Miracle-dz/UniFAR-FTUs huggingface dataset:
Glycogen Storage Disease type Ib: a paediatric case report.

AIM This paper addresses the need of the dental literature to document cases of Glycogen Storage Disease (GSD) type Ib with focus on the paediatric management of oral and dental problems and the potential complications arising from the increased susceptibility to bacterial infections, cariogenicity, and blood diathesis. METHODS Previous medical and dental papers on GSD type Ib published from 1980 to 2006 have been searched in the electronic databases PubMed and EMBASE using keywords of the NLM's Thesaurus, with the aim to retrieve important implications of treatment and preventive measures for an evidence-based multidisciplinary medical and dental management of the oral health problems of a 9-year old boy affected by GSD type Ib. CASE REPORT Oral and dental manifestations observed during a period of two years and relative treatments are reported from our case. Data on pharmacologic, oral hygienic, dietary, and surgical preventive measures for the control of recurrent oral infections, dental caries, gingival inflammation and risk of surgical bleeding are described. In addition, an electronic microscope structural analysis at SEM of the enamel tooth surface was conducted to identify any characteristic difference in the enamel architecture of this GSD type Ib affected patient compared with the normal enamel structure. CONCLUSION The oral manifestations of our case included uncommon findings such as multiple deep oral ulcers of the tongue, labial and vestibular mucosa, which could be related with a severe impairment of the neutrophil-related immune system of the patient. At SEM, the enamel of the deciduous teeth showed dark spots of hypomineralisation which are the first published data of an enamel-related susceptibility to dental caries. The quality of life of our patient increased thanks to the oral and dental treatments and preventive oral health measures given at clinical appointments. The dentist should be prepared to face the possible complications of surgery in these patients and the hospital setting seems to allow for this clinical safety.
The underside of the cerebral cortex: layer V/VI spiny inverted neurons

This paper presents an account of past and current research on spiny inverted neurons – alternatively also known as ‘inverted pyramidal neurons’– in rats, rabbits and cats. In our laboratory, we have studied these cells with a battery of techniques suited for light and electron microscopy, including Nissl staining, Golgi impregnation, dye intracellular filling and axon retrograde track‐tracing. Our results show that spiny inverted neurons make up less than 8.5 and 5.5% of all cortical neurons in the primary and secondary rabbit visual cortex, respectively. Infragranular spiny inverted neurons constitute 15 and 8.5% of infragranular neurons in the same animal and areas. Spiny inverted neurons congregate at layers V–VI in all studied species. Studies have also revealed that spiny inverted neurons are excitatory neurons which furnish axons for various cortico‐cortical, cortico‐claustral and cortico‐striatal projections, but not for non‐telencephalic centres such as the lateral and medial geniculate nuclei, the colliculi or the pons. As a group, each subset of inverted cells contributing to a given projection is located below the pyramidal neurons whose axons furnish the same centre. Spiny inverted neurons are particularly conspicuous as a source of the backward cortico‐cortical projection to primary visual cortex and from this to the claustrum. Indeed, they constitute up to 82% of the infragranular cells that furnish these projections. Spiny inverted neurons may be classified into three subtypes according to the point of origin of the axon on the cell: the somatic basal pole which faces the cortical outer surface, the somatic flank and the reverse apical dendrite. As seen with electron microscopy, the axon initial segments of these subtypes are distinct from one another, not only in length and thickness, but also in the number of received synaptic boutons. All of these anatomical features together may support a synaptic‐input integration which is peculiar to spiny inverted neurons. In this way, two differently qualified streams of axonal output may coexist in a projection which arises from a particular infragranular point within a given cortical area; one stream would be furnished by the typical pyramidal neurons, whereas spiny inverted neurons would constitute the other source of distinct information flow.
Longitudinal modes in classical Yang-Mills plasma.

A classical nonperturbative study of the longitudinal modes in a Yang-Mills [SU(2)] plasma is carried out. A new periodic non-Abelian mode is found which alternates with the usual Abelian plasma mode. When the non-Abelian terms in the equations become large, chaotic behavior sets in.
Domains of health-related quality of life important to patients with giant cell arteritis.

OBJECTIVE To determine aspects of quality of life (QOL) important to people with giant cell arteritis (GCA). METHODS We explored the domains of QOL affected by GCA in audiotaped focus groups. We then created an Importance Rating Questionnaire (IRQ) by constructing questions related to the domains most frequently mentioned. Of 214 GCA patients to whom the IRQ was sent, 145 (68%) responded. We calculated frequencies of responses and then ranked items by the proportion selecting the top category of importance and also according to a mean item rank. We compared the domains of QOL covered by the IRQ with those in the Short Form 12 (SF-12). RESULTS The highest rated QOL item was "losing sight in both eyes permanently. " Of the top 20 items, 12 were in domains not covered directly by the SF-12. CONCLUSION We have identified aspects of QOL important to GCA patients. Assessment of QOL in GCA should include vision and other domains that are not included in standard QOL questionnaires.
A Longitudinal Study of the Impact of Childhood and Adolescent Physical Activity Experiences on Adult Physical Activity Perceptions and Behaviors

The authors describe the influence of childhood and adolescent physical activity on adult physical activity attitudes and behaviors. They conducted one-on-one, semistructured, in-depth interviews with 16 men and 15 women from a longitudinal growth and development study and a follow-up investigation 25 years later. They used thematic analyses to analyze the data. Three themes emerged from the men's interviews: significant others, size and maturation, and physical ability, with clear distinctions among the active, average, and inactive men. The themes transitions, body image concerns, and significant others influenced the active, average, and inactive women. Specific relationships, circumstances, and attitudes formed in childhood and adolescence influenced adult physical activity predilection and behaviors.
Pursuing “model care” of transient ischemic attacks

It is never good to be a “high-risk” patient. For health care systems, however, high-risk patient populations provide high-value opportunities to improve outcomes. Patients with TIA represent such a targetable high-risk group. The high short-term risk of stroke after TIA and substantial proportion of strokes preceded by TIA1 imply that focusing on risk reduction in these patients can meaningfully reduce stroke incidence. The prevalence of systems designed to reduce stroke risk after TIA reflects the importance of this opportunity. In this issue of Neurology®, Ranta and Barber2 present a narrative review of 7 such systems. These models originate from 5 different countries and differ considerably in their design and implementation.
Migrant Workers and Labor Market Segmentation in Japan

Segmentation of the migrant work force in Japan is determined largely by institutional factors put in place by the 1990 Immigration Law Reform. In contrast to conventional discussions which compare migrant workers with native workers, this discussion focuses on the segmentation of the labor market among migrant workers themselves. These workers form an ethnically diverse mass in the Japanese labor market today. They are diverse not only in the time of arrival and other characteristics but also in terms of treatment under Japan's immigration control practice. This discussion describes the nature of labor market segmentation among migrant workers and examines the reasons for differentiated working conditions.
TECHNIQUES FOR DIAGNOSING OSTEOPOROSIS: A SYSTEMATIC REVIEW OF COST-EFFECTIVENESS STUDIES

Objectives: The study question was whether dual-energy X-ray absorptiometry (DXA) alone is more cost-effective for identifying postmenopausal women with osteoporosis than a two-step procedure with quantitative ultrasound sonography (QUS) plus DXA. To answer this question, a systematic review was performed. Methods: Electronic databases (PubMed, INAHTA, Health Evidence Network, NIHR, the Health Technology Assessment program, the NHS Economic Evaluation Database, Research Papers in Economics, Web of Science, Scopus, and EconLit) were searched for cost-effectiveness publications. Two independent reviewers selected eligible publications based on the inclusion/exclusion criteria. Quality assessment of economic evaluations was undertaken using the Drummond checklist. Results: Seven journal articles and four reports were reviewed. The cost per true positive case diagnosed by DXA was found to be higher than that for diagnosis by QUS+DXA in two articles. In one article it was found to be lower. In three studies, the results were not conclusive. These articles were characterized by the differences in the types of devices, parameters and thresholds on the QUS and DXA tests and the unit costs of the DXA and QUS tests as well as by variability in the sensitivity and specificity of the techniques and the prevalence of osteoporosis. Conclusions: The publications reviewed did not provide clear-cut evidence for drawing conclusions about which screening test may be more cost-effective for identifying postmenopausal women with osteoporosis.
Fludarabine and cyclophosphamide using an attenuated dose schedule is a highly effective regimen for patients with indolent lymphoid malignancies

Preclinical data have supported the use of fludarabine and cyclophosphamide (FC) in combination for the treatment of indolent lymphoid malignancies. Previously reported schedules were highly effective, but were complicated by significant myelotoxicity and infectious complications. In the current study, the authors analyzed their experience with an attenuated dose regimen to determine whether equivalent efficacy could be achieved with reduced toxicity.
Sound and Light Levels Are Similarly Disruptive in ICU and non‐ICU Wards

BACKGROUND: Hospitalized patients frequently report poor sleep, partly due to the inpatient environment. In‐hospital sound and light levels are not well described on non‐intensive care unit (non‐ICU) wards. Although non‐ICU wards may have lower average and peak noise levels, sound level changes (SLCs), which are important in disrupting sleep, may still be a substantial problem. OBJECTIVE: To compare ambient sound and light levels, including SLCs, in ICU and non‐ICU environments. DESIGN: Observational study. SETTING: Tertiary‐care hospital. MEASUREMENTS: Sound measurements of 0.5 Hz were analyzed to provide average hourly sound levels, sound peaks, and SLCs ≥17.5 decibels (dB). For light data, measurements taken at 2‐minute intervals provided average and maximum light levels. RESULTS: The ICU rooms were louder than non‐ICU wards; hourly averages ranged from 56.1 ± 1.3 dB to 60.3 ± 1.7 dB in the ICU, 47.3 ± 3.7 dB to 55.1 ± 3.7 dB on the telemetry floor, and 44.6 ± 2.1 dB to 53.7 ± 3.6 dB on the general ward. However, SLCs ≥ 17.5 dB were not statistically different (ICU, 203.9 ± 28.8 times; non‐ICU, 270.9 ± 39.5; P = 0.11). In both ICU and non‐ICU wards, average daytime light levels were <250 lux, and peak light levels occurred in the afternoon and early evening. CONCLUSIONS: Quieter, non‐ICU wards have as many SLCs as ICUs do, which has implications for quality improvement measurements. Efforts to further reduce average noise levels might be counterproductive. Light levels in the hospital (ICU and non‐ICU) may not be optimal for maintenance of a normal circadian rhythm for most people.
What Drives Conversion of Tropical Forest in Carrasco Province, Bolivia?

Abstract The conversion of closed forest (CCF) in Carrasco Province, Bolivia, was monitored using a series of four midresolution satellite images from 1986 to 2002. The conversion of forests into nonforests from 1986 to 2002 was 1.5% annually. Inclusion of conversions into open forest doubles the annual CCF rate to 3.1%. Five predictors of CCF were tested in a spatial model: land tenure regime, distance from roads, distance from settlements, topography, and soil suitability for farming. Only three out of the five predictors tested were found to be reliable predictors of CCF: land tenure regime, distance from roads, and distance from settlements. University reserve and indigenous land show substantially less CCF than national park and untitled land. In addition the spatial model shows that the greater the distance of forest from roads or settlements, the less CCF. Topography and soil suitability for farming lack predictive power for CCF and are therefore excluded from the spatial model.
Extravascular lung water in patients with congestive heart failure. Difference between patients with acute and chronic myocardial disease.

Extravascular lung water was assessed using a double-indicator dilution technique (thermal-green dye) in 15 men who had radiographic and clinical evidence of cardiogenic interstitial and alveolar pulmonary edema. Eight patients had suffered an acute myocardial infarction and seven had a history of chronic congestive cardiomyopathy and worsening of previous clinical signs and symptoms. At the same level of pulmonary capillary wedge pressure and similar arterial oxygen tensions extravascular lung water was significantly greater in the group with chronic congestive cardiomyopathy (p less than .05). The two groups did not differ with respect to systemic arterial pressure, resistance, or cardiac index.
Molecular Dynamics and Docking Studies on Cardiac Troponin C

Abstract Cardiac troponin C (cTnC) is the Ca2+ dependent switch for contraction in heart muscle making it a potential target for drug research in the therapy of heart failure. Calcium binding on Troponin C (TnC) triggers a series of conformational changes exposing a hydrophobic pocket in the N-domain of TnC (cNTnC), which leads to force generation. Mutations and acidic pH have been related to altering the sensitivity of TnC affecting the efficiency of the heart. Bepridil, identified as a calcium sensitizer to TnC, has been experimentally found to bind to the N-domain pocket of TnC but with negative cooperativity. Screening and de novo design were carried out using LUDI and AUTOLUDI programs in this work to identify and design potential ligands that can bind to the hydrophobic pocket of TnC. Two docking centers and multiple searching radii including 5Å, 5. 5Å, 6Å, 6. 5Å, 7. 0Å and 7. 5Å were used in LUDI to screen the ZINC database. Based on the LUDI docking results, 8 molecules were identified from the database with good potential to bind into the binding pocket and they were used as template molecules to generate a series of new molecules by AUTOLUDI design. Out of all the newly-designed molecules, 14 new ligands were recognized to be potential ligands that can bind and fit well into the binding pocket. These molecules can be used as starting molecules to develop TnC ligands. The binding stability and binding affinity of these molecules to the protein was further analyzed by molecular dynamics simulations. The results show that the binding energies, interactions and complex stabilities of 6 ligands are comparable to or better than bepridil.
Oral contraceptive use and glucose metabolism in a national sample of women in the united states.

OBJECTIVE This study was undertaken to determine whether users of oral contraceptives in a nationally representative population of US women had elevated levels of measures of glucose metabolism. STUDY DESIGN Cross-sectional data from the Third National Health and Nutrition Examination Survey (1988-1994) included hemoglobin A(1c) levels and fasting glucose, insulin, and C-peptide levels. Means were compared among those who had never used oral contraceptives, current users of oral contraceptives, and former users of oral contraceptives, with and without adjustment for potential confounders. RESULTS The vast majority of current users of oral contraceptives were using low-dose estrogen formulations. The two most common preparations were a triphasic formulation containing 0. 035 mg ethinyl estradiol and 0.5, 0.75, and 1 mg norethindrone (23. 9%) and a monophasic formulation containing 0.035 ethinyl estradiol and 1 mg norethindrone (20.7%). Current users of oral contraceptives did not have elevated values for any of the four measures of glucose metabolism. Hemoglobin A(1c) level and fasting glucose, insulin, and C-peptide levels were not related to duration of current use, age at which use began, or major formulation type. Among women who were former users of oral contraceptives there was no evidence of higher values among those who had recently ceased use. CONCLUSION Oral contraceptive formulations currently available in the United States are not associated with an adverse glucose metabolic profile.
Posttranscriptional mechanisms account for differences in steady state levels of insulin receptor messenger RNA in different cells.

Previous studies have indicated that, in general, the insulin receptor gene is expressed at a level in cells reflecting the level of insulin receptors on the cellular surface. For instance, insulin-responsive tissues, such as hepatocytes, express high levels of both insulin receptor protein and mRNA relative to less responsive cells, such as fibroblasts. Moreover, in the cells of a patient (Minn1) with severe insulin resistance and very low levels of insulin receptors, it has been shown that insulin receptor gene transcripts are virtually undetectable. Our earlier studies of the insulin receptor gene promoter suggested that the differences in the steady state level of insulin receptor gene transcripts in different cells could be transcriptionally mediated. In this study we have attempted to assess the relative contribution of transcriptional and posttranscriptional mechanisms in determining steady state levels of insulin receptor mRNA in various cells, including Minn 1 fibroblasts. Using nuclear run-on assays, we have determined that the level of nascent insulin receptor gene transcripts is roughly equal in different cells, including Minn1 fibroblasts. Therefore, transcriptional differences do not seem to account for the dramatic differences in steady state levels of insulin receptor mRNA in different cells, and there is no evidence in support of a transcriptional defect in Minn1's insulin receptor gene alleles. However, the rate of insulin receptor mRNA turnover varies significantly in different cells, ranging from an mRNA half-life of as little as 2 h in fibroblast and IM9 (lymphocytic) cells up to 8 h in HepG2 (liver) cells, and accounts in part for the observed differences in steady state insulin receptor mRNA. (ABSTRACT TRUNCATED AT 250 WORDS)
Survival of Patients with Dementia

The evidence on survival in dementia is summarized. There are no reliable data on survival after onset of dementia or after first contact with medical services. People with dementia in outpatient clinics and nursing homes have 2‐year survival rates of 75% (range 60%‐95%) and 50% (range 30%‐65%), respectively. Differences in survival between patients with senile dementia of the Alzheimer's type (SDAT) and multi infarct dementia (MID) are small. Women in nursing homes have a better prognosis than men (2‐year survival rates, 60% vs 40%). Dementia patients have a considerable excess mortality when compared to the vital statistics. There is no evidence for improvement of survival rates during recent decades. Recommendations for future studies are made
Soccer training programme improved the body composition of pre‐adolescent boys and increased their satisfaction with their body image

It is recognised that physical activity improves nutritional status and body image perception and reduces the risk of disease and body dissatisfaction (1-2). Although the relationship between physical activity and body composition and body image perception have been extensively investigated in adults, few studies on the effects of specific sporting activities on children or adolescents have so far been reported and those that have been carried out have reported contradictory results (1). Our study had two aims. Firstly we wanted to gain a better understanding of the body composition and body image perception of 60 pre-adolescent boys by carrying out an evaluation of their anthropometric characteristics and body image perception before and after a 12-week soccer (football) training programme. Secondly we were keen to analyse any possible differences between age groups by looking at 24 boys of nine years of age and 36 boys of 10 years of age. All the children who attended two soccer schools for four hours per week volunteered to participate in the study. They were all born in the province of Ferrara in Northern Italy. Written, informed consent was obtained from their parents and the study was approved by the Ethics Committee of Ferrara University. This article is protected by copyright. All rights reserved.This article is protected by copyright. All rights reserved. Language: en
Voxel based morphometry reveals specific gray matter changes in primary dystonia

The present study assessed patterns of brain tissue alterations in different types of primary dystonia using voxel‐based morphometry (VBM). Nine patients with primary generalized dystonia (GD), 11 patients with primary cervical dystonia (CD), and 11 patients with primary focal hand dystonia (FHD) as well as 31 age and gender‐matched controls were included. When compared with healthy controls, patients with primary dystonia (n = 31) showed gray matter volume increase bilaterally in the globus pallidus internus, nucleus accumbens, prefrontal cortex, as well as unilaterally in the left inferior parietal lobe. This is the first study using VBM in patients with different types of primary dystonia, showing a common pattern of gray matter changes. © 2007 Movement Disorder Society
On a collection of fishes made by Mr. Alan Owston in the deep waters of Japan

The writers have recently received from Mr. Alan Owston, of Yokohama, well known as a shipmaster and as a collector in natural history, a very remarkable series of fishes obtained on long lines in the depths of Sagami bay and Siiruga bay, Japan. The present paper contains notes on these species, together with field notes of Mr. Owston. The specimen of Mitsukurina otvstoni and some of the others have been sent to the United States National Museum. The rest are in the collection of Leland Stanford Junior University.