nils-herrmann/clean_scirepeval huggingface dataset:
Outcome of congenital acute lymphoblastic leukemia treated on the Interfant-99 protocol.

Acute lymphoblastic leukemia (ALL) diagnosed in the first month of life (congenital ALL) is very rare. Although congenital ALL is often assumed to be fatal, no studies have been published on outcome except for case reports. The present study reports the outcome of 30 patients with congenital ALL treated with the uniform Interfant-99 protocol, a hybrid regimen combining ALL treatment with elements designed for treatment of acute myeloid leukemia. Congenital ALL was characterized by a higher white blood cell count and a strong trend for higher incidence of MLL rearrangements and CD10-negative B-lineage ALL compared with older infants. Induction failure rate was 13% and not significantly different from that in older infants (7%, P = .14), but relapse rate was significantly higher in congenital ALL patients (2-year cumulative incidence [SE] was 60.0 [9.3] vs 34.2 [2.3], P < .001). Two-year event-free survival and survival of congenital ALL patients treated with this protocol was 20% (SE 9.1%). Early death in complete remission and treatment delays resulting from toxicity were not different. The survival of 17% after last follow-up, combined with a toxicity profile comparable with that in older infants, justifies treating congenital ALL with curative intent.
Haemodialysing babies weighing <8 kg with the Newcastle infant dialysis and ultrafiltration system (Nidus): comparison with peritoneal and conventional haemodialysis

To compare the efficacy of the Newcastle infant dialysis and ultrafiltration system (Nidus) with peritoneal dialysis (PD) and conventional haemodialysis (HD) in infants weighing <8 kg. We compared the urea, creatinine and phosphate clearances, the ultrafiltration precision, and the safety of the Nidus machine with PD in 7 piglets weighing 1–8 kg, in a planned randomised cross-over trial in babies, and in babies for whom no other therapy existed, some of whom later graduated to conventional HD. Two babies entered the randomised trial; 1 recovered rapidly on PD, the other remained on the Nidus as PD failed. Additionally, 9 babies were treated on the Nidus on humanitarian grounds: 3 because of failed PD, and 3 with permanent kidney failure later converted to conventional HD. We haemodialysed 10 babies weighing between 1.8 and 5.9 kg for 2,475 h during 354 Nidus sessions without any clinically important incidents, and without detectable haemolysis. Single-lumen vascular access was used with no blood priming of circuits. The urea, creatinine and phosphate clearances using the Nidus were around 1.5 to 2.0 ml/min in piglets and babies, and were consistently higher than PD clearances, which ranged from about 0.2 to 0.8 ml/min (p ≤ 0.0002 for each chemical). Ultrafiltration was achieved to microlitre precision by the Nidus, but varied widely with PD. Fluid removal using conventional HD was imprecise and resulted in some hypovolaemic episodes requiring correction. The Nidus can provide HD in the Pediatric Intensive Care Unit (PICU) and outpatient intermittent HD without blood priming for babies weighing <8 kg, It generates higher dialysis clearances than PD, and delivers more precise ultrafiltration control than either PD or conventional HD.
Development of a genotype 325-specific proCPU/TAFI ELISA.

A Thr/Ile polymorphism at position 325 in the coding region of proCPU has been reported. Immunological assays, fully characterized (including genotype dependency), are required for the quantitation of proCPU levels.



We have generated a panel of monoclonal antibodies against human, plasma-derived proCPU. Two combinations exhibiting distinct reactivities were selected for measurement of proCPU in plasma. T12D11/T28G6-HRP yielded values of 10.1+/-3.1 microg/mL (mean+/-SD, n=86; normal donors), and T32F6/T9G12-HRP yielded values of 5.4+/-3.0 microg/mL. Grouping according to the 325 genotype demonstrated that T12D11/T28G6-HRP was independent to this polymorphism whereas T32F6/T9G12-HRP revealed a complete lack of reactivity with the Ile/Ile genotype (ie, 0.0+/-0.0, 4.2+/-1.7, and 7.3+/-2.9 microg/mL for the Ile/Ile, Ile/Thr, and Thr/Thr isoforms, respectively). Commercially available antigen assays appeared to be partially dependent on the 325 genotype (eg, 44+/-8.9% and 100+/-30% for the Ile/Ile and Thr/Thr isoforms, respectively).



Our data demonstrate that great care should be taken when evaluating proCPU antigen values as a putative causative agent or as a diagnostic risk marker for cardiovascular events.
Safety of phosphatidylserine containing omega3 fatty acids in ADHD children: a double-blind placebo-controlled trial followed by an open-label extension.

To evaluate the safety of phosphatidylserine (PS) enriched with omega3 fatty acids, mainly eicosapentaenoic (PS-Omega3) in children with attention-deficit hyperactivity disorder (ADHD).



Two hundred children diagnosed with ADHD were randomised to receive either PS-Omega3 (300mg PS-Omega3/day) or placebo for 15 weeks. One hundred and fifty children continued into an open-label extension for an additional 15 weeks in which they all consumed PS-Omega3 (150mg PS-Omega3/day). Standard blood biochemical and haematological safety parameters, blood pressure, heart rate, weight and height were evaluated. Adverse events and the Side Effect Rating Scale were also assessed.



One hundred and sixty-two participants completed the double-blind phase. No significant differences were noted between the two study groups in any of the safety parameters evaluated. One hundred and forty participants completed the open-label phase. At the end of this phase, no significant changes from baseline were observed in any of the studied parameters among participants who consumed PS-Omega3 for 30 weeks.



Study results demonstrate that consumption of PS-Omega3 by children with ADHD, as indicated in a 30-week evaluation period, is safe and well tolerated, without any negative effect on body weight or growth.
A multifaceted health-service intervention in remote Aboriginal communities: 3-year follow-up of the impact on diabetes care.

To examine the trends in processes of diabetes care and in participant outcomes after an intervention in two remote regions of Australia.



Follow-up study over 3 years.



Seven health centres in the Tiwi Islands and the Katherine West region of the Northern Territory.



137 Aboriginal people with type 2 diabetes.



Implementation of a multifaceted trial, including transfer of purchasing and planning responsibility to local health boards, the development and dissemination of clinical guidelines supported by electronic registers, recall and reminder systems and associated staff training, and audit and feedback.



Trends in the proportion of Aboriginal people receiving services in accordance with clinical guidelines and in the proportion for whom specified levels of blood pressure and glycosylated haemoglobin (HbA(1c)) were achieved; health staff perceptions of barriers to effective service delivery.



An initial improvement in overall service levels from 40% to 49% was not fully sustained over the 3-year period. The overall proportion of services delivered varied from 22% to 64% between communities and over time. The proportion of participants whose most recent HbA(1c) level was less than 7% improved from 19% to 32%, but there was little change in blood pressure control. Perceived barriers to service delivery included discontinuities in staffing, lack of work-practice support and patients' acceptance of services.



Multifaceted interventions can improve quality of care in this environment, but achieving sustainable, high-quality care in a range of services and local conditions presents particular challenges. Developing and testing strategies for consistent and sustained improvement should be a priority for service providers and researchers.
Effect of the GABAB agonist baclofen in patients with symptoms and duodeno-gastro-oesophageal reflux refractory to proton pump inhibitors

A subset of patients with gastro-oesophageal reflux disease (GORD) with refractory symptoms during therapy with proton pump inhibitors (PPIs), have persistent non-acid duodenogastro-oesophageal reflux (duodenal reflux). The aim of the present study was to investigate the effect of the GABAB receptor agonist baclofen, which was shown to inhibit the occurrence of transient lower oesophageal sphincter relaxations (TLOSRs) in patients with persistent non-acid duodenal reflux during PPI therapy.  Patients were eligible for the study if they had persistent reflux symptoms, normal pH monitoring, and pathological Bilitec monitoring during PPI treatment. Upper gastrointestinal endoscopy and reflux symptom score were performed at the beginning of the study. Baclofen 5 mg three times daily was associated with treatment, and was increased by 5 mg every fourth day until a maintenance dose of 20 mg three times daily was reached. A reflux symptom questionnaire, ambulatory pH monitoring, and Bilitec monitoring were repeated four days later while PPI and baclofen were continued. All data are given as mean (SEM) or median (interquartile range) and were compared using the Student’s t test or the MannWhitney U test.  Sixteen patients (11 women, mean age 46 (3) years) with persistent heartburn or regurgitation for at least three months, in spite of PPI therapy, were included in the study. Erosive oesophagitis was present in seven patients (five with grade 1, two with grade 2). Under PPI therapy alone, all patients had normal acid exposure (0.3 (0.05; 2.2)% of the time) but pathological duodenal reflux exposure (13.8 (11.8; 15.5)% of the time). After addition of baclofen 20 mg three times daily, acid exposure was similar (0.4 (0.15; 2.3)% of the time; NS) but duodenal reflux had significantly decreased (6.1 (0.8; 10.3)% of the time; p,0.05). The number of duodenal reflux episodes and the number of longlasting duodenal reflux episodes (.5 minutes) was decreased, respectively, from 23 (14.5; 34) to 12 (5; 21) (p = 0.06) and from 5 (3; 8) to 2 (0.5;4.5) (p,0.05). The cumulative severity score for 14 reflux symptoms decreased from 10.3 (1.7) to 5.8 (1.3) (p,0.01). Four patients reported mild side effects of nausea or drowsiness.  The GABAB receptor agonist baclofen improves duodenal reflux and associated reflux symptoms that persist during PPI therapy.
Artificial Liver Support System Improves Short- and Long-Term Outcomes of Patients With HBV-Associated Acute-on-Chronic Liver Failure

For patients with acute-on-chronic liver failure (ACLF), artificial liver support system (ALSS) may help prolong lifespan and function as a bridge to liver transplantation (LT), but data on its long-term benefit are lacking. We conducted this prospective, controlled study to determine the efficacy of ALSS and the predictors of mortality in patients with hepatitis B virus (HBV)-associated ACLF.From January 2003 to December 2007, a total of 234 patients with HBV-associated ACLF not eligible for LT were enrolled in our study. They were allocated to receive either plasma exchange centered ALSS plus standard medical therapy (SMT) (ALSS group, n=104) or SMT alone (control group, n=130). All the patients were followed-up for at least 5 years, or until death.At 90 days, the survival rate of ALSS group was higher than that of the control group (62/104 [60%] vs 61/130 [47%], respectively; P<0.05). Median survival was 879 days in the ALSS group (43% survival at 5 years) and 649 days in the control group (31% survival at 5 years, log-rank P<0.05). ALSS was found to be associated with favorable outcome of these patients by both univariate and multivariate analysis. Multivariate Cox regression analysis also revealed that lower serum sodium levels, higher grades of encephalopathy, presence of cirrhosis, hepatorenal syndrome, and higher model for end-stage liver disease scores were independent predictors for both 90-day and 5-year mortality due to ACLF.Our findings suggest that ALSS is safe and may improve the short- and long-term prognosis of patients with HBV-associated ACLF.
Azúcar y nervios: explanatory models and treatment experiences of Hispanics with diabetes and depression.

This study examined the explanatory models of depression, perceived relationships between diabetes and depression, and depression treatment experiences of low-income, Spanish-speaking, Hispanics with diabetes and depression. A purposive sample (n=19) was selected from participants enrolled in a randomized controlled trial conducted in Los Angeles, California (United States) testing the effectiveness of a health services quality improvement intervention. Four focus groups followed by 10 in-depth semi-structured qualitative interviews were conducted. Data were analyzed using the methodology of coding, consensus, co-occurrence, and comparison, an analytical strategy rooted in grounded theory. Depression was perceived as a serious condition linked to the accumulation of social stressors. Somatic and anxiety-like symptoms and the cultural idiom of nervios were central themes in low-income Hispanics' explanatory models of depression. The perceived reciprocal relationships between diabetes and depression highlighted the multiple pathways by which these two illnesses impact each other and support the integration of diabetes and depression treatments. Concerns about depression treatments included fears about the addictive and harmful properties of antidepressants, worries about taking too many pills, and the stigma attached to taking psychotropic medications. This study provides important insights about the cultural and social dynamics that shape low-income Hispanics' illness and treatment experiences and support the use of patient-centered approaches to reduce the morbidity and mortality associated with diabetes and depression.
Association between perceived life chaos and medication adherence in a postmyocardial infarction population.

The benefits of medication adherence to control cardiovascular disease (CVD) are well defined, yet multiple studies have identified poor adherence. The influence of life chaos on medication adherence is unknown. Because this is a novel application of an instrument, our preliminary objective was to understand patient factors associated with chaos. The main objective was to evaluate the extent to which an instrument designed to measure life chaos is associated with CVD-medication nonadherence.



Using baseline data from an ongoing randomized trial to improve postmyocardial infarction (MI) management, multivariable logistic regression identified the association between life chaos and CVD-medication nonadherence. Patients had hypertension and a myocardial infarction in the past 3 years (n=406). Nearly 43% reported CVD-medication nonadherence in the past month. In simple linear regression, the following were associated with higher life chaos: medication nonadherence (β=1.86; 95% confidence interval [CI], 0.96-2.76), female sex (β=1.22; 95% CI [0.22-2.24]), minority race (β=1.72; 95% CI [0.78-2.66]), having less than high school education (β=2.05; 95% CI [0.71-3.39]), low health literacy (β=2.06; 95% CI [0.86-3.26]), and inadequate financial status (β=1.93; 95% CI [0.87-3.00]). Being married (β=-2.09, 95% CI [-3.03 to -1.15]) was associated with lower life chaos. As chaos quartile increased, patients exhibited more nonadherence. In logistic regression, adjusting for sex, race, marital status, employment, education, health literacy, and financial status, a 1-unit life chaos increase was associated with a 7% increase (odds ratio, 1.07; 95% CI [1.02-1.12]) in odds of reporting medication nonadherence.



Our results suggest that life chaos may be an important determinant of medication adherence. Life chaos screenings could identify those at risk for nonadherence.
A brief mindfulness-based cognitive behavioral intervention improves sexual functioning versus wait-list control in women treated for gynecologic cancer.

GOAL
The goal of this study was to evaluate a mindfulness-based cognitive behavioral intervention for sexual dysfunction in gynecologic cancer survivors compared to a wait-list control group.



Thirty-one survivors of endometrial or cervical cancer (mean age 54.0, range 31-64) who self-reported significant and distressing sexual desire and/or sexual arousal concerns were assigned either to three, 90-minute mindfulness-based cognitive behavior therapy sessions or two months of wait-list control prior to entering the treatment arm. Validated measures of sexual response, sexual distress, and mood, as well as laboratory-evoked physiological and subjective sexual arousal were assessed at pre-, one month post-, and 6-months following treatment.



There were no significant effects of the wait-list condition on any measure. Treatment led to significant improvements in all domains of sexual response, and a trend towards significance for reducing sexual distress. Perception of genital arousal during an erotic film was also significantly increased following the intervention despite no change in physiologically-measured sexual arousal.



A brief mindfulness-based intervention was effective for improving sexual functioning. Geographic restrictions permitted only a select sample of survivors to participate, thus, the generalizability of the findings is limited. Future studies should aim to develop online modalities for treatment administration to overcome this limitation.
Metabolic Control With the Bio-inspired Artificial Pancreas in Adults With Type 1 Diabetes: A 24-Hour Randomized Controlled Crossover Study.

The Bio-inspired Artificial Pancreas (BiAP) is a closed-loop insulin delivery system based on a mathematical model of beta-cell physiology and implemented in a microchip within a low-powered handheld device. We aimed to evaluate the safety and efficacy of the BiAP over 24 hours, followed by a substudy assessing the safety of the algorithm without and with partial meal announcement. Changes in lactate and 3-hydroxybutyrate concentrations were investigated for the first time during closed-loop.



This is a prospective randomized controlled open-label crossover study. Participants were randomly assigned to attend either a 24-hour closed-loop visit connected to the BiAP system or a 24-hour open-loop visit (standard insulin pump therapy). The primary outcome was percentage time spent in target range (3.9-10 mmol/l) measured by sensor glucose. Secondary outcomes included percentage time in hypoglycemia (<3.9 mmol/l) and hyperglycemia (>10 mmol/l). Participants were invited to attend for an additional visit to assess the BiAP without and with partial meal announcements.



A total of 12 adults with type 1 diabetes completed the study (58% female, mean [SD] age 45  years, BMI 25  kg/m(2), duration of diabetes 22  years and HbA1c 7.4 [0.7]% [58 (8) mmol/mol]). The median (IQR) percentage time in target did not differ between closed-loop and open-loop (71% vs 66.9%, P = .9). Closed-loop reduced time spent in hypoglycemia from 17.9% to 3.0% (P < .01), but increased time was spent in hyperglycemia (10% vs 28.9%, P = .01). The percentage time in target was higher when all meals were announced during closed-loop compared to no or partial meal announcement (65.7% [53.6-80.5] vs 45.5% [38.2-68.3], P = .12).



The BiAP is safe and achieved equivalent time in target as measured by sensor glucose, with improvement in hypoglycemia, when compared to standard pump therapy.
Variation in dinucleotide (GT) repeat sequence in the first exon of the STAT6 gene is associated with atopic asthma and differentially regulates the promoter activity in vitro.

U pregulation of the IL-4/IL-13 mediated Th2 response is a characteristic of allergic diseases such as asthma, a common and often debilitating disease. STAT6 is a critical signalling molecule in the Th2 signalling pathway, and mice lacking STAT6 are protected from allergic pulmonary manifestations. The importance of STAT6 in asthma is also evident from studies showing that STAT6 gene expression is markedly upregulated in airway epithelial cells in asthma. STAT6 is important in the expression of VCAM-1 in endothelial cells and of chemokines, such as eotaxin, in epithelial cells following stimulation with IL-4 and IL-13. As a consequence, STAT6 has been considered a strong candidate for predisposition to atopic asthma. Indeed, the human STAT6 gene is mapped to chromosome 12q13.32q14.1, a region linked to total serum IgE concentration and atopy in several populations. A number of common polymorphisms have been identified, including a GT repeat in exon 1 and three common SNPs (G4219A, A4491G, and A4671G; GenBank AF067575) in the 39 untranslated region of the human STAT6 gene. Although all four of these polymorphisms have been shown to be associated with allergic phenotypes in various populations, their functional relevance remains unclear. Dinucleotide repeats are the most frequent of the simple repeats distributed throughout the human genome, and many of these exhibit length polymorphisms. Investigations into the effect of dinucleotide repeats on gene expression have shown enhanced or decreased transcriptional activity 12 in the context of different genes and cell types. These regulatory effects have been proposed to be due, in part, to the fact that dinucleotide repeats have potential to form alternative DNA structures, such as Z-, Hand cruciform DNA. The Z-DNA sequences in human genes tend to be located near transcription sites, which makes it possible that they play a role in the transcriptional control of genes. Because of their close proximity to the transcription start site, we first conducted a case2control association analysis of the GT repeat variants and atopic phenotypes in a white population, and then investigated their potential functional effects on the regulation of the activity of the STAT6 promoter. We report, for the first time, that the GT13 repeat variant in exon 1 of STAT6 is associated with asthma and total serum IgE level in a white study population, and that variation in the length of repeat variants differentially regulates the promoter activity in vitro.
Moderate sodium restriction with angiotensin converting enzyme inhibitor in essential hypertension: a double blind study.

Fifteen unselected patients who had essential hypertension and whose average supine blood pressure when they were not receiving any treatment and their usual sodium intake was 162/107 mm Hg were treated with captopril 50 mg twice daily. After one month's treatment their supine blood pressure had decreased to 149/94 mm Hg. They were then instructed to reduce their sodium intake to about 80 mmol(mEq)/day. After two weeks of moderate sodium restriction they were entered into a double blind randomised crossover study comparing the effect of 10 Slow Sodium tablets (100 mmol sodium chloride) with matching placebo tablets while continuing to take captopril and restrict sodium in their diet. After one month of taking placebo their mean supine blood pressure was 137/88 mm Hg with a urinary sodium excretion of 83 mmol/24 h, while after one month of taking Slow Sodium tablets their mean supine blood pressure was 150/97 mm Hg (p less than 0.001) with a sodium excretion of 183 mmol/24 h. The mean supine blood pressure during moderate sodium restriction therefore decreased by 9% and correlated significantly with the reduction in urinary sodium excretion. These results suggest that the combination of treatment with a moderate but practical reduction in sodium intake and an angiotensin converting enzyme inhibitor is effective in decreasing the blood pressure in patients with essential hypertension. This combined approach overcomes some of the objections that have been made to salt restriction alone and to converting enzyme inhibitors alone.