scholarly journals The Relationship between Hemophilia A Severity and Hemophilia Activities List

2021 ◽  
Author(s):  
Faizal Muhammad

Hemophilia is a hereditary bleeding disorder due to clotting factors deficiency. Its clinical manifestations including spontaneous and recurrent joints and muscle bleeding. Thus, hemophilia can limit the patients’ daily activities. This study aims to assess the relationship of hemophilia A severity on daily activities and the Hemophilia Activities List (HAL). The research subjects were thirty men with hemophilia A aged 18 years old or older who went to the Hematology-Oncology Clinic of Dr. Moewardi General Hospital during February - September 2020. Standardized seven aspects of routine activities with high-risk for bleeding event were assessed using the HAL questionnaire including lying down/ sitting/ kneeling/ standing, functions of the legs, functions of the arms, use of transportation, self-care, household tasks, leisure activities and sports. Based on the frequency of activity difficulty due to hemophilia A, each average score of HAL aspect was categorized into never (100% - 76%); rarely (75% - 51%), sometimes (50% - 26%), and impossible (25% - 0%). Based on Factor VIII level, hemophilia A severity was categorized into mild, moderate, and severe. Spearman’s correlation test was used for statistical analysis. The result showed significant correlation (p < 0.05) on five aspects, including lying down/ sitting/ kneeling/ standing, functions of the legs, use of transportation, self-care, and household tasks. The aspects of arms functions and leisure sports activities were not significantly correlated (p > 0.05). Nevertheless, these two aspects showed positive sufficient (r = 0.330) and weak (r = 0.177) correlation respectively. Joint and muscle bleeding are an undeniable pathological event in hemophilia patients. Hemophilia A severity positively correlates with the bleeding event frequency in the essential routine musculoskeletal activities. According to the HAL questionnaire, it needs to be a concern for clinicians and patient education to prevent bleeding in any high-risk musculoskeletal activities.

2016 ◽  
Author(s):  
Aric Parnes ◽  
Lisa Rotenstein

Hemophilia is a family of rare bleeding disorders characterized by deficiency of clotting factors. Hemophilia A is an inherited deficiency of factor VIII, whereas hemophilia B (Christmas disease) represents a deficiency of factor IX. Both hemophilia A and B are X-linked diseases, with hemophilia A accounting for 80 to 85% of cases and hemophilia B 15 to 20%. Although hemophilia has historically referred to deficiencies of factors VIII and IX, it is important to recognize that similar bleeding disorders can occur with other missing clotting factors, although this is far more rare. This review covers the definition, history, epidemiology, biology/genetics, clinical manifestations, diagnosis, differential diagnosis, treatment, complications, measures of quality of care, and prognosis of hemophilia, as well as future directions. Figures show the clotting cascade, the genetic makeup of severe hemophilia A, an algorithm for diagnosing hemophilia, and hemophilic arthropathy in a patient’s knees. Tables list severity in hemophilia A and B, treatment of acute bleeding in hemophilia A and B, frequency of dosing in acute bleeding, and treatment of acute bleeding with inhibitors. This review contains 4 highly rendered figures, 4 tables, and 59 references.


1979 ◽  
Vol 42 (03) ◽  
pp. 825-831 ◽  
Author(s):  
Jean-Pierre Allain

SummaryIn order to determine the correlation between different doses of F. VIII and their clinical effect,. 70 children with severe hemophilia A were studied after treatment with single doses of cryoprecipitate. The relationship between plasma F. VIII levels or doses calculated in u/ kg of body weight and clinical results followed an exponential curve. Plasma F. VIII levels of 0.35 and 0.53 u/ml corresponded to 95 and 99% satisfactory treatment, respectively. Similar clinical results were obtained with 20 and 31 u/kg. When the in vivo recovery of F. VIII after lyophilized cryoprecipitate was 0.015 u/ml for each u/kg injected, plasma F. VIII levels of 0.30 and 0.47 u/ml respectively were achieved. Since home treatment is largely based on single infusions of F. VIII, it is suggested that moderate and severe hemorrhages be treated with a dose which will provide a plasma F. VIII level of 0.5 u/ml.


Author(s):  
S. V. Petrov

This article is devoted to the study of the relationship, mutual influence of team building and team leadership established by the author of the article, the competencies of leaders in the field of team building, leadership styles, including with regard to the process of forming and maintaining teams, are established that the process of creating an effective team is an integral part of the leader’s daily activities.


Author(s):  
Ani Media Harumi ◽  
Kasiati Kasiati

Abstract: The purpose of this study is to analyze the relationship of age high risk with the incidence ofbleeding post partum in dr. M. Soewandhie Surabaya hospital. This research is analytic with an approachof a sectional cross. The study population was all post partum mothers in the Dr. M. Soewandhie Surabayahospital in January 2016 to March 2017, which amounts to an average of 1840respondents while theresearch sample number 182. Measuring collection sheet data obtained by systematic random sampling.The Study was conducted Chi-Square test obtained mean count X2 (0,00) is less than á (0.05) thenH0 is rejected and H1 accepted it means that there is a relationship between the age of high risk withbleeding post partum. Conclusion, there is a relationship between the age of high risk with the incidenceof bleeding post partum in the Spaceof the Maternity room Dr. Moch. Soewandhie Surabayahospital.


2020 ◽  
Author(s):  
Amir Karami ◽  
Brandon Bookstaver ◽  
Melissa Nolan

BACKGROUND The COVID-19 pandemic has impacted nearly all aspects of life and has posed significant threats to international health and the economy. Given the rapidly unfolding nature of the current pandemic, there is an urgent need to streamline literature synthesis of the growing scientific research to elucidate targeted solutions. While traditional systematic literature review studies provide valuable insights, these studies have restrictions, including analyzing a limited number of papers, having various biases, being time-consuming and labor-intensive, focusing on a few topics, incapable of trend analysis, and lack of data-driven tools. OBJECTIVE This study fills the mentioned restrictions in the literature and practice by analyzing two biomedical concepts, clinical manifestations of disease and therapeutic chemical compounds, with text mining methods in a corpus containing COVID-19 research papers and find associations between the two biomedical concepts. METHODS This research has collected papers representing COVID-19 pre-prints and peer-reviewed research published in 2020. We used frequency analysis to find highly frequent manifestations and therapeutic chemicals, representing the importance of the two biomedical concepts. This study also applied topic modeling to find the relationship between the two biomedical concepts. RESULTS We analyzed 9,298 research papers published through May 5, 2020 and found 3,645 disease-related and 2,434 chemical-related articles. The most frequent clinical manifestations of disease terminology included COVID-19, SARS, cancer, pneumonia, fever, and cough. The most frequent chemical-related terminology included Lopinavir, Ritonavir, Oxygen, Chloroquine, Remdesivir, and water. Topic modeling provided 25 categories showing relationships between our two overarching categories. These categories represent statistically significant associations between multiple aspects of each category, some connections of which were novel and not previously identified by the scientific community. CONCLUSIONS Appreciation of this context is vital due to the lack of a systematic large-scale literature review survey and the importance of fast literature review during the current COVID-19 pandemic for developing treatments. This study is beneficial to researchers for obtaining a macro-level picture of literature, to educators for knowing the scope of literature, to journals for exploring most discussed disease symptoms and pharmaceutical targets, and to policymakers and funding agencies for creating scientific strategic plans regarding COVID-19.


2020 ◽  
Vol 4 (Supplement_1) ◽  
pp. 331-332
Author(s):  
Jacqueline Guzman ◽  
Yuliana Soto ◽  
David Marquez ◽  
Susan Aguinaga

Abstract Latinos have high risk of Alzheimer’s disease and related dementias (ADRD). Self-rated health (SRH) has been used to predict cognitive decline. Early detection of executive function changes may help identify those at higher risk of cognitive decline. The purpose of this study was to examine the relationship between SRH and executive function in Latinos. Latinos (N=333, 84.4% female, Mage= 64.9 ± 7.08) from the BAILA randomized controlled trial self-rated their health as 1) poor/fair, 2) good, and 3) very good/excellent. Executive function was assessed by the Trail-making B, Verbal Fluency, Stroop C & CW, and the Digit Modality tests and stratified by SRH. One-way analysis of variance showed that the effect of SRH was significant for Trails B, F(2,298)=4.01, p=.019 and Stroop CW, F(2,298)=3.07, p=.048. Tukey’s test indicated that participants who rated their health as fair/poor took longer to complete Trails B (M=196.78±83.0 seconds) compared to those who rated their health as good (M=185.25 ± 85.1 seconds) and very good/excellent (M=149.25±95.3 seconds). Stroop CW results demonstrated that those in the fair/poor health category scored lower (M=17.22±6.6) than those in good (M=19.70±8.5 words/minutes) and very good/excellent health categories (M=18.73±8.2 words/minute). In sum, the results suggest SRH is related to executive function such that lower categories of SRH are indicative of poorer executive function. SRH might be used as a proxy for executive function and as a tool that community leaders can use to identify individuals at high risk of ADRD in need of behavioral interventions.


Toxins ◽  
2021 ◽  
Vol 13 (2) ◽  
pp. 78
Author(s):  
Lachlan A. Bourke ◽  
Christina N. Zdenek ◽  
Edgar Neri-Castro ◽  
Melisa Bénard-Valle ◽  
Alejandro Alagón ◽  
...  

The toxin composition of snake venoms and, thus, their functional activity, can vary between and within species. Intraspecific venom variation across a species’ geographic range is a major concern for antivenom treatment of envenomations, particularly for countries like French Guiana that lack a locally produced antivenom. Bothrops asper and Bothrops atrox are the most medically significant species of snakes in Latin America, both producing a variety of clinical manifestations, including systemic bleeding. These pathophysiological actions are due to the activation by the venom of the blood clotting factors Factor X and prothrombin, thereby causing severe consumptive coagulopathy. Both species are extremely wide-ranging, and previous studies have shown their venoms to exhibit regional venom variation. In this study, we investigate the differential coagulotoxic effects on human plasma of six venoms (four B. asper and two B. atrox samples) from different geographic locations, spanning from Mexico to Peru. We assessed how the venom variation of these venom samples affects neutralisation by five regionally available antivenoms: Antivipmyn, Antivipmyn-Tri, PoliVal-ICP, Bothrofav, and Soro Antibotrópico (SAB). The results revealed both inter- and intraspecific variations in the clotting activity of the venoms. These variations in turn resulted in significant variation in antivenom efficacy against the coagulotoxic effects of these venoms. Due to variations in the venoms used in the antivenom production process, antivenoms differed in their species-specific or geographical neutralisation capacity. Some antivenoms (PoliVal-ICP, Bothrofav, and SAB) showed species-specific patterns of neutralisation, while another antivenom (Antivipmyn) showed geographic-specific patterns of neutralisation. This study adds to current knowledge of Bothrops venoms and also illustrates the importance of considering evolutionary biology when developing antivenoms. Therefore, these results have tangible, real-world implications by aiding evidence-based design of antivenoms for treatment of the envenomed patient. We stress that these in vitro studies must be backed by future in vivo studies and clinical trials before therapeutic guidelines are issued regarding specific antivenom use in a clinical setting.


2021 ◽  
Vol 23 (3) ◽  
Author(s):  
Frank Zhu ◽  
Jocelyn Y. Ang

Abstract Purpose of Review Provide an updated review of the clinical management and diagnosis of Kawasaki disease with inclusion of potential diagnostic difficulties with multisystem inflammatory syndrome in children (MIS-C) given the ongoing COVID-19 pandemic. Recent Findings Adjunctive corticosteroid therapy has been shown to reduce the rate of coronary artery dilation in children at high risk for IVIG resistance in multiple Japanese clinical studies (most notably RAISE study group). Additional adjunctive therapies (etanercept, infliximab, cyclosporin) may also provide limited benefit, but data is limited to single studies and subgroups of patients with cardiac abnormalities. The efficacy of other agents (atorvastatin, doxycycline) is currently being investigated. MIS-C is a clinically distinct entity from KD with broad clinical manifestations and multiorgan involvement (cardiac, GI, hematologic, dermatologic, respiratory, renal). MIS-C with Kawasaki manifestations is more commonly seen in children < 5 years of age. Summary The 2017 American Heart Association (AHA) treatment guidelines have included changes in aspirin dosing (including both 80–100 mg/kg/day and 30–50 mg/kg/day treatment options), consideration of the use of adjuvant corticosteroid therapy in patients at high risk of IVIG resistance, and the change in steroid regimen for refractory KD to include both pulse-dose IVMP and longer course of prednisolone with an oral taper. A significant proportion of children diagnosed with MIS-C, a post-infectious syndrome of SARS-CoV-2 infection, meet criteria for Kawasaki disease. Further investigation is warranted to further delineate these conditions and optimize treatment of these conditions given the ongoing COVID-19 pandemic.


2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Minjae Yoon ◽  
Jaewon Oh ◽  
Kyeong-Hyeon Chun ◽  
Chan Joo Lee ◽  
Seok-Min Kang

AbstractImmunosuppressive therapy can decrease rejection episodes and increase the risk of severe and fatal infections in heart transplantation (HT) recipients. Immunosuppressive therapy can also decrease the absolute lymphocyte count (ALC), but the relationship between early post-transplant ALC and early cytomegalovirus (CMV) infection is largely unknown, especially in HT. We retrospectively analyzed 58 HT recipients who tested positive for CMV IgG antibody and received basiliximab induction therapy. We collected preoperative and 2-month postoperative data on ALC and CMV load. The CMV load > 1200 IU/mL was used as the cutoff value to define early CMV infection. Post-transplant lymphopenia was defined as an ALC of < 500 cells/μL at postoperative day (POD) #7. On POD #7, 29 (50.0%) patients had post-transplant lymphopenia and 29 (50.0%) patients did not. The incidence of CMV infection within 1 or 2 months of HT was higher in the post-transplant lymphopenia group than in the non-lymphopenia group (82.8% vs. 48.3%, P = 0.013; 89.7% vs. 65.5%, P = 0.028, respectively). ALC < 500 cells/μL on POD #7 was an independent risk factor for early CMV infection within 1 month of HT (odds ratio, 4.14; 95% confidence interval, 1.16–14.77; P = 0.029). A low ALC after HT was associated with a high risk of early CMV infection. Post-transplant ALC monitoring is simple and inexpensive and can help identify patients at high risk of early CMV infection.


2021 ◽  
pp. 0044118X2110028
Author(s):  
Heather Mary Quinlan ◽  
Kellie Lynn Hadden ◽  
David Paul Storey

The purpose of the current study was to explore whether selfcompassion predicted psychological distress over and above childhood maltreatment and attachment orientation in high-risk youths. Fifty-one youths (31 males, 20 females) aged 17 to 24, recruited from a community non-profit organization in St. John’s, Newfoundland and Labrador, Canada, were administered validated measures of childhood maltreatment, attachment orientation, self-compassion, and psychological distress. Results indicated that self-compassion was inversely associated with childhood maltreatment, attachment anxiety, attachment avoidance, and psychological distress. However, results did not support the hypothesis that self-compassion was a significant predictor of psychological distress over and above attachment anxiety and childhood maltreatment in high-risk youths. Our results indicated that self-compassion is not well developed in street-involved youths and may be a vital intervention target to heal negative internalized views of the self, while maintaining vigilance to threats inherent in the street environment.


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