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bleeding diathesis

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https://www.readbyqxmd.com/read/27909619/the-role-of-ultra-mini-percutaneous-nephrolithotomy-in-the-treatment-of-kidney-stones
#1
Abdulkadir Tepeler, İsmail Başıbüyük, Muhammed Tosun, Abdullah Armağan
OBJECTIVE: In our study we aimed to evaluate outcomes of ultra-mini-percutaneous nephrolithotomy (UMP) performed for the treatment of kidney stones. MATERIAL AND METHODS: Between October 2014 and September 2015, a single surgeon performed UMP on a total of 49 consecutive patients with symptomatic kidney stones at our center. Pregnants, anticogulant users, patients with bleeding diathesis pyonephrosis, and partial/complete staghorn stones were excluded from the study...
December 2016: Turkish Journal of Urology
https://www.readbyqxmd.com/read/27889498/bloc-2-subunit-hps6-deficiency-affects-the-tubulation-and-secretion-of-von-willebrand-factor-from-mouse-endothelial-cells
#2
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
https://www.readbyqxmd.com/read/27866582/bleeding-diatheses-approach-to-the-patient-who-bleeds-or-has-abnormal-coagulation
#3
REVIEW
Marcia Paddock, John Chapin
Many complex elements contribute to normal hemostasis, and an imbalance of these elements may lead to abnormal bleeding. In addition to evaluating medication effects, the hematologist must evaluate for congenital or acquired deficiencies in coagulation factors and platelet disorders. This evaluation should include a thorough bleeding history with careful attention to prior hemostatic challenges and common laboratory testing, including coagulation studies and/or functional platelet assays. An accurate diagnosis of a bleeding diathesis and selection of appropriate treatment are greatly aided by a basic understanding of the mechanisms of disease and the tests used to diagnose them...
December 2016: Primary Care
https://www.readbyqxmd.com/read/27829879/hermansky-pudlak-syndrome-in-pregnancy-a-case-report
#4
Lydia Yusuf, Srivasavi Dukka, Etienne Ciantar
Hermansky-Pudlak syndrome is a rare autosomal recessive disorder estimated to affect 1 in 500,000 to 1,000,000 individuals worldwide. Clinically, it presents as oculocutaneous albinism combined with bleeding diathesis. This is due to the absence of dense bodies in platelets causing a delayed secondary response resulting in prolonged bleeding time despite normal platelet count and coagulation factors. This has consequences for major bleeding, the risk of which is high at delivery. In the longer term, the condition is also associated with the development of pulmonary fibrosis, inflammatory bowel disorders caused by granulomatous colitis and renal failure...
December 2016: Obstetric Medicine
https://www.readbyqxmd.com/read/27780690/risk-of-atrial-fibrillation-and-bleeding-diathesis-associated-with-ibrutinib-treatment-a-systematic-review-and-pooled-analysis-of-four-randomized-controlled-trials
#5
Seongseok Yun, Nicole D Vincelette, Utkarsh Acharya, Ivo Abraham
BACKGROUND: Clinical trials raised concern that ibrutinib may increase the risk of atrial fibrillation/flutter (Afib/Aflutter) and major bleeding. However, the association has not been statistically validated, and there is no consensus regarding optimal management of anticoagulation among patients receiving ibrutinib who develop Afib/Aflutter. We performed a systematic review and pooled analysis to precisely assess the risk of Afib/Aflutter and bleeding associated with ibrutinib treatment in patients with hematologic malignancies...
September 19, 2016: Clinical Lymphoma, Myeloma & Leukemia
https://www.readbyqxmd.com/read/27745620/topical-hemostatic-agents-what-the-oral-and-maxillofacial-surgeon-needs-to-know
#6
Patrick J Vezeau
Hemostasis is a key step in safe and predictable surgery. Knowledge of normal blood clotting mechanisms and abnormal diathesis is necessary to anticipate potential problems during and after surgery. As an adjunct to bleeding control, topical hemostatic agents have long been used in all surgical disciplines. This article provides a brief review of hemostasis and a topical summary of different classes of topical hemostatic agents useful to oral and maxillofacial surgery, including indications and potential complications/side effects...
November 2016: Oral and Maxillofacial Surgery Clinics of North America
https://www.readbyqxmd.com/read/27695173/acute-myeloid-leukemia-presenting-as-galactorrhea
#7
K Rakul Nambiar, Sreejith G Nair, R Nandini Devi
Acute myeloid leukemia (AML) presents with symptoms related to pancytopenia (weakness, infections, bleeding diathesis) and organ infiltration with leukemic cells. Galactorrhea is an uncommon manifestation of AML. We report a case of AML presenting with galactorrhea.
October 2016: Proceedings of the Baylor University Medical Center
https://www.readbyqxmd.com/read/27687927/existence-of-bovine-neonatal-pancytopenia-before-the-year-2005-retrospective-evaluation-of-215-cases-of-haemorrhagic-diathesis-in-cattle
#8
A Stoll, A Pfitzner-Friedrich, F Reichmann, J Rauschendorfer, A Roessler, G Rademacher, G Knubben-Schweizer, C Sauter-Louis
Haemorrhagic diathesis (HD) in cattle is a relatively rare syndrome that can have many different causes. With the occurrence of bovine neonatal pancytopenia (BNP) in 2007, the number of cases of HD in cattle has increased. This led to an enhanced interest in diseases presenting with bleeding disorders. The possible causes of HD in cattle, the clinical findings, and the course of various diseases are described and evaluated. Furthermore, we determined whether cases of BNP occurred before the introduction of the vaccine Pregsure BVD since its widespread use was associated with the syndrome...
October 2016: Veterinary Journal
https://www.readbyqxmd.com/read/27676646/evidence-of-both-von-willebrand-factor-deposition-and-factor-v-deposition-onto-al-amyloid-as-the-cause-of-a-severe-bleeding-diathesis
#9
Jonathan S Harrison, Shellaine R Frazier, Dianne D McConnell, Senan John Yasar, Nataliya Melnyk, Gratian Salaru
Acquired coagulopathies are common; uncommonly, adsorption of coagulation factors from the circulation into the tissues by pathologic amyloid exceeds the body's ability to produce factor and results in acquired factor deficiency. When amyloidosis does cause a coagulopathy, it is most often acquired factor X deficiency, but there are rare reports of amyloidosis being associated with other acquired factor deficiencies. We investigated a case of a severe bleeding diathesis, the cause of which was combined acquired factor V deficiency and concomitant acquired von Willebrand syndrome...
September 24, 2016: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/27634287/rotational-thromboelastometry-can-detect-factor-xiii-deficiency-and-bleeding-diathesis-in-patients-with-cirrhosis
#10
Sotiria Bedreli, Jan-Peter Sowa, Saraa Malek, Sandra Blomeyer, Antonios Katsounas, Guido Gerken, Fuat H Saner, Ali Canbay
BACKGROUND & AIMS: Patients with progressive liver disease exhibit complex coagulation disorders. Factor XIII plays a crucial role in the last steps of haemostasis, and its deficiency is associated with an increased incidence of bleeding diathesis. However, current conventional coagulation tests cannot detect factor XIII deficiency. In this study, we examined factor XIII activity and the ability of rotational thromboelastometry to detect factor XIII deficiency and bleeding diathesis in patients with cirrhosis...
September 16, 2016: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/27615056/hemophilic-pseudotumor-in-a-non-hemophilic-patient-treated-with-a-hybrid-procedure-of-preoperative-embolization-of-the-feeding-arteries-followed-by-surgical-resection-a-case-report
#11
Sorcha Allen, Craig B Reeder, Mark J Kransdorf, Christopher P Beauchamp, Matthew A Zarka, Farouk Mookadam
INTRODUCTION: Hemophilic pseudotumor is a rare but well documented complication seen in approximately 1-2% of patients with hemophilia. The incidence continues to decrease, likely because of increasingly sophisticated techniques in managing factor deficiency. We present a case of hemophilic pseudotumor in a patient without hemophilia, an exceptionally rare entity, and outline a hybrid approach to treatment. PRESENTATION OF CASE: The patient presented with a left sided iliopsoas mass and associated radiculopathy, with a history of a poorly characterized bleeding diathesis and Noonan's syndrome...
2016: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/27609734/life-threatening-hemorrhage-from-acquired-hemophilia-a-as-a-presenting-manifestation-of-prostate-cancer
#12
Chirag Sheth, Amandeep Gill, Sumeet Sekhon
Acquired factor VIII deficiency (acquired hemophilia A) is a rare condition characterized by the acquisition of autoantibodies that affect the clotting activity of factor VIII (fVIII). The most common manifestation in affected patients is a hemorrhagic diathesis. This disorder is associated with autoimmune diseases, pregnancy, postpartum period, drugs, and malignancy. Management of this condition begins with attempts to arrest an acute bleed based on the site and severity of bleeding and inhibitor titer. The next priority is eradication of the fVIII antibodies using immunosuppressive therapies...
2016: Journal of Community Hospital Internal Medicine Perspectives
https://www.readbyqxmd.com/read/27599665/transcatheter-closure-of-pda-in-premature-babies-less-than-2-kg
#13
Nazmi Narin, Özge Pamukçu, Ali Baykan, Mustafa Argun, Abdullah Özyurt, Adnan Bayram, Kazım Üzüm
OBJECTIVE: Our hypothesis was that percutaneous PDA closure in babies less than 2 kg was a safe and effective method. The aim of this study is to share our experience in transcatheter PDA closure in infants whose body weight is less than 2 kg in order to support our hypothesis. METHODS: Between July 1997 and October 2014, 382 percutaneous PDA closures were done in our center. Nineteen patients who weighed less than 2 kg were included in this retrospectively study...
September 2, 2016: Anatolian Journal of Cardiology
https://www.readbyqxmd.com/read/27597910/intraperitoneal-hemorrhage-in-a-pregnant-woman-with-hyperemesis-gravidarum-vitamin-k-deficiency-as-a-possible-cause
#14
Yosuke Baba, Hiroyuki Morisawa, Koyomi Saito, Hironori Takahashi, Kazuma Rifu, Shigeki Matsubara
Hyperemesis gravidarum can cause various vitamin deficiencies. Vitamin K deficiency can lead to coagulopathy or hemorrhagic diathesis. A nulliparous Japanese woman with hyperemesis gravidarum at 10(5/7) weeks was admitted with giant myoma, intestinal obstruction, and abdominal pain. Treatment for a degenerative myoma was instituted with intravenous antibiotics. The abdominal pain ameliorated, but intestinal obstruction persisted. At 16(6/7) weeks, we performed laparotomy for release of intestinal obstruction, when intraabdominal bleeding of 110 mL existed...
2016: Case Reports in Obstetrics and Gynecology
https://www.readbyqxmd.com/read/27593200/ngs-based-100-gene-panel-of-hypopigmentation-identifies-mutations-in-chinese-hermansky-pudlak-syndrome-patients
#15
Aihua Wei, Yefeng Yuan, Dayong Bai, Jing Ma, Zhenhua Hao, Yingzi Zhang, Jiaying Yu, Zhiyong Zhou, Lin Yang, Xiumin Yang, Li Li, Wei Li
Hermansky-Pudlak syndrome (HPS) is a rare recessive disorder characterized by hypopigmentation, bleeding diathesis, and other symptoms due to multiple defects in lysosome-related organelles. Ten HPS subtypes have been identified with mutations in HPS1 to HPS10. Only four patients with HPS-1 have been reported in Chinese population. Using next-generation sequencing (NGS), we have screened 100 hypopigmentation genes and identified four HPS-1, two HPS-3, one HPS-5, and three HPS-6 in Chinese HPS patients with typical ocular or oculocutaneous albinism and the absence of platelet dense granules together with other variable phenotypes...
November 2016: Pigment Cell & Melanoma Research
https://www.readbyqxmd.com/read/27529121/pulmonary-fibrosis-in-hermansky-pudlak-syndrome
#16
Glenn W Vicary, Yeidyly Vergne, Alberto Santiago-Cornier, Lisa R Young, Jesse Roman
Hermansky-Pudlak syndrome (HPS) is a rare autosomal recessive genetic disorder characterized by oculocutaneous albinism and a bleeding diathesis due to platelet dysfunction. More than 50% of cases worldwide are diagnosed on the Caribbean island of Puerto Rico. Genetic testing plays a growing role in diagnosis; however, not all patients with HPS have identified genetic mutations. In Puerto Rico, patients with HPS are often identified shortly after birth by their albinism, although the degree of hypopigmentation is highly variable...
October 2016: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/27473743/prozone-effect-in-the-diagnosis-of-lupus-anticoagulant-for-the-lupus-anticoagulant-hypoprothrombinemia-syndrome
#17
Jing Jin, James L Zehnder
OBJECTIVES: The main clinical sequela of a lupus anticoagulant is increased thrombosis risk. However, bleeding due to lupus anticoagulant-hypoprothrombinemia syndrome is a rare but well-described manifestation of antiphospholipid syndrome. The association of acute acquired hypoprothrombinemia is caused by a lupus anticoagulant's specificity to prothrombin, which results in clearance of prothrombin and bleeding due to hypoprothrombinemia (usually <10% of normal). Severe life-threatening bleeding is most frequently reported in children with systemic lupus erythematosus or in healthy children after viral infection...
August 2016: American Journal of Clinical Pathology
https://www.readbyqxmd.com/read/27471233/impaired-von-willebrand-factor-adhesion-and-platelet-response-in-thrombospondin-2-knockout-mice
#18
Nina Kristofik, Nicole E Calabro, Weiming Tian, Aaron Meng, Susan MacLauchlan, Yinong Wang, Christopher K Breuer, George Tellides, Laura E Niklason, Themis R Kyriakides
Interactions between collagenous extracellular matrices and von Willebrand factor (VWF) are critical for hemostasis and thrombosis. In the present study, we investigated the contribution of an extracellular matrix (ECM) abnormality to the bleeding diathesis in thrombospondin-2 (TSP2) knockout (KO) mice. First, we performed adoptive bone marrow transplantation and observed that introduction of wild-type (WT) marrow into lethally irradiated TSP2 KO mice did not rescue the bleeding diathesis. However, platelets in transplanted mice displayed an inherent aggregation defect, which complicated interpretation...
September 22, 2016: Blood
https://www.readbyqxmd.com/read/27403770/light-transmission-aggregometry-does-not-correlate-with-the-severity-of-%C3%AE-granule-platelet-storage-pool-deficiency
#19
Gary M Woods, Elizabeth L Kudron, Kyle Davis, Joseph Stanek, Bryce A Kerlin, Sarah H O'Brien
Delta-granule platelet storage pool deficiency (δ-PSPD) is a poorly studied bleeding diathesis resulting from either decreased granule content or decreased average number of platelet δ-granules. Light transmission aggregometry (LTA) is commonly used to evaluate for δ-PSPD and platelet electron microscopy (EM) is used to confirm the diagnosis. Currently, little data exist examining the relationship between the likelihood of abnormal platelet aggregation findings, severity of δ-granule deficiency on platelet EM, and severity of bleeding symptoms in patients with δ-PSPD...
October 2016: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/27400185/mean-platelet-volume-and-mortality-risk-in-a-national-incident-hemodialysis-cohort
#20
Steven Kim, Miklos Z Molnar, Gregg C Fonarow, Elani Streja, Jiaxi Wang, Daniel L Gillen, Rajnish Mehrotra, Steven M Brunelli, Csaba P Kovesdy, Kamyar Kalantar-Zadeh, Connie M Rhee
BACKGROUND: Higher mean platelet volume (MPV) is an indicator of larger, reactive platelets, and has been associated with a higher risk of thrombosis and cardiovascular events in the general population. Hemodialysis patients have a higher risk for cardiovascular death and predisposition to platelet dysfunction (thrombosis and bleeding diathesis), but the relationship between MPV and mortality in this population is unknown. METHODS: Among a 5-year cohort (1/2007-12/2011) of 149,118 incident hemodialysis patients from a large national dialysis organization, we examined the association between MPV and all-cause mortality...
October 1, 2016: International Journal of Cardiology
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