bluuebunny/crossref_metadata_2025_split huggingface dataset:
Cerebrospinal fluid β‐glucocerebrosidase activity is reduced in parkinson's disease patients
Authors: Parnetti Lucilla , Paciotti Silvia , Eusebi Paolo , Dardis Andrea , Zampieri Stefania , Chiasserini Davide , Tasegian Anna , Tambasco Nicola , Bembi Bruno , Calabresi Paolo , Beccari Tommaso 
Published: August 2017

AbstractBackgroundReduced β‐glucocerebrosidase activity was observed in postmortem brains of both GBA1 mutation carrier and noncarrier Parkinson's disease patients, suggesting that lower β‐glucocerebrosidase activity is a key feature in the pathogenesis of PD. The objectives of this study were to confirm whether there is reduced β‐glucocerebrosidase activity in the CSF of GBA1 mutation carrier and noncarrier PD patients and verify if other lysosomal enzymes show altered activity in the CSF.MethodsCSF β‐glucocerebrosidase, cathepsin D, and β‐hexosaminidase activities were measured in 79 PD and 61 healthy controls from the BioFIND cohort. The whole GBA1 gene was sequenced.ResultsEnzyme activities were normalized according to CSF protein content (specific activity). β‐glucocerebrosidase specific activity was significantly decreased in PD versus controls (‐28%, P < 0.001). GBA1 mutations were found in 10 of 79 PD patients (12.7%) and 3 of 61 controls (4.9%). GBA1 mutation carrier PD patients showed significantly lower β‐glucocerebrosidase specific activity versus noncarriers. β‐glucocerebrosidase specific activity was also decreased in noncarrier PD patients versus controls (‐25%, P < 0.001). Cathepsin D specific activity was lower in PD versus controls (‐21%, P < 0.001). β‐Hexosaminidase showed a similar trend. β‐Glucocerebrosidase specific activity fairly discriminated PD from controls (area under the curve, 0.72; sensitivity, 0.67; specificity, 0.77). A combination of β‐glucocerebrosidase, cathepsin D, and β‐hexosaminidase improved diagnostic accuracy (area under the curve, 0.77; sensitivity, 0.71; specificity, 0.85). Lower β‐glucocerebrosidase and β‐hexosaminidase specific activities were associated with worse cognitive performance.ConclusionsCSF β‐glucocerebrosidase activity is reduced in PD patients independent of their GBA1 mutation carrier status. Cathepsin D and β‐hexosaminidase were also decreased. The possible link between altered CSF lysosomal enzyme activities and cognitive decline deserves further investigation. © 2017 International Parkinson and Movement Disorder Society
How does parkinson's disease begin? Perspectives on neuroanatomical pathways, prions, and histology
Authors: Borghammer Per 
Published: August 2017

ABSTRACTParkinson's disease (PD) is a multisystem disorder with involvement of the peripheral nervous system. Misfolding and aggregation of α‐synuclein is central to the pathogenesis of PD, and it has been postulated that the disease may originate in olfactory and gastrointestinal nerve terminals. The prion‐like behavior of α‐synuclein has been convincingly demonstrated in vitro and in animal models of PD. Lewy‐type pathology have been detected in peripheral organs many years prior to PD diagnosis, and 2 independent studies have now suggested that truncal vagotomy may be protective against the disorder. Other lines of evidence are difficult to reconcile with a peripheral onset of PD, most importantly the relative scarcity of post mortem cases with isolated gastrointestinal α‐synuclein pathology without concomitant CNS pathology. This Scientific Perspectives article revisits some important topics with implications for the dual‐hit hypothesis. An account of the neuroanatomical pathways necessary for stereotypical α‐synuclein spreading is presented. Parallels to the existing knowledge on true prion disorders, including Creutzfeld‐Jakob disease, are examined. Finally, the vagotomy studies and the somewhat inconsistent findings in the growing literature on peripheral α‐synuclein pathology are discussed. It is concluded that the dual‐hit hypothesis remains a potential explanation for PD pathogenesis, but several issues need to be resolved before more firm conclusions can be drawn. © 2017 International Parkinson and Movement Disorder Society
Reconstruction of midface defects with the osteocutaneous radial forearm flap: Evaluation of long term outcomes including patient reported quality of life
Authors: Connolly Timothy M. , Sweeny Larissa , Greene Benjamin , Morlandt Anthony , Carroll William R. , Rosenthal Eben L. 
Published: August 2017

AbstractBackgroundMaxillectomy defects significantly impair quality of life. Prosthetics can overcome some of these issues, but has limitations. The role of the osteocutaneous radial forearm free flap (OC‐RFFF) has been established for reconstruction of smaller maxillectomy defects, but its role in larger defects is not well defined. We aim to evaluate outcomes after midface reconstruction utilizing the OC‐RFFF.MethodsRetrospective review of prospective database collected between 2005 and 2014 of midface reconstruction using OC‐RFFF in a tertiary care centre. Donor site complications and acute and long‐term recipient site complications were measured. Health related quality of life was assessed using the University of Washington Quality of Life (UW‐QOL) Questionnaire.ResultsA total of 68 midface defects were reconstructed using the OC‐RFFF. Acute recipient site complications included three flap failures (4%), and two additional microvascular revision cases for vascular compromise. Late recipient complications included fistula (n = 10, 14%), ectropion (n = 7, 10%), diplopia (n = 6, 9%) and exposed hardware (n = 5, 7%). Resection of cheek skin or orbital rim correlated with orbital complications. The incidence of fistula was not affected by defect size or prior radiation. There were two donor site infections and no instances of forearm fracture. Patients undergoing OC‐RFFF repair had mean scores for UW‐QOL outcomes higher than published rates of obturator quality of life.ConclusionThe OC‐RFFF is suited to a variety of midface defects and can be combined with hardware to reconstruct the orbital floor. Recipient site complications are common, but donor site morbidity is low and outcomes, including HR‐QOL, are acceptable.
Breast reconstruction with DIEP and SIEA flaps in patients with prior abdominal liposuction
Authors: Zavlin Dmitry , Jubbal Kevin T. , Ellsworth Warren A. , Spiegel Aldona J. 
Published: August 2017

AbstractIntroductionSuction‐assisted lipectomy (SAL) has been considered a relative contraindication for autologous breast reconstruction due to reservations about size and integrity of perforator vessels. Such patients are often not considered ideal candidates for breast reconstruction utilizing deep inferior epigastric perforator (DIEP) and superficial inferior epigastric artery (SIEA) flaps. The aim of this article is to describe our experience with these flaps after SAL.MethodsRetrospectively, patient charts from 2005 to 2015 were analyzed and 9 patients (13 flaps) were identified who received breast reconstruction after prior donor‐site SAL. Eight patients underwent DIEP and 1 patient an SIEA flap breast reconstruction. The patients' average age was 47.7 (ranging 33–64) years and their BMI 26.0 (ranging 21.1–36.5). Preoperative radiologic studies were obtained for all patients via either Doppler ultrasound or cross‐sectional imaging to assess abdominal perforators. Abdominal SAL took place between 2 and 20 years before reconstruction.ResultsOn average, 2.4 (ranging 1–4) perforators with a mean diameter of 0.68 mm (ranging 0.2–2.5 mm) were included per DIEP flap and anastomosed to the internal mammary arteries. Median follow‐up period was 1.2 (ranging 0.5–9.6) years without any flap loss, flap necrosis, hematoma, or unplanned reoperation. One breast seroma and one fat necrosis occurred. All patients had a successful reconstruction and finished treatment at our institution.ConclusionOur results show that DIEP and SIEA flaps are safe and effective options for breast reconstruction in patients with previous abdominal SAL. Extensive preoperative patient evaluation and perforator imaging is important in identifying suitable candidates within this patient population.
The bipedicled medial plantar flap: Vascular enhancement of a reverse flow Y–V medial plantar flap by the inclusion of a metatarsal artery perforator for the reconstruction of a forefoot defect—A case report
Authors: Scaglioni Mario F. , Franchi Alberto , Uyulmaz Semra , Giovanoli Pietro 
Published: August 2017

AbstractThe pedicled medial plantar (MP) flap is an optimal solution for defects involving the weight‐bearing areas of the foot. However, venous congestion is a frequently encountered complication, especially with the reverse‐flow variants of the flap, and several strategies have been reported in literature both to prevent and to manage it. In this article, we present the use of a medial plantar flap based distally on the lateral plantar artery with the adjunct of an extra metatarsal perforator from the dorsalis pedis artery as a vascular enhancement to avoid the venous congestion and to improve the overall circulation. This bipedicled flap was successfully advanced to cover a 5 × 3 cm soft‐tissue defect located at the level of the first and second metatarsal heads of the right foot resulting from the excision of a squamous cell carcinoma in an 80‐year‐old male patient with a history of cardiovascular disease. The postoperative course was uneventful. The patient was discharged 7 days after the operation and was able to walk after 6 weeks wearing normal shoes. We believe that preserving plantar metatarsal perforators during the harvesting of an MP flap based distally on the lateral plantar artery may be a precious adjunct as it increases blood supply, especially the venous drainage of the flap.
Novel Body Shape Descriptors for Abdominal Adiposity Prediction Using Magnetic Resonance Images and Stereovision Body Images
Authors: Sun Jingjing , Xu Bugao , Lee Jane , Freeland‐Graves Jeanne H. 
Published: August 2017

ObjectiveThe purpose of this study was to design novel shape descriptors based on three‐dimensional (3D) body images and to use these parameters to establish prediction models for abdominal adiposity.MethodsSixty‐six men and fifty‐five women were recruited for abdominal magnetic resonance imaging (MRI) and 3D whole‐body imaging. Volumes of abdominal visceral adipose tissue (VAT) and subcutaneous adipose tissue (SAT) were measured from MRI sequences by using a fully automated algorithm. The shape descriptors were measured on the 3D body images by using the software developed in this study. Multiple regression analysis was employed on the training data set (70% of the total participants) to develop predictive models for VAT and SAT, with potential predictors selected from age, BMI, and the body shape descriptors. The validation data set (30%) was used for the validation of the predictive models.ResultsThirteen body shape descriptors exhibited high correlations (P < 0.01) with abdominal adiposity. The optimal predictive equations for VAT and SAT were determined separately for men and women.ConclusionsNovel body shape descriptors defined on 3D body images can effectively predict abdominal adiposity quantified by MRI.
High‐dose treatment for malignant rhabdoid tumor of the kidney: No evidence for improved survival—The Gesellschaft für Pädiatrische Onkologie und Hämatologie (GPOH) experience
Authors: Furtwängler Rhoikos , Kager Leo , Melchior Patrick , Rübe Christian , Ebinger Martin , Nourkami‐Tutdibi Nasenien , Niggli Felix , Warmann Steven , Hubertus Jochen , Amman Gabriele , Leuschner Ivo , Vokuhl Christian , Graf Norbert , Frühwald Michael C. 
Published: August 2017

AbstractBackgroundMalignant rhabdoid tumor of the kidney (MRTK) is the most aggressive childhood renal tumor with overall survival (OS) rates ranging from 22% to 42%. Whether high‐dose chemotherapy with autologous stem‐cell transplantation (HDSCT) in an intensive first‐line treatment offers additional benefit is an ongoing discussion.MethodsA retrospective analysis of all 58 patients with MRTK from Austria, Switzerland, and Germany treated in the framework of consecutive, prospective renal/rhabdoid tumor studies SIOP9/GPO, SIOP93‐01/GPOH (where SIOP is International Society of Pediatric Oncology and GPOH is German Society of Pediatric Oncology and Hematology), SIOP2001/GPOH, and European Rhabdoid Tumor Registry from 1991 to 2014.ResultsMedian age at diagnosis was 11 months. Fifty percent of patients had metastases or multifocal disease at diagnosis (Stage IV). Local stage distribution was as follows: not done/I/II/III—1/6/11/40. Fifteen (26%) patients underwent upfront surgery. Thirty‐seven (64%) patients achieved a complete remission, 17 (29%) relapsed, 34 (59%) died of disease progression, and two (3%) died of treatment‐related complication. Mean time to the first event was 3.5 months. Two‐year EFS/OS (where EFS is event‐free survival) for the whole group was 37 ± 6%/38 ± 6%. Metastases/multifocal disease, younger age, and local stage III were associated with significantly inferior survival. Eleven (19%) patients underwent HDSCT (carboplatin + thiotepa, n = 6; carboplatin + etoposide + melphalan, n = 4; others, n = 1); 2‐year OS in this group was 60 ± 15% compared to 34 ± 8% in the non‐HDSCT group (P = 0.064). However, the time needed from radiologic to histologic diagnosis, stem‐cell harvest, and HDSCT must also be taken into account to avoid selection bias by excluding the highest risk group with early progression (<90 days). Thus, 2‐year EFS only for patients without progression until day 90 was 60 ± 16% consolidated by HDSCT compared to 62 ± 11% without (P = 0.8).ConclusionOur retrospective analysis suggests comparable outcomes for patients with and without HDSCT, if adjusted for early disease progression.
Exercise right heart catheterization for pulmonary hypertension identified on screening echocardiography in adult survivors of childhood cancer: A report from the St. Jude Lifetime Cohort
Authors: Armstrong Gregory T. , Tolle James J. , Piana Robert , Santucci Aimee , Leathers James , Ness Kirsten K. , Mulrooney Daniel A. , Green Daniel M. , Joshi Vijaya M. , Robison Leslie L , Hudson Melissa M. , Lenihan Daniel 
Published: August 2017

AbstractPulmonary hypertension, determined noninvasively by tricuspid regurgitant jet velocity on Doppler echocardiography, was previously identified in 25% of long‐term survivors who received chest‐directed radiotherapy. To validate noninvasively defined pulmonary hypertension, survivors (mean age 48 years), exposed to chest radiotherapy, underwent right heart catheterization with planned cardiopulmonary exercise testing during catheterization. Eight participants had an elevated mean pulmonary artery pressure at rest (≥25 mm Hg) or with subsequent exercise (>30 mm Hg), evidence of hemodynamically confirmed pulmonary hypertension by right heart catheterization. Cardiopulmonary exercise testing further defined the magnitude and etiology of cardiopulmonary limitations in this life‐threatening late effect.
Oral health and hematopoietic stem cell transplantation: A longitudinal evaluation of the first 28 days
Authors: Doss Laura M. , Dandoy Christopher E. , Kramer Kathi , Pate Abigail , Flesch Laura , El‐Bietar Javier , Lane Adam , Davies Stella M. , Thikkurissy Sarat 
Published: August 2017

AbstractBackgroundMucositis is well described after pediatric hematopoietic stem cell transplant (HSCT) but other aspects of oral health such as dental plaque and gingivitis are poorly understood. The aim of this study was to describe dental plaque, gingivitis, and mucositis early after HSCT.MethodsWe conducted a prospective longitudinal observational study to describe dental plaque, gingivitis, and mucositis in the peritransplant period. We conducted comprehensive oral evaluations that included the Miyazaki tongue coating, modified simplified oral hygiene, modified gingivitis of Suomi and Barbano, and mucosal ulceration indices at baseline on days 0, +7, +14, and +28.ResultsData were collected from 19 patients with a median age of 8.0 years (5.1–12.8) at time of HSCT. Sixteen patients (85%) had plaque accumulation that progressively worsened, 16 (85%) developed severe gingival inflammation, 13 (68%) developed mucositis, and 11 (58%) had oral ulcerations. All oral indices worsened from baseline during the study period. Gingivitis and oral plaque persisted in most patients at day +28 while mucositis and oral ulcerations slightly improved.DiscussionGingivitis, dental plaque, mucositis, and oral ulcerations are common after HSCT. Additional studies are needed to ascertain methods that decrease plaque and gingivitis development and severity.
Hypercalcaemia secondary to ectopic parathyroid hormone expression in an adolescent with metastatic alveolar rhabdomyosarcoma
Authors: Brooks Rachael , Lord Christopher , Davies Justin H. , Gray Juliet C. 
Published: August 2017

AbstractWe report the case of a 14‐year‐old male with metastatic alveolar rhabdomyosarcoma, presenting with hypercalcaemia (3.89 mmol/l) and elevated parathyroid hormone (PTH) level (10.2 pmol/l). Imaging demonstrated extensive bony lytic damage, with “floating teeth” in the mandible. Normalisation of calcium levels and bony reformation of the mandible occurred following chemotherapy; PTH levels decreased initially but remained above normal levels. Imaging did not demonstrate any evidence of parathyroid abnormality. Tumour ectopic PTH secretion is a very rare cause of hypercalcaemia of malignancy in children. Hypercalcaemia with an elevated PTH, in the absence of parathyroid‐related cause, should prompt investigation for underlying malignancy.
Sirolimus therapy in the treatment of pseudomyogenic hemangioendothelioma
Authors: Gabor Krisztina Mita , Sapi Zoltan , Tiszlavicz Lilla Gyorgyi , Fige Anita , Bereczki Csaba , Bartyik Katalin 
Published: August 2017

AbstractPseudomyogenic hemangioendothelioma (PMH) is a rare, mostly indolent vascular tumor. Extensive cases are treated with amputation as chemotherapy seems to be ineffective. Recently, promising results were published using mammalian target of rapamycin (mTOR) inhibitors in tumors of vascular origin. Here, we present a case of a child with advanced PMH relapsing after surgery and chemotherapy. Sirolimus achieved significant clinical improvement and stabilization of the lesions without any remarkable toxicity. This case contributes to the growing evidence regarding the efficacy of mTOR inhibitors, such as sirolimus, in multifocal PMH.
The amniotic fluid as a source of mesenchymal stem cells with lung‐specific characteristics
Authors: Lesage Flore , Zia Silvia , Jiménez Julio , Deprest Jan , Toelen Jaan 
Published: August 2017

What is already known about this topic?

Fetal tissues are interesting sources of stem cells for autologous therapy in a neonatal context.
AF‐MSC are a heterogeneous stem cell population of which the exact origin remains elusive.
What does this study add?

This study identifies AF‐MSC with lung‐specific characteristics.
This study provides a reliable culture method that supports the expansion of lung‐specific AF‐MSC.
Chitosan for wastewater treatment
Authors: Desbrières Jacques , Guibal Eric 
Published: August 2017

AbstractChitosan (an amino‐polysaccharide obtained from deacetylation of chitin, the major constituent of crustaceous shells and insect cuticles) presents a cationic character in acidic media allowing its dissolution, its shaping and possible ion‐exchange interactions with anionic compounds (a property applied in adsorption and coagulation–flocculation processes). In neutral media, non‐protonated amino groups allow complexation of metal cations or organic chemicals. These different properties explain the interest taken by the scientific community in using this biopolymer. In solution it contributes to complex metals and their recovery by complexation‐assisted ultrafiltration. It can also be used to coagulate–flocculate organic compounds (as anionic dyes). In the solid state, it can be used for metal ion adsorption, as well as adsorption of organic compounds (dyes, pesticides, drugs, endocrine disruptors, etc.). The adsorption and coagulation–flocculation processes will be compared and examples considered. Moreover, it is noteworthy that the thermal degradation of this type of material is also more environmentally friendly than that of conventional synthetic resins (production of hazardous by‐products, etc.), a supplementary advantage of these biopolymer‐based sorbents. Combined with its ability to be chemically or physically modified improving the potential and phase separation of chitosan‐based materials, all these properties mean it is an excellent candidate for wastewater treatment. © 2017 Society of Chemical Industry
Insulin Mediated Activation of PI3K/Akt Signalling Pathway Modifies the Proteomic Cargo of Extracellular Vesicles
Authors: Liem Michael , Ang Ching‐Seng , Mathivanan Suresh 
Published: August 2017

AbstractEpidemiological studies suggest that diabetes and obesity increases the risk of colorectal cancer (CRC) and lowers the patient survival rate. An important attribute in diabetes and obesity is the presence of high levels of growth factors including insulin in blood which can activate the PI3K/Akt signalling pathway. Dysregulation of PI3K/Akt signalling pathway leads to sustained proliferative signals thereby allowing the cells susceptible to cancer. Extracellular vesicles (EVs), secreted nanovesicles of endocytic origin, are implicated in mediating the transfer of oncogenic cargo in the tumour microenvironment. In this study, CRC cells were treated with insulin to activate PI3K/Akt signaling pathway. Insulin treatment significantly increased the number of EVs secreted by CRC cells. Furthermore, pAkt was exclusively packaged in EVs secreted by PI3K/Akt activated cells. Quantitative proteomics analysis confirmed that the protein cargo of EVs are modified upon activation of PI3K/Akt signaling pathway. Bioinformatics analysis highlighted the enrichment of proteins implicated in cell proliferation in EVs secreted by PI3K/Akt activated cells. Furthermore, incubation of EVs secreted by PI3K/Akt activated cells induced proliferation in recipient CRC cells. These findings suggest that EVs can amplify the signal provided by the growth factors in the tumor microenvironment and hence aid in cancer progression.
Side chain engineering of naphthalene diimide–bithiophene‐based polymer acceptors in all‐polymer solar cells
Authors: Wu Xia , Tang Yumin , Wang Yuxi , Liu Xiaoyuan , Liu Changmei , Zhang Xiaonan , Yang Yingguo , Gao Xingyu , Chen Fei , Guo Xugang , Chen Zhi‐Kuan 
Published: August 2017

ABSTRACTTwo novel polymeric acceptors based on naphthalene diimide (NDI) and 2.2′‐bithiophene, named as P(NDI2THD‐T2) and P(NDI2TOD‐T2), were designed and synthesized for all polymer solar cells application. The structural and electronic properties of the two acceptors were modulated through side‐chain engineering of the NDI units. The optoelectronic properties of the polymers and the morphologies of the blend films composed of the polymer acceptors and a donor polymer PTB7‐Th were systemically investigated. With thiophene groups introduced into the side chains of the NDI units, both polymers showed wider absorption from 350 nm to 900 nm, compared with the reference polymer acceptor of N2200. No redshift of absorption spectra from solutions to films indicated reduced aggregation of the polymers due to the steric hindrance effect of thiophene rings in the side chains. The photovoltaic performance were characterized for devices in a configuration of ITO/PEDOT:PSS/PTB7‐Th:acceptors/2,9‐bis(3‐(dimethylamino)propyl)anthra[2,1,9‐def:6,5,10‐def]diisoquinoline‐1,3,8,10(2H,9H)‐tetraone (PDIN)/Al. With the addition of diphenyl ether as an additive, the power conversion efficiencies (PCEs) of 2.73% and 4.75% for P(NDI2THD‐T2) and P(NDI2TOD‐T2) based devices were achieved, respectively. The latter showed improved Jsc, Fill Factor (FF), and PCE compared with N2200 based devices. © 2017 Wiley Periodicals, Inc. J. Polym. Sci., Part A: Polym. Chem. 2017, 55, 3679–3689