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https://www.readbyqxmd.com/read/27913547/new-treatment-approaches-to-von-willebrand-disease
#1
Michelle Lavin, James S O'Donnell
von Willebrand disease (VWD) is the commonest inherited bleeding disorder and results from either a quantitative or qualitative deficiency in the plasma glycoprotein von Willebrand factor (VWF). Recent large cohort studies have significantly enhanced our understanding of the molecular mechanisms involved in the pathogenesis of VWD. In contrast, however, there have been relatively few advances in the therapeutic options available for the treatment of bleeding in patients with VWD. Established treatment options include tranexamic acid, 1-deamino-8-d-arginine vasopressin (DDAVP), and plasma-derived VWF concentrates...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27913544/treatment-of-rare-factor-deficiencies-in-2016
#2
Flora Peyvandi, Marzia Menegatti
Rare bleeding disorders (RBDs) are a heterogeneous group of coagulation disorders characterized by fibrinogen, prothrombin, factors V, VII, X, XI, or XIII (FV, FVII, FX, FXI, or FXIII, respectively), and the combined factor V + VIII and vitamin K-dependent proteins deficiencies, representing roughly 5% of all bleeding disorders. They are usually transmitted as autosomal, recessive disorders, and the prevalence of the severe forms could range from 1 case in 500 000 for FVII up to 1 in 2-3 million for FXIII in the general population...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27913535/reversing-vitamin-k-antagonists-making-the-old-new-again
#3
Sabine Eichinger
Vitamin K antagonists (VKAs) are commonly used for the prevention and treatment of thrombotic disorders. The response to VKAs is highly variable due to their specific interaction with the vitamin K cycle, and hence interference with hepatic synthesis of vitamin K-dependent coagulation factors. Monitoring the anticoagulant effect of VKAs by assessing the patient's international normalized ratio (INR) is essential because complications are closely related to the intensity of anticoagulation. Treatment with VKAs contains a substantial risk of bleeding with a high case fatality rate...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27913488/platelets-in-liver-and-renal-disease
#4
Michele P Lambert
This review will discuss how 2 common and morbid conditions, renal disease and liver disease, alter platelet number and function. It will review the impact of thrombocytopenia on bleeding complications in patients with these disorders and whether the low platelet count actually correlates with bleeding risk. Emerging data also suggest that platelets are much more than bystanders in both renal and liver disease, but instead play an active role in the pathobiology of these disorders. This review will briefly cover the emerging information on novel roles of platelets in the biology of renal and liver disease...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27913486/heavy-menstrual-bleeding-work-up-and-management
#5
Andra H James
Heavy menstrual bleeding (HMB), which is the preferred term for menorrhagia, affects ∼90% of women with an underlying bleeding disorder and ∼70% of women on anticoagulation. HMB can be predicted on the basis of clots of ≥1 inch diameter, low ferritin, and "flooding" (a change of pad or tampon more frequently than hourly). The goal of the work-up is to determine whether there is a uterine/endometrial cause, a disorder of ovulation, or a disorder of coagulation. HMB manifest by flooding and/or prolonged menses, or HMB accompanied by a personal or family history of bleeding is very suggestive of a bleeding disorder and should prompt a referral to a hematologist...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27913485/congenital-and-acquired-bleeding-disorders-in-pregnancy
#6
Terry B Gernsheimer
Obstetrical hematology represents challenges not only for the patient, but also for her progeny. In particular, bleeding disorders, both congenital and acquired, not only present problems both for delivery and in the immediate postpartum period, but also may have significant implications for the fetus and neonate. Women with congenital bleeding disorders or who are carriers of X-linked or autosomal disorders should be counseled prior to conception so that pregnancy can be safely undertaken with careful preparation...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27913478/thrombosis-in-the-setting-of-obesity-or-inflammatory-bowel-disease
#7
Steven R Lentz
Obesity and inflammatory bowel disease (IBD) are systemic inflammatory disorders that predispose to arterial and venous thrombosis through similar prothrombotic mechanisms. Obesity and IBD are chronic risk factors that lead to a persistently elevated risk of thrombosis, although the thrombotic risk with IBD appears to wax and wane with disease severity. Because of the lack of high-quality evidence to guide management decisions, approaches to the prevention and treatment of thrombosis in patients with obesity or IBD are based on extrapolation from general guidelines for antithrombotic therapy...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27908513/small-bowel-imaging-computed-tomography-enterography-magnetic-resonance-enterography-angiography-and-nuclear-medicine
#8
REVIEW
Jeff L Fidler, Ajit H Goenka, Chad J Fleming, James C Andrews
Radiology examinations play a major role in the diagnosis, management, and surveillance of small bowel diseases and are complementary to endoscopic techniques. Computed tomography enterography and magnetic resonance enterography are the cross-sectional imaging studies of choice for many small bowel diseases. Angiography still plays an important role for catheter-directed therapies. With the emergence of hybrid imaging techniques, radionuclide imaging has shown promise for the evaluation of small bowel bleeding and Crohn disease and may play a larger role in the future...
January 2017: Gastrointestinal Endoscopy Clinics of North America
https://www.readbyqxmd.com/read/27906097/a-10-year-old-child-presenting-with-syndromic-paucity-of-bile-ducts-alagille-syndrome-a-case-report
#9
Girish Kumar Pati, Ayaskanta Singh, Preetam Nath, Jimmy Narayan, Pradeep Kumar Padhi, Prasanta Kumar Parida, Kaumudee Pattnaik, Chittaranjan Panda, Shivaram Prasad Singh
BACKGROUND: Alagille syndrome, a rare genetic disorder with autosomal dominant transmission, manifests with five major features: paucity of interlobular bile ducts, characteristic facies, posterior embryotoxon, vertebral defects, and peripheral pulmonary stenosis. Globally, only 500 cases have so far been reported, with only five cases reported in the Indian subcontinent. Rarely, Alagille syndrome also presents with skin manifestations and early-onset chronic liver disease, which was found in our case...
November 30, 2016: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/27906086/massive-adrenal-vein-aneurysm-mimicking-an-adrenal-tumor-in-a-patient-with-hemophilia-a-a-case-report-and-review-of-the-literature
#10
Richard Sleightholm, Steven Wahlmeier, Jeffrey S Carson, Andjela Drincic, Audrey Lazenby, Jason M Foster
BACKGROUND: Visceral venous aneurysms are exceedingly rare, and until now, there have been no reports of this phenomenon in the adrenal vasculature. This report details the first adrenal venous aneurysm reported in the literature. The aneurysm presented as an 18-cm mass that was initially suspected to be a hematoma or tumor on the basis of the complex medical history of the patient, which included hemophilia A and testicular cancer. After surgical excision, pathologic examination confirmed this mass to be a 15...
December 1, 2016: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/27904630/a-survey-of-the-therapeutic-effects-of-vitamin-e-suppositories-on-vaginal-atrophy-in-postmenopausal-women
#11
Aazam Parnan Emamverdikhan, Nahid Golmakani, Sayyed ASajadi Tabassi, Malihe Hassanzadeh, Nooriyeh Sharifi, Mohammad Taghi Shakeri
BACKGROUND: Menopause is associated with various complications such as depression, sleep disorders, and genitourinary atrophy. Vaginal atrophy occurs due to the loss of steroid hormones, and its major symptoms include vaginal dryness, itching, dyspareunia, and bleeding after intercourse. According to the literature, vitamin E plays a key role in estrogen stability. The aim of this study was to compare the effects of vitamin E suppositories and conjugated estrogen vaginal cream on vaginal atrophy...
September 2016: Iranian Journal of Nursing and Midwifery Research
https://www.readbyqxmd.com/read/27903446/polycystic-ovary-syndrome-an-under-recognized-cause-of-abnormal-uterine-bleeding-in-adolescents-admitted-to-a-children-s-hospital
#12
Sofya Maslyanskaya, Hina J Talib, Jennifer L Northridge, Amanda M Jacobs, Chanelle Coble, Susan M Coupey
STUDY OBJECTIVE: To evaluate whether ovulatory dysfunction due to polycystic ovary syndrome (PCOS) is a common underlying etiology of abnormal uterine bleeding (AUB) in adolescents requiring hospitalization and to explore etiology, treatment and complications of AUB with severe anemia in adolescents. DESIGN: We identified females aged 8 to 20 years admitted to a children's hospital for treatment of AUB from January 2000 to December 2014. Our hospital protocol advises hormonal testing for PCOS and other disorders prior to treatment for AUB...
November 26, 2016: Journal of Pediatric and Adolescent Gynecology
https://www.readbyqxmd.com/read/27903389/-dabigatran-versus-warfarin-for-the-prevention-of-stroke-in-chinese-patients-with-nonvalvular-atrial-fibrillation-chinese-subpopulation-analysis-of-re-ly
#13
X Gao, Y M Yang, J Zhu, Y Dai, H Q Tan
Objective: This analysis was performed to evaluate the efficacy in stroke prevention and safety of dabigatran in Chinese nonvalvular atrial fibrillation(NVAF) patients enrolled in RE-LY trial. Methods: RE-LY was an prospective, open-label, randomized, multicenter study. From March 2006 to March 2009, 541 atrial fibrillation patients at risk of stroke were recruited from 13 medical centers in China. Patients randomly received, in a blinded fashion, fixed doses of dabigatran-110 mg or 150 mg twice daily or, in an unblinded fashion, adjusted-dose warfarin...
November 24, 2016: Zhonghua Xin Xue Guan Bing za Zhi
https://www.readbyqxmd.com/read/27903265/associations-among-oral-health-related-quality-of-life-subjective-symptoms-clinical-status-and-self-rated-oral-health-in-japanese-university-students-a-cross-sectional-study
#14
Mayu Yamane-Takeuchi, Daisuke Ekuni, Shinsuke Mizutani, Kota Kataoka, Ayano Taniguchi-Tabata, Tetsuji Azuma, Michiko Furuta, Takaaki Tomofuji, Yoshiaki Iwasaki, Manabu Morita
BACKGROUND: The present study aimed to elucidate the associations among self-rated oral health, clinical oral health status, oral health behaviors, subjective oral symptoms, and oral health-related quality of life (OHRQoL) in a group of Japanese university students. METHODS: Of 2051 participants, 2027 (98.83%) students received an optional oral examination and answered a questionnaire including items regarding age, sex, self-rated oral health, oral health behaviors, subjective oral symptoms, and OHRQoL [The Oral Health Impact Profile (OHIP)-14]...
November 30, 2016: BMC Oral Health
https://www.readbyqxmd.com/read/27901126/low-level-light-treatment-ameliorates-immune-thrombocytopenia
#15
Jingke Yang, Qi Zhang, Peiyu Li, Tingting Dong, Mei X Wu
Immune thrombocytopenia (ITP) is an immune-mediated acquired bleeding disorder characterized by abnormally low platelet counts. We reported here the ability of low-level light treatment (LLLT) to alleviate ITP in mice. The treatment is based on noninvasive whole body illumination 30 min a day for a few consecutive days by near infrared light (830 nm) transmitted by an array of light-emitting diodes (LEDs). LLLT significantly lifted the nadir of platelet counts and restored tail bleeding time when applied to two passive ITP models induced by anti-CD41 antibody...
November 30, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27900416/-intensive-care-treatment-after-aneurysmal-subarachnoid-hemorrhage
#16
U Jaschinski
Aneurysmal subarachnoid hemorrhage (SAH) is a devastating disease and nearly one third of patients die in the acute phase. Due to the bleeding event, a hyperactive sympathetic nervous system and an uncontrolled inflammatory response have a profound local and systemic impact on other organ functions. Neuroendocrinological disorders and cardiopulmonary morbidity are dominant. Despite a decrease in hospital mortality for high volume centers, a high proportion of survivors suffer from neurological deficits. Knowledge of the pathophysiology of vasospasms in the later stages of the disease has increased...
November 29, 2016: Der Anaesthesist
https://www.readbyqxmd.com/read/27894493/optimal-reversal-of-novel-anticoagulants-in-trauma
#17
REVIEW
Jason Weinberger, Mark Cipolle
The incidence of patients with trauma on novel oral anticoagulants (NOACs) for the treatment of thromboembolic disorders is increasing. In severe bleeding or hemorrhage into critical spaces, urgent reversal of this underlying pharmacologic coagulopathy becomes paramount. Optimal reversal strategy for commonly used NOACs is still evolving. Basic tenets of evaluation of patients with trauma and resuscitation remain the same. Clinical outcomes data in bleeding human patients with trauma are lacking, but are needed to establish efficacy and safety in these treatments...
January 2017: Critical Care Clinics
https://www.readbyqxmd.com/read/27894217/diagnosis-clinical-manifestations-and-management-of-rare-bleeding-disorders-in-iran
#18
Akbar Dorgalaleh, Sayed Ezatolla Rafiee Alavi, Shadi Tabibian, Shahrzad Soori, Es'hagh Moradi, Taregh Bamedi, Mansour Asadi, Masumeh Jalalvand, Morteza Shamsizadeh
BACKGROUND: Rare bleeding disorders (RBDs) are heterogeneous disorders, mostly inherited in an autosomal recessive pattern. Iran is a Mideast country with a high rate of consanguinity that has a high rate of RBDs. OBJECTIVE: In this study, we present prevalence and clinical presentation as well as management and genetic defects of Iranian patients with RBDs. METHODS: For this study, all relevant publications were searched in Medlin until 2015...
November 28, 2016: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/27891349/unusual-presentation-of-acute-leukaemia-a-tripod-of-cases
#19
Manjari Kishore, Vijay Kumar, Sadhna Marwah, Abhay S Nigam
Acute Leukemia is one of the common haematological malignancies encountered with varied clinical and haematological presentation. In acute leukaemia, complications like bleeding and infection cause significant morbidity and mortality, thus overshadowing the thromboembolic events. Among the various malignant haematological disorders, the association of thromboembolic events is often noted with acute promyelocytic leukemia, though the overall frequency of such events remains very low. Acute Lymphoblastic Leukemia (ALL) is, however, more common than Acute non-lymphoblastic Leukaemia...
October 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/27889498/bloc-2-subunit-hps6-deficiency-affects-the-tubulation-and-secretion-of-von-willebrand-factor-from-mouse-endothelial-cells
#20
Jing Ma, Zhe Zhang, Lin Yang, Janos Kriston-Vizi, Daniel F Cutler, Wei Li
Hermansky-Pudlak syndrome (HPS) is a recessive disorder with bleeding diathesis, which has been linked to platelet granule defects. Both platelet granules and endothelial Weibel-Palade bodies (WPBs) are members of lysosome-related organelles (LROs) whose formation is regulated by HPS protein associated complexes such as BLOC (biogenesis of lysosome organelles complex) -1, -2, -3, AP-3 (adaptor protein complex-3) and HOPS (homotypic fusion and protein sorting complex). Von Willebrand factor (VWF) is critical to hemostasis, which is stored in a highly-multimerized form as tubules in the WPBs...
October 21, 2016: Journal of Genetics and Genomics, Yi Chuan Xue Bao
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