bluuebunny/crossref_metadata_2025_split huggingface dataset:
Karpaltunnelsyndrom und ATTR-Amyloidose
Authors: Hahn Katharina , Urban Peter , Meliß Rolf Rüdiger , Axmann Hans-Detlef , Siebert Frank , Röcken Christoph 
Published: November 2018

Zusammenfassung
Hintergrund Das Karpaltunnelsyndrom ist das häufigste Kompressionssyndrom
eines peripheren Nervs. Unter anderem wird es durch Transthyretin-Amyloid
verursacht, das sich in Karpaltunnelgeweben ablagert. Die meisten Betroffenen
sind älter als 50 Jahre. Das Karpaltunnelsyndrom stellt häufig das erste Symptom
einer Transthyretin-Amyloidose dar, die im schlimmsten Fall zur
Amyloid-Kardiomyopathie mit den Symptomen einer Herzinsuffizienz führt und die
Lebensqualität und Lebenszeit einschränkt. Für diesen Artikel wurden alle
histologischen Gewebeproben von Karpaltunnelgewebe aus den Jahren 2010 bis 2018
nach den klinischen Patienteneigenschaften Alter und Geschlecht ausgewertet.
Durch die gezielte Auswertung des Transthyretin-Amyloidgehalts in verschiedenen
Regionen des Karpaltunnels konnte eine Empfehlung zur Resektion für die optimale
histologische Diagnostik herausgearbeitet werden.
Material und Methoden Im Amyloidregister Kiel werden alle Fälle unter
anderem nach Amyloidart und vorliegendem Gewebe archiviert. Für die Auswertung
lagen 582 Resektate von Patientinnen und Patienten mit Transthyretin-Amyloid im
Karpaltunnelgewebe aus den Jahren 2010 bis Anfang 2018 vor. Ergänzend wurde der
Transthyretin-Amyloidgehalt aus zwei verschiedenen Regionen des Karpaltunnels
(Synovialisgewebe und Retinaculum flexorum) miteinander verglichen.
Ergebnisse Es lagen mehrheitlich Resektate von Frauen vor (53 %). Das
mediane Alter betrug 78 Jahre für das gesamte Kollektiv, 77 Jahre für Männer und
79 Jahre für Frauen. Die Resektate des Retinaculum flexorum enthielten
signifikant mehr Amyloid (9,66 % Amyloid) als die Resektate aus dem
Synovialisgewebe des Karpaltunnels (2,10 % Amyloid). Die Prävalenz der durch
ATTR-Amyloid-bedingten Karpaltunnelsyndrome beträgt 11,66 %.
Schlussfolgerungen Sowohl Männer als auch Frauen erkranken an einem
Karpaltunnelsyndrom, das durch Transthyretin-Amyloid verursacht wird.
Insbesondere im Alter von über 50 Jahren sollte im Rahmen der ätiologischen
Abklärung des Karpaltunnelsyndroms an Amyloidablagerungen als Auslöser gedacht
werden. Die frühzeitige histologische Diagnostik ist in Hinblick auf das Risiko
einer kardialen Amyloidose von hoher Relevanz. Für eine frühzeitige und korrekte
Diagnose der Transthyretin-Amyloidose im Karpaltunnelgewebe eignet sich vor
allem die histologische Untersuchung des Retinaculum flexorum, so dass von
diesem immer eine Probe-Exzision entnommen und histologisch untersucht werden
sollte.
Diffuse Alveolar Hemorrhage and Pulmonary Vasculitides: Histopathologic Findings
Authors: Scapa Jason , Fishbein Gregory , Wallace W. , Fishbein Michael 
Published: November 2018

AbstractVasculitides are a heterogeneous group of disorders in which inflammation of blood vessel walls is present at least some time during the course of the disease. Vasculitides can affect any caliber or type of vessel in many anatomic sites; however, the disease can alter more than just vasculature. Given the diversity of vasculitides, in 2012, a revised classification system was proposed to categorize vasculitides by the type of vessel involved including size, function, and structural attributes. In the lung, vasculitis impacts both the pulmonary vessels and parenchyma. Extrapulmonary involvement, particularly with concomitant kidney involvement, is a frequent occurrence. Pulmonary vasculitides often present with hemoptysis, pathologically manifested as diffuse alveolar hemorrhage (DAH) with or without capillaritis and can be life threatening when diffuse throughout the lungs. Etiologies for DAH include both primary and secondary vasculitides, along with collagen-vascular diseases, infection, and drug toxicity. Therefore, diagnosing the specific vasculitic etiology often relies on comprehensive assessment of all clinical, laboratory/serological, imaging, and histopathologic features that may be present. The most common primary pulmonary vasculitides often affect small vessels and are associated with circulating antineutrophilic cytoplasmic antibodies (ANCAs). In the 2012 classification, these include granulomatosis with polyangiitis (formerly Wegener granulomatosis), eosinophilic granulomatosis with polyangiitis (formerly Churg–Strauss' syndrome), and microscopic polyangiitis. Other less frequent vasculitides that are non-ANCA associated or affect medium- to large-sized vessels can have pulmonary involvement. These entities are usually associated with extrapulmonary disease and include polyarteritis nodosa, Takayasu's arteritis, Behçet's disease, and antibasement membrane antibody disease (formerly Goodpasture's syndrome). Although all vasculitides have vessel wall inflammation at some phase in the disease process, their histopathologic findings are as diverse as the group of diseases themselves. The characteristic histologic findings of the pulmonary vasculitides will be reviewed here.
Antiglomerular Basement Membrane Disease
Authors: Pusey Charles , McAdoo Stephen 
Published: November 2018

AbstractAntiglomerular basement membrane (anti-GBM) disease is a rare but life-threatening autoimmune vasculitis that is characterized by the development of pathogenic autoantibodies to type IV collagen antigens expressed in the glomerular and alveolar basement membranes. Once deposited in tissue, these autoantibodies incite a local capillaritis which manifests as rapidly progressive glomerulonephritis (GN) in 80 to 90% of patients, and with concurrent alveolar hemorrhage in ∼50%. A small proportion of cases may present with pulmonary disease in isolation. Serological testing for anti-GBM antibodies may facilitate rapid diagnosis, though renal biopsy is often required to confirm the presence of necrotizing or crescentic GN and linear deposition of autoantibody on the glomerular basement membrane. Alveolar hemorrhage may be evident clinically, or detected on imaging, pulmonary function testing, or bronchoscopy. Prompt treatment with plasmapheresis, cyclophosphamide, and steroids is usually indicated to remove pathogenic autoantibodies, to prevent their ongoing production, and to ameliorate end-organ inflammation. Alveolar hemorrhage is usually responsive to this treatment, and long-term respiratory sequelae are uncommon. Renal prognosis is more variable, though with aggressive treatment, independent renal function is maintained at 1 year in more than 80% of patients not requiring renal replacement therapy at presentation. Relapse in uncommon in anti-GBM disease, unless there is a concomitant antineutrophil cytoplasm antibody (present in 30–40%), in which case maintenance immunosuppression is recommended.
Eosinophilic Granulomatosis with Polyangiitis (Churg–Strauss)
Authors: Nguyen Yann , Guillevin Loïc 
Published: November 2018

AbstractEosinophilic granulomatosis with polyangiitis (EGPA), formerly called Churg–Strauss syndrome, is a systemic necrotizing vasculitis of small- and medium-size vessels, characterized by asthma and blood eosinophilia. EGPA typically occurs in patients with preexisting asthma, and involves the skin, lungs, and peripheral nerves. Poor-prognosis factors (i.e., involvement(s) of the gastrointestinal tract, heart, and/or kidney) have been assessed with the Five-Factor Score (FFS). One-third of the patients have anti-myeloperoxidase antineutrophil cytoplasm antibodies, and their presence seems to differentiate between two phenotypes, with different clinical characteristics and prognoses. Overall survival has improved markedly since the use of glucocorticoids and immunosuppressants, but relapse rates remain high. All patients require glucocorticoids, and for those with severe/refractory disease and FFS-defined poor prognoses, immunosuppressants should be used (cyclophosphamide for induction and azathioprine for maintenance therapy). Recent advances in EGPA management, including several novel immunomodulatory drugs and targeted biotherapies, were or are being evaluated to improve EGPA patients' prognoses.
Microscopic Polyangiitis: New Insights into Pathogenesis, Clinical Features and Therapy
Authors: Karras Alexandre 
Published: November 2018

AbstractMicroscopic polyangiitis (MPA) is one of the main clinical presentations of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides. Although the disease is defined by clinical and pathological criteria, the anti-myeloperoxidase (MPO) specificity of ANCAs is observed in almost 80% of MPA patients. The direct pathogenic role of anti-MPO antibodies has been proven in animal models, in which the disease was transmitted by transfer of anti-MPO antibodies or anti-MPO–specific splenocytes. The most frequently affected organs in this disease are the kidneys and the lungs. Necrotizing and crescentic glomerulonephritis can be revealed by rapidly progressive renal failure, but kidney injury can be more slowly progressive and lead to end-stage renal disease without major extrarenal manifestations. The most frequent pulmonary manifestation is diffuse alveolar hemorrhage, but some patients may present with chronic interstitial fibrosis leading to respiratory failure. General signs such as fever and weight loss, muscular and articular symptoms, peripheral neuropathy, and cutaneous involvement may also reveal the disease. Although the relapse rate is quite low after induction of remission, 5-year mortality is 25%, with even higher mortality rates in older patients (> 65 years old), or those with significant kidney dysfunction. Iatrogenic causes (particularly infections) are an important cause of deaths in these vulnerable patients. Future studies are warranted to determine the optimal maintenance immunosuppressive regimen to minimize side effects of immunosuppression.
Arterial Variation: Higher Bifurcation of the Brachial Artery into Radial and Ulnar Arteries in the Arm of a Male Corpse
Authors: de Oliveira Juliano , Ferreira Mônica , Cunha Marcos 
Published: November 2018

AbstractThe objective of this study was to describe a morphological variation of the bifurcation of the brachial artery, which was located at a higher level in the right arm of a male corpse. This variation was found during a dissection process performed at the anatomy laboratory of the Centro Universitário de Volta Redonda, in the state of Rio de Janeiro, Brazil, by graduates in medicine during a course of dissection conducted by the Academic League of Human Anatomy of the institution. The brachial artery was located medial in the arm, as usual, but its bifurcation into the radial and ulnar arteries happened prematurely, midway down the diaphysis. The morphological variation has previously been described by other authors; its occurrence is not well established yet, since there are articles that say it is common, and others that say it is rare. The implication of this morphological variation in medical practice is related to the measure of blood pressure in the arm; this higher bifurcation of the brachial artery can be confusing for the health provider measuring the blood pressure of a patient. This article describes a morphological variation and hopes to help to elucidate its patterns.
Survival Analysis in Elderly Patients with Glioblastomas: The Influence of Clinical Status, Tumor and Surgical Features
Authors: Sousa Ulysses , Oliveira Matheus , Heringer Lindolfo , Botelho Ricardo , Rotta José 
Published: November 2018

Introduction Glioblastomas are malignant neoplasms, notorious for their poor prognosis. We have conducted a survival analysis in a sample of elderly patients with glioblastomas.
Methods The sample of the present study consisted of elderly patients consecutively admitted from January 2014 to January 2016 (24 months) at the Hospital do Servidor Público Estadual de São Paulo. We have evaluated the impact of age, Karnofsky scale (KS) score, tumor location, and occurrence of perioperative complications.
Results A total of 42 patients were analyzed. Of these, 23 (54.7%) were men, and 19 (45.3%) were women. Patients > 60 years old, with low KS score, deep-seated tumors, and those with perioperative complications had worst outcomes.
Discussion and conclusion Surgery, perioperative chemotherapy and radiotherapy add survival time and quality of life to these patients. In patients with low KS score, isolated radiotherapy and/or chemotherapy might be adequate. Decreasing perioperative complications is essential to adequately deliver adjuvant therapy in elderly patients.
Anatomical Variation of Facial Artery Branch: A Case Report
Authors: Brasil Guilherme , Mazon Josete 
Published: November 2018

Introduction The facial artery and its branches are the major vessels that supply blood to the face region. This artery and its branches can present variations in path and branching pattern and thus complicate the location of these arteries during invasive procedures. There is still a great need to inform and clarify the variant or unusual organization of the display of these arteries.
Case Report During the dissection of the head and neck region of a cadaver, an anomalous branch of the unilateral facial artery was observed in the superior labial artery.
Conclusion The lack of knowledge about the possible pathways of the facial artery and its branches can lead to errors in surgical procedures or fillers, causing severe complications to the facial structures.
The geographic mosaic of coevolution in mutualistic networks
Authors: Medeiros Lucas P. , Garcia Guilherme , Thompson John N. , Guimarães Paulo R. 
Published: November 2018

Significance
The reciprocal evolution of interacting species, or coevolution, generates impressive adaptations in pairs of species across geographic regions. However, we currently do not understand how coevolution shapes adaptations in large groups of species that interact not just locally but also across ecosystems. We use a mathematical model of coevolution and network tools to show that gene flow resulting from movement of individuals among populations may favor, rather than swamp, reciprocal adaptation in mutualisms, especially in large and heterogeneous networks typical of pollination and seed dispersal interactions. Our results suggest that the disruption of gene flow, fueled by human activities, may undermine long-term adaptation in mutualistic assemblages, with severe consequences for the functioning of ecological systems.
A flexible organic reflectance oximeter array
Authors: Khan Yasser , Han Donggeon , Pierre Adrien , Ting Jonathan , Wang Xingchun , Lochner Claire M. , Bovo Gianluca , Yaacobi-Gross Nir , Newsome Chris , Wilson Richard , Arias Ana C. 
Published: November 2018

Significance
The optical method to determine oxygen saturation in blood is limited to only tissues that can be transilluminated. The status quo provides a single-point measurement and lacks 2D oxygenation mapping capability. We use organic printed optoelectronics in a flexible array configuration that senses reflected light from tissue. Our reflectance oximeter is used beyond conventional sensing locations and accurately measures oxygen saturation on the forehead. In a full system implementation, coupled with a mathematical model, we create 2D oxygenation maps of adult forearms under pressure-cuff–induced ischemia. Our skin-like flexible sensor system has the potential to transform oxygenation monitoring of tissues, wounds, skin grafts, and transplanted organs.
Paraconsistency, self-extensionality, modality
Authors: Avron Arnon , Zamansky Anna 
Published: November 2018

Abstract
Paraconsistent logics are logics that, in contrast to classical and intuitionistic logic, do not trivialize inconsistent theories. In this paper we take a paraconsistent view on two famous modal logics: B and S5. We use for this a well-known general method for turning modal logics to paraconsistent logics by defining a new (paraconsistent) negation as $\neg \varphi =_{Def} \sim \Box \varphi$ (where $\sim$ is the classical negation). We show that while that makes both B and S5 members of the well-studied family of paraconsistent C-systems, they differ from most other C-systems in having the important replacement property (which means that equivalence of formulas implies their congruence). We further show that B is a very robust C-system in the sense that almost any axiom which has been considered in the context of C-systems is either already a theorem of B or its addition to B leads to a logic that is no longer paraconsistent. There is exactly one notable exception, and the result of adding this exception to B leads to the other logic studied here, S5.
Neocucurbitaria keratinophila: An emerging opportunistic fungus causing superficial mycosis in Spain
Authors: Valenzuela-Lopez Nicomedes , Cano-Lira José F , Stchigel Alberto M , Rivero-Menendez Olga , Alastruey-Izquierdo Ana , Guarro Josep 
Published: November 2018

Abstract
Although there have been few reports of opportunistic infections (superficial and systemic) caused by coelomycetous fungi, they are becoming more frequent. Neocucurbitaria keratinophila (formerly Pyrenochaeta keratinophila), characterized by producing pycnidial conidiomata and small hyaline conidia, seems to be an emergent opportunistic pathogen in Spain. Since this fungus was first reported from human keratitis, eight strains have been isolates from clinical cases in Spain. This is a retrospective study of these fungal strains, including phenotypic and molecular characterizations, and in vitro antifungal susceptibility assays. These clinical strains were identified by sequencing four phylogenetic markers such as the internal transcribed spacer region (ITS1-5.8S-ITS2) and fragments of the 28S nrRNA (LSU), beta-tubulin (tub2), and RNA polymerase II subunit 2 (rpb2) genes, and by morphological characterization. All the strains tested were susceptible to the majority of antifungals, being isavuconazole the only drug that showed a poor antifungal activity.
Unintended specificity of an engineered ligand-binding protein facilitated by unpredicted plasticity of the protein fold
Authors: Day Austin L , Greisen Per , Doyle Lindsey , Schena Alberto , Stella Nephi , Johnsson Kai , Baker David , Stoddard Barry 
Published: November 2018

Abstract
Attempts to create novel ligand-binding proteins often focus on formation of a binding pocket with shape complementarity against the desired ligand (particularly for compounds that lack distinct polar moieties). Although designed proteins often exhibit binding of the desired ligand, in some cases they display unintended recognition behavior. One such designed protein, that was originally intended to bind tetrahydrocannabinol (THC), was found instead to display binding of 25-hydroxy-cholecalciferol (25-D3) and was subjected to biochemical characterization, further selections for enhanced 25-D3 binding affinity and crystallographic analyses. The deviation in specificity is due in part to unexpected altertion of its conformation, corresponding to a significant change of the orientation of an α-helix and an equally large movement of a loop, both of which flank the designed ligand-binding pocket. Those changes led to engineered protein constructs that exhibit significantly more contacts and complementarity towards the 25-D3 ligand than the initial designed protein had been predicted to form towards its intended THC ligand. Molecular dynamics simulations imply that the initial computationally designed mutations may contribute to the movement of the helix. These analyses collectively indicate that accurate prediction and control of backbone dynamics conformation, through a combination of improved conformational sampling and/or de novo structure design, represents a key area of further development for the design and optimization of engineered ligand-binding proteins.
Pharmacological Activation of PXR and CAR Downregulates Distinct Bile Acid-Metabolizing Intestinal Bacteria and Alters Bile Acid Homeostasis
Authors: Dempsey Joseph L , Wang Dongfang , Siginir Gunseli , Fei Qiang , Raftery Daniel , Gu Haiwei , Yue Cui Julia 
Published: November 2018

AbstractThe gut microbiome regulates important host metabolic pathways including xenobiotic metabolism and intermediary metabolism, such as the conversion of primary bile acids (BAs) into secondary BAs. The nuclear receptors pregnane X receptor (PXR) and constitutive androstane receptor (CAR) are well-known regulators for xenobiotic biotransformation in liver. However, little is known regarding the potential effects of PXR and CAR on the composition and function of the gut microbiome. To test our hypothesis that activation of PXR and CAR regulates gut microbiota and secondary BA synthesis, 9-week-old male conventional and germ-free mice were orally gavaged with corn oil, PXR agonist PCN (75 mg/kg), or CAR agonist TCPOBOP (3 mg/kg) once daily for 4 days. PCN and TCPOBOP decreased two taxa in the Bifidobacterium genus, which corresponded with decreased gene abundance of the BA-deconjugating enzyme bile salt hydrolase. In liver and small intestinal content of germ-free mice, there was a TCPOBOP-mediated increase in total, primary, and conjugated BAs corresponding with increased Cyp7a1 mRNA. Bifidobacterium, Dorea, Peptociccaceae, Anaeroplasma, and Ruminococcus positively correlated with T-UDCA in LIC, but negatively correlated with T-CDCA in serum. In conclusion, PXR and CAR activation downregulates BA-metabolizing bacteria in the intestine and modulates BA homeostasis in a gut microbiota-dependent manner.
Satisfactory outcomes after gradual deformity correction of delayed onset Blount disease using the Ilizarov device
Authors: Fahmy Fahmy Samir , Fathi Hossam 
Published: November 2018

Background:
Blount disease is a developmental disorder affecting the posteromedial part of the upper tibial growth plate resulting in complex knee deformity. Surgical correction of such deformity is challenging for orthopaedic surgeons. The goal of this study was to evaluate the results of gradual deformity correction of delayed-onset Blount disease using the Ilizarov device.

Methods:
Sixteen knees in 13 patients with delayed-onset Blount disease were operated for gradual deformity correction using the Ilizarov fixator at Zagazig University Hospital between June 2013 and May 2017. Clinical (tibial torsion, shortening, and range of knee motion) and radiographic parameters (tibiofemoral angle, mechanical axis deviation, and medial proximal tibial angle) were used for evaluation. A P-value of <0.05 was statistically significant.

Results:
The mean follow-up period was 24.4±7.1 mo. All patients were satisfied with the final range of knee motion, with a mean of 125±8.1 degrees of flexion. Internal tibial torsion and limb-length discrepancy were significantly improved at the end of the follow-up. There was a statistically significant difference between the preoperative and the final mean of the radiographic measurements. Nine patients had pin track infection, and one patient had common peroneal nerve neurapraxia that completely resolved.

Conclusions:
Gradual deformity correction of delayed onset Blount disease using the Ilizarov device had satisfactory clinical and radiographic outcomes with a low incidence of serious surgical complications.