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https://www.readbyqxmd.com/read/28211970/brain-hemorrhages-in-jacobsen-syndrome-a-retrospective-review-of-six-cases-and-clinical-recommendations
#1
Paul Grossfeld
Jacobsen syndrome is a rare chromosomal disorder caused by distal deletions in the long arm of chromosome 11. All patients with Jacobsen syndrome have Paris-Trousseau syndrome, a bleeding disorder that causes neonatal thrombocytopenia, and persistent platelet dysfunction. Despite that, to date there are no reported cases of hemorrhagic strokes occurring in patients with Jacobsen syndrome. In the last 6 years at least six cases of brain hemorrhages in patients with Jacobsen syndrome have occurred. In this report, we perform a retrospective review of these six cases...
March 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28210912/inhibitory-effects-of-emodin-thymol-and-astragalin-on-leptospira-interrogans-induced-inflammatory-response-in-the-uterine-and-endometrium-epithelial-cells-of-mice
#2
Wenlong Zhang, Xiaojie Lu, Wei Wang, Zhuang Ding, Yunhe Fu, Xiaofei Zhou, Naisheng Zhang, Yongguo Cao
Leptospirosis is a systemic infection that causes, among others, acute kidney injury, acute liver disease, muscle pain, vasculitis, bleeding disorders, and reproductive loss. In an effort to reduce uterine inflammatory responses induced by Leptospira, we evaluated the anti-inflammation effects of emodin, thymol, and astragalin in a mouse model. Our results showed that treatment with emodin, thymol, and astragalin alleviated uterine inflammation induced by leptospira infection via suppression of pro-inflammatory cytokine expression and prevented tissue damage...
February 16, 2017: Inflammation
https://www.readbyqxmd.com/read/28210642/high-output-heart-failure-contributing-to-recurrent-epistaxis-kiesselbach-area-syndrome-in-a-patient-with-hereditary-hemorrhagic-telangiectasia
#3
Venugopal Brijmohan Bhattad, Jennifer N Bowman, Hemang B Panchal, Timir K Paul
Hereditary hemorrhagic telangiectasia (HHT), also known as Osler-Weber-Rendu syndrome, is a rare genetic blood disorder that leads to abnormal bleeding due to absent capillaries and multiple abnormal blood vessels known as arteriovenous malformations. A feature of HHT is high-output heart failure due to multiple arteriovenous malformations. High-output heart failure can lead to recurrent epistaxis Kiesselbach area syndrome (REKAS), further exacerbating heart failure through increased blood loss and resultant anemia...
January 2017: Journal of Investigative Medicine High Impact Case Reports
https://www.readbyqxmd.com/read/28208056/oral-rehabilitation-of-a-patient-with-ectodermal-dysplasia-treated-with-fresh-frozen-bone-allografts-and-computer-guided-implant-placement-a-clinical-case-report
#4
Carlo Maiorana, Pier Paolo Poli, Carlo Poggio, Paola Barbieri, Mario Beretta
Ectodermal dysplasia (ED) is an inherited disorder characterized by abnormality of ectodermally derived structures. A recurrent oral finding is oligodontia, which in turn leads to a severely hypotrophic alveolar process with typical knife-edge morphology and adverse ridge contours. This unfavorable anatomy can seriously hamper proper implant placement. Fresh-frozen bone (FFB) allografts recently have been proposed to augment the residual bone volume for implant placement purposes; however, scientific evidence concerning the use of FFB to treat ED patients is absent...
January 20, 2017: Journal of Oral and Maxillofacial Surgery
https://www.readbyqxmd.com/read/28205029/stray-energy-transfer-during-endoscopy
#5
Edward L Jones, Amin Madani, Douglas M Overbey, Asimina Kiourti, Satheesh Bojja-Venkatakrishnan, Dean J Mikami, Jeffrey W Hazey, Todd R Arcomano, Thomas N Robinson
INTRODUCTION: Endoscopy is the standard tool for the evaluation and treatment of gastrointestinal disorders. While the risk of complication is low, the use of energy devices can increase complications by 100-fold. The mechanism of increased injury and presence of stray energy is unknown. The purpose of the study was to determine if stray energy transfer occurs during endoscopy and if so, to define strategies to minimize the risk of energy complications. METHODS AND PROCEDURES: A gastroscope was introduced into the stomach of an anesthetized pig...
February 15, 2017: Surgical Endoscopy
https://www.readbyqxmd.com/read/28203325/inherited-thrombocytopenia-with-a-different-type-of-gene-mutation-a-brief-literature-review-and-two-case-studies
#6
Mohammad Taghi Arzanian
Hereditary thrombocytopenias are rare bleeding disorders, which cause a deficiency of platelets in early infancy. This group of disorders is sometimes associated with abnormal phenotypes, like absence of radius. Diagnosis of this type of thrombocytopenia is usually difficult; other causes of thrombocytopenia, such as immune disorders and infections, must be ruled out. The symptoms of hereditary thrombocytopenia also vary from seldom and mild to severe bleeding and occasionally may first occur in late childhood...
October 2016: Iranian Journal of Pediatrics
https://www.readbyqxmd.com/read/28202865/repeated-diffuse-alveolar-hemorrhage-in-a-patient-with-hemophilia-b
#7
Hajime Kasai, Jiro Terada, Hiromasa Hoshi, Takashi Urushibara, Fumiaki Kato, Rintaro Nishimura, Koichiro Tatsumi
Diffuse alveolar hemorrhage (DAH) is a life-threatening complication that occurs in association with various diseases including coagulation disorders. In rare cases, it is caused by hemophilia. A 48-year-old man was admitted to our hospital for a third time due to DAH. Although the cause of DAH could not be identified by bronchoscopy or laboratory tests, a good response to corticosteroids suggested idiopathic DAH with pulmonary capillaritis. The patient was diagnosed with hemophilia B based on the results of a detailed inquiry, a mildly prolonged activated partial thromboplastin time, and low factor IX activity...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28198025/haemoglobin-responses-to-transfusion-in-severe-iron-deficiency-anaemia-potential-impact-of-gastrointestinal-disorders
#8
X Bosch, E Montori, M Guerra-García, J Costa-Rodríguez, M H Quintanilla, P E Tolosa-Chapasian, P Moreno, N Guasch, A López-Soto
BACKGROUND AND OBJECTIVES: Red blood cell (RBC) transfusion may be justified in iron deficiency anaemia (IDA) when an increase in oxygen delivery is needed, as sometimes occurs in subjects with haemoglobin <8·0 mg/dL, serious comorbidities or at risk of cardiovascular instability. Earlier investigations showed that some patients with severe IDA requiring transfusion had lower than expected post-transfusion haemoglobin levels with poorer clinical outcomes than other patients. After hypothesizing that haemoglobin responses to transfusion were different and that the underlying gastrointestinal (GI) disorders causing IDA could be a confounder explaining this association, these responses were analysed in a prospective cohort of IDA adults referred for outpatient GI investigation...
February 15, 2017: Vox Sanguinis
https://www.readbyqxmd.com/read/28194493/oral-health-of-adult-patients-undergoing-hematopoietic-cell-transplantation-pre-transplant-assessment-and-care
#9
REVIEW
Agnieszka Bogusławska-Kapała, Kazimierz Hałaburda, Ewa Rusyan, Hubert Gołąbek, Izabela Strużycka
Hematopoietic cell transplantation (HCT) is now one of the frequent procedures used for treatment of malignant and non-malignant blood diseases, autoimmune disorders, and certain solid tumors. Despite improvements of therapeutic protocols, HCT still carries a high risk of non-relapse mortality due to early and late complications. Side effects of the therapy regimen frequently occur in the oral cavity and often significantly decrease the patients' quality of life. The complications may result from or may be exacerbated by improper oral preparation of the patient before transplantation...
February 13, 2017: Annals of Hematology
https://www.readbyqxmd.com/read/28194178/role-of-helicobacter-pylori-eradication-therapy-on-platelet-recovery-in-chronic-immune-thrombocytopenic-purpura
#10
Khan Sheema, Ujjan Ikramdin, Naz Arshi, Naz Farah, Sheikh Imran
Background. Idiopathic thrombocytopenic purpura (ITP) is a bleeding disorder in which the immune system destroys native platelets. In this condition an autoantibody is generated against a platelet antigen. ITP affects women more often than men and is more common in children than adults. Objective. To assess the effect of Helicobacter pylori eradication therapy (HPET) on platelet count in Helicobacter pylori associated chronic immune thrombocytopenic purpura (chronic ITP) in adult. Materials and Methods. It is an interventional prospective study conducted at Liaquat University of Medical and Health Sciences, Jamshoro, from 2014 to 2015...
2017: Gastroenterology Research and Practice
https://www.readbyqxmd.com/read/28193763/neurologic-involvement-in-patients-with-atypical-chediak-higashi-disease
#11
Wendy J Introne, Wendy Westbroek, Catherine A Groden, Vikas Bhambhani, Gretchen A Golas, Eva H Baker, Tanya J Lehky, Joseph Snow, Shira G Ziegler, May Christine V Malicdan, David R Adams, Heidi M Dorward, Richard A Hess, Marjan Huizing, William A Gahl, Camilo Toro
OBJECTIVE: To delineate the developmental and progressive neurodegenerative features in 9 young adults with the atypical form of Chediak-Higashi disease (CHD) enrolled in a natural history study. METHODS: Patients with atypical clinical features, but diagnostically confirmed CHD by standard evaluation of blood smears and molecular genotyping, underwent complete neurologic evaluation, MRI of the brain, electrophysiologic examination, and neuropsychological testing...
14, 2017: Neurology
https://www.readbyqxmd.com/read/28188927/xanthohumol-isolated-from-humulus-lupulus-prevents-thrombosis-without-increased-bleeding-risk-by-inhibiting-platelet-activation-and-mtdna-release
#12
Guang Xin, Zeliang Wei, Chengjie Ji, Huajie Zheng, Jun Gu, Limei Ma, Wenfang Huang, Susan L Morris-Natschke, Jwu-Lai Yeh, Rui Zhang, Chaoyi Qin, Li Wen, Zhihua Xing, Yu Cao, Qing Xia, Ke Li, Hai Niu, Kuo-Hsiung Lee, Wen Huang
AIM: As the global population has reached 7 billion and the baby boom generation reaches old age, thrombosis has become the major contributor to the global disease burden. It has been reported that, in moderate doses, beer may protect against thrombosis. Xanthohumol (XN), an antioxidant, is found at high concentrations in hop cones (Humulus lupulus L.) and is a common ingredient of beer. Here, the aim of the present work was to investigate the effects of XN on antithrombotic and antiplatelet activities, and study its mechanism...
February 7, 2017: Free Radical Biology & Medicine
https://www.readbyqxmd.com/read/28187737/evaluation-of-a-web-based-registry-of-inherited-bleeding-disorders-a-descriptive-study-of-the-brazilian-experience-with-hemovidaweb-coagulopatias
#13
Suely Meireles Rezende, Silvia Helena Lacerda Rodrigues, Kelly Neves Pinheiro Brito, Diego Lima Quintino da Silva, Marcos Lázaro Santo, Bárbara de Jesus Simões, Guilherme Genovez, Helder Teixeira Melo, João Paulo Baccara Araújo, Danila Augusta Accioly Varella Barca
BACKGROUND: Inherited bleeding disorders (IBD) consist of a group of rare heterogeneous diseases, which require treatment for life. Management of these disorders is complex and costly. Therefore, good quality data of the affected population is crucial to guide policy planning. The aim of this manuscript is to describe the impact of a national, web-based registry - the Hemovidaweb Coagulopatias (HWC) - in the management of the IBD in Brazil. METHODS: The system was developed in PHP 5...
February 10, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28187047/is-extracorporeal-co2-removal-really-safe-and-less-invasive-observation-of-blood-injury-and-coagulation-impairment-during-ecco2r
#14
Johannes Kalbhenn, Nadine Neuffer, Barbara Zieger, Axel Schmutz
Extracorporeal CO2-Removal (ECCO2R) is promoted with attributes like "safe" and "less invasive" compared to (high-flow) veno-venous ECMO-Systems. With our experience in coagulation disorders during ECMO-therapy with this observational study we for the first time prospectively evaluate hemolysis and coagulation disorders during ECCO2R. Eight consecutive patients with predominant hypercapnic respiratory failure were treated with the Hemolung® Respiratory Assist System (RAS) (Alung-Technologies, Pittsburg, USA)...
February 7, 2017: ASAIO Journal: a Peer-reviewed Journal of the American Society for Artificial Internal Organs
https://www.readbyqxmd.com/read/28186870/total-hip-arthroplasty-via-the-direct-anterior-approach-with-kerboull-type-acetabular-reinforcement-device-for-an-elderly-female-with-factor-xi-deficiency
#15
Kei Sano, Yasuhiro Homma, Tomonori Baba, Jun Ando, Mikio Matsumoto, Hideo Kobayashi, Takahito Yuasa, Kazuo Kaneko
We present a case of successful and uncomplicated total hip arthroplasty with an acetabular reinforcement device in an elderly patient with hip osteoarthritis already diagnosed with factor XI deficiency, which is a very rare bleeding disorder and at high risk of post-operative haemorrhage, and it poses a substantial challenge to surgeons as a consequence of the specific risks of infection and fixation failure. Moreover, bone fragility in elderly patient increases potential risk of adverse event. Fresh frozen plasma was used to supplement factor XI activity...
2017: SICOT-J
https://www.readbyqxmd.com/read/28184956/-hemorrhagic-shock-general-principles
#16
T I Eiben, V Fuhrmann, B Saugel, S Kluge
Bleeding associated with hemorrhagic shock is often seen in emergency medical services or in the intensive care unit. Identifying the origin of the bleeding and additional disorders helps to determine the degree of the hemorrhagic shock. In order to be effective, the initial therapy until blood products are available needs to be differentiated to be effective in terms of hemodynamic stabilization and coagulation. Crystalloidal and colloidal solutions should be used carefully since those solutions bear a risk within themselves...
February 9, 2017: Der Internist
https://www.readbyqxmd.com/read/28184053/gastrointestinal-diseases-and-their-oro-dental-manifestations-part-4-peutz-jeghers-syndrome
#17
S E Korsse, M E van Leerdam, E Dekker
Peutz-Jeghers syndrome (PJS) is a rare, autosomal dominant inherited disorder, caused by germline mutations in the LKB1 tumour suppressor gene. It is clinically characterised by distinct perioral mucocutaneous pigmentations, gastrointestinal polyposis and an increased cancer risk in adult life. Hamartomatous polyps can develop already in the first decade of life and may cause various complications, including abdominal pain, bleeding, anaemia, and acute intestinal obstruction. Furthermore, patients have an increased risk for developing cancer, both in the gastrointestinal tract as in other organs...
February 10, 2017: British Dental Journal
https://www.readbyqxmd.com/read/28183693/prophylaxis-usage-bleeding-rates-and-joint-outcomes-of-hemophilia-1999-2010-a-surveillance-project
#18
Marilyn J Manco-Johnson, J Michael Soucie, Joan C Gill
This analysis of the United States Hemophilia Treatment Center Network and the Centers for Disease Control and Prevention surveillance registry assessed trends in prophylaxis use and its impact on key indicators of arthropathy across the life span among participants with severe hemophilia A. Data on demographics, clinical characteristics and outcomes were collected prospectively between 1999 and 2010 at annual clinical visits to 134 hemophilia treatment centers. Trends in treatment and outcomes were evaluated using cross-sectional and longitudinal analyses...
February 9, 2017: Blood
https://www.readbyqxmd.com/read/28183556/blood-loss-and-transfusion-requirements-with-epsilon-aminocaproic-acid-use-during-cranial-vault-reconstruction-surgery
#19
Mark E Thompson, Charles Saadeh, Phillip Watkins, Laszlo Nagy, Joshua Demke
OBJECTIVE: To determine whether epsilon-aminocaproic acid (EACA) load of 50 mg∙kg(-1) before skin incision, and infusion of 25 mg∙kg(-1)∙h(-1) until skin closure during cranial vault reconstruction (CVR) were associated with decreased estimated blood loss and transfusion requirements. BACKGROUND: Antifibrinolytic medications decrease bleeding and transfusion requirements during cardiothoracic and orthopedic surgeries with high blood loss, but practical reductions in blood loss and transfusion requirements have not been consistently realized in children undergoing CVR...
February 2017: Journal of Clinical Anesthesia
https://www.readbyqxmd.com/read/28182323/ibrutinib-associated-bleeding-pathogenesis-management-and-risk-reduction-strategies
#20
REVIEW
Joseph J Shatzel, Sven R Olson, Derrick L Tao, Owen J T McCarty, Alexey V Danilov, Thomas G DeLoughery
Ibrutinib is an irreversible inhibitor of Bruton's tyrosine kinase (Btk) that has proven to be an effective therapeutic agent for multiple B-cell mediated lymphoproliferative disorders. Ibrutinib, however, carries an increased bleeding risk compared to standard chemotherapy. Bleeding events range from minor mucocutaneous bleeding to life-threatening hemorrhage, due in large part to the effects of ibrutinib on several distinct platelet signaling pathways. There is currently minimal data to guide clinicians regarding the use of ibrutinib in patients at high risk for bleeding or on anticoagulant or antiplatelet therapy...
February 9, 2017: Journal of Thrombosis and Haemostasis: JTH
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