15 research outputs found
Renewable, ethical? Assessing the energy justice potential of renewable electricity
Energy justice is increasingly being used as a framework to conceptualize the impacts of energy decision making in more holistic ways and to consider the social implications in terms of existing ethical values. Similarly, renewable energy technologies are increasingly being promoted for their environmental and social benefits. However, little work has been done to systematically examine the extent to which, in what ways and in what contexts, renewable energy technologies can contribute to achieving energy justice. This paper assesses the potential of renewable electricity technologies to address energy justice in various global contexts via a systematic review of existing studies analyzed in terms of the principles and dimensions of energy justice. Based on publications including peer reviewed academic literature, books, and in some cases reports by government or international organizations, we assess renewable electricity technologies in both grid integrated and off-grid use contexts. We conduct our investigation through the rubric of the affirmative and prohibitive principles of energy justice and in terms of its temporal, geographic, socio-political, economic, and technological dimensions. Renewable electricity technology development has and continue to have different impacts in different social contexts, and by considering the different impacts explicitly across global contexts, including differences between rural and urban contexts, this paper contributes to identifying and understanding how, in what ways, and in what particular conditions and circumstances renewable electricity technologies may correspond with or work to promote energy justice
Sonographic features of spermatic cord leiomyosarcoma
Leiomyosarcoma of the spermatic cord is a rare tumor, with 55 reported cases to date.(1) There are limited reports on the imaging findings, particularly the sonographic appearance of this rare tumor. We report the sonographic and histopathologic features of a case of spermatic cord leiomyosarcoma and discuss the role of sonography in demonstration and diagnosis of the mass
Segmental testicular infarction as a complication of varicocelectomy: Sonographic findings
Segmental testicular infarction is a rare clinical entity with various causes. We report the gray-scale and color Doppler sonographic findings of a case of segmental testicular infarction associated with varicocelectomy. (C) 2006 Wiley Periodicals, Inc
RUNX2 mutations in cleidocranial dysplasia
The runt-related transcription factor 2 gene (RUNX2), which is also known as CBFA1, is a master regulatory gene in bone formation. Mutations in RUNX2 have been identified in cleidocranial dysplasia (CCD) patients. CCD is a rare autosomal dominant skeletal dysplasia that is characterized by delayed closure of cranial sutures, aplastic or hypoplastic clavicle formation, short stature, and dental anomalies, including malocclusion, supernumerary teeth, and delayed eruption of permanent teeth. In this study, we recruited three de novo CCD families and performed mutational analysis of the RUNX2 gene as a candidate gene approach. The mutational study revealed three disease-causing mutations: a missense mutation (c.674G>A, p.Arg225Gln), a frameshift mutation (c.1119delC, p.Arg374Glyfs*), and a nonsense mutation (c.1171C>T, p.Arg391*). Clinical examination revealed a unique dental phenotype (no typical supernumerary teeth, but duplication of anterior teeth) in one patient. We believe that this finding will broaden the understanding of the mechanism of supernumerary teeth formation and CCD-related phenotypes
STIM1 and SLC24A4 Are Critical for Enamel Maturation
Dental enamel formation depends upon the transcellular transport of Ca2+ by ameloblasts, but little is known about the molecular mechanism, or even if the same process is operative during the secretory and maturation stages of amelogenesis. Identifying mutations in genes involved in Ca2+ homeostasis that cause inherited enamel defects can provide insights into the molecular participants and potential mechanisms of Ca2+ handling by ameloblasts. Stromal Interaction Molecule 1 (STIM1) is an ER transmembrane protein that activates membrane-specific Ca2+ influx in response to the depletion of ER Ca2+ stores. Solute carrier family 24, member 4 (SLC24A4), is a Na+/K+/Ca2+ transporter that exchanges intracellular Ca2+ and K+ for extracellular Na+. We identified a proband with syndromic hypomaturation enamel defects caused by a homozygous C to T transition (g.232598C>T c.1276C>T p.Arg426Cys) in STIM1, and a proband with isolated hypomaturation enamel defects caused by a homozygous C to T transition (g.124552C>T; c.437C>T; p.Ala146Val) in SLC24A4. Immunohistochemistry of developing mouse molars and incisors showed positive STIM1 and SLC24A4 signal specifically in maturation-stage ameloblasts. We conclude that enamel maturation is dependent upon STIM1 and SLC24A4 function, and that there are important differences in the Ca2+ transcellular transport systems used by secretory- and maturation-stage ameloblasts
Can SUVmax values of Ga-68-PSMA PET/CT scan predict the clinically significant prostate cancer?
Purpose The intensity of prostate-specific membrane antigen (PSMA) expression increases as the tumor grade increases and the uptake of Ga-68-PSMA is higher in high-grade tumors. The aim of the present study was to evaluate the correlation of preoperative tracer uptake of primary tumor to Gleason Score in patients who underwent prostatectomy. Patients and methods We retrospectively evaluated 141 patients who had Ga-68-PSMA positron emission tomography/computed tomography (PET/CT) imaging and who underwent prostatectomy. All patients had a diagnosis of prostate cancer on the basis of 10-24 cores transrectal ultrasound-guided biopsy (TRUS-Bx). Histological assessment was performed according to the New Contemporary Prostate Cancer Grading System. All patients had a prostate-specific antigen (PSA) level measurement within maximum of 28 days before Ga-68-PSMA PET/CT. Region of interests were drawn manually around the prostate gland, avoiding the bladder activity, to calculate the maximum standardized uptake values (SUVmax) values. Results The median PSA values for all patients were 10.0 ng/ml. PSA values for low-risk patients were significantly lower than those of high-risk patients (P<0.001). There were 41.1% upgrades and 7.8% downgrades following prostatectomy in terms of Grade Groups. According to the final pathology reports, 21% (n=16) of patients moved from a low-risk level (grade groups 1+2) to a high-risk level (grade groups 3+4+5). The median SUVmax value was 8.8, ranging from 2.1 to 62.4. There was a strong correlation between SUVmax values and grade groups (Pearson rho=0.66) (P<0.001). The mean SUVmax values of high-risk patients were significantly higher than those of low-risk patients (18.9 +/- 12.1 vs. 7.16 +/- 6.2, respectively) (P<0.001). Receiver operation characteristic curve analysis of SUVmax at the cut-off value of 9.1 showed a high sensitivity (78%) and specificity (81%) for detection of high risk disease. Conclusion SUVmax values correlate significantly with the grade groups of the primary tumor. The intraprostatic accumulation sites may predict clinically significant cancer and potentially serve as a target for biopsy sampling in conjunction with mpMRI in selected patients
LAMB3 Mutations Causing Autosomal-dominant Amelogenesis Imperfecta
Amelogenesis imperfecta (AI) can be either isolated or part of a larger syndrome. Junctional epidermolysis bullosa (JEB) is a collection of autosomal-recessive disorders featuring AI associated with skin fragility and other symptoms. JEB is a recessive syndrome usually caused by mutations in both alleles of COL17A1, LAMA3, LAMB3, or LAMC2. In rare cases, heterozygous carriers in JEB kindreds display enamel malformations in the absence of skin fragility (isolated AI). We recruited two kindreds with autosomal-dominant amelogenesis imperfecta (ADAI) characterized by generalized severe enamel hypoplasia with deep linear grooves and pits. Whole-exome sequencing of both probands identified novel heterozygous mutations in the last exon of LAMB3 that likely truncated the protein. The mutations perfectly segregated with the enamel defects in both families. In Family 1, an 8-bp deletion (c.3446_3453del GACTGGAG) shifted the reading frame (p.Gly 1149Glufs*8). In Family 2, a single nucleotide substitution (c.C3431A) generated an in-frame translation termination codon (p.Ser1144*). We conclude that enamel formation is particularly sensitive to defects in hemidesmosome/basement-membrane complexes and that syndromic and non-syndromic forms of AI can be etiologically related