bluuebunny/crossref_metadata_2025_split huggingface dataset:
Neurosurgical experience of managing optic pathway gliomas
Authors: Hill Ciaran Scott , Khan Mehdi , Phipps Kim , Green Katherine , Hargrave Darren , Aquilina Kristian 
Published: February 2021

AbstractBackgroundOptic pathway gliomas (OPGs), also known as visual pathway gliomas, are debilitating tumors that account for 3–5% of all pediatric brain tumors. They are most commonly WHO grade 1 pilocytic astrocytomas and frequently occur in patients with neurofibromatosis type 1. The location of these tumors results in visual loss and blindness, endocrine and hypothalamic dysfunction, hydrocephalus, and premature death. Their involvement of the visual pathways and proximity to other eloquent brain structures typically precludes complete resection or optimal radiation dosing without incurring significant neurological injury. There are various surgical interventions that can be performed in relation to these lesions including biopsy, cerebrospinal fluid diversion, and partial or radical resection, but their role is a source of debate. This study catalogues our surgical experience and patient outcomes in order to support decision-making in this challenging pathology.MethodsA retrospective review of all cases of OPGs treated in a single center from July 1990 to July 2020. Data was collected on patient demographics, radiographic findings, pathology, and management including surgical interventions. Outcome data included survival, visual function, endocrine, and hypothalamic dysfunction.ResultsOne hundred twenty-one patients with OPG were identified, and 50 of these patients underwent a total of 104 surgical procedures. These included biopsy (31), subtotal or gross total resection (20 operations in 17 patients), cyst drainage (17), Ommaya reservoir insertion (9), or cerebrospinal fluid diversion (27). During the study period, there was 6% overall mortality, 18% hypothalamic dysfunction, 20% endocrine dysfunction, and 42% had some cognitive dysfunction. At diagnosis 75% of patients had good or moderate visual function in at least one eye, and overall, this improved to 83% at the end of the study period. In comparison the worst eye had good or moderate visual function in 56%, and this reduced to 53%. Baseline and final visual function were poorer in patients who had a surgical resection, but improvements in vision were still found—particularly in the best eye.Discussion/conclusionOPG are debilitating childhood tumor that have lifelong consequences in terms of visual function and endocrinopathies/hypothalamic dysfunction; this can result in substantial patient morbidity. Decisions regarding management and the role of surgery in this condition are challenging and include cerebrospinal fluid diversion, biopsy, and in highly select cases cystic decompression or surgical resection. In this paper, we review our own experience, outcomes, and surgical philosophy.
Heterotopic ossification in primary total hip arthroplasty using the posterolateral compared to the direct lateral approach
Authors: van Erp J. H. J. , Massier J. R. A. , Truijen S. , Bekkers J. E. J. , Snijders T. E. , de Gast A. 
Published: February 2021

Abstract
Purpose
Total hip arthroplasty (THA) is a successful procedure. However, in time, heterotopic ossification (HO) can form due to, amongst others, soft tissue damage. This can lead to pain and impairment. This study compares the formations of HO between patients who underwent either THA with the posterolateral approach (PA) or with the direct lateral approach (DLA). Our hypothesis is that patients who underwent THA with a PA form less HO compared to THA patients who underwent DLA.

Methods
In this prospective cohort study, 296 consecutive patients were included who underwent THA. A total of 127 patients underwent THA with the PA and 169 with the DLA. This was dependent on the surgeon’s preference and experience. More than 95% of patients had primary osteoarthritis as the primary diagnosis. Clinical outcomes were scored using the Numeric Rating Scale (NRS) and Harris Hip Score (HHS), radiological HO were scored using the Brooker classification. Follow-up was performed at 1 and 6 years postoperatively.

Results
Two hundred and fifty-eight patients (87%) completed the 6-year follow-up. HO formation occurred more in patients who underwent DLA, compared to PA (43(30%) vs. 21(18%), p = 0.024) after 6 years. However, the presence of severe HO (Brooker 3–4) was equal between the DLA and PA (7 vs. 5,p = 0.551). After 6 years the HHS and NRS for patient satisfaction were statistically significant higher after the PA (95.2 and 8.9, respectively) compared to the DLA (91.6 and 8.5, respectively) (p < 0.001 and p = 0.003, respectively). The NRS for load pain was statistically significant lower in the PA group (0.5) compared to the DLA group (1.2) (p = 0.004). The NRS for rest pain was equal: 0.3 in the PA group and 0.5 in the DLA group.

Conclusion
THA with the PA causes less HO formation than the DLA.

Trial registration
Registrated as HipVit trial, NL 32832.100.10, R-10.17D/HIPVIT 1. Central Commission Human-Related research (CCMO) Registry.
Chromosome X aneusomy and androgen receptor gene copy number aberrations in apocrine carcinoma of the breast
Authors: Cremonini Anna , Saragoni Luca , Morandi Luca , Corradini Angelo G. , Ravaioli Caterina , Di Oto Enrico , Limarzi Francesco , Sanchez Alejandro M. , Cucchi Maria C. , Masetti Riccardo , Quinn Cecily , Foschini Maria P. 
Published: February 2021

AbstractCarcinomas with apocrine differentiation (CAD) of the breast are rare tumours typically presenting high immunohistochemical expression of androgen receptor (AR) which is a target molecule for personalised therapy. To date, no studies have evaluated the genetic changes that are associated with AR immunohistochemical expression in CADs. The present work aims to characterise AR status in CADs. Twenty CAD tumours were studied with immunohistochemistry, in situ fluorescence hybridization and DNA methylation analysis, to evaluate AR expression and its regulator status. All tumours demonstrated high AR immunohistochemical expression, with over 95% of the neoplastic cells showing AR positivity in 19/20 cases. CADs showed AR gene copy loss in a percentage of neoplastic cells ranging from 5 to 84% (mean 48.93%). AR regulator genes, including the MAGE family, UXT and FLNA, presented variable methylation levels, but were mainly hypomethylated and therefore all transcriptionally active. The results of this study indicate that CADs present AR monosomy, paralleled by higher transcriptional activity of the gene with potential to influence response to AR deprivation therapy.
Practice variations in the management of childhood nephrotic syndrome in the Netherlands
Authors: Schijvens Anne M. , van der Weerd Lucie , van Wijk Joanna A. E. , Bouts Antonia H. M. , Keijzer-Veen Mandy G. , Dorresteijn Eiske M. , Schreuder Michiel F. 
Published: February 2021

AbstractNephrotic syndrome in childhood is a common entity in the field of pediatric nephrology. The optimal treatment of children with nephrotic syndrome is often debated. Previously conducted studies have shown significant variability in nephrotic syndrome management, especially in the choice of steroid-sparing drugs. In the Netherlands, a practice guideline on the management of childhood nephrotic syndrome has been available since 2010. The aim of this study was to identify practice variations and opportunities to improve clinical practice of childhood nephrotic syndrome in the Netherlands. A digital structured survey among Dutch pediatricians and pediatric nephrologists was performed, including questions regarding the initial treatment, relapse treatment, kidney biopsy, additional immunosuppressive treatment, and supportive care. Among the 51 responses, uniformity was seen in the management of a first presentation and first relapse. Wide variation was found in the tapering of steroids after alternate day dosing. Most pediatricians and pediatric nephrologists (83%) would perform a kidney biopsy in case of steroid-resistant nephrotic syndrome, whereas for frequent relapsing and steroid-dependent nephrotic syndrome this was 22% and 41%, respectively. Variation was reported in the steroid-sparing treatment. Finally, significant differences were present in the supportive treatment of nephrotic syndrome.Conclusion: Substantial variation was present in the management of nephrotic syndrome in the Netherlands. Differences were identified in steroid tapering, use of steroid coverage during stress, choice of steroid-sparing agents, and biopsy practice. To promote guideline adherence and reduce practice variation, factors driving this variation should be assessed and resolved.

What is Known:• National and international guidelines are available to guide the management of childhood nephrotic syndrome.• Several aspects of the management of childhood nephrotic syndrome, including the choice of steroid-sparing drugs and biopsy practice, are controversial and often debated among physicians.What is New:• Significant practice variation is present in the management of childhood nephrotic syndrome in the Netherlands, especially in the treatment of FRNS, SDNS, and SRNS.• The recommendation on the steroid treatment of a first episode of nephrotic syndrome in the KDIGO guideline leaves room for interpretation and is likely the cause of substantial differences in steroid-tapering practices among Dutch pediatricians and pediatric nephrologists.
Quality of life among advanced cancer patients in Vietnam: a multicenter cross-sectional study
Authors: Huyen Bui Thanh , Van Anh Pham Thi , Duong Le Dai , The Than Ha Ngoc , Guo Ping , Van Thuc Pham , Khue Luong Ngoc , Krakauer Eric L. , Harding Richard 
Published: February 2021

Abstract
Purpose
Cancer is a leading cause of death in Vietnam. To maximize quality of life (QOL) at the end of life, valid and clinically useful instruments are needed to assess palliative care needs and the effectiveness of palliative care interventions.

Methods
We aimed to (i) determine psychometric properties of the Vietnamese version of the WHO abbreviated quality of life scale (WHOQOL-BREFVN) among advanced cancer patients, (ii) measure HR-QOL, and (iii) identify predictors of HR-QOL. We collected demographic, clinical, and HR-QOL data from stage III/IV adult cancer patients at two major Vietnamese cancer centers. We determined the internal consistency (Cronbach’s alpha), construct validity (confirmatory factor analysis (CFA)), and discriminant validity (known-groups comparison) of the Vietnamese instrument. HR-QOL was analyzed descriptively. Multinomial logistic regressions identified predictors of HR-QOL.

Results
A total of 825 patients participated. Missing data were completely at random (MCAR) (chi-square = 14.270, df = 14, p = 0.430). Cronbach’s alpha for all items was 0.904. CFA loadings of physical, psychological, social relationship, and environment domains onto HR-QOL were 0.81, 0.82, 0.34, and 0.75, respectively. Prediction of scores differed significantly by functional status (Wilks’ lambda = 0.784, chi-square = 197.546, df = 4, p < 0.01, correct prediction = 74.6%). HR-QOL was reported as very bad/bad by n = 188 patients (22.8%) and general health as very bad/bad by n = 430 (52.1%). Multinomial logistic regression (likelihood ratio test: chi-square = 35.494, df = 24, p = 0.061, correct prediction = 62.2%) and the Pearson correlations revealed worse HR-QOL was associated with inpatient status, high ECOG score, and having dependent children.

Conclusion
The Vietnamese version of the WHOQOL-BREF has excellent internal consistency reliability and sound construct and discriminant validity in advanced cancer patients. Advanced cancer inpatients, those with dependent children, and those with poorer physical function appear to have the greatest palliative care needs.
Mycoplasma pneumoniae may cause dyspnoea and hospitalisations in young healthy adults
Authors: Metsälä Riku , Ala-Korpi Solja , Rannikko Juha , Helminen Merja , Renko Marjo 
Published: February 2021

AbstractPolymerase chain reaction (PCR)-based diagnostics for Mycoplasma pneumoniae (M. pneumoniae) from the respiratory tract has become widely available, but the interpretation of the results remains unclear. M. pneumoniae has been suggested to cause mainly mild and self-limiting infections or asymptomatic carriage. However, systematic analyses of the association between PCR results and clinical findings are scarce. This study aimed to clarify the clinical features of PCR-positive M. pneumoniae infections in a hospital setting. We reviewed 103 PCR-positive patients cared for in a university hospital during a 3-year period. Data on age, sex, health condition, acute symptoms, other pathogens found, laboratory and X-ray results and treatments were collected. Over 85% of the patients had a triad of typical symptoms: fever, cough and shortness of breath. Symptoms in the upper respiratory tract were rare. In 91% of the cases, M. pneumoniae was the only pathogen found. The highest incidence was found in the age group of 30–40 years, and 68% of the patients did not have any underlying diseases. Most patients were initially empirically treated with beta-lactam antibiotics and needed 2–4 changes in their treatment. Only 6% were discharged without an antibiotic effective against M. pneumoniae. This study shows that M. pneumoniae often led to hospitalisation and that patients needed appropriate antimicrobial treatment to recover. Mixed infections were rare, and situations that could be interpreted as carriage did not occur.
Impact of combined use of intraoperative MRI and awake microsurgical resection on patients with gliomas: a systematic review and meta-analysis
Authors: Tuleasca Constantin , Leroy Henri-Arthur , Peciu-Florianu Iulia , Strachowski Ondine , Derre Benoit , Levivier Marc , Schulder Michael , Reyns Nicolas 
Published: February 2021

AbstractMicrosurgical resection of primary brain tumors located within or near eloquent areas is challenging. Primary aim is to preserve neurological function, while maximizing the extent of resection (EOR), to optimize long-term neurooncological outcomes and quality of life. Here, we review the combined integration of awake craniotomy and intraoperative MRI (IoMRI) for primary brain tumors, due to their multiple challenges. A systematic review of the literature was performed, in accordance with the Prisma guidelines. Were included 13 series and a total number of 527 patients, who underwent 541 surgeries. We paid particular attention to operative time, rate of intraoperative seizures, rate of initial complete resection at the time of first IoMRI, the final complete gross total resection (GTR, complete radiological resection rates), and the immediate and definitive postoperative neurological complications. The mean duration of surgery was 6.3 h (median 7.05, range 3.8–7.9). The intraoperative seizure rate was 3.7% (range 1.4–6; I^2 = 0%, P heterogeneity = 0.569, standard error = 0.012, p = 0.002). The intraoperative complete resection rate at the time of first IoMRI was 35.2% (range 25.7–44.7; I^2 = 66.73%, P heterogeneity = 0.004, standard error = 0.048, p < 0.001). The rate of patients who underwent supplementary resection after one or several IoMRI was 46% (range 39.8–52.2; I^2 = 8.49%, P heterogeneity = 0.364, standard error = 0.032, p < 0.001). The GTR rate at discharge was 56.3% (range 47.5–65.1; I^2 = 60.19%, P heterogeneity = 0.01, standard error = 0.045, p < 0.001). The rate of immediate postoperative complications was 27.4% (range 15.2–39.6; I^2 = 92.62%, P heterogeneity < 0.001, standard error = 0.062, p < 0.001). The rate of permanent postoperative complications was 4.1% (range 1.3–6.9; I^2 = 38.52%, P heterogeneity = 0.123, standard error = 0.014, p = 0.004). Combined use of awake craniotomy and IoMRI can help in maximizing brain tumor resection in selected patients. The technical obstacles to doing so are not severe and can be managed by experienced neurosurgery and anesthesiology teams. The benefits of bringing these technologies to bear on patients with brain tumors in or near language areas are obvious. The lack of equipoise on this topic by experienced practitioners will make it difficult to do a prospective, randomized, clinical trial. In the opinion of the authors, such a trial would be unnecessary and would deprive some patients of the benefits of the best available methods for their tumor resections.
Understanding the Reasons for Deferring ART Among Patients Diagnosed Under the Same-Day-ART Policy in Johannesburg, South Africa
Authors: Onoya Dorina , Sineke Tembeka , Mokhele Idah , Bor Jacob , Fox Matthew P. , Miot Jacqui 
Published: February 2021

AbstractWe aimed to examine the correlates of antiretroviral therapy (ART) deferral to inform ART demand creation and retention interventions for patients diagnosed with HIV during the Universal Test and Treat (UTT) policy in South Africa. We conducted a cohort study enrolling newly diagnosed HIV-positive adults (≥ 18 years), at four primary healthcare clinics in Johannesburg between October 2017 and August 2018. Patients were interviewed immediately after HIV diagnosis, and ART initiation was determined through medical record review up to six-months post-test. ART deferral was defined as not starting ART six months after HIV diagnosis. Participants who were not on ART six-months post-test were traced and interviewed telephonically to determine reasons for ART deferral. Modified Poisson regression was used to evaluate correlates of six-months ART deferral. We adjusted for baseline demographic and clinical factors. We present crude and adjusted risk ratios (aRR) associated with ART deferral. Overall, 99/652 (15.2%) had deferred ART by six months, 20.5% men and 12.2% women. Baseline predictors of ART deferral were older age at diagnosis (adjusted risk ratio (aRR) 1.5 for 30–39.9 vs 18–29.9 years, 95% confidence intervals (CI): 1.0–2.2), disclosure of intentions to test for HIV (aRR 2.2 non-disclosure vs disclosure to a partner/spouse, 95% CI: 1.4–3.6) and HIV testing history (aRR 1.7 for  > 12 months vs < 12 months/no prior test, 95% CI: 1.0–2.8). Additionally, having a primary house in another country (aRR 2.1 vs current house, 95% CI: 1.4–3.1) and testing alone (RR 4.6 vs partner/spouse support, 95% CI: 1.2–18.3) predicted ART deferral among men. Among the 43/99 six-months interviews, women (71.4%) were more likely to self-report ART initiation than men (RR 0.4, 95% CI: 0.2–0.8) and participants who relocated within SA (RR 2.1 vs not relocated, 95% CI: 1.2–3.5) were more likely to still not be on ART. Under the treat-all ART policy, nearly 15.2% of study participants deferred ART initiation up to six months after the HIV diagnosis. Our analysis highlighted the need to pay particular attention to patients who show little social preparation for HIV testing and mobile populations.
Current recommendations for clinical surveillance and genetic testing in rhabdoid tumor predisposition: a report from the SIOPE Host Genome Working Group
Authors: Frühwald M. C. , Nemes K. , Boztug H. , Cornips M. C. A. , Evans D. G. , Farah R. , Glentis S. , Jorgensen M. , Katsibardi K. , Hirsch S. , Jahnukainen K. , Kventsel I. , Kerl K. , Kratz C. P. , Pajtler K. W. , Kordes U. , Ridola V. , Stutz E. , Bourdeaut F. 
Published: February 2021

AbstractThe rhabdoid tumor (RT) predisposition syndromes 1 and 2 (RTPS1 and 2) are rare genetic conditions rendering young children vulnerable to an increased risk of RT, malignant neoplasms affecting the kidney, miscellaneous soft-part tissues, the liver and the central nervous system (Atypical Teratoid Rhabdoid Tumors, ATRT). Both, RTPS1&2 are due to pathogenic variants (PV) in genes encoding constituents of the BAF chromatin remodeling complex, i.e. SMARCB1 (RTPS1) and SMARCA4 (RTPS2). In contrast to other genetic disorders related to PVs in SMARCB1 and SMARCA4 such as Coffin-Siris Syndrome, RTPS1&2 are characterized by a predominance of truncating PVs, terminating transcription thus explaining a specific cancer risk. The penetrance of RTPS1 early in life is high and associated with a poor survival. However, few unaffected carriers may be encountered. Beyond RT, the tumor spectrum may be larger than initially suspected, and cancer surveillance offered to unaffected carriers (siblings or parents) and long-term survivors of RT is still a matter of discussion. RTPS2 exposes female carriers to an ill-defined risk of small cell carcinoma of the ovaries, hypercalcemic type (SCCOHT), which may appear in prepubertal females. RT surveillance protocols for these rare families have not been established. To address unresolved issues in the care of individuals with RTPS and to propose appropriate surveillance guidelines in childhood, the SIOPe Host Genome working group invited pediatric oncologists and geneticists to contribute to an expert meeting. The current manuscript summarizes conclusions of the panel discussion, including consented statements as well as non-evidence-based proposals for validation in the future.
An Obata-type theorem on compact Einstein manifolds with boundary
Authors: Akutagawa Kazuo 
Published: February 2021

AbstractWe show a kind of Obata-type theorem on a compact Einstein n-manifold $$(W, \bar{g})$$

(
W
,

g

¯

)

with smooth boundary $$\partial W$$

∂
W

. Assume that the boundary $$\partial W$$

∂
W

is minimal in $$(W, \bar{g})$$

(
W
,

g

¯

)

. If $$(\partial W, \bar{g}|_{\partial W})$$

(
∂
W
,

g

¯

|

∂
W

)

is not conformally diffeomorphic to $$(S^{n-1}, g_S)$$

(

S

n
-
1

,

g
S

)

, then for any Einstein metric $$\check{g} \in [\bar{g}]$$

g
ˇ

∈

[

g

¯

]

with the minimal boundary condition, we have that, up to rescaling, $$\check{g} = \bar{g}$$

g
ˇ

=

g

¯

. Here, $$g_S$$

g
S

and $$[\bar{g}]$$

[

g

¯

]

denote respectively the standard round metric on the $$(n-1)$$

(
n
-
1
)

-sphere $$S^{n-1}$$

S

n
-
1

and the conformal class of $$\bar{g}$$

g

¯

. Moreover, if we assume that $$\partial W \subset (W, \bar{g})$$

∂
W
⊂
(
W
,

g

¯

)

is totally geodesic, we also show a Gursky-Han type inequality for the relative Yamabe constant of $$(W, \partial W, [\bar{g}])$$

(
W
,
∂
W
,
[

g

¯

]
)

.
Age as a merit in admission decisions for higher education
Authors: Sandsør Astrid Marie Jorde , Hovdhaugen Elisabeth , Bøckmann Ester 
Published: February 2021

AbstractThis paper uses register data to study how a particular age reward feature affects admission into two highly competitive study programs: medicine and law. The Norwegian admission system to higher education is centralized, and applicants compete in two quotas: one quota almost entirely based on grade point average from upper secondary education and one quota where students can compete with improved grades and where being older automatically increases the chance of acceptance, by awarding age points. For these study programs, we find that the admission system creates a waiting game, as gaining admission in the second quota is nearly impossible without accumulating a substantial amount of age points. If age predicts completion in higher education, this waiting game might be justified. However, if anything, we find the opposite to be true. Our paper suggests that age should carry less weight in admission decisions and that countries and/or higher education institutions should carefully consider how their admission system affects student incentives and how applicants are selected.