Miracle-dz/UniFAR-FTUs huggingface dataset:
Progression of cardiomyopathy after liver transplantation in patients with familial amyloidotic polyneuropathy, Portuguese type.

BACKGROUND Transthyretin amyloidosis is today an accepted indication for orthotopic liver transplantation (OLT). For several mutations progression of the cardiomyopathy has been observed after OLT. The aim of this study was to assess the course of cardiac involvement in Swedish familial amyloidotic polyneuropathy (FAP), Portuguese type, after OLT. By comparison of the echocardiographic findings before OLT with those obtained after, the course of the heart involvement was followed. METHODS Twenty-three patients, who had undergone OLT and were examined with echocardiography 1-12 months before OLT, were available for the study. Twenty-one patients were examined 12-27 months after OLT, and 12 were re-examined 52-71 months after OLT. Two-dimensional and M-mode echocardiography were performed in accordance with the standards of the American Society of Echocardiography. RESULTS A significantly increased septal and left ventricular posterior wall thickness and a significantly increased left atrial dimension was observed at the post-OLT examinations, indicating a progression of the amyloid heart disease. This increase of the cardiac involvement was neither correlated to waiting time for OLT or to pre-operative signs of cardiomyopathy. CONCLUSIONS Even though the production of the amyloidogenic-mutated transthyretin is stopped by OLT, the cardiomyopathy may progress after the operation even for the Portuguese type of FAP. The increase of the septal and left ventricular posterior wall thickness after OLT is not restricted to patients with signs of left ventricular hypertrophy before the transplantation. The findings have important implications for the follow-up of FAP patients after OLT.
Ambiguity Aversion and Underdiversification

We examine asset allocation decisions under smooth ambiguity aversion when an investor has a prior degree of belief in the domestic capital asset pricing model (CAPM). Different from a Bayesian approach, the investor separately relies on the conditional distribution of returns and on the posterior over parameters to make decisions, rather than on the predictive distribution of returns that integrates priors and likelihood information. We find that in the perspective of U.S. investors, ambiguity aversion generates strong home bias in equity holdings, regardless of beliefs in the CAPM or risk aversion. Results become stronger under regime-switching investment opportunities.
Promising results of a pilot trial of a GD2 directed anti-idiotypic antibody as a vaccine for high risk neuroblastoma.

8511 Background: High risk neuroblastoma has a dismal outcome despite intensive therapy including stem cell transplant. We conducted the first pediatric trial of mAb1A7, an anti-id antibody directed against anti-GD2, 14G2a, as a vaccine for high risk neuroblastoma. METHODS Thirty one patients with high risk neuroblastoma (26 stage 4, 5 stage 3) who achieved first or subsequent complete response (16 in CR1, 7 in CR2) or very good partial response (3 in VGPR1, 5 in VGPR2) were entered into this trial. Patients received SQ injection of mAb1A7 (Titan Pharmaceutical Inc.) + QS-21 (Aquila Pharmaceutical Inc.) q2 weeks × 4, q m × 11. RESULTS The treatment was well tolerated with only transient local reactions, transient fever and chills in 4 patients and serum sickness in 1. There was no neuropathic pain which is often seen with infusion of anti-GD2. Despite prior intensive treatment including stem cell transplantation in all but 4 patients, all 31 patients generated anti-mAb1A7. The mean peak anti-mAb1A7 was 665 ± 390 ng /ml (83-1467). Anti-mAb1A7 inhibited the binding of mAb1A7 (Ab2) to 14G2A (Ab1) in 31/31. The peak inhibition was observed between weeks 22 and 62, at which time, immune sera from 14/31 patients inhibited binding of Ab2 to Ab1 by > 90% at a 160- fold dilution. Immunoaffinity purified Ab3 from 5/5 patients bound purified GD2 and was predominantly IgG. More importantly, immune sera from 9/13 patients tested after 37 weeks of treatment, displayed CDC activity. Furthermore, purified Ab3 from 6/8 patients mediated ADCC against neuroblastoma. To date, 17 of 20 patients who enrolled during first remission have no evidence of disease progression at 32+ to 52+ months (median + 47 m) from study entry, while only 1 of 11 patients who enrolled during 2nd remission remains progression free. CONCLUSIONS mAb1A7 + QS21 is safe and effective in inducing biologically active anti-GD2 in heavily pre-treated neuroblastoma patients and may be useful in controlling minimal residual disease. No significant financial relationships to disclose.
Temporal corneal phacoemulsification in patients with filtered glaucoma.

OBJECTIVE To evaluate the effect of temporal clear corneal phacoemulsification on intraocular pressure (IOP) in eyes that underwent prior trabeculectomy. DESIGN Retrospective case-control study. PATIENTS Forty consecutive patients who underwent temporal clear corneal phacoemulsification subsequent to trabeculectomy (trabeculectomy-phacoemulsification group) were identified, and 40 control patients who underwent trabeculectomy alone (trabeculectomy group) were matched to the case patients for length of follow-up, age, IOP, number of antiglaucoma medications, number of 5-fluorouracil injections, race, sex, and diagnosis. MAIN OUTCOME MEASURES Intraocular pressure before vs 1 year after phacoemulsification in the trabeculectomy-phacoemulsification group compared with IOP in the trabeculectomy group and survival analysis of IOP control after trabeculectomy in the 2 groups. RESULTS In the trabeculectomy-phacoemulsification group, IOP 1 year after phacoemulsification was not significantly different from the prephacoemulsification IOP value (P = .65). Kaplan-Meier survival analysis showed that the rates of IOP control 3, 6, and 9 years after trabeculectomy in the trabeculectomy-phacoemulsification group were 80%, 66%, and 44%, respectively; in the trabeculectomy group, these were 79%, 69%, and 55%, respectively. These survival curves were not statistically different (P = .55). CONCLUSION Cataract surgery by temporal clear corneal phacoemulsification in eyes with filtering blebs after trabeculectomy does not adversely affect long-term IOP control.
Mosaic inv dup(8p) marker chromosome with stable neocentromere suggests neocentromerization is a post-zygotic event.

Marker chromosomes containing active human neocentromeres have been described in individuals where the chromosomes are non-mosaic, suggesting that they are mitotically stable, but also in individuals where there is mosaicism, raising the possibility of neocentromere instability. We report two independently ascertained individuals who are mosaic for a supernumerary marker chromosome, shown by reverse chromosome painting to have an 8p origin, resulting in mosaicism for tetrasomy 8p23.1-->pter in the patient. The markers have a primary constriction but show no detectable centromeric alpha-satellite DNA. The marker in Patient 1 demonstrated no centromere protein CENP-B binding, but associated with nine different functionally critical centromere proteins. Investigation of peripheral blood lymphocytes from this patient on five separate occasions over a 13-year period showed 23-46% mosaicism for the marker chromosome with no decrease in incidence. In vitro investigation of primary and secondary sub-clones of a lymphoblast cell line derived from the patient demonstrated 100% stability of the marker chromosome indicating that neocentromere instability is unlikely to be responsible for the mosaicism in the patient. This and other available data support a general model of neocentromerization as a post-zygotic event, irrespective of whether the supernumerary chromosome fragment has arisen during meiosis or post-fertilization at mitosis.
INCREASING ISOLATION OF PROTECTED AREAS IN TROPICAL FORESTS OVER THE PAST TWENTY YEARS

4 UNEP World Conservation Monitoring Centre, Cambridge, UK Abstract. Protected areas are one of the cornerstones for conserving the world's re- maining biodiversity, most of which occurs in tropical forests. We use multiple sources of satellite data to estimate the extent of forest habitat and loss over the last 20 years within and surrounding 198 of the most highly protected areas (IUCN status 1 and 2) located throughout the world's tropical forests. In the early 1980s, surrounding habitat in the 50- km unprotected or less highly protected ''buffers'' enhanced the protected areas' effective size and their capacity to conserve richness of forest-obligate species above the hypothetical case of complete isolation. However, in nearly 70% of the surrounding buffers, the area of forest habitat declined during the last 20 years, while 25% experienced declines within their administrative boundaries. The loss of habitat occurred in all tropical regions, but protected areas in South and Southeast Asia were most severely affected because of rela- tively low surrounding forest habitat in the early 1980s and high subsequent loss, particularly in dry tropical forests. The future ability of protected areas to maintain current species richness depends on integrating reserve management within the land use dynamics of their larger regional settings.
Educational tourism in agriculture and identity of farm successors

Although farm visiting is gaining popularity as a type of experience-oriented tourism, the economic viability of this emerging service has not been fully established. To make it viable, it is not enough to approach educational tourism solely from a technical viewpoint, we also need to approach it from the aspect of the operator’s identity, a factor that has not been addressed fully in the arena of either rural tourism or tourism economics. Thus, by presenting a conceptual economic framework that explains the connection between a farmer’s identity and the level of diversified activity including tourism, this article has evaluated, based on a questionnaire survey of next-generation successors working on-farm at Educational Dairy Farms in Japan, the hypothesis that those farmers who have enlarged their identity will be able to successfully develop diversified activities. The results clarify factors that lead to enlarged identity, including formation of both a wider perspective and more extensive human networks from social learning opportunities, and female initiative within the activity. In this context, the issue of identity formation should be properly positioned in the field of innovation-oriented capability building of farm management.
Buoyancy and maximal diving depth in penguins: do they control inhaling air volume?

Using a newly developed data logger to measure acceleration, we demonstrate that free-ranging king and Adélie penguins only beat their flippers substantially during the first part of descent or when they were presumed to be chasing prey at the bottom of dives. Flipper beating stopped during the latter part of ascent: at 29+/-9 % (mean +/- S.D.) of dive depth (mean dive depth=136.8+/-145.1 m, N=425 dives) in king penguins, and at 52+/-20 % of dive depth (mean dive depth=72.9+/-70.5 m, N=664 dives) in Adélie penguins. Propulsive swim speeds of both species were approximately 2 m s(-1) during dives; however, a marked increase in speed, up to approximately 2.9 m s(-1), sometimes occurred in king penguins during the passive ascending periods. During the prolonged ascending, oblique ascent angle and slowdown near the surface may represent one way to avoid the potential risk of decompression sickness. Biomechanical calculations for data from free-ranging king and Adélie penguins indicate that the air volume of the birds (respiratory system and plumage) can provide enough buoyancy for the passive ascent. When comparing the passive ascents for shallow and deep dives, there is a positive correlation between air volume and the depth of the dive. This suggests that penguins regulate their air volume to optimize the costs and benefits of buoyancy.
Detection of hepatitis C virus RNA from gingival crevicular fluid and its relation to virus presence in saliva.

BACKGROUND To search for a possible source of hepatitis C virus (HCV) in saliva, the presence and shedding patterns of HCV in gingival crevicular fluid (GCF) and saliva of HCV viremic patients were assessed based on clinical, biochemical, histological, virological, and oral health parameters. METHODS Saliva and GCF samples of 50 HCV viremic patients were collected to detect HCV RNA by a modified commercial polymerase chain reaction (PCR) assay. Clinical oral examination was performed and periodontal status at the collection sites was monitored. The results were correlated to specified parameters. RESULTS HCV RNA was detected in 59% (29/49) of the GCF specimens and in 35% (17/48) of the saliva specimens. In saliva specimens, HCV RNA was detected only in cases which also had detectable HCV RNA in the GCF samples (P=0.00002) and was significantly related to the presence of blood in saliva (P=0.03). Higher, but not significant, values of oral clinical parameters at the sites of fluid collection were found in GCF specimens harboring HCV RNA. In GCF specimens with no blood detected, HCV RNA was more often present in cases with higher plasma viral load (P=0.05). CONCLUSIONS The results suggest that besides blood, the other most probable source of HCV in saliva is GCF. Unknown endogenous HCV inhibitory mechanisms in the oral cavity may explain the discrepancies in HCV appearance between saliva and GCF. The results provide a biologic basis for further investigation of the role of HCV in the pathogenesis of periodontal disease.
Late-onset Tay-Sachs disease presenting as catatonic schizophrenia: diagnostic and treatment issues.

BACKGROUND It is not commonly appreciated that patients with hexosaminidase A deficiency (Tay-Sachs disease) can first present in adulthood with psychiatric illness. METHOD A 17-year-old non-Jewish male patient was referred with a history of treatment-resistant catatonic schizophrenia. We uncovered coexistent neurologic abnormalities and evidence of cognitive decline. Metabolic screening revealed a severe deficiency of beta-hexosaminidase A. We reviewed the literature on late-onset gangliosidosis with attention to (1) the nature of the associated psychiatric and neurologic abnormalities and (2) the treatment of psychosis in these patients. RESULTS The patient's catatonia responded promptly to benzodiazepine therapy. Treatment with neuroleptic medication resulted in the rapid development of neuroleptic malignant syndrome. The patient was thereafter maintained on lorazepam with resolution of his acute psychiatric disturbances and unexpected improvement in his neurologic status. CONCLUSION Patients with hexosaminidase deficiency may first present in adolescence or adulthood with psychiatric illness, particularly schizophrenic-like psychosis. The presence of speech disturbance, gait abnormalities, movement disorders, and cognitive decline may indicate an underlying metabolic disorder. The use of traditional neuroleptics to treat the psychosis in such individuals may produce an unacceptably high risk/benefit ratio. Benzodiazepines may ameliorate the psychiatric and neurologic abnormalities in these patients.
Unique insertional translocation in a childhood Wilms' tumor survivor detected when his daughter developed bilateral retinoblastoma

Retinoblastoma and Wilms' tumor are rare childhood embryonic tumors associated with loss or inactivation of tumor suppressor genes, RB1 located within 13q14, and WT1 located within 11p13. Interchromosomal insertional translocations occur rarely, and such rearrangements within RB1 or WT1, even rarer. We report a unique family in which an insertional translocation of a chromosomal segment that included band 13q14 inserted into 11p13 caused childhood Wilms' tumor in the father, and whose child developed bilateral retinoblastoma. This is the first case of an insertional translocation that caused both tumors. This insertional translocation had significant consequences for genetic counseling and in utero diagnosis. The estimated risk for an offspring of this father to develop Wilms' tumor is up to 50%, to develop retinoblastoma up to 25%, to have neither tumor 25%, and to have both tumors 0%. © 2003 Wiley‐Liss, Inc.
Optimum exposure ranges for computed dental radiography.

OBJECTIVE Computed dental radiography (CDR; Schick Technologies Inc, Lond Island City, NY, USA) can be used with any dental X-ray generator. The optimum exposure at various tube voltage settings was studied. METHODS Images were made of a dental QA jaw phantom and a standard aluminium stepwedge. Exposures were made between 50 and 90 kVp at 10 kVp intervals. Two contrast indices were calculated from the bone stepwedge pixel values: CI(1), the ratio between the highest and lowest pixel values, and CI(2), the difference between them. RESULTS Contrast indices were greatest at low kVp. The gradual decrease in CI(1) with increased exposure demonstrated that the optimum exposure range was always relatively wide. Maximum CI(2) values were found at exposures of 27, 17, 15, 11 and 9microC kg-1 at 50, 60, 70, 80 and 90 kVp, respectively, at the centre of the optimum exposure range. Pixel values for each aluminium step increased both with increased exposure and with increased kVp. The longest contrast scale was obtained at 11.0, 9.6, 8.7, 7.2 and 7.0 microC kg-1 at 50, 60, 70, 80 and 90 kVp. The steepest slopes were obtained either with thin aluminium steps or at low kVp. CONCLUSIONS CDR is a fast CCD-based system and is capable of operating at a wide range of kVp settings.
Prophylactic positive end-expiratory pressure and postintubation hemodynamics: an interventional, randomized study.

OBJECTIVE To investigate the hemodynamic and outcome effects of implementing prophylactic positive end-expiratory pressure (PEEP) versus zero end-expiratory pressure (ZEEP) in patients during the postintubation period in the emergency setting. METHODS The present study was a prospective, single-centre, interventional, single-blinded randomized trial performed in a 16-bed medical intensive care unit. The study cohort consisted of consecutive patients who urgently required intubation. During the postintubation period, patients received either 5 cmH2O PEEP or ZEEP. The primary aim was to assess the variation in mean arterial pressure (MAP) from baseline up to 90 min postintubation. The secondary aim was to determine the mean duration of intubation, level of MAP support after intubation and 28-day mortality. RESULTS Seventy-five consecutive patients with similar mean (+/- SD) baseline characteristics and preintubation MAP (76+/-18 mmHg in the ZEEP group and 78.5+/-23 mmHg in the PEEP group, P=Not significant [NS]) were studied. The final analysis was performed in 33 patients in the ZEEP group and 30 patients in the PEEP group. Regarding outcome measures following intubation, delta MAP (ie, the difference between the lowest MAP values from baseline) was not differentially affected in either group (P=NS); the mean durations of intubation were similar (ZEEP 9.2+/-8.5 days versus PEEP 9.2+/-8.8 days, P=NS); 28-day mortality was not discriminative (ZEEP 14 of 33, PEEP nine of 30; P=NS); and levels of MAP support after intubation were comparable between the two groups. CONCLUSION In the present trial, there was no evidence that implementing a prophylactic PEEP of 5 cmH2O adversely affects short-term hemodynamics or outcome in medical intensive care patients during the postintubation period.
AFRICAN WOMEN AS GLOBAL LEADERS: CHALLENGES AND PROSPECTS

The women in traditional African societies suffered discrimination and prejudice in their quest to participate in leadership. This paper examined the attitude of modern Africans toward s allowing female equal access as their male counterparts to leadership positions in global organisations. The study was conducted among 100 part time students of a tertiary institution who worked under male and female leaders. The participants were purposeivey selected into a quasi-experimental group. They were asked to express a preference between a male leader and a female leader assuming they were the ones to decide who to hire to fill positions of CEOs in two simulated global organisations. They were also requested to rate their female or male leaders using Multifactor Leadership Questionnaire (MLQ). The result of the study revealed that women were still unfavourably perceived as fit for leadership positions. The reasons for the negative perception towards women participation in leadership was discussed, the qualities that make African women good leaders were analysed, and efforts that can be made to increase women’s leadership participation in Africa were also suggested.
Narcotic-exposed neonates in a First Nations population in northwestern Ontario: incidence and implications.

OBJECTIVE To document the incidence of neonatal abstinence syndrome (NAS) and the rate of narcotic use during pregnancy in northwestern Ontario, where narcotic abuse is a growing social and medical problem. DESIGN Retrospective chart review. SETTING The Sioux Lookout Meno Ya Win Health Centre catchment area in northwestern Ontario. PARTICIPANTS Mothers and neonates for the 482 live births that took place in the 18-month study period (January 2009 to June 2010). MAIN OUTCOME MEASURES Maternal drug use and neonatal outcomes were documented. RESULTS The incidence of narcotic (oxycodone) abuse during pregnancy increased from a low of 8.4% at the beginning of the study period to a high of 17.2% by mid-2010. Narcotic-using mothers were more likely to also use nicotine and alcohol, to have premature deliveries, and to be episodic users. Narcotic-exposed neonates experienced NAS 29.5% of the time; daily maternal use was associated with a higher rate of NAS (66.0%). While all infants roomed in with their mothers, exposed infants were more likely to require transfer to a tertiary care nursery. Infants with severe NAS were treated with oral morphine and had significantly longer hospital stays compared with the entire cohort (4.5 vs 1.5 days, P = .004). Narcotic abuse during pregnancy in our region is not currently associated with increased rates of HIV or hepatitis C infection, as intravenous route of administration is less common at present than intranasal and oral ingestion. CONCLUSION Narcotic abuse during pregnancy is a considerable problem in First Nations communities in northwestern Ontario. Community-based initiatives need to be developed to address this issue, and medical and nursing staff need to develop surveillance, assessment, and therapeutic responses. Passive neonatal addiction and withdrawal result from maternal narcotic use during pregnancy. Rates of opioid use among pregnant Canadian women are unknown.
Structure-Function Relationships in l-Amino Acid Deaminase, a Flavoprotein Belonging to a Novel Class of Biotechnologically Relevant Enzymes*

l-Amino acid deaminase from Proteus myxofaciens (PmaLAAD) is a membrane flavoenzyme that catalyzes the deamination of neutral and aromatic l-amino acids into α-keto acids and ammonia. PmaLAAD does not use dioxygen to re-oxidize reduced FADH2 and thus does not produce hydrogen peroxide; instead, it uses a cytochrome b-like protein as an electron acceptor. Although the overall fold of this enzyme resembles that of known amine or amino acid oxidases, it shows the following specific structural features: an additional novel α+β subdomain placed close to the putative transmembrane α-helix and to the active-site entrance; an FAD isoalloxazine ring exposed to solvent; and a large and accessible active site suitable to bind large hydrophobic substrates. In addition, PmaLAAD requires substrate-induced conformational changes of part of the active site, particularly in Arg-316 and Phe-318, to achieve the correct geometry for catalysis. These studies are expected to pave the way for rationally improving the versatility of this flavoenzyme, which is critical for biocatalysis of enantiomerically pure amino acids.