Varicella Zoster Virus CNS disease in hematopoietic cell transplantation: A single center experience

Background: Varciella Zoster Virus (VZV) can lead to serious complications in Hematopoietic Cell Transplant (HCT) recipients. Central nervous system (CNS) VZV can be one of the most devastating infections in transplant recipients, yet little is known about this rare disease. Objectives: To describe CNS VZV in the post-transplant period and to define potential risk factors in the HCT population. Methods: We reviewed the course of all patients who received a first HCT at the Fred Hutchinson Cancer Center (FHCRC) in Seattle, WA from 1/1996 through 12/2007. Data were collected retrospectively using the Long-Term Follow-Up database, which includes on-site examinations, outside records, laboratory tests, and yearly questionnaires. Patients were classified as CNS VZV if they had laboratory confirmation of VZV in the cerebrospinal fluid (CSF), or had zoster with associated clinical and laboratory findings consistent with CNS disease. Results: A total of six patients developed VZV CNS disease during the evaluation period (table 1). Diagnosis was confirmed in 3/6 by detection of VZV in CSF by PCR. All other patients had a clinical diagnosis based on the presence of CNS symptoms, zoster, lymphocytic pleiocytosis, and response to IV acyclovir. Patients who developed CNS disease had a mean age of 42 years (range 34-51) at time of transplant. CNS disease developed at a mean of 9 months posttransplantation (range 0.5-24 months), and severity varied, ranging from meningitis (3/6) to encephalitis/myelitis (3/6). All had active graft-versus host disease (GHVD) and all were being treated with immunosuppressive therapy at time of diagnosis. Fever and headache were the most common symptoms, but patients who developed focal CNS findings or seizures (3/6) had a more complicated clinical course. While most patients presented with classic VZV/zoster skin lesions, 2/6 patients had no dermatologic findings associated with their presentation. Four (66%) of patients who developed VZV CNS disease died, two related to VZV complications despite aggressive antiviral therapy. Conclusions: In this cohort of HCT patients, VZV CNS disease was a rare complication. Mortality due to CNS VZV is high, particularly in patients who develop focal neurologic findings or seizures. Even in the absence of skin lesions, VZV CNS disease should be considered in patients who develop fevers and neurologic symptoms.

The Lausanne experience with the Wichita fusion nail for arthrodesis of the knee

Background: Arthrodesis of the knee by intramedullary fixation hasbeen reported to have a higher rate of success than external fixationor compression plating. Antegrade nailing however can lead to complicationsdue to the different diameters of the medullary canals, fracturesduring insertion, poor rotational stability, breakage of the IM-nailand insufficient compression at the fusion site.Method: This retrospective study reports all knee fusions performedby the same orthopaedic surgeon with the Wichita (Stryker) fusion nail(WFN) from 2004 to 2010. The Wichita nail is a short nail with a deviceat the knee which allows for coupling of differently sized and interlockedfemoral and tibial components and at the same time for compression.Results: We report of 18 patients with a mean follow up of 28 months(range 3-71 months). Infected TKA was the most common indicationfor arthrodesis in 9 cases. The remaining reasons included asepticfailed TKA in 3 cases, 2 patients after fracture, 1 patient with neurologicalinstability after knee dislocation, 1 patient after tumoral resectionand 1 non union after failed arthrodesis with long antegrade nail.Finally 1 patient with bilateral congenital knee dislocation operated onboth sides. As expected, patients receiving the WFN had undergonea large number of previous knee surgeries with a mean of 3.8 (range0-8) procedures per patient. The complication rate was 27% (5 of 18).Two patients had persistent pain requiring revision surgery to increasestability with plating. One case of periprosthetic fracture needed openreduction and internal fixation. 2 patients with superficial hematomawere treated one with open drainage and the other with physiotherapy.Infection was erradicated in all septic cases, we found no new infectionand the fusion rate was 100%.Conclusion: The results in these often difficult cases are satisfyingand we think that this technique is a valid alternative to the otherknown techniques of knee fusion in patients with a poor bone stockand fragile soft tissues.

The experience of transplantation for patients and their significant ones: A difficult encounter of two worlds

Objectives This paper reports on a longitudinal qualitative study exploring concerns of 60 patients before and after transplantation. Methods Semi-structured interviews were conducted without time constraints in a protected space out of the hospital. Qualitative analysis was performed. Results Prior to transplantation, all patients talked freely about negative feelings, stigmatisation, being misunderstood by others, loneliness and culpability caused by increasing physical dependency or abandoned roles. They mentioned alternative ways to cope (magic, spirituality), and even expressed their right to let go. In a subset of 13 patients, significant ones allowed themselves in the interview, or were integrated on the request of the patients. In this modified setting, two illness-worlds were confronted. If common themes were mentioned (e.g., modified life plans, restricted space, physical and psychological barriers), they were experienced differently. Fear of transplantation or guilt towards the donors was overtly expressed, often for the first time. Mutual hiding of anxiety in order to protect loved ones or to prevent loss of control was disclosed. The significant ones talked about accumulated stress and exhaustion related to the physical degradation of the patient, fear of the unpredictable evolution of illness and financial problems, and stressed their difficulty to adapt adequately to the fluctuating state of the patient. After transplantation, other themes emerged, where difficulty in disclosure was observed: intensive care and near death experiences, being a transplanted person, debt to the donor and his/her family, fear of rejection. Conclusions With the self-imposed strategy of hiding concerns to protect one another, a discrepancy between two illness-worlds was created. When concerns were confronted during the interviews, a new mutual understanding emerged. Patients and their families stated the need for sharing concerns in the course of illness.

Eight years experience from a skeletal dysplasia referral center in a tertiary hospital in Southern India: A model for the diagnosis and treatment of rare diseases in a developing country.

We report on a series of 514 consecutive diagnoses of skeletal dysplasia made over an 8-year period at a tertiary hospital in Kerala, India. The most common diagnostic groups were dysostosis multiplex group (n = 73) followed by FGFR3 (n = 49) and osteogenesis imperfecta and decreased bone density group (n = 41). Molecular confirmation was obtained in 109 cases. Clinical and radiographic evaluation was obtained in close diagnostic collaboration with expert groups abroad through Internet communication for difficult cases. This has allowed for targeted biochemical and molecular studies leading to the correct identification of rare or novel conditions, which has not only helped affected families by allowing for improved genetic counseling and prenatal diagnosis but also resulted in several scientific contributions. We conclude that (1) the spectrum of genetic bone disease in Kerala, India, is similar to that of other parts of the world, but recessive entities may be more frequent because of widespread consanguinity; (2) prenatal detection of skeletal dysplasias remains relatively rare because of limited access to expert prenatal ultrasound facilities; (3) because of the low accessibility to molecular tests, precise clinical-radiographic phenotyping remains the mainstay of diagnosis and counseling and of gatekeeping to efficient laboratory testing; (4) good phenotyping allows, a significant contribution to the recognition and characterization of novel entities. We suggest that the tight collaboration between a local reference center with dedicated personnel and expert diagnostic networks may be a proficient model to bring current diagnostics to developing countries. © 2014 Wiley Periodicals, Inc.

Experience with angiotensin II antagonists in hypertensive patients.

1. The availability of orally active specific angiotensin receptor antagonists (AT1 antagonists) has opened new therapeutic choices and provided probes to test the specific role of the renin-angiotensin system in the pathogenesis of cardiovascular disease. 2. The data available so far suggest that the antihypertensive efficacy of angiotensin receptor antagonists is comparable to that of angiotensin-converting enzyme (ACE) inhibitors. This provides further evidence that this latter class of drugs exerts its effect mainly through blockade of the renin-angiotensin enzymatic cascade. As expected, the association of a diuretic exerts an equally strong additive effect to the antihypertensive efficacy of both classes of drugs. 3. The most common side effect of ACE inhibitors, dry cough, does not occur with AT1 antagonists, which confirms the long-held view that this untoward effect of the ACE inhibitors is due to renin-angiotensin-independent mechanisms. 4. Long-term studies with morbidity/mortality outcome results are needed, before a definite position can be assigned to this newcomer in the orchestra of modern antihypertensive drugs. Notwithstanding, this new class of agents already represents an exciting new addition to our therapeutic armamentarium.

Evaluation of the indication for surgical extraction of third molars according to the oral surgeon and the primary care dentist. Experience in the Master of Oral Surgery and Implantology at Barcelona University Dental School

Introduction: Third molar extraction is the most frequent procedure in oral surgery. The present study evaluates the indication of third molar extraction as established by the primary care dentist (PCD) and the oral surgeon, and compares the justification for extraction with the principal reason for patient consultation. Patients and method: A descriptive study was made of 319 patients subjected to surgical removal of a third molar in the context of the Master of Oral Surgery and Implantology (Barcelona University Dental School, Barcelona, Spain) between July 2004 and March 2005. The following parameters were evaluated: sex, age, molar, type of impaction, position according to the classifications of Pell and Gregory and of Winter, and the reasons justifying extraction. Results: The lower third molars were the most commonly extracted molars (73.7%). A total of 69.6% of the teeth were covered by soft tissues only. Fifty-six percent of the lower molars corresponded to Pell and Gregory Class IIB, while 42.1% were in the vertical position. The most common reason for patient reference to our Service of Oral Surgery on the part of the PCD was prophylactic removal (51.0% versus 46.1% in the case of the oral surgeon). Discussion and conclusions. Our results show prophylaxis to be the principal indication of third molar extraction, followed by orthodontic reasons. Regarding third molars with associated clinical symptoms or signs, infectious disease-including pericoronitis- was the pathology most often observed by the oral surgeon, followed by caries. This order of frequency was seen to invert in the case of third molars referred for extraction by the PCD. A vertical position predominated among the third molars with associated pathology

Sudden cardiac death among general population and sport related population in forensic experience.

PURPOSE: The goal of the study was to assess the causes and analyze the cases of sudden cardiac death (SCD) victims referred to the department of forensic medicine in Lausanne, with a particular focus on sports-related fatalities including also leisure sporting activities. To date, no such published assessment has been done nor for Switzerland nor for the central Europe. METHODS: This is a retrospective study based on autopsy records of SCD victims, from 10 to 50 years of age, performed at the University Centre of Legal Medicine in Lausanne from 1995 to 2010. The study population was divided into two groups: sport-related (SR) and not sport-related (NSR) SCDs. RESULTS: During the study period, 188 cases of SCD were recorded: 166 (88%) were NSR and 22 (12%) SR. The mean age of the 188 victims was 37.3 ± 10.1 years, with the majority of the cases being male (79%). A cause of death was established in 84%, and the pathology responsible for death varied according to the age of the victims. In the NSR group, the mean age was 38.2 ± 9.2 years and there was 82% of male. Coronary artery disease (CAD) was the main diagnosis in the victims aged 30-50 years. The majority of morphologically normal hearts were observed in the 15-29 year age range. There was no case in the 10-14 year age range. In the SR group, 91% of victims died during leisure sporting activities. In this group the mean age was 30.5 ± 13.5 years, with the majority being male (82%). The main cause of death was CAD, with 6 cases (27%) and a mean age of 40.8 ± 5.5 years. The youngest victim with CAD was 33 years old. A morphologically normal heart was observed in 5 cases (23%), with a mean age of 24.4 ± 14.9 years. The most frequently implicated sporting activities were hiking (26%) and swimming (17%). CONCLUSION: In this study, CAD was the most common cause of death in both groups. Although this pathology most often affects adults over 35 years of age, there were also some victims under 35 years of age in both groups. SCDs during sport are mostly related to leisure sporting activities, for which preventive measures are not yet usually established. This study highlights also the need to inform both athletes and non athletes of the cardiovascular risks during sport activities and the role of a forensic autopsy and registries involving forensic pathologists for SR SCD.

Female common lizards (Lacerta vivipara) do not adjust their sex-biased investment in relation to the adult sex ratio.

Sex allocation theory predicts that facultative maternal investment in the rare sex should be favoured by natural selection when breeders experience predictable variation in adult sex ratios (ASRs). We found significant spatial and predictable interannual changes in local ASRs within a natural population of the common lizard where the mean ASR is female-biased, thus validating the key assumptions of adaptive sex ratio models. We tested for facultative maternal investment in the rare sex during and after an experimental perturbation of the ASR by creating populations with female-biased or male-biased ASR. Mothers did not adjust their clutch sex ratio during or after the ASR perturbation, but produced sons with a higher body condition in male-biased populations. However, this differential sex allocation did not result in growth or survival differences in offspring. Our results thus contradict the predictions of adaptive models and challenge the idea that facultative investment in the rare sex might be a mechanism regulating the population sex ratio.

Helicopter emergency medical rescue for the traumatized: experience in the metropolitan region of Campinas, Brazil

OBJECTIVE: To analyze the profile of patients served by the air medical rescue system in the Metropolitan Region of Campinas, evaluating: triage and mobilization criteria; response time; on-site care and transport time; invasive procedures performed in the Pre-Hospital Care (PHC); severity of patients; morbidity and mortality.METHODS: We conducted a prospective, descriptive study in which we analyzed medical records of patients rescued between July 2010 and December 2012. During this period, 242 victims were taken to the HC-Unicamp. Of the 242 patients, 22 were excluded from the study.RESULTS: of the 220 cases evaluated, 173 (78.6%) were male, with a mean age of 32 years. Blunt trauma was the most prevalent (207 cases - 94.1%), motorcycle accidents being the most common mechanisms of injury (66 cases - 30%), followed by motor vehicle collisions (51 cases - 23.2%). The average response time was 10 ± 4 minutes and the averaged total pre-hospital time was 42 ± 11 minutes. The mean values of the trauma indices were: RTS = 6.2 ± 2.2; ISS = 19.2 ± 12.6; and TRISS = 0.78 ± 0.3. Tracheal intubation in the pre-hospital environment was performed in 77 cases (35%); 43 patients (19.5%) had RTS of 7.84 and ISSd"9, being classified as over-triaged. Of all patients admitted, the mortality was 15.9% (35 cases).CONCLUSION: studies of air medical rescue in Brazil are required due to the investments made in the pre-hospital care in a country without an organized trauma system. The high rate of over-triage found highlights the need to improve the triage and mobilization criteria.

Tuberculosis in renal transplant patients: The experience of a single center in Medellín-Colombia, 2005-2013

Introduction: Tuberculosis is a common opportunistic infection in renal transplant patients. Objective: To obtain a clinical and laboratory description of transplant patients diagnosed with tuberculosis and their response to treatment during a period ranging from 2005 to 2013 at the Pablo Tobón Uribe Hospital. Methods: Retrospective and descriptive study. Results: In 641 renal transplants, tuberculosis was confirmed in 12 cases. Of these, 25% had a history of acute rejection, and 50% had creatinine levels greater than 1.5 mg/dl prior to infection. The disease typically presented as pulmonary (50%) and disseminated (33.3%). The first phase of treatment consisted of 3 months of HZRE (isoniazid, pyrazinamide, rifampicin and ethambutol) in 75% of the cases and HZME (isoniazid, pyrazinamide, moxifloxacin and ethambutol) in 25% of the cases. During the second phase of the treatment, 75% of the cases received isoniazid and rifampicin, and 25% of the cases received isoniazid and ethambutol. The length of treatment varied between 6 and 18 months. In 41.7% of patients, hepatotoxicity was associated with the beginning of anti-tuberculosis therapy. During a year-long follow-up, renal function remained stable, and the mortality rate was 16.7%. Conclusion: Tuberculosis in the renal transplant population studied caused diverse nonspecific symptoms. Pulmonary and disseminated tuberculosis were the most frequent forms and required prolonged treatment. Antituberculosis medications had a high toxicity and mortality. This infection must be considered when patients present with a febrile syndrome of unknown origin, especially during the first year after renal transplant.

An exploration of the experience of implementing the second step violence prevention curriculum in one primary general learning disabilities class /

Violence has always been a part of the human experience, and therefore, a popular topic for research. It is a controversial issue, mostly because the possible sources of violent behaviour are so varied, encompassing both biological and environmental factors. However, very little disagreement is found regarding the severity of this societal problem. Most researchers agree that the number and intensity of aggressive acts among adults and children is growing. Not surprisingly, many educational policies, programs, and curricula have been developed to address this concern. The research favours programs which address the root causes of violence and seek to prevent rather than provide consequences for the undesirable behaviour. But what makes a violence prevention program effective? How should educators choose among the many curricula on the market? After reviewing the literature surrounding violence prevention programs and their effectiveness, The Second Step Violence Prevention Curriculum surfaced as unique in many ways. It was designed to address the root causes of violence in an active, student-centred way. Empathy training, anger management, interpersonal cognitive problem solving, and behavioural social skills form the basis of this program. Published in 1992, the program has been the topic of limited research, almost entirely carried out using quantitative methodologies.The purpose of this study was to understand what happens when the Second Step Violence Prevention Curriculum is implemented with a group of students and teachers. I was not seeking a statistical correlation between the frequency of violence and program delivery, as in most prior research. Rather, I wished to gain a deeper understanding of the impact ofthe program through the eyes of the participants. The Second Step Program was taught to a small, primary level, general learning disabilities class by a teacher and student teacher. Data were gathered using interviews with the teachers, personal observations, staff reports, and my own journal. Common themes across the four types of data collection emerged during the study, and these themes were isolated and explored for meaning. Findings indicate that the program does not offer a "quick fix" to this serious problem. However, several important discoveries were made. The teachers feU that the program was effective despite a lack of concrete evidence to support this claim. They used the Second Step strategies outside their actual instructional time and felt it made them better educators and disciplinarians. The students did not display a marked change in their behaviour during or after the program implementation, but they were better able to speak about their actions, the source of their aggression, and the alternatives which were available. Although they were not yet transferring their knowledge into positive action,a heightened awareness was evident. Finally, staff reports and my own journal led me to a deeper understanding ofhow perception frames reality. The perception that the program was working led everyone to feel more empowered when a violent incident occurred, and efforts were made to address the cause rather than merely to offer consequences. A general feeling that we were addressing the problem in a productive way was prevalent among the staff and students involved. The findings from this investigation have many implications for research and practice. Further study into the realm of violence prevention is greatly needed, using a balance of quantitative and qualitative methodologies. Such a serious problem can only be effectively addressed with a greater understanding of its complexities. This study also demonstrates the overall positive impact of the Second Step Violence Prevention Curriculum and, therefore, supports its continued use in our schools.

Clinical experience of clobazam as add-on treatment in a cohort of adults with epilepsy

Objective: Epilepsy is a common neurologic disorder affecting 1% of the world population with one-third of these patients failing to have seizure control for more than one year. Clobazam is a long-acting benzodiazepine used worldwide for the treatment of epilepsy. This antiepileptic drug has demonstrated great clinical benefits with mild side effects. The objective of this study was to better understand the efficacy of clobazam treatment on adult patients with refractory epilepsy. Design: A retrospective review of 44 adult patients with diagnosis of epilepsy that were seen at our Epilepsy Clinic between January 2014 and May 2015. Setting: An outpatient epilepsy clinic at the Hospital Universitario Fundación Santa Fe de Bogota, Colombia. Participants: 44 adult patients with diagnosis of epilepsy. Measurements: Seizure frequency, adverse effects and the use of concomitant AEDs were reviewed in each of the patient´s clinical charts. Results: The responder rate of patients with clobazam was 52% at 3 months, 50% at 6 months and 55% at 12 month. Seizure freedom rates at 3, 6 and 12 months were 18%, 25% and 25% respectively. Clobazam related adverse events occurred only in four patients (9%) at the end of the twelve months with somnolence being the most common. Conclusion: These findings suggest that clobazam treatment in adult patients with focal or generalized epilepsy is effective and safe. Its use should be considered early when first-line agents fail to provide seizure control.

Harmonization of policies and training systems in Europe for urban development – The Experience of LUDA Project

The work developed in LUDA’s project has clearly showed that, in a European economic and cultural diversified frame, crossed by recent and not so recent historical challenging processes, the issues of the urban affairs certainly have different layouts, but, as a matter of fact, we can assume that in their essence they are common to all regions. Identifying a set of common problems is not difficult: the Luda’s; the disadjustment between people and goods mobility, the difficult articulation between space and development sustainability the fragile features of the urban space in its complexity, the responsible social management of current migrations etc.

Sex differences in common marmosets (Callithrix jacchus) in response to an unfamiliar food task

There is a growing body of information on sex differences In callitrichid behaviour that includes the animals' performance in food tasks. For example, both reproductive and non-reproductive adult females have been found to be more successful than adult males in solving food tasks. In this study ten adult male and ten adult female common marmosets (Callithrix jacchus), housed individually, were tested with an unfamiliar task that involved the extraction of an embedded food. The task was to open a plastic canister that contained a raisin; the open end was covered with parchment paper. Each marmoset was given 15 trials in three blocks of 5 consecutive days. We measured the latency for each animal to open the lid and get the raisin-by one of five strategies that spontaneously emerged. The females learned the task faster and more efficiently than males; all the females opened the canister on day 1, for instance, in contrast to seven of the males on the same day. Females also progressively decreased the time that they took to open the tube. The final latency on day 15, for instance, was significantly shorter for the females. These results are consistent with relevant literature for callitrichids and cannot be accounted for in terms of differences in mental abilities, strength, hand morphology, or energy requirements. Further investigation is necessary to clarify the reasons for these differences.

Amygdalar function reflects common individual differences in emotion and pain regulation success

Although the co-occurrence of negative affect and pain is well recognized, the mechanism underlying their association is unclear. To examine whether a common self-regulatory ability impacts the experience of both emotion and pain, we integrated neuroimaging, behavioral, and physiological measures obtained from three assessments separated by substantial temporal intervals. Out results demonstrated that individual differences in emotion regulation ability, as indexed by an objective measure of emotional state, corrugator electromyography, predicted self-reported success while regulating pain. In both emotion and pain paradigms, the amygdala reflected regulatory success. Notably, we found that greater emotion regulation success was associated with greater change of amygdalar activity following pain regulation. Furthermore, individual differences in degree of amygdalar change following emotion regulation were a strong predictor of pain regulation success, as well as of the degree of amygdalar engagement following pain regulation. These findings suggest that common individual differences in emotion and pain regulatory success are reflected in a neural structure known to contribute to appraisal processes.