bluuebunny/crossref_metadata_2025_split huggingface dataset:
The Mode of Action of Heparin in Plasma
Authors: Béguin Suzette , Lindhout Theo , Hemker H Coenraad 
Published: July 2018

SummaryThe influence of heparin on prothrombin conversion in plasma was estimated by measuring prothrombin disappearance with the aid of staphylocoagulase or by calculation from the thrombin generation curve, compensating for simultaneous thrombin inactivation by plasma protease inhibitors. In thromboplastin-activated plasma the effect of heparin on prothrombinase (factor Xa-factor Va-phospholipid) is negligible compared to that on thrombin probably because both the extrinsic factor X activating complex and phospholipid bound factor Xa are insensitive to AT III-heparin action. In contact-activated plasma prothrombinase generation is inhibited by heparin, because heparin lowers the ambient concentrations of thrombin so that the feedback activation of factor VIII by thrombin is diminished. Consequently, the delayed factor Xa generation causes a lag phase in prothrombinase generation. We conclude that heparin in plasma acts by its thrombin scavenging properties. No direct action on prothrombinase or on the factor X activating enzyme needs to be assumed if one takes into account the effect of thrombin depletion on thrombin-mediated feedback reactions
Antithrombin Milano, Single Amino Acid Substitution at the Reactive Site, Arg393 to Cys
Authors: Erdjument H , Lane D A , Ireland H , Di Marzo V , Panico M , Morris H R , Tripodi A , Mannucci P M 
Published: July 2018

SummaryAntithrombin Milano is an unusual antithrombin variant, exhibiting an abnormal, fast moving component on crossed immunoelectrophoresis (in the absence of heparin). Antithrombin isolated from the propositus could be resolved into two peaks on anion-exchange chromatography; anti thrombin Milano peak 1 of Mr ∼60,000 which could inhibit thrombin, and antithrombin Milano peak 2 of Mr ∼120,000 which was inactive. The latter component also reacted with antisera to both antithrombin and albumin on immunoblotting. Under reducing conditions, the ∼120,000 Mr component migrated on SDS-PAGE as two distinct bands with Mr ∼60,000, one of which reacted with antiserum to antithrombin and the other (of slower mobility) of which reacted with antiserum to albumin only. These and other results established the ∼120,000 Mr component to be an inactive, disulphide-linked variant antithrombin and albumin complex. The variant antithrombin was isolated, following reduction and S-carboxy-methylation, by reverse-phase HPLC and then it was fragmented with CNBr. A major CNBr pool containing the sequence Gly339-Met423 was treated with trypsin, followed by V8 protease. The resulting peptides were analysed by fast atom bombardment mass spectrometry (Fab-MS) mapping. A peptide of molecular mass 1086, corresponding to the normal sequence Ala382-Arg393, was almost absent from the mass spectrum, but an additional peptide of mass number 1772 was present. These results are almost identical to those found in another variant antithrombin, North-wick Park (Erdjument et al., J Biol Chem, 262: 13381, 1987; Erdjument et al., J Biol Chem, 263: 5589-5593, 1988), indicating the same single amino acid substitution of Arg393 to Cys.
Normal Plasmic Cleavage of the γ-Chain Variant of “Fibrinogen Saga” with an Arg–275 to His Substitution
Authors: Yamazumi Kensuke , Matsuda Michio , Terukina Shigeharu , Onohara Shingo 
Published: July 2018

SummaryWe have identified a γ-Arg–275 to His substitution in an abnormal fibrinogen designated as “fibrinogen Saga” characterized by impaired fibrin monomer polymerization. By chromatofocusing chromatography, we isolated normal and abnormal fragment Dl populations separately from the plasmic-calcium digests of fibrinogen derived from the propositus, a heterozygote for the abnormality. We found that both normal and abnormal fragment D1’s were similarly protected from digestion by plasmin in the presence of calcium ions and further degraded to fragments D2 and D3 due to cleavage of the γ-chain remnant when calcium ions were replaced by chelating agents. Abnormal fragment D1 failed to inhibit both thrombin-clotting of normal fibrinogen and polymerization of normal fibrin monomer, while normal D1 exhibited marked inhibitory activities. In an aberrant peptide comprising residues γ–274–302 isolated by HPLC from the lysyl endopeptidase-digests of abnormal fragment D1, we identified a His substituting for an Arg at position 2, which corresponds to position 275 of the mutant γ-chain
5HT-Kinetics and Sensitivity of Human Blood Platelets: Variations with Age, Gender and Platelet Number
Authors: Pletscher Alfred , Fetkovska Natalia , Amstein Ruth , Ferracin Fabrizia , Regenass Martin , BÜhler Fritz R 
Published: July 2018

SummaryPlatelet shape change and aggregation as well as serotonin (5–hydroxytryptamine; 5HT) content in platelets, spontaneous release of 5HT, 5HT plasma levels, urinary excretion of 5–hydroxyindoleacetic acid (5HIAA) and plasma (3–thromboglobulin (β–TG) were investigated in 45 healthy subjects (27 men, 18 women). Platelets from women were more susceptible to aggregation (both by 5HT and ADP) than platelets from men. However, in men, 5HT-induced shape change and aggregation as well as plasma β–TG concentration increased with age. In men, 5HT–induced platelet aggregation correlated positively with 5HT plasma levels. An inverse relationship was found in men between platelet number on the one hand and platelet 5HT content, 5HT release, 5HT plasma levels and urinary 5HIAA excretion rates on the other hand. In all subjects 5HT-induced platelet aggregation correlated negatively with platelet numberThese results indicate that age and gender must be considered in designing clinical studies on 5HT and in evaluating human platelet 5HT kinetics. The platelet number seems to be related to 5HT kinetics of platelets and their reaction to 5HT in men. An age-dependent change in the reactivity of platelets to 5HT (rather than their absolute 5HT sensitivity) may contribute to the enhanced platelet turnover and higher incidence of cardiac events in older men
Percutaneous Instrumentation of a Complex Lumbar Spine Fracture with Bilateral Pedicle Dissociation: Case Report and Technical Note
Authors: Urakov Timur , Vanni Steven , Luther Evan 
Published: July 2018

Background and Study Objective Complex traumatic lumbar spine fractures are difficult to manage and typically occur in younger patients. Surgical immobilization for unstable fractures is an accepted treatment but can lead to future adjacent-level disease. Furthermore, large variations in fracture morphology create significant difficulties when attempting fixation. Therefore, a surgical approach that considers both long-term outcomes and fracture type is of utmost importance. We present a novel technique for percutaneous fixation without interbody or posterolateral fusion in a young patient with bilateral pedicle dissociations and an acute-onset incomplete neurologic deficit.
Case Description A 20-year-old man involved in a motorcycle accident presented with unilateral right lower extremity paresis and sensory loss with intact rectal tone and no saddle anesthesia. Lumbar computed tomography (CT) demonstrated L2 and L3 fractures associated with bilateral pedicle dislocations. Lumbar magnetic resonance imaging showed draping of the conus medullaris/cauda equina anteriorly over the kyphotic deformity at L2 with minimal associated canal stenosis at L2 and L3. He was treated with emergent percutaneous fixation of the fracture segment without interbody or posterolateral fusion. Decompression was not performed because of the negligible amount of canal stenosis and high likelihood of cerebrospinal fluid leakage due to dural tears from the fractures. Surgical fixation of the L2 vertebra was achieved by cannulating the left pedicle with an oversized tap while holding the right pedicle in place with a normal tap and then driving screws into the left and right pedicles, respectively, thus reducing the free-floating fracture segment. At 18 months after surgery, a follow-up CT demonstrated good cortication across the prior pedicle fractures, and the instrumentation was removed without any obvious signs of instability or disruption of the alignment at the thoracolumbar junction.
Conclusion We present a novel technique for percutaneous reduction and fixation of bilateral pedicle fractures with significant dissociation from the vertebral body, associated neural compression from the kyphotic deformity, and minimal spinal canal stenosis. Furthermore, we argue that early fixation and reduction of the fracture prevented irreversible neurologic compromise, and the absence of interbody or posterolateral fusion ultimately preserved the spinal mobility of the patient once the hardware was removed.
Sporadic Schwannomatosis: A Systematic Review Following the 2005 Consensus Statement
Authors: Victor Jan , Poujade Thibault , Hollevoet Nadine , Chick Gregoire 
Published: July 2018

Aims To identify the frequency of reports of sporadic schwannomatosis, the types of patients affected, and the nerves affected.
Patients and Methods We identified all case reports and case series that reported on patients with sporadic schwannomatosis according to established criteria.
Results The initial search yielded 1,597 studies, of which 15 were included. A total of 38 of 55 individuals met the inclusion criteria. The mean age of the patients was 48 years; 41% were male. Thirty-three patients had peripheral nerve tumors, and 17 had spinal tumors. Twelve had tumors in both locations. Tumor distribution was unilateral in 25 of 30 cases (83.3%) and segmental (limited to one limb or five or fewer contiguous segments of the spine) in 28 of 38 cases (73.7%).
Conclusion This systematic review quantified the number of individuals who meet the criteria for sporadic schwannomatosis and better described this population to facilitate the classification of neurofibromatosis in regard to the 2005 consensus statement. Unilateral or segmental distribution of nerve tumors are key aspects when dealing with multiple noncutaneous schwannomas without involvement of the vestibular nerve.
Calvian Endo-pen: New Coagulation Forceps for Endoscopic Endonasal Transsphenoidal Surgery
Authors: Rosahl Steffen , Kellner Geralf , Gerlach Rüdiger 
Published: July 2018

Objective Over the past few years bipolar electrocoagulation techniques in neurosurgery have been continually improving. However, limited access during endoscopic endonasal transsphenoidal surgery (EETS) for central skull base pathologies and the requirement of very precise coagulation in that dedicated anatomical area requires further refinement of bipolar coagulation instruments. We describe our experience (effectiveness of coagulation, intraoperative handling, and the use as a dissecting tool) with a new type of coagulation forceps, the Calvian endo-pen (Sutter Medizintechnik, Freiburg, Germany) during EETS.
Method From June to August 2015, 12 patients with central skull base lesions (9 with a pituitary adenoma and 1 each with epidermoid, hemangioma, and juvenile angiofibroma) were operated on with the Calvian endo-pen.
Results The application of the Calvian endo-pen was feasible in all cases. The angled thin tips proved to be very effective and precise for soft tissue coagulation to achieve hemostasis. Even very small vessels could be occluded selectively. It was also helpful for outward dissection in separating normal from tumor tissue.
Conclusion The use of the Calvian endo-pen is safe and effective during EETS for central skull base pathologies. Its ease in intraoperative handling (maneuverability, cleaning) and precise coagulation makes it a promising instrument for EETS.
Persistent Iliosacral Joint Syndrome following Instrumentation to the Sacropelvis in Patients with Adult Spinal Deformity
Authors: Diesing Daniela , Franke Joerg , Tschoeke Sven , Schultheiß Rolf , Scheufler Kai 
Published: July 2018

Background and Objective Persistent sacroiliac joint syndrome (PSIJS) may complicate adult spinal deformity surgery (ASDS). This study assesses the relationship between clinical/morphometric parameters and PSIJS following ASDS including pelvic fixation and the therapeutic efficacy of secondary iliosacral fusion (ISF).
Methods Perioperative health-related quality of life (HRQOL) outcomes (Oswestry Disability Index, Short Form 12-item health survey, version 2 scores) at 6, 12, and 24 months, and radiographic studies were analyzed retrospectively in a cohort of 71 consecutive patients undergoing ASDS. PSIJS was confirmed in nine individuals (12.7%) by placebo-controlled dual sacroiliac joint (SIJ) blocks. The relationships between global and regional spinopelvic morphometry, PSIJS, and HRQOL outcomes were assessed by logistic regression and receiver operating characteristic curve (ROC) analysis.
Results PSIJS, independently causing significantly reduced improvement in HRQOL scores (p < 0.001) 6 months postoperatively, warranted secondary ISF in nine patients (12.7%) within 12 months of index surgery, without evidence of progressive SIJ arthrosis, pseudarthrosis, or hardware issues. Eight of nine patients undergoing secondary ISF reported≥ 70% pain reduction at 24 months. Logistic regression/ROC analysis revealed a close association between PSIJS and nonharmonious postoperative L4–S1 fractional lordosis (p < 0.0001), pelvic incidence angle > 53 degrees, hip arthrosis, and preexistent advanced SIJ arthrosis (p < 0.01).
Conclusion PSIJS may negatively impact the clinical outcome of ASDS. Recurrent preoperative SIJ syndrome requiring interventional treatment, preexisting hip and SIJ arthrosis, insufficient restoration of L4–S1 fractional lordosis, and high pelvic incidence predispose to PSIJS. PSIJS may potentially be avoided by restoring physiologic lumbosacral geometry and S2 sacral alar-iliac screw fixation during index surgery. Secondary ISF appears to be effective in reducing pain and physical impairment due to PSIJS.
Metastasis from Intracranial Mesenchymal Chondrosarcoma: Report of a Rare Case
Authors: Singh Daljit , Saran Ravindra , Singh Hukum , Srivastava Arvind , Jagetia Anita , Dutta Gautam 
Published: July 2018

AbstractChondrosarcoma is a rare malignant tumor originating from cartilaginous tissue with a tendency to localize in the epiphysis of long and pelvic bones. Only 7% of all chondrosarcomas originate in the craniocervical region. Metastasis from intracranial chondrosarcoma is extremely rare with only two previously reported cases. We report on a young patient with intracranial chondrosarcoma who presented with extracranial metastasis 2 years after surgical excision of the primary lesion. Gross total excision combined with radiotherapy so far has led to a favorable outcome.
Langerhans Cell Histiocytosis of the Adult Cervical Spine: A Case Report and Literature Review
Authors: Hewer Ekkehard , Ulrich Christian , Schär Ralph 
Published: July 2018

AbstractA 36-year-old man was diagnosed with Langerhans cell histiocytosis (LCH) of the cervical spine with a unifocal expansive osteolytic lesion of C4. The surgical management with a 2-year follow-up and a review of the literature on LCH of the cervical spine are presented. Although a rare condition, LCH is an important differential diagnosis of any osteolytic lesion in the cervical spine with localized pain in a young adult patient. Review of the literature suggests a higher prevalence of LCH lesions affecting the cervical spine as compared with the thoracic or lumbar spine than historically reported.
Vertebral Arteriovenous Fistula: A Rare Complication Following Transpedicular Occipitocervical Fixation in a Patient with Atlantoaxial Dislocation
Authors: Li Zhixiao , Li Ming , Qian Rongjun 
Published: July 2018

AbstractVertebral arteriovenous fistula (AVF), a complication of a vertebral artery injury (VAI), is a rare but serious complication of upper cervical spine fixation surgery. We report a case of a 59-year-old female patient who had a vertebral AVF following transpedicular occipitocervical fixation surgery. Endovascular embolization of the AVF was successfully performed using ethylene vinyl alcohol. From this case we learned that preoperative evaluation of the course of the vertebral artery is necessary, and vertebral artery embolism is an effective and safe method to treat vertebral AVF after proof of a patent second vertebral artery.
Mpl-Ligand and the Regulation of Megakaryocytopoiesis
Authors: Debili Najet , Villeval J.-L. , Vainchenker W. , Wendling Françoise , Cohen-Solal Karine 
Published: July 2018

SummaryAfter 35 years of research, the physiological regulator of platelet production has been isolated and its gene cloned. This discovery originates from studies performed with the myeloproliferative leukaemia virus (MPLV), a murine retrovirus which induces an acute myeloproliferative syndrome in adult mice. MPLV carries the v-mpl oncogene in its genome. This oncogene corresponds to a truncated form of the c-mpl proto-oncogene. c-mpl encodes a cytokine receptor (Mpl-R) belonging to the hematopoietin receptor superfamily. Among the hematopoietic cell lineages, Mpl-R is preferentially expressed on late MK progenitors, megakaryocytes and platelets. The ligand for Mpl-R, called Mpl-L, thrombopoietin (TPO), megakaryocyte growth and development factor (MGDF) or megapoietin, is a gly-cosylated hormone of 322 amino acids in man which comprises two domains; the N-terminus domain shares 50% similarity with erythropoietin and is respon-sible for the biological activity; the C-terminus part is required for secretion. In vitro Mpl-L is capable of directly inducing both the proliferation of MK progenitors and the terminal maturation of megakaryocytes. MK grown in vitro in presence of Mpl-L can shed platelets. Notwithstanding its major action on megakaryocytopoiesis and thrombocytopoiesis, Mpl-L also potentiates the action of other cytokines on early hematopoietic progenitors. The plasma level of Mpl-L inversely correlates with the platelet and MK mass. However, in the two major organs (liver and kidney) which synthesize Mpl-L, there is no regulation at the transcription level of the synthesis of Mpl-L by the platelet demand, suggesting that regulation of the plasma level occurs by uptake and consumption at the level of platelets. A deregulation of Mpl-L synthesis obtained by infecting, murine hematopoietic stem cells by a retrovirus containing the coding sequence for Mpl-L induces a disease which has many common features with idiopathic myelofibrosis in man. Therefore, Mpl-L might be a very useful therapeutical cytokine to treat thrombocytopenia, but these data emphasize that chronic exposure to Mpl-L may lead to a severe disease. Thus, human clinical trials will require well controlled administration protocols.
Spontane Faktor-VIII-Inhibitoren
Authors: Lechner K. , Huhmann Iris 
Published: July 2018

ZusammenfassungAls spontane (nichthämophile) Faktor-Vlll-lnhibitoren werden Antikörper gegen Faktor VIII bei zuvor gerinnungsnormalen Patienten bezeichnet. Meist ist der F-Vlll-Mangel schwer und dementsprechend die Blutungsneigung ausgeprägt. Die Pathogenese ist heterogen. Laborchemisch findet sich typischerweise eine verlängerte aPTT und ein isolierter F-Vlll-Mangel. Die Antikörper sind polyklonal und zumeist Typ-Il-Inhibitoren. Die Therapie gliedert sich in die Beherrschung der akuten Blutung (hochkonzentrierte humane F-Vlll-Konzentrate, porciner F VIII, aktivierte Prothrombinkomplexpräparate, rekombinanter F Vlla, evtl. zusätzlich Plasmapherese und Immunadsorption - zur Reduktion des Inhibitors) und in die Langzeitreduktion des Titers mit Immunsuppressiva.
Erworbene Faktor-V-Inhibitoren
Authors: Tribl B. , Lechner K. , Knöbl P. 
Published: July 2018

ZusammenfassungErworbene Faktor-V-Inhibitoren sind eine seltene Gerinnungsstörung. Weltweit wurden bisher 72 Fälle publiziert. Die Inzidenz scheint jedoch zuzunehmen, was wahrscheinlich auf den gehäuften Einsatz von Fibrinklebern zurückzuführen ist. Diese Metaanalyse untersucht die prädiponierenden Faktoren, die Pathogenese, diagnostische Methoden, klinisches Erscheinungsbild, therapeutische Möglichkeiten und den langfristigen Verlauf aller bisher publizierten Fälle von Faktor-V-Inhibitoren.
Therapieergebnisse der chronischen Autoimmunthrombozytopenie bei Erwachsenen
Authors: Barz D. , Reinhardt K. M. , Freund M. , Anders O. 
Published: July 2018

ZusammmenfassungIn einer retrospektiven Untersuchung wurden die klinischen Befunde und der Therapieerfolg von 131 Patienten mit chronischer Autoimmunthrombozytopenie (97 Frauen, Altersmedian 29 Jahre, und 34 Männer, Altersmedian 32 Jahre, Range 20 bis 80 Jahre), die in einem Zeitraum von 36 Jahren (1960 bis 1995) be-handelt wurden, ermittelt. Es wurden die therapeutische Ansprechrate, die Re-misssionsrate und die Langzeiterfolgsrate nach verschiedenen Therapieregimen der chronischen Autoimmunthrombozytopenie erfaßt.Die Splenektomie ergab die besten Ergebnisse mit einer Ansprechrate von 90% und einer Langzeiterfolgsrate von 74%. Die Ansprechrate und die Langzeiterfolgsrate betrug nach der Behandlung mit Prednisolon 69% und 16%, nach Azathioprin 52% und 27%, nach Vinchstin 36% und 10%. Mit Ascorbinsäure wurde keine Remission erzielt. Nach der Therapie mit Prednisolon, nach Splenektomie und nach immunsuppressiver Behandlung erreichten 110 Patienten (84%) eine Remission. 21 Patienten (16%) sprachen nicht mit einem dauerhaften Thrombozytenanstieg an. Die Therapie der refraktären chronischen Autoimmunthrombozytopenie mit Blutungskomplikationen ist heute noch ein therapeutisch mitunter schwierig zu lösendes Problem.
Gene Polymorphisms in FAS (Rs3740286 and Rs4064) Are Involved in Endometriosis Development in Brazilian Women, but not those in CASP8 (rs13416436 and rs2037815)
Authors: Pissetti Cristina Wide , Tanaka Sarah Cristina Sato Vaz , Hortolani Andrezza Cristina Cancian , Marqui Alessandra Bernadete Trovó de 
Published: July 2018

Objective The present study aims to investigate the association between caspase-8 (CASP8) (rs13416436 and rs2037815) and Fas cell surface death receptor (FAS) (rs3740286 and rs4064) polymorphisms with endometriosis in Brazilian women.
Methods In the present case-control study, 45 women with a diagnosis of endometriosis and 78 normal healthy women as a control group were included. The genotyping was determined by real-time polymerase chain reaction (PCR) with Taqman hydrolysis probes (Thermo Fisher Scientific, Darmstadt, Germany). Genotypic and allelic frequencies were analyzed using Chi-squared (χ2) test. In order to determine the inheritance models and haplotypes ,SNPStats (Institut Català d'Oncologia, Barcelona, Spain) was used. Levels of 5% (p = 0.05) were considered statistically significant.
Results No significant difference was observed in genotypic or allelic frequencies between control and endometriosis groups for rs13416436 and rs2037815 (CASP8 gene). On the other hand, a significant difference between rs3740286 and rs4064 (FAS gene) was found. Regarding polymorphisms in the FAS gene, a statistically significant difference was found in co-dominant and dominant models. Only the haplotype containing the rs3740286A and rs4064G alleles in the FAS gene were statistically significant.
Conclusion The polymorphisms in the CASP8 gene were not associated with endometriosis. The results indicate an association between FAS gene polymorphisms and the risk of developing endometriosis.