bluuebunny/crossref_metadata_2025_split huggingface dataset:
Institutional Experience with Spiral Intestinal Lengthening and Tailoring
Authors: Coletta Riccardo , Aldeiri Bashar , Morabito Antonino 
Published: June 2018

Aim The aim of this study was to report our initial experience using spiral intestinal lengthening and tailoring (SILT) technique in selected cases of short bowel syndrome (SBS).
Materials and Methods We analyzed all cases of SBS underwent SILT in our unit since the introduction of the procedure in 2012. We retrospectively analyzed patients' demographics, pre- and postprocedure bowel length, surgical complications, and postoperative parenteral nutrition (PN) requirements. Data were compared using independent samples, Mann–Whitney's U-test.
Results Five children with SBS underwent SILT between 2012 and 2017. Median age at procedure was 8.3 months (4.5–16). Preoperative small bowel length measured a median of 22 cm (17.5–50) with a median diameter of 4 cm (3.5–4.6). SILT allowed a median increase in length of 56% (10–15 cm; p = 0.03) and tailoring of the dilated segment providing a reduction in diameter of 50% (4.3–2.1 cm; p = 0.01). No major complications related to SILT were encountered and none of the children required further surgical intervention following a median follow-up of 26 months (14.5–41). Interestingly, we observed a significant reduction of PN requirement at 6 months (p = 0.008) associated with liver function preservation during the follow-up period.
Conclusion In our experience, SILT is a promising adjunct in the surgical management of SBS. It can be used to tailor and lengthen mildly dilated segments of the bowel where other procedures are technically challenging, with a view to reduce the risk of intestinal failure associated liver disease and thereby improving chances for quality survival. Further studies are needed to investigate long-term outcomes of SILT in pediatric SBS.
Quality of Life in Parents of Children Born with Esophageal Atresia
Authors: Witt Stefanie , Dellenmark-Blom Michaela , Dingemann Jens , Dingemann Carmen , Ure Benno M. , Gomez Barbara , Bullinger Monika , Quitmann Julia 
Published: June 2018

Introduction For parents of chronically ill children, the experiences of caregiving are challenged by increased demands and restrictions imposed by their child's disease. Therefore, this study aims to investigate the quality of life (QoL) in parents of children born with esophageal atresia (EA) and to explore associated factors.
Materials and Methods Parents of children (2–17 years) with EA recruited from two German pediatric hospitals participated in this cross-sectional study about QoL in EA. Data on QoL, sociodemographic, and clinical characteristics were collected from parents and children. Parental QoL was assessed using the Short-Form 8 questionnaire, containing eight dimensions aggregated to a mental and physical health summary score which was compared with German representative population norms.
Results Forty-nine families (47 mothers and 40 fathers) participated in the study. Compared with German population norms, both mothers and fathers showed significantly lower mental component score (MCS) but no differences in physical component score (PCS). Within the study sample, parents of younger children (2–7 years), severe EA, or high school/kindergarten absence had lower MCSs compared with those with older, less severe, and less absent children. Parental female gender was associated with lower MCS as well as lower family income.
Conclusion Parents of children with EA reported lower mental health compared with the general population, especially mothers, and parents of young children, with severe EA, and a frequent school/kindergarten absence. This shows that parents may experience substantial emotional burden and highlights the need for psychosocial support for EA parents, especially in the first years.
Review of Early Postoperative Metrics for Children Undergoing Resection of Congenital Pulmonary Airway Malformations and Report of Pleuropulmonary Blastoma at a Single Institution
Authors: Vandewalle Robert J. , Easton Joseph C. , Burns R. Cartland , Gray Brian W. , Rescorla Frederick J. 
Published: June 2018

Purpose The purpose of this study is to describe a single institution's 11-year experience treating children with congenital pulmonary airway malformations (CPAMs) and pleuropulmonary blastoma (PPB).
Materials and Methods An institutional database was sampled for all patients aged 0 to 18 years from January 1, 2005, to December 31, 2015. Patients with a pathologic diagnosis of CPAM or PPB during this period were reviewed.
Results A total of 51 patients with a pathologic diagnosis of CPAM (n = 45; 88.2%) or PPB (n = 6; 11.8%) underwent surgical resection. Among patients treated for PPB, one death occurred approximately 13 months after diagnosis. Although four patients with PPB (four out of six; 66.7%) had radiographic indicators highly suggestive of malignancy prior to surgery, two had a preoperative diagnosis of CPAM (two out of six; 33.3%). Twenty-four patients (24 out of 45; 53.3%) with CPAM underwent resection after developing symptoms and 21 (21 out of 45; 46.7%) were symptomatic at the time of surgery. Mann–Whitney's tests revealed a statistically significant difference in postoperative length of stay (median: 6 vs. 3 days; p < 0.001) and days with thoracostomy tube in place (median 3 vs. 2 days; p = 0.003) for symptomatic versus asymptomatic patients, respectively.
Conclusion CPAM patients appear to recover faster from surgery, if performed before the onset of symptoms. There may be a benefit to waiting until at least 3 months of age to complete resection in the asymptomatic patient. A low threshold for resection should be maintained in patients where delineating CPAM from PPB is difficult.
Biomechanical Comparison of the Temporalis Muscle Fascia, the Fascia Lata, and the Dura Mater
Authors: Pukšec Mirjana , Semenski Damir , Ježek Damir , Brnčić Mladen , Karlović Sven , Jakovčević Antonia , Bosanac Goran , Jurlina Martin 
Published: June 2018

AbstractThe purpose of our research is to prove that elastic biomechanical characteristics of the temporalis muscle fascia are comparable to those of the fascia lata, which makes the temporalis muscle fascia adequate material for dural reconstruction in the region of the anterior cranial fossa. Fifteen fresh human cadavers, with age range from 33 to 83 years (median age: 64 years; mean age: 64.28 years), were included in the biomechanical study. Biomechanical stretching test with the comparison of elasticity among the tissues of the temporalis muscle fascia, the fascia lata, and the dura was performed. The samples were stretched up to the value of 6% of the total sample length and subsequently were further stretched to the maximum value of force. The value of extension at its elastic limit for the each sample was extrapolated from the force–extension curve and was 6.3% of the total sample length for the fascia lata (stress value of 14.61 MPa), 7.4% for the dura (stress value of 6.91 MPa), and 8% for the temporalis muscle fascia (stress value of 2.09 MPa). The dura and temporalis muscle fascia shared the same biomechanical behavior pattern up to the value of their elastic limit, just opposite to that of the fascia lata, which proved to be the stiffest among the three investigated tissues. There was a statistically significant difference in the extension of the samples at the value of the elastic limit for the fascia lata in comparison to the temporalis muscle fascia and the dura (p = 0.002; Kruskal–Wallis test). Beyond the value of elastic limit, the temporalis muscle fascia proved to be by far the most elastic tissue in comparison to the fascia lata and the dura. The value of extension at its maximum value of force for the each sample was extrapolated from the force–extension curve and was 9.9% of the sample's total length for the dura (stress value of 10.02 MPa), 11.2% for the fascia lata (stress value of 23.03 MPa), and 18.5% (stress value of 3.88 MPa) for the temporalis muscle fascia. There was a statistically significant difference in stress values at the maximum value of force between the dura and the temporalis muscle fascia (p = 0.001; Mann–Whitney U test) and between the dura and the fascia lata (p < 0.001; Mann–Whitney U test). Because of its elasticity and similarity in its mechanical behavior to the dura, the temporalis muscle fascia can be considered the most suitable tissue for dural reconstruction.
Chinese rhetoric
Authors: Yeung Lorrita 
Published: June 2018

AbstractThis study investigates the question of Chinese indirection as a result of the use of modality expressions, which is conventionally believed to be the hallmark of Chinese rhetoric (e.g.Young, 1994;Bond, 1991;Powers and Gong, 1994). The present research compares and contrasts the degree of assertiveness as reflected in the patterns of modality in two corpora of expert Chinese and English argumentative writing on the same controversial subject. Corpus evidence shows that contrary to expectations, the Chinese writers are significantly more assertive than the English in arguing their case. The frequency of use and distribution patterns of intensifiers present both quantitative and qualitative evidence for the rhetorical differences, which may be accounted for culturally.
Revisiting Judicial Empowerment in the European Union
Authors: Pavone Tommaso 
Published: June 2018

AbstractJudicial empowerment is often cited as a driver of transnational governance, particularly in the European Union. In this view, lower national courts enthusiastically began referring cases to the European Court of Justice to acquire new powers of judicial review. Revisiting this argument, I argue that path dependent, everyday practices within domestic judiciaries stemming from insufficient training in European Union law, workload pressures, and cultural aversions to judicial review can resist Europeanization even when it would lead to empowerment. The argument is evaluated via a critical case study of judicial practice in Italy that is placed in a broader comparative context.
Evidence for a Posterior Parietal Cortex Contribution to Spatial but not Temporal Numerosity Perception
Authors: Cavdaroglu Seda , Knops André 
Published: June 2018

Abstract
Posterior parietal cortex (PPC) is thought to encode and represent the number of objects in a visual scene (i.e., numerosity). Whether this representation is shared for simultaneous and sequential stimuli (i.e., mode independency) is debated. We tested the existence of a common neural substrate for the encoding of these modes using fMRI. While both modes elicited overlapping BOLD response in occipital areas, only simultaneous numerosities significantly activated PPC. Unique activation for sequential numerosities was found in bilateral temporal areas. Multivoxel pattern analysis revealed numerosity selectivity in PPC only for simultaneous numerosities and revealed differential encoding of presentation modes. Voxel-wise numerosity tuning functions for simultaneous numerosities in occipital and parietal ROIs revealed increasing numerosity selectivity along an occipito-to-parietal gradient. Our results suggest that the parietal cortex is involved in the extraction of spatial but not temporal numerosity and question the idea of commonly used cortical circuits for a mode-independent numerosity representation.
Plasma fractalkine levels are associated with renal inflammation and outcomes in immunoglobulin A nephropathy
Authors: Luo Ran , Guo Shui-Ming , Li Yue-Qiang , Yang Yi , Li Meng-Lan , Han Min , He Xiao-Feng , Ge Shu-Wang , Xu Gang 
Published: June 2018

AbstractBackgroundA recognized noninvasive biomarker to improve risk stratification of immunoglobulin A nephropathy (IgAN) patients is scarce. Fractalkine has been shown to play a key role in glomerular disease as chemoattractant, adhesion and even fibrosis factor. The current study assessed the possibility of plasma fractalkine as a novel biomarker in IgAN patients.MethodsPlasma fractalkine was measured in 229 patients with renal biopsy consistent IgAN from 2012 to 2014, and clinical, pathological and prognostic relationships were analyzed.ResultsThe plasma fractalkine levels in IgAN patients were significantly correlated with the creatinine level and 24-h urine protein by both univariate and multivariate analysis. Mesangial hypercellularity was still significantly correlated with the plasma fractalkine levels even after adjustment for other potential predictor variables by multivariate analysis. In addition, the counts of CD20+ B cells or CD68+ macrophage in renal biopsies of IgAN patients were significantly correlated with the plasma fractalkine levels, but not CD4+ and CD8+ T cells. Finally, we concluded that patients with higher plasma fractalkine levels had higher risk of poor renal outcome compared with those with lower plasma fractalkine levels. No association was observed between the CX3CR1 polymorphisms and clinical parameters including plasma fractalkine levels and prognosis. Recombinant fractalkine induced mesangial cells extracellular matrix synthesis and promoted the migration of microphage cells RAW264.7.ConclusionsPlasma fractalkine levels were associated with creatinine level, 24-h urine protein, mesangial hypercellularity pathological damage, the CD68+ macrophage and CD20+ B cell infiltration in renal tissue and renal outcome in IgAN patients. Plasma fractalkine might be a potential prognosis novel predictor in Chinese patients with IgAN.
Clinical Image Coregistration Variability on a Dedicated Radiosurgery Unit
Authors: Ruschin Mark , Sahgal Arjun , Soliman Hany , Myrehaug Sten , Tseng Chia-Lin , Bola Ruby , Yeboah Collins , Sarfehnia Arman , Chugh Brige , Eriksson Markus , Nordström Håkan , Lee Young 
Published: June 2018

Abstract

BACKGROUND
On a new dedicated radiosurgery unit enabling frameless treatments, a cone-beam computed tomography (CBCT) can be used for stereotactic definition. Since magnetic resonance imaging (MRI) is used to delineate target, reproducible MRI-to-CBCT coregistration is vital for accurate target localization.

OBJECTIVE
To evaluate reproducibility of image coregistration in patient images.

METHODS
Three types of coregistration (source-to-target) were analyzed: (1) MRI-to-CT; (2) MRI-to-CBCT; and (3) CT-to-CBCT. For each patient (n = 15), each coregistration type was independently performed 5 to 30 times (total: 465 coregistrations). Each coregistration yielded a transformation matrix, which was subsequently applied to transform every point in the source image to stereotactic coordinates. Two metrics were measured: (1) target registration error (TRE): mean distance between the registered position of each target point and the average registration position of that point; (2) compound registration error (CRE): mean spatial difference between stereotactic coordinates using (A) MRI-to-CT-to-CBCT and (B) MRI-to-CBCT.

RESULTS
The median (range) of TRE was 0.11 mm (0.06-0.22 mm), 0.17 mm (0.10-0.36 mm), and 0.12 mm (0.08-0.21 mm) for MRI-to-CT, MRI-to-CBCT, and CT-to-CBCT, respectively. The TRE for MRI-to-CBCT was statistically higher than the other 2 methods (P < .01). The median (range) of CRE was 0.44 mm (0.22-0.59 mm). The maximum point CRE between patients ranged from 0.37-1.15 mm when considering all MRI points, but reduced to 0.31-0.90 mm within the central 16 cm. The CRE varied across the image volume, and typically was minimized near the center.

CONCLUSION
The variation in image coregistration is within 0.2 mm, indicating a high degree of reproducibility. The CRE varies throughout the head but is submillimeter in the central 16 cm region.
Cilia protein IFT88 regulates extracellular protease activity by optimizing LRP‐1–mediated endocytosis
Authors: Coveney Clarissa R. , Collins Isabella , Fie Megan Mc , Chanalaris Anastasios , Yamamoto Kazuhiro , Wann Angus K. T. 
Published: June 2018

ABSTRACTMatrix protease activity is fundamental to developmental tissue patterning and remains influential in adult homeostasis. In cartilage, the principal matrix proteoglycan is aggrecan, the protease‐mediated catabolism of which defines arthritis; however, the pathophysiologic mechanisms that drive aberrant aggrecanolytic activity remain unclear. Human ciliopathies exhibit altered matrix, which has been proposed to be the result of dysregulated hedgehog signaling that is tuned within the primary cilium. Here, we report that disruption of intraflagellar transport protein 88 (IFT88), a core ciliary trafficking protein, increases chondrocyte aggrecanase activity invitro.Wefind that the receptor for protease endocytosis in chondrocytes, LDL receptor‐related protein 1 (LRP‐1), is unevenly distributed over the cell membrane, often concentrated at the site of cilia assembly. Hypomorphic mutation of IFT88 disturbs this apparent hot spot for protease uptake, increases receptor shedding, and results in a reduced rate of protease clearance from the extracellular space. We propose that IFT88 and/or the cilium regulates the extracellular remodeling of matrix—independently of Hedgehog regulation—by enabling rapid LRP‐1–mediated endocytosis of proteases, potentially by supporting the creation of a ciliary pocket. This result highlights new roles for the cilium's machinery in matrix turnover and LRP‐1 function, with potential relevance in a range of diseases.—Coveney, C. R., Collins, I., Mc Fie, M., Chanalaris, A., Yamamoto, K., Wann, A. K. T. Cilia protein IFT88 regulates extracellular protease activity by optimizing LRP‐1–mediated endocytosis.FASEB J.32, 6771–6782 (2018).www.fasebj.org
Deletion of the stress‐response protein REDD1 promotes ceramide‐induced retinal cell death and JNK activation
Authors: Dai Weiwei , Miller William P. , Toro Allyson L. , Black Adam J. , Dierschke Sadie K. , Feehan Robert P. , Kimball Scot R. , Dennis Michael D. 
Published: June 2018

ABSTRACTThe role of dyslipidemia in the development of retinal dysfunction remains poorly understood. Using an animal model of diet‐induced obesity/pre‐type 2 diabetes, we investigated molecular defects in the retina arising from consumption of a diet high in saturated fats and sugars (i.e., a Western diet). We found that feeding mice a Western diet increased the abundance of retinal sphingolipids, attenuated protein kinase B (Akt) phosphorylation, enhanced JNK activation, and increased retinal cell death. When we used palmitate or C6‐ceramide (Cer) to assess sphingolipid‐mediated signaling in cultured murine and human cells, we observed similar effects on Akt, JNK, and cell death. Furthermore, both Western diet and C6‐Cer exposure enhanced expression of the stress‐response protein regulated in development and DNA damage response 1 (REDD1) and loss of REDD1 increased C6‐Cer‐induced JNK activation and cell death. Exogenous REDD1 expression repressed JNK‐mediated phosphorylation in cultured cells. We found that thioredoxin‐interacting protein (TXNIP) expression was elevated in REDD1–deficient cell lines and C6‐Cer promoted TXNIP expression in both wild‐type and REDD1–deficient cells. Likewise, TXNIP knockdown attenuated JNK activation and caspase 3 cleavage after either C6‐Cer exposure or REDD1 deletion. The results support a model wherein Cer‐induced REDD1 expression attenuates TXNIP‐dependent JNK activation and retinal cell death.—Dai, W., Miller, W. P., Toro, A. L., Black, A. J., Dierschke, S. K., Feehan, R. P., Kimball, S. R., Dennis, M. D. Deletion of the stress‐response protein REDD1 promotes ceramide‐induced retinal cell death and JNK activation. FASEB J. 32, 6883–6897 (2018). www.fasebj.org
Pigmented Villonodular Synovitis of the Knee: An Underappreciated Source of Pain in Children and Adolescents
Authors: Willimon Samuel Clifton , Busch Michael T. , Perkins Crystal A. 
Published: June 2018

Background:
Pigmented villonodular synovitis (PVNS) is a benign proliferative synovial disorder most commonly described in adults. The purpose of this study is to describe the presentation and management of a large single-center series of pediatric patients with PVNS of the knee.

Methods:
A retrospective review was performed of consecutive pediatric patients treated for PVNS at a single institution from 2001 to 2016. Inclusion criteria were patients below 20 years of age with surgical treatment of histologically proven PVNS of the knee. Review of the electronic medical record was utilized to gather demographic data, clinical presentation, affected joint, imaging findings, treatment, and disease persistence.

Results:
Over the 16-year study period, 17 pediatric patients with an average age of 11 years (range, 3 to 19 y) were treated for PVNS of the knee. The average duration of symptoms before orthopaedic evaluation was 16 months. More than half of the children were misdiagnosed with a variety of rheumatologic or orthopaedic conditions before their diagnosis of PVNS. On the basis of magnetic resonance imaging (MRI), PVNS was included as a potential diagnosis in 12 of 17 patients (71%). A total of 27 surgeries were performed in 17 patients. Ten patients were treated with a single surgery and 2 patients were treated with staged biopsy followed by synovectomy. Five patients required ≥2 therapeutic surgeries. The majority of procedures were arthroscopic synovectomies (89%). One patient required open resection of a mass in the posterior compartment of the knee and 1 patient had a manipulation under anesthesia for arthrofibrosis. Ten patients were noted to have nodular disease, 4 diffuse, and 3 mixed. At an average 15-month follow-up, 15 patients (88%) were considered to be disease-free based on clinical examination and/or follow-up MRI. Two patients (12%) had MRI studies following their last surgery with findings consistent with persistent PVNS, but only a single patient had persistent symptoms.

Conclusions:
This case series of pediatric patients with PVNS of the knee represents the largest single-center cohort in the literature. Although previously considered a disease of young adult patients, PVNS should be considered in pediatric patients with an insidious onset of joint swelling with or without pain. Symptoms may be present for months to years before diagnosis. Patients are frequently misdiagnosed, most commonly with rheumatologic diseases, bleeding disorders, or septic arthritis. MRI with gradient echo sequences is the diagnostic imaging study of choice and arthroscopic synovectomy produces good outcomes with low rates of symptomatic disease persistence.

Level of Evidence:
Level IV.