Chronic myeloproliferative disorders such as polycythemia vera (PV), essential thrombocytosis (ET), and idiopathic myelofibrosis arise from clonal proliferation of neoplastic stem cells in the bone marrow. Matrix metalloproteinases (MMPs) are a family of zinc-dependent endopeptidases that have potential to degrade all types of extracellular matrix (ECM) and also play a role in remodeling of the ECM. It is known that MMPs play a role in bone marrow remodeling.The primary goal of our study is to explore the relationship between chronic myeloproliferative diseases and some of MMP gene polymorphisms. The demonstration of a relationship will help to understand whether these polymorphisms may be a potential early diagnosis marker of the diseases.Patients were selected from outpatient clinics of Turgut Ozal University Hospital, Ankara, Turkey, between December 2010 and May 2011. Twenty-eight patients that previously diagnosed and followed-up with PV, 17 with secondary polycythemia (SP), and 12 with ET were enrolled in the study, along with a control group of 22 healthy people.DNA was isolated from peripheral blood. Using polymerase chain reaction–restriction fragment length polymorphism method, MMP2 and MMP9 gene polymorphisms were analyzed with agarose gel electrophoresis. There was a statistically significant difference between the study groups and the control group in terms of Gln279Arg polymorphisms rates of MMP9. The highest MMP9 Gln279Arg polymorphism rate was observed in the ET group. But nobody from the control group had polymorphic MMP9. There was no statistically significant difference between the groups in terms of MMP2-735 C > T polymorphism rates.In conclusion, MMP9 gene Gln279Arg polymorphism was associated with ET, SP, and PV diseases. Hence, we believe that these gene polymorphisms may play a role in the mechanism of bone marrow fibrosis and may be a factor that increases the risk of thrombosis. Illumination of the molecular basis of the relationship between MMP-thrombosis and MMP-fibrosis provides a better understanding of the pathophysiology of PV and ET diseases and will allow new approaches to diagnosis and treatment. 相似文献
Fibrodysplasia ossificans progressiva (FOP) is a rare genetic disorder whose most debilitating pathology is progressive and cumulative heterotopic ossification (HO) of skeletal muscles, ligaments, tendons, and fascia. FOP is caused by mutations in the type I BMP receptor gene ACVR1, which enable ACVR1 to utilize its natural antagonist, activin A, as an agonistic ligand. The physiological relevance of this property is underscored by the fact that HO in FOP is exquisitely dependent on activation of FOP-mutant ACVR1 by activin A, an effect countered by inhibition of anti–activin A via monoclonal antibody treatment. Hence, we surmised that anti-ACVR1 antibodies that block activation of ACVR1 by ligands should also inhibit HO in FOP and provide an additional therapeutic option for this condition. Therefore, we generated anti-ACVR1 monoclonal antibodies that block ACVR1’s activation by its ligands. Surprisingly, in vivo, these anti-ACVR1 antibodies stimulated HO and activated signaling of FOP-mutant ACVR1. This property was restricted to FOP-mutant ACVR1 and resulted from anti-ACVR1 antibody–mediated dimerization of ACVR1. Conversely, wild-type ACVR1 was inhibited by anti-ACVR1 antibodies. These results uncover an additional property of FOP-mutant ACVR1 and indicate that anti-ACVR1 antibodies should not be considered as therapeutics for FOP. 相似文献
Objective: Respiratory syncytial virus (RSV) lower respiratory tract infection is the most common viral infection in childhood. RSV-infected infants demonstrate various radiographic findings. The aim of the study is to evaluate whether assessment of radiological characteristics of lower tract infection due to RSV may be a predictor of clinical outcome in newborns.
Methods: The study included 36 newborn infants hospitalized for lower tract infection. In order to detect RSV, RSV Respi-Strip (Coris Bioconcept Organization) test kits were used on admission. Chest X-rays and clinical characteristics of the study group were reviewed.
Results: Of 36 patients hospitalized for lower tract infection from October 2012 to April 2013, 18 (50%) newborns were infected with RSV. The study included 36 neonates. Patients with RSV-positive infants at admission had greater need for respiratory support, supplemental oxygen and prolonged stay in the NICU. Newborns with an atelectasis pattern on admission chest radiograph had greater at RSV-positive infants.
Conclusion: Chest radiological patterns with lower respiratory tract infection in newborn infants due to RSV are a predictor of clinical outcome. 相似文献
Hemophilia is a hereditary disease with impaired blood coagulation due to a genetic deficiency of blood coagulation factors. The development of inhibitors further complicates the course of the disease and management. The case is here reported of a haemophilia patient who presented with coexisting development of high titer inhibitor with Gastrointestinal Stromal Tumor (GIST) diagnosis and was admitted with upper gastrointestinal system bleeding. The patient had no prior history of inhibitor presence. During all procedures including surgery, excellent hemostasis was achieved with rFVIIa treatment and no hemorrhagic complication was observed. To the best of our knowledge, this constitutes the first reported case of GIST associated with inhibitor development in a hemophilia A patient. 相似文献
[Purpose] The application of kinesio taping may improve strength and performance, inhibit
and facilitate motor activity, and increase range of motion. The aim of this study was to
compare the effects of kinesio taping facilitation and inhibition applications on
spatiotemporal knee kinematics and kinetics during walking activity in healthy subjects.
[Subjects and Methods] A three-dimensional quantitative gait evaluation was performed
without tape and with, facilitation and inhibition kinesio taping application on the knee.
Sixteen healthy female college students (age, 19.7 ± 0.4 years; height, 1.64 ± 3.4 cm;
body mass, 51.5 ± 4.8 kg) participated in the study. [Results] Spatiotemporal parameters
(cadence, walking speed, stride length) were significantly different among the trials.
Knee joint sagittal plane range of motion was not different with either kinesio taping
application. Knee external flexion moment during the early stance phase decreased
significantly with facilitation kinesio taping and increased with the inhibition kinesio
taping. Knee external extension moment during the mid-stance phase increased with
facilitation kinesio taping. Knee power parameters, eccentric activity in the rectus
femoris during the pre-swing phase was significantly increased with inhibition kinesio
taping application, while eccentric activity of the hamstrings during the terminal swing
of gait was decreased. [Conclusion] These findings showed that facilitation kinesio taping
application affected the terminal stance phase and that inhibition kinesio taping
influenced the terminal swing phase compared with the no tape condition.Key words: Kinesio tape, Knee joint, Gait analysis相似文献
This study evaluated the root canal seal achieved by irradiation with an erbium, chromium:yttrium‐scandium‐gallium‐garnet laser, and the optimal output power to remove debris and the smear layer were determined. One hundred mandibular premolar teeth were prepared and divided into four groups. Group 1 was not lased but was irrigated with 5 mL of 5.25% NaOCl and 5 mL of 17% ethylenediaminetetraacetic acid. Group 2 was irradiated at a panel setting of 1 W, group 3 at 2 W and group 4 at 2.5 W, with a 50% water level and 48% air‐cooling level. Root canals were obturated by cold lateral compaction, and apical microleakage was measured using a fluid filtration model. The remaining debris and smear layer were evaluated via scanning electron microscopy. Statistically significant differences were detected between groups. Irradiation at 1 and 2 W using an erbium, chromium:yttrium‐scandium‐gallium‐garnet laser produced a seal superior to that of the other treatments. 相似文献