bluuebunny/crossref_metadata_2025_split huggingface dataset:
A systematic review of current studies in patients with familial hypercholesterolemia by use of national familial hypercholesterolemia registries
Authors: Mundal Liv , Retterstøl Kjetil 
Published: April 2016

Purpose of review
More than 25 million are expected to have familial hypercholesterolemia worldwide. The risk of cardiovascular disease (CVD) in familial hypercholesterolemia is not clear and register studies represent a valuable tool to get such data, which will be discussed in the present paper.

Recent finding
A systematic review of current familial hypercholesterolemia studies, by use of National familial hypercholesterolemia registries was performed from 1980 to 2016. This review shows that familial hypercholesterolemia patients have a high prevalence of CVD also in the time period after statins became available. The patient group does not reach recommended target values for lipids, and have a significantly higher CVD mortality as compared with the general population according to age and sex.

Summary
The review underscores the importance of establishing National familial hypercholesterolemia registries with complete datasets on familial hypercholesterolemia patients to improve early diagnostics, therapeutics and long-term follow-up to prevent the premature CVD events and deaths in this patient group.
Should apolipoprotein B replace LDL cholesterol as therapeutic targets are lowered?
Authors: Meeusen Jeffrey W. , Donato Leslie J. , Jaffe Allan S. 
Published: April 2016

Purpose of review
The success of LDL cholesterol (LDL-C) as a predictor of atherosclerotic cardiovascular disease and a therapeutic target is indisputable. Apolipoprotein B (apoB) is a more contemporary and physiologically relevant measure of atherogenic lipoproteins. This report summarizes recent comparisons of apoB and LDL-C as biomarkers of cardiovascular risk.

Recent findings
Multiple recent reports have found that LDL-C methods perform poorly at low concentrations (<70 mg/dl). Several meta-analyses from randomized controlled trials and large prospective observational studies have found that apoB and LDL-C provide equivalent information on risk of cardiovascular disease. More innovative analyses have asserted that apoB is a superior indicator of actual risk when apoB and LDL-C disagree.

Summary
ApoB is more analytically robust and standardized biomarker than LDL-C. Large population studies have found that apoB is at worst clinically equivalent to LDL-C and likely superior when disagreement exists. Realistically, many obstacles prevent the wide spread adoption of apoB and for now providers and their patients must weigh the costs and benefits of apoB.
Which PCDAI Version Best Reflects Intestinal Inflammation in Pediatric Crohn Disease?
Authors: Turner Dan , Levine Arie , Walters Thomas D. , Focht Gili , Otley Anthony , López Victor Navas , Koletzko Sibylle , Baldassano Robert , Mack David , Hyams Jeffrey , Griffiths Anne M. 
Published: April 2016

ABSTRACTIntroduction:There is increasing interest in measuring mucosal inflammation in Crohn disease (CD), but there are minimal data correlating the Pediatric Crohn's Disease Activity Index (PCDAI) versions (PCDAI, weighted Pediatric Crohn's Disease Activity Index [wPCDAI], abbreviated Pediatric Crohn's Disease Activity Index [abbrPCDAI], and the short Pediatric Crohn's Disease Activity Index [shPCDAI]) with mucosal inflammation.Methods:We aimed to compare the 4 PCDAI versions head to head with endoscopic degree of inflammation as measured by the Simple Endoscopic Score for Crohn's Disease (SES‐CD), fecal calprotectin, erythrocyte sedimentation rate (ESR), and C‐reactive protein (CRP) and to explore cut‐off values that are associated with mucosal healing. We used the prospectively collected data from the ImageKids study on 100 children with CD undergoing colonoscopy and from the Growth Relapse and Outcomes with Therapy study (n = 222), in which 145 children had calprotectin data at week 12 after diagnosis.Results:All 4 PCDAI versions had fair correlation with the SES‐CD (r = 0.42–0.45, all P < 0.001) and CRP (r = 0.32–0.45, all P < 0.01); the wPCDAI and PCDAI were superior to the shorter versions when comparing the blood tests. All versions had poor correlation with calprotectin, and only the wPCDAI reached significance (r = 0.26, P = 0.002 vs r = 0.15, P = 0.07 for PCDAI; r = 0.08, P = 0.37 for shPCDAI; r = 0.06, P = 0.5 for abbrPCDAI). The best cut‐off to identify endoscopic mucosal healing was <12.5 points for the wPCDAI (sensitivity 58% and specificity 84%) and <10 for PCDAI (sensitivity 63% and specificity 77%).Conclusions:The more feasible wPCDAI and the PCDAI had comparable correlation with measures of endoscopic inflammation. These were slightly superior to the other 2 shorter versions, but still none of the PCDAI versions can give a valid assessment of mucosal healing.
When Partial Hospitalization Fails
Authors: Beard Courtney , Hearon Bridget A. , Lee Josephine , Kopeski Lynne M. , Busch Alisa B. , Björgvinsson Thröstur 
Published: April 2016

Abstract
Partial hospitalization is an understudied bridge between outpatient and inpatient care. One of its primary functions is to prevent the need for inpatient hospitalization. We examined potential demographic and clinical risk factors for inpatient hospitalization for current partial hospital patients. We conducted separate multiple logistic regression analyses for patients referred from inpatient care and the community. For individuals referred from inpatient care, suicidal ideation and greater psychotic symptoms upon admission to the partial program were associated with acute inpatient re-hospitalization. For individuals referred from the community, suicidal ideation and worse relationship functioning upon partial hospital admission were significant risk factors for inpatient hospitalization. Number of previous inpatient hospitalizations and greater substance abuse were not associated with inpatient hospitalization in either sample. Implications at the provider and program level are discussed.
Value of Routine Eye Examinations in Asymptomatic Patients
Authors: Irving Elizabeth L. , Harris Joel D. , Machan Carolyn M. , Robinson Barbara E. , Hrynchak Patricia K. , Leat Susan J. , Lillakas Linda 
Published: April 2016

ABSTRACTPurposeTo determine if routine eye examinations in asymptomatic patients result in spectacle prescription change, new critical diagnosis, or new management of existing conditions. We also investigate whether age and time between assessments (assessment interval) impact detection rates.MethodsThe Waterloo Eye Study (WatES) database was created from a retrospective file review of 6397 patients seen at the University of Waterloo Optometry Clinic. Significant changes since the previous assessment (significant change) were defined as a change in spectacle prescription, presence of a new critical diagnosis, or a new management. Significant change, assessment interval, and age were extracted from the database for all asymptomatic patients presenting for a routine eye examination. The frequency of patients with significant change and the median assessment interval were determined for different age groups.ResultsOf 2656 asymptomatic patients, 1078 (41%) patients had spectacle prescription changes, 434 (16%) patients had new critical diagnoses, 809 (31%) patients had new managements, and 1535 (58%) patients had at least one of these (significant change). Median assessment intervals were 2.9 and 2.8 years for age groups 40 to <65 years and 20 to <40 years, respectively, approximately 1.5 years for patients 7 to <20, and between 1 and 1.5 years for patients <7 or >64. Controlling for assessment interval and sex, increasing age was associated with having a significant change (OR = 1.03, 95% CI 1.029–1.037). Similarly, controlling for age and sex, increased assessment interval was associated with having a significant change (OR = 1.06, 95% CI 1.02–1.11).ConclusionsIn asymptomatic patients, comprehensive routine optometric eye examinations detect a significant number of new eye conditions and/or result in management changes. The number detected increases with age and assessment interval.
Neuroimaging Rates for Closed Head Trauma in a Community Hospital
Authors: Rothman Steven M. , Alander Sarah W. 
Published: April 2016

Objectives
We aimed to characterize the utility of neuroimaging for head trauma in a suburban community hospital and determine whether imaging practices conform to most recent pediatric guidelines.

Methods
The electronic medical record was surveyed for computed tomographic and magnetic resonance imaging head scans on patients aged 1 to 18 years who were evaluated for trauma. The query included the following: date, sex, type of scan (computed tomography or magnetic resonance imaging), age, patient location, reason for scan, Glasgow Coma Scale (GCS) score (if entered), result, and text from physician's notes.

Results
A total of 2679 patients were identified. Within this cohort was a maximum of 29 surgical patients, of whom 8 required a surgical procedure but not neurosurgery among the 592 patients who had a GCS score of 14–15 entered, 2 were confirmed/possible neurosurgical patients, giving a neurosurgical rate of 0.34%. When the GCS 3–13 patient group was analyzed, the relative risk of requiring neurosurgery climbed to 52. Using an established algorithm for pediatric head trauma imaging would have reduced the number of scanned patients to 533. The individual cost of identifying the 29 surgical patients in our population exceeded $31,000.

Conclusions
Our rate of serious lesions in GCS 14–15 patients was identical to a larger prospective study in urban teaching hospitals. Using their previously described algorithm might have reduced the number of patients scanned by more than 70% and saved close to $750,000 for the study period.
An Unexpected Fish Bite
Authors: Berkowitz Tal , Goldsmith Michael Paul 
Published: April 2016

Abstract
We report a case of a 22-month-old boy who suffered a piranha bite, amputating his fourth distal phalanx. His finger was irrigated and closed with a flap under sedation, but we could find little evidence during a brief literature search of what prophylactic antibiotics, if any, to provide. Because reports of infections from piranha bites are lacking, we examined studies evaluating oral flora from fish, as well as flora cultured from aquariums. In conclusion, if infection is present or prophylaxis is strongly desired, a broad spectrum oral antibiotic that covers Gram-positive and Gram-negative bacteria including Pseudomonas and Aeromonas species, such as ciprofloxacin, would be recommended; however, there is little evidence to suggest that prophylactic antibiotics provide benefit over appropriate wound management.
Relationship Between Central Hypotonia and Motor Development in Infants Attending a High-Risk Neonatal Neurology Clinic
Authors: Segal Idan , Peylan Tali , Sucre Jennifer , Levi Loren , Bassan Haim 
Published: April 2016

Purpose:
To study the relationship between central hypotonia and motor development, and to determine the relative contribution of nuchal, truncal, and appendicular hypotonia domains to motor development.

Methods:
Appendicular, nuchal, and truncal tones of high-risk infants were assessed, as was their psychomotor developmental index (PDI). Infants with peripheral hypotonia were excluded.

Results:
We included 164 infants (mean age 9.6 ± 4 months), 36 with normal tone in all 3 domains and 128 with central hypotonia. Twenty-six of the latter had hypotonia in 1 domain and 102 had multiple combinations of 3 domains. Hypotonia domains were distributed as follows: truncal (n = 115), appendicular (n = 93), and nuchal (n = 70). Each domain was significantly associated with PDI scores (P < .001) but not with a later diagnosis of cerebral palsy. On linear regression, nuchal hypotonia had the strongest contribution to PDI scores (β = −0.6 [nuchal], −0.45 [appendicular], and −0.4 [truncal], P < .001).

Conclusions:
Central hypotonia, especially nuchal tone, is associated with lowered motor development scores.
Association of Polymorphisms in the Regulatory Region of the Cyclophilin a Gene (PPIA) with Gene Expression and HIV/AIDS Disease Progression
Authors: Madlala Paradise , Singh Ravesh , An Ping , Werner Lise , Mlisana Koleka , Abdool Karim Salim S. , Winkler Cheryl A. , Ndung'u Thumbi 
Published: April 2016

Background:
Human cyclophilin A (CypA) encoded by peptidyl prolyl isomerase A gene (PPIA), enhances HIV-1 replication by aiding capsid uncoating. The association of genetic variation in the PPIA regulatory region with susceptibility to HIV-1 infection, disease progression, and gene expression among black South Africans at risk for infection or infected with HIV-1 is unknown.

Methods:
We genotyped 539 participants from 2 longitudinal study cohorts of black South Africans at high risk for infection or infected with HIV-1 for PPIA regulatory single nucleotide polymorphisms by polymerase chain reaction–restriction fragment length polymorphism.

Results:
Minor allele (G) of SNP rs6850 (rs6850 G) significantly associated with higher viral loads (mean 4.85 versus 4.46 log copies/mL, P = 0.0006) and lower CD4+ T-cell counts (mean 506 versus 557 cells/μL, P = 0.0256) during the acute phase of infection in the Centre for the AIDS Programme of Research in South Africa (CAPRISA) 002 cohort. Consistently, rs6850 G significantly associated with higher viral loads (mean 4.49 versus 4.01 log copies/mL, P < 0.0001) and lower CD4+ T-cell counts (mean 442 versus 494 cells/μL, P = 0.0002) during the early chronic phase of infection in the CAPRISA 002 cohort; rs6850 G further associated significantly with rapid CD4+ T-cell decline in the CAPRISA 002 cohort (P = 0.0481) and Sinikithemba chronic infection cohort (P = 0.0156). Interestingly, rs6850 G significantly associated with elevated CypA mRNA levels in HIV-1–positive individuals (P = 0.0061).

Conclusions:
These data suggest that rs6850 G enhances HIV-1 replication through upregulation of CypA expression following HIV-1 infection. The data support ongoing efforts to develop anti–HIV-1 drugs that block interaction of HIV-1 and cellular proteins.
Thoracic Incidental Findings in Preoperative Computed Tomography Evaluation for Transcatheter Aortic Valve Implantation (TAVI)
Authors: Hussien Amira F. , Jeudy Jean , Kligerman Seth J. , White Charles S. 
Published: April 2016

Background:
Many older patients with aortic stenosis are excluded from surgical intervention due to multiple comorbidities. For these patients, transcatheter aortic valve implantation (TAVI) is a viable treatment alternative. Cardiothoracic computed tomography (CT) provides detailed anatomic assessment of the aortic root structures for patient selection, preprocedural planning, and intraoperative decision-making; however, nonaortic incidental findings are frequently detected. The prevalence and clinical significance of these findings have not been well described.

Materials and Methods:
This retrospective study examined incidental findings in 209 consecutive patients (96 women and 113 men; mean age, 70 y) with aortic stenosis undergoing preoperative cardiothoracic CT evaluation for TAVI during a 3-year period. Incidental findings were recorded, and medical records were reviewed for follow-up diagnostic examinations.

Results:
Incidental findings, both significant and nonsignificant, were observed in 85.6% of patients (179/209). Noncalcified pulmonary nodules were the most common incidental finding occurring in 59/209 (28.2%) patients, followed by pleural effusion and emphysema both of which occurred in 34/209 (16.3%) patients, respectively.

Conclusions:
Cardiothoracic CT obtained for evaluation before TAVI has a high frequency of incidental findings.
An Algorithm for the Management of Concomitant Maxillofacial, Laryngeal, and Cervical Spine Trauma
Authors: Tan Joshua Yuan Wang , Khoo Wan Xin , Hing Eileen Chor Hoong , Yap Yan Lin , Lee Hanjing , Nallathamby Vigneswaran , Thong Mark Kim Thye , Ong Wei Chen , Lim Jane , Lim Thiam Chye 
Published: April 2016

Abstract
Concomitant maxillofacial, laryngeal and cervical spine injuries may occur after high-energy accidents. Although this presentation is uncommon, the multiple injuries may compromise airway, breathing, circulation, and neurologic function. We identified 8 adult trauma patients admitted to the National University Hospital with the concomitant injuries. We reviewed the patient data and existing literature to identify the important factors that must be considered for management. Seven resulted from high velocity accidents, whereas 1 was assaulted. An algorithm that prioritizes in-tandem diagnosis and acute management of the adult trauma patient with maxillofacial, laryngeal, and cervical spine trauma was developed. The first priority is to assess airway, breathing, and circulation with cervical spine immobilization. Early diagnosis of patients with severe laryngeal injury, confirmation by video endoscopy, and establishing a surgical airway prevents airway obstruction or even a laryngotracheal dissociation. Urgent computed tomography scans of the head and neck are essential for definitive diagnosis and surgical planning for the 3 injuries. Prudent sequencing of surgery is important to avoid complications and to achieve better functional outcomes.
When the Mannequin Dies, Creation and Exploration of a Theoretical Framework Using a Mixed Methods Approach
Authors: Tripathy Shreepada , Miller Karen H. , Berkenbosch John W. , McKinley Tara F. , Boland Kimberly A. , Brown Seth A. , Calhoun Aaron W. 
Published: April 2016

Introduction
Controversy exists in the simulation community as to the emotional and educational ramifications of mannequin death due to learner action or inaction. No theoretical framework to guide future investigations of learner actions currently exists. The purpose of our study was to generate a model of the learner experience of mannequin death using a mixed methods approach.

Methods
The study consisted of an initial focus group phase composed of 11 learners who had previously experienced mannequin death due to action or inaction on the part of learners as defined by Leighton (Clin Simul Nurs. 2009;5(2):e59–e62). Transcripts were analyzed using grounded theory to generate a list of relevant themes that were further organized into a theoretical framework. With the use of this framework, a survey was generated and distributed to additional learners who had experienced mannequin death due to action or inaction. Results were analyzed using a mixed methods approach.

Results
Forty-one clinicians completed the survey. A correlation was found between the emotional experience of mannequin death and degree of presession anxiety (P < 0.001). Debriefing was found to significantly reduce negative emotion and enhance satisfaction. Sixty-nine percent of respondents indicated that mannequin death enhanced learning. These results were used to modify our framework.

Conclusions
Using the previous approach, we created a model of the effect of mannequin death on the educational and psychological state of learners. We offer the final model as a guide to future research regarding the learner experience of mannequin death.
Simulation for Milestone Assessment
Authors: Frey-Vogel Ariel S. , Scott-Vernaglia Shannon E. , Carter Lindsay P. , Huang Grace C. 
Published: April 2016

Introduction
Determining clinical competency on the milestones requires direct observation of residents, which is difficult for faculty members who are also providing patient care. Simulation can potentially represent an effective standardized tool for high-stakes assessment. Using a longitudinal simulation curriculum with formative and summative components, we conducted a pilot investigation to examine whether (1) performance on the formative cases predicted performance on the summative cases and (2) performance on the summative cases correlated with the clinical competency committee’s (CCC) milestone placement.

Methods
We developed 6 formative and 6 matched summative cases for pediatric interns that covered core pediatric topics. The interns progressed through the formative cases in pairs during the course of the academic year and then through the summative cases back to back individually at the end of the year. The interns were evaluated using a competency-based simulation evaluation. We determined the relationship between the formative and summative scores and between the summative scores and those from the CCC.

Results
The relationship between formative and summative scores was not statistically significant. There was a statistically significant relationship between summative and CCC scores.

Conclusions
Significant methodological limitations preclude definite conclusions about the predictive power of simulation cases for Pediatric Milestones-based assessment. However, our work is an example of how simulation is a potentially useful tool for assessing residents’ skill development on the Pediatric Milestones. More rigorous research is needed to determine the extent to which simulation can be used for high-stakes, milestones-based assessment.
A Minimally Invasive Strategy for Mirizzi Syndrome Type II: Combined Endoscopic With Laparoscopic Approach
Authors: Yuan Haicheng , Yuan Tian , Sun Xiangyu , Zheng Mingwei 
Published: April 2016

Background:
Mirizzi syndrome (MS) is a rare complication of the gallstone disease. Despite the fact that successful laparoscopic treatments have been reported for MS type I, open surgery remains the gold standard approach for MS type II because of the technical difficulties involved.

Objective:
The aim of this study is to determine the best technique for patients with MS type II by comparing the duration of surgery, loss of blood, rates of complication, duration of hospitalization, and outcomes of long-term follow-up.

Materials and Methods:
From January 2009 to September 2014, combined endoscopic retrograde cholangiopancreatography with laparoscopic surgery for MS type II was implemented. Patients’ demographics and treatment outcomes were collected prospectively and during the follow-up.

Results:
Forty-nine patients with MS type II were managed with this strategy. Laparoscopic subtotal cholecystectomy was successfully performed in all the patients without conversion or morbidity. When compared with a historical cohort of 57 patients who underwent a surgery for MS, this group of patients had significantly shorter duration of hospitalization (7.21±1.61 vs. 15.31±3.82 d, P<0.01). It also showed less blood loss (162.81±40.83 vs. 207.55±37.01 mL, P=0.425) and less postoperative complications (10.20% vs. 14.04%, P=0.594), although the duration of surgery (165.73±54.33 vs. 156.04±48.61 min, P=0.334) was longer, but these were not statistically significant. However, no significant difference in the rate of recurrence (4.55% vs. 9.52%, P=0.522) of choledocholithiasis was found.

Conclusions:
The MS type II can be effectively managed with a laparoscopic surgery combined with preoperative endoscopic retrograde cholangiopancreatography, which is feasible and minimally invasive.