bluuebunny/crossref_metadata_2025_split huggingface dataset:
Magnetic Resonance Imaging in Symptomatic Children With Hereditary Multiple Exostoses of the Hip
Authors: Duque Orozco Maria d.P. , Abousamra Oussama , Rogers Kenneth J. , Thacker Mihir M. 
Published: May 2016

Background:
Magnetic resonance imaging (MRI) is useful in evaluating nontraumatic hip pain. It provides information about associated injuries like labral/chondral tears or ischiofemoral impingement (IFI). However, in hereditary multiple exostoses (HME) there has been no report about MRI findings in symptomatic children with hip involvement.

Methods:
Records of children with HME and hip osteochondromas, who had hip MRI/magnetic resonance arthrography, were reviewed. The presence of chondral lesions and labral tears, as well the presence of IFI, was recorded. IFI was defined as edema or fatty replacement/atrophy in the quadratus femoris muscle or decrease of the space for this muscle between the ischium and the proximal femur. The measurements used to determine the space included the ischiofemoral space, the quadratus femoris space, and the minimum ischiofemoral space (MIFS). All measurements were performed on axial T1-weighted images.

Results:
Ten children were included (4 males, 6 females). In 2 patients, MRI was unilateral, therefore a total of 18 hips were analyzed. The indication for MRI was hip pain. Mean age, when MRI was performed, was 11.7 years. Labral tears were found in 44% (8/18) and chondral lesions in 33% (6/18) of the hips. The mean ischiofemoral space was 17.2 mm (SD, 7.3), the mean quadratus femoris space was 14.9 mm (SD, 5.3), and the mean MIFS was of 12.8 mm (SD, 5.9). IFI was seen in 44% (8/18) of hips. Two patients had bilateral IFI. MIFS was <10 mm in all hips with IFI (8/8). Of these hips, 88% (7/8) had edema of the quadratus femoris muscle and 38% (3/8) had fatty replacement/atrophy in the muscle. Osteochondromas were seen in the lesser trochanter in all hips with IFI (8/8) and in the ischium in 50% of them (4/8).

Conclusions:
In symptomatic children with HME of the hip, MRI is helpful in detecting the source of pain. A high percentage of these children have IFI and intra-articular lesions. These findings can play an important role in the indication and planning of the surgical approach.

Level of Evidence:
Level IV—diagnostic study.
Flexible Intramedullary Nails for Femur Fractures in Pediatric Patients Heavier Than 100 Pounds
Authors: Shaha James , Cage Jason M. , Black Sheena , Wimberly Robert L. , Shaha Steven H. , Riccio Anthony I. 
Published: May 2016

Background:
Flexible intramedullary nailing (FIMN) of femoral shaft fractures in children >100 pounds remains controversial. The purpose of this study is to assess the relationship between patient weight and alignment at radiographic union following Ender’s FIMN of pediatric femoral shaft fractures.

Methods:
An IRB approved, retrospective review of all patients who sustained a femoral shaft fracture treated by retrograde, stainless-steel Ender’s FIMN was performed at a level 1 pediatric trauma center from 2005 to 2012. Preoperative radiographs were analyzed to determine fracture pattern, location, and isthmic canal diameter. Patient weight was measured on presentation to the emergency room. Radiographs at bony union were reviewed to measure shortening, coronal angulation, and sagittal angulation.

Results:
A total of 261 children underwent Ender’s FIMN for femoral shaft fractures during the study period. There were 24 patients who weighed ≥100 lbs and 237 patients who weighed <100 lbs. There were no significant differences in sex (75% vs. 73% male), fracture stability (42.6% vs. 41.7% length unstable), or fracture patterns between the 2 groups. The ≥100 lbs group was significantly older (10.6 vs. 8.0 y, P<0.001). There were no significant differences in final coronal angulation (1.5 vs. 3.0 degrees), sagittal angulation (2.8 vs. 3.1 degrees), or shortening (3.4 vs. 3.5 mm) between the 2 groups. There were significantly more nail removals in the <100 lbs group (81.4% vs. 66.7%, P<0.01). Four percent of the population (10 patients) weighed ≥120 lbs and aside from age (11.4 vs. 8.1 y, P<0.01), there were no significant demographic or fracture pattern differences between this group and the remaining population. This heaviest group demonstrated no significant difference in shortening (3.3 vs. 3.5 mm), coronal angulation (0.8 vs. 3.0 degrees), or sagittal angulation (0.7 vs. 3.2 degrees) at radiographic union when compared with the lighter patients.

Conclusions:
Stainless-steel Ender’s FIMN is an effective treatment for pediatric femoral shaft fractures in patients ≥100 pounds with excellent radiographic outcomes and no increased risk for malunion.

Level of Evidence:
Therapeutic Level III.
Three-dimensional EOS Analysis of Apical Vertebral Rotation in Adolescent Idiopathic Scoliosis
Authors: Kato So , Debaud Charlotte , Zeller Reinhard D. 
Published: May 2016

Background:
Apical vertebral rotation (AVR) is increasingly recognized as one of the important radiographic parameters in adolescent idiopathic scoliosis (AIS). EOS enables us to precisely measure AVR by 3-dimensional reconstruction. The objective of the present study was to describe the postoperative correction and the long-term follow-up of AVR in posterior spinal fusion with direct vertebral rotation and elucidate the factors that affected the correction.

Methods:
We retrospectively reviewed 153 consecutive posterior spinal fusion surgeries for AIS performed between 2009 and 2012. Among them, 55 patients who fulfilled the study inclusion criteria with complete preoperative, immediate postoperative, and last follow-up (>1 y) EOS images were included in the present study. EOS 3-dimentional reconstructions were undertaken for each patient. Postoperative AVR correction and the loss of correction were calculated.

Results:
Preoperative AVR of the major curve averaged 19 degrees (SD=7 degrees), and AVR on immediate postoperative images averaged 9 degrees (SD=6 degrees, P<0.001). AVR at final follow-up averaged 11 degrees (SD=6 degrees, P=0.06). Postoperative correction was larger in all-screw construct than in hybrid construct (55% vs. 36%, P=0.03).

Conclusions:
The present study is the first study to measure AVR in a large population of AIS patients using EOS 3-dimensional reconstruction. We report the correction magnitude was significantly affected by the construct.

Level of Evidence:
Level IV—therapeutic study (case series).
Pseudarthrosis Repair Using Autologous Cultured Osteoblasts in Complex Type-1 Neurofibromatosis Spinal Deformity
Authors: Kim Young-Hoon , Reoyan Genriech N. , Ha Kee-Yong , Kim Chul-Kyu 
Published: May 2016

Study Design.
A case report and literature review.

Objective.
To report a late dislocation of the vertebra caused by progressive dural ectasia combined with type-1 neurofibromatosis (NF-1) and the clinical results of pseudarthrosis repairs using autologous cultured osteoblasts.

Summary of Background Data.
NF-1 is a well-known genetic disorder that is commonly characterized by spinal deformities including kyphoscoliosis. Late dislocation of the vertebra resulting from progressive dural ectasia after surgical correction of NF-1 scoliosis is a very rare occurrence, and pseudarthrosis frequently develops after surgical intervention for this complex spinal deformity.

Methods.
A 32-year-old female patient with NF-1 scoliosis underwent surgical correction with posterior instrumented fusion. Seventeen years later, dislocation of the lumbar spine with implant failure resulting from massive progressive dural ectasia was observed. She underwent anterior interbody fusion three times and posterior instrumented fusion four times for pseudarthrosis followed by surgical deformity correction. For the last operation, autologous cultured osteoblasts were used as a therapeutic approach to repair the pseudarthrosis, and a three-dimensional printing technique was used to understand the surgical anatomy of the dislocated lumbar spine in detail.

Results.
After the final operation, bone union was achieved and confirmed by clinical and radiological examination.

Conclusion.
Spine surgeons should be knowledgeable about the possibility of late destabilization of the spine, due to pulsatile dural ectasia, and a high rate of pseudarthrosis in neurofibromatosis. Autologous cultured osteoblasts may prove to be a modality that can be applied pseudarthrosis repair to treat complex spinal deformity.

Level of Evidence: 5
Checkpoint inhibitors and gastrointestinal immune-related adverse events
Authors: Pernot Simon , Ramtohul Toulsie , Taieb Julien 
Published: May 2016

Purpose of review
Recent development of checkpoint inhibitors is a challenge for oncologists. Indeed, it leads to specific immune adverse events, close to autoimmune disorders, which require a specific management. Colitis is one of the most frequent immune adverse events, in particular with anticytotoxic T-lymphocyte-associated antigen 4 (CTLA-4) therapy.

Recent findings
Severe colitis is frequent with immune checkpoint inhibitors and leads in a few cases to bowel perforation and death. This review focuses on specific pathogenic pathway and recent findings on risk factors and managements of colitis.

Summary
Anti-CTLA-4 antibodies are the most involved immune checkpoint inhibitors in colitis, and the combinations with anti-programmed death ligand 1 dramatically increase the rate of colitis. The early use of budesonide, and in some cases corticosteroids and/or infliximab should be recommended, as colitis is responsive to infliximab in almost all cases. Immune-related colitis shares some characteristics with inflammatory bowel disease but with little specificity. In particular, it has been recently showed that gut microbiota could interact with anti-CTLA-4 treatment to modulate efficacy but also to induce colitis. This opens the way for preventive or curative treatments capable of inducing modulation of the microbiota or fecal transplantation.
High prevalence of frequent attendance in the over 65s
Authors: McMahon C. Geraldine , Power Foley Megan , Robinson David , O’Donnell Kate , Poulton Miriam , Kenny Rose A. , Bennett Kathleen 
Published: May 2016

Introduction
Characteristics of older frequent users of Emergency Departments (EDs) are poorly understood. Our aim was to examine the characteristics of the ED frequent attenders (FAs) by age (under 65 and over 65 years).

Methods
We examined the prevalence of FA attending the ED of an Urban Teaching Hospital in a cross-sectional study between 2009 and 2011. FA was defined as an individual who presented to the ED four or more times over a 12-month period. Randomly selected groups of FA and non-FA from two age groups (under 65 and over 65 years) were then examined to compare the characteristics between older FAs and non-FAs and older FAs and younger FAs. Logistic regression was used to calculate the odds ratio and 95% confidence intervals for 12-month mortality in FA compared with non-FA aged at least 65 years.

Results
Overall, 137 150 ED attendances were recorded between 2009 and 2011. A total of 21.6% were aged at least 65 years, 4.4% of whom were FAs, accounting for 18.4% of attendances by patients older than 65 years. There was a bimodal age distribution of FA (mean±SD; under 65 years 40±12.7; and over 65 years 76.9±7.4). Older FAs were five times more likely to present outside normal working hours and 5.5 times more likely to require admission. Cardiovascular emergencies were the most common complaint, in contrast with the younger FA group, where injury and psychosocial conditions dominated. The odds ratio for death at 12 months was 2.07 (95% confidence interval 0.93–4.63; P=0.07), adjusting for age and sex.

Conclusion
One-in-five ED patients older than 65 years of age are FAs. Older FAs largely present with complex medical conditions. Enhanced access to expert gerontology assessment should be considered as part of effective intervention strategies for older ED users.
Menstrual management and reproductive concerns in adolescent and young adult women with underlying hematologic or oncologic disease
Authors: Quinn Sheila M. , Louis-Jacques Jennifer 
Published: May 2016

Purpose of review
Heavy menstrual bleeding is common among adolescent and young adult women, and can affect health-related quality of life. The cause of heavy menstrual bleeding is not uncommonly because of an underlying hematologic or oncologic disease process, which substantially influences the way patients are counseled and treated.

Recent findings
Options for menstrual management are more numerous today than ever before and range from minimizing monthly blood loss to suppressing the cycle altogether. However, an underlying bleeding disorder or malignancy can introduce many nuances and limits in individual patient care, which this review highlights. Additionally, because survival rates for adolescent and young adult cancers are improving, more of these patients are planning for lives after their disease, which may include starting or adding to a family. Options for fertility preservation during cancer therapy regimens are solidifying and both primary practitioners and subspecialists should be aware of the possibilities.

Summary
Patients with underlying hematologic or oncologic disease require management of menstrual bleeding, but also deserve a comprehensive evaluation and counseling regarding their individualized contraceptive needs and fertility preservation options during their reproductive years. This review employs the latest evidence from current literature to help guide clinicians caring for this unique demographic.
Nongenomic regulation of gene expression
Authors: Iglesias-Platas Isabel , Monk David 
Published: May 2016

Purpose of review
The purpose of this review is to highlight the recent advances in epigenetic regulation and chromatin biology for a better understanding of gene regulation related to human disease.

Recent findings
Alterations to chromatin influence genomic function, including gene transcription. At its most simple level, this involves DNA methylation and posttranscriptional histone modifications. However, recent developments in biochemical and molecular techniques have revealed that transcriptional regulation is far more complex, involving combinations of histone modifications and discriminating transcription factor binding, and long-range chromatin loops with enhancers, to generate a multifaceted code. Here, we describe the most recent advances, culminating in the example of genomic imprinting, the parent-of-origin monoallelic expression that utilizes the majority of these mechanisms to attain one active and one repressed allele.

Summary
It is becoming increasingly evident that epigenetic mechanisms work in unison to maintain tight control of gene expression and genome function. With the wealth of knowledge gained from recent molecular studies, future goals should focus on the application of this information in deciphering their role in developmental diseases.
Comparison of Primary Care Provider Office Hours and Pediatric Emergency Department Return Visits
Authors: Rogers Brent D. , Attia Magdy W. 
Published: May 2016

Objective
The aim of this study was to evaluate the influence of primary care office hours of operation on 48-hour return visits (RVs) to a pediatric emergency department (ED). We compared characteristics of patients who return with those who follow up outpatient to determine the feasibility of opening off-hour clinics to decrease the RV rate.

Methods
The study was a retrospective chart review of patients presenting to a pediatric ED for a 3-year period. A subset of patients with a hospital-affiliated primary care provider was evaluated to compare those with 48-hour ED RVs with those with office follow-up.

Results
Patients with a hospital-affiliated primary care provider had 30,231 visits, of whom 842 had a 48-hour return (2.79%). A significant number (48.5%) of those who returned had seen their primary care doctor between emergency visits. The percentage of RVs occurring at night (55.7%) was slightly lower than the percentage of all visits occurring off hours (58.1%). Patients with more acute presentation at initial visit (emergency severity index level acuity 2, >20 orders placed) were more likely to follow up with their provider than return to the ED.

Conclusions
The findings from this study show no significant increase in RVs during the evening and overnight hours and many patients with outpatient follow-up before returning to the ED. Opening a clinic at our hospital during nontraditional hours would not likely significantly decrease RV rate.
Auricular Acupuncture for the Treatment of Pediatric Migraines in the Emergency Department
Authors: Graff Danielle M. , McDonald Mark J. 
Published: May 2016

Objectives
Headache remains a frequent complaint for children presenting to a pediatric emergency department (ED). Typical treatments include oral or intravenous medications, but do not always relieve pain. An alternative intervention is auricular acupuncture. It has been shown to be effective in the treatment of migraines in adults. The objective of this study was to evaluate the utility and adverse effects of auricular acupuncture in the treatment of pediatric migraines in the ED.

Methods
This was a prospective, interventional, cohort study of patients 8 to 18 years of age. Efficacious ear points were located by needle contact or electrical point finder with attention to 2 migraine lines on the ear. ASP gold semipermanent ear needles were placed in the efficacious points and patients were monitored for 15 minutes. The primary outcome was the change in preintervention and postintervention pain scores using a numerical self-reported pain visual analog scale (VAS).

Results
Nineteen patients elected to enroll in the study. The mean change in the VAS scores was both clinically and statistically significant at 7.03 (interquartile range, 6–8.5) with a P value of less than 0.001. Two patients elected to withdraw from the study secondary to incomplete resolution of migraine pain despite improvement in VAS scores. There were no known adverse events.

Conclusions
With all subjects showing improvement or resolution of migraine headache, this pilot study introduces an alternative intervention to pediatric migraine management. Further studies are needed to evaluate the duration of symptom resolution and comparative effectiveness; auricular acupuncture seems to be a valid alternative.
Correction of Severely Contracted Nose
Authors: Oh Young Hwan , Seo Je Won , Oh Se Joon , Oum Ick Tae , Kim Gukjin , Kim Jun , Kim Sa Yong , Shin Seung Woo , Park DaEun , Kim Kenneth K. 
Published: May 2016

Background:
Severely contracted nose is manifested with a tight and hardened nasal envelope. Expansion of the contracted skin is an important first step in correcting these revision cases. The underlying weak lower lateral cartilage makes the tip projection structurally difficult to achieve and maintain without rigid supporting cartilage grafting.

Methods:
A total 59 of patients were treated with isolated adipose-derived stromal cells before revision surgery to soften the nasal envelope. Adipose tissues were digested at 37°C with sterile 0.075% collagenase type 2. The average isolated adipose-derived stromal cell count of each serial injection was 5 × 106 cells (total injection volume, 0.5 ml; 1 × 107 cells/ml). Intraoperatively, the lower lateral cartilage was released from surrounding scar tissue to allow for advancement. Rib cartilage and other autologous grafts were used in reconstruction of the internal framework.

Results:
The follow-up period ranged from January of 2009 to April of 2014. The mean follow-up period was 10 months. Fifty-one of 59 patients were satisfied with their results. Eight patients underwent revision surgery for the following: infection (two patients), deviation (one patient), warping (two patients), and cosmetic dissatisfaction (three patients). There were two cases of additional warping, but the patients refused revision surgery. Nine patients required additional adipose-derived stromal cell injections at the tip.

Conclusions:
The combination of isolated fat grafting to soften the nasal skin envelope and rigid tip support results in correction of silicone-induced contracted nose. There were no incidences of recurrent nasal contraction or ischemic injury.
CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.
Outcomes of Living Donor Liver Transplantation Alone for Patients on Maintenance Renal Replacement Therapy in Japan
Authors: Eguchi Susumu , Furukawa Hiroyuki , Uemoto Shinji , Umeshita Koji , Imamura Hajime , Soyama Akihiko , Shimamura Tsuyoshi , Isaji Shuji , Ogura Yasuhiro , Egawa Hiroto , Kawachi Shigeyuki , Kasahara Mureo , Nagano Hiroaki , Ku Yonson , Ohdan Hideki , Maehara Yoshihiko , Sato Shuntaro , Inomata Yukihiro 
Published: May 2016

Background
Because simultaneous liver and kidney transplantation has been limited as a standard practice because of a severe shortage of deceased donors in Japan, living donor (LD) liver transplantation alone (LTA) is indicated in most recipients with maintenance renal replacement therapy (MRRT).

Methods
A retrospective nationwide survey of LD LTA was performed for liver transplant patients on MRRT. The characteristics of donors and recipients, postoperative complications, survival rate, and causes of death were analyzed.

Results
In the adult cases (n = 28), the overall survival rate at 1 year and 5 years were 66.1% and 57.3%, respectively. When compared with those adults without MRRT (n = 237), it was significantly worse. In the 7 pediatric cases, the overall survival rate at 1 and 5 years were both 83.3%. Three adult recipients died of nonaneurysm cerebral hemorrhage after 1 year and 1 adult recipient died of acute heart failure after 7 months. In adult recipients with MRRT, graft weight versus standard liver volume, and duration and blood loss in LTA surgery were associated with poor outcomes after LD LTA. Multivariate analysis revealed that MRRT was highest hazard ratio on patient survival after LD LTA.

Conclusions
Early post-LD LTA mortality was higher in patients with MRRT than in those without MRRT with characteristic causes. Smaller grafts for size and a complicated surgery were associated with poor outcome after LD LTA. Thus, LD LTA in adult patients on MRRT should be carefully treated with meticulous postoperative management and follow-up.
SignaFish: a zebrafish-specific signaling pathway resource
Authors: Csályi Kitti , Fazekas Dávid , Kadlecsik Tamás , Türei Dénes , Gul Leila , Horváth Balázs , Módos Dezső , Demeter Amanda , Pápai Nóra , Lenti Katalin , Csermely Péter , Vellai Tibor , Korcsmáros Tamás , Varga Máté 
Published: May 2016

AbstractUnderstanding living systems requires an in depth knowledge of the signaling networks that drive cellular homeostasis, regulate intercellular communication and contribute to cell fates during development. Several resources exist to provide high-throughput datasets or manually curated interaction information from human or invertebrate model organisms. We previously developed SignaLink, a uniformly curated, multi-layered signaling resource containing information for human and for the model organisms nematodeCaenorhabditis elegansand fruit flyDrosophila melanogaster. Until now, the use of the SignaLink database for zebrafish pathway analysis was limited. To overcome this limitation we created SignaFish (http://signafish.org), a fish-specific signaling resource, built using the concept of SignaLink. SignaFish contains more than 200 curation based signaling interactions, 132 further interactions listed in other resources, and it also lists potential miRNA based regulatory connections for 7 major signaling pathways. From the SignaFish website, users can reach other web resources, such as ZFIN. SignaFish provides signaling or signaling-related interactions that can be examined for each gene, or downloaded for each signaling pathway. We believe that the SignaFish resource will serve as a novel navigating point for experimental design and evaluation for the zebrafish community and for researchers focusing on non-model fish species, such as cyclids.