Miracle-dz/UniFAR-FTUs huggingface dataset:
The six‐minute walk test is a valuable measure of functional change following chemotherapy for AL (light‐chain) cardiac amyloidosis

Light chain amyloidosis (AL) is characterized by tissue deposition of mis-folded protein fibrils, which leads to organ dysfunction (Merlini & Bellotti, 2003). Cardiac involvement is common and represents the most common cause of death in AL (Comenzo et al, 2012; Kastritis & Dimopoulos, 2016). The 6-minute walk test (6MWT) is a well-established outcome measure in a variety of diseases including heart failure, idiopathic pulmonary hypertension and sickle cell-associated pulmonary hypertension (Cazeau et al, 2001; Abraham, 2002; American Thoracic Society [ATS] 2002, Anthi et al, 2007). The objective of this study was to determine the impact of chemotherapy on changes in the 6MWT in patients with AL cardiac amyloidosis. Data were retrospectively collected from 22 consecutive AL amyloid patients with cardiac involvement. Cardiac AL was confirmed in all cases. Subjects performed the 6MWT in a standardized manner according to the ATS guidelines, at diagnosis and at the end of planned chemotherapy (ATS 2002). All patients were untreated at time of diagnosis. Patients who were clinically unable to walk at baseline were scored as zero metres (m) walked. Patients with multiple myeloma were excluded. All patients received bortezomib-based chemotherapy and 12 patients (55%) received autologous haematopoietic cell transplantation after induction therapy at a median of 9 months (range, 4–16 months) post-treatment initiation. Modified cardiac biomarker staging was defined by elevated brain natriuretic peptide (BNP; >100 pg/ml) and troponin I (>0 1 ng/ml). Stage I was defined as no elevation, stage II was defined by elevation in either BNP or troponin I and stage III was defined by elevation in both markers (Sanchorawala et al, 2015). To determine cardiac response, BNP was converted to NT-proBNP by a conversion factor of 1:3 5. Cardiac response was defined as a decrease of >30% and 85 pg/ml in BNP (with minimum baseline 185 pg/ml) or a decrease ≥2 in New York Heart Association (NYHA) class in subjects designated as NYHA class 3 or 4 at baseline. Cardiac progression was defined as either a >30% and 85 pg/ml increase in BNP, ≥33% increase in troponin I, or ≥10% decrease in ejection fraction (EF) (Palladini et al, 2012; Girnius et al, 2014). Haematological responses (HR) were defined according to consensus guidelines (Comenzo et al, 2012). Table I. Patient baseline characteristics.
Iron‐chelating therapy with deferasirox in transfusion‐dependent, higher risk myelodysplastic syndromes: a retrospective, multicentre study

Iron chelation is controversial in higher risk myelodysplastic syndromes (HR‐MDS), outside the allogeneic transplant setting. We conducted a retrospective, multicentre study in 51 patients with transfusion‐dependent, intermediate‐to‐very high risk MDS, according to the revised international prognostic scoring system, treated with the oral iron chelating agent deferasirox (DFX). Thirty‐six patients (71%) received azacitidine concomitantly. DFX was given at a median dose of 1000 mg/day (range 375–2500 mg) for a median of 11 months (range 0·4–75). Eight patients (16%) showed grade 2–3 toxicities (renal or gastrointestinal), 4 of whom (8%) required drug interruption. Median ferritin levels decreased from 1709 μg/l at baseline to 1100 μg/l after 12 months of treatment (P = 0·02). Seventeen patients showed abnormal transaminase levels at baseline, which improved or normalized under DFX treatment in eight cases. One patient showed a remarkable haematological improvement. At a median follow up of 35·3 months, median overall survival was 37·5 months. The results of this first survey of DFX in HR‐MDS are comparable, in terms of safety and efficacy, with those observed in lower‐risk MDS. Though larger, prospective studies are required to demonstrate real clinical benefits, our data suggest that DFX is feasible and might be considered in a selected cohort of HR‐MDS patients.
Excess early postnatal weight gain leads to thicker and stiffer arteries in young children.

BACKGROUND Although early life growth pattern is associated with cardiovascular disease later in life, it is unknown whether vascular changes associated with excess early weight gain already occur in early childhood. METHODS In the Wheezing-Illnesses-Study-Leidsche-Rijn (WHISTLER) birth cohort, weight and height from birth to 3 months of age were used to calculate Z-scores of individual weight and length gain rates. In the first 333 children who turned 5 years old, intima-media thickness, distensibility, and elastic modulus of the carotid artery were measured ultrasonographically. The association between weight gain rate for length gain rate (WLG), as a measure of excess weight gain, and vascular characteristics was assessed by generalized linear modeling. Interaction between birth size and WLG was tested. RESULTS Per 1 SD increase in WLG, carotid intima-media thickness was 5.1 μm (95% confidence interval, 1.0-9.2; P value = .01) higher (adjusted for age, gender, current height, and observer). The thinner the children were at birth, the stiffer the arteries were with increasing WLG (interaction between birth size and WLG-distensibility: P = .04; elastic modulus: P = .03). CONCLUSION Excess early postnatal weight gain leads to vascular changes already in early childhood, characterized by thicker arterial walls. In children who are relatively thin at birth, excess early postnatal weight gain also leads to stiffer arteries. This supports the view that cardiovascular disease risk is associated with growth pattern early in life.
Childhood Stunting and Mortality Between 36 and 64 Years: The British 1946 Birth Cohort Study

Background: Our aim was to examine the associations between childhood or adult height and adult mortality. Methods: In the prospective British 1946 Birth Cohort Study, childhood height was measured at 2, 4, 6, 7, 11, and 15 years, and adult height was measured at 36 years. Deaths were reported from the national health service register. Results: A total of 3877 study members (1963 male) contributed 106,333 person-years of follow-up; 391 deaths (228 male) were reported between the ages of 36 and 64 years. The strongest sex-adjusted association between height and mortality between ages 36 and 64 years was seen for height at age 6 years. The association was nonlinear; only study members in the shortest quintile at 6 years had a higher relative risk of adult mortality compared with those in the tallest quintile. By contemporary growth standards, 5.7% (n = 188) had heights at 6 years less than the second percentile, and a further 15.0% (n = 490) had heights between the second to ninth percentiles; these groups had higher adult mortality than all other study members (hazard ratio, 2.18; 95% confidence interval, 1.52–3.13; P < .001; and hazard ratio, 1.42; 95% confidence interval, 1.08–1.88; P = .01, respectively). Several determinants of childhood stunting (height at 6 years less than the second percentile) were directly associated with adult mortality; these included shorter parental heights and adverse early life nutrition and housing. Conclusions: British men and women born in 1946 were relatively stunted as children by contemporary standards. Those who were short at age 6 years had substantially higher mortality 30 to 60 years later. Furthermore, they accounted for the well-recognized inverse association between adult height and mortality.
Learning and teaching in clinical practice Patient safety and technology-driven medication e A qualitative study on how graduate nursing students navigate through complex medication administration

Background: The technology-driven medication process is complex, involving advanced technologies, patient participation and increased safety measures. Medication administration errors are frequently reported, with nurses implicated in 26e38% of in-hospital cases. This points to the need for new ways of educating nursing students in today's medication administration. Aim: To explore nursing students' experiences and competences with the technology-driven medication administration process. Methods: 16 pre-graduate nursing students were included in two focus group interviews which were recorded, transcribed and analyzed using the systematic horizontal phenomenological-hermeneutic template methodology. Results: The interviews uncovered that understanding the technologies; professionalism and patient safety are three crucial elements in the medication process. The students expressed positivity and confidence in using technology, but were fearful of committing serious medication errors. From the nursing students' perspective, experienced nurses deviate from existing guidelines, leaving them feeling isolated in practical learning situations. Conclusion: Having an unclear nursing role model for the technology-driven medication process, nursing students face difficulties in identifying and adopting best practices. The impact of using technology on the frequency, type and severity of medication errors; the technologies implications on nursing professionalism and the nurses ability to secure patient adherence to the medication process, still remains to be studied. © 2014 Elsevier Ltd. All rights reserved.
Weight loss induced by Roux-en-Y gastric bypass but not laparoscopic adjustable gastric banding increases circulating bile acids.

CONTEXT It has been hypothesized that increased plasma bile acids (BAs) contribute to metabolic improvements after Roux-en-Y gastric bypass (RYGB) surgery by the G protein-coupled receptor TGR5-mediated effects on glucagon-like peptide-1 secretion and thyroid hormones. OBJECTIVE The objective of this study was to evaluate the importance of bariatric surgery-induced alterations in BA physiology on factors that regulate glucose homeostasis (insulin secretion and sensitivity) and energy metabolism (resting energy expenditure and thyroid hormone axis). DESIGN, PARTICIPANTS, INTERVENTION, AND MAIN OUTCOME MEASURE: Eighteen extremely obese subjects were studied before and after 20% weight loss, induced by either laparoscopic adjustable gastric banding (LAGB) (n = 10) or RYGB surgery (n = 8). RESULTS Plasma BAs more than doubled after RYGB [fasting: 1.08 (0.26-1.42) to 2.28 (1.59-3.28) μmol/L, P = . 03; postprandial: 2.46 ± 1.59 to 6.00 ± 2.75 μmol/L, P = . 01] but were either lower or did not change after LAGB [fasting: 1.80 (1.49-2.19) to 0.92 (0.73-1.15) μmol/L, P = . 02; postprandial: 3.71 ± 2.61 to 2.82 ± 1.75 μmol/L, P = . 14]. Skeletal muscle expression of TGR5 targets, Kir6.2 and cyclooxygenase IV, increased after RYGB but not LAGB. Surgery-induced changes in BAs were associated with increased peak postprandial plasma glucagon-like peptide-1 (r(2) = 0.509, P = . 001) and decreased serum TSH (r(2) = 0.562, P < . 001) but did not correlate with the change in insulin response to a meal (r(2) = 0.013, P = . 658), insulin sensitivity (assessed as insulin stimulated glucose disposal during a hyperinsulinemic-euglycemic clamp procedure) (r(2) = 0.001, P = . 995), or resting energy expenditure (r(2) = 0.004, P = . 807). CONCLUSIONS Compared with LAGB, RYGB increases circulating BAs and TGR5 signaling, but this increase in BAs is not a significant predictor of changes in glucose homeostasis or energy metabolism.
Guidelines for the use of imaging in the management of patients with myeloma

The role of imaging in myeloma has gained increasing importance over the past few years. The recently revised definition of myeloma from the International Myeloma Working Group (IMWG) includes cross sectional imaging as a method to define bone disease and also incorporates its use in the disease definition for patients with suspected smouldering myeloma. The National Institute for Health and Care Excellence myeloma guidelines also recommend cross sectional imaging for patients with suspected myeloma. There is also increasing use of imaging in disease assessments and the International Myeloma Working Group has recently incorporated imaging in defining new response categories of minimal residual disease negativity, with or without imaging‐based evidence of disease. Plain X‐rays have previously been the standard imaging modality included in a myeloma work up at presentation but evidence is mounting for use of cross‐sectional modalities such as computed tomography (CT), magnetic resonance imaging (MRI) and 18fluoro‐deoxyglucose (18F‐FDG) positron emission tomography (PET)/CT. Funding and therefore availability of newer imaging techniques remains a barrier. Here, we propose an evidence‐based approach to the use and technical application of the latest imaging modalities at diagnosis and in the follow‐up of patients with myeloma and plasmacytoma.
Assessment of OPG/RANK/RANKL gene expression levels in peripheral blood mononuclear cells (PBMC) after treatment with strontium ranelate and ibandronate in patients with postmenopausal osteoporosis.

CONTEXT Recent research results have confirmed the high significance of the OPG/RANK/RANKL system in the development of bone diseases. AIM The aim of the reported study was to assess gene expression levels of the OPG/RANK/RANKL system in peripheral blood mononuclear cells (PBMCs) after strontium ranelate (SR) and ibandronate administered to patients with postmenopausal osteoporosis. PATIENTS AND METHODS A total of 89 postmenopausal women, aged 51 to 85 years, patients of the Outpatient Clinic of Osteoporosis of the Military Teaching Hospital in Lodz, were enrolled into the study. The patients were randomly assigned to different medical therapies: ibandronate and SR. Patients of the control group received only calcium and vitamin D₃ supplements. Patient visits were repeated after 3 and 6 months. Measurements of serum alkaline phosphatase concentrations and of RNA expression in PBMCs as well as of total serum calcium and phosphate levels and of their 24-hour urine excretion rates were carried out in material, collected at baseline and after 3 and 6 months of the therapy. Densitometry of the left hip and of the lumbar spine was done at the baseline visit and after 6 months. RESULTS The differences in gene expressions of RANKL and RANK were not significant during the study period and did not differ between the groups in a statistically significant manner. No OPG gene expression was observed in PBMCs of patients in any of the studied groups and at any time point. The tendency of correlation (P = .07) was observed between decreasing RANK gene expression and increasing bone mineral density in the patients treated with SR. CONCLUSIONS Both ibandronate and SR do not seem to cause any significant changes in gene expression levels of OPG/RANK/RANKL in PBMCs during the first 6 months of treatment.
Raising the thermoelectric performance of p-type PbS with endotaxial nanostructuring and valence-band offset engineering using CdS and ZnS.

We have investigated in detail the effect of CdS and ZnS as second phases on the thermoelectric properties of p-type PbS. We report a ZT of ~1.3 at 923 K for 2.5 at. % Na-doped p-type PbS with endotaxially nanostructured 3.0 at. % CdS. We attribute the high ZT to the combination of broad-based phonon scattering on multiple length scales to reduce (lattice) thermal conductivity and favorable charge transport through coherent interfaces between the PbS matrix and metal sulfide nanophase precipitates, which maintains the requisite high carrier conductivity and the associated power factor. Similar to large ionically bonded metal sulfides (ZnS, CaS, and SrS), the covalently bonded CdS can also effectively reduce the lattice thermal conductivity in p-type PbS. The presence of ubiquitous nanostructuring was confirmed by transmission electron microscopy. Valence and conduction band energy levels of the NaCl-type metal sulfides, MS (M = Pb, Cd, Zn, Ca, and Sr) were calculated from density functional theory to gain insight into the band alignment between PbS and the second phases in these materials. The hole transport is controlled by band offset minimization through the alignment of valence bands between the host PbS and the embedded second phases, MS (M = Cd, Zn, Ca, and Sr). The smallest valence band offset of about 0.13 eV at 0 K was found between PbS and CdS which is diminished further by thermal band broadening at elevated temperature. This allows carrier transport between the endotaxially aligned components (i.e., matrix and nanostructure), thus minimizing significant deterioration of the hole mobility and power factor. We conclude the thermoelectric performance of the PbS system and, by extension, other systems can be enhanced by means of a closely coupled phonon-blocking/electron-transmitting approach through embedding endotaxially nanostructured second phases.
Atypical femoral fractures shortly after osteonecrosis of the jaw in a postmenopausal woman taking alendronate for osteoporosis.

CONTEXT Bisphosphonates effectively increase bone mineral density and reduce fracture risk in patients with osteoporosis, but there are concerns about osteonecrosis of the jaw (ONJ) and atypical femoral fractures (AFFs) in the long-term users. So far both complications have not been reported as occurring simultaneously in an osteoporotic individual on oral alendronate. OBJECTIVE The aim of this study was to report a postmenopausal woman presenting with concomitant ONJ and AFF on oral alendronate treatment. SUBJECT, MEASURES, AND RESULT: The patient was a 63-year-old woman with a history of rheumatoid arthritis for 30 years and diabetes for 3 years. Spinal compression fractures at levels L3 and L4 were documented, and she took alendronate 70 mg weekly for 7 years. She is the first case whose dental periapical imaging and pelvic radiography documented her ONJ and AFF, which developed subsequently within 6 months. CONCLUSIONS This case report supports the association of both ONJ and AFF with long-term oral bisphosphonate therapy.
Das Konzept „Optische Qualität“– ein neues Paradigma in der Augenheilkunde

Zusammenfassung Bei der Bewertung von refraktiv-chirurgischen Verfahren erwies sich der Hochkontrastvisus als alleiniger Parameter haufig als unzureichend, da er den fur die Patientenzufriedenheit so wichtigen subjektiven Seheindruck oft nur unzureichend widerspiegeln vermochte. So ergab sich mit dem Konzept der „Optischen Qualitat“ ein neues Paradigma in der Augenheilkunde. Ein bei der Evaluation der optischen Qualitat auftretendes Problem ist die Tatsache, dass sich „optische Qualitat“ nicht direkt messen lasst. Es ist daher eine Definition uber messbare Surrogatparameter (Operationalisierung) notwendig. Die optische Qualitat wird von Faktoren auf mehreren Ebenen beeinflusst: Die Ebene „Anatomie“ stellt die Eingangsebene dar. Sie beeinflusst direkt die optischen Eigenschaften des Auges, die sich wiederum auf die Funktion des Auges auswirken. Auf der letzten Ebene steht der subjektive Seheindruck des Patienten. Jeder der einzelnen Ebenen wird durch messbare Parameter reprasentiert (Anatomie: z. B. Hornhauttopografie, Hornhautreflexion; Optik: z. B. Wellenfrontfehler; Funktion: z. B. Visus, Kontrastund Blendempfindlichkeit; Wahrnehmung: z. B. Fragebogenwerte fur Symptomintensitat und Zufriedenheit). Wir stellen nachfolgend die Zusammenhange zwischen den einzelnen Ebenen und ihre Bedeutung fur die refraktiv-chirurgische Praxis dar.
Occupational Balance: A Scoping Review of Current Research and Identified Knowledge Gaps

This article reports a comprehensive review of the research conducted regarding occupational balance. A scoping study method was used to explore and describe current research about occupational balance and to identify research gaps. Twenty-two articles published between 2009 and 2014 met the inclusion criteria. The articles reported studies conducted in eight countries on four continents, but the majority were conducted in Europe and North America. The articles contributed to knowledge about the concept itself, its importance, levels of occupational balance, and the relationship between occupational balance and health and well-being. They also described what is important for occupational balance or how to enhance/create/recreate it and differences between people. Several research gaps were identified which include the need for studies about perceptions of occupational balance among people beyond western societies. Furthermore, systematic research is warranted with regards to levels of occupational balance, and how to enhance it.
Low urinary levels of angiotensin‐converting enzyme 2 may contribute to albuminuria in children with sickle cell anaemia

Clinical trials have shown that angiotensin-converting enzyme inhibitors (ACEi) effectively reduce albuminuria in patients with sickle cell anaemia (SCA) (Haymann et al, 2017). However, the utilization of ACEi in SCA was drawn from experience with diabetic nephropathy. No studies have evaluated renin-angiotensin system (RAS) components in patients with SCA, and whether RAS activity plays a role in albuminuria. Animal models of sickle cell disease (SCD) have demonstrated changes in the levels of RAS components (dos Santos et al, 2014; Roy et al, 2018). However, there is currently no data available regarding RAS molecules in humans with SCD, which is crucial to understand the role of RAS components in sickle cell nephropathy, and to identify therapeutic targets. This study sought to evaluate urinary levels of RAS components and the association of these molecules with albuminuria in children with SCA. Detailed methods are provided in Appendix S1. A crosssectional study of 72 children selected from the Minas Gerais state SCA cohort were assigned to two groups: (i) 36 children with SCA who had persistent albuminuria (SCA-PA); (ii) 36 children with SCA with normal albuminuria (SCANA) on hydroxycarbamide therapy, sex and age-matched with PA group. The study protocol was approved by our Ethics Committee. Medical records were reviewed to obtain demographic, clinical, laboratory and management data. Random spot urine specimens were collected during routine visits. Albumin/creatinine ratio and total urinary protein
Effects of nonsteroidal anti-inflammatory drugs on serum proinflammatory cytokines in the treatment of ankylosing spondylitis.

OBJECTIVE This study was conducted to investigate the correlation between serum levels of proinflammatory cytokines and the clinical efficacy of nonsteroidal anti-inflammatory drugs (NSAIDs) in patients with ankylosing spondylitis (AS). METHODS A total of 148 patients with AS were selected and received NSAID treatment. ELISA was used to assess cytokine levels, and patients were assigned into the following groups: positively effective; effective; moderately effective; and ineffective. Spearman and Pearson correlation analyses were used for correlation analysis. RESULTS The erythrocyte sedimentation rates (ESR), C-reactive protein (CRP) levels, and immunoglobulin A (IgA) levels of the case group after NSAID treatment were markedly lower than those before NSAID treatment. After treatment, the levels of interleukin (IL)-6, IL-17, and tumor necrosis factor (TNF)-α were markedly reduced, while IL-10 levels increased in the positively effective, effective, and moderately effective groups, and IL-12 levels decreased in the positively effective and effective groups. In addition, the levels of IL-6 and TNF-α were correlated with a greater number in the efficacy indexes and clinical parameters, followed by IL-10 levels, while the levels of IL-17 and IL-12 had relatively weaker correlations with these indexes and parameters. CONCLUSION NSAIDs could promote the clinical efficacy of treatment for ankylosing spondylitis by regulating serum levels of proinflammatory cytokines.