bluuebunny/crossref_metadata_2025 huggingface dataset:
Congenital malignant melanoma- the unique entity of pediatric melanoma: two case reports and literature review
Authors: Xu Kailun , Sun Zhaoyun , Li Can , Wang Lifeng , Guan Wenbin
Published: February 2022

Background: Pediatric malignant melanoma (MM) is categorized by the age as congenital, childhood and adolescent subtypes. Congenital melanoma is present at birth or in the first year of life. It is an exceedingly rare entity for the low incidence and difficult diagnosis. Here, we present two cases of congenital malignant melanoma. Besides, we describe the special clinicopathologic and molecular features of pediatric MM especially congenital sub-type by reviewing the literature. Case presentation: We report two cases of congenital malignant melanoma. One is a boy patient who had been born with a mass in the occipital scalp, the other newborn girl presented with a mass at birth on her right shoulder. Two patients all underwent the excision of the nodular lesion. Histological evaluation revealed the boy case is pigmented epithelioid melanocytoma (PEM), a unique low-grade variant of malignant melanoma of the skin. The other is congenital melanoma. After the operation, the boy patient presented with cervical lymph node metastasis on the third postoperative month, but is still alive and is developing well. The girl patient recovered, with no recurrence or metastasis since follow-up.Conclusions: Congenital melanoma is a rare sub-type of pediatric melanoma. Accurate diagnosis of congenital MM is challenging and requires the combination of clinical presentation, pathologic and molecular findings. The cases we offered will contribute to the understanding of epidemiological features and molecular biology of congenital MM and pediatric MM.
Promoting sleep and other simple, non-pharmacological interventions for preventing and treating ICU Delirium: case report
Authors: Chou Shinn-Te , Pogach Melanie , Rock Laura K.
Published: February 2022

Delirium is common among critically ill patients and it prolongs the length of ICU and hospital stays and is associated with worse outcomes, including cognitive impairment and increased mortality. No medications have been shown to have a consistent therapeutic benefit. Delirium is a form of brain dysfunction characterized by the acute onset of fluctuating mental status, inattention, disorganized thinking, and altered level of consciousness. These features are also seen in states of acute sleep deprivation. It is believed that the state of delirium contributes to disrupted sleep just as sleep deprivation is considered a modifiable risk factor for delirium. Improving sleep conditions and other nonpharmacologic interventions to promote sleep may be an underused approach to preventing and treating delirium in critical illness.
Management of Complications After Skin Surgery Relevant for Melanoma in the Trunk and Extremities Under the COVID-19 Pandemic: A Case Series Report
Authors: Huang Yinglai , Carlsson Lena , Jogeland Karin , Samuelsson Marianne , Larsson Lars , Jonsborg Catarina
Published: February 2022

Background: Patients with melanoma have been found to be at greater risk of adverse outcomes including mortality after contacting COVID-19. Management of postsurgical complications presented additional challenges by potentially increasing exposure to COVID-19 through repeated inpatient admissions to hospital during the pandemic. We report four cases for which skin flaps, lymph ligation and split-thickness skin graft (STSG) were successfully used in the treatment of complications in the trunk and extremities after wide local excision (WLE). This study details the operative experience in management of postsurgical complications for melanoma in the trunk and extremities during a 6-month period at the height of the COVID-19 pandemic Case presentation: We present 4 cases detailing management of complications that occurred after wide local excisions performed for melanoma during Feb. to Oct.-2020. Case 1: a 90-year-old man who experienced wound dehiscence and necrosis on the shoulder after non- radical excision for an aggressive melanoma, underwent the side-to-side closure after ellipse formed WLE with modified tangent-to-circle method. Case 2: an 80-year-old man who had undergone excision for melanoma in his left upper arm, histopathology did not show radically. Two weeks after the excision, he underwent a WLE and direct reconstruction with double rotation skin flap. Case 3: a 55-year-old man that experienced a large wound dehiscence on his back due to WLE. He underwent an advanced double skin flap operation. Case 4: a 36-year-old woman who had a lymphorrhea and graft necrosis after WLE and STSG on the right lower leg. A combination of micro lymph ligation and re- STSG was performed. One month after the operation, all wounds had healed. There was no clinical evidence of tumor recurrence after eight months post procedure. Conclusions: Severe complications (e.g., large wound dehiscence, necrosis or lymphorrhea) following wide local excision of melanoma are infrequent but must be swiftly and appropriately managed, especially during the COVID-19 pandemic to decrease the likelihood of COVID-19 infection and impaired oncology outcomes from delaying systemic cancer therapy due to the complications in primary interventions.
Glycogen storage disease Ia complicated with multiple hepatic adenomas: a case report and literature review
Authors: Tian Sufang , zhang Wanyu , Fan Wei , Zhang Zhonglin
Published: February 2022

Background: Glycogen storage diseases (GSDs) are a rare group of autosomal recessive genetic disease. The pathogenesis of GSD is derangement of carbohydrate metabolism and results in abundant glycogen accumulation within tissues. Clinically, patients suffering from GSD are characterized with growth retardation, puberty delay, hyperuricemia, hypoglycemia and hepatomegaly, occasionally, complicated with hepatic adenoma.Case presentation: A 29-year-old man was admitted to our hospital with enlarged liver masses after a 2-year-long follow-up. Physical examinations showed he was 160cm in height, 50kg in weight and many tophi in his joints. Laboratory examinations showed hypoglycemia, elevated uric acid and free fatty acids, but negative tumor markers. After reviewing his clinical history, GSD Ia was suspected for the underlying disease and complicated with multiple hepatocellular neoplasm. The precise diagnosis was made finally by combining pathology examination, biochemical assays and whole exome sequencing (WES).Conclusions: The present case would be conductive for general pathologists to recognize the clinicopathological features of GSD and prompt diagnosis. On patients’ side, it will be vital for their timely follow-up and management.
Exploring intervention components grounded in critical race theory, harm reduction, and self-determination theory: A qualitative study with Black and Latino persons living with HIV in the United States
Authors: Cluesman Sabrina , Gwadz Marya
Published: February 2022

Background
. The persistence of racial/ethnic disparities in the United States in rates of engagement along the HIV care continuum signals the need for novel approaches. We recently described a new integrated conceptual model (ICM) that combines critical race theory, harm reduction, and self-determination theory. This ICM guided the development of six behavioral intervention components tested in optimization trial (Core component, Component A: motivational interviewing sessions, Component B: pre-adherence skill building, Component C: peer mentorship, Component D: focused support groups, Component E: navigation). The present qualitative exploratory study describes participants’ perspectives on the components’ acceptability, feasibility, and impact.
Methods
. Participants were African American/Black and Latino PLWH poorly engaged in HIV care and with non-suppressed HIV viral load in New York City. From a larger optimization trial (N = 512), we randomly selected 46 participants for in-depth semi-structured interviews. Interviews were audio-recorded and transcribed verbatim, and data were analyzed using directed content analysis. Structured data on components’ acceptability and feasibility were collected.
Results
. On average, participants were 49 years old (SD = 9) and had lived with HIV for 19 years (SD = 7). Most were male (78%) and African American/Black (76%). Participants reported a constellation of serious mainly social and structural challenges to HIV management including chronic poverty, unstable housing, and stigma. Components were found acceptable (> 70% rated them as quite a bit to very helpful). Feasibility was high: attendance rates for each component was > 80%. For all components, a non-judgmental, pressure-free, and structurally and culturally salient approach was seen as vital and described as lacking in most medical/social service settings. Prominent aspects of components, consistent with the ICM, included establishing trust (core); developing intrinsic motivation and self-reflection (Component A); learning/practicing adherence strategies and habits (Component B); reducing social isolation via peer role models (Component C); reflecting on salient goals and challenges with peers without stigma (Component D); and circumventing structural barriers to HIV management, including related to systemic racism (Component E).
Conclusions
. The ICM has potential to improve the acceptability and feasibility of interventions and services for AABL-PLWH, key underpinnings of intervention/treatment effectiveness. Future study of the ICM is warranted to address racial/ethnic health disparities.
Spatial Phase Synchronisation of Pistachio Alternate Bearing
Authors: Sakai Kenshi , Brown Patrick , Rosenstock Todd , Upadhyaya Shrini , Hastings Alan
Published: February 2022

Collective dynamics of chaotic oscillators has attracted much attention in many fields even including agriculture and forestry. Alternate bearing of tree crops is a phenomenon in which a year of heavy yield is followed by a light yield. This phenomenon has been modelled using a tent map known as a resource budget model (RBM). We applied in-phase/out-of-pahse analysis to yield data of a 9,562 pistachio plants population and revealed phase transitions and mode locking in the orchard. Using a developed network model consisting of diffusively coupled chaotic oscillators (RBMs) on which common noise imposed, we theoretically confirm the phase transitions mode locking and 1/3 power-law scaling in the yield data. Here, we demonstrate how three essential factors, i.e. common noise, direct coupling, and cropping coefficient gradient, explain the spatial synchrony of the orchard.
Plasmablastic Lymphoma in HIV-Positive Patients: A Literature Review and Results of a Russian Multi-Center Retrospective Study
Authors: Popova Marina Olegovna , Tsygankov I.V. , Gudozhnikova Ya.V. , Rogacheva Yu.A. , Volkov N.P. , Lepik K.V. , Demchenkova M.V. , Grigoreva M.V. , Efirkina A.Yu. , Shneider T.V. , Kopeikina Yu.V. , Stepanova S.A. , Potapenko V.G. , Klimovich A.V. , Medvedeva N.V. , Kolesnikova M.A. , Pospelova T.I. , Mikhailova N.B. , Baikov V.V. , Kulagin A.D.
Published: February 2022

Background. Plasmablastic lymphoma (PBL) is a rare lympho-proliferative disease which is almost exclusively associated with immunodeficiency. Most ample experience of chemotherapy and hematopoietic stem cells transplantation (HSCT) in this lymphoma variant has been accumulated in HIV-positive patients. Aim. To describe the current approaches to PBL diagnosis and treatment in HIV-positive patients as well as to provide the results of the first multi-center retrospective study on PBL epidemiology and therapy efficacy in HIV-positive patients in the Russian Federation. Materials & Methods. The study included 26 HIV-positive patients with PBL who were treated and followed-up at 5 Russian centers during 2012-2019. The present study is a part of multi-center retrospective study on lymphoma epidemiology in HIV-positive patients in Russia. Results. PBL accounted for 9.5 % of all lymphomas in HIV-positive patients enrolled in multi-center retrospective study on lymphoma epidemiology in HIV-positive patients in Russia. Epidemiological characteristics of these patients corresponded to those described in previously published literature: the disease being diagnosed mainly at late stages (88 %), oral and nasal mucosa lesions with a common involvement of facial bones (65 %), and lack of optimal HIV-infection control (66.7 %). Most commonly, the patients received EPOCH-like treatment as first-line therapy (50 %). However, the efficacy of primary therapy appeared to be low. Overall survival (OS) and progression-free survival (PFS) during a year after first-line therapy onset was 57 % and 46 %, respectively. Bortezomib included in first-line therapy was associated with a trend to a more favorable prognosis. Half of patients showed a lymphoma relapse or progression after first-line therapy. Most used second-line regimen was DHAP. Overall response to second-line therapy was 38.5 %. After second-line therapy onset, 1-year OS and PFS were 26 % and 15 %, respectively. Conclusion. HIV-positive patients with PBL have poor prognosis. Efforts to improve the prognosis for HIV-positive patients with PBL should be aimed at increasing the efficacy of first-line therapy and should involve the use of intensive chemotherapy regimens with bortezomib. The role of auto-and allo-HSCTs in the treatment of PBL has not been clearly determined, however, PBL patients, despite their HIV-infec-tion, should be regarded as auto-HSCT-eligible in the first remission and allo-HSCT-eligible in case of relapse. Further prospective multi-center studies are needed to optimize the treatment of HIV-positive patients with PBL.
Russian Prospective Non-Randomized Clinical Study on Dose Reduction of Tyrosine Kinase Inhibitors with Subsequent Complete Therapy Discontinuation in Chronic Myeloid Leukemia Patients with Stable Deep Molecular Response (READIT-2020): Background, Aim, Main Objectives, Design, and Expected Results
Authors: Turkina A.G. , Gurianova Margarita Anatolevna , Chelysheva E.Yu. , Shukhov O.A.
Published: February 2022

Background. A withdrawal of tyrosine kinase inhibitor (TKI) therapy in chronic myeloid leukemia (CML) patients with optimal response, especially in patients with drug toxicity, is a matter of current interest. According to the results of numerous clinical studies, the probability of sustaining treatment-free remission (TFR) in CML patients with deep molecular response (DMR) is about 40-60 %. Great attention has recently been paid to personalized therapy consisting in TKI dose modification aimed at reducing or preventing therapy adverse events. Many large retrospective studies showed that reduced TKI doses in CML patients with major molecular response (MMR) and DMR is a safe therapy option. The follow-up of patients receiving reduced TKI doses is also carried out under prospective clinical studies as a stage prior to therapy discontinuation. This approach shows that the probability of sustaining TFR after the stage of TKI dose reduction is about 70 % which is higher than that after the withdrawal of standard TKI doses. Aim. To present the background, aim, and mai n objectives of the study as well as the design and expected results. Materials & Methods. READIT-2020 (Russian prospective study of REduction And DIscontinuation Treatment of TKI) is a Russian prospective non-randomized clinical study with the main aim of developing a safe management regimen for CML patients with MMR and DMR, who receive reduced TKI doses, with subsequent follow-up in the period of TFR under the control of minimal residual disease. The study is going to enroll 100 patients. Each stage of the clinical study will include a regular molecular genetic monitoring at the central laboratory (National Research Center for Hematology, Moscow). The primary objective is to assess survival without MMR loss (BCR-ABL > 0.1 %) both on reduced TKI doses and during TFR. The primary endpoint is the follow-up period of 12 months after TKI discontinuation. Trial Registration No.: NCT04578847 (Clinicaltrial.gov).
The Role of BAALC-Expressing Leukemia Precursor Cells in the Pathogenesis of Myelodysplastic Syndromes
Authors: Mamaev Nikolai Nikolaevich , Latypova M.V. , Shakirova A.I. , Gindina T.L. , Kanunnikov M.M. , Tsvetkov N.Yu. , Barkhatov I.M. , Bondarenko S.N. , Vladovskaya M.D. , Morozova E.V.
Published: February 2022

The present paper provides evidence for a high detection rate of BAALC gene overexpression, also combined with WT1 gene overexpression, in patients with myelodysplastic syndromes (MDS) and FISH-verified chromosome defects. The BAALC and WT1 gene expression profiling of 16 MDS patients (6 out of them received allogeneic hematopoietic stem cell transplantation) showed an increased BAALC expression in 14 patients. The expression level in 2 patients was near the cut-off. Low expression levels were identified in a female patient with isolated 5q deletion in karyotype and also with its combination with complex karyotype. On the other hand, the highest expression levels were reported in patients with normal karyotype and 3q26 locus rearrangement, which was associated with EVI1 gene overexpression. Since the BAALC expression level, at least in patients with the major (except for М3 and М7) FAB-variants of acute myeloid leukemias (AML), was closely associated with BAALC-producing precursor cells of leukemia clone, a profound study of this phenomenon in MDS patients seems to be important for understanding the finest mechanisms underlying the pathogenesis of AML and AML relapses on the level of precursor cells.
Correlation of the Number of TGFβF1-Expressing Atypical Megakaryocytes with the Degree of Bone Marrow Stroma Fibrosis and Osteosclerosis in Patients with Essential Thrombocythemia and Different Stages of Primary Myelofibrosis
Authors: Chebotarev Dmitrii Ilich , Kovrigina A.M. , Melikyan A.L.
Published: February 2022

Background. As morphological pattern of bone marrow (BM) biopsy samples at advanced stages of clonal evolution in essential thrombocythemia (ET) appears similar to that in the development of post-thrombocythemic myelofibrosis and primary myelofibrosis (PMF), the expression of fibro-genesis factors by atypical megakaryocytes (MKC) acquires increased interest. Aim. To study the expression of the transforming growth factor TGFβF1 by atypical MKC; to relate the number of TGFβF1-positive MKCs with the degree of BM stroma fibrosis and trabecular bone changes in patients with ET and different PMF stages. Materials & Methods. BM biopsy samples of ET and PMF patients, obtained before cytoreductive therapy, were subjected to histochemical study with Gomori stain and Masson trichrome as well as to CD42b and TGFβF1 antibody immu-nohistochemical assays. The degree of myelofibrosis and osteosclerosis was estimated by semi-quantitative method in accordance with the European Consensus guidelines. The morphological characteristics of atypical MKC included the comparative evaluation of nuclear-cytoplasmic ratio. Results. The number of MKCs with high nuclear-cytoplasmic ratio was significantly higher in BM biopsy samples of patients with pre-fibrosis/early PMF (pre-PMF) stage and fibrosis stage of PMF (f-PMF) compared with BM biopsy samples of ET patients. The analysis of TGFβF1 expression showed different numbers of positive MKCs in the study groups. The matching of the number of TGFβF1-positive MKCs with the degree of myelofibrosis and osteosclerosis, with no regard to nosologic entities, revealed significant moderate correlation between these features (r = 0.431, p = 0.001 and r = 0.499, p = 0.001, respectively). In 55 % of pre-PMF patients’ BM biopsy samples, histochemical study with Masson trichrome stain visualized minimal immature osteoid deposits on bone trabeculae. Similar changes were also identified in f-PMF patients’ BM biopsy samples, whereas the ET patients’ samples featured none of them. Conclusion. The results of the study prove that the pathological clone of MKC with TGFβF1 expression affects myelofibrosis and osteosclerosis processes whose manifestation in BM biopsy samples is associated with the number of TGFβF1-expressing atypical MKCs.
Autologous Hematopoietic Stem Cell Transplantation in Multiple Myeloma with Renal Impairment
Authors: Firsova Maiya Valerevna , Mendeleeva L.P. , Solov’ev M.V. , Mironova D.A. , Kuzmina L.A. , Savchenko V.G.
Published: February 2022

Aim. To study the efficacy and adverse event spectrum of high-dose chemotherapy with subsequent autologous hematopoietic stem cell transplantation (auto-HSCT) in multiple myeloma (MM) patients with acute renal impairment, including hemodialysis (HD) dependence. Materials & Methods. The retrospective single-center study enrolled 64 MM patients (30 men and 34 women) with renal impairment, aged 19-65 years (median 54 years), who received auto-HSCT in the period from 2013 to 2019. Newly diagnosed patients had a median creatinine of 462 nmol/L and a median glomerular filtration rate of 10 ml/min/1,73 m2 (CKD-EPI). HD dependence was reported in 23 (36 %) patients on diagnosis date. As a result of the induction therapy, in 13 (57 %) out of 23 patients HD could be discontinued. Prior to auto-HSCT, overall antitumor response was 91 % (complete remission was 45 %), overall renal response was 80 % (complete renal response was 28 %). In the course of auto-HSCT 10 patients remained HD dependent. Two groups were analyzed: “HD-” (program HD-independent patients during auto-HSCT, n = 54) and “HD+” (program HD-dependent recipients of auto-HSCT, n = 10). Results. Herpes virus infection reactivation and reversible toxic encephalopathy were observed significantly more often in “HD+” than in “HD-” group (30 % vs. 6 %, p = 0.04 and 20 % vs. 0 %, p = 0.02, respectively). HD-dependent patients required red blood cell transfusion significantly more often than HD-independent patients (100 % vs. 35 % of cases; p = 0.0001). In 100 days after auto-HSCT, overall antitumor response increased from 91 % to 96 %, the rate of complete remission increased from 45 % to 64 %. After auto-HSCT the rate of complete renal response increased from 28 % to 34 %, however, overall renal response remained within the range of 80 %. After auto-HSCT, in a single case HD was discontinued. As a result of the treatment, 14 (61 %) patients became HD-independent. Transplantation-associated mortality was not reported. During median follow-up of 48 months, 5-year overall survival was 70 % and 5-year disease-free survival was 42 %. Conclusion. Auto-HSCT is a feasible, safe, and effective treatment of MM patients with acute renal impairment. Induction therapy with subsequent auto-HSCT resulted in less need for HD which was 36 % at MM onset and 14 % on completing the treatment.
Thermodynamic limit and boundary energy of the spin-1 Heisenberg chain with non-diagonal boundary fields
Authors: Zheng Zhihan , Sun Pei , Xu Xiaotian , Yang Tao , Cao Junpeng , Yang Wen-Li
Published: February 2022

The thermodynamic limit and boundary energy of the isotropic spin-1 Heisenberg chain with non-diagonal boundary fields are studied. The finite size scaling properties of the inhomogeneous term in the T-Q relation at the ground state are calculated by the density matrix renormalization group. Based on our findings, the boundary energy of the system in the thermodynamic limit can be obtained from Bethe ansatz equations of a related model with parallel boundary fields. These results can be generalized to the SU(2) symmetric high spin Heisenberg model directly.
Etica y corrupción: Percepciones de los estudiantes de la Universidad Nacional Agraria la Molina
Authors: Barriga Barriga Tomás Carlos Manuel
Published: February 2022

La ética es considerada buena y deseable; la corrupción es considerada mala e indeseable; sin embargo, los límites entre ambas no siempre están claramente delimitados. Ambos conceptos se analizan desde las perspectivas occidental y oriental, con la intención de establecer las posibles causas de la corrupción, su normalización en la sociedad moderna, los niveles de tolerancia frente a ella y la percepción que existe de los jóvenes universitarios de sí mismos, y de quien administra el Estado. Se pretende establecer argumentos racionales que sustenten la necesidad de la ética para una vida humana con significado y, como consecuencia, proponer mecanismos para combatir la corrupción a un nivel personal y social.
Research Advancements on Fluorinated and Non-Fluorinated 4-
Phenyl(thio)ureido-Substituted 2,2-Dimethylchromans Acting as Inhibitors
of Insulin Release and Smooth Muscle Relaxants
Authors: Pirotte Bernard , Florence Xavier , Goffin Eric , Leleux Fabienne , Lebrun Philippe
Published: February 2022

Aims:

The present study aimed at characterizing the impact of the presence or absence of
fluorine atoms on the phenyl and benzopyran rings of 4-phenyl(thio)ureido-substituted 2,2-
dimethylchromans on their ability to inhibit insulin release from pancreatic β-cells or to relax vascular
smooth muscle cells.

Methods:

Most compounds were found to inhibit insulin secretion and to provoke a marked
myorelaxant activity.

Results:

The lack of a fluorine or chlorine atom at the 6-position of the 2,2-dimethylchroman core
structure reduced the inhibitory activity on the pancreatic endocrine tissue. One of the most active
compounds on both tissues, compound 11h (BPDZ 678), was selected for further pharmacological
investigations.

Conclusion:

The biological data suggested that 11h mainly expressed the profile of a KATP channel
opener on pancreatic β-cells, although a calcium entry blockade effect was also observed. On vascular
smooth muscle cells, 11h behaved as a calcium entry blocker.
Hypomyelination with Atrophy of Basal Ganglia and Cerebellum (HABC)
Due to UFM1 Mutation in Roma Patients - Severe Early Encephalopathy
with Stridor and Severe Hearing and Visual Impairment. A Single
Center Experience
Authors: Ivanov Ivan , Pacheva Iliyana , Yordanova Ralitsa , Sotkova Iglika , Galabova Fani , Gaberova Katerina , Panova Margarita , Gheneva Ina , Tsvetanova Tsvetelina , Noneva Katerina , Dimitrova Diana , Markov Stoyan , Sapundzhiev Nikolay , Bichev Stoyan , Savov Alexey
Published: February 2022

Background:

Hypomyelination with atrophy of the basal ganglia and cerebellum (H-ABC)
is a neurodegenerative disease with neurodevelopmental delay, motor, and speech regression, pronounced
extrapyramidal syndrome, and sensory deficits due to TUBB4A mutation. In 2017, a severe
variant was described in 16 Roma infants due to mutation in UFM1.

Objective:

The objective of this study is to expand the clinical manifestations of H-ABC due to UFM1
mutation and suggest clues for clinical diagnosis.

Methodology:

Retrospective analysis of all 9 cases with H-ABC due to c.-273_-271delTCA mutation
in UFM1 treated during 2013-2020 in a Neuropediatric Ward in Plovdiv, Bulgaria.

Results:

Presentation is no later than 2 months with inspiratory stridor, impaired sucking, swallowing,
vision and hearing, and reduced active movements. By the age of 10 months, a monomorphic disease
was observed: microcephaly (6/9), malnutrition (5/9), muscle hypertonia (9/9) and axial hypotonia
(4/9), progressing to opisthotonus (6/9), dystonic posturing (5/9), nystagmoid ocular movements (6/9),
epileptic seizures (4/9), non-epileptic spells (3/9). Dysphagia (7/9), inspiratory stridor (9/9), dyspnea
(5/9), bradypnea (5/9), apnea (2/9) were major signs. Vision and hearing were never achieved or lost
by 4-8 mo. Neurodevelopment was absent or minimal with subsequent regression after 2-5 mo. Brain
imaging revealed cortical atrophy (7/9), atrophic ventricular dilatation (4/9), macrocisterna magna
(5/9), reduced myelination (6/6), corpus callosum atrophy (3/6) and abnormal putamen and caput nuclei
caudati. The age at death was between 8 and 18 mo.

Conclusion:

Roma patients with severe encephalopathy in early infancy with stridor, opisthotonus,
bradypnea, severe hearing and visual impairment should be tested for the Roma founder mutation of
H-ABC in UFM1.
Um breve olhar sobre a gramaticalização dos marcadores discursivos "depois" e "agora"
Authors: Bastos Eloísa
Published: February 2022

Asthetitlesuggests,thepresentworkaimstoidentifyanddescribethedifferentstagesofgrammaticalizationcrossedbythediscursivemarkersafterandnow.Withthispurposeinmind,wewillestablishacomparisonbetweenthediscursivevaluesthatthesemarkersexhibitedbetweenthe13thandthe16thcenturiesandthediscursivevaluesthatconveyinContemporaryPortuguese.Fromthefirstlookatourcorpus,wehypothesizethatvalueslikecause-consequence,counter-expectation,orevenspatiallocation,identifiedforthesemarkersinthepresentcentury,arenotyetvisibleinthe13ththrough16thcenturies,containingmerelythefunctionofatemporaladverb.Therefore,wewillbeinterestedinprovingthishypothesisbyidentifyingandclassifyingtheexamplescollectedinlinewiththegrammaticalizationprinciplesproposedbyHopper(1987)andwiththegrammaticalizationmechanisms suggested, later, by Heine (2003).
Gebelerin Grip Aşısı Etkinliği ve Güvenirliği Hakkında Bilgi Düzeyi
Authors: OKTAY Sabri , ARSLAN Mehmet , EMRE Nilüfer , EDİRNE Tamer , ÖZŞAHİN Aysun
Published: February 2022

Amaç: Mevsimsel grip özellikle risk gruplarında sağlık üzerine doğrudan etkisinin yanı sıra, sosyal ve ekonomik açılardan da önemli kayıplara neden olan bir enfeksiyon hastalığıdır. Gribe bağlı 2. ve 3. trimesterde ciddi komplikasyonlar, sekonder pnömoni, ölü doğum, akut solunum yetmezliği riski ve erken doğum görülebilmektedir. Grip aşısının, gebelik sırasında son derece etkili ve güvenli olduğu bilinmektedir. Bu çalışmadaki amacımız, Pamukkale Üniversitesi kadın hastalıkları ve doğum polikliniğinde takip edilen gebelerin, grip aşısının etkinliği ve güvenilirliği hakkında bilgi düzeylerini değerlendirmektir. Yöntem: Araştırmamız, Pamukkale Üniversitesi Tıp Fakültesi Hastanesi Kadın Hastalıkları ve Doğum polikliniğe 2017 Haziran-Ağustos aylarında başvuran gebelerde yapılmıştır. Araştırma, tanımlayıcı bir çalışmadır. Çoğul gebelik olanlar, Türkçe bilmeyenler, çalışmaya katılmak istemeyenler çalışmaya dahil edilmemiştir. Bulgular: Çalışmamıza katılan 205 kişinin yaş ortalaması 28,8 + 0,89 ve %56,6’sı (n=116) ev hanımı idi. Gebelerin %50,7’si (n=104) grip aşısının etkinliği ve güvenirliği hakkında bilgiye sahip olmadığını belirtmiştir. Çalışmamıza katılanların %10,2’sinin (n=21) gebe kalmadan önce grip aşısı yaptırdığını, %70,2’sinin (n=144) grip aşısının gebeler için zararlı olduğunu düşündükleri saptanmıştır. Katılımcıların %66,3’ü (n=136) gebelik sürecinde grip aşısı olması gerektiğini aile hekiminden öğrenmiştir. Katılımcıların eğitim düzeyi arttıkça, grip aşısının hangi sağlık kuruluşunda yapılacağını bilme durumu istatiksel anlamlı bir şekilde artmıştır (p=0,03). Grip aşısının, etkili ve güvenli bir korunma yöntemi olduğunu düşünenler, istatiksel anlamlı olarak daha fazla gebe kalmadan önce grip aşısı yaptırmıştı (p<0,001). Grip aşısının etkili ve güvenli bir korunma yöntemi olmadığını düşünenler, istatiksel anlamlı olarak daha fazla gebeyken grip aşısı yaptırmanın zararlı olduğunu düşünüyordu (p<0,001). Sonuç: Çalışmaya katılan gebelerin, yaklaşık yarısı grip aşısının güvenirliği hakkında bilgiye sahip olmadığını ve büyük çoğunluğunun grip aşısının gebeler için zararlı olduğunu düşündükleri saptanmıştır. Ancak grip aşısını etkili ve güvenli bulan gebeler, daha fazla oranda gebelik öncesinde dönemde grip aşılarını yaptırmışlardı. Ayrıca eğitim düzeyi artışı, grip aşısının yapıldığı merkezleri bilme oranında etkili bulunmuştur.
Designing a mobile user experience student knowledge management system using Lean UX
Authors: Kartiko Condro , Arrasyid Hesa Abda , Wardhana Ariq Cahya
Published: February 2022

Student Affairs at Telkom Purwokerto Institute of Technology has a lot of information and knowledge that all students need to know. However, this information and knowledge have not been maximally conveyed to students. Student affairs have a scope of work to handle the Student Creativity Program (PKM), Student Organization (Ormawa), scholarships, Student Activity Transcripts (TAK), and lectures. Therefore, one of the efforts to facilitate Student Affairs in conveying various knowledge is a Student Knowledge Management System mobile application. User Experience student KMS mobile application designed for mobile devices that focus on interface design. The development of this application interface uses the Lean UX method. The Lean UX method has four stages: Declare Assumptions, Create an MVP, Run an Experiment, and Feedback and Research. The application design is evaluated using the Enhanced Cognitive Walkthrough (ECW) to produce a prototype of a knowledge management system or Knowledge Management System for IT Telkom Purwokerto student affairs according to the user's wishes. The test results show success in all tasks, so it can be concluded that the application design has achieved a good user experience.
An analysis of proportional reasoning ability of junior high school students
Authors: Mardika Fitria , Mahmudi Ali
Published: February 2022

This study aims to describe proportional reasoning skills and students’ difficul­ties in solving proportional reasoning problems. The population of this research was 3480 seventh-grade students of state junior high schools in the Yogyakarta region. The samples were 158 students chosen using proportionate stratified random sampling that represent four categories of school (very high, high, me­dium, low) based on the average scores of the national examination in mathe­matics in 2016. Data were collected through essay tests and interviews. Data were analyzed quantitatively and qualitatively. The result showed that the stu­dents in very high and high category schools had moderate proportional rea­soning ability. Students in medium and low category schools had low propor­tional reasoning ability. Students’ difficulties in solving proportional reasoning problems vary based on the type of the problem, such as the difficulty in finding the multiplicative relationship contained in the problem, the difficulty in under­standing the value of inverse proportion, and the difficulty in explaining the obtained solution of the problems.
An analysis of mathematics understanding of prospective student-teachers of mathematics
Authors: Mahmudi Ali
Published: February 2022

This quantitative-qualitative descriptive study aims to reveal the mathematics understanding of prospective student-teachers, describe the types of their mistakes in mathematics understanding, and recommend appropriate teaching of mathematics understanding. The subjects were 34 first-year students taking the Geometry course in the Mathematics Education Study Program, at a State University in Yogyakarta, Indonesia. The test instrument was consisting of four items, two of which were about the problems of procedural instrumental understanding and the other two were about the problems of relational understanding. The data were analyzed through the stages of reducing data, presenting data, and concluding data. The results show that students' mathematical understanding is categorized as good (87.75%). Students' procedural instrumental understanding (87.75%) is better than their relational understanding (77.52%) which is in good and sufficient categories, respectively. Some students made mistakes in representing theorems, performing mathematical procedural using appropriate concepts/theorems, and interpreting conclusions or proofs using appropriate notation and representation. It is recommended that mathematical learning should prioritize relational understanding by exploring a concept with other concepts and using various representations, situations, and contexts. Learning should focus more on relational understanding, namely discussing a concept and its relationship with other concepts with different representations, situations, and contexts.
Sleep disorders in cancer patients: experience from Rafael Institute Sleep Observatory (Preprint)
Authors: scher Nathaniel , Guetta Liath , Yahiaoui Safia , Terzioglu Mathilde , Butaye Emilie , Henriques Kathy , Alavoine Marie , Elarar Ayala , Guetta Andre , Toledano Alain
Published: February 2022

BACKGROUND

Sleep disorders (SDs) are a common occurrence in the general population. Yet today it is clearly agreed that SDs Liath represent both a cancer risk factor and a biological consequence of the.

OBJECTIVE

The aim of this study is to assess the impact on quality of life, identify the type of disorder and its causes in order to offer an adapted and personalized care plan.

METHODS

In a survey completed during the COVID-19 lockdown, 2,000 hours of interviews were collected by remote consultations. During these calls, we administered a sleep questionnaire. This questionnaire was inspired by the STOP BANG questionnaire enquired about 6 items. The demographic details of each patient (age, sex), the nature of the pathology, their past treatments, the ongoing cancer treatment, the mood, whether or not the patient is anxious or depressed and the use of sleeping drug pills were analyzed. A univariate analysis was performed according to the presence or absence of fatigue. Chi-square test was applied to assess possible differences of variables link to sleep disturbance between patients complaining of fatigue with those without fatigue. The same test was then used to analyse patients on hormone therapy and those without for two types of cancer, breast cancer and prostate cancer

RESULTS

905 patients were included in this study. The average age was 66.7 with 67% women. 142 patients declared being overweight. Breast cancer was the most frequently cancer reported. Nocturnal awakening was reported by 70% of patients, fatigue by 50% of patients, difficulty getting to sleep by 38% of patients, snoring reported by an independent observer in 38% of patients and apnea reported by an independent observer in 9% of patients. The univariate analysis showed that the feeling of tiredness was significantly greater in patients reporting difficulty in getting to sleep (p<0.01), pain (p<0.001), frequent awakening (p<0.001) and who were not receiving cancer treatment (p<0.001). The univariate analysis showed that patients receiving breast cancer treatment and who were under hormone therapy reported difficulty getting to sleep (p=0.04) and pain (p =0.05). In a univariate analysis of patients treated for prostate cancer, being overweight was the only factor reported that had a statistically significant value.

CONCLUSIONS

Our preliminary data support and are consistent with data in the literature as regards the importance of sleep disorders in oncology. This justifies the usefulness of a diagnosis and early treatment of sleep disorders in patients with cancer. The Rafael Institute sleep observatory will enable patients to be identified and treated.
Study of Impact of Digital marketing on Consumer Buying Behaviour for Electronic Goods
Authors: Tirpude Raman
Published: February 2022

Abstract: The buying behavior of consumer is changing at a faster rate in the customer oriented market environment. Consumer behaviour differs when it involves the merchandise, price, features, quality, packaging, buying behaviour, status, generation, age of the customer etc. however, youth is that the most intricate group to correspond with. The changing preferences of the present day youth affects the buying pattern with trends and fashion. Therefore, Marketers spend crores of rupees and invest an excessive amount of time on marketing research per annum to spot and predict the changing youth behaviour. Now digital platforms has posed many challenges to the marketer in the retail segment. The present generation is more fascinated with the web shopping than the traditional buying. The marketers are forced to introduce the innovative way of selling due to the pressure of the younger generation’s buying behaviour. Customers use the web not only to shop for the merchandise online, but also to match prices, product features and after sale service facilities the desire receive. In addition to the tremendous potential of the E-business market, the web provides a singular opportunity for companies to more efficiently reach existing and potential customers. The popular digital retailing companies in India are Myntra, Flipkart Snap deal, Amazon.com and e-Bay etc. The five dominant factors which influence consumer perceptions for digitalshopping are information, easy to use, satisfaction, security, proper utilization of available information to compare the different products. Keywords: Customer, Digital shopping, Digital marketing, Buying Behaviour
Online Voting System Using Java and SQL
Authors: Sai Medasani Harshith , Kumar Vallepu Anil
Published: February 2022

Abstract: An online voting system is being developed that is both threats free and user-friendly. The online voting system is intended for people living in different places around the world to vote for their representatives. We have the option of having an election with paper ballots or an automated ballot election here in our world. The automated ballot elections have become the norm in recent years. Electronic voting is also currently used. The online voting system has evolved to a point that it can replace the online polling system with accurate and direct voting. A user logs in for this online voting process by providing their first name, last name, email address, password, and thumb (because these things are all unique to them). In this system, every user has just one vote and if they use their votes repeatedly, it means their votes will be considered invalid. We can reduce time and money by using this online voting system and it is very secure, efficient and safe. Keywords: java, SQL Database, JavaScript, Wamp server
Fast and Reliable Vision-Based Navigation for Real Time Kinematic Applications
Authors: Amami Mustafa M.
Published: February 2022

Abstract: Automatic Image Matching (AIM) is the term used to identify the automatic detection of corresponding points located on the overlapping areas of multiple images. AIM is extensively used with Mobile Mapping System (MMS) for different engineering applications, such as highway infrastructure mapping, monitoring of road surface quality and markings, telecommunication, emergency response, and collecting data for Geographical Information Systems (GIS). Robotics community and Simultaneous Localization And Mapping (SLAM) based applications are other important areas that require fact and welldistributed AIM for robust vision navigation solutions. Different robust feature detection methods are commonly used for AIM, such as Scale Invariant Feature Transform (SIFT), Principal Component Analysis (PCA)–SIFT and Speeded Up Robust Features (SURF). The performance of such techniques have been widely investigated and compared, showing high capability to provide reliable and precise results. However, these techniques are still limited to be used for real and nearly real time SLAM based applications, such as intelligent Robots and low-cost Unmanned Aircraft Vehicles (UAV) based on vision navigation. The main limitations of these AIM techniques are represented in the relatively long processing time and the random distribution of matched points over the common area between images. This paper works on overcoming these two limitations, providing extremely fast AIM with well- distributed common points for robust real time vision navigation. Digital image pyramid, Epipolar line and 2D transformation have been utilized for limiting the size of search windows significantly and determining the rotating angle and scale level of features, reducing the overall processing time considerably. Using limited number of well-distributed common points has also helped to speed up the automatic matching besides providing robust vision navigation solution. The idea has been tested with terrestrial MMS images, and surveying UAV aerial images. The results reflect the high capability of the followed technique in providing fast and robust AIM for real-time SLAM based applications. Keywords: Automatic Image Matching, Epipolar Line, Image Pyramid, SLAM, Vision Navigation, Real Time, Vision Navigation.
Formulation of Hand Made Soap by using Goat Milk
Authors: There Uma Ganesh , Kalambe Swanand , Choudhary Vikas
Published: February 2022

Abstract: Hand Made Soap is a mingle of both science and art by combining scientific knowledge and artistic creature. Costeffective, skin and eco-friendly handmade soap has been developed by a cold process of saponification by using food-grade, natural ingredients with vegetable oils such as coconut oil, palm oil with active ingredients such as goat milk, and essential oil. The addition of fresh goat milk is the main challenge due to heat generation by caustic lye during the saponification process. The caustic soda in soap formula is caramelize the sugar in the milk, turning soap a bright brown color or curdles the milk. Hence, the study has been initiated and developed a standardized formula with desired qualities, color, and texture. The experiment studied physio-chemical parameters and dermatological tests of five different formulations of goat milk soap. Keywords: Hand Made Soap, Saponification, cold process, goat milk, vegetable oils.
Similarity Analysis of User Trajectories Based on Haversine Distance and Needleman Wunsch Algorithm
Authors: Jamhuri Mohammad , Irawan Mohammad Isa , Mukhlash Imam
Published: February 2022

Abstract: In this paper, we discuss the similarity between two trajectories using the Needleman Wunsch algorithm. The calculation steps are interpolating the trajectory, calculating the distance between the trajectory coordinates, identifying the equivalent length, transforming trajectories into a sequence of alphabetic letters, aligning the sequences, and measuring the magnitude of the similarity based on the alignment results. The similarity obtained is compared directly to the length of the trajectories shared by the two lines. The calculation results show that the accuracy of the alignment method reaches more than 90%. Abstrak: Dalam tulisan ini dibahas cara perhitungan persentase kesamaan dari dua buah lintasan menggunakan algoritma Needleman Wunsch dan perhitungan secara manual berdasarkan irisan dari lintasan-lintasan tersebut. Pada perhitungan menggunakan algoritma Needleman Wunsch, tahapan-tahapan yang dilakukan adalah menginterpolasi lintasan, menghitung jarak antara titik-titik koordinat dari kedua lintasan, mengidentifikasi jarak yang ekivalen, mengubah lintasan menjadi sekuens huruf alfabet, menyejajarkan sekuens, dan menentukan besarnya kesamaan berdasarkan hasil penyejajaran. Kesamaan yang diperoleh dari metode penyejajaran dibandingkan secara langsung dengan panjang jalur yang dilalui bersama oleh kedua lintasan, hasil perhitungan menunjukkan bahwa akurasi metode penyejajaran mencapai lebih dari 90%.
Peningkatan Kualitas Pelatihan Dengan Metode Servqual dan Quality Function Deployment: Studi Kasus di Perusahaan Reasuransi
Authors: Halimmah Zarica
Published: February 2022

PT. Reinsurance Indonesia Utama (Persero) is the first reinsurance company of the government (BUMN). As a company that is very concerned about their employees, PT. Reinsurance Indonesia Utama (Persero) intensively held some training for their employee. But unfortunately, there is no evaluation regarding the satisfaction of the participants. Therefore, this research was conducted to determine the level of participant satisfaction using the ServQual method and proceed with using the Importance-Performance Analysis (IPA) method to determine the attributes that need improvement. After knowing the attributes that need improvement, the research continuing with QFD method to find out the improvement efforts that need to be done. The questionnaire was used as a data collection method and involved 70 respondents from PT. Reinsurance Indonesia Utama (Persero). The results of ServQual method showed that all attributes tested were negative. This indicates that the services provided have not satisfied the participants. The study continued with the IPA method and obtained 5 attributes in quadrant A that needed improvement. The five attributes are then spelt out in the HOQ matrix and obtained 6 technical responses as the company's response to the participants' needs.