Erdheim-Chester disease: 25-year history with early CNS involvement.

We report a case of Erdheim-Chester disease (ECD) with a 25-year history following initial presentation with diabetes insipidus and brainstem involvement. The exceptionally long history is particularly notable, given that ECD is a life-threatening disorder and there is a recognised association between central nervous system involvement and poor outcome. The case is a timely reminder of the presenting features of the condition, given the emergence of potential new treatment options.

Virulence and vertical transmission of two genotypically and geographically diverse isolates of Trypanosoma cruzi in mice.

Although principally considered a vector-borne disease, the vertical transmission of Trypanosoma cruzi from mother to child is now recognized as a significant and increasing threat to human health. Despite its importance, significant gaps exist in our understanding of the relationships between genotype, virulence, and the extent of vertical transmission of this pathogen. To better understand these relationships, we describe the comparison of a South American-derived Type I isolate (BS) of T. cruzi to a Type IIa isolate (SCI) of from North America for virulence and frequency of vertical transmission in BALB/c and outbred mice. Assays performed in BALB/c mice conclusively confirm the comparatively greater virulence of the BS isolate. Breeding experiments demonstrated a reciprocal relationship between virulence and the frequency of vertical transmission, with the pups born to Type IIa SCI-infected female mice testing positive at twice the frequency (66%) as those infected with the Type I BS (33%). Experiments carried out in BALB/c mice confirmed that an active infection with the SCI isolate generated immunity against a BS challenge. These results confirm that significant differences in the extent of vertical transfer can exist between T. cruzi isolates and contradicts the hypothesis that such transmission is a function of elevated maternal blood parasitemias. This study also provides support for some of the current hypotheses on attenuation during a pathogen's evolution from vector-borne to vertical transmission. We suggest that T. cruzi may provide a useful model for the study of the adaptive dynamics of a zoonotic human pathogen.

Application of raisin extracts as preservatives in liquid bread and bread systems.

Interest in natural ingredients with multifunctions in food has led to the investigation of the antimicrobial activity of raisins, a traditional baking ingredient. Water and 60% ethanol extracts, and raisin juice concentrate (RJC, a commercial food ingredient) were assessed for their ability to inhibit the growth of ropy-bread-causing Bacillus species. The ability of these additives and raisin paste were tested for antifungal activity in liquid bread model system and bread. In the liquid bread model, concentrations as low as 20 and 36 mg crude extract or RJC per gram media significantly (P < 0.05) reduced the populations of B. licheniformis and B. subtilis, respectively. A significant reduction in the population of Aspergillus flavus and Penicillium chrysogenum was achieved at 80 and 470 mg crude extract per gram media, respectively. Bread formulas containing 7.5% of a water extract (equivalent to 70 mg extract per gram dough) or RJC (70 mg/g dough) produced bread that had equivalent mold-free shelf life. The mean mold-free shelf life of the bread containing 7.5% water extract was 18.1 +/- 3.3 d at room temperature while the negative control was mold free for 9.4 +/- 2.4 d. The antifungal efficacy of the extracts in bread was equivalent to 0.24% calcium propionate in 21 d of storage. Doubling the concentration of the extract did not improve the mold-retarding property in bread. The bread containing raisin paste, the percentage of which in dough was equivalent to 15% raisin extract, exhibited a stronger antifungal activity than did the extracts in bread.

Treatment-induced stable, moderate reduction in blood cell counts correlate to disease control in early rheumatoid arthritis.

BACKGROUND: Treatment of rheumatoid arthritis (RA) has become more intensive, thereby raising concerns regarding toxicities, including leucopenia. The objective was to analyse cell counts obtained as routine surveillance for adverse effects to assess the effect of intensive treatment and treatment dosage and to examine correlations to disease activity scores. METHODS: Patients with early RA were treated with combinations of disease-modifying anti-inflammatory drugs according to pre-defined rules, with dose adjustments contingent on residual disease activity and tolerance. RESULTS: Mean leucocyte, neutrophil and platelet counts fell with levels that correlated to disease activity scores. The strongest correlation was between platelets and disease activity scores. There was a modest, inverse correlation between methotrexate dose and monocyte and lymphocyte counts. No serious toxicity associated with the therapy was seen. CONCLUSION: Moderate reductions in cell counts are well tolerated in RA and appear to contribute to disease control.

A compact strip-line pulsed power generator for isentropic compression experiments.

Veloce is a medium-voltage, high-current, compact pulsed power generator developed for isentropic and shock compression experiments. Because of its increased availability and ease of operation, Veloce is well suited for studying isentropic compression experiments (ICE) in much greater detail than previously allowed with larger pulsed power machines such as the Z accelerator. Since the compact pulsed power technology used for dynamic material experiments has not been previously used, it is necessary to examine several key issues to ensure that accurate results are obtained. In the present experiments, issues such as panel and sample preparation, uniformity of loading, and edge effects were extensively examined. In addition, magnetohydrodynamic simulations using the ALEGRA code were performed to interpret the experimental results and to design improved sample/panel configurations. Examples of recent ICE studies on aluminum are presented.

The host-parasite neuroimmunoendocrine network in schistosomiasis: consequences to the host and the parasite.

The physiological interactions during the course of any immune response are complex. Infection induces antigen-specific recognition by the immune system, which is consequently charged with the responsibility of marshalling the appropriate effector responses necessary to destroy the pathogen, or at the very least inhibit its progression. Obviously, the immune system should accomplish this while minimizing collateral damage to the host or it risks, winning a Pyrrhic victory. As our understanding of the neuroendocrine system grows, it has become increasingly clear that this complex network of neurotransmitters, hormones and cytokines plays an important role in mediating immunity. Schistosomes present an especially complex relationship between pathogen and these physiological systems, with hormonally dependent host factors such as sex and age correlated with parasite success. In this report, we review the current literature on sex and age associations between infection and progression of disease. We then follow with a discussion on interactions between the host neuroendocrine and immune systems. We also speculate on strategies to apply this knowledge to novel treatment strategies. Results argue for a complex network comprising the immune, endocrinological and nervous systems of both host and schistosome in the regulation of the plural outcomes of infection.

Regulation of hepatic microcirculation in stepwise liver resection.

BACKGROUND: After liver resection a small-for-size syndrome may result from the reduction of liver volume and additional liver damage caused by hepatic hyperperfusion. Therefore the influence of the extent of liver resection on liver perfusion is investigated. MATERIAL AND METHODS: A stepwise liver resection (removal of 30%, 70%, 90%, 95% and 97% of the liver) was performed under inhalation anaesthesia with isoflurane in 6 male Lewis rats. Besides systemic arterial and venous blood pressure the portal pressure and flow was measured and the sinusoidal perfusion was visualized. Sinusoidal diameter, intersinusoidal diameter and functional capillary density were determined. RESULTS AND CONCLUSIONS: A decrease in the portal flow but an increase in the portal pressure was observed. Sinusoidal diameter showed a steady but low increase when up to 70% of the liver was removed but a high increase after 90% or more of the liver was resected. This indicates a decompensation of a regulatory mechanism of sinusoidal perfusion.

Prevalence of Neospora caninum infection in Australian (NSW) dairy cattle estimated by a newly validated ELISA for milk.

AIM: To determine the performance characteristics of an Institut Pourquier (IP) enzyme-linked immunosorbent assay (ELISA) for the detection of antibodies against Neospora caninum in bovine milk and subsequent determination of the prevalence of N. caninum infection in New South Wales (NSW) dairy cattle. METHODS: Matching serum and milk samples from 93 cattle were assayed in two commercially available ELISAs for the detection of anti-N. caninum antibodies. Serum test results of one ELISA (IDEXX) were used to determine the N. caninum infection status of the cattle. Optimised cut-off values for the IP ELISA using milk samples were determined by two-graph receiver operating characteristic (TG-ROC) analysis and then applied to a representative sample of 398 milk samples from dairy herds around NSW. RESULTS: When this ELISA was applied to a representative collection of 398 milk samples from dairy cattle across NSW it demonstrated a 21.1% prevalence of N. caninum infection in those cattle. From the TG-ROC analysis an IP ELISA protocol was derived which suggested a cut-off threshold that would allow milk testing with 97% sensitivity and specificity, respectively, relative to serum testing. CONCLUSIONS: The prevalence of N. caninum in NSW dairy cattle was higher than previously believed. When used on individual milk samples this ELISA demonstrated high sensitivity and specificity and so could be used to accurately identify N. caninum infection. TG-ROC analysis of the IP ELISA optimised the protocol and prescribed cut-off values enabling the ELISA to be used for the screening of N. caninum antibodies in the milk of dairy cattle.

Performance characteristics and optimisation of cut-off values of two enzyme-linked immunosorbent assays for the detection of antibodies to Neospora caninum in the serum of cattle.

AIM: To determine the performance characteristics of two enzyme-linked immunosorbent assays (ELISAs) manufactured by Institut Pourquier (IP) for the detection of antibodies against Neospora caninum in bovine sera. METHODS: Sera from 526 cattle were assayed in two ELISAs (IP) for the detection of anti-N. caninum antibodies. Results from a further ELISA (IDEXX) were used to provide the "gold standard"N. caninum infection status of the cattle and the ELISA results assessed by two-graph receiver operating characteristic (TG-ROC) analysis. RESULTS: TG-ROC analysis suggested changes to one of the IP ELISA protocols, arriving at a cut-off threshold that was different to the one recommended by the manufacturer. With that change, both of the ELISAs performed with high sensitivity and specificity (in excess of 98%) for bovine sera. CONCLUSIONS: The analysis of the two IP ELISAs when used on individual bovine sera demonstrated high sensitivity and specificity. TG-ROC analyses optimised the cut-off point suggested by the manufacturer for one of these commercial diagnostic assays and found agreement with the manufacturer's cut-off regarding the other assay. This will help with the accurate identification of infected animals and thereby contributing to the control of neosporosis.

Neospora abortions in dairy cattle: diagnosis, mode of transmission and control.

AIM: To determine the contribution of Neospora caninum to abortions on a dairy farm in NSW (Australia), determine the mode of transmission and develop and trial a control option for infection. METHODS: Two whole herd bleeds were conducted 12 months apart and the association between serological status and abortion events were calculated for a number of bovine abortifacients. Family trees were constructed for N. caninum seropositive cattle in the herd. Some N. caninum seropositive cows were culled from the herd and no female offspring was retained from seropositive cows. RESULTS: At the first whole-herd bleed in December 2002 a seroprevalence of 10.2% for N. caninum infection was detected. Cows with N. caninum infection were 13 times more likely to abort than uninfected ones. Seventy-five percent of seropositive animals in the herd were related, suggesting a high degree of congenital infection/transmission. Only 15% of infections were likely to be postnatally acquired. Selective culling of seropositive cows and not breeding from them reduced the number of seropositive animals. Only one newly sero-converted cow was detected at the second whole-herd bleed 12 months later. CONCLUSIONS: Seroepidemiological approaches were able to establish a high degree of association between N. caninum infection and low-level abortion in the dairy herd. Vertical transmission of infection was the predominant mode of infection and hence control efforts aimed at selectively culling seropositive animals from the herd were highly successful in reducing the level of infection.

The effect of stimulus height on visual discrimination in horses.

This study investigated the effect of stimulus height on the ability of horses to learn a simple visual discrimination task. Eight horses were trained to perform a two-choice, black/white discrimination with stimuli presented at one of two heights: ground level or at a height of 70 cm from the ground. The height at which the stimuli were presented was alternated from one session to the next. All trials within a single session were presented at the same height. The criterion for learning was four consecutive sessions of 70% correct responses. Performance was found to be better when stimuli were presented at ground level with respect to the number of trials taken to reach the criterion (P < 0.05), percentage of correct first choices (P < 0.01), and repeated errors made (P < 0.01). Thus, training horses to carry out tasks of visual discrimination could be enhanced by placing the stimuli on the ground. In addition, the results of the present study suggest that the visual appearance of ground surfaces is an important factor in both horse management and training.

Use of a wave reverberation technique to infer the density compression of shocked liquid deuterium to 75 GPa.

A novel approach was developed to probe density compression of liquid deuterium (L-D2) along the principal Hugoniot. Relative transit times of shock waves reverberating within the sample are shown to be sensitive to the compression due to the first shock. This technique has proven to be more sensitive than the conventional method of inferring density from the shock and mass velocity, at least in this high-pressure regime. Results in the range of 22-75 GPa indicate an approximately fourfold density compression, and provide data to differentiate between proposed theories for hydrogen and its isotopes.

Estimation of actinide skeletal content in humans based on bone samples collected at autopsy.

The USTUR has developed simple linear and multiple regression models for estimating skeletal actinide concentrations on the basis of bone samples collected at autopsies of non-whole body tissue donors. Bone samples usually collected include a clavicle, the patella(e), one or more ribs, the sternum, and a vertebral wedge cut from within the abdominal cavity. The described models were derived by regression analyses with the analytical results from those bones and the entire skeletons of eight whole body donations to the USTUR. With the model, skeletal concentrations of 238Pu, (239+240)Pu, and 241Am can be estimated from wet or ashed actinide concentrations in one to five of the bones usually collected at autopsy and analyzed. Application of the models to a selected USTUR non-whole body donation (Case 0240) indicated that the skeletal actinide concentration estimates were reasonably precise and that there was good agreement between the results from individual bones with wet or ashed actinide concentrations. The USTUR will apply the model that is based on wet concentrations of bones to estimate skeletal concentrations of actinides in all non-whole body autopsy cases for the sake of consistency because of the large number of early cases for which ashed weights of bones were not recorded.

Equation of state measurements in liquid deuterium to 70 GPa.

Using intense magnetic pressure, a method was developed to launch flyer plates to velocities in excess of 20 km/s. This technique was used to perform plate-impact, shock wave experiments on cryogenic liquid deuterium ( L-D(2)) to examine its high-pressure equation of state. Using an impedance matching method, Hugoniot measurements were obtained in the pressure range of 30-70 GPa. The results of these experiments disagree with previously reported Hugoniot measurements of L-D(2) in the pressure range above approximately 40 GPa, but are in good agreement with first principles, ab initio models for hydrogen and its isotopes.

Prevalence survey of multiple sclerosis in the Australian Capital Territory.

AIM: This study sought to obtain an estimate of the prevalence of multiple sclerosis (MS) in the Australian Capital Territory (ACT), a largely urban region that differs climatically and socioeconomically from other Australian cities examined in previous MS surveys. METHODS: Prevalence day was chosen to coincide with the 1996 National Census. All ACT neurologists' records for the previous 5 years were examined and cases of MS were classified according to the published diagnostic criteria of Rose et al. and Poser et al. RESULTS: By the criteria of Rose et al., as used in previous Australian surveys of MS, prevalence was 79.9/100,000 (95% confidence interval (CI) = 63.4-99.2) for females, 32.8 (22.7-46.2) for males and 56.7 (43.1-74.1) for all people, standardized to the 1996 population. Standardized to the 1981 population for direct comparison with 1981 surveys in New South Wales, the prevalence of MS in the ACT was still unexpectedly high, particularly for females. Using the criteria of Poser et al., the prevalence of MS standardized to the 1996 population was 70.6/ 100,000 (95% CI = 58.4-85.3) for females, 28.0 (20.3-37.8) for males and 49.5 (42.2-58.2) for all people. There was evidence from a relatively short duration of disease in the ACT sample that some persons with long-standing MS had been missed in the survey and therefore that the prevalence of MS observed in the ACT was an underestimate. CONCLUSIONS: The survey found an unexpectedly high prevalence of MS in the ACT. Possible reasons for this are discussed. There was no evidence that the advent of magnetic resonance imaging had increased the numbers of persons diagnosed with MS in the present survey.

Meeting the needs of abused older women? A british columbia and yukon transition house survey.

ABSTRACT Older women victims of violence by a partner or family member are more often categorized as victims of elder abuse, not victims of family violence. Their needs are assessed, if at all, by social and health service agencies, often with little knowledge or understanding of domestic violence, while domestic violence policy initiatives, advocacy, and services focus on younger women and children. A survey of domestic violence services in British Columbia and the Yukon found that women over 60 years of age were a very small fraction, generally less than two percent, of women served, and those 50 to 59 years of age were also substantially under-represented. Only four percent of respondents had special services for older women. Comments from respondents confirm that abused older women face enormous difficulties in trying to establish a safe and secure future. It was also evident that better interaction is needed between those providing support and advocacy for abused women, and those who provide health and social services for older persons. A majority of the shelters provided community education to a wide range of agencies. Most have only partial accessibility to women with mobility handicaps.

Aneurysmal intracerebral hemorrhage: clinical outcome after emergent surgical treatment.

BACKGROUND: Management guidelines and recovery potentials for individuals presenting with poor clinical condition owing to an aneurysmal intracerebral hemorrhage are not well established. METHODS: We assessed the outcome of a consecutive series of 6 patients with aneurysmal intracerebral hemorrhages presenting with Hunt and Hess clinical grade IV or V selected to undergo emergent hematoma evacuation and aneurysm clip obliteration. Their presenting clinical condition and neuroradiology were assessed, as well as early surgical results and follow-up outcome. RESULTS: The 2 women and 4 men were 30 to 59 years old. All 6 patients had profound neurological deficits on admission, with Glasgow Coma Scale scores of 4 to 9. Aneurysmal intracerebral hemorrhage diameters measured from 6 to 8 cm and were associated with 5 to 15 mm of midline shift. Surgical intervention was initiated within 3 hours of ictus in 5 patients and within 7 hours in one patient. All 6 patients survived, demonstrated clinical improvement postoperatively, and were transferred to rehabilitation centers 19 to 30 days after presentation. The Glasgow Outcome Scores were 2 to 3 with a minimum follow-up interval of 12 months. Four patients returned home and resumed independent activities of daily living; one required partial supervision. One patient remained in a chronic care facility. CONCLUSION: An acute management protocol, including aggressive neurosurgical intervention, with craniotomy for hematoma evacuation and aneurysm clip obliteration, can result in good neurological outcome in carefully selected, poor-grade patients with aneurysmal intracerebral hemorrhage.

Food-bound B12 absorption and serum total homocysteine in patients with low serum B12 levels.

This study was undertaken to determine whether measurements of serum total homocysteine (Hcys) and bound B12 absorption are useful in determining which patients with low- or low-normal levels of serum B12 are B12 deficient. In 40 patients with low or borderline serum levels of B12, food-bound B12 absorptions were determined using a body counter in an iron room, and were related to serum total Hcys levels. Food-bound B12 absorption was decreased in 16 patients and in an additional four, absorption of the free vitamin was also decreased. Homocysteine levels were elevated in four of the 16; in three of the four who had both decreased bound and free B12 absorptions, Hcys was elevated. If elevation of the Hcys level indicates tissue deficiency of B12, the 75% incidence of normal levels of Hcys in these patients with low food-bound B12 absorptions suggests the existence of a cohort of patients who may be at risk to develop, but have not yet developed, B12 deficiency. Only long term follow-up will reveal how many ultimately will become B12 deficient.

Patient management in head injury care: a nursing perspective.

Through a review of the literature, this article provides a consideration of nursing responsibilities and nursing care in the management of patients with head injuries. A brief review of the pathophysiology of head injury is followed by a description of intracranial pressure (ICP) monitoring and cerebral perfusion pressure (CPP) measurement, using the Camino fibreoptic catheter and monitor, with exploration of pulse and trend waveforms and their significance in assessing cerebral compliance. Jugular venous bulb saturation (SjO2) monitoring adds another dimension to the assessment of neurological damage by demonstrating cerebral tissue perfusion and cerebral metabolic rate, and this is also discussed. After identification of assessment as a crucial part of the nursing role, aspects of nursing care of head-injured patients are discussed. The discussion of respiratory support measures includes the controversial use of hyperventilation in reducing ICP, the prevention of hypoxia, the risk of respiratory complications, the use of suctioning and turning to prevent these and the problems associated with these nursing care measures. Other aspects of care are also considered, including careful positioning and temperature control, and the benefits of mild hypothermia.