bluuebunny/crossref_metadata_2025_split huggingface dataset:
Sequential measurements of IGF-I serum concentrations in adolescents with Laron syndrome treated with recombinant human IGF-I (rhIGF-I)
Authors: Breil Thomas , Kneppo Carolin , Bettendorf Markus , Müller Hermann L. , Kapelari Klaus , Schnabel Dirk , Woelfle Joachim 
Published: July 2018

Abstract

Background
Recombinant human insulin-like growth factor 1 (rhIGF-I) has been approved as an orphan drug for the treatment of growth failure in children and adolescents with severe primary IGF-I deficiency (SPIGFD) with little pharmacokinetic data available. Therefore, sequential measurements of serum IGF-I, glucose, potassium, insulin and cortisol were performed in patients treated with rhIGF-I to evaluate their significance in safety and efficacy.

Methods
Repetitive blood samples were taken after meals before and 30, 60, 120, 180 and 360 min after rhIGF-I injections in two male patients with Laron syndrome at times of dose adjustments.

Results
Maximal IGF-I concentrations were observed 2 h after injections (495 ng/mL) and concentrations were still higher 6 h after injections than at baseline (303 ng/mL vs. 137 ng/mL). Thirteen percent of all and 33% of maximum IGF-I concentrations were greater than +2 standard deviation score (SDS) calculated for bone age (BA) (IGF-I SDS BA) rather than chronological age (CA) as BA was significantly delayed to CA by 3.2 years (p=0.0007). Height velocities correlated with individual maximum IGF-I SDS BA (ρ=0.735; p<0.0001). Serum insulin, cortisol and glucose did not correlate with IGF-I concentrations, but serum potassium showed a negative correlation (ρ=−0.364; p<0.0001) with IGF-I concentrations.

Conclusions
Sequential measurements of serum IGF-I, glucose and potassium in patients with Laron syndrome may aid in optimizing and individualizing rhIGF-I treatment. IGF-I concentrations should be referenced according to BA which better reflects the biological age. The inverse correlation of IGF-I and serum potassium concentrations after injections of rhIGF-I has not been reported before and warrants further consideration.
Single center experience of biotinidase deficiency: 259 patients and six novel mutations
Authors: Canda Ebru , Yazici Havva , Er Esra , Kose Melis , Basol Gunes , Onay Huseyin , Ucar Sema Kalkan , Habif Sara , Ozkinay Ferda , Coker Mahmut 
Published: July 2018

Abstract

Background
Biotinidase deficiency (BD) is an autosomal recessively inherited disorder of biotin recycling. It is classified into two levels based on the biotinidase enzyme activity: partial deficiency (10%–30% enzyme activity) and profound deficiency (0%–10% enzyme activity). The aims of this study were to evaluate our patients with BD, identify the spectrum of biotinidase (BTD) gene mutations in Turkish patients and to determine the clinical and laboratory findings of our patients and their follow-up period.

Methods
A total of 259 patients who were diagnosed with BD were enrolled in the study. One hundred and forty-eight patients were male (57.1%), and 111 patients were female (42.9%).

Results
The number of patients detected by newborn screening was 221 (85.3%). By family screening, 31 (12%) patients were diagnosed with BD. Seven patients (2.7%) had different initial complaints and were diagnosed with BD. Partial BD was detected in 186 (71.8%) patients, and the profound deficiency was detected in 73 (28.2%) patients. Most of our patients were asymptomatic. The most commonly found variants were p.D444H, p.R157H, c.98_104delinsTCC. The novel mutations which were detected in this study are p.D401N(c.1201G>A), p.A82G (c.245C>G), p.F128S(c.383T>C), c617_619del/TTG (p.Val207del), p.A287T(c.859G>A), p.S491H(c.1471A>G). The most common mutation was p.R157H in profound BD and p.D444H in partial BD. All diagnosed patients were treated with biotin.

Conclusions
The diagnosis of BD should be based on plasma biotinidase activity and molecular analysis. We determined the clinical and genetic spectra of a large group of patients with BD from Western Turkey. The frequent mutations in our study were similar to the literature. In this study, six novel mutations were described.
Modelling the dynamic pattern of surface area in basketball and its effects on team performance
Authors: Metulini Rodolfo , Manisera Marica , Zuccolotto Paola 
Published: July 2018

AbstractBecause of the advent of GPS techniques, a wide range of scientific literature on Sport Science is nowadays devoted to the analysis of players’ movement in relation to team performance in the context of big data analytics. A specific research question regards whether certain patterns of space among players affect team performance, from both an offensive and a defensive perspective. Using a time series of basketball players’ coordinates, we focus on the dynamics of the surface area of the five players on the court with a two-fold purpose: (i) to give tools allowing a detailed description and analysis of a game with respect to surface areas dynamics and (ii) to investigate its influence on the points made by both the team and the opponent. We propose a three-step procedure integrating different statistical modelling approaches. Specifically, we first employ a Markov Switching Model (MSM) to detect structural changes in the surface area. Then, we perform descriptive analyses in order to highlight associations between regimes and relevant game variables. Finally, we assess the relation between the regime probabilities and the scored points by means of Vector Auto Regressive (VAR) models. We carry out the proposed procedure using real data and, in the analyzed case studies, we find that structural changes are strongly associated to offensive and defensive game phases and that there is some association between the surface area dynamics and the points scored by the team and the opponent.
Redetermination of methyl 4-(4-chlorophenyl)-2,7,7-trimethyl-5-oxo-1,4,5,6,7,8-hexahydro-quinoline-3-carboxylate, C20H22ClNO3
Authors: Wang Qian-Qian , Wang Da-Xiang , Liu Qian-Qian 
Published: July 2018

Abstract
C20H22ClNO3, monoclinic, P21/n (no. 14), a = 9.275(5) Å, b = 18.213(9) Å, c = 11.403(6) Å, β = 109.718(9)°, V = 1813.4(16) Å3, Z = 4, R
gt(F) = 0.0483, wR
ref(F
2) = 0.1346, T = 296(2) K.
Crystal structure of 2,5-bis(4-(10H-phenothiazin-10-yl)phenyl)-1,3,4-oxadiazole, C38H24N4OS2
Authors: Wu Qiong , Zhang Heng-Qiang , Ramanaskanda Braveenth , Chai Kyu-Yun 
Published: July 2018

Abstract
C38H24N4OS2, monoclinic, P21/c (no. 14), a = 10.9621(6) Å, b = 19.7851(11) Å, c = 14.1928(9) Å, β = 100.957(6)°, V = 3022.1(3) Å3, Z = 4, R
gt(F) = 0.0528, wR
ref(F
2) = 0.1294, T = 172.99(10) K.
Gedächtnismechanismen in Drosophila
Authors: Felsenberg Johannes , Owald David 
Published: July 2018

ZusammenfassungZielgerichtetes Verhalten wird durch neuronale Schaltkreise im Gehirn gesteuert. Erfahrungen können die Dynamiken dieser neuronalen Schaltkreise verändern, indem spezifische synaptische Verbindungen modifiziert werden. Die Lokalisierung einer solchen lerninduzierten synaptischen Plastizität hat sich jedoch bis heute als anspruchsvoll erwiesen. Technische Fortschritte in der Kontrolle und Messung von neuronaler Aktivität haben es nun ermöglicht, die dem Lernen und der Gedächtnisbildung zugrunde liegenden Strukturen im Gehirn des ModelsystemsDrosophila in vivozu charakterisieren. Die Taufliege (Drosophila melanogaster)hat ein numerisch einfacheres Gehirn, was die Erforschung identifizierter Netzwerkkomponenten realisierbar macht. In diesem Artikel erörtern wir die Mechanismen, auf denen assoziatives Lernen, Gedächtnisabruf sowie die Reevaluierung von existierenden Gedächtnissen inDrosophilaberuhen.
Motion detection: cells, circuits and algorithms
Authors: Ramos-Traslosheros Giordano , Henning Miriam , Silies Marion 
Published: July 2018

Abstract
Many animals use visual motion cues to inform different behaviors. The basis for motion detection is the comparison of light signals over space and time. How a nervous system performs such spatiotemporal correlations has long been considered a paradigmatic neural computation. Here, we will first describe classical models of motion detection and introduce core motion detecting circuits in Drosophila. Direct measurements of the response properties of the first direction-selective cells in the Drosophila visual system have revealed new insights about the implementation of motion detection algorithms. Recent data suggest a combination of two mechanisms, a nonlinear enhancement of signals moving into the preferred direction, as well as a suppression of signals moving into the opposite direction. These findings as well as a functional analysis of the circuit components have shown that the microcircuits that process elementary motion are more complex than anticipated. Building on this, we have the opportunity to understand detailed properties of elementary, yet intricate microcircuits.
Enhancement of eucalypt pulp yield through extended impregnation cooking
Authors: Souza Gustavo B. de , Colodette Jorge Luiz , Borges Gomes Fernando José , Carvalho Danila Morais de 
Published: July 2018

AbstractThe improvement caused by eucalypt chip impregnation on kraft pulping performance was assessed for terminating the cook at kappa in the range of 15–27 and at controlled residual effective alkali (REA) of 6–8 g/L NaOH. Extended impregnation cooking of eucalypt chips (EIC) increased about 1 %lignin- and HexA-freescreen yield gains in relation to conventional cooking (CC), regardless of kappa number in the range of 15–27. The EIC technology allows for cooking eucalypt wood to kappa number up to 27, without rejects production, but without significant improvement inlignin- and HexA-freescreen yield and with larger chlorine dioxide (ClO2) consume during bleaching. The optimum kappa number for both CC and EIC cooking was about 19 with similar refinability and strength properties for both technologies, CC and EIC. It was concluded that extended impregnation cooking is an attractive technique for enhancing bleached eucalypt Kraft pulp yield.
Thermal and natural aging of bagasse paper sheets coated with gelatin
Authors: El-Sakhawy Mohamed , Nashy EL-Shahat H. A. , El-Gendy Ahmed , Kamel Samir 
Published: July 2018

AbstractThe utilization of the solid tannery wastes as a promoting agent to improve paper sheet properties was investigated. Solid tannery wastes from pickled hide shavings are treated to remove acid and salts, and hydrolyzed at pH 10 by using potassium carbonate. The produced hydrolysable collagen (gelatin) was used as coating for paper sheets. The gelatin concentrations from 0.5–2.5 % were used as coating solution and its effect on thermal aging and mechanical properties was studied. A direct proportion between gelatin concentration and improvements of paper mechanical properties was observed. A kinetic study based on paper strength properties confirmed the stabilizating effect of gelatin on paper sheets. Paper sheets coated with 2.5 % gelatin solution were thermally aged at temperatures range between 100–200 °C for different time intervals from 1–6 h, and after that it was subjected to natural aging for two years. Gelatin coating improves the paper sheets mechanical properties and thermal stability.
How big is the potato (Solanum tuberosum L.) yield gap in Sub-Saharan Africa and why? A participatory approach
Authors: Harahagazwe Dieudonné , Condori Bruno , Barreda Carolina , Bararyenya Astère , Byarugaba Arinaitwe Abel , Kude Danbaba Anthony , Lung’aho Charles , Martinho Carolino , Mbiri Daniel , Nasona Bouwe , Ochieng Bruce , Onditi John , Randrianaivoarivony Jean Marc , Tankou Christopher M. , Worku Alemu , Schulte-Geldermann Elmar , Mares Victor , Mendiburu Felipe de , Quiroz Roberto Quiroz 
Published: July 2018

Abstract
According to potato experts from ten Sub-Saharan Africa (SSA) countries working together in a community of practice (CoP) over a 3-years period, potato farmers across SSA can increase their current annual production of 10.8 million metric tons by 140% if they had access to high quality seed along with improved management practices. This paper describes this innovative new methodology tested on potato for the first time, combining modelling and a comprehensive online survey through a CoP. The intent was to overcome the paucity of experimental information required for crop modelling. Researchers, whose data contributed to estimating model parameters, participated in the study using Solanum, a crop model developed by the International Potato Center (CIP). The first finding was that model parameters estimated through participatory modelling using experts’ knowledge were good approximations of those obtained experimentally. The estimated yield gap was 58 Mg ha-1, of which 35 corresponded to a research gap (potential yield minus research yield) and 24 to farmers’ gap (research yield minus farmer’s yield). Over a 6-month period, SurveyMonkey, a Web-based platform was used to assess yield gap drivers. The survey revealed that poor quality seed and bacterial wilt were the main yield gap drivers as perceived by survey respondents.
Agro-ecological distribution of the phenotypic diversity of aerial yam (Dioscorea bulbifera L.) in Cameroon using multivariate analysis: prospect for germplasm conservation and improvement
Authors: Kouam Eric B. , Avana-Tientcheu Marie L. , Lekeumo Vanessa D. , Akitio Hermine M. , Khasa Damase P. , Pasquet Remy S. 
Published: July 2018

Abstract
Aerial yam (Dioscorea bulbifera L.) is a crop of great economic importance and an excellent candidate for improving food security in developing countries. Understanding the genetic variability of any crop species is a decisive step for its improvement and requires characterization and evaluation of available germplasm. The objectives of this study were to determine the extent of genetic variability, estimate the association between agromorphological traits and clustering among 57 genotypes of aerial yam from three distinct agro-ecological zones in Cameroon using multivariate analysis. Thirty nine characters (23 qualitative and 16 quantitative) were used for the study. Significant differences in genetic diversity indices were found. Accessions from the bimodal humid forest zone (Na = 2.08, He = 0.27) showed significantly lower diversity compared to both western highland (Na = 2.30, He = 0.34) and humid monomodal forest zones (Na = 2.57, He = 0.32). Means values of most quantitative traits also showed significant differences between agro-ecological zones. Batingla-3 and Bawouwoua-1 had important bulbil yield, reaching 3500 g / plant. Significant associations were found between many traits. The use of the Unweighted Pair Group Method with Arithmetic Mean allowed the distribution of the 57 genotypes into six distinct clusters with the clustering pattern not showing any parallelism with location sites or agro-ecological zones. Mahalanobis D2 statistics revealed the highest inter-cluster distance between cluster II and VI. Accessions of these clusters are potential parents for future breeding programs. This study showed that aerial yam from Cameroon has an enormous wealth of traits variation, indicating huge potential for its genetic improvement through selection and hybridization.
Understandable robots - What, Why, and How
Authors: Hellström Thomas , Bensch Suna 
Published: July 2018

Abstract
As robots become more and more capable and autonomous, there is an increasing need for humans to understand what the robots do and think. In this paper, we investigate what such understanding means and includes, and how robots can be designed to support understanding. After an in-depth survey of related earlier work, we discuss examples showing that understanding includes not only the intentions of the robot, but also desires, knowledge, beliefs, emotions, perceptions, capabilities, and limitations of the robot. The term understanding is formally defined, and the term communicative actions is defined to denote the various ways in which a robot may support a human’s understanding of the robot. A novel model of interaction for understanding is presented. The model describes how both human and robot may utilize a first or higher-order theory of mind to understand each other and perform communicative actions in order to support the other’s understanding. It also describes simpler cases in which the robot performs static communicative actions in order to support the human’s understanding of the robot. In general, communicative actions performed by the robot aim at reducing the mismatch between the mind of the robot, and the robot’s inferred model of the human’s model of the mind of the robot. Based on the proposed model, a set of questions are formulated, to serve as support when developing and implementing the model in real interacting robots.
Comparative studies of energy saving polymers and fabrication of high performance transparent polymer by solvent bonding
Authors: Aadhy S.P. , Sinega T. Hema , Karthikeyan C. , Akshay S. , Kumar Pitchan Mohan , Bhowmik Shantanu 
Published: July 2018

Abstract
This work investigates the possibility of using polyetherimide (PEI) as an energy saving alternative to glass, polymethylmethacrylate (PMMA) and polycarbonate (PC) by carrying out heat transfer analysis and suggests vaporized solvent bonding as a viable bonding technique for the fabrication of PEI. By heat transfer analysis using building energy simulation, it is observed that less energy is expended for space-conditioning of a building with windows made of PEI when compared to glass, PMMA and PC. The compression moulding technique is used to mould PEI and fabrication is done using a solvent mixture of dimethyl sulfoxide and tetrahydrofuran in 1:1 ratio. The optical properties of the bonded specimen are studied using UV-visible spectrophotometry and it is found that PEI does not allow UV wavelength radiation to pass through while transmitting visible wavelengths. The mechanical strength of the bond is tested using lap shear tensile strength test and the type of failure is observed to be cohesive from the structure. This is indicative of the fact that using this particular solvent to bond PEI results in the maximum possible strength.
Thermal degradation kinetics of oxo-degradable PP/PLA blends
Authors: Mandal Dev K. , Bhunia Haripada , Bajpai Pramod K. 
Published: July 2018

Abstract
In this article, the influence of polylactide and pro-oxidant on the thermal stability, degradation kinetics, and lifetime of polypropylene has been investigated using thermogravimetric analysis under nitrogen atmosphere at four different heating rates (i.e. 5, 10, 15, and 20°C/min). The kinetic parameters of degradation were studied over a temperature range of 30–550°C. The derivative thermogravimetric curves have indicated single stage and two stage degradation processes. The activation energy was evaluated by using the Kissinger, Kim-Park, and Flynn-Wall methods under the nitrogen atmosphere. The activation energy value of polypropylene was much higher than that of polylactide. Addition of polylactide and pro-oxidant in polypropylene decreased the activation energy. The lifetime of polypropylene has also decreased with the addition of polylactide and pro-oxidant.
Adjuvant Pressurized IntraPeritoneal Aerosol Chemotherapy (PIPAC) in resected high-risk colon cancer patients – study protocol for the PIPAC-OPC3 Trial. A prospective, controlled phase 2 Study
Authors: Graversen Martin , Detlefsen Sönke , Fristrup Claus , Pfeiffer Per , Mortensen Michael Bau 
Published: July 2018

AbstractBackgroundPeritoneal metastasis (PM) is the second most common site of recurrence in colon cancer (CC) patients and accounts for approximately one-third of all recurrences. Patients with T4 or intraperitoneal perforated colon cancers have an increased risk of developing PM, and since manifest PM is difficult to treat, high-risk patients should be offered prophylactic treatment. Here, we propose a study of adjuvant oxaliplatin administered as pressurized intraperitoneal aerosol chemotherapy (PIPAC OX) in patients with high-risk colon cancer (T4, perforated tumors, ovarian metastasis).MethodsPIPAC-OPC3 CC is a non-randomized, non-blinded phase 2 cohort study designed to treat high-risk colon cancer patients with adjuvant PIPAC-directed therapy. Based on an expected 90 % peritoneal recurrence-free survival with adjuvant PIPAC against the estimated 75 % without, 60 patients are needed (α: 0.05, power: 0.8). Eligible patients will receive two PIPAC treatments with oxaliplatin (92 mg/m2) at 4–6 week intervals. During laparoscopy, the peritoneum is biopsied at two locations, and peritoneal lavage with 500 mL of saline and laparoscopic ultrasound is performed. The patients are screened for adverse medical events and surgery-related complications after each PIPAC procedure. After the second PIPAC procedure, the patients will be examined in the outpatient clinic and followed with CT scans 12, 24 and 36 months after resection. The primary outcome of the PIPAC-OPC3 CC trial is to evaluate if PIPAC-directed adjuvant therapy can reduce the risk of PM. Secondary outcomes include the number of conversions from positive to negative peritoneal lavage cytology after one PIPAC procedure, completion rate of two adjuvant PIPAC treatments, toxicity and complication rate and recurrence-free and overall survival rates after 1, 3 and 5 years.ResultsIt is expected that PIPAC-directed adjuvant therapy can provide an absolute risk reduction of 15 % regarding the development of PM in high-risk colon cancer patients, and that this may result in increased survival rates. We expect that free intraperitoneal tumor cells (FITC) may be detected by peritoneal lavage performed just prior to the administration of PIPAC-directed therapy, and that this treatment may convert FITC-positive patients to a FITC-negative status.ConclusionsThis study may provide important knowledge to be used in designing additional studies on PIPAC in the adjuvant setting of other primary cancers.Trial registrationClinicalTrials.gov Identifier NCT03280511 (2017-09-12). European Clinical Trials Database (EudraCT) 2017-002637-37.
PIPAC EstoK 01: Pressurized IntraPeritoneal Aerosol Chemotherapy with cisplatin and doxorubicin (PIPAC C/D) in gastric peritoneal metastasis: a randomized and multicenter phase II study
Authors: Eveno Clarisse , Jouvin Ingrid , Pocard Marc 
Published: July 2018

AbstractBackgroundPeritoneal metastasis (PM) from gastric cancer often remains undiagnosed until it reaches an advanced stage. Despite curative management combining perioperative systemic chemotherapy, cytoreductive surgery (CRS), and hyperthermic intraperitoneal chemotherapy (HIPEC), treated patients’ 5 year survival rate remains under 20 % when patients are carefully selected. Palliative intravenous chemotherapy in patients with non-resectable cancer is frequently associated with poor long-term benefit and an estimated survival time below 1 year. Recently, two retrospectives studies reported that Pressurized IntraPeritoneal Aerosol Chemotherapy (PIPAC) improves patients’ overall survival without impairing their quality of life (QoL). This promising result needs however to be studied on large randomized clinical trial to validate the effect of PIPAC on survival and QoL of patients with gastric PM.MethodsPIPAC EstoK 01 is a prospective, open, randomized multicenter phase II clinical study with two arms that aims at evaluating the effects of PIPAC with doxorubicin and cisplatin on patients with PM of gastric cancer with peritoneal cancer index (PCI)>8, treated with systemic chemotherapy between two PIPAC procedures. Patients were randomized at the end of explorative laparoscopy and after signing a written consent. Patients received in the first experimental arm a treatment associating PIPAC and systemic chemotherapy (1 PIPAC then 2 IV Chemo) and systemic chemotherapy only in the control arm. Primary endpoint was progression-free survival from the date of surgery to the date of death, or to the end of the 5 year follow-up. Secondary endpoint was 2 year overall survival, morbidity, QoL and secondary resectability rate. The number of patients randomized was calculated to be 94.Trial registrationRetrospectively registered.