Hemophilia type A is a rare inherited bleeding disorder with a diversity of clinical manifestations ranging from persistent bleeding after minor trauma, spontaneous deep muscle or joint hemorrhage, to intracranial hemorrhage. As an X-linked disorder, hemophilia is rare in females and therefore there is little experience with pregnancy and no standardized guidelines to prevent bleeding antepartum, at delivery, and postpartum. We report the clinical course and management of a woman with severe hemophilia A who on two occasions had uncomplicated pregnancies and vaginal deliveries at term utilizing bolus recombinant factor VIII concentrate.
In this paper we are elaborating the concept of disease detection in the human body using the nail image of human fingers and analyzing the data from the image on the basis of nail color and texture. Fingernail has to be detected from the entire hand region using distribution density of the nail color pixels on the surface of nail. The methodology for creating a finger nail detection system involves removing the skin area from shiny/glossy nail portion; this is known as image segmentation concept that separates the specific object. Image segmentation is the method of dividing the image pixels into homogenous region.
2575 Background: Use of platelet (plt) transfusions to treat and prevent bleeding varies widely between hospitals and by medical and surgical services. Standard indications include active bleeding with thrombocytopenia or plt dysfunction, pre or peri-invasive procedure, and prophylaxis for low plt counts. Rising demand for plt transfusions and donor shortage, coupled with the risks of transfusion (including infectious disease transmission and alloimmunization) are concerns which often lead to strict regulation of plt transfusion in hospitals. In order to evaluate appropriate use of plt transfusion based on Newark Beth Israel Medical Center transfusion guidelines, a review of plt use was undertaken at this tertiary care hospital. Design: A retrospective review was performed of plt utilization over a 3 month period from October to December 2009. All charts of hospitalized and outpatient patients receiving plt transfusions were reviewed to determine reasons for plt transfusion. Pre-transfusion plt values, site/service ordering plt transfusions, number of units transfused and cost were determined. Results: 421 plt units were transfused to 125 patients (51.6% female), mean age 44 years (yrs.) (range 0–89). All plt transfusions were single donor units. The mean plt count prior to transfusion for all procedures was 127,000, well above hospital guidelines. The majority of plt utilized were by cardiothoracic (CT) surgery (168/421, 40%) with the highest cost (Table 1). 124/421(29%) of transfusions occurred pre- or peri- invasive procedure, with 88/124 (71%) of those transfusions occurring prior or peri- cardio-thoracic procedure. 83/421 (20%) of transfusions had no clear indication based on hospital guidelines, predominately ordered by CT surgery and occurring post-op for asymptomatic thrombocytopenia (cost $45, 650). The mean plt count at which transfusion was found to have no indication was 55,000 (range 25,000–105,000). 136/421(32%) of the cases were prophylactic transfusions with a plt count < 20,000, with 121/136 (89%) in the oncology patients, and the rest in the medical pts due to sepsis. 114/421(27%) of the transfusions were for bleeding. Only 5 patients, 3 in the CT group, and 2 in neonate group had plt dysfunction as the indication for transfusion prior to procedure. The lowest incidence of plt transfusions without an indication was in the adult oncology department. Conclusion: Platelet utilization varied by departments. CT surgery followed by neonatal and pediatric oncology are the principal users of plt in our tertiary care medical center. CT surgery, general surgery, and neonatal services had the highest pre-transfusion plt counts. As 20% of all transfusions had no clinical reason for plt use (no bleeding, invasive procedure, or severely low plt count) the opportunities may exist for lower platelet usage by educating physicians about compliance to transfusion guidelines in order to decrease the risks associated with transfusion and resultant complications. Disclosure: No relevant conflicts of interest to declare.
2289 Introduction: Primary prophylaxis treatment for hemophilia (hem) patients (pts) during childhood has become the standard of care, however there is little consensus on the guidelines for adult management. Currently depending on the physician preference and experience, an adult hem pt can either be treated in response to bleeding (on-demand(OD)), or with regular infusion of clotting factor to prevent bleeding and further joint damage (secondary prophylaxis(P)). This study was undertaken to assess the differences in annual bleeds rates and utilization of clotting factor concentrates between P and OD treatment strategies in adults. Methods: A retrospective study of the 46 patients with moderate or severe hem at the Newark Beth Israel Comprehensive Hemophilia Center was undertaken. Data on treatment and outcomes were evaluated for pts treated with P and those treated with OD. A chart review was done to evaluate the extent of joint arthropathy, incidence of bleeds per calendar year, type of treatment P or OD, and quantity of factor utilized annually. Data was also collected on the utilization of analgesics medications, employment history, and psychiatric history. Results: The data was evaluable for 5 Hemophilia B pts, 4 severe, and 1 moderate; and 41 Hemophilia A pts, 25 severe, and 16 moderate. Forty-six percent (21/46) of the hem pts were receiving P as adults. Fifteen percent (7/46) hem pts had received P as a child or adolescent, and continued into adulthood. The pts ages ranged from 18 to 66 years of age. The mean age of pts receiving P was 32 years, while it was 34 years in the OD group. There was no difference in the average number of target joints between the two groups, with a mean of 2 target joints per patient. Fifty-two percent of hem on P (11/21) had 0–1 bleed per month, while only 40% of OD pts (12/25) had 0–1 bleed per month. In the P group 48% (10/21) had more than 1 bleed per month, while 60% in the OD group had more than 1 bleed per month. Overall there was no statically significant difference between the incidence of annual bleeds between the two groups, p=0.51. Forty-six percent of patients (21/46) were taking analgesic managements, and of these 48% (10/21) were on P, p=.806. Thirty-three percent (15/46) of the pts were unemployed or not enrolled as a student, and more than half (8/15) of those pts were on P treatment. Twenty-four percent (11/46) of hem pts were found to have a psychiatric history with either depression, or anxiety, of which 82% were on P. In 43 of the 46 hem pts the amount of factor utilized over a 5-year period (2006–2010) was available for analysis. Patients receiving P utilized more factor than pts on OD, 385,124 units versus 115,367 units, p =.0013. Conclusion: The initiation of primary prophylaxis in childhood has decreased much of the morbidity associated with hemophilia, however the benefit of secondary prophylaxis has not been clearly established. The treatment of hemophilia is costly, and there are both medical and economical advantages and disadvantages. Prophylaxis in this older population of moderate and severe hem pts did not have an impact on the reduction of annual bleeds, or the number of target joints, though pts on P utilized more units of clotting factor concentrate. Additionally, our pts did not have improvement in quality of life factors such as pain alleviation, increased employment, or mental well-being. Lack of benefit may be obscured as these pts who had not received primary prophylaxis, already had significant joint damage. Studies on continuation of P or OD in pts who have received primary prophylaxis will be necessary to determine long term prophylaxis in these pts. Disclosures: No relevant conflicts of interest to declare.
Beta arrestins are a family of adaptor proteins that help in the regulation of signaling and trafficking of various G protein coupled receptors (GPCRs). Six oxadiazole derivatives taken from literature are analyzed for anti-cancer properties. The toxicity profiles of all the drugs were similar to Tamoxifen used as control. Data shows that compounds 2, 4, and 6 exhibited comparably significant molecular interactions with the cancerous protein for further consideration.
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