Among DLTX patients, colonization with Burkholderia cepacia was present in 22 patients and was associated with poorer outcome (1- and 5-year survival 60 and 36% in B. cepacia patients vs. 86 and 64% in non-cepacia patients). DLTX patients younger than age 20 (n=22) had a similar survival to patients age 20 or older (n=90). Being on a ventilator at the time of transplant was not associated with poorer survival (n=8). BOS affects increasing numbers of survivors with time. Five CF patients have been retransplanted due to BOS with one operative death and 1-year survival of 60%., Conclusions: DLTX has acceptable long term survival in CF adults and children with end stage disease. CF patients colonized with B. cepacia have a worse outcome but transplantation is still warranted.

The objective of the present study was to evaluate the effect of degree of saturation of fat incorporated into broiler diets on performance and body fatty acid (FA) profile. The various degrees of saturation were achieved by using regular soybean oil (SO) and hydrogenated soybean oil (HSO), mixed at different proportions. The work was carried out on commercial broilers (Experiment 1) and on lines of chickens divergently selected for high (HF) or low (LF) abdominal fat (Experiment 2). Daily BW gain and gain:feed ratio increased and the amount of feed intake decreased as the dietary fat saturation decreased. Digestibility of total fat and of each of the FA was lowest in the HSO group and reached maximal values when 23% or more of the added oil was SO. The AMEn values of the diets were almost parallel to fat digestibility. The performance of the HF and LF chickens was affected by the degree of saturation similarly to that observed for the commercial stock. The degree of dietary fat saturation had very little effect on saturated FA (C16:0 and C18:0) in body lipids, reduced the level of monoenoic FA (C16:1 and C18:1), and raised that of polyunsaturated FA (PUFA) (C18:2, C18:3, and C20:4). Monoenoic FA were higher, whereas PUFA were lower in the HF than in the LF line. The improved AMEn in diets containing unsaturated fat is probably due to higher fat digestibility, direct deposition of PUFA in body lipids, and lower lipogenesis, associated with lower heat production.

Background: There are few congenital anomalies of the heart that have benefited more from thorough anatomic analysis than the complex anomaly known as atrioventricular septal defect in the setting of common atrioventricular junction. Recent advances in understanding the anatomy of this lesion have led to alternative methods of repairing these defects., Methods: The medical records of 21 consecutive patients undergoing repair of complete atrioventricular septal defect have been reviewed. Nine of these patients had a standard one- or two-patch repair, and 12 had direct closure of the ventricular element of the defect., Results: Direct closure resulted in significantly shorter pump and cross-clamp times. Follow-up for an average of 34 months suggests that when direct closure can be performed, the results are comparable with those of the more standard technique., Conclusions: Our initial success with this approach is encouraging; however, longer follow-up is required to establish whether it will be broadly applicable.

Background: Between June 1997 and November 1993, 100 consecutive thymectomies for myasthenia gravis were performed at University of North Carolina Hospitals in Chapel Hill., Methods: A consistent, planned protocol involving preoperative, intraoperative, and postoperative care was followed. All thymectomies were performed through a median sternotomy with removal of all visible thymus and perithymic fat in the anterior mediastinum., Results: There was no perioperative mortality or longterm morbidity. Mean postoperative hospital stay was 6.3 days (range, 3 to 18 days). Ninety-six percent of the patients were extubated the day of the operation, and all patients were extubated within 24 hours. Mean postoperative intensive care unit stay was 1.2 days (range, 1 to 4 days). After a mean follow-up of 65 months (range, 1 to 199 months), 78% of all patients are improved by at least one modified Osserman classification when their current status is compared with their worst preoperative disease severity. In fact, 69% of patients with mild disease preoperatively (class I, II, or III maximal severity) are in pharmacologic remission (asymptomatic without regular medication), whereas 29% of patients with severe disease (class IV or V) are in remission (p = 0.0001)., Conclusions: Our programmatic approach to thymectomy through a sternotomy has shown minimal morbidity and mortality. It is beneficial to myasthenics at both ends of the age and severity spectrum.

Background: This study examined results of lung transplantation after previous thoracic surgical procedures., Methods: Twenty percent of the 69 isolated lung transplantations performed at the University of North Carolina between January 1990 and June 1993 were in patients who had undergone a previous thoracic surgical procedure, and an additional 10% had undergone a previous chest tube placement., Results: No statistically significant increase in morbidity or mortality was observed between those having undergone a previous procedure or chest tube placement and all other patients. Specifically, the length of intubation, length of hospital stay, hospital mortality, or the number of patients who experienced major early complications was not significantly different between these groups. A statistically significant increase in the number of blood products used was observed in the patients with previous thoracic surgical procedures but not with patient having had previous chest tube placements. However, when the data were reanalyzed with respect to the use of cardiopulmonary bypass, those patients requiring bypass had a markedly poorer outcome that reached statistical significance in all of the parameters studied: hospital death, incidence of major complications, length of intubation, hospital stay, incidence of bleeding, and number of blood products used., Conclusions: We conclude that although increased bleeding may be encountered, lung transplantation can be performed successfully in patients who have had previous thoracic surgical procedures without increased major morbidity or mortality; however, the use of cardiopulmonary bypass has been associated with significantly increased morbidity and mortality in our patient population.

Current United Network for Organ Sharing policy requires listing lung transplant recipients with an acceptable donor weight range, but lung size is a function of height, age, sex, and race. Frequently, lung transplant recipients are underweight, which results in a large discrepancy between donor and recipient weights. We reviewed our experience with size discrepancy between donors (D) and recipients (R) of 49 double-lung transplant (DLTX) procedures since July 1990. Pneumoreduction procedures were performed in 11 recipients of lungs judged to be too large at the time of DLTX (right middle lobectomy, 2; lingulectomy, 2; both, 6; right middle lobectomy and bilateral apical resections, 1). Predicted forced vital capacity (FVC) and total lung capacity (TLC) of donors and recipients were calculated. Donors were larger than recipients in general (D:R height = 1.02; D:R weight = 1.46), and, as a result, recipient-predicted lung volumes were smaller than donor-predicted lung volumes (D:R FVC = 1.1; D:R TLC = 1.1). Recipients undergoing pneumoreduction procedures had a significantly greater size discrepancy between donors and recipients; thus, both the ratio of D:R and the difference between D and R predicted FVC and TLC were significantly greater among recipients who underwent pneumoreduction, compared with nonreduced recipients. For recipients in the pneumoreduction group, predicted FVC and TLC were recalculated, with a proportionate amount subtracted based on the number of pulmonary segments removed. When the "corrected" FVC and TLC of the donors were compared with recipient-predicted FVC and TLC, there was no longer any significant difference between reduced and non-reduced groups, which implies that visual estimate of size mismatch at surgery is an accurate measure of size discrepancy. Post-DLTX spirometry showed identical improvement in FVC in patients who had pneumoreduction and those who did not, and survival at 6 months was identical in both groups. We conclude that pneumoreduction had no adverse effect on survival or post-DLTX spirometry, allowing safe use of larger donors in small recipients. Also, because lung size is more a function of height than weight, this study challenges the United Network for Organ Sharing practice of listing recipients with an acceptable donor weight range.

Patients with cystic fibrosis pose particular challenges for lung transplant surgeons. Earlier reports from North American centers suggested that patients with cystic fibrosis were at greater risk for heart-lung or isolated lung transplantation than other patients with end-stage pulmonary disease. During a 3 1/2 year period, 44 patients with end-stage lung disease resulting from cystic fibrosis underwent double lung transplantation at this institution. During the same interval, 18 patients with cystic fibrosis died while waiting for lung transplantation. The ages of the recipients ranged from 8 to 45 years, and mean forced expiratory volume in 1 second was 21% predicted. Seven patients had Pseudomonas cepacia bacteria before transplantation. Bilateral sequential implantation with omentopexy was used in all patients. There were no operative deaths, although two patients required urgent retransplantation because of graft failure. Cardiopulmonary bypass was necessary in six procedures in five patients and was associated with an increased blood transfusion requirement, longer postoperative ventilation, and longer hospital stay. Actuarial survival was 85% at 1 year and 67% at 2 years. Infection was the most common cause of death within 6 months of transplantation (Pseudomonas cepacia pneumonia was the cause of death in two patients), and bronchiolitis obliterans was the most common cause of death after 6 months. Actuarial freedom from development of clinically significant bronchiolitis obliterans was 59% at 2 years. Results of pulmonary function tests improved substantially in survivors, with forced expiratory volume in 1 second averaging 78% predicted 2 years after transplantation. Double lung transplantation can be accomplished with acceptable morbidity and mortality in patients with cystic fibrosis.

As lung transplantation has become more successful, the selection criteria have broadened; however, some relative contraindications to lung transplantation are controversial. Some programs consider mechanical ventilation to be a major contraindication to lung transplantation because airway colonization with bacteria may lead to nosocomial infection and respiratory muscle deconditioning may necessitate prolonged postoperative ventilatory support. We report our experience of seven double lung transplant procedures on six patients requiring mechanical ventilation. Five patients with cystic fibrosis required preoperative mechanical ventilation for 7 to 19 days (mean, 10.7 days). One patient with acute lung injury required 115 days of preoperative mechanical ventilatory support. Only the latter patient required prolonged (27 days) postoperative mechanical ventilation because of respiratory muscle weakness; the others were extubated in 1 to 19 days (mean, 7.8 days). No early complications related to bacterial infection were seen. Two patients required temporary hemodialysis for transient kidney failure. Three patients had postoperative neurologic residua; one patient had a transient hemiparesis, and seizures developed in two patients. One patient died 3 months after transplantation from severe central nervous system complications with no evidence of pulmonary problems; and two patients died 17 months after transplantation, one of them receiving a second double lung transplant for obliterative bronchiolitis. Except for the patient who required prolonged preoperative ventilatory support, mechanical ventilation did not appear to play a role in the outcome of these patients. The posttransplantation hospital stay and hospital charges for patients requiring pretransplantation ventilatory support were not significantly different from those for other lung transplant recipients.(ABSTRACT TRUNCATED AT 250 WORDS)

An anomalous course of the left brachiocephalic vein beneath the aortic arch was identified in 7 patients undergoing surgical repair of congenital cardiac malformations. Six of these patients had the morphologic features of tetralogy of Fallot including severe obstruction to the right ventricular outflow tract and a right aortic arch. A review of the literature reveals this to be an uncommon anomaly. When it is present, however, it is frequently associated with a ventricular septal defect, obstruction of the right ventricular outflow, and aortic arch anomalies. This anomaly can be documented by echocardiography, at cardiac catheterization, or intraoperatively. The presence of a subaortic left brachiocephalic vein may have implications for the conduct of surgical procedures, especially in the setting of tetralogy of Fallot.

Analysis of salivary digoxin using a rapid dry chemistry, enzyme-labeled immunometric assay (ELIA) was compared with fluorescence polarization immunoassay (FPIA). Saliva and serum samples were obtained from 40 hospitalized patients who were taking digoxin chronically and from 8 patients just prior to treatment with digoxin. Unstimulated saliva samples were collected from 20 patients; however, saliva volumes from 10 pediatric patients were inadequate to permit analysis by FPIA, and 1 other had unmeasurable concentrations by both methods. Stimulated saliva was collected by having patients chew a small piece of Parafilm for 1-2 min. Salivary digoxin was analyzed using the same procedure recommended for serum digoxin by each manufacturer. There were no significant differences found between ELIA and FPIA determinations of unstimulated or stimulated salivary digoxin, serum digoxin, or saliva/serum concentration ratios. The saliva/serum ratio of the unstimulated group was approximately twice that of the stimulated group (p less than 0.01) by both methods, suggesting that salivary digoxin concentration decreases with increased saliva production rate. Excellent correlations were found between ELIA and FPIA salivary digoxin concentrations and between stimulated saliva and serum concentrations by both assays. Weaker correlations were observed between unstimulated saliva and serum concentrations. There was no evidence of assay interference with either method in eight nondigitalized patients, each taking an average of 6.5 medications. The ELIA appears to provide equivalent results compared with the FPIA for the determination of salivary digoxin concentration. Further investigations are needed before salivary digoxin concentration monitoring can be recommended as an acceptable alternative to serum monitoring.

Since January 1990, we have performed 29 isolated lung transplantations in 28 patients with end-stage lung disease (12 single, 16 bilateral). Recipient diagnoses were: cystic fibrosis (11), chronic obstructive pulmonary disease (6), pulmonary fibrosis (6), eosinophilic granulomatosis (1), postinfectious lung disease (1), adult respiratory distress syndrome (1), and primary pulmonary hypertension (2). There have been four deaths, two in patients with pulmonary fibrosis and two in patients with primary pulmonary hypertension. Four patients have undergone transplantation while on ventilatory support for respiratory failure (2 with cystic fibrosis, 1 having redo lung transplantation with cystic fibrosis, and 1 with adult respiratory distress syndrome); all of these have survived. Six patients required cardiopulmonary bypass, which was associated with increased transfusion requirement. All patients 2 months after discharge have returned to an active life-style, except for 2 patients who currently await retransplantation. Preoperative pulmonary rehabilitation has resulted in significant improvement in exercise performance in all patients. Immunosuppression consists of cyclosporine, azathioprine, and antilymphoblast globulin (University of Minnesota), withholding systemic steroids in the early postoperative period. We have employed bronchial omentopexy in all but four transplants; there has been one partial bronchial dehiscence, two instances of bronchomalacia requiring internal stenting, and one airway stenosis. Cytomegalovirus disease has been seen frequently (15 cases), but has responded well to treatment with ganciclovir. Other complication shave included one drug-related prolonged postoperative ventilation, thrombosis of a left lung after bilateral lung transplantation requiring retransplantation, five episodes of unilateral phrenic nerve palsy after bilateral lung transplantation (4 resolved), and the requirement of massive transfusion (greater than 10 units) in 5 patients.(ABSTRACT TRUNCATED AT 250 WORDS)

The relationship of nutritional status, self-perceived chewing ability, dental status, and social isolation was examined. Seventy-three ambulatory, elderly (means = 86 years) veterans were studied. Parameters of nutritional status included intakes of protein, carbohydrate, fat, and total calories, and hemoglobin, serum albumin, total lymphocyte count, and height/weight ratio were determined. Dental status was measured, and self-perceived chewing problems and social isolation were assessed by interview. Results showed a significant correlation between perceived chewing problems and diminished protein and total caloric intake and increased carbohydrate intake. No association was found between measured dental status and nutritional status. Social isolation was weakly correlated with greater protein and calorie intake. These results support the contention that the presence of self-perceived chewing problems are more reliable than the quality of the dentition itself as an indicator of altered nutritional status.

Although a variety of long-term, probably immunologically induced pulmonary changes have been described in recipients of both combined heart-lung and bone marrow transplantation, pulmonary infections continue to remain causes of significant morbidity and mortality as well. Herein we describe three patients (two heart-lung and one bone marrow transplant recipient) who had bronchocentric granulomatous mycosis, a tissue manifestation of fungal infection not previously described in the setting of a transplant host. Although one patient was being treated successfully with antifungal agents for his mucormycosis, two other patients eventually died of invasive aspergillosis. This emphasizes that although this process is histologically somewhat similar to bronchocentric granulomatosis, a high index of suspicion for infection needs to be maintained when this pathologic process is identified in a transplant host.

In an effort to define the optimal preventive antibiotics for gunshot wounds to the abdomen, 100 consecutive patients were randomized while in the emergency department to receive either combination ampicillin, amikacin, and clindamycin, combination doxycycline and penicillin, or carbenicillin (Groups I, II, and III, respectively). Antibiotics were continued for 5 days in the presence of distal ileal or colonic injury. The study groups were comparable in age, sex, incidence of shock, degree of peritoneal contamination, and abdominal trauma index. The incidence of infection was not statistically different among the groups. Enterobacteriacea were the predominant offenders, although anaerobes were identified in all groups. The critical risk factor was distal ileal or colon injury. This study indicates that a regimen employing a single agent whose spectrum includes both aerobes and anaerobes is as effective as more expensive and potentially toxic multiagent regimens.