Miracle-dz/UniFAR-FTUs huggingface dataset:
Dilated cardiomyopathy in childhood: prognostic features and outcome

The natural history of dilated cardiomyopathy in children is difficult to predict due to the heterogeneous character of the disease. The outcome in infants and children is highly variable from complete recovery to death. In this study, 40 children diagnosed with dilated cardiomyopathy between 1995 and 2004 in our paediatric cardiology unit were reviewed with respect to clinical course and outcome, retrospectively. The medical history of these patients with dilated cardiomyopathy was reviewed to determine age, gender, family history, preceding viral illness, duration of symptoms before the diagnosis, symptoms and signs at presentation, treatment and outcome. The diagnosis was made on the basis of cardiomegaly and evidence of poor left ventricular function by echocardiography. Median age at diagnosis was 14 months, ranging from 2 months to 8 years. At presentation, 28 patients (70%) were under and twelve (30%) were above the age of two years.Twenty-eight (70%) patients had signs of congestive heart failure. Mean duration of follow-up was 40 ± 24 months (ranging from 6 months to 9 years), 21 patients (52.5%) recovered, 17 patients (42.5%) had residual disease and two (5%) died. The cause of death in both patients was progressive cardiac failure. Sixteen of 28 patients (57%) who were below the age of two years and five of 12 patients (42%) who were above the age of two years at presentation recovered.The rate of recovery was significantly different between the two age groups (p < 0.05). Seventeen of 21 (81%) patients with a history of recent viral illness at presentation recovered. The mean duration of the disease among those who recovered was 11 ± 8.3 months. Five of 19 (26%) patients without recent viral illness recovered.The mean duration of the disease in this group was 22 ± 12 months.There was a significant difference between the two groups with respect to recovery and recovery time (p < 0.05). During the first 6 months after diagnosis, there was a significant difference between the patients who recovered and the patients who had residual disease with respect to improvement in the left ventricular FS (22 ± 3.5%, 15.2 ± 2.8%, respectively) (p < 0.05). In conclusion, in this study, the rate of recovery and survival is higher than in previous studies. A good outcome is related to age at presentation (≤ two years old), a history of viral disease within three months of presentation and improvement in ventricular function during the first 6 months after diagnosis. Intractable heart failure has an adverse effect on the outcome.
Functional and anatomic consequences of subretinal dosing in the cynomolgus macaque.

OBJECTIVE To characterize functional and anatomic sequelae of a bleb induced by subretinal injection. METHODS Subretinal injections (100 μL) of balanced salt solution were placed in the superotemporal macula of 1 eye in 3 cynomolgus macaques. Fellow eyes received intravitreal injections (100 μL) of balanced salt solution. Fundus photography, ocular coherence tomography, and multifocal electroretinography were performed before and immediately after injection and again at intervals up to 3 months postinjection. Histopathologic analyses included transmission electron microscopy and immunohistochemistry for glial fibrillary acidic protein, rhodopsin, M/L-cone opsin, and S-cone opsin. RESULTS Retinas were reattached by 2 days postinjection (seen by ocular coherence tomography). Multifocal electroretinography waveforms were suppressed post-subretinal injection within the subretinal injection bleb and, surprisingly, also in regions far peripheral to this area. Multifocal electroretinography amplitudes were nearly completely recovered by 90 days. The spectral-domain ocular coherence tomography inner segment-outer segment line had decreased reflectivity at 92 days. Glial fibrillary acidic protein and S-cone opsin staining were unaffected. Rhodopsin and M/L-cone opsins were partially displaced into the inner segments. Transmission electron microscopy revealed disorganization of the outer segment rod (but not cone) discs. At all postinjection intervals, eyes with intravitreal injection were similar to baseline. CONCLUSIONS Subretinal injection is a promising route for drug delivery to the eye. Three months post-subretinal injection, retinal function was nearly recovered, although reorganization of the outer segment rod disc remained disrupted. Understanding the functional and anatomic effects of subretinal injection is important for interpretation of the effects of compounds delivered to the subretinal space. CLINICAL RELEVANCE Subretinal injection is a new potential route for drug delivery to the eye. Separating drug effects from the procedural effects is critical.
Spontaneous and posed emotional facial expressions following severe traumatic brain injury

Aim: The current study aimed to test the intensity of spontaneous emotional expressions and the accuracy of posed emotional expressions in patients with severe traumatic brain injury (TBI). Method: Twenty-three participants with TBI and 27 matched control participants were asked to relate personal angry, happy, and sad events (spontaneous expressivity) and to pose angry, happy, and sad expressions in response to a photo or word cue (posed expressivity). Their emotional facial expressions were coded via judges' ratings. Results: Participants with TBI had less intense sad expressions when relating a sad event than did control participants. No group difference emerged in the happy and angry events, the latter possibly due to differentially low interrater reliability for anger ratings. Participants with TBI were impaired in their ability to pose sad emotions. Conclusions: These preliminary findings suggest that patients with TBI are impaired at expressing sad expressions either spontaneously or deliberately. This may reflect difficulties in the initiation or suppression of facial expression as well as an impaired semantic knowledge of the facial configuration of sad expression.
Vitamin A status among children in China

Abstract Objective The objective of this study was to assess the prevalence of vitamin A deficiency (VAD) in children under 6 years old in China and to identify risk groups for VAD. Design A cross-sectional survey was conducted in 14 provinces from coastal, inland and western geographic areas in China. Setting One city (urban) and two counties (rural) were randomly selected from each province as survey areas. Subjects About 200 children aged 0–6 years were randomly selected in each survey area. A blood sample was collected from each child. Data on sociodemographics and nutrition were obtained by interview of the mother or principal caregiver. Fluorescence microanalysis was used to analyse serum retinol concentration. Results VAD (serum retinol < 0.7 μmol l− 1) was observed in 957 out of 7826 children aged 0–6 years (12.2% of the entire study population), whereas severe VAD (serum retinol < 0.35 μmol l− 1) was found in 39 children (0.5%). The highest prevalences of VAD at >1 year of age were observed among children of mothers with minority ethnicity (22.7%) or poor education (19.8%) and in the poor western area (17.4%). Conclusions VAD is a nutritional problem in children in China. Children living in the poor western area, having a mother with minority ethnicity or a mother with poor education have a high risk of VAD.
Mouse vs human prostate : the realities of comparative anatomy Anatomy and histology of the prostate

This review is focused on mouse models for prostate cancer that have been designed on the basis of genetic alterations that are frequently found in human prostate cancer. It begins with an analysis of the similarities and differences in the gross and microscopic anatomy of the mouse and human prostate glands, and extends to the pathologies induced in the genetically manipulated mouse prostate in comparison with the sporadic development of the disease in humans. Major achievements have been made in modeling human prostate cancer in mice in recent years. There are models which display slow, temporal development of increasingly severe preneoplastic lesions, which are remarkably restricted to the prostate gland, a property similar to the aging-related progression of these lesions in humans. Other models rapidly progress to local invasive adenocarcinoma, and, in some of them metastasis is manifested subsequently with defined kinetics. Global assessment of molecular changes in the prostate of the genetically manipulated mice is increasingly underscoring the validity of the models through identification of ‘signature’ genes which are associated with the organ-confined primary or distant metastases of human prostate cancer. Taken together, various ‘natural’ models depicting stages of the disease, ranging from the early preneoplastic lesions to metastatic prostate cancer, now provide new tools both for exploring the molecular mechanism underlying prostate cancer and for development or testing of new targeted therapies. Endocrine-Related Cancer (2004) 11 225–254
A mouse model of basal‐like breast carcinoma with metaplastic elements

Breast cancers arising in carriers of germline BRCA1 mutations frequently have a basal‐like phenotype. Basal‐like cancers are characterized by high histological grade, central necrotic areas, foci with metaplastic differentiation, lack of hormone receptor and HER2 (ErbB2) expression, and consistent positivity for basal markers, including CK5/6, CK14, and EGFR. We have used germline manipulation to generate a conditional mouse model of Brca1 deficiency. Transgenic expression of Cre recombinase in the mammary gland of these mice results in deletion of exons encoding the C‐terminus of Brca1 and leads to tumour formation when combined with heterozygosity for a p53 mutation. Histologically, these mammary gland tumours were characterized by high histological grade, central necrotic areas, and presence of homologous metaplastic elements. These metaplastic elements consisted of neoplastic spindle cells or squamous cell differentiation in the form of keratin pearls or individual cell keratinization. Immunohistochemical analysis revealed expression of basal‐like markers in all cases. The tumour phenotype generated in our mouse model was compared with published data on human basal‐like breast carcinomas and also with metaplastic breast cancers with a basal‐like phenotype; the comparison showed that we have generated a mouse model of basal‐like breast cancer, which should prove useful in testing new and targeted treatments for this type of breast cancer. Copyright © 2007 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.
Long-term anti-tumor necrosis factor antibody therapy in rheumatoid arthritis patients sensitizes the pituitary gland and favors adrenal androgen secretion.

OBJECTIVE New insights into the role of tumor necrosis factor (TNF) in the pathogenesis of rheumatoid arthritis (RA) have expanded our understanding about the possible mechanisms by which anti-TNF antibody therapy reduces local synovial inflammation. Beyond local effects, anti-TNF treatment may modulate systemic antiinflammatory pathways such as the hypothalamic-pituitary-adrenal (HPA) axis. This longitudinal anti-TNF therapy study was designed to assess these effects in RA patients. METHODS RA patients were given 5 infusions of anti-TNF at weeks 0, 2, 6, 10, and 14, with followup observation until week 16. We measured serum levels of interleukin-6 (IL-6), adrenocorticotropic hormone (ACTH), 17-hydroxyprogesterone (17[OH]progesterone), cortisol, cortisone, androstenedione (ASD), dehydroepiandrosterone (DHEA), and DHEA sulfate in 19 RA patients. RESULTS Upon treatment with anti-TNF, we observed a fast decrease in the levels of serum IL-6, particularly in RA patients who did not receive parallel prednisolone treatment (P = 0.043). In these RA patients who had not received prednisolone, the mean serum ACTH levels sharply increased after every injection of anti-TNF, which indicates a sensitization of the pituitary gland (not observed for the adrenal gland). During treatment, the ratio of serum cortisol to serum ACTH decreased, which also indicates a sensitization of the pituitary gland (P < 0.001), and which was paralleled by constant cortisol secretion. The adrenal androgen ASD significantly increased relative to its precursor 17(OH)progesterone (P = 0.013) and relative to cortisol (P = 0.009), which indicates a normalization of adrenal androgen production. The comparison of patients previously treated with prednisolone and those without previous prednisolone revealed marked differences in the central and adrenal level of this endocrine axis during long-term anti-TNF therapy. CONCLUSION Long-term therapy with anti-TNF sensitizes the pituitary gland and improves adrenal androgen secretion in patients who have not previously received prednisolone treatment. These changes are indicative of normalization of the HPA axis and must therefore be considered as evidence of an additional antiinflammatory influence of anti-TNF treatment in patients with RA.
Effects of ovarian steroid hormones on histochemically demonstrable lipids in the rat uterine epithelium.

Neutral lipids unsaturated lipids phospholipids and fatty acids were investigated histochemically in the rat uterine epithelium during the estrus cycle under a range of experimental conditions. During the normal estrus cycle neutral fats were the dominant lipids in the uterine epithelium reaching their maximum at diestrus and proestrus before declining to the low levels of metestrus. Distribution patterns of unsaturated lipids paralleled that of the neutral lipids. In ovariectomized mature rats given 17-beta- estradiel either by itself or in combination with medroxyprogesterone acetate neutral lipids and unsaturated lipids were below control levels while phospholipids were increased. Medroxyprogesterone acetate however increased levels of neutral lipids and unsaturated lipids and decreased phospholipids. It is suggested that during the estrus cycle of the rat high concentrations of uterine epithelial lipid during late diestrus and proestrus reflect the increased plasma progesterone of early diestrus acting in a low plasma estrogen environment.
Why the Quiet Revolution Was "Quiet": The Catholic Church's Reaction to the Secularization of Nationalism in Quebec after 1960

québécois contemporain, de 1940 à nos jours,” dans La croix et le nouveau monde. Histoire religieuse des francophones d’Amérique du nord, dir. Guy-Marie Oury, 217-36, (Montréal: Éditions C.L.D./ C.M.D., 1987), p. 224. Despite these impressive figures, the Church was already in the midst of a crisis in vocations, organization, and morale. See Jean Hamelin, Histoire du catholicisme québécois. Le XXe siècle. Tome 2. De 1940 à nos jours, (Montréal: Boréal Express, 1984), pp. 160-91.
Numerical Simulation of Transient Response in 3-D Multi-Channel Nanowire MOSFETs Submitted to Heavy Ion Irradiation

The bulk MOSFET scaling has recently encountered significant limitations, mainly related to the gate oxide (SiO2) leakage currents (Gusev et al., 2006; Taur et al., 1997), the large increase of parasitic short channel effects and the dramatic mobility reduction (Fischetti & Laux, 2001) due to highly doped Silicon substrates precisely used to reduce these short channel effects. Technological solutions have been proposed in order to continue to use the “bulk solution” until the 32 nm ITRS node (ITRS, 2009). Most of these solutions envisage the introduction of high-permittivity gate dielectric stacks (to reduce the gate leakage, (Gusev et al., 2006; Houssa, 2004), midgap metal gate (to suppress the Silicon gate polydepletioninduced parasitic capacitances) and strained Silicon channel (to increase carrier mobility (Rim et al., 1998). However, in parallel to these efforts, alternative solutions to replace the conventional bulk MOSFET architecture have been proposed and studied in the recent literature. One solution is the radical change of the device architecture such as in MultipleGate devices introducing additional gate electrodes: 2 (double-gate), 3 (FinFET or trigate) or 4 (gate-all-around, completely surrounding the channel). Silicon nanowires MOSFETs with gate-all-around (GAA) provide an original and very promising architecture to further increase the integration density and performances of nano-devices (Park & Colinge, 2002). These structures exhibit a superior control of short channel effects resulting from an exceptional electrostatic coupling between the conduction channel and the surrounding gate electrode. As a result, intrinsic channels can be used leading to higher mobilities and drain currents. 3D Multi-Channel MOSFETs (MCFETs) have been recently proposed to achieve a higher current drivability and a significant enhancement of the on-state current over the off-state current ratio (ION/IOFF) (Bernard et al., 2007; Ernst et al., 2006) compared to conventional single channel devices. MCFETs combine the advantages of excellent control of shortchannel effects with a high on-state current due to a multiple-gate architecture and the 3-D integration of vertically stacked channels. GAA devices with ultra-thin and narrow channels (about 10 nm) are seen as the ideal architecture for off-state current control of sub-10 nm gate lengths (Ernst et al., 2006). Meanwhile, the current density per surface of such a device is limited by the lithography pitch, which dictates the distance between nanowires. The
Ovarian steroids, stem cells and uterine leiomyoma: therapeutic implications.

BACKGROUND Uterine leiomyoma is the most common benign tumor in women and is thought to arise from the clonal expansion of a single myometrial smooth muscle cell transformed by a cellular insult. Leiomyomas cause a variety of symptoms, including abnormal uterine bleeding, pelvic pain, bladder or bowel dysfunction, and recurrent pregnancy loss, and are the most common indication for hysterectomy in the USA. A slow rate of cell proliferation, combined with the production of copious amounts of extracellular matrix, accounts for tumor expansion. A common salient feature of leiomyomas is their responsiveness to steroid hormones, thus providing an opportunity for intervention. METHODS A comprehensive search of PUBMED was conducted to identify peer-reviewed literature published since 1980 pertinent to the roles of steroid hormones and somatic stem cells in leiomyoma, including literature on therapeutics that target steroid hormone action in leiomyoma. Reviewed articles were restricted to English language only. Studies in both animals and humans were reviewed for the manuscript. RESULTS Estrogen stimulates the growth of leiomyomas, which are exposed to this hormone not only through ovarian steroidogenesis, but also through local conversion of androgens by aromatase within the tumors themselves. The primary action of estrogen, together with its receptor estrogen receptor α (ERα), is likely mediated via induction of progesterone receptor (PR) expression, thereby allowing leiomyoma responsiveness to progesterone. Progesterone has been shown to stimulate the growth of leiomyoma through a set of key genes that regulate both apoptosis and proliferation. Given these findings, aromatase inhibitors and antiprogestins have been developed for the treatment of leiomyoma, but neither treatment results in complete regression of leiomyoma, and tumors recur after treatment is stopped. Recently, distinct cell populations were discovered in leiomyomas; a small population showed stem-progenitor cell properties, and was found to be essential for ovarian steroid-dependent growth of leiomyomas. Interestingly, these stem-progenitor cells were deficient in ERα and PR and instead relied on the strikingly higher levels of these receptors in surrounding differentiated cells to mediate estrogen and progesterone action via paracrine signaling. CONCLUSIONS It has been well established that estrogen and progesterone are involved in the proliferation and maintenance of uterine leiomyoma, and the majority of medical treatments currently available for leiomyoma work by inhibiting steroid hormone production or action. A pitfall of these therapeutics is that they decrease leiomyoma size, but do not completely eradicate them, and tumors tend to regrow once treatment is stopped. The recent discovery of stem cells and their paracrine interactions with more differentiated cell populations within leiomyoma has the potential to provide the missing link between developing therapeutics that temper leiomyoma growth and those that eradicate them.
Uptake of amino acids by the marine centric diatom Cyclotella cryptica.

The transport systems for all of the amino acids studied, with the exception of isoleucine, obey saturation kinetics. The strong inhibition of the process by cyanide and dinitrophenol provides evidence that energy is required. Additional evidence for the requirement of energy for amino acid transport is the strong temperature dependence (Q10 about 2) and the high concentration ratios measured for transported amino acids.Transport parameters (Ks and Vmax) for several amino acids were determined from data for two different concentration ranges. The results of the kinetic studies indicate the presence of two or more transport systems that become saturated at different substrate concentrations for each of the amino acids. The presence of a high affinity – high capacity transport system for arginine indicates that this amino acid may be utilized efficiently by the diatom when present at low concentrations in the natural environment.Interactions between pairs of amino acids suggest that several transport system.
A Simple Procedure for the Radioimmunoassay of 17α-Hydroxyprogesterone in Serum: Comparison with an Immunological Purification Technique

A simple radioimmunoassay for the measurement of 17α-hydroxyprogesterone in 0·1–0·25 ml serum is described. An antibody prepared against 17α-hydroxyprogesterone 3(O-carboxymethyl)-oxime-BSA as immunogen was used. A correlation of the 17α-hydroxyprogesterone concentration found in the serum of normal subjects and of patients with that obtained by a more complex immunological purification method was good (r = 0·924, p < 0·001). Accuracy, precision, and specificity were acceptable with the simple method. Normal values for infants, children, and adults have been established, and the individual concentrations of 17α-hydroxyprogesterone in the serum of patients with congenital adrenal hyperplasia were well separated from normal values.