bluuebunny/crossref_metadata_2025_split huggingface dataset:
Therapeutic challenges in a patient with the simple virilizing (SV) form of congenital adrenal hyperplasia (CAH) due to the P30L/I172N genotype
Authors: Tankoska Maja , Anastasovska Violeta , Krstevska-Konstantinova Marina , Naydenov Michel , Kocova Mirjana 
Published: April 2019

Abstract

Background
Steroid 21-hydroxylase deficiency is an autosomal recessive disorder, present in 90–95% of all cases with congenital adrenal hyperplasia (CAH). The classical simple virilizing (SV) form of the disease causes virilization of the external genitalia in newborn females and pseudo-precocious puberty in both sexes, due to reactive androgen overproduction.

Case presentation
We describe a 3.5-year-old girl presenting with pubarche, P2 according to Tanner, advanced bone age of 6 years and 10 months, and high serum levels of 17-hydroxyprogesterone (17-OHP). Molecular analysis of the nine most common pseudogene-derived CYP21A2 point mutations was performed in the patient and her family members using the polymerase chain reaction/amplification-created restriction site (PCR/ACRS) method. We detected the P30L/I172N genotype in the patient. She had inherited a mild P30L mutation from her mother and a severe I172N mutation from her father.

Conclusions
Although the CAH phenotype is determined by the allele that produces most of the enzyme activity and the mild non-classical (NC) phenotype should be expected, the mild P30L known to be more virilizing probably induced the classical SV phenotype in our patient. A continuous regimen of hydrocortisone at a recommended dose failed to decrease the 17-OHP sufficiently. Careful tapering of the dose did not help, and her pubic hair advanced to P3 according to Tanner. Individually tailored treatment is warranted in this patient.
Educational Series in Congenital Heart Disease: Three-dimensional echocardiography in congenital heart disease
Authors: Simpson John M , van den Bosch Annemien 
Published: April 2019

AbstractThree-dimensional echocardiography is a valuable tool for the assessment of cardiac function where it permits calculation of chamber volume and function. The anatomy of valvar and septal structures can be presented in unique and intuitive ways to enhance surgical planning. Guidance of interventional procedures using the technique has now become established in many clinical settings. Enhancements of image processing to include intracavity flow, image fusion and true 3D displays look set to further improve the contribution of this modality to care of the patient with congenital heart disease.
Atskyrimo (khōrismos) doktrinos kritika Aristotelio Metafizikoje
Authors: Petuška Virgilijus 
Published: April 2019

[straipsnis ir santrauka lietuvių kalba; santrauka anglų kalba]
Straipsnyje nagrinėjama atskyrimo (khōrismos) sąvoka (tezė, kad eidai ir skaičiai egzistuoja atskirai (khōris) nuo jusliškai suvokiamų esybių) bei jos kritika Aristotelio Metafizikoje. Straipsnyje siekiama įrodyti, kad nors Aristotelis Metafizikoje eidų ir skaičių atskyrimo doktrinas kritikavo skyrium viena nuo kitos (kaip dvi Platono metafizinio mokymo dalis), vis dėlto jas laikė neatskiriamai susijusiomis – iš skaičių atskyrimo, pasak Aristotelio, kildintinas ir eidų atskyrimas jusliškai suvokiamų objektų atžvilgiu. Ši straipsnio pagrindinė tezė grindžiama bendra Metafizikos knygų M ir N problemine struktūra, Aristotelio pateikiamais liudijimais apie eidų teorijos atsiradimą bei šių liudijimų interpretacija remiantis senovės graikų mąstymui būdingu skaičiaus fenomeno suvokimu.
Why Kant is a Weak Conceptualist
Authors: Baranovas Ruslanas 
Published: April 2019

[full article, abstract in English; abstract in Lithuanian]
The question whether Kant is a conceptualist has attracted significant attention of Kant scholars in recent decades. I present all three dominant positions in the debate (strong conceptualism, weak conceptualism, nonconceptualism) and argue that strong conceptualism and nonconceptualism are less plausible interpretations of Kant’s philosophy. I argue that the first cannot explain Kant’s commitments related to the incongruents, animals, and infants. The second one, meanwhile, cannot explain Kant’s argument on causation against Hume. At the end of the paper, I try to show that the key to a plausible and convincing interpretation of Kant as a weak conceptualist is the distinction between categories and empirical concepts.
Reduction in Unintended Extubations in a Level IV Neonatal Intensive Care Unit
Authors: Galiote John P. , Ridoré Michelande , Carman Jessica , Zell Lisa , Brant Karen , Gayle Cheyenne , Short Billie L. , Klugman Darren , Soghier Lamia 
Published: April 2019

OBJECTIVES:
Unintended extubations (UEs) lead to significant morbidity in neonates. A quality improvement project was initiated in response to high rates in our level IV NICU. We targeted creating and sustaining UE rates below the published standard of 1 per 100 ventilator days.

METHODS:
This project spanned 4 time periods: baseline, epoch 1 (December 2010–May 2012), sustain, and epoch 2 (May 2015–December 2017) by using standard quality improvement methodology. Epoch 1 interventions included real-time analysis of UE events, standardization of taping, patient positioning and movement, accurate event reporting, and change in nomenclature. Epoch 2 interventions included reduction in daily chest radiographs (CXRs) and development of a high-risk tool. Patient and event characteristics were statistically compared across time points.

RESULTS:
Of the 612 UE events recorded over 10 years, 249 UEs occurred from May 2011 to 2017 involving 184 unique patients. UE rates decreased by 43% (from 1.75 to 0.99 per 100 ventilator days; epoch 1) and were sustained until a notable spike. Epoch 2 interventions led to a further 31% rate reduction. Single CXR use decreased by half. Median corrected gestational age at the time of an event was 35 weeks (interquartile range: 29–41). Seventy percent of infants experiencing an UE required reintubation, 29% had a previous event, and 9% had a code event.

CONCLUSIONS:
A decrease in UE below benchmarks can be achieved and sustained by standardization and mitigation interventions. This decline was also accompanied by a reduction in use of CXRs without increasing UE events.
La autocorrelación espacial y el desarrollo de la geografía cuantitativa
Authors: Siabato Willington , Guzmán-Manrique Jhon 
Published: April 2019

¿Cómo varía UN fenómeno a través del espacio geográfico? Esta es la pregunta que ayuda a resolver la autocorrelación espacial. En este editorial se presenta una breve introducción al concepto de autocorrelación espacial y a los métodos de análisis usados para clasificar y cuantificar el comportamiento de un fenómeno a través del espacio. Además, se presenta una contextualización histórica sobre su origen y desarrollo, y una serie de elementos que se espera ayude a aclarar las posibles confusiones entre la correlación y autocorrelación espacial. Las descripciones y conceptos expuestos no son desarrollados con rigurosidad matemática ni detallando la formalización de cada método, por el contrario, se presentan como discusión conceptual que describe los fundamentos y detalles que se consideran necesarios para conocer y comprender la base de los principales métodos estadísticos espaciales hasta ahora desarrollados para analizar la variación de los fenómenos geográficos. El objetivo de estas notas es que sirvan de soporte para cursos introductorios de análisis espacial.
Еfficacy of Combined Treatment of Epithelial and Endothelial Corneal Dystrophy Using Corneal Crosslinking and Automated Posterior Lamellar Keratoplasty
Authors: Marvanova L. R. 
Published: April 2019

The purpose: to establish a combined approach to the treatment of patients with epithelial and endothelial cornea dystrophy (EED) based on a comparative study of the results of one-step and two-step methods. Patients and methods. The study included 75 patients (81 eyes) with corneal EED, who underwent surgical treatment at the Ufa Eye Research Institute from 2011 to 2016. The patients were divided into two groups — the main group consisted of 43 (46 eyes, 57 %) patients, who underwent the first stage of the CC before surgical treatment of EED, the second stage after 1–12 months — automated posterior lamellar keratoplasty (APLK). In the control group, isolated APLK was performed in 32 (35 eyes, 43 %) patients. Results. After CC (1–10 days) in the main group an increase in corneal thickness due to edema enhancement in the cornea stroma was observed in patients with stage I–III of the corneal EED. After 3 months, a decrease in corneal thickness was recorded in patients of the main group with I-II stages of the disease (p < 0.05), after 6 months — in all stages of the corneal EED compared with the control group (p < 0.05). According to optical coherence tomography (OCT), a decrease in the total cornea thickness in both зфешутеы groups was noted within 12 months after APLK: in the main group from 667 ± 65 μm initially to 594 ± 31 μm, in the control group, where there was a pronounced corneal edema from 787 ± 56 to 612 ± 67 μm. Conclusion. Corneal cross-linking in patients with I–III stages of corneal EED provides improvement of the cornea, manifested in reducing edema and its thickness. It allows to delay the implementation of the automated posterior lamellar keratoplasty without corneal deterioration in patients with stage I up to 6–12 months, in patients with stage II–III up to 3–6 months.
Мodern Approaches to the Treatment and Prevention of Recurrent of Ophthalmic Herpes. Review
Authors: Zaynutdinova G. Ch. 
Published: April 2019

The review focuses on modern treatment aspects and prevention of ophthalmic herpes.The generalized experience of the clinical nucleoside administration, the basic antiviral drugs in the treatment of this pathology, is presented in detail. Information about indications, side effects and various schemes for their systemic and local application is provided. Questions on the duration of nucleoside administration in various clinical forms of eye herpes are considered. A meta-analysis of the comparative therapeutic nucleoside efficacy based on the results of more than 100 placebo-controlled randomized trials is presented. Special attention is paid to the rare but severe eye pathology, the acute retinal necrosis. Also data on the indications and contraindications of corticosteroids and on the current view of immunotherapy of ophthalmic herpes is presented. The question of the modern approach to the disease prevention recurrences is considered in detail, particularly, the results of the nucleoside administration have a high level of evidence, and, herpetic vaccines in the rehabilitation of patients with herpes infection are not widely used due to the lack of reliable methods for evaluating the efficacy. The information on the search for new effective drugs that have a targeted effect on certain patient cell receptors that can inhibit the penetration and spread of the herpes virus that can cause a controlled immune response is provided. Expanding the arsenal of effective and safe antiviral therapy will definitely optimize the treatment of ophthalmic herpes and prevent the occurrence of its recurrence and severe outcomes.
Morphometric Analysis of Corneal Nerves Condition after Laser Keratorefractive Surgery
Authors: Avetisov S. E. , Tyurina A. A. , Surnina Z. V. , Dovgileva O. M. , Sukhanova E. V. 
Published: April 2019

Purpose: to conduct a morphometric assessment of the corneal nerves state after laser keratorefractive surgery using laser confocal microscopy and the software Liner 1.2S. Patients and methods. 40 eyes of 20 patients received LASIK to correct a mean refractive error of –4.5 ± 1.9 diopters (range, –2.25 to –8.25 diopters). The stroma was ablated with a Teneo Technolas 317P excimer laser. Corneas were examined by laser corneal confocal microscopy (HRT III with Rostock Cornea Module) before and in 1, 3, and 6 months after LASIK. Images were analyzed with software that automatically computes coefficients of anisotropy and orientation symmetry of corneal nerves. Result. At 1 and 3 months after surgery, the center corneal nerves were completely absent. At 1, 3 and 6 months after surgery, the paracentral corneal nerves were detected and the coefficient of anisotropy were statistically significantly reduced compared with the preoperative rates. At 6 months after surgery, both central and peripheral corneal nerves were detected and the coefficient of anisotropy were statistically significantly reduced compared with the preoperative rates. Conclusion. As a result of the study, the central corneal nerves were first detected at 6 months after surgery, whereas the paracentral cornea nerves were detected already after 1 month. At 6 months after surgery, the coefficient of anisotropy in the central of cornea statistically significant decreased compared to the preoperative rates. At all stages of the study after surgery, the anisotropy coefficient in the cornea paracentral statistically significant decreased compared with preoperative.
Etiology and outcome of patients with viral-induced acute liver failure
Authors: Nabi Tauseef , Rafiq Nadeema 
Published: April 2019

Background: Acute liver failure (ALF) is a rare medical emergency and devastating clinical syndrome associated with high mortality in the absence of immediate intensive supportive care, specific treatment, or liver transplantation. Viral hepatitis is still one of the main causes of ALF in the India as well in world. We aimed to determine the etiology of Viral-ALF and to compare the outcome and clinical and biochemical variables in patients with hepatitis E and non HEV group in this prospective study.Methods: A total of 37 patients with a diagnosis of viral-ALF were included in the study. The variables evaluated were demographic, signs and symptoms, biochemical parameters, severity of liver injury, outcome, complications and duration of hospital stay.Results: Out of 37 Viral-ALF patients, Acute HEV-induced ALF (48.6%) was most common followed by HBV (24.3%) and HAV (21.6%). There were significantly more females in HEV group (61.1%) than non HEV group (21.1%) (P = 0.014). Overall mortality was 20 (54.1%). Mortality was higher in non HEV group (73.7%) than HEV group (33.3%) (P = 0.015). The mean age in HEV group was 30±12.7 years and in non HEV group was 38.1±10.4 years (P = 0.042). Bilirubin, ALT, mean grade of coma and interval between jaundice and encephalopathy were significantly higher in non HEV group than HEV group. MELD Score was higher in non HEV group 32.6±7.9 than HEV group 26.3±7.2 (P = 0.012). Sepsis and renal failure occurred more frequently in non HEV group than HEV group. Duration of hospital stay was also significantly more in non HEV group 11.3±3.3 days versus HEV group was 7.9 ± 2.9 (P = 0.002).Conclusions: HEV was the most frequently associated with Viral-ALF. HEV related ALF disproportionately affected young women. Mortality was higher in non HEV group (73.7%) than HEV group (33.3%). The marked difference in spontaneous survival between HEV and non HEV group can be explained by the severity of hepatic dysfunction on admission and more frequent complications.
Secondary hemophagocytic lymphohistiocytosis: a rare case report
Authors: Balamurugan Venkataraman , Jayanthi Bharathi Vidhya 
Published: April 2019

Haemophagocytic lymphohistiocytosis (HLH) is a clinicopathologic syndrome, characterised by hyperinflammation due to inherited or acquired defects in the immune function, leading to unchecked proliferation of histiocytes and lymphocytes resulting in multiorgan dysfunction. HLH can be primary (familial) occurring in young children caused by underlying genetic defects in natural killer cells/cytotoxic T cells or secondary HLH occurring in older children or adults following infections, rheumatological disorders or malignancies. HLH is a medical emergency, having varied clinical presentations and lacks a pathognomonic clinical or laboratory abnormality. Clinical presentations include unexplained fever, hepatomegaly, splenomegaly, skin rash, cytopenias, liver dysfunction, coagulation abnormality and neurological manifestations. It carries a poor prognosis. Early diagnosis based on HLH 2004 criteria and initiation of treatment is crucial in the management strategy, which is likely to improve the outcome of this life-threatening disease. The treatment strategies include immunosuppressive drugs, immunomodulatory therapy and autologous hematopoietic stem cell transplant in selected cases. Here with authors report a case of young adult, presenting with fever, thrombocytopenia, splenomegaly, and multi organ dysfunction, diagnosed as a case of secondary HLH based on the HLH 2004 guidelines.
Local facial flaps: a workhorse for reconstruction of facial malignancies defects
Authors: Rolekar Nikita G. , Goil Pradeep , Rao Jagdeep 
Published: April 2019

<p class="abstract"><strong>Background:</strong> Face represents complete personality of a human being, so adequate cosmetic correction of facial defects arising due to skin malignancy is very important. After excision, treatment option varies according to size and location of defect for small- or moderately-sized circular defects. In our study, we have evaluated versatility of local flap of face.</p><p class="abstract"><strong>Methods:</strong> We have included 30 cases of skin malignancy on face in our Institute within the period of June 2016 to May 2018. 20 patients (66.6%) were male and 10 patients (33.3%) were female, in age group from 45 to 65 years. 17 cases had basal cell carcinoma, 10 cases had squamous cell carcinoma and 3 cases had malignant melanoma. Tumours were excised with safe margins and defects reconstructed with local facial flaps. We evaluated the early postoperative complications and after 6months, cosmetic outcome and patient satisfaction was evaluated. </p><p class="abstract"><strong>Results:</strong> 30 patients with small to medium-sized defect (3-6 cm), 8 cases were managed with V-Y advancement flap, 7 cases nasolabial flap, 8 cases forehead flap and, 4 cases Limberg flap, 4 cases cheek advancement flap. Postoperatively none of the cases had complications and the functional and aesthetic outcomes were quite acceptable. Aesthetic results were excellent in 15 patients (50%), while in 8 patients good results (27%) and 5 patient fair results (17%) after 6 month.</p><p class="abstract"><strong>Conclusions:</strong> Local facial flaps are the simple and best option to reconstruct the small to medium size facial malignancies defects and provides excellent skin colour and texture match with gives good aesthetic result.</p>
FORMATION OF DUTY BEHAVIOR WITH CONSIDERATION OF THE PRESENCE OF THE NON-GOVERNMENTAL ECONOMY
Authors: Kuzmynchuk Nataliia , Nazarova Tetiana , Drugova Olena , Vyadrova N. G. , Chkheailo Anna 
Published: April 2019

In this study, the main attention is paid to the problem of "shadowing" the Ukrainian economy during the crisis period. Theoretical approaches to determining the functions of taxes and factors affecting real tax revenues were studied. A scientific study was carried out that will reveal the motives of the behavior of taxpayers, who are explicitly or implicitly laid in the basis of approaches to the formation of the mechanism of taxation and tax policy of the state. Consequently, we tried to determine motives of the behavior of taxpayers, which explicitly or implicitly form the basis of approaches to the formation of the tax mechanism and the tax policy of the state. So, we tried to determine the level of the shadow economy, taking into account the modeling of the dynamics of tax behavior of economic entities, which, of course, will allow to increase the efficiency of management of the budget process, in particular, planning of budget expenditures to regional authorities.The article defines the necessity of improving the tools of state regulation of the economy, taking into account the specific conditions of development, and creates scientific and methodological support for the formation of concepts, strategies and programs of socio-economic development of the country and regions by the authorities. The theoretical positions concerning the role of the state in the regulation of fiscal-budgetary relations in the light of the evolution of the views of the known economic schools are outlined, and the expediency of introducing the views of well-known economic schools into practice has been outlined, which will help to develop regulatory measures to overcome negative phenomena in the economyIt is generally agreed that evasion of payment of taxes and fees leads to a number of negative consequences for both state power, and for the country's society (the threat of development, the collapse of the national economy, the negative influence on the distribution of resources redistribution between the spheres of economy, the threat to democratic values and social institutions). Systematically conceptualized the motives of the behavior of the subtitles, which were used as the basis for the formation of the mechanism of the podcasting and subjective policy of the state. Factors influencing the regulatory policy of the country and the factors influencing the adoption of decisions by the parties associated with the subtraction of subtractions are considered. The structure of the mechanization of the formation of a subtle behavior is proposed. The problems of tax evasion were identified and countermeasures were proposed to facilitate the development of an effective state anti-political policy in Ukraine.