Miracle-dz/UniFAR-FTUs huggingface dataset:
Familial clustering of non-nuclear autoantibodies and C3 and C4 complement components in systemic lupus erythematosus.

OBJECTIVE To determine whether key features of systemic lupus erythematosus (SLE), namely, production of non-nuclear antibodies (anti-C1q and anticardiolipin antibodies [aCL]) and depletion of complement components C3 and C4, aggregate in families. In addition, we examined relationships between anti-C1q and C3 and C4 levels. METHODS The study cohort comprised 1,037 predominantly white (82%) nuclear families in which at least 1 member had SLE. Associations of antibody measurements between probands and their unaffected siblings were examined using parametric and nonparametric analyses, along with associations between unaffected siblings and their parents. The heritability of anti-C1q, C3, and C4 was estimated, and interdependencies between these factors were examined in a regression model accounting for the family structure of the data set. RESULTS We demonstrated associations between siblings for anti-C1q (odds ratio [OR] 3.74, 95% confidence interval [95% CI] 2.65, 5.28) and IgG and IgM aCL (OR 4.08, 95% CI 1.83, 5.13 and OR 2.06, 95% CI 1.46, 2.91, respectively) and, for anti-C1q, association between unaffected parents and their unaffected offspring (OR 4.34, 95% CI 2.16, 8.72). We also demonstrated significant heritability of anti-C1q, C3, and C4 (approximately 45%). Anti-C1q was negatively associated with C3 and C4 in SLE probands but not in their healthy relatives. CONCLUSION Non-nuclear antibodies and C3 and C4 cluster within the families of SLE probands, suggesting that specific autoantibody formation is partly genetically determined, even if the total genetic effect in unaffected relatives is insufficient to cause disease. Anti-C1q antibodies accelerate C3 and C4 depletion in patients with SLE but have no effect in the absence of disease.
An orally bioavailable spleen tyrosine kinase inhibitor delays disease progression and prolongs survival in murine lupus.

OBJECTIVE To assess whether R788, an orally bioavailable small molecule inhibitor of spleen tyrosine kinase (Syk)-dependent signaling, could modulate disease in lupus-prone (NZB x NZW)F1 (NZB/NZW) mice via inhibition of Fc receptor (FcR) and B cell receptor signaling. METHODS R788 was administered to NZB/NZW mice before and after disease onset. Proteinuria, blood urea nitrogen levels, and autoantibody titers were examined periodically, and overall survival and renal pathologic features were assessed following long-term treatment (24-34 weeks). The distribution and immunophenotype of various splenic T cell and B cell subpopulations were evaluated at the time of study termination. Arthus responses in NZB/NZW mice pretreated with R788 or Fc-blocking antibody (anti-CD16/32) were also examined. RESULTS When R788 was administered prior to or after disease onset, it delayed the onset of proteinuria and azotemia, reduced renal pathology and kidney infiltrates, and significantly prolonged survival of lupus-prone NZB/NZW mice; autoantibody titers were minimally affected throughout the study. Dose-dependent reductions in the numbers of CD4+ activated T cells expressing high levels of CD44 or CD69 were apparent in spleens from R788-treated mice. Minimal effects on the numbers of naive T cells expressing CD62 ligand and total CD8+ T cells per spleen were observed following long-term drug treatment. R788 pretreatment resulted in reduced Arthus responses in NZB/NZW mice, similar to results obtained in mice pretreated with FcR-blocking antibody. CONCLUSION We demonstrate that a novel Syk-selective inhibitor prevents the development of renal disease and treats established murine lupus nephritis. These data suggest that Syk inhibitors may be of therapeutic benefit in human lupus and related disorders.
Successful liver transplantation following veno‐arterial extracorporeal membrane oxygenation in a child with fulminant Wilson disease and severe pulmonary hemorrhage: A case report

Son SK, Oh SH, Kim KM, Lee YJ, Jhang WK, Park SJ, Shin HJ, Park J‐J, Kim TH, Kim DY, Hwang S, Park K‐M, Lee Y‐J, Lee S‐G. Successful liver transplantation following veno‐arterial extracorporeal membrane oxygenation in a child with fulminant Wilson disease and severe pulmonary hemorrhage: A case report. Pediatr Transplantation 2011. © 2011 John Wiley & Sons A/S.
Psychosocial Assessment Tool 2.0_General: Validity of a psychosocial risk screener in a pediatric kidney transplant sample

Pai ALH, Tackett A, Ittenbach RF, Goebel J. Psychosocial Assessment Tool 2. 0_General: Validity of a psychosocial risk screener in a pediatric kidney transplant sample. Pediatr Transplantation 2012: 16: 92–98. © 2011 John Wiley & Sons A/S.
Effects of long-term strontium ranelate treatment on the risk of nonvertebral and vertebral fractures in postmenopausal osteoporosis: Results of a five-year, randomized, placebo-controlled trial.

OBJECTIVE This study was undertaken to assess the effect of strontium ranelate on nonvertebral and vertebral fractures in postmenopausal women with osteoporosis in a 5-year, double-blind, placebo-controlled trial. METHODS A total of 5,091 postmenopausal women with osteoporosis were randomized to receive either strontium ranelate at 2 gm/day or placebo for 5 years. The main efficacy criterion was the incidence of nonvertebral fractures. In addition, incidence of hip fractures was assessed, by post hoc analysis, in the subset of 1,128 patients who were at high risk of fractures (age 74 years or older with lumbar spine and femoral neck bone mineral density T scores -2.4 or less). The incidence of new vertebral fractures was assessed, using the semiquantitative method described by Genant, in the 3,646 patients in whom spinal radiography (a nonmandatory procedure) was performed during the course of the study. Fracture data were analyzed using the Kaplan-Meier survival method. RESULTS Of the 5,091 patients, 2,714 (53%) completed the study up to 5 years. The risk of nonvertebral fracture was reduced by 15% in the strontium ranelate group compared with the placebo group (relative risk 0.85 [95% confidence interval 0.73-0.99]). The risk of hip fracture was decreased by 43% (relative risk 0.57 [95% confidence interval 0.33-0.97]), and the risk of vertebral fracture was decreased by 24% (relative risk 0.76 [95% CI 0.65-0.88]) in the strontium ranelate group. After 5 years, the safety profile of strontium ranelate remained unchanged compared with the 3-year findings. CONCLUSION Our findings indicate that treatment of postmenopausal osteoporosis with strontium ranelate results in a sustained reduction in the incidence of osteoporotic nonvertebral fractures, including hip fractures, and vertebral fractures over 5 years.
A New Trick for an Old Dog: Applying Developmental Trajectories to Inform Drug Use Progression

The frequent criticisms of the “gateway hypothesis” have led scholars to note the importance of considering the role of intra-individual change for drug use progression. While studies employing drug use trajectories have added considerably to our understanding of drug use comorbidity, the extent to which trajectories inform drug use progression remains largely unknown despite the fact that there are several theoretical reasons to suspect that intra-individual change is important to the gateway phenomenon. The current study employs latent class growth models using a sample from the Boys Town study of adolescent drug and drinking behavior. The results demonstrate that knowing how gateway drug use changes over time provides important information above and beyond knowing frequency of gateway use for predicting harder drug use trajectories. Implications of the empirical findings and directions for future research are discussed.
Fitness, fatigue, disease activity, and quality of life in pediatric lupus.

OBJECTIVE To measure aerobic fitness (maximum oxygen consumption [VO(2peak)]), fatigue, quality of life (QOL), and disease activity in young persons with systemic lupus erythematosus (SLE), and to determine an equation for predicting VO(2peak) from the distance walked in 6 minutes (6MW). METHODS Fifteen young patients ages 12-19 years with SLE participated. VO(2) was measured by a graded treadmill exercise test. Submaximal exercise intensity was determined from the ventilatory anaerobic threshold. Submaximal aerobic capacity was measured using the 6MW. Patient questionnaires included measures of fatigue, QOL, and physical activity. Physician questionnaires included the Systemic Lupus Erythematosus Disease Activity Index and the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index. RESULTS Compared with age-matched norms, our patients had moderate impairment in aerobic fitness, with a mean +/- SD VO(2peak) of 31.1 +/- 7.9 ml/minute/kg and a mean 6MW distance Z score of -2.4 +/- 2.3. The regression equation to predict VO(2peak) (ml/minute/kg) from 6MW was as follows: 57.1 + [0.038 x distance (meters)] + (-0.35 x maximal heart rate) (R = 0.67, P = 0.027). Ten subjects (67%) reported significant fatigue. There was no significant correlation of fatigue with fitness measures. Neither fatigue nor fitness was significantly correlated with disease activity, disease damage, or QOL measures. CONCLUSION Young SLE patients have lower aerobic fitness than reference norms. The 6MW may be used as a marker of fitness, but it is preferable to determine VO(2) with a graded exercise test. Fatigue is a significant symptom in young SLE patients. The application of fatigue measures in young persons is exploratory. The relationship between fatigue and aerobic fitness is not clear.
Adaptive sliding mode dynamic controller with integrator in the loop for nonholonomic wheeled mobile robot trajectory tracking

In this paper, novel adaptive sliding mode dynamic controller with integrator in the loop is proposed for nonholonomic wheeled mobile robot (WMR). The modified kinematics controller is used to generate kinematics velocities of WMR which are subsequently used as the input to adaptive dynamic controller. Actuator dynamics are also derived to generate actuator voltage of WMR through torque and velocity vectors. Stability of both kinematics and dynamic controller is presented using Lyapunov stability analysis. The proposed scheme is verified and validated using computer simulations for tracking the desired trajectory of WMR. The performance of proposed scheme is compared with standard backstepping kinematics controller and classical sliding mode control. In addition, the performance is further compared with standard backstepping kinematics controller with adaptive sliding mode controller without integrator. It is shown that the proposed scheme exhibits zero steady state error, fast error convergence and robustness in the presence of continuous disturbances and uncertainties.
Reproductive intentions and fertility rates.

An evaluation of whether the stated desire to terminate childbearing is a valid predictor of fertility using national data from 134 surveys conducted over the past 10-15 years reveals a strong relationship between the total fertility rate and the percentage of women who want no more children even when the comparison is restricted to developeing countries only. More than two-thirds of this strong association acts through contraceptive prevalence. Further analyses confirm the relationship between intention and fertility across time for those countries for which there were two sources of data about five years apart (on average). The conclusion reached is that the porportion of women reporting that they want no more children has high predictive validity and is therefore a useful tool for short-term fertility forecasting. (SUMMARY IN FRE AND SPA)
Academic achievement, BMI, and fast food intake of American and Japanese college students

Purpose – The purpose of this paper is to investigate the relationship between grade point average (GPA), body mass index (BMI), and fast food intake, and to test five different hypotheses regarding these target variables.Design/methodology/approach – In total, 57 American and 72 Japanese college students are recruited from two different universities and the participants provide their gender, age, weekly fast food intake, GPA, and their actual height and weight are measured.Findings – It is found that GPA is negatively correlated with BMI and fast food intake, and BMI and fast food intake are positively correlated in the American sample. A negative correlation between GPA and fast food intake is also found in the Japanese sample. Although no differences of GPA are found in regard to the physiques of both American and Japanese samples, a marginally significant difference of GPA is found in the American sample in regard to their fast food intake. In the Japanese sample, a significant difference of GPA is fo.
Cooperative tracking and estimation of linear multi-agent systems with a dynamic leader via iterative learning

This paper presents a new iterative learning design approach for cooperative tracking and estimation of linear multi-agent systems with a dynamic leader. The input of the dynamic leader is unavailable to all follower agents. Distributed iterative learning controllers, based on the relative state information of neighbouring agents, are proposed for tracking the dynamic leader, and meanwhile identifying the unknown input of the leader. Stability and convergence of the proposed controller are established using Lyapunov–Krasovskii theory. Further, this result is extended to the output feedback case where only partial states can be measured. A local observer is constructed to recover the unmeasurable states. Then, distributed iterative learning tracking controllers, based on the relative observed states of neighbouring agents, are devised. For both cases, the main advantage of the proposed controllers allows for tracking the dynamic leader for undirected graphs without knowing the input of the leader, and meanwhile identifying the unknown input of the leader using distributed iterative learning laws. An example is given to show the efficacy and utility of the theoretical results.
Shorter sentences for drug mules: The early impact of the sentencing guidelines in England and Wales

Abstract Aims: In February 2012, new sentencing guidelines for drug offences became effective in all courts in England and Wales. An explicit aim was to reduce the length of sentences for drug “mules” and so make them more proportionate. Methods: This article examines their early impact drawing on data from the Court Proceedings Database and the Crown Court Sentencing Survey for importing/exporting a Class A drug. Findings: Overall, the guidelines have achieved their intended aim. The length of the average custodial sentence for drug trafficking fell following the introduction of the guidelines, largely due to taking defendants' roles into account. Notably, three-quarters of those in “lesser” roles received sentences less than four years, representing an important change. Nonetheless, around 10% of mules received very long sentences due to the continued use of drug weight in sentencing. Conclusion: The new guidelines represent an internationally important innovation in drug policy reform.
An EM-based identification algorithm for a class of hybrid systems with application to power electronics

In this paper we present an identification algorithm for a class of continuous-time hybrid systems. In such systems, both continuous-time and discrete-time dynamics are involved. We apply the expectation-maximisation algorithm to obtain the maximum likelihood estimate of the parameters of a discrete-time model expressed in incremental form. The main advantage of this approach is that the continuous-time parameters can directly be recovered. The technique is particularly well suited to fast-sampling rates. As an application, we focus on a standard identification problem in power electronics. In this field, our proposed algorithm is of importance since accurate modelling of power converters is required in high- performance applications and for fault diagnosis. As an illustrative example, and to verify the performance of our proposed algorithm, we apply our results to a flying capacitor multicell converter.
Age- and sex-related patterns of serum interferon-alpha activity in lupus families.

OBJECTIVE Interferon-alpha (IFNalpha) levels are elevated in many patients with systemic lupus erythematosus (SLE) and may play a primary role in its pathogenesis. The purpose of this study was to determine whether serum IFNalpha activity in SLE patients and their healthy first-degree relatives is highest in early adulthood, when the incidence of SLE is greatest. METHODS Serum samples from 315 SLE patients, 359 healthy first-degree relatives, and 141 healthy unrelated donors were measured for IFNalpha activity using a functional reporter cell assay. IFNalpha activity was analyzed in relation to age, and subgroups with high levels of IFNalpha activity were identified within the large data sets using a Mann-Whitney sliding window segmentation algorithm. The significance of each subgrouping was ranked by Kruskal-Wallis testing. RESULTS Age was inversely correlated with IFNalpha activity in female SLE patients (r = -0.20, P = 0.001) as well as their healthy female first-degree relatives (r = -0.16, P = 0.02). In male patients and their healthy male first-degree relatives, there was no significant overall correlation between age and serum IFNalpha activity. The segmentation algorithm revealed significantly increased IFNalpha activity between the ages of 12 and 22 years in female SLE patients and between the ages of 16 and 29 years in male SLE patients. Both male and female healthy first-degree relatives had significantly decreased IFNalpha activity after the age of 50 years. CONCLUSION Serum IFNalpha activity is higher in younger individuals in the SLE family cohorts, and this tendency is accentuated in affected individuals. This age-related pattern of IFNalpha activity may contribute to the increased incidence of SLE in early adulthood, and interestingly, males and females had similar age-related patterns of IFNalpha activity.
A pilot study to evaluate the safety and efficacy of the long-acting interleukin-1 inhibitor rilonacept (interleukin-1 Trap) in patients with familial cold autoinflammatory syndrome.

OBJECTIVE Familial cold autoinflammatory syndrome (FCAS) is caused by mutations in the CIAS1 gene, leading to excessive secretion of interleukin-1beta (IL-1beta), which is associated with cold-induced fevers, joint pain, and systemic inflammation. This pilot study was conducted to assess the safety and efficacy of rilonacept (IL-1 Trap), a long-acting IL-1 receptor fusion protein, in patients with FCAS. METHODS Five patients with FCAS were studied in an open-label trial. All patients received an initial loading dose of 300 mg of rilonacept by subcutaneous injection, were evaluated 6 and 10 days later for clinical efficacy, and remained off treatment until a clinical flare occurred. At the time of flare, patients were again treated with 300 mg of rilonacept and then given maintenance doses of 100 mg/week. Patients whose FCAS was not completely controlled were allowed a dosage increase to 160 mg/week and then further to 320 mg/week during an intrapatient dosage-escalation phase. Safety, disease activity measures (daily diary reports of rash, joint pain and/or swelling, and fevers), health quality measures (Short Form 36 health survey questionnaire), and serum markers of inflammation (erythrocyte sedimentation rate [ESR], high-sensitivity C-reactive protein [hsCRP], serum amyloid A [SAA], and IL-6) were determined at 3, 6, 9, 12, and 24 months after initiation of rilonacept and were compared with baseline values. RESULTS In all patients, clinical symptoms typically induced by cold (rash, fever, and joint pain/swelling) improved within days of rilonacept administration. Markers of inflammation (ESR, hsCRP, and SAA) showed statistically significant reductions (P < 0.01, P < 0.001, and P < 0.001, respectively) at doses of 100 mg. Dosage escalation to 160 mg and 320 mg resulted in subjectively better control of the rash and joint pain. Furthermore, levels of the acute-phase reactants ESR, hsCRP, and SAA were lower at the higher doses; the difference was statistically significant only for the ESR. All patients continued taking the study drug. The drug was well-tolerated. Weight gain in 2 patients was noted. No study drug-related serious adverse events were seen. CONCLUSION In this study, we present 2-year safety and efficacy data on rilonacept treatment in 5 patients with FCAS. The dramatic improvement in clinical and laboratory measures of inflammation, the sustained response, and the good tolerability suggest that this drug may be a promising therapeutic option in patients with FCAS, and the data led to the design of a phase III study in this patient population.
Relation between relative growth rate, endogenous gibberellins, and the response to applied gibberellic acid for Plantago major.

Relationships between relative growth rate (RGR), endogenous gibberellin (GA) concentration and the response to application of gibberellic acid (GA(3) ) were studied for two inbred lines of Plantago major L. , which differed in RGR. A4, the fast-growing inbred line, had a higher free GA concentration than the slow-growing W9, as analyzed by enzyme immunoassay. GA(3) application increased total plant weight and RGR(3) particularly for the slow-growing line. Chlorophyll a content and photosynthetic activity per unit leaf area were decreased, while transpiration rate was unaffected by GA(3) application. The increase in RGR by GA(3) application was associated with an increased leaf weight ratio; specific leaf area and percentage of dry matter in the leaves were only temporarily affected. Root respiration rate per unit dry weight was unaffected. The correlation between low RGR, low GA concentration and high responsiveness to applied GA(3) supports the contention that gibberellins are involved in the regulation of RGR. However, the transient influence of GA(3) application on some growth components suggests the involvement of other regulatory factors in addition to GA.
Implementing the Short-Term Assessment of Risk and Treatability (START) in a forensic mental health service †

Aims and Method To evaluate the practical utility and face validity of a new risk assessment guideline, the Short-Term Assessment of Risk and Treatability (START), within a forensic mental health service. Staff attended training and subsequently used the START to assess and formulate risk. Staff then completed an evaluation questionnaire about their experiences of using the START. Results The study proved useful in evaluating the practical utility and face validity of the START. the START demonstrated both good practical utility and face validity. Areas of difficulty in completing the START were identified and areas for developing the START were highlighted. Clinical Implications The service subsequently implemented a revised version of the START into routine practice. the results of the pilot study suggest that the START can usefully assist in structuring risk judgements in practice.