Factor V11 deficiency: A rare cause of nasal bleeding

Factor VII deficiency is a rare inherited disorder. Clinically the patient presents with bleeding tendencies. Diagnosis is made by prolonged prothrombin time, normal activated partial thromboplastin time and low functions factor VII assay or factor VII antigen. Therapy involves factor VII concentrates, recombinant factor VII, fresh frozen plasma and fibrinolytic inhibitors. We present a 6 years old boy with nose bleed for six months of whom prothrombin time was prolonged with functional factor VII assay of less than 1% confirming factor VII deficiency. He was managed with fresh frozen plasma, blood transfusion, tranexamic acid. Factor VII deficiency even though rare should be sought out in children presenting with bleeding. Keywords: Factor VII deficiency; coagulation; hemorrhage.

Peran Musik Tradisi Kelintang Tungkal Sebagai Prosesi Malam Beinai Masyarakat Kampung Nelayan Kabupaten Tanjung Jabung Timur

This study aims to know the role of the Kelintang Tungkal music tradition as a musical tradition that lives and develops in the Kampung Nelayan Sub-District as a form of social integration and a developing musical structure. The structure is seen from the perspective of anthropologogical music, that Kelintang Tungkal was originally used by the community as accompaniment music in medical ceremonies but at this time the ceremony was no longer played because the myth of the local community that rolled the Kelintang Tungkal community had a strong magical element, so that these types of blows may not be made or played anywhere. Then the local community changed the function of the Kelintang Tungkal as an entertainment activity for the local community at wedding parties, welcoming traditional guests and as dance accompaniment music. Most of the data collected in this study were obtained from research on places and performances, interviews, and references related to writing about tungkal latitude, and were analyzed with qualitative approach. This study concludes that there has been a change in the Kelintang Tungkal performance that was originally used for medical ceremonies, because the functional factor of the Kelintang Tungkal performance turned into entertainment.

A life dedicated to medicine

The article discusses the work of the founder of modern anatomy Andreas Vesalius (1514-1564) on the culmination of his scientific and pedagogical activity - the work "De humani corporis fabrica", the historical conditions in which this book was written, and the difficulties created by the classical anatomy of Galen. It is noted that the work of Vesalius played an important role not only in the development of anatomy and medicine but in general in the formation and development of universal human innovative ideas. Unlike his predecessors, Vesalius preferred the method of dissection in anatomy; the study of anatomy on human cadavers, often contrary to the laws of that time, the explanation of the details of the subject precisely on cadaveric material, of course, could not fail to bear fruit. Along with this, it is noted that Vesalius was a skilled physician and surgeon of his time. Putting forward the functional factor in the study of organs, Vesalius achieved more voluminous and versatile scientific details. Throughout his career, Vesalius fought against the negative manifestation of Galen's ideas, which turned into medical dogma and achieved a detailed correction of about 200 Galen's mistakes. Of course, this was not positively received from the conservative-reactionary and very influential scientific circles, scientific and pedagogical activities, as well as the very existence of Vesalius simply as a person, was attacked. As a person who dedicated his life entirely to medicine and anatomy, Vesalius eventually fell victim to these conservative forces. Analysis of modern literary data on the main work of Vesalius shows that his scientific and pedagogical legacy still needs to be studied and discussed.

MloDisDB: a manually curated database of the relations between membraneless organelles and diseases

Cells are compartmentalized by numerous membrane-bounded organelles and membraneless organelles (MLOs) to ensure temporal and spatial regulation of various biological processes. A number of MLOs, such as nucleoli, nuclear speckles and stress granules, exist as liquid droplets within the cells and arise from the condensation of proteins and RNAs via liquid–liquid phase separation (LLPS). By concentrating certain proteins and RNAs, MLOs accelerate biochemical reactions and protect cells during stress, and dysfunction of MLOs is associated with various pathological processes. With the development in this field, more and more relations between the MLOs and diseases have been described; however, these results have not been made available in a centralized resource. Herein, we build MloDisDB, a database which aims to gather the relations between MLOs and diseases from dispersed literature. In addition, the relations between LLPS and diseases were included as well. Currently, MloDisDB contains 771 curated entries from 607 publications; each entry in MloDisDB contains detailed information about the MLO, the disease and the functional factor in the relation. Furthermore, an efficient and user-friendly interface for users to search, browse and download all entries was provided. MloDisDB is the first comprehensive database of the relations between MLOs and diseases so far, and the database is freely accessible at http://mlodis.phasep.pro/.

The Evolution of Hemophilia Care: Clinical and Laboratory Advances, Opportunities, and Challenges

Hemophilia A (HA) and B (HB) are X-linked bleeding disorders caused by mutations in the F8 or F9 gene that result in the absence, or reduced activity, of the corresponding clotting factor. The severity of bleeding and related complications is proportional to the amount of residual circulating functional factor. The development of a safe and effective hemophilia treatment lasted several decades and has been mainly based on clotting factor replacement. Advances in the engineering and manufacturing of clotting concentrates have led to the widespread availability of extended half-life products that reduced the number of intravenous infusions needed to achieve adequate trough levels. The recent development of new nonfactor replacement treatments and biotechnology techniques has offered therapeutic alternatives for hemophilia patients with and without inhibitors. These are characterized by an easier route of administration, low immunogenicity, and, regarding gene therapy and cell-based treatments, potential long-term protection from bleeding after a single treatment course. In this review, we analyze recent progresses in the management of hemophilia and discuss opportunities and challenges.

0700 Prediction Of Surgical Outcome Using Respiratory Pattern Clafification

Introduction: Introduction A diagnosis of the surgical indication is important in Sleep surgery. In otolaryngology regions, it is usually considered that a site of occlusion diagnosis by DISE. However, for OSA patients with aging, heart failure and neurologic disease, the effect with the surgical treatment is still low. In these OSA patients, it is a reason that a functional factor is associated as well as an anatomical factor. Similarly, without clear complications including Aging etc, there are a lot of the OSA patients whom a functional factor is associated with. OSA is multi-factorial disease according to Wellman model, OSA is associated with the functional factor including loop gain and the ability of the upper airway to dilate associated with ventilatory drive, arousal threshold only other than the anatomical factor of the upper airway. By normal PSG, the respiratory event is classified as obstructive, mixed and central. However, there are a lot of events that a functional factor is associated with, even if it is a respiratory event to be diagnosed as an obstructive respiratory event. An effect of sleep surgery is expected only for an anatomical factor. Therefore, in the case of sleep surgery, we must diagnose a pure obstructive respiratory event precisely. Purpose of this study: To clarify a respiratory pattern to influence surgical success. Methods: Methods 26 surgical patients with Pharyngeal surgeries diagnosed as OSA by PSG + Pes measurements were enrolled. We distinguish respiratory event as Pure obstructive event or not, using Pes signal pattern and EEG arousal timing. Results: Results Rate of pure obstructive event of all respiratory event varied by an individual patient. Mean rate of pure obstructive event is 57.3%. We find significant difference of AHI improvement rate between two groups which Rate of pure obstructive event shows more than 55% or less than 55%. Conclusion: Conclusion Breathing pattern is variety and pure obstructive event is few than ever considered. Respiratory pattern influence surgical success. Support Non