bluuebunny/crossref_metadata_2025_split huggingface dataset:
Primary Extraosseous Spinal Ewing Sarcomas
Authors: Amelot Aymeric , Peyre Matthieu , Mokhtari Karima , Carpentier Alexandre , Nouet Aurélien , Bielle Franck , Clemenceau Stephane , Kalamarides Michel , Mathon Bertrand 
Published: November 2020

Study Design.
Single-center retrospective study

Objective.
We discuss the widespread misdiagnosis of primary extraosseous spinal Ewing Sarcomas (PESES) to begnin tumors leading to poor treatment

Summary of Background Data.
PESES is a particular entity of spinal Ewing sarcoma (SES) appearing in a similar shape and features to benign tumors such as schwannomas. This imaging mimicry and subsequent possible misdiagnosis lead to primary surgery, without neoadjuvant chemotherapy, which remains deleterious for survival and progression.

Methods.
We identified a total of 13 patients: seven women (53.8%) and six men operated between 2001 and 2018 for PESES and initially misdiagnosed as schwannomas or ependymomas

Results.
The mean age of our series was 35.8 years (range, 18.1–47.2 years). The first clinical symptom was neuralgia (61.5%) followed or associated with nerves deficits (38.5%). Median progression-free survival (PFS) was 31.7 months (SD 5.8). Tumor recurrence rates at 1 and 3 years were respectively 21.2% (SD 3.1) and 60.1% (SD 15.8). Median overall survival (OS) was 61.5 months (SD 16.27). The 1-year, 2-year, and 5-year survival estimates were 100.0%, 88.9% (SD 10.5), and 44.4% (SD 16.6). Six patients (46.13%) died following their SES. In univariate analyses, patients with metastastic PESES had a significantly lower OS than others (41.2 months, P = 0.03).

Conclusion.
PESES must be ruled out at diagnosis of a spinal tumor when facing a fast-growing lesion with neurological deficits in a young adult. Thoracoabdominopelvic extension should be carried out. Presurgical biopsy must be performed. In case of PESES, neoadjuvant chemotherapy must be established before considering surgical intervention.

Level of Evidence: 4
Sagittal Alignment in Patients with Thoracic Myelopathy Caused by the Ossification of the Ligamentum Flavum
Authors: Chang Sam Yeol , Kim Yeonho , Kim Junho , Kim Hyoungmin , Kim Ho-Joong , Yeom Jin S. , Lee Choon-Ki , Chang Bong-Soon 
Published: November 2020

Study design.
Retrospective case–control study

Objective.
The aim of this study was to describe and compare the sagittal spinal alignment between patients with and without the ossification of the ligamentum flavum in the thoracic spine (TOLF).

Summary of Background Data.
Although mechanical factors play an important role in the development of TOLF, limited evidence exists on the association of sagittal spinal alignment and TOLF in the literature.

Methods.
The present study assessed the preoperative sagittal alignment parameters in consecutive patients who underwent posterior decompression for TOLF in a single institution between January 2014 and December 2019. The sagittal parameters of the patients with TOLF were compared to those of the age- and sex-matched control group with lumbar spondylosis.

Results.
The TOLF group (n = 43 [23 men, 20 women]), with a mean age of 69.5 (range: 41–86) years, and the control group (n = 86) were compared. The TOLF group had a significantly smaller sacral slope (27.60 ± 7.49 vs. 30.61 ± 8.15, P = 0.045) and lumbar lordosis (36.84 ± 13.63 vs. 45.08 ± 9.90, P < 0.001) and a larger pelvic incidence minus lumbar lordosis (PI-LL, 8.06 ± 15.05 vs.1.00 ± 11.34, P = 0.004) than the control group. Moreover, the thoracic kyphosis was smaller in the TOLF group, although the difference was not statistically significant (25.73 ± 11.29 vs. 28.22 ± 9.34, P = 0.187). The TOLF group had a significantly smaller slope angle at the inflection point (11.97 ± 5.85 vs. 15.78 ± 5.62, P < 0.001) and a higher tendency to have a Roussouly type 2 morphology (46.5% vs. 36.0%, P = 0.252) than the control group.

Conclusion.
This is the first study to describe the sagittal alignment in patients with TOLF. The TOLF group showed a hypolordotic spine with a larger PI-LL mismatch compared to the age- and sex-matched control group with lumbar spondylosis.

Level of Evidence: 4
Disparity of human immunodeficiency virus incidence and drug resistance in college student, non-student youth and older men who have sex with men: a cross-sectional study from seven major cities of China
Authors: Zhang Jing , Xu Jun-Jie , Chu Zhen-Xing , Hu Qing-Hai , Han Xiao-Xu , Zhao Bin , Jiang Yong-Jun , Geng Wen-Qing , Shang Hong 
Published: November 2020

Abstract

Background:
Human immunodeficiency virus (HIV) prevalence among student men who have sex with men (MSM) in college is more than 5.0% and keeps on increasing in China. This study aims to clarify the proportion of HIV recent infection, its propeller and the source among college student MSM.

Methods:
We conducted a multicenter cross-sectional study in seven major Chinese cities during 2012-2013. HIV recent infections (≤ 168 days) and incidence was measured and estimated by BED HIV-1 capture enzyme immunoassay (BED-CEIA) testing strategy. HIV-related behaviors and transmitted drug resistance (TDR) were investigated and compared between the college student MSM, <25-year-old non-student youth MSM (NSYM), and ≥25-year-old non-student non-youth MSM (NSNYM), using structured survey, and analyses of drug resistance.

Results:
Overall, 4,496 (4496/4526, 99.3%) were eligible for enrollment, comprising 565 college student MSM, 1,094 NSYM, and 2,837 NSNYM. The proportion of HIV recent infection were 70.3% (26/37), 50.8% (65/128) and 35.1% (95/271), the HIV incidence rate were 10.0 (95% CI: 6.2-13.9)/100PY, 12.9 (95% CI: 9.8-16.1)/100PY, 6.8 (95% CI: 5.4-8.2)/100PY, and TDR prevalence were 7.4% (2/27), 2.0%, (2/98) and 4.9% (11/226), among student MSM, NSYM, and NSNYM, respectively. Among HIV positive student MSM with age< 21-year-old, the proportion of HIV recent infection is 90.9% (10/11). Factors independently associated with HIV recent infection in student MSM was usage of recreational drug in the past 6 months (AOR: 2.5; 95% CI: 1.0–5.8).

Conclusions:
College student MSM had higher proportion of HIV recent infection and TDR than the youth and older MSM in China during 2012-2013. The HIV infections were more likely to happen during the early year of college life among student MSM.
Clinical outcomes of people living with human immunodeficiency virus (HIV) with diffuse large B-cell lymphoma (DLBCL) in Shanghai, China
Authors: Sun Jian-Jun , Liu Li , Wang Jiang-Rong , Shen Yin-Zhong , Qi Tang-Kai , Wang Zhen-Yan , Tang Yang , Song Wei , Chen Jun , Zhang Ren-Fang 
Published: November 2020

Abstract

Background:
Numerous studies have focused on lymphoma among patients infected with human immunodeficiency virus (HIV). However, little is known about the treatment options and survival rate of lymphoma in the Chinese people living with HIV (PLHIV). Our study aimed to investigate the prognosis and compare outcome of dose-adjusted etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin, and rituximab (DA-EPOCH-R) with standard cyclophosphamide, doxorubicin, vincristine, prednisone and rituximab(R-CHOP) as front line therapy for PLHIV with diffuse large B-cell lymphoma (DLBCL) receiving modern combined antiretroviral therapy (cART).

Methods:
A retrospective analysis evaluating PLHIV with DLBCL was performed in Shanghai Public Health Clinical Center from July 2012 to September 2019. The demographic and clinical data were collected, and overall survival (OS) and progression-free survival (PFS) analyses of patients receiving R-CHOP or DA-EPOCH-R therapy were performed by Kaplan-Meier analysis. Additionally, a Cox multiple regression model was constructed to identify related factors for OS.

Results:
A total of 54 eligible patients were included in the final analysis with a median follow-up of 14 months (interquartile range [IQR]: 8–29 months). The proportion of high international prognostic index (IPI) patients was much larger in the DA-EPOCH-R group (n = 29) than that in the R-CHOP group (n = 25). The CD4 cell counts and HIV RNA levels were not significantly different between the two groups. The 2-year OS for all patients was 73%. However, OS was not significantly different between the two groups, with a 2-year OS rate of 78% for the DA-EPOCH-R group and 66% for the R-CHOP group. Only an IPI greater than 3 was associated with a decrease in OS, with a hazard ratio of 5.0. The occurrence of grade 3 and 4 adverse events of chemotherapy was not significantly different between the two groups.

Conclusions:
Outcomes of R-CHOP therapy do not differ from those of DA-EPOCH-R therapy. No HIV-related factors were found to be associated with the OS of PLHIV in the modern cART era.
Hypopigmented Mycosis Fungoides Mimicking Vitiligo
Authors: Kim Jin Cheol , Kim You Chan 
Published: November 2020

Abstract:
Hypopigmented mycosis fungoides (HMF) is a clinical variant of MF with a presentation similar to other hypopigmented diseases, especially vitiligo. In this article, we report an adult case of HMF mimicking vitiligo. A 53-year-old man presented with an asymptomatic well-defined focal and hypopigmented patch with erythematous to brownish macules on the flank which had been developing over several months without other cutaneous findings. He had no past medical or trauma history. Skin biopsy from the hypopigmented patch indicated a slightly band-like, superficial dermal infiltrate of lymphocytes with mild cytologic atypia and epidermotropism. Fontana-Masson and Mart-1 stains showed a decrease in the epidermal pigment and the number of basal melanocytes. In addition, CD4 and CD8 stains were positive, predominantly the CD8 stain, and loss of CD7 stain was noted in the epidermal atypical lymphocytes. A T-cell receptor gene rearrangement study from the hyperpigmented area showed monoclonality. Finally, we diagnosed the patient with HMF. After about 17 months of treatment with narrow-band ultraviolet B, the hypopigmented lesion had notably improved in both the clinical and histological aspects. The clinical appearance of our case was similar to vitiligo while clinical improvement was also exceptionally similar to the skin findings from follicular repigmentation after narrow-band ultraviolet B treatment in vitiligo. Therefore, dermatologists should consider the clinical differential diagnosis of HMF in patients with an asymptomatic hypopigmentation, especially in dark-skinned Asian patients.
Recurrent Antineutrophil Cytoplasmic Antibody–Associated Vasculitis Induced by Levamisole-Adulterated Cocaine
Authors: Macias Alonso Begoña , Bashyam Arjun M. , Eginli Ariana N. , Ahn Christine S. , McMichael Amy J. 
Published: November 2020

Abstract:
Levamisole, an anthelmintic and immunomodulatory drug, was withdrawn from the US market in 1999 due to adverse effects, including agranulocytosis and vasculitis. In recent years, levamisole has been used as a common cocaine adulterant, and its use has led to an autoimmune syndrome characterized by an antineutrophil cytoplasmic antibody–associated vasculitis presenting with necrotic retiform purpura on the face and extremities. We present a case of recurrent levamisole-induced vasculitis initially misdiagnosed as systemic lupus erythematosus to highlight this easily misdiagnosed entity and to demonstrate re-exposure leading to recurrent vasculitis with more extensive clinical manifestations.
Comparison of Corneal Biomechanical Properties and Corneal Tomography Between Customized and Accelerated Corneal Crosslinking in Eyes with Keratoconus
Authors: Nishida Tomoya , Kojima Takashi , Kataoka Takahiro , Isogai Naoki , Yoshida Yoko , Nakamura Tomoaki 
Published: November 2020

Purpose:
To compare the changes in corneal biomechanical properties and corneal tomography between transepithelial customized corneal crosslinking (C-CXL) and epithelium-off accelerated corneal crosslinking (A-CXL) in eyes with keratoconus.

Methods:
Twenty eyes in 20 consecutive patients who underwent C-CXL (C-CXL group) and 20 eyes in 20 patients who underwent A-CXL (A-CXL group) were included in this retrospective comparative study. The corneal biomechanical properties were analyzed using a Scheimpflug-based tonometer, and all corneas were examined by anterior segment optical coherence tomography (AS-OCT) before and 3 months after surgery. The corneal biomechanical parameters analyzed were the maximum inverse radius, deformation amplitude (DA) ratio max (2 mm), stiffness parameter at applanation 1, and integrated radius. The AS-OCT parameters analyzed included average keratometry, corneal astigmatism, maximum keratometry reading (Kmax), higher-order irregularity, and asymmetry.

Results:
In the C-CXL group, there were significant improvements in biomechanical parameters, including the maximum inverse radius, the DA ratio max (2 mm), and the integrated radius after surgery (P = 0.037, P = 0.002, and P = 0.003, respectively). In the C-CXL group, there was a significant decrease in the Kmax, higher-order irregularity, and asymmetry components (P = 0.014, P = 0.008, and P = 0.016, respectively). The biomechanical properties and AS-OCT parameters did not change significantly in the A-CXL group after surgery. According to multiple regression analyses, C-CXL had a greater effect than A-CXL in improving the maximum inverse radius, DA ratio max (2 mm), integrated radius, Kmax, asymmetry component, and higher-order irregularity component.

Conclusions:
C-CXL might improve the biomechanical properties and irregular shape of the cornea from the early postoperative period to a greater extent than A-CXL.
Inhibition of Fibrotic Contraction by Sirolimus (Rapamycin) in an Ex Vivo Model of Thyroid Eye Disease
Authors: Roos Jonathan C. P. , Eglitis Viesturs , Murthy Rachna 
Published: November 2020

Background:
Thyroid eye disease (TED) is characterized by orbital inflammation and complicated by extraocular muscle fibrosis. Treatment with rapamycin/sirolimus has been reported to improve ocular motility and disease manifestations in TED. Whether this resulted from a primary antifibrotic effect on fibroblasts or was secondary to immune-suppression is unclear.

Methods:
In vitro contractility studies of primary orbital fibroblasts. Cells from patients with TED and controls were treated with rapamycin [mechanistic target of rapamycin an (mTOR) inhibitor] and MHY1485 (an mTOR stimulator) as well as inhibitors upstream in the same signaling cascade (saracatinib and befatinib).

Results:
At concentrations consistent with the therapeutic dosing range in humans, rapamycin/sirolimus significantly reduces fibrosis in orbital fibroblasts from TED patients and controls in vitro. This effect is separate from, and in addition to, its immune suppressive effect. mTOR-driven fibrotic activity is greater in TED-derived fibroblasts and can be blocked also upstream of mTOR by inhibition of src. There was no adverse effect on cell survival.

Conclusion:
The authors present evidence for a direct antifibrotic effect of rapamycin/sirolimus in primary orbital fibroblasts. Targeting mTOR signaling presents a further and adjunctive treatment of TED alongside other immune-suppressive agents. By acting downstream of IGF1-R, sirolimus may offer a cost-effective alternative to teprotumumab therapy. Clinical case reports, now supplemented by this in vitro evidence, support the initiation of a clinical trial to treat the fibrotic sequelae of TED with this already-approved agent. Such an “off-the-shelf” therapy is a welcome prospect for TED treatment, particularly one available at a low price.
Volumetric Versus Empirical Determination of Enucleation Implant Size
Authors: Custer Philip L. , Maamari Robi N. , Huecker Julia B. , Gordon Mae O. 
Published: November 2020

Purpose:
To compare the use of sizing implants versus intraoperative volume measurements for individualization of enucleation implant size, and to identify preoperative factors affecting the choice of implant.

Methods:
In this retrospective observational cohort study, 2 implant sizing methods were compared using clinical records and photographs in 139 patients who underwent enucleation between 2007 and 2016.

Results:
There was no difference in mean implant (p = 0.6562) or prosthetic (p = 0.1990) enophthalmos between the 2 methods, or when comparing patients with and without conjunctival-Tenons fibrosis. The incidence and severity of superior sulcus deformity was similar between the methods (p = 0.6394). Preoperative phthisis (p < 0.0001) and intraoperative conjunctival-Tenons fibrosis (p = 0.0010) were more common in the sizing implant group. Among all study patients, mean implant size was larger in males (21.3 mm, n = 80) than females (20.7 mm, n = 59) (p = 0.0027). Implants >20 mm were more frequently inserted in patients with a wider Hertel exophthalmometry base (80% of males >95 mm; 77% of females >97 mm). Approximately 21.0% of patients demonstrated ≥8 mm of implant enophthalmos, suggesting an undersized implant. The authors estimate that 11% of patients could have received a sphere larger than 22 mm.

Conclusions:
Individualization of enucleation implant size can reduce the incidence of anophthalmic socket syndrome. The empirical use of sizing implants provided similar results as intraoperative volumetric measurements, when evaluated by postoperative superior sulcus depth and prosthetic or implant enophthalmos. Preoperative exophthalmometry may facilitate selection of implant size. Preexisting conjunctival-Tenon fascial fibrosis often did not limit implant size and was not associated with a higher prevalence of wound breakdown or implant exposure.
Toe Absence Related to Verrucous Carcinoma
Authors: Zheng Xiao-Li , Yi Fei , Jiang Yi-Qun , Wang Qian-Qiu , Liang Guan-Zhao , Yin Yue-Ping , Zhang Guo-Yi 
Published: November 2020

Abstract

Introduction:
Verrucous carcinoma is a rare variant of squamous cell carcinoma. It generally grows exogenously and bone erosion is rare.
Here we present a case of verrucous carcinoma on the foot with toe absence.

Case presentation:
A 64-year-old man presented with a 6-year history of a slowly growing exogenous hyperkeratotic verrucous lesion on the right fifth toe, which had been absent for 1 year. Before the lesion appeared, he had undergone nail extraction on this toe. The culture of fungi and mycobacteria were negative. Three-dimensional computed tomography showed that the fifth toe of the right foot was absent. Histopathological examination showed that the lesion was exogenous and verrucous and exhibited moderate keratinocyte hyperplasia. Combined with clinical manifestation and histopathological examination, verrucous carcinoma is suggested. The lesion was surgically excised, and the patient was followed up for more than 6 months without recurrence.

Discussion:
Verrucous carcinoma is an exophytic tumor with verrucous growth at the beginning, which can invade deep tissues and even cause limb mutilation. When encountering a patient with a wart-like plaque on the foot that responds poorly to conventional treatments, clinicians should maintain a high degree of clinical vigilance and a low threshold for biopsy.

Conclusion:
We observed a rare outcome of toe absence associated with verrucous carcinoma. Therefore, early diagnosis of verrucous carcinoma is very important. Surgical excision is an effective treatment at present. Extensive resection is usually required to avoid recurrence.
Role of Tight Junctions and Their Protein Expression in Atopic Dermatitis
Authors: Namrata Kunwar , Bai Bing-Xue 
Published: November 2020

Abstract
Atopic dermatitis (AD) is a chronic inflammatory skin disease with xerosis, itchiness, as well as interconnection with immunoglobulin E (Ig E), mediated foods including airborne allergies. AD is not only related to the diminished stratum corneum barrier but also presents with an unusual expression of tight junctions (TJs) proteins. TJ barrier dysfunction leads to impairment in the stratum corneum (SC) barrier. The significant role of TJs in the epidermal barrier as indicated by Claudin-1 (Cldn-1) deficient mice that undergo high transepidermal water loss (TEWL) and skin dehydration. In atopic dermatitis, downregulation of Cldn-1 was observed due to inflammation. Still, a lack of distinct understanding exists in considering tight junction barrier impairment as a cause or outcome in atopic dermatitis. This review summarizes TJs main role in skin barrier function and TJ proteins (TJPs) expression observed in AD patients.
A Study of Normal Epidermal Melanocyte Distribution
Authors: Sun Kai-Lv , Liu Wan , Gao Xiao-Man , Yang Min , Chang Jian-Min 
Published: November 2020

Abstract

Objective:
To elucidate the epidermal melanocyte distribution according to sex, age, and body part with the goal of providing benchmark data for the diagnosis and therapeutic effect evaluation of pigmentary skin diseases.

Methods:
Epidermal melanocytes and keratinocytes were assessed using direct immunofluorescence staining, and the melanocyte density and epidermal thickness were calculated. The obtained data were statistically analyzed using SPSS Version 20.0 software. An independent-samples t test was used to compare the data between two groups, while data among three or more groups were compared by one-way analysis of variance. Data correlations were evaluated using Pearson correlation analysis.

Results:
Melanocytes were uniformly distributed among the keratinocytes in the basal layer, and the average ratio of melanocytes to keratinocytes was 1:7. Among them, the ratio in males was 1:6.5 and that in females was 1:7.4, with no significant difference (P = 0.127). The melanocyte density gradually declined as age increased; the ratio was 1:5.8 before 50 years of age without an obvious downtrend. The average melanocyte density was 1:7.9 within 51 to 65 years of age and 1:8.5 at >65 years of age, and the difference was statistically significant (P < 0.01). Obvious differences were found in the melanocyte density among different body parts; in descending order, these densities were as follows: face (1:4.0) >neck (1:5.1) >hip (1:5.7) >upper limb (1:7.4) >lower limb (1:8.3) >lower back (1:9.2) >thorax and abdomen (1:9.9). The melanocyte density was not related to the epidermal thickness.

Conclusion:
The melanocyte density showed a declining trend with age and significantly changed after 50 years of age. The melanocyte density was associated with body part; specifically, the density in the face, neck, and hip was higher than that in the limbs and torso. However, the melanocyte density was not associated with sex or epidermal thickness.
Kidney Stones Are Prevalent in Individuals with Pseudoxanthoma Elasticum, a Genetic Ectopic Mineralization Disorder
Authors: Ralph Douglas , Allawh Rina , Terry Ian F. , Terry Sharon F. , Uitto Jouni , Li Qiao-Li 
Published: November 2020

Abstract

Objective:
Pseudoxanthoma elasticum (PXE) is a rare genetic disorder caused by loss-of-function mutations in the ABCC6 gene. While PXE is characterized by ectopic mineralization of connective tissues clinically affecting the skin, eyes, and cardiovascular system, kidney stones were reported in some individuals with PXE. The aim of this study is to determine whether kidney stones are an incidental finding or a frequent manifestation of PXE.

Methods:
We first investigated the genetic basis of two siblings diagnosed with PXE. The younger patient presented with recurrent kidney stones since 8 years old. Secondly, to address whether kidney stones are associated with PXE, the prevalence of kidney stones in a survey cohort of 563 respondents with PXE was compared to that of a general U.S. population survey, National Health and Nutrition Examination Survey, with 28,629 participants.

Results:
Genetic analysis in both patients identified compound heterozygous mutations in ABCC6, c.2787+1G>T, and c.3774_3775insC. The analysis of participants 20 years old and older revealed that 23.4% of PXE patients had previously had a kidney stone, a significant increase compared to 9.2% in the general population (P < 0.01). In addition, 17.8% of PXE patients reported their first kidney stone episode before age of 18 years old.

Conclusions:
PXE correlates with an increased risk of developing kidney stones with considerable morbidity and health-care cost.