bluuebunny/crossref_metadata_2025_split huggingface dataset:
Heterozygous inactivating CaSR mutations causing neonatal hyperparathyroidism: function, inheritance and phenotype
Authors: Glaudo Markus , Letz Saskia , Quinkler Marcus , Bogner Ulrich , Elbelt Ulf , Strasburger Christian J , Schnabel Dirk , Lankes Erwin , Scheel Sandra , Feldkamp Joachim , Haag Christine , Schulze Egbert , Frank-Raue Karin , Raue Friedhelm , Mayr Bernhard , Schöfl Christof 
Published: September 2016

BackgroundHomozygous inactivating mutations of the calcium-sensing receptor (CaSR) lead to neonatal severe hyperparathyroidism (NSHPT), whereas heterozygous inactivating mutations result in familial hypocalciuric hypercalcemia (FHH). It is unknown why in some cases heterozygousCaSRmutations cause neonatal hyperparathyroidism (NHPT) clinically similar to NSHPT but with only moderately elevated serum calcium.MethodsA literature survey was conducted to identify patients with heterozygousCaSRmutations and NHPT. The common NHPT CaSR mutants R185Q and R227L were compared with 15 mutants causing only FHH in the heterozygous state. We studiedin vitrocalcium signaling including the functional consequences of co-expression of mutant and wild-type (wt) CaSR, patients’ phenotype, age of disease manifestation and mode of inheritance.ResultsAll inactivating CaSR mutants impaired calcium signaling of wt-CaSR regardless of the patients’ clinical phenotype. The absolute intracellular calcium signaling response to physiologic extracellular calcium concentrationsin vitroshowed a high correlation with patients’ serum calcium concentrationsin vivo, which is similar in NHPT and FHH patients with the same genotype. Pedigrees of FHH families revealed that paternal inheritanceper sedoes not necessarily lead to NHPT but may only cause FHH.ConclusionsThere is a significant correlation betweenin vitrofunctional impairment of the CaSR at physiologic calcium concentrations and the severity of alterations in calcium homeostasis in patients. Whether a particular genotype leads to NHPT or FHH appears to depend on additional predisposing genetic or environmental factors. An individual therapeutic approach appears to be warranted for NHPT patients.
Risk of Autism Associated With Hyperbilirubinemia and Phototherapy
Authors: Wu Yvonne W. , Kuzniewicz Michael W. , Croen Lisa , Walsh Eileen M. , McCulloch Charles E. , Newman Thomas B. 
Published: September 2016

OBJECTIVE:Whether neonatal hyperbilirubinemia and/or phototherapy increase the risk of autism spectrum disorder (ASD) is unclear. We sought to quantify the risk of ASD associated with elevated total serum bilirubin (TSB) levels and with phototherapy.METHODS:In a retrospective cohort study of 525 409 infants born at ≥35 weeks’ gestation in 15 Kaiser Permanente Northern California (KPNC) hospitals, 1995–2011, we obtained all TSB levels and determined which infants received phototherapy. From the KPNC Autism Registry, we identified patients with ASD diagnosed at a KPNC Autism Center, by a clinical specialist, or by a pediatrician. We calculated Cox proportional hazard ratios (HRs) for time to diagnosis of ASD, adjusting for confounding factors.RESULTS:Among infants in the birth cohort, 2% had at least 1 TSB level ≥20 mg/dL, and 8% received phototherapy. The rate of ASD was 13 per 1000 births. Crude analyses revealed an association between TSB ≥20 and ASD (relative risk: 1.4; 95% confidence interval [CI]: 1.1–1.6), and between phototherapy and ASD (relative risk: 1.7; 95% CI: 1.5–1.8). After adjusting for confounders, TSB ≥20 (HR: 1.09; 95% CI: 0.89–1.35) and phototherapy (HR: 1.10; 95% CI: 0.98–1.24) were no longer significantly associated with ASD. Independent risk factors for ASD included maternal and paternal age; maternal and paternal higher education; male sex; birth weight &lt;2500 g or ≥4200 g; and later year of birth.CONCLUSIONS:After adjustment for the effects of sociodemographic factors and birth weight, neither hyperbilirubinemia nor phototherapy was an independent risk factor for ASD.
Dialectal elements in the vocabulary of the Uyghur Khanate inscriptions
Authors: Aydin Erhan 
Published: September 2016

One of the significant problems with Old Turkic inscriptions is that it is not known by which peoples’ or tribe’s Turkic language the inscriptions were written in. Although among the clans and persons who wrote and erected the large inscriptions of the Turkic and Uyghur Khanates, those of Köl Tegin, Bilge Kaghan, Şine Usu, Tariat, Tes and Karabalghasun I were identified, the peoples or clans having erected the other inscriptions are mostly unknown. The most serious problem encountered by researchers in consideration of the tribal seals present in the inscriptions is the uncertainty whether the seal belonged to the tribe that wrote or erected the inscription, or the tribe that was in power at that time.
This paper investigates the inscriptions of the Uyghur Khanate. Our scrutiny is based on the examination of the peculiarities of the Uyghur Khanate inscriptions which cannot be observed in any other inscriptions of Mongolia, Yenisei, Altai and Kyrgyzstan. By substituting these peculiar words with other words to be found in other inscriptions, an attempt has been made to prove that these words are Uyghur dialectal words. After an inquiry whether the words were used subsequent to the runic period, etymological suggestions concerning the words have also been put forward.
The Organoleptic and Smoked Catfish Histology from Pre-cooking
Authors: Yuliastri Venny , Suwandi Ruddy , Uju U. 
Published: September 2016

<p>Catfish is one of the main commodities in fresh water aquaculture. Indonesia catfish<br />production increased 37,49% in 2010 until 2014. Protein content of catfish is 17.7-26.7%<br />and fat about 0.95 until 11.5%. The objective of this study was to determine the best<br />organoleptic and to study the changes of tissue structure of catfish caused by process precooking<br />and smoking process. Precooked Catfish with variation 5, 10 and 15 minute;<br />temperature of 100oC, and smoked for 7 hours with a temperature of 90°C, analyzed in<br />laboratory. The results organoleptic consumers is the favored smoke catfish without any<br />pre – cooking and catfish-results of pre-cooking for 5 minutes i.e. ten successive sense<br />8.66; 7.66 smell 8.46; 7.8appearance 8.13;5.93 texture 6.86; 6.93. Organoleptic low on </p><p>pre-cooked for 15 minutes the sense of 5.66 smell 7 appearance of 2.93 texture 5.53.<br />Pre–cooked for 15 minutes has the highest water content of 26.33%. Pre–cooked for 5<br />minutes with the lowest value of 16.23%. smoked catfish without any pre–cooking has<br />the highest value of aw 0.82. Pre–cooked for 15 minutes had the lowest value of 0.78.<br />Observation on histology showed fresh catfish the structure of the connective tissue still<br />compact. Precooked catfish, myomer begins damage, catfish smoke without pre-cooking<br />is characterized by the formation of fibrils wafy fibers are separated from one another and<br />pre-cooking smoked catfish, myoseptum damaged and lead to the distance inter myomer<br />but myomer still intact.<br />Keywords : Muscle, sensory analysis, water activity,water content</p>
The Use of Alginate in Lemon Extract Effervescent Powder Production
Authors: Murdinah M. 
Published: September 2016

<p>Study on the use of alginate in lemon (Citrus medica var lemon) extract effervescent<br />powder production has conducted. The aims of the research are to determine the optimum<br />concentration of alginate used in lemon extract effervescent powder to produced best<br />product and acceptance consumen.The lemon extract effervescent powder formula<br />consisted of lemon extract powder, sucrose, aspartame, salt and effervescent mix (citric<br />acid-tartrat acid-sodium bicarbonat). The alginate used in this study was extracted from<br />Sargassum filipendula sea weed. The concentration of alginate used in lemon effervescent<br />powder production was varied from 1; 2; 3 and 4%. The parameters observed to see the<br />quality of the product were moisture content, ash content, pH, viscosity and organoleptic </p><p>of dietary fiber, sugar content, vitamin C content, total titratable acids, TPC and E.Coli<br />to the best product. The result showed that the higher the concentration of alginate used<br />in lemon effervescent powder production, the higher viscousness and the lower the<br />organoleptic value. The optimum concentration of alginate used in the lemon extract<br />effervescent powder processing was 1%. The characteristic this product 7.60% moisture<br />content, 0.86% insoluble dietary fiber , 7.92% soluble dietary fiber, 3.74% sugar content,<br />55,26 mg/100 g vitamin C, 134.15 mL 0.1 NaOH/100 mL total titratable acids, 20 cPs<br />viscosity, &lt;2.5x102 coloni/mL TPC and E.Coli negative.<br />Keywords: Alginate, characteristic, effervescent powder, lemon extract</p>
Factors influencing atherogenic indices in type 2 diabetic women in northwestern Algeria
Authors: Diaf Mustapha , Khaled Boumediene Méghit 
Published: September 2016

<p class="abstract"><strong>Background:</strong> <span lang="EN-GB">Type 2 diabetic women are at higher risk of developing atherogenic dyslipidemia. The major possible risk factors are obesity, abdominal fat accumulation and poor glycaemic control. However, menopause-related changes could be another determinant. The aim of this study was to evaluate the interrelationships of these risk factors and their independent effects on atherogenic indices in type 2 diabetes women</span>.</p><p class="abstract"><strong>Methods:</strong> <span lang="EN-GB">A prospective, cross-sectional study, which includes 160 women agreed to participate in this study. Anthropometrics, biochemical parameters and blood pressure were measured. Atherogenic indices - total cholesterol-to-high-density lipoprotein cholesterol ratio (TC/HDL) and apolipoprotein (apo) B-to-apo A1 ratio, were calculated. Individual risk factors were examined in relationship to these atherogenic indices using correlation tests and logistic regression</span><span lang="EN-IN">. </span><span lang="EN-IN"> </span></p><p class="abstract"><strong>Results:</strong> <span lang="EN-GB">23.12% of the participants were normal weight and 76.87% were overweight/obese. The overall mean age was 57.70±11.16 years. Diabetes duration (˃5years), anthropometric parameters, poor glycaemic control, high apo B and high level of low-density lipoprotein (LDL) were found to be significant determinants of atherogenic indices changes. </span><span lang="EN-GB">The TC/HDL ratio was weakly associated with both BMI and waist circumference. However, the apo B/apo A1 ratio provided positive correlations with anthropometric parameters, especially with waist circumference (<em>p</em>=0.185, r=0.108, r<sup>2</sup>=0.012), and this, in both pre and post-menopausal type 2 diabetic women</span>.</p><p class="abstract"><strong>Conclusions:</strong> <span lang="EN-GB">The atherogenic risk, estimated by TC/HDL and apo B/apo A1 ratios, becomes more severe with higher anthropometric parameters (BMI and waist circumference), diabetes duration and poor glycaemic control in type 2 diabetes women and this during both premenopausal and postmenopausal periods</span><span lang="EN-IN">. </span></p>
Synchronously detected seminoma of both testis in a middle aged male with bilateral cryptorchidism
Authors: Afsar Nabila , Wase Abdul , Fatima S. Nikhath , Abid Zakia 
Published: September 2016

<p class="abstract">Bilateral testicular tumours are rare and 80% occur metachronously. Synchronous bilateral testicular tumours are rare, and bilateral synchronous testicular tumours in bilateral cryptorchidism are extremely rare with only few cases reported previously. A 36 year old male patient presented to surgical OP with complaints of pain in scrotal region of both sides since 6-8 months. He was asymptomatic previously except for complaints of empty scrotal sac. Magnetic resonance imaging performed 6 months back revealed bilateral large oval to mildly irregular inguinal swellings with iso to hyperintense areas measuring 36×23×20 mm on right side and 34×26×20 mm on left side and patient was diagnosed with bilateral undescended testis. Bilateral orchidectomy was performed and the resected specimen was subjected to histopathologic examination. Gross visual examination of the bilateral orchidectomy specimen revealed the larger specimen to measure 7×4×1 cm with grey coloured external surface. Cut section revealed testis measuring 3.5×2 cm and surrounded with fibro adipose tissue. The testicle shows grey brown and grey white areas surrounding a central yellow nodule measuring 1×0.8 cm. The other mass had a yellowish nodular external surface and measured 5.5×4×1 cm. On cut section, normal testicular tissue could not be identified and the entire testis appeared to be replaced by a solid yellowish white homogenous tumour. Sections from the yellowish smaller mass revealed tumour composed of sheets and trabeculae of large polygonal cells with large vesicular nuclei with prominent nucleoli and abundant clear to vacuolated cytoplasm. Focal collections of histiocytes and occasional multinucleated giant cells were also seen. Sections from the other larger mass showed a thick fibrotic tunica albuginea with sclerosed and hypoplastic seminiferous tubules in areas of dense hyaline fibrosis. In the centre, a well circumscribed tumour was seen showing similar histologic features as in the contralateral mass. Epididymis did not show tumour infiltration.</p>
Level of serum lactate dehydrogenase in oral submucous fibrosis
Authors: Swamy K. Mallikarjuna , Ganiger Arati 
Published: September 2016

<p class="abstract"><strong>Background:</strong> Oral cancer is one of the most common form of malignancies in India. In many cases it develops at the site of premalignant lesion. Of all oral premalignant conditions, oral submucous fibrosis (OSMF) is of greater concern because of its disabling nature and relative greater chances of malignant transformation. Transformation of normal tissue to premalignant lesion and further to oral cancer results in alteration in glycolytic pathway and hence the lactate dehydrogenase (LDH) levels. The aim of this study was to estimate the LDH levels in serum of subjects with OSMF and to compare them with healthy controls and to correlate the relationship between pathogenesis of OSMF and the LDH enzyme.</p><p class="abstract"><strong>Methods:</strong> It is a case control study. The study included 40 diagnosed cases of OSMF and 40 matched healthy controls. Venous blood of 3 ml was collected in both cases and controls. Serum was separated by centrifugation and LDH was estimated by using standard kits. Statistical analysis was done using student ‘t’ test. Pearson's correlation was performed to establish the relationship between study variables. </p><p class="abstract"><strong>Results:</strong> It was observed that serum LDH levels were significantly increased in cases of OSMF as compared to controls (p &lt;0.005).</p><p><strong>Conclusions:</strong> Serum LDH was significantly increased in OSMF and can be used as a valuable biochemical marker in prognosis of OSMF.</p>
A clinical study of symptomatic profile and response in objective and subjective parameters to proton pump inhibitor in laryngopharyngeal reflux
Authors: Y. Kavitha , Dutta Angshuman , K. Sabarigirish , Kumar Joish Upendra 
Published: September 2016

<p class="abstract"><strong>Background:</strong> The study was conducted to identify the various clinical presentations and findings in cases of LPR and also to study the role of proton pump inhibitor in the management of laryngopharyngeal reflux by observing the effect of proton pump inhibitor on reflux finding score (RFI) and reflux symptom index (RSI).</p><p class="abstract"><strong>Methods:</strong> A prospective, interventional, cohort study of 50 diagnosed cases of LPR with RSI &gt;13 and RFS &gt;7 (based on fibreoptic laryngoscopic findings) at the time of initial presentation was done. They were treated with a 6 month course of oral cap omeprazole (20 mg twice daily 30 minutes before meals) and followed up at 0, 4, 8 and 24 weeks for any improvement in RSI and RFS scores. </p><p class="abstract"><strong>Results:</strong> Most frequent symptoms detected in the cases were frequent clearing of throat, dry cough and sensation of lump in the throat all of which showed significant improvement at follow up at 4 weeks. Most common finding on fibreoptic laryngoscopy was erythema and diffuse laryngeal oedema, both of which showed significant improvement on follow up.There was also a significant change in RSI and RFS after receiving Omeprazole at 4 weeks, 8 weeks and at 24 weeks duration (p &lt;0.0001).</p><p><strong>Conclusions:</strong> This study dealt mainly with study of combination of clinical features that LPR presents time line of improvement of these features with capsule omeprazole. It is proposed that a presumptive diagnosis of LPR can be made based on the criteria of RSI and patients be given an empirical therapeutic trial including behavioral and dietary recommendations and 6 months of twice-daily proton-pump inhibitor therapy for an excellent clinical response.</p>
Widening techniques for stomal stenosis: Y-V advancement vs. two circular incisions technique
Authors: Kamath P. Shrinath D. , Cherian Elizabeth , Bhat Vadish S. 
Published: September 2016

<p class="abstract"><strong>Background:</strong> Tracheostomal stenosis following total laryngectomy for carcinoma of larynx is a challenging condition for an otolaryngologist, especially when the patient requires post-operative radiotherapy. Critical narrowing of the stoma needs surgical widening. Though there are few surgical techniques described for the same, re-stenosis has been observed in many. We conducted a comparative study of two widening techniques: Y-V advancement and two circular incisions technique.</p><p class="abstract"><strong>Methods:</strong> A prospective study was conducted, to assess the outcome of 2 surgical techniques: Y-V advancement and two circular incisions technique in patients with tracheostomal stenosis following total laryngectomy. Initially three patients were subjected to Y-V advancement technique for stomal dilatation. But all three patients presented with re-stenosis in a mean period of 8 months and were then subjected to stomal widening by the double circular incisions technique. The next five patients who presented with stomal stenosis underwent widening by two circular incisions technique. All 8 patients were followed up for one year postoperatively. </p><p class="abstract"><strong>Results:</strong> Among the 8 patients, 3 patients who presented initially underwent stomal widening by the Y-V advancement technique. All 3 patients presented with re-stenosis in a mean period of 8 months post-operatively. Stomal widening using the two circular incisions technique was adopted for these three patients and another 5 patients who presented with stomal stenosis. All 8 patients were found to be maintaining an adequate stoma on one year post-operative follow up.</p><p><strong>Conclusions:</strong> In our experience, stomal widening by the two circular incisions technique has found to be effective in maintaining an adequate stoma with no features of re-stenosis on a one year postoperative follow up. However a larger study is desirable to conclusion of the same. </p>
Comercio Justo: ¿Contradictio in Adiecto?
Authors: Ushakova Tatsiana 
Published: September 2016

El presente estudio pretende analizar el concepto “comercio justo” en tres ámbitos de conocimiento muy distintos. La reflexión se construye en torno a tres “variaciones” inspiradas en fuentes de registros casi incompatibles. La primera variación presenta una aproximación al comercio justo desde la perspectiva lingüística, y plantea la posibilidad de considerar el término como oxímoron, por analogía con el término “industria cultural”, acuñado por Horkheimer y Adorno, y de alguna manera, privado de existencia hoy en día. La segunda variación retoma el razonamiento anterior desde un ángulo filosófico, anclado en la teoría crítica como punto de partida, y con referencia a la idea de la negación, propia de la dialéctica de Hegel. Finalmente, la tercera variación se fundamenta en un argumento jurídico, al construir la oposición en las ideas de “comercio global”, desde el enfoque intergubernamental de la OMC, y de “comercio justo”, desde la plataforma de la OMCJ.
Con todo, no se aspira a dar una respuesta, sino a buscarla. ¿Qué es el comercio justo? ¿Un oxímoron que permite “la estetización de la realidad social”? ¿Un objeto del análisis dialéctico? ¿Un mito en el contexto del comercio global?
Las Investigaciones Sociojurídicas Acerca de la Eficacia y Efectividad del Derecho; Algunas Alternativas Metodológicas
Authors: Acosta Rolando Pavó 
Published: September 2016

Uno de los temas fundamentales de la Sociología Jurídica presente y futura lo constituye el de la eficacia y efectividad del Derecho, puesto que tiene que ver con la realización del Derecho objetivo y de los derechos subjetivos de los individuos, los grupos sociales, las empresas y de otros sujetos, y en consecuencia con la legitimidad de los sistemas políticos y con el desarrollo económico sustentable.
La noción sobre eficacia del Derecho, las diferencias y coincidencias entre eficacia del Derecho, efectividad de las normas jurídicas y eficiencia del Derecho, los factores jurídicos y otras causas que determinan el grado de eficacia de las normas jurídicas y de los derechos subjetivos tales son las fundamentales cuestiones a las que se ofrece respuesta en el presente trabajo. Este trabajo también propone varios modelos que contienen las dimensiones y variables que deben tenerse en cuenta en la medición empírica sobre la eficacia de las leyes y en la determinación de los factores jurídicos que determinan sobre los niveles de eficacia y la ineficacia de las leyes.
ABCD analysis of Dye-doped Polymers for Photonic Applications
Authors: Aithal Shubhrajyotsna , Aithal P. S. 
Published: September 2016

<div><p><em>Photonics is a subject of scientific study on generating, controlling, harvesting and detecting the beam of photons or light with a purpose of creating, manipulating, storing, transmitting and detecting information using nonlinear optical properties of materials. This paper is a new attempt to integrate scientific research and social research by analysing the characteristics of dye-doped polymer films for photonics applications. The analysing framework called ABCD framework to analyse any business concepts, business systems, technology, strategy, engineering material, technology or even an idea systematically by identifying the advantages, benefits, constraints, and disadvantages under various determinant issues and listing the constituent critical elements under each construct. In this paper, as per the ABCD framework, the various determinant issues related to the use of dye-doped polymer films for photonic applications through focus group method are determined as affecting factors under : (1) Material Issues, (2) Application Issues, (3) Commercialization Issues, (4) Production/Service providers Issues, (5) Customer Issues, and (6) Environmental/Social Issues. The constituent critical elements of these factors are listed under the four constructs - advantages, benefits, constraints and disadvantages of the ABCD technique and tabulated. The analysis has brought about 204 critical constituent elements which satisfy the success of this analysis methodology. <strong></strong></em></p></div>