bluuebunny/crossref_metadata_2025_split huggingface dataset:
Treatment of hyperthyroidism with antithyroid drugs corrects mild neutropenia in Graves' disease
Authors: Aggarwal N. , Tee S. A. , Saqib W. , Fretwell T. , Summerfield G.P. , Razvi S. 
Published: June 2016

SummaryContextNeutropenia secondary to antithyroid drug (ATD) therapy in Graves' disease (GD) is well recognized. However, the effect of hyperthyroidism, prior to and after ATD therapy, on neutrophil counts in patients with GD is unclear.ObjectiveTo study the prevalence of neutropenia in newly diagnosed untreated GD and the effect of ATD on the neutrophil count.DesignProspective study from August 2010 to December 2014.SettingEndocrinology outpatient clinic in a single centre.PatientsConsecutive patients (n = 206) with newly diagnosed GD.InterventionATD therapy.Main outcome measuresPrevalence and factors predicting neutropenia (<2 × 109/l) and change in neutrophil counts following ATD.ResultsAt diagnosis, 29 (14·1%) of GD individuals had neutropenia. Non‐Caucasians [odds ratio (95% CI) of 4·06 (1·14–14·45), P = 0·03] and patients with higher serum thyroid hormone levels [OR 1·07 (1·02–1·13), P = 0·002 for serum FT3] were the only independent predictors of neutropenia. All patients with neutropenia had normalized blood neutrophil levels after achieving euthyroidism with ATD therapy. In patients in whom data were available posteuthyroidism (n = 149), change in neutrophil count after achieving euthyroidism was independently related to reduction in thyroid hormone levels (P < 0·01).ConclusionsGD is associated with neutropenia in one in seven patients at diagnosis, especially in non‐Caucasians and those with higher serum thyroid hormone levels. Neutrophil counts increase with treatment with ATD and are related to reduction in thyroid hormone concentrations. It is therefore important to check neutrophil levels in newly diagnosed patients with GD prior to commencing ATD therapy as otherwise low levels may incorrectly be attributed to ATD therapy.
Interventions to improve supervised contact visits between children in out of home care and their parents: a systematic review
Authors: Bullen Tracey , Taplin Stephanie , McArthur Morag , Humphreys Cathy , Kertesz Margaret 
Published: June 2016

AbstractAlthough the importance of contact between children in care and their parents, when safe, is accepted, there is limited research about supervised face‐to‐face contact. There is no literature that has systematically critiqued how supervised contact can be best delivered. The aim of this systematic review was to evaluate the evidence for interventions aimed at improving the quality of contact visits between parents and their children who are in out‐of‐home care. Twelve studies were included in this review. Each study was graded and assigned scores according to the presence or absence of each of seven criteria. The studies demonstrated key similarities in the types of interventions provided, although delivery varied across group, individual and educational interventions. Parents reported improved capacity to manage their emotions and parents' satisfaction with the programmes was high. Although there was a lack of large scale, methodologically rigorous studies with long‐term follow‐up, some promising findings were identified: the literature indicates individual family support and group programmes have the potential to improve parent–child relationships and the quality of contact visits. This review suggests that future studies build on current evidence by addressing their methodological limitations and evaluating interventions that can be tailored to meet the needs of individual families.
“Emplotted Narratives” and Structured “Behavioral Observations” Supporting the Diagnosis of Willis‐Ekbom Disease/Restless Legs Syndrome in Children with Neurodevelopmental Conditions
Authors: Ipsiroglu Osman S. , Beyzaei Nadia , Berger Mai , Wagner Alexandra L. , Dhalla Sophia , Garden Jennifer , Stockler Sylvia 
Published: June 2016

SummaryBackgroundWillis‐Ekbom disease/restless legs syndrome (WED/RLS) seems to be a frequent cause of intractable chronic insomnia (ICI) but is under‐recognized in children/adolescents with neurodevelopmental conditions (NDCs), as many patients do not have the ability to express the underlying “urge‐to‐move”. In light of this, we aim to develop a protocol for behavioral observations supporting the diagnosis of WED/RLS.MethodsWe investigated 26 pediatric patients (age 1–16 years, median 8) with NDCs, ICI and evidence of familial WED/RLS employing (1) “emplotted narratives” for description of the various “urge‐to‐move” presentations and (2) self‐description and “behavioral observations” during a “suggested clinical immobilization test” (SCIT).ResultsParental narratives reflected typical WED/RLS‐related “urge‐to‐move” symptoms during day‐, bed‐, and nighttime in all patients. Fifteen out of 26 patients could describe the “urge‐to‐move” during the SCIT. Ten out of 26 patients, unable to describe their symptoms due to cognitive disabilities, showed patterns of “relieving‐movements” upon observation. Sensory processing abnormalities were reported in all patients, with tactile sensitivities (26/26) (including shifted pain threshold) as the most common sensory domain.Conclusion“Emplotted narratives” and structured “behavioral observations” support recognition of familial WED/RLS associated movement patterns and provide a useful tool for the diagnosis of WED/RLS in children with NDCs in a clinical office setting.
Transplantation from haploidentical donor is not inferior to that from identical sibling donor for patients with chronic myeloid leukemia in blast crisis or chronic phase from blast crisis
Authors: Ma Yan‐Ru , Huang Xiao‐Jun , Xu Zheng‐Li , Liu Kai‐Yan , Chen Huan , Zhang Xiao‐Hui , Han Wei , Chen Yu‐Hong , Wang Feng‐Rong , Wang Jing‐Zhi , Wang Yu , Chen Yao , Yan Chen‐Hua , Xu Lan‐Ping 
Published: June 2016

AbstractObjectivesAllogeneic hematopoietic stem cell transplantation (HSCT) is the only curative therapy for chronic myeloid leukemia (CML) patients in blast crisis (BC), and haploidentical donors (HID) are immediately available for most patients. We compared the outcomes of HID transplantation with those of matched related donor (MRD) transplantation in a cohort study.Patients and MethodsA total of 90 consecutive patients who received allogeneic HSCT because of CML‐BC were investigated retrospectively. A total of 67 patients underwent transplantation from HID and 23 from MRD. Survival outcomes were compared between the two cohorts.ResultsOf the 90 patients, 86 patients were engrafted. Three‐year overall survival (OS) and relapse‐free survival (RFS) were comparable between HID and MRD recipients (OS: 60.0% vs 55.3%, respectively, P=.580; RFS: 51.1% vs 47.8%, respectively, P=.512). Three‐year incidences of transplant‐related mortality (TRM) and relapse did not differ between HID and MRD recipients (relapse: 21.0% vs 26.1%, respectively, P=.626; TRM: 27.9% vs 26.1%, respectively, P=.937). In multivariate analyses, previous chemotherapy history and not achieving CHR before HSCT are independent adverse predictors of OS.ConclusionsFor CML‐blast crisis or chronic phase from blast crisis patients, HID transplantation achieves comparable survival to MRD transplantation. HID donors can be regarded as regular donors for these special patients at selected centers.
Macrovesicular steatosis in living related liver donors: correlation of biopsy findings with CT liver attenuation index and body mass index
Authors: Jehangir Maham , Nazir Rashed , Jang Aisha , Rana Atif , Rafique Salman , Dar Faisal Saud 
Published: June 2016

AbstractBackgroundHepatic steatosis threatens post‐transplant graft survival; therefore, pre‐operative quantification of steatosis is crucial. Gold standard for evaluation is donor liver biopsy but it is invasive. An alternative non‐invasive method is a calculation of CT liver attenuation index. BMI can be an independent factor predicting grade of steatosis but it is necessary to re‐define appropriate BMI cut‐off points that are specific for Asians.ObjectiveTo retrospectively analyze CT LAI and BMI for quantitative assessment of macrovesicular steatosis in living related liver donors, using histological analysis as gold standard.Materials and MethodsA radiologist blinded to histological grading calculated mean CT hepatic attenuation in 48 potential living related liver donors.ResultsCT‐derived LAI correctly predicted steatosis in all except 1 patient. Parametric analysis for CT LAI and BMI showed overall weak positive correlation. No significant association was found between BMI and biopsy findings.ConclusionLiver biopsy remains a gold standard for evaluation of steatosis. CT LAI of ≤0 correlates well with significant hepatic steatosis and biopsy may be avoided in such cases. Biopsy may be reserved for patients with CT LAI between 1 and 5. BMI alone is not a good predictor of hepatic steatosis in our study population.
Patient‐reported outcomes in liver transplant recipients with hepatocellular carcinoma
Authors: Kensinger Clark D. , Feurer Irene D. , O'Dell Heather W. , LaNeve David C. , Simmons Lindsey , Pinson C. Wright , Moore Derek E. 
Published: June 2016

AbstractBackgroundThe effect of awarding MELD exception points for hepatocellular carcinoma (HCC) on patient‐reported outcomes (PROs) is unknown. We evaluated the physical and mental health‐related quality of life (HRQOL) and symptoms of anxiety and depression in liver transplant recipients with HCC compared to patients without HCC.MethodsThe single‐center sample measured PROs before and after transplant, which included 1521 multisurvey measurement points among 502 adults (67% male, 28% HCC, follow‐up time: <1–131 months). Data were analyzed using multivariable mixed‐effects models.ResultsLongitudinal PRO values did not differ between persons who received HCC exception points and those who did not have HCC. Patients with HCC who did not receive exception points had reduced physical HRQOL (P=.016), a late decline in mental HRQOL, and delayed reduction in anxiety (time‐by‐outcome interaction P<.050) compared to patients with HCC who received exception points.ConclusionTransplant recipients who received HCC exception points had PROs that were comparable to those of patients without HCC, and reported better physical HRQOL and reduced symptoms of anxiety compared to patients with HCC who did not receive exception points. These analyses demonstrate the impact of HCC exception points on PROs, and may help inform policy regarding HCC exception point allocation.
Pharmacokinetics and pharmacodynamics of dapagliflozin in children and adolescents with type 2 diabetes mellitus
Authors: Tirucherai G. S. , LaCreta F. , Ismat F. A. , Tang W. , Boulton D. W. 
Published: June 2016

AimsTo evaluate the pharmacokinetic (PK)/pharmacodynamic (PD) and safety profile of dapagliflozin in paediatric patients aged 10–17 years with type 2 diabetes mellitus (T2DM).MethodsPatients were randomized to a single oral dose of dapagliflozin 2.5, 5 or 10 mg. The PK characteristics for individual patients were derived by non‐compartmental methods. Urinary glucose excretion (UGE), fasting plasma glucose (FPG) and ease of swallowing were also evaluated.ResultsA total of 24 patients with a mean (range) body weight of 99.7 (61.5–169.5) kg received dapagliflozin. Dapagliflozin was rapidly absorbed after oral administration (median time to maximum plasma concentration ∼1.5 h) and systemic exposures to dapagliflozin and its 3‐O‐glucuronide metabolite appeared dose‐proportional. The mean 24‐h UGE increased in a dose‐related manner (52.8, 62.4 and 89.0 g for the 2.5, 5 and 10 mg groups, respectively). Mean FPG concentrations were lower for all dose groups on day 2 (6.9, 6.2 and 6.8 mmol/l for 2.5, 5 and 10 mg groups, respectively) than they were predose on day 1 (9.5, 8.5 and 8.2 mmol/l for 2.5, 5 and 10 mg groups, respectively). Six patients (25%) experienced ≥1 adverse event (AE), however, there was no dose‐related pattern. All AEs occurred only once and most were mild in intensity. Nearly all patients (n = 23; 95.8%) reported easy swallowing of the dapagliflozin tablets.ConclusionsDapagliflozin was well tolerated in this paediatric population, with no significant safety findings. PK/PD characteristics were similar to those observed in adults with T2DM, thereby supporting the hypothesis that the same dapagliflozin dosage as that used in adults can be evaluated in future phase III paediatric studies.
Is behaviour in a novel environment associated with bodily state in brown troutSalmo truttafry?
Authors: Näslund Joacim , Sandquist Lin , Johnsson Jörgen I. 
Published: June 2016

AbstractEnergetic status and body size are inconstant bodily states often considered to have feedback effects on behaviour. Feedbacks can be negative, like starvation‐threshold feedbacks (lower state values = increased risk taking due to higher energy need, and vice versa), or positive, like state‐dependent safety feedbacks (higher state values = lower vulnerability when expressing risky behaviours, and vice versa). Few studies have investigated feedback effects during the early‐life survival bottleneck of territorial species, when rapid growth is particularly important to maintain competitive ability. We investigated effects of body size and energetic status on the emergence latency into an unknown environment in highly territorial brown trout fry. We manipulated energetic status using high and low food rations in different combinations over two consecutive periods. In contrast to results from other small‐sized fishes, no general behavioural effects of energetic state were detected, but larger fish had on average shorter emergence latency. Starvation‐threshold feedbacks were not supported, but the size effect favours the presence of a state‐dependent safety feedback. When individuals were split into clusters characterised by long (>34 s) and short (<34 s) emergence latency, a state‐dependent safety feedback, based on energetic status, was found in the short‐latency cluster. Positive state‐dependent feedbacks may reflect the high level of competition among wild trout fry during the first summer, particularly among the more active individuals (i.e., from the short latency cluster). High state levels could be utilised to gain advantages over lesser competitors (e.g., to establish territories), particularly if the overall risk of starvation is higher than the risk of predation.
Partnering to change the world for people with haemophilia: 6th Haemophilia Global Summit, Prague, Czech Republic, 24–26th September 2015
Authors: Astermark Jan , Hart Dan , Lobet Sébastien , Blatný Jan , d'Oiron Roseline , Kenet Gili , Dolan Gerry , Libotte Valérie , Hermans Cedric 
Published: June 2016

AbstractThe 6th Haemophilia Global Summit was held in Prague, Czech Republic, in September 2015. The programme was designed by an independent Scientific Steering Committee of haemophilia experts and aimed to share optimal management strategies for haemophilia at all life stages, explore recent potential advances in the management of haemophilia A and B and discuss challenges in haemophilia care. In this supplement from the meeting, Dan Hart reviews the lessons that can be learnt from cost‐constrained environments with regard to improving care for people with haemophilia globally. Sébastien Lobet discusses the importance of physical activity for optimising care and Roseline d'Oiron and Jan Blatný consider the role of real‐world data in understanding the effect of treatment in a clinical setting over the long term and the true impact of treatment on the day‐to‐day life of the patient. Gili Kenet addresses the current challenges relating to the optimal management of prophylaxis, and Gerry Dolan and Cedric Hermans discuss the value of pharmacokinetic (PK) parameters in informing treatment decisions. Cedric Hermans and Valérie Libotte explore the importance of considering social and occupational development factors as an integral part of haemophilia care, and Jan Astermark reviews key strategies to predict and prevent inhibitor development.
Effects of chemical and microbial additives on clostridium development in sugarcane (Saccharum officinarum L.) ensiled with lime
Authors: Custódio Letícia , Morais Greiciele , Daniel João L. P. , Pauly Thomas , Nussio Luiz G. 
Published: June 2016

AbstractThe aim of this study was to compare strategies to prevent clostridium growth in sugarcane silages added with lime. In Experiment 1, treatments were (as fed basis): no additives (Control), 15 g lime kg−1 (L), 15 g lime kg−1 + 1.5 g sodium benzoate kg−1 (L+B), 15 g lime kg−1 + 0.7 g sodium nitrite kg−1 (L+N), 15 g lime kg−1 + Lactobacillus buchneri (5 × 105 cfu g−1) (L+LB) and 15 g lime kg−1 + Lactobacillus plantarum (5 × 105 cfu g−1) (L+LP). Plastic buckets (20 L) were used as experimental silos. Silage fermentative losses and aerobic stability were also evaluated. Lime treatment was not effective in decreasing dry matter (DM) losses compared with control silage (121 and 158 g kg−1, respectively). On the other hand, L+LB, L+B, L+LP and L+N treatments significantly decreased DM losses (70, 73, 87 and 98 g kg−1, respectively). The aerobic stability of silages L (172 h), L+B (155 h) and L+N (223 h) was substantially higher compared to those treated with L+LB (49 h), L+LP (48 h) and control (52 h). Control silages had low counts of clostridia (log 3.3 cfu g−1) whereas L and L+LB treated silages reached very high clostridium counts (log 6.7 and log 6.0 cfu g−1, respectively). The remaining treatments resulted in intermediate values. In Experiment 2, sugarcane treated with lime (15 g kg−1) received the following doses of sodium nitrite prior to ensiling: 0, 0.5, 1.0 and 1.5 g kg−1 as fed. Treatments did not affect clostridium counts but decreased butyric acid and 2,3‐butanediol. The silage with the highest nitrite dose decreased the butyric acid content substantially compared to other treatments but the final level (5.9 g kg−1 DM) was above the target value for well‐fermented silage. None of additives combined with lime were able to provide completely butyric acid free silages.
Identification of mutations in theF8andF9gene in families with haemophilia using targeted high‐throughput sequencing
Authors: Lyu C. , Xue F. , Liu X. , Liu W. , Fu R. , Sun T. , Wu R. , Zhang L. , Li H. , Zhang D. , Yang R. , Zhang L. 
Published: June 2016

IntroductionAt present, many methods are available for the genetic diagnosis of haemophilia, including indirect linkage analysis, direct sequencing. However, these methods are time‐consuming, labourious, and limited in their application. Therefore, the development of new, more effective techniques is necessary.AimTo detect theF8andF9gene mutations in patients with haemophilia and their female relatives in 29 haemophilia A (HA) and 11 haemophilia B (HB) families.MethodsFVIII:C and FIX:C were analyzed using one‐stage method, and factor VIII and factor IX inhibitors were tested using the Bethesda method. Intron 22 and one inversions were identified using long‐distance polymerase chain reaction (PCR) and standardPCR. Non‐inversion mutations of theF8andF9gene were identified by targeted high‐throughput sequencing. All mutations were verified by Sanger sequencing.ResultsIntron 22 inversion was detected in eightHAfamilies and intron one inversion was detected in oneHAfamily. Apart from the inversion mutations, 20 mutations were identified inHAfamilies, including 17 previously reported and three novel mutations: c.5724G>A (p.Trp1908*), c.6116‐1_6120delGAGTGTinsTCC(p.Lys2039Ilefs*13), and c.5220‐2A>C. We found a complex rearrangement inHA: intron one inversion concomitant with exon one deletion. InHB, eight recurrent mutations were detected, including six missense mutations and two nonsense mutations.ConclusionTargeted high‐throughput sequencing is an effective technique to detect theF8andF9gene mutations, especially for the discovery of novel mutations. The method is highly accurate, time‐saving and shows great advantage in uncovering large deletion mutations and also in distinguishing the wild‐type genotype and heterozygous large deletions.
Practical aspects of factor concentrate use in patients with von Willebrand disease undergoing invasive procedures: a European survey
Authors: Windyga J. , Dolan G. , Altisent C. , Katsarou O. , López Fernández M.‐F. , Zülfikar B. ,   the EHTSB
Published: June 2016

IntroductionThe bleeding propensity in von Willebrand disease (VWD) is usually moderate or mild and patients with VWD do not need continuous treatment, but do require extra increased haemostatic cover when undergoing dental or surgical procedures. Desmopressin can be effective in certain patient groups and this has been considered in a previous publication.AimThis paper now seeks to evaluate current knowledge and practice in the use of factor concentrate in the management of VWD patients undergoing invasive procedures.MethodsA literature search was performed on the use of factor concentrates to cover invasive procedures and a survey of current practice in a number of specialist haematology centres across Europe represented by the European Haemophilia Strategy Board was conducted.ResultsOur review of the literature and the results of the survey showed considerable heterogeneity in treatment regimens, and a lack of consistency in reporting of the variables that determine factor concentrate dosing and monitoring.ConclusionBy analysing the literature, examining guidelines and using consensus deliberation, this survey allowed the group to develop recommendations for management of VWD patients undergoing invasive procedures.
Usefulness of the Total Thrombus‐Formation Analysis System (T‐TAS) in the diagnosis and characterization of von Willebrand disease
Authors: Daidone V. , Barbon G. , Cattini M. G. , Pontara E. , Romualdi C. , Di Pasquale I. , Hosokawa K. , Casonato A. 
Published: June 2016

IntroductionThe heterogeneity of von Willebrand disease (VWD) makes its diagnosis a difficult task.MethodsWe report here on the usefulness of a microchip‐based flow‐chamber system, the total thrombus‐formation analysis system (T‐TAS), in the identification and characterization of VWD. Thirty VWD patients and 20 healthy subjects were studied with the T‐TAS platelet (PL) and atherome (AR) microchips developed for the in vitro assessment of platelet thrombus formation and fibrin‐rich platelet thrombus formation respectively.ResultsSamples from severe type 1 VWD, characterized by von Willebrand factor (VWF) levels below 10 U dL−1, failed to occlude either the PL or the AR chip capillaries, while the occlusion times were normal in patients with mild type 1 VWD (VWF above 25 U dL−1). PL and/or AR chip occlusion occurred, but took longer than normal, for samples from type Vicenza and type 1 VWD patients, whose VWF levels ranged between 10 and 25 U dL−1. No PL or AR chip capillary occlusion was seen for samples from patients with type 2A or 2B VWD featuring the absence of large VWF multimers, whereas no abnormalities emerged for type 2B patients with normal multimer patterns.ConclusionThe T‐TAS appears to be sensitive mainly to plasma VWF concentration and the presence of large multimers. Failure of the PL and AR chips to become occluded points to a lack of large VWF multimers, or type 1 VWD with VWF levels below 10 U dL−1. Although the T‐TAS does not assure a precise VWD diagnosis, it does point us in the right direction, and thus seems a useful global preliminary test.