Miracle-dz/UniFAR-FTUs huggingface dataset:
Dilated cardiomyopathy caused by LMNA mutations. Clinical and morphological studies.

BACKGROUND Dilated cardiomyopathy (DCM) is familial in about 20-35% of patients. The most frequently encountered mutations associated with DCM are found in LMNA. AIM To define the frequency of LMNA mutations in a series of consecutive DCM patients and to evaluate the phenotype of mutation carriers. METHODS We screened the 12 exons of LMNA in a series of 61 Polish patients with DCM diagnosed angiographically, as well as in two DCM families. RESULTS Two mutations were detected in 5 mutation carriers (D192G in one proband and Y481Stop in one proband and 3 of his offspring), which represents 3.3% (2/61) of the DCM patients. These mutations were absent from 100 controls. The D192G mutation was found in a 26-year-old patient with mild DCM and heart failure leading to death within two years after onset of symptoms. Mild conduction disease was also present. Ultrastructural analysis of the endomyocardial biopsy showed a striking alteration of nuclear morphology. This finding can explain nuclear fragility and is in agreement with the pathophysiological mechanical hypothesis of LMNA mutations. All four Y481Stop mutation-carriers were affected. Three phenotypes were found: in the proband, cardiac dysrhythmia and pacemaker requirement preceded DCM leading to heart transplantation; the proband's 13-year old daughter had conduction disease (2nd degree A-V block) with subtle skeletal muscle involvement documented by immunofluorescence study; ventricular arrhythmia was detected in the proband's son at the age of 11 and in the proband's daughter at the age of 18. Serum creatine kinase was normal in all mutation carriers.
Painful ascites

Background: In predicted severe acute pancreatitis, many patients develop organ failure and recover without local complications, and mortality is only 14–30%. It has been suggested that half of patients with progressive early organ failure may die, but there are no data to relate death or local complications to duration of early (week 1) organ failure. Aims: To determine mortality rates in patients with transient (<48 hours) and persistent (>48 hours) early organ failure and to show whether persistent organ failure predicts death or local complications. Patients: A total of 290 patients with predicted severe acute pancreatitis previously studied in a trial of lexipafant, recruited from 78 hospitals through 18 centres in the UK. Method: Manual review of trial database to determine: the presence of organ failure (Marshall score ⩾2) on each of the first seven days in hospital, duration of organ failure, and outcome of pancreatitis (death, complications by Atlanta criteria). Results: Early organ failure was present in 174 (60%) patients. After transient organ failure (n = 71), outcome was good: one death and 29% local complications. Persistent organ failure (n = 103) was followed by 36 deaths and 77% local complications, irrespective of onset of organ failure on admission or later during the first week. Conclusion: Duration of organ failure during the first week of predicted severe acute pancreatitis is strongly associated with the risk of death or local complications. Resolution of organ failure within 48 hours suggests a good prognosis; persistent organ failure is a marker for subsequent death or local complications.
Communication, and concurrency with logic-based restriction inside a calculus of structures

It is well known that we can use structural proof theory to refine, or generalize, existing paradigmatic computational primitives, or to discover new ones. Under such a point of view we keep developing a programme whose goal is establishing a correspondence between proof-search of a logical system and computations in a process algebra. We give a purely logical account of a process algebra operation which strictly includes the behavior of restriction on actions we find in Milner CCS. This is possible inside a logical system in the Calculus of Structures of Deep Inference endowed with a self-dual quantifier. Using proof-search of cut-free proofs of such a logical system we show how to solve reachability problems in a process algebra that subsumes a significant fragment of Milner CCS.
Evaluation of erectile dysfunction in permanent pacemaker implanted patients with cardiac rhythm disorder prediagnosis

Background and aims To evaluate the anxiety, depression and related psychogenic erectile dysfunction that might be developed before and after pacemaker implantation in patients with cardiac arrhythmias. Material and methods Thirty permanent pacemaker implanted male patients, were enrolled to study between September 2006 and September 2008. Erectile function domain questions of International Index of Erectile Function (IIEF‐6) and Hospital Anxiety and Depression Scale (HAD) questionnaires were applied to patients, 6 months before pacemaker implantation (BP6) and on month 1 (AP1) and 6 after application (AP6). Patients were included in a multidisciplinary cardiac rehabilitation-adaptation program with a duration of 1–2 months. Patients were evaluated in subgroups. Results Mean age was 51.5 ± 10.3. Most frequent diagnosis was observed as AV block in etiology. The mean IIEF values were changed 22.8→20.2→24.6 in BP6, AP1 and AP6 time frames consecutively. However, the mean HAD-Anxiety scores were evaluated as 8.1→17.0→7.3 and the mean HAD-Depression as 3.9→7.9→8.9 consecutively in the same time frames. Conclusion Cardiac arrhythmia plus permanent pacemaker implantation, increased anxiety and depression of patients and decreased erectile function at AP1; however, the improvement in cardiac symptoms at AP6 with the possible positive effects of rehabilitation program, helps to reduce anxiety and increased IIEF scores, although there was still a slight increase in depression levels.
Perceived Nursing Work Environment of Critical Care Nurses

Background:Different concepts and measures have been used to evaluate the work environment of nurses in hospital settings. There is increasing need for updated measurement tools that reflect the evolving nature of the work environment. Objectives:To report the psychometric properties of the Perceived Nursing Work Environment (PNWE) instrument, and to compare these results with those of other scales derived from the same background instrument: the Nursing Work Index—Revised. Methods:The Nursing Work Index—Revised was used in a national survey of critical care nurses. Exploratory principal component analysis with orthogonal rotation was conducted. Psychometric properties were examined. Construct validity was evaluated by comparing the scores for a subsample of nurses in magnet and nonmagnet hospitals. Results:Surveys obtained from 68 hospitals across the nation had a mean response rate of 41%, resulting in a sample of 2,324 participants. The PNWE, with its seven subscales and 42 items, explained 53% of the total variance of the Nursing Work Index—Revised. The subscales exhibited moderate to high reliabilities ranging from . 70 to . 91, except for one subscale (.56), and generally low intercorrelation. A similar version of the nurse–physician collaboration subscale was found in three of the other four scales compared. The positive scheduling climate subscale was a unique finding. Nurses employed at magnet hospitals had more positive perceived work environments than those employed at nonmagnet hospitals, and showed higher mean scores in four of the seven subscales (p < .05). Conclusions:The PNWE, a measure of the work environment as perceived by nurses, represents the latest version of the Nursing Work Index—Revised based on current practice. In this study, it exhibited sound psychometric properties. Further application and testing of the PNWE in various patient care settings is recommended.
The effect of dexmedetomidine added to preemptive (2% lignocaine with adrenaline) infiltration on intraoperative hemodynamics and postoperative pain after ambulatory maxillofacial surgeries under general anesthesia

Background: Lignocaine + adrenaline; a local anesthetic agent; frequently used for perilesional infiltration, maintains the stable hemodynamics and decreases the postoperative pain after maxillofacial surgery. α2agonists have peripheral analgesic effects. This prospective study was to evaluate the effectiveness of perilesional dexmedetomidine administered preincisionally in addition to conventional lignocaine adrenaline combinations for reconstructive maxillofacial surgery in an ambulatory care setting. Materials and Methods: 76, American Society of Anesthesiologists I-II patients scheduled for unilateral traumatic maxillofacial surgeries were randomly allocated into group DL (n = 38) receiving 15 cc of 2% lignocaine + adrenaline (1:200,000) mixed with 1 μg/kg dexmedetomidine and group PL receiving 15 cc of 2% lignocaine + adrenaline with normal saline (placebo) via local wound infiltration 5 min prior to skin incision. Perioperative hemodynamics, time to first analgesic use, total analgesic need, bleeding, and side effects were recorded for each patient. Results: Dosage of supplemental propofol; total perioperative, postoperative, and postanesthesia care unit (PACU) fentanyl consumption was significantly lower (P = 0.0001, P= 0.0001, P= 0.0001, P= 0.004, respectively) in dexmedetomine treated group than placebo. Rescue analgesic requirement was significantly earlier in group PL than group DL. Group DL patients suffered from significantly less (P = 0.02) bleeding and surgeon's satisfaction score was also high in this group. Discharge from PACU was significantly earlier in group DL. Intraoperative hemodynamic parameters were significantly lower in group DL (P < 0.05) without any appreciable side effects. Conclusion: Thus, prior dexmedetomidine local infiltration at the site of maxillofacial trauma has significantly reduced bleeding from wound site; perioperative fentanyl, propofol consumption, and subsequently ensured earlier discharge from PACU, better surgeon's satisfaction score with better hemodynamic control and lesser side effects.
Unequal Error Protection of Memories in LDPC Decoders

Memories are one of the most critical components of many systems: due to exposure to energetic particles, fabrication defects and aging they are subject to various kinds of permanent and transient errors. In this scenario, Unequal error protection (UEP) techniques have been proposed in the past to encode stored information, allowing to detect and possibly recover from errors during load operations, while offering different levels of protection to partitions of codewords according to their importance. Low-density parity-check (LDPC) codes are used in many communication standards to encode the transmitted information: at reception, LDPC decoders heavily rely on memories to store and correct the received information. To ensure efficient and reliable decoding of information, the need to protect the memories used in LDPC decoders is of primary importance. In this paper we present a study on how to efficiently design UEP techniques for LDPC decoder memories. The devised UEP method is divided in four adjustable levels, each one offering a different degree of protection. The full UEP, along with simplified versions, has been implemented within an existing decoder and its area occupation and power consumption evaluated. Comparison with the literature on the subject shows an unmatched level of protection from errors at a small complexity and energy cost.
Structure, function, and biology of SHIP proteins.

One of the “simple” curiosities of life, at least for a biological scientist, is how the growth and development of a complete organism from a single cell is controlled. Part of the answer includes growth factor receptors and their respective ligands, which transduce signals across cell membranes and into the nucleus for transcriptional readout. The molecular nature of these intracellular signals determines the type of signals, the pathways involved, subsequent regulatory interactions, and eventual transcription factor activation/repression. Hematopoietic cell development has been a fruitful system for the
Study on temperature measurement of two-electrode TIG arc plasma

Measurements of arc plasma are important for determining the associated physical properties. Such measurements usually involve the use of spectroscopic techniques to measure temperature distribution in free-burning arcs. Most studies have reported the temperature of axially symmetric arc plasma using Abel inversion. This method cannot be used for axially asymmetric arc plasma such as two-electrode TIG arc plasma. It is a complex phenomenon because the arc plasma generated from each electrode is affected by the other due to electromagnetic force. We measured the temperature distribution of tandem TIG arc plasma three dimensionally with a one camera rotation system. The measurement method includes the multi-directional detections and the image reconstruction technique: maximum likelihood–expectation maximization. We evaluated the influence of the number of detection directions on the reconstruction by virtual intensity distribution and found that the sufficient direction number is 6. As a result, we could measure the temperature distribution of the inclined, drawn and coupled arc plasma provided by a two-electrode TIG arc.
A historic issue of the Annals: three papers examine paracetamol in osteoarthritis

The CONSORT statement for the full reporting of clinical trials should be consistently applied Although current European League Against Rheumatism (EULAR)1 and American College of Rheumatology (ACR)2 guidelines both support paracetamol (acetaminophen) as the first line oral analgesic for patients with knee osteoarthritis (OA), until now there has been a paucity of clinical trial data to confirm the efficacy of paracetamol in large joint OA. This issue of the Annals is unique in containing one meta-analysis and two large placebo controlled studies that examine evidence for the efficacy of paracetamol in OA. Never in the 127 years of paracetamol’s existence have so much trial data on OA been reported. Because of the marked placebo effect in pain trials3 comparison with a placebo is clearly important to determine the true efficacy of any analgesic. Therefore it is surprising that despite the widespread use and support of paracetamol in OA until now there have been only four placebo controlled trials of this drug in OA. The first two studies reported in 19834 and in 19955 both demonstrated the superiority of paracetamol over placebo for pain relief in large joint OA using parallel group designs. However, the first study contained only 25 patients with knee OA followed up for just 6 weeks.4 The second study was larger (60 patients) but was of just 1 week’s duration and contained a mixed population with knee or hip OA.5 A third parallel group study was reported in 2003.6 This compared paracetamol, diclofenac, and placebo over a 12 week period in 82 patients with knee OA but was the first negative study for paracetamol, showing no difference between paracetamol and placebo using Western Ontario McMaster Osteoarthritis Index (WOMAC) assessments. Most recently, Pincus et al carried out a crossover study …
Biologically Different Subgroups of Invasive Ductal Carcinoma of the Pancreas

Purpose: Among invasive ductal carcinomas of the pancreas (IDCP), there is a morphologically characteristic subgroup accompanied by abundant intraductal carcinoma components (ICCs). With the aim of determining whether ICC-rich IDCP are biologically different from ICC-poor IDCP, the expression status of Dpc4 protein was analyzed. Experimental Design: A total of 43 IDCP was subdivided into two groups: (a) ICC-rich IDCP (ICCs area occupies ≥10% of the entire tumorous area); and (b) ICC-poor IDCP (with <10% of ICCs area). A total of 10 invasive carcinomas derived from intraductal papillary-mucinous neoplasms (ICs from IPMNs) were also analyzed. Each invasive and intraductal carcinoma area was then evaluated for Dpc4 protein status by immunohistochemistry. Results: In a total of 43 IDCP, there were 23 ICC-rich IDCP and 20 ICC-poor IDCP. Dpc4-positive immunostaining was observed in the invasive carcinoma component of ICC-rich IDCP, ICC-poor IDCP, and ICs from IPMN in 18 of 23 (78%), 4 of 20 (20%), and 7 of 10 (70%) cases, respectively. In the intraductal component, positive staining for Dpc4 was found in 20 of 23 (87%), 3 of 7 (41%), and 8 of 10 (80%) cases, respectively. Dpc4 expression was found in both the invasive and ICC components of ICC-rich IDCP, similar to that found in IC derived from IPMN, whereas the expression of Dpc4 was largely diminished in ICC-poor IDCP. Conclusions: Morphologically distinct subgroups of invasive ductal carcinomas of the pancreas, namely ICC-rich IDCP and ICC-poor IDCP, are also biologically distinguishable as revealed by the differential expression of Dpc4.
Bestrophin 1 gene analysis and associated clinical findings in a Chinese patient with Best vitelliform macular dystrophy

The aim of the present study was to investigate the clinical characteristics and the underlying genetic causes of Best vitelliform macular dystrophy (BVMD) in a sporadic case in a Chinese patient. A 10-year-old boy was diagnosed with BVMD; complete ophthalmic examinations were performed, including best-corrected visual acuity, intraocular pressure, slit-lamp examination, fundus photograph, optical coherence tomography and fundus fluorescein angiography imaging. Genomic DNA was extracted from leukocytes of the peripheral blood collected from this patient and his family members. DNA samples from 200 unrelated subjects from the Chinese population were used as controls. A total of 11 exons of the bestrophin 1 (BEST1) gene were amplified by polymerase chain reaction and directly sequenced. The results revealed that the patient presented with yellowish lesions in the macular area. Heterozygous mutations c.292G>A (p.Glu98Lys) in exon 4 and c.1608C>T (p.Thr536Thr) in exon 10 of the BEST1 gene were identified in this sporadic case; however, this was not identified in any of his unaffected family members or in the normal controls. The c.292G>A (p.Glu98Lys) mutation has not been previously reported, whereas the c.1608C>T (p.Thr536Thr) mutation is a previously characterized single nucleotide polymorphism (SNP). In conclusion, BEST1 gene mutations and polymorphisms have been reported in diverse ethnic groups, and the present study identified a novel BEST1 gene mutation and an SNP that occurred simultaneously in a Chinese patient with BVMD.
Laminin isoforms in human embryonic stem cells: synthesis, receptor usage and growth support

To reveal the functional intrinsic niche of human embryonic stem cells (hESC) we examined the production of basement membrane (BM) proteins and the presence of their receptors in feeder‐free cell culture conditions. In addition, we investigated binding of hESCs to purified human BM proteins and identified the receptors mediating these contacts. Also, we tested whether purified human laminin (Lm) isoforms have a role in hESC self‐renewal and growth in short‐term cultures. The results show that hESCs synthesize Lm α1 and Lm α5 chains together with Lm β1 and γ1 chains suggesting the production of Lms‐111 and ‐511 into the culture medium and deposits on cells. hESCs contain functionally important integrin (Int) subunits, Int β1, α3, α6, α5, β5 and αV, as well as the Lm α5 receptor, Lutheran (Lu) glycoprotein and its truncated form, basal cell adhesion molecule (B‐CAM). In cell adhesion experiments, Int β1 was crucial for adhesion to most of the purified human BM proteins. Lu/B‐CAM mediated adhesion to Lm‐511 together with Int α3β1, and was essential for the adhesion of hESCs to embryonic feeder cells. Adhesion to Lm‐411 was mediated by Int α6β1. Lm‐511 supported hESC growth in defined medium equally well as Matrigel. These results provide consequential information of the biological role of BM in hESCs, warranting further investigation of BM biology of human pluripotent stem cells.
Pregnancy Following Bariatric Surgery

Gastric bypass surgery for morbid obesity is considered an appropriate intervention when other weight-loss measures have proven unsuccessful. Weight loss often brings about improvement in overall health by lessening the effects of obesity-related comorbidities such as chronic hypertension and diabetes. In fact, the ability to become pregnant is enhanced, as weight loss often allows for a normalization of sex hormones. However, the nutrition challenges brought about by the surgery may have a profound impact on maternal health and pregnancy outcome. Surgical procedures for morbid obesity may be classified according to the digestive aftereffects brought about by the particular procedure. These categories include the “restrictive” procedures, “restrictive-malabsorptive” procedures, and the less common “malabsorptive” procedures. Deficiencies in iron, vitamin B12, folate, and calcium can result in maternal complications, such as severe anemia, and in fetal complications, such as neural tube defect, intrauterine growth restriction, and failure to thrive. Nutrient supplementation following bariatric surgery and close supervision before, during, and after pregnancy can help prevent nutrition-related complications and improve maternal and fetal health.
Helicobacter pylori and precancerous conditions of the stomach: the frequency of infection in a cross-sectional study of 79 consecutive patients with chronic antral gastritis in Yaoundé, Cameroon

Introduction The study aimed at determining the different types of precancerous conditions of the stomach and searches the frequency of Helicobacter pylori in these lesions in patients with chronic antral gastritis in Yaounde, Cameroon. Methods Five gastric biopsies were performed during upper gastrointestinal endoscopy for pathology and fixed in formol 10% before being coated in paraffin. Both the modified Giemsa and Periodic acid of Shift – Alkaline blue stains were used for the histological diagnosis of Helicobacter pylori infection. Hematoxylyn and eosin stain was used to determine the activity of gastritis, atrophic gastritis and intestinal metaplasia in accordance to the Sydney's classification of gastritis. Data were analysed using both the Epi info 6.04 and Excel 2007 softwares. Means and their standard deviations, medians and their interquartiles (IQR) were calculated. Proportions were established for qualitative variables and chi square analysis done in this study with a p value set at 0.05. Results Seventy-nine patients with chronic antral gastritis were enrolled, of which 43 (54.4%) were male, median age: 43 years (range from 21 to 70 years). The rate of atrophic gastritis was 74.7% (59/79). The activity of atrophic gastritis was mild in 47.5% (28/59) of cases, moderate in 47.5% (28/59) and severe in 5% (5/59). Intestinal metaplasia and follicular gastritis were present in 6.3% (5/79), and 10.1% (8/79), respectively. Concerning Helicobacter pylori infection, 71.2% (42/59) of patients with atrophic gastritis tested positive against 28.8% (17/59) who tested negative (p = 0.00003). Helicobacter pylori infection was related to the severity of gastric atrophy (p = 0.0001). Among patients with intestinal metaplasia and follicular gastritis, the proportion of those who tested positive for Helicobacter pylori infection was 80% (4/5), and 75% (6/8), respectively. There were no significant differences in the occurrence of atrophic gastritis according to age groups (p = 0.908). Conclusion This study concludes that atrophic gastritis, which is most often caused by Helicobacter pylori, is the most frequent precancerous condition of stomach in Cameroon. Routine gastric sampling for pathologic analysis is mandatory for effective diagnosis and surveillance of Helicobacter pylori infection and precancerous conditions of the stomach.