Miracle-dz/UniFAR-FTUs huggingface dataset:
Incipient CADASIL.

BACKGROUND Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is caused by mutations in the NOTCH3 gene. Knowledge of disease expression in young adult NOTCH3 mutation carriers (MCs) is limited. OBJECTIVE To characterize clinical, neuropsychological, and radiological status in NOTCH3 MCs younger than 35 years. DESIGN Clinical characterization and blinded survey comparing MCs with non-MCs. SETTING Referral center. PARTICIPANTS Individuals younger than 35 years who were at a 50% risk of a NOTCH3 mutation, from our CADASIL database. Thirteen individuals, from 8 families, met the criteria. METHODS Comprehensive clinical, genetic, neuropsychological, and radiological investigations. Magnetic resonance images were scored according to a standardized white matter hyperintensities rating scale. RESULTS Six individuals, from 5 families, were MCs. Clinical symptoms consisted of migraine (with aura), stroke, and stroke-like episodes. We did not find evidence for psychiatric disturbances, functional disability, or cognitive dysfunction, compared with non-MCs. Radiologically, a characteristic magnetic resonance imaging lesion pattern emerged for all MCs. This comprised white matter hyperintensities in the anterior temporal lobes, the frontal lobes, and the periventricular frontal caps. CONCLUSIONS Migraine (with aura) and stroke can present in NOTCH3 MCs younger than 35 years; however, more importantly, physical function and cognition are intact. Possible subtle cognitive dysfunction needs to be assessed in a larger study. White matter hyperintensities on magnetic resonance imaging are characteristic, and are consistently visualized from the age of 21 years and onward. Awareness of the clinical and radiological features of CADASIL in those younger than 35 years should increase early diagnosis and allow for customized counseling of young adults from families with CADASIL.
Knowledge About Schizophrenia and Attitudes Towards People with Schizophrenia in Greece

Background: This study was the first national survey ever conducted in Greece aiming to monitor the level of stigma Greeks hold against people with schizophrenia (PWS). Aim: To investigate sources, degree of knowledge and attitudes towards PWS in Greece. Method: A cross-sectional nationwide survey was conducted by face-to-face household interviews ( n = 1,199, aged 15 years and over). Results: Television was the main source of information (65.9%). Negative portrayals of PWS were recalled by 60.5%. Only 27.7% attributed schizophrenia to a combination of psychosocial, genetic and environmental factors. Respondents believed that PWS are dangerous (74.6%), have split personalities (81.3%) and cannot work (83.2%). Regarding attitudes, a negative relationship between closeness and social distance was observed. Most respondents (92.1%) would not marry someone with schizophrenia, half (50.5%) would be disturbed by working with PWS and one third (32.9%) would feel afraid to start a conversation. Urban residence and higher education were generally associated with better knowledge and more positive attitudes towards PWS. Conclusions: Knowledge about schizophrenia in Greece is poor. The Greek public has stigmatizing attitudes towards PWS. Educational interventions should especially target rural and semi-urban residents of a lower educational level. The role of television can be crucial.
Brokers of crisis: the everyday uncertainty of Eastern Congo's mineral négociants*

ABSTRACT Eastern Democratic Republic of the Congo's (DRC) artisanal mining sector is often linked to the violent conflicts that have beset Central Africa for over two decades. While many analyses emphasise its ‘criminal’ and ‘illegal’ nature, less attention has been paid to the ambiguity of this economy, most prominently incarnated by the intermediate mineral traders called négociants. Focusing on their entrepreneurship, networks and everyday activities, this essay offers a more nuanced understanding of local mineral trade in the context of a ‘crisis economy’ framed by competing governable orders. It investigates the uncertainty along eastern DRC's mineral supply chains, that are undergoing major regulatory changes to curb the trade of so-called ‘conflict minerals’. Drawing from extensive fieldwork, this essay demonstrates how this uncertainty shapes the négociants’ role as brokers of socio-economic life in the provinces of North and South Kivu.
A Robust Point-Matching Algorithm Based on Integrated Spatial Structure Constraint for Remote Sensing Image Registration

Feature matching, which refers to finding the correct correspondences from two sets of features, is an important step in feature-based image registration. In this letter, an accurate and highly robust point-matching algorithm, which is called the integrated spatial structure constraint, is proposed. We establish a set of tentative correspondences using the scale-invariant feature transform algorithm and then focus on increasing the number of correct correspondences (inliers) and removing incorrect correspondences (outliers). First, a global structure constraint, i.e., the shape context, is constructed for each correspondence out of the tentative set to increase the number of inliers and raise the correct rate simultaneously. Then, a local structure constraint based on the triangle area representation is utilized on the neighboring points of each correspondence to remove outliers. Experimental results compared with four state-of-the-art methods demonstrate that the proposed algorithm is robust and can achieve preferable results in terms of both matching accuracy and quantity of inliers.
Leadership Education in Public Administration: Finding the Fit between Purpose and Approach

Abstract This article asserts that leadership education programs can be strengthened by questioning assumptions about the nature of leadership and the skills it requires and by using pedagogical approaches that are a good fit with the knowledge and skills students are expected to master. The authors argue that differing views and assumptions about leadership and how it can be practiced successfully may suggest different educational approaches. The authors address four approaches to public sector leadership—trait, situational, transformational, and value-based—and compare the skills and abilities, representative literature, and teaching techniques associated with each approach; they argue that value-based leadership is particularly important in developing a model for public leadership education. They also consider whether special leadership responsibilities and approaches derive from the public sector context, and, if so, how public affairs educators might infuse leadership education with these elements.
Relationship of Lymph Node Micrometastasis and Micropapillary Component and Their Joint Influence on Prognosis of Patients With Stage I Lung Adenocarcinoma

This study aimed to investigate the relationship between lymph node micrometastasis and histologic patterns of adenocarcinoma, with a particular focus on their joint effect on prognosis. We retrospectively reviewed 235 patients with stage I adenocarcinoma from January 2009 to December 2009. Lymph node micrometastasis was evaluated by immunohistochemical staining for cytokeratin (AE1/AE3) and thyroid transcription factor-1. A logistic regression model was applied to confirm the predictive factors of micrometastasis. Survival analysis was performed to evaluate the effect of micrometastasis on prognosis. Lymph node micrometastasis was observed in 35 patients (15%). Patients with micrometastasis had significantly worse recurrence-free survival (P<0.001) and overall survival (P<0.001) compared with those without micrometastasis. Micropapillary component was confirmed as an independent predictor of increased frequency of micrometastasis (P<0.001). Among 62 patients with adenocarcinoma with a micropapillary component, 23 (37%) had lymph node micrometastasis. Micropapillary-positive/micrometastasis-positive patients had significantly worse survival compared with micropapillary-positive/micrometastasis-negative patients (RFS, P=0.039; OS, P=0.002) and micropapillary-negative patients (recurrence-free survival, P<0.001; overall survival, P<0.001). Moreover, the presence of micrometastasis correlated with a higher risk of locoregional recurrence (P=0.031) rather than distant recurrence (P=0.456) in micropapillary-positive patients. In summary, lymph node micrometastasis was more frequently observed in adenocarcinoma with a micropapillary component. Moreover, lymph node micrometastasis could provide helpful prognostic information in patients with resected stage I lung adenocarcinoma with a micropapillary component; thus, immunohistochemical detection of micrometastatic tumor cells in lymph nodes should be recommended.
Comparison of Registered and Reported Outcomes in Randomized Clinical Trials Published in Anesthesiology Journals

BACKGROUND: Randomized clinical trials (RCTs) provide high-quality evidence for clinical decision-making. Trial registration is one of the many tools used to improve the reporting of RCTs by reducing publication bias and selective outcome reporting bias. The purpose of our study is to examine whether RCTs published in the top 6 general anesthesiology journals were adequately registered and whether the reported primary and secondary outcomes corresponded to the originally registered outcomes. METHODS: Following a prespecified protocol, an electronic database was used to systematically screen and extract data from RCTs published in the top 6 general anesthesiology journals by impact factor (Anaesthesia, Anesthesia & Analgesia, Anesthesiology, British Journal of Anaesthesia, Canadian Journal of Anesthesia, and European Journal of Anaesthesiology) during the years 2007, 2010, 2013, and 2015. A manual search of each journal’s Table of Contents was performed (in duplicate) to identify eligible RCTs. An adequately registered trial was defined as being registered in a publicly available trials registry before the first patient being enrolled with an unambiguously defined primary outcome. For adequately registered trials, the outcomes registered in the trial registry were compared with the outcomes reported in the article, with outcome discrepancies documented and analyzed by the type of discrepancy. RESULTS: During the 4 years studied, there were 860 RCTs identified, with 102 RCTs determined to be adequately registered (12%). The proportion of adequately registered trials increased over time, with 38% of RCTs being adequately registered in 2015. The most common reason in 2015 for inadequate registration was registering the RCT after the first patient had already been enrolled. Among adequately registered trials, 92% had at least 1 primary or secondary outcome discrepancy. In 2015, 42% of RCTs had at least 1 primary outcome discrepancy, while 90% of RCTs had at least 1 secondary outcome discrepancy. CONCLUSIONS: Despite trial registration being an accepted best practice, RCTs published in anesthesiology journals have a high rate of inadequate registration. While mandating trial registration has increased the proportion of adequately registered trials over time, there is still an unacceptably high proportion of inadequately registered RCTs. Among adequately registered trials, there are high rates of discrepancies between registered and reported outcomes, suggesting a need to compare a published RCT with its trial registry entry to be able to fully assess the quality of the study. If clinicians base their decisions on evidence distorted by primary outcome switching, patient care could be negatively affected.
A Micromethod for Determination of Plasma Pyridoxal Phosphate and Its Use in Assessment of Storage Stability of the Vitamer

A widely used macromethod employing tyrosine apodecarboxylase for measurement of pyridoxal phosphate (PLP) concentration in 0.5–1.0-ml plasma was modified to a microscale utilizing 0.1-ml plasma. Mean PLP levels in 12 plasma samples were 160.6 ± 32.8 pmol/ml (mean ± SD) when analyzed by the macro-method, and were not significantly different compared to those obtained by the micromethod (158.4 ± 28.2 pmol/ ml). Results of the two methods were significantly correlated (r = +0.97, p < 0.001). Plasma PLP concentrations of 11 samples determined by the micromethod (&OV0335; = 151.8 ± 30.0 pmol/ml) were similar and significantly correlated (r = +0.95, p < 0.001) to levels measured in the same samples 1–2 years earlier (&OV0335; = 145.1 ± 26.2 pmol/ml). This suggests that plasma PLP content of the samples was stable for up to 2 years of storage when the micro-method was utilized for analysis. The strong significant correlation between macro- and micromethods attests that the micromethod is a reliable alternative to the macromethod. The micromethod is useful in instances where only small samples of plasma are available for measurement of PLP.
Adherence of Families to a Group A Streptococcal Pharyngitis Protocol Used in a Pediatric Emergency Department

Objectives: In an effort to limit inappropriate antibiotic use for children with pharyngitis, our pediatric emergency department (PED) has implemented a strep throat protocol using preprinted prescriptions given to families pending pharyngeal swab results. We sought to determine the rate of adherence of families managed with this protocol and to identify whether clinical features are associated with adherence. Methods: We conducted a prospective cohort study of children aged 2 to 17 years presented to the PED with suspected group A streptococcal (GAS) pharyngitis. Prescription-filling activity was tracked using a novel carbon-copy prescription and compared with throat swab result. Health records were reviewed for demographic and clinical information. Adherence was defined as prescriptions being filled after notification of a positive swab result and prescriptions not being filled when throat swab result was negative. Results: Three hundred nine children were screened for GAS pharyngitis. One hundred swabs (32.4%) were positive for GAS pharyngitis, of which 15 prescriptions were filled before swab results. No record of prescription filling was found for 37 of the children with positive swab results. Only 9 families (4.3%) filled the prescription when the swab result was negative. Overall, 247 families (80.2%) were adherent to the protocol. Families of children aged 2 to 5 years were more likely to be adherent than those aged 13 to 17 years (odds ratio, 3.5; 95% confidence interval, 1.15-10.66). Conclusions: Most families are adherent to our GAS pharyngitis protocol. Very few families filled prescriptions when the swab result was negative. Age was the only factor influencing adherence. Our current GAS pharyngitis protocol is an effective management strategy for children presenting with pharyngitis to the PED.
Anticoagulants for the treatment of recurrent pregnancy loss in women without antiphospholipid syndrome.

BACKGROUND Since hypercoagulability might result in recurrent pregnancy loss, anticoagulant agents could potentially increase the live-birth rate in subsequent pregnancies in women with either inherited thrombophilia or unexplained pregnancy loss. OBJECTIVES To evaluate the efficacy and safety of anticoagulant agents, such as aspirin and heparin, in women with a history of at least two spontaneous miscarriages or one later intrauterine fetal death without apparent causes other than inherited thrombophilias. SEARCH STRATEGY We searched the Cochrane Pregnancy and Childbirth Group trials register (March 2004), the Central Register of Controlled Trials (The Cochrane Library, Issue 1, 2004), MEDLINE (January 1966 to March 2004), and EMBASE (1980 to March 2004). We scanned bibliographies of all located articles for any unidentified articles. SELECTION CRITERIA Randomised and quasi-randomised controlled trials that assessed the effect of anticoagulant treatment on the live-birth rate in women with a history of at least two spontaneous miscarriages or one later intrauterine fetal death without apparent causes other than inherited thrombophilias were eligible. Interventions included aspirin, unfractionated heparin, and low molecular weight heparin for the prevention of birth loss. One treatment could be compared with another or with placebo. DATA COLLECTION AND ANALYSIS Two authors assessed the trials for inclusion in the review and extracted the data. Data were entered into the Review Manager software and double checked. MAIN RESULTS Two studies (242 participants) were included in the review and for both of them data were extracted for the subgroups of women fulfilling the inclusion criteria of the review. In one study, 54 pregnant women with recurrent spontaneous abortion without detectable anticardiolipin antibodies were randomised to low-dose aspirin or placebo. Similar live-birth rates were observed with aspirin and placebo (relative risk (RR) 1.00, 95% confidence interval (CI) 0.78 to 1.29). In another study, a subgroup of 20 women who had had a previous fetal loss after the 20th week and had a thrombophilic defect were randomised to enoxaparin or aspirin. Enoxaparin treatment resulted in an increased live-birth rate, as compared to low-dose aspirin, RR 10.00, 95% CI 1.56 to 64.20). AUTHORS' CONCLUSIONS The evidence on the efficacy and safety of thromboprophylaxis with aspirin and heparin in women with a history of at least two spontaneous miscarriages or one later intrauterine fetal death without apparent causes other than inherited thrombophilias is too limited to recommend the use of anticoagulants in this setting. Large, randomised, placebo-controlled trials are urgently needed.
Human, rodent, and canine pancreatic beta-cells express a sodium channel alpha 1-subunit related to a fetal brain isoform.

Voltage-dependent, tetrodotoxin-sensitive Na+ currents have been identified by the patch-clamp technique in rodent, canine, and human pancreatic beta-cells, but their exact role in insulin secretion remains uncertain. Misler et al. (Diabetes 41:1221-28, 1992) recently reviewed data showing that human and rat beta-cell action potentials differ, in that trains of Na(+)-dependent action potentials are seen in human (and canine) cells, but the vast majority of rat beta-cell Na+ channels are inactivated. We have now identified Na+ channel alpha 1-subunit mRNAs expressed in normal adult human, canine, and rat islets, and two insulinoma cell lines, by mRNA amplification (reverse transcription followed by polymerase chain reaction). cDNA sequencing showed that all amplified human islet products and the majority of rodent islet cDNAs are most closely related to the rat brain III alpha 1-subunit isoform, previously found to be expressed primarily in fetal rat brain. Canine islets expressed both brain II and brain III isoforms. Reverse transcription followed by polymerase chain reaction experiments with hamster and mouse insulinoma cell lines also showed expression of the message related to the rat brain III isoform. In situ hybridization of human pancreas sections using a partial human Na+ channel III cDNA probe showed the message to be expressed in the majority of islet cells, and not in the acinar tissue, confirming its presence in pancreatic beta-cells. (ABSTRACT TRUNCATED AT 250 WORDS)
Hidradenitis suppurativa: a disease with male predominance in Tunisia.

BACKGROUND hidradenitis suppurativa (HS) is a chronic inflammatory, suppurating, fistulizing, and scar-producing disease of apocrine gland-bearing skin. The diagnosis is primarily clinical, based on the presence of both sinus tracts and abscesses with a characteristic distribution. OBJECTIVE Review of epidemiological, clinical, and prognostic characteristics of HS and discussion of the etiopathogenic aspects of this chronic problem. PATIENTS AND METHODS We retrospectively report all cases of HS followed at the Department of Dermatology between January 1985 and December 2008. RESULTS Eleven patients (10 male and 1 female), with a mean age of 35.2 years (range 21 dash, vertical53 years) at HS diagnosis were followed for HS. The average age of disease onset was 23.9 years. The median delay between onset of symptoms and diagnosis was 144 months (range 1 dash, vertical408 months). Clinical features showed inflamed discharging papules or nodules, painful tender erythematous nodules, and double-ended comedones. The disease mainly affected the axillary, anal, perineal, and genital areas. Histologically, dermal features showed active folliculitis or abscess, sinus tract formation, fibrosis, and granuloma formation. Pathological associations (Darier's disease and Down syndrome) were noted in two patients. Treatment consisted of antibiotics in eight patients, retinoids (1 mg/kg/ day) in three patients, and surgery in three patients. The mean follow-up was 13 months (range 2 dash, vertical30 months). Recurrence of lesions was observed in all patients approximately 1 month after treatment withdrawal. The Down syndrome patient developed vaginal hydrocele of the testis as a complication of his staphylococcic ulcers. In all cases healing occurred with substantial scarring. DISCUSSION An obvious male predominance was noted in our patients as well as a delay in the diagnosis of HS, which could be explained by non-recognition of the disease by non-specialists that see the patients at the primary-care level.
Translocation of Hormone-sensitive Lipase and Perilipin upon Lipolytic Stimulation of Rat Adipocytes*

Adipocyte lipolysis was compared with hormone-sensitive lipase (HSL)/perilipin subcellular distribution and perilipin phosphorylation using Western blot analysis. Under basal conditions, HSL resided predominantly in the cytosol and unphosphorylated perilipin upon the lipid droplet. Upon lipolytic stimulation of adipocytes isolated from young rats with the β-adrenergic agonist, isoproterenol, HSL translocated from the cytosol to the lipid droplet, but there was no movement of perilipin from the droplet to the cytosol; however, perilipin phosphorylation was observed. By contrast, upon lipolytic stimulation and perilipin phosphorylation in cells from more mature rats, there was no HSL translocation but a significant movement of perilipin away from the lipid droplet. Adipocytes from younger rats had markedly greater rates of lipolysis than those from the older rats. Thus high rates of lipolysis require translocation of HSL to the lipid droplet and translocation of HSL and perilipin can occur independently of each other. A loss of the ability to translocate HSL to the lipid droplet probably contributes to the diminished lipolytic response to catecholamines with age.
Three week results of transforaminal epidural steroid injection in patients with chronic unilateral low back related leg pain: The relation to MRI findings and clinical features.

BACKGROUND Transforaminal epidural steroid injection (TESI) is a frequently used intervention for lumbar radicular pain. OBJECTIVE To evaluate the value of MRI findings, neurologic assessment and the Slump test (neurodynamic test) as predictors of treatment response to TESI. METHOD One hundred subjects (mean age 58 [SD13], 54% females) were included in this trial. The sample was stratified by location of disc herniaton, grade of nerve root compression, clinically assessed neurologic deficit and positive Slump test.Treatment response was primarily evaluated by Visual Analogue Scale for leg pain after three weeks. Predictive value for each stratum was analyzed using logistic regression after the sample was dichotomized into definite treatment response (≥ 50% reduction of pain) and negative response (≤ 0% reduction) to TESI (the 1-49% reduction group was excluded). RESULTS The overall definite treatment response rate was 27%. The Slump test was the only predictor of the response to TESI (p= 0.031). The definite treatment response rates for subjects with positive and negative Slump test were 33% and 15%, respectively. CONCLUSION In patients with chronic low back related leg pain, MRI findings and neurologic assessment results failed to predict treatment response, whereas a positive Slump test predicted the best 3-week response to TESI.