Miracle-dz/UniFAR-FTUs huggingface dataset:
Pediatric Cochlear Implantation in Auditory Neuropathy

Objective Auditory neuropathy (AN) is characterized by varying degrees of sensorineural hearing loss, an absent or severely abnormal auditory brainstem response, and normal otoacoustic emissions. The nomenclature for this condition reflects the concept that the site of lesion is proximal to the cochlea (e.g., cochlear nerve). Given this hypothesis, it is reasonable to expect limited benefit from cochlear implantation in patients with AN. However, a growing body of evidence shows the striking benefits of cochlear implantation in AN. To explore this topic, we reviewed our population of children with AN and, specifically, the performance results in those children having undergone cochlear implantation. Study Design A retrospective case review of those patients diagnosed with AN from 1993 to 2001. Setting A tertiary pediatric referral center. Patients A diagnosis of AN reported from the Center for Hearing and Deafness Research, Cincinnati, OH, database. Results Eighteen patients were diagnosed with AN (11 girls, 7 boys), with 3 sets of siblings, including 1 set of identical twins. Four patients with AN underwent implantation in the previous 5 years. Twelve out of the 18 patients had classic risk factors for AN (e.g., prematurity and hyperbilirubinemia). The degree of hearing loss varied in our patients, with a majority showing severe to profound deficits. All children with implants showed improvement in auditory and verbal development, but this improvement was variable. Conclusion The success of cochlear implantation in these patients suggests that some children with AN have an auditory system lesion that can be compensated for by cochlear implantation. This implies either an inner hair cell or inner hair cell–cochlear nerve junctional pathology that can be overcome by direct electrical stimulation.
Mutations in CYP11B1 gene: Phenotype–genotype correlations

11β‐hydroxylase deficiency, an autosomal recessive disorder, is the second most common cause of congenital adrenal hyperplasia. We studied four subjects with classic 11β‐hydroxylase deficiency and severe hypertension: a 46,XX affected subject from a Turkish family with severe ambiguity of the external genitalia and hypertension, and three affected 46,XY subjects from a Dominican kindred with isosexual precocious puberty and severe hypertension. The affected subjects had significantly elevated plasma 11‐desoxycortisol, 11‐desoxycorticosterone, Δ4‐androstenedione, and testosterone. To determine the molecular genetic defects, genomic DNA was isolated from the leukocytes of affected subjects and their family members. The encoding region of the 11β‐hydroxylase gene (CYP11B1) was amplified by PCR with specific primers. Using single‐stranded DNA conformational polymorphism (SSCP) and DNA sequencing, a nonsense mutation in exon 6 of CYP11B1 in the affected 46,XX subject from the Turkish family was identified, where a cytosine was substituted by a thymidine, resulting in the replacement of glutamine (CAG) by a stop codon (TAG) at amino acid position 338 (Q338X). In the three 46,XY Dominican boys, the mutation was also a nonsense mutation in exon 6 of CYP11B1, where a cytosine was substituted by a thymidine, resulting in the replacement of glutamine (CAG) by a stop codon (TAG) at amino acid position 356 (Q356X). Both mutations result in the biosynthesis of a truncated 11β‐hydroxylase protein with loss of enzymatic activity. Heterozygosity was determined in family members of both probands including parents and siblings. These results indicate that mutations of CYP11B1 in these subjects are responsible for their clinical syndromes. © 2003 Wiley‐Liss, Inc.
MANAGEMENT OF ADVANCED RETINOBLASTOMA WITH INTRAVENOUS CHEMOTHERAPY THEN INTRA-ARTERIAL CHEMOTHERAPY AS ALTERNATIVE TO ENUCLEATION

Purpose: To determine the efficacy of primary intravenous chemotherapy (IVC) plus secondary intraarterial chemotherapy (IAC) for patients with advanced retinoblastoma. Methods: Retrospective, nonrandomized interventional case series of 14 patients with retinoblastoma managed with primary systemic IVC (vincristine, etoposide, and carboplatin for 6 cycles) followed by secondary IAC (melphalan for 1–6 cycles). Results: Fourteen patients with advanced retinoblastoma classified by the International Classification of Retinoblastoma as Group D (n = 6, 43%) or Group E (n = 8, 57%) were treated with IVC as primary treatment and subsequent secondary IAC as rescue or consolidation therapy. The IAC was given for recurrent retinoblastoma and/or subretinal/vitreous seeds in 13 eyes (93%) and for persistent viable retinoblastoma in 1 eye (7%). Enucleation was the alternative option. The mean interval between IVC completion and IAC start was 40 weeks (median, 11 weeks; range, 2–170 weeks) and the mean number of IAC cycles was 3 (median, 3; range, 1–6). After primary IVC plus secondary IAC, globe salvage was achieved in 8 patients (57%) at mean 2-year follow-up. There was no evidence of retinoblastoma metastasis or death and no sign of second cancer or life-threatening complication. Conclusion: For advanced retinoblastoma (Groups D and E) in which enucleation is the alternative option, primary systemic IVC followed by secondary focal IAC provides globe salvage in 57% of the eyes and with no metastatic event.
Māori, a Politicized Identity: Indigenous Identity, Voter Turnout, Protest, and Political Party Support in Aotearoa New Zealand

Political struggles are important to the identities of many indigenous peoples. This article examines identity as a predictor of crucial political outcomes—voter turnout, support for protest, and political party support—for Māori, the indigenous peoples of Aotearoa (New Zealand). We analyzed data from a national probability sample of Māori (N = 663) that included a scale of subjective identification with various aspects of Māori identity: the Multidimensional Model of Māori Identity and Cultural Engagement. Use of the scale allowed us to examine the facets of ethnic identity that predict political mobilization for indigenous peoples. As expected, the identity domain relating to political struggle, Socio-Political Consciousness, was positively associated with support for left-wing parties and Māori rights protest but negatively associated with support for the right-wing party. However, Socio-Political Consciousness did not relate to voter turnout. These results demonstrate the importance of ethnic identity as a key predictor of political behaviors for indigenous peoples.
A Series Stacked IGBT Switch With Robustness Against Short-Circuit Fault for Pulsed Power Applications

The limited value of the insolated-gate bipolar transistors (IGBTs) blocking voltage is an important issue for applying these devices to the high-voltage power converters. An effective solution is to use a series configuration of these devices in order to achieve higher voltage ratings in addition to save the interesting features such as fast rising time. To reach this goal, acceptable equal voltage sharing for the IGBTs are provided by the series stacking schemes in the normal operation. However, a considerable difference in their voltage level will be occurred in the short-circuit condition. Although the IGBT can withstand the short-circuit current in a defined time, the occurred high voltage stress for the IGBT is fatal in such condition. To solve the mentioned problem, this paper proposes a short-circuit protection system suitable for the series stacked IGBTs. Using this proposal, the safe voltage level for IGBTs as well as the adequate time for the performance of the detection and elimination of the short-circuit fault will be provided. Each IGBT can have a different short-circuit current in the active region since the proposed scheme makes the IGBTs independent in terms of the current in the series structure. An external resistor added to the IGBTs emitter improves the equality of the IGBTs short-circuit currents and consequently their voltage sharing. This resistor controls the system short-circuit current by changing the operating point of IGBTs in active region in the short-circuit fault. The experimental and simulation results show that the safe condition is provided for the IGBTs under the short-circuit fault.
Co-composting of rose oil processing waste with caged layer manure and straw or sawdust: Effects of carbon source and C/N ratio on decomposition

Rose oil is a specific essential oil that is produced mainly for the cosmetics industry in a few selected locations around the world. Rose oil production is a water distillation process from petals of Rosa damascena Mill. Since the oil content of the rose petals of this variety is between 0.3–0.4% (w/w), almost 4000 to 3000 kg of rose petals are needed to produce 1 kg of rose oil. Rose oil production is a seasonal activity and takes place during the relatively short period where the roses are blooming. As a result, large quantities of solid waste are produced over a limited time interval. This research aims: (i) to determine the possibilities of aerobic co-composting as a waste management option for rose oil processing waste with caged layer manure; (ii) to identify effects of different carbon sources – straw or sawdust on co-composting of rose oil processing waste and caged layer manure, which are both readily available in Isparta, where significant rose oil production also takes place; (iii) to determine the effects of different C/N ratios on co-composting by the means of organic matter decomposition and dry matter loss. Composting experiments were carried out by 12 identical laboratory-scale composting reactors (60 L) simultaneously. The results of the study showed that the best results were obtained with a mixture consisting of 50% rose oil processing waste, 64% caged layer manure and 15% straw wet weight in terms of organic matter loss (66%) and dry matter loss (38%).
Targeting Fel d 1 to FcgammaRI induces a novel variation of the T(H)2 response in subjects with cat allergy.

BACKGROUND Induction of CD4+ T cells that produce IL-10 or IFN-gamma is central to the protective effects of conventional allergen immunotherapy. OBJECTIVE We examined the T-cell modulatory capacity of a fusion protein (H22-Fel d 1) that targets Fel d 1 to the high-affinity IgG receptor (FcgammaRI) on antigen-presenting cells. METHODS Monocyte-derived dendritic cells pulsed with H22-Fel d 1 were analyzed for surface phenotype and cytokine secretion by flow cytometry and cytometric bead assay, respectively. CD4+ T cells generated after coculture with H22-Fel d 1-pulsed dendritic cells were analyzed at the single-cell level by flow cytometry after intracellular cytokine staining. The T-cell repertoire was compared for subjects with (IgE+) and without cat allergy (IgE(neg)IgG(neg)), including subjects with a modified T(H)2 response (IgE(neg)IgG+). RESULTS H22-Fel d 1 induced a semimature phenotype in dendritic cells in conjunction with a selective increase in IL-5+ and IL-10+ CD4+ T cells compared with nonreceptor-targeted Fel d 1. Amplified T cells included diverse subtypes characteristic of T(H)0 (IL-5+IFN-gamma+), regulatory T(H)1 (IL-10+IFN-gamma+) and regulatory T(H)2 (IL-10+IL-5+ cells. T-cell qualitative changes were restricted to subjects with allergy and were distinct from a modified T(H)2 response. Blocking IL-10 induced by H22-Fel d 1 selectively increased IL-5+ CD4+ T cells, suggesting that T(H)2 responses were controlled. CONCLUSION Targeting Fel d 1 to FcgammaRI induces a novel variation of the T(H)2 response that incorporates major elements of a protective T-cell response.
Health-Related Quality of Life in Children Operated for Pediatric Cataracts

Purpose To evaluate health-related quality of life (HRQOL) in children who had surgery for pediatric cataracts. Methods Twenty-five children were divided into 2 groups according to the age at the time of survey (younger than 6 years in group A and 7 years and older in group B). A questionnaire that had 1 part appraising the child's perception and 3 parts evaluating the functional, social, and surgical apprehensions of parents was given to the participants. The questions in the first part were answered by parents in group A (proxy test) and by children in group B (self test). The correlations between the results and patient characteristics were evaluated statistically. Results Mean (range) score for the first part was 79.5 (58-97) in group A and 70 (56-86) in group B. Functional, social, and surgical scores were 42.5 (0-80), 45 (5-100), and 63 (12.5-100) in group A and 40 (10-90), 70 (40-85), and 75 (25-100) in group B. Functional, social, or surgical scores were not significantly different between groups. Social apprehensions were significantly higher in parents of patients with preoperative strabismus in group B. Functional, social, surgical apprehensions, and children's thoughts were not correlated significantly with age, sex, family history, laterality, associated nystagmus, aphakia or pseudophakia, postoperative optical rehabilitation, or the need for additional surgery in the groups. Conclusions Although pediatric cataracts are known to have a great impact on children's life socially and functionally, there was no significant relationship between patient characteristics and HRQOL. The associated preoperative strabismus causes more social concern in the parents.
RAD50 Expression Is Associated with Poor Clinical Outcomes after Radiotherapy for Resected Non–small Cell Lung Cancer

Purpose: Although postoperative radiotherapy is often used to maintain local control after surgical resection and chemotherapy for locally advanced non–small cell lung cancer (NSCLC), both locoregional failure and distant metastasis remain problematic. The mechanisms of therapeutic resistance remain poorly understood. Experimental Design: We used reverse-phase protein arrays (RPPA) to profile the baseline expression of 170 total and phosphorylated proteins in 70 NSCLC cell lines to categorize pathways that may contribute to radiation resistance. Significant markers identified by RPPA were further analyzed in tissue microarrays (TMA) of specimens from 127 patients with NSCLC who had received surgery before receiving postoperative radiotherapy. Cox regression analysis and log-rank tests were used to identify potential predictive factors. We then validated the biological function of the markers in NSCLC cell lines in vitro. Results: Of the 170 proteins or phospho-proteins profiled, a subset of 12 proteins was found to correlate with radiation response parameters. TMA analysis of the 12 proteins showing the greatest differences in expression in the RPPA analysis demonstrated that RAD50 had the strongest correlation with distant relapse-free survival, locoregional relapse-free survival, and disease-free survival in patients with NSCLC. We confirmed that knockdown of RAD50 sensitized NSCLC cells to radiation and that upregulation of RAD50 increased radioresistance in in vitro experiments. Conclusions: Upregulated RAD50 may be a predictor of radioresistance in patients with lung cancer who received radiotherapy. Clin Cancer Res; 24(2); 341–50. ©2017 AACR.
Understanding Ethical Consumption: Types and Antecedents

Ethical consumption incorporates a variety of consumer practices, which have been fragmentarily investigated, so far. This research study introduces the overall concept of Ethical Consumption (EC) with the concurrent investigation of its three types, namely Positive, Negative and Discursive Ethical Consumption (PEC, NEC, DEC). The demographic, attitudinal and psychographic antecedents of each type were examined. The results of a survey conducted in Thessaloniki urban area, Greece with a stratified sample of 420 consumers are presented. The results revealed that Greeks do not frequently engage in any of the three types. The analyses of the results indicated the demographic, attitudinal and psychographic antecedents of the EC types and enlightened the insights and inter-relations in the overall domain. PEC is endorsed by middle aged, highly educated women, who receive relatively high family incomes. Consumers, who are less indifferent to ethical consumption issues, less sceptical about ethical products, more willing and capable of exercising their political interests and influencing the political system, are more likely to get engaged in PEC. NEC is endorsed by highly educated consumers, who feel more politically powerful, less ethically indifferent and hold higher post-materialist values than their counterparts. DEC is endorsed by young, post-graduate professionals. Consumers, who are less indifferent to ethical consumption issues, less sceptical about ethical products, less materialists and feel more politically powerful, are more likely to get engaged in DEC.
Normal Brain Maturation Characterized With Age-Related T2 Relaxation Times: An Attempt to Develop a Quantitative Imaging Measure for Clinical Use

Objectives:We studied age-related changes in T2 relaxation times from the normal maturating human brain under routine clinical MR examination conditions. Materials and Methods:In 70 healthy subjects aged between 3 weeks and 39 years, T2 maps of the brain in which the intensity of each pixel corresponded to T2 relaxation times were generated based on magnetic resonance imaging data collected with a triple spin echo sequence. T2 relaxation times in white matter (WM) and gray matter (GM) were measured in 6 distinctive regions of interest of the T2 maps. The age dependence of the T2 values was mathematically simulated using a biexponential function. Results:T2 values were largest at the age of 3 weeks (maximum: approximately 400 milliseconds for WM and 200 milliseconds for GM) and decreased continuously with increasing age, faster in the first few months and slower thereafter, until values achieved between 95 and 110 milliseconds for WM and 88 and 95 milliseconds for GM in adults. The relationship between T2 values and age could be well simulated using a biexponential function (R2 > 0.92). Conclusions:T2 relaxation time correlates well with the progress of brain maturation. The used biexponential function reflects the dynamic development of myelination in newborns and young children as well as the maturation of myelination during adolescence and could be used to develop a “normal” reference for neuroradiological diagnoses.
Educating Caribbean librarians to provide library education in a dynamic information environment

The dynamism of the information sector driven by ICT and the resulting impact on library and information science professionals have resulted in significant changes in how teaching librarians in the Caribbean are educated. This research was conducted to: describe the education of teaching librarians; examine the changes in education; and investigate how teaching librarians develop and hone their teaching competencies. A mixed-method approach was used to garner quantitative data from 45 librarians and qualitative data from relevant documents in two library schools. The findings show there were significant changes as new courses and programs were developed, online programs were implemented and faculty qualifications upgraded. These changes were somewhat effective, however, teacher-librarians, who developed teaching skills mainly from teacher training, expressed a need for additional technology skills to make them more effective. It was recommended that LIS schools need to diversify their programs to make the content and the activities more aligned to the day to day practices of these teacher librarians. Educating Caribbean Librarians to Provide Library Education in a Dynamic Information Environment
How do patients who participate in cancer support groups differ from those who do not?

Research has shown that cancer patients in general benefit from support group participation. However, few patients attend such groups. This study investigated differences between participants of a community cancer support group and a random selection of non‐participants from the Cancer Registry. Data were collected through mail survey, and included variables identified through past research and variables derived from Leventhal's self‐regulatory model of illness representations and the theory of planned behaviour. Sixty‐three support group participants and 44 comparison sample respondents were recruited. Support group participants were more likely to be female, without a partner, younger, and to have more education and formal support than non‐participants. They held more favourable views of support groups, believed that significant others were favourable towards participation, and perceived less difficulty in joining a group. They furthermore used more active, adaptive coping strategies and felt more control over their cancer, but were more distressed and anxious. Non‐participants reported more support from a special person. A multivariate logistic regression analysis showed only psychosocial variables to be independent predictors of participation. As psychological variables are amenable to change, increase of appropriate support group participation should be possible, for instance by addressing patients' beliefs about support groups. Copyright © 2005 John Wiley & Sons, Ltd.
Synchronous incidental gastrointestinal stromal and epithelial malignant tumors.

AIM To investigate the incidence of incidental gastrointestinal stromal tumor (GIST) and its etiopathogenesis. METHODS From January 1, 2000 to December 31, 2007, 13804 cases of gastrointestinal epithelial malignant tumor (EMT) and 521 cases of pancreatic adenocarcinoma (PAC) were successfully treated with surgery at the Department of General Surgery and the Department of Thoracic Surgery, West China Hospital, Sichuan University, China. The clinical and pathologic data of 311 cases of primary GIST, including 257 cases with clinical GIST and 54 cases of incidental GIST were analyzed. RESULTS Of the 311 patients, 54 had incidental GIST, accounting for 17.4%. Of these tumors, 27 were found in 1.13% patients with esophageal squamous cell carcinoma (ESCC), 22 in 0.53% patients with gastric adenocarcinoma (GAC), 2 in 0.38% patients with PAC, 2 in 0.03% patients with colorectal adenocarcinoma, and 1 in one patient with GAC accompanying ESCC, respectively. Patients with incidental GIST presented symptoms indistinguishable from those with EMT. All incidental GIST lesions were small in size, and the majority had a low mitotic activity while only 1.9% (5/257) of clinical GIST lesions had a high risk. CONCLUSION Incidental GIST may occur synchronously with other tumors and has a high prevalence in males. Surgery is its best treatment modality.
In-depth study on all forms of violence against women: Report of the Secretary General

Violence against women persists in every country in the world as a pervasive violation of human rights and a major impediment to achieving gender equality. Such violence is unacceptable, whether perpetrated by the State and its agents or by family members or strangers, in the public or private sphere, in peacetime or in times of conflict. The Secretary-General has stated that as long as violence against women continues, we cannot claim to be making real progress towards equality, development and peace.