keyword
MENU ▼
Read by QxMD icon Read
search

Bleeding disorders

keyword
https://www.readbyqxmd.com/read/29773783/cholesteryl-ester-storage-disease-fatal-outcome-without-causal-therapy-in-a-female-patient-with-the-preventable-sequelae-of-progressive-liver-disease-after-many-years-of-mild-symptoms
#1
Ali Canbay, Meike N Müller, Stathis Philippou, Guido Gerken, Andreas Tromm
BACKGROUND Cholesteryl ester storage disease (CESD), also known as lysosomal acid lipase deficiency (LAL-D), is a rare autosomal-recessive inheritable lysosomal storage disease. Since 2015, a causal treatment with sebelipase alfa, which replaces the missing LAL enzyme, has been approved. We report a fatal course of LAL-D in a female patient. CASE REPORT In 1979, CESD was first diagnosed in a 13-year-old female with marked hepatomegaly. At that time, no specific treatment for CESD was available and the spontaneous course of the disease had to be awaited...
May 18, 2018: American Journal of Case Reports
https://www.readbyqxmd.com/read/29772593/-coagulation-monitoring-and-bleeding-management-in-cardiac-surgery
#2
Berthold Bein, Robert Schiewe
The transfusion of allogeneic blood products is associated with increased morbidity and mortality. An impaired hemostasis is frequently found in patients undergoing cardiac surgery and may in turn cause bleeding and transfusions. A goal directed coagulation management addressing the often complex coagulation disorders needs sophisticated diagnostics. This may improve both patients' outcome and costs. Recent data suggest that coagulation management based on a rational algorithm is more effective than traditional therapy based on conventional laboratory variables such as PT and INR...
May 2018: Anästhesiologie, Intensivmedizin, Notfallmedizin, Schmerztherapie: AINS
https://www.readbyqxmd.com/read/29770858/-management-of-anticoagulants-in-ophthalmic-surgery-a-survey-among-ophthalmic-surgeons-in-germany
#3
N Feltgen, B Mele, T Dietlein, C Erb, A Eckstein, A Hager, A Heiligenhaus, H Helbig, H Hoerauf, E Hoffmann, D Pauleikhoff, M Schittkowski, B Seitz, C Sucker, S Suffo, U Schaudig, F Tost, S Thurau, P Walter, J Koscielny
INTRODUCTION: As our population ages and comorbidities rise, ophthalmic surgeons are increasingly faced with patients on anticoagulant therapy or with clotting disorders. The ophthalmic surgeon has to weigh the perioperative risk of haemorrhage when anticoagulation continues against the risk of thromboembolism caused by discontinuation or changing the patient's medication (bridging, switching, cessation). There are currently no guidelines or recommendations. METHODS: A survey was sent to the DOG (German Ophthalmologic Society) divisions and associated surgical organizations to determine the status quo...
May 16, 2018: Der Ophthalmologe: Zeitschrift der Deutschen Ophthalmologischen Gesellschaft
https://www.readbyqxmd.com/read/29769259/novel-therapeutics-for-hemophilia-and-other-bleeding-disorders
#4
Michael U Callaghan, Robert Sidonio, Steven W Pipe
Hemophilia and von Willebrand disease are the most common congenital bleeding disorders. Treatment for these disorders has focused on replacement of the missing coagulation factor to prevent or treat bleeding. New technologies and insights into hemostasis have driven the development of many promising new therapies for hemophilia and von Willebrand disease. Emerging bypass agents including zymogen-like factor IXa and Xa molecules are in development and a bispecific antibody, emicizumab, demonstrated efficacy in a phase III trial in people with hemophilia A and inhibitors...
May 16, 2018: Blood
https://www.readbyqxmd.com/read/29766740/factors-predictive-of-postoperative-acute-respiratory-failure-following-inpatient-sinus-surgery
#5
Brittany N Burton, Sapideh Gilani, Matthew W Swisher, Richard D Urman, Ulrich H Schmidt, Rodney A Gabriel
OBJECTIVE: The impact of perioperative risk factors on outcomes following outpatient sinus surgery is well defined; however, risk factors and outcomes following inpatient surgery remain poorly understood. We aimed to define risk factors of postoperative acute respiratory failure following inpatient sinus surgery. METHODS: Utilizing data from the Nationwide Inpatient Sample Database from the years 2010 to 2014, we identified patients (≥18 years of age) with an Internal Classification of Disease, Ninth Revision, Clinical Modification (ICD-9-CM) procedure code of sinus surgery...
May 1, 2018: Annals of Otology, Rhinology, and Laryngology
https://www.readbyqxmd.com/read/29765293/recurrent-bleedings-in-newborn-a-factor-vii-deficiency-case-report
#6
Kim Cattivelli, Cristina Distefano, Lorenza Bonetti, Sophie Testa, Simona Maria Siboni, Alessandro Plebani, Carlo Poggiani
Background: Major hemorrhages in newborns can be caused by several conditions, and knowledge of the differential diagnosis is essential in order to ensure prompt recognition and appropriate treatment. Case Report: We describe the case of a male newborn experiencing recurrent hemorrhages from the first days of life. Laboratory findings showed normal platelet count, hepatic function, and C-reactive protein. Coagulation tests detected an isolated prothrombin time (PT) prolongation and severe factor VII (FVII) deficiency...
April 2018: Transfusion Medicine and Hemotherapy
https://www.readbyqxmd.com/read/29762905/pharmacokinetics-and-the-transition-to-extended-half-life-factor-concentrates-communication-from-the-ssc-of-the-isth
#7
Margaret V Ragni, Stacy E Croteau, Massimo Morfini, Marjon H Cnossen, Alfonso Iorio
Extended half-life proteins (EHL) are increasingly used in clinical practice, but there is no standardized approach to sampling, interpretation, and implementation of pharmacokinetics (PK) data to maximize treatment benefit. The goal of EHL treatment is to attain a trough level sufficient to protect against spontaneous bleeds and reduce infusion frequency and limitations on individual activity and lifestyle. Performing classical PK assessments requires multiple blood samples which is burdensome for patients and providers...
May 15, 2018: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/29762845/peritoneal-dialysis-effectively-removes-toxic-substances-and-improves-liver-functions-of-liver-failure-patients
#8
W-X Zhao, X-M Liu, C-M Yu, H Xu, J-R Dai, H-Y Chen, L Li, F Chen, Y-L Ou, Z-K Zhao
OBJECTIVE: Liver failure (LF) is a clinically complex disorder that characterizes with hepatic dysfunction. This study aimed at observing the therapeutic effects of peritoneal dialysis on liver function in LF patients. PATIENTS AND METHODS: This study involves 62 patients diagnosed as LF hospitalized from February 2005 to December 2016. The 62 LF patients were randomly divided into 3 groups, including artificial liver applying plasma exchange group (PE, n = 28), peritoneal dialysis group (PD, n = 22), and conservative treatment group (CT, n=12)...
April 2018: European Review for Medical and Pharmacological Sciences
https://www.readbyqxmd.com/read/29760930/prevalence-of-thrombocytopenia-before-and-after-initiation-of-haart-among-hiv-infected-patients-at-black-lion-specialized-hospital-addis-ababa-ethiopia-a-cross-sectional-study
#9
Gashaw Garedew Woldeamanuel, Diresibachew Haile Wondimu
Background: Hematological abnormalities are common in HIV positive patients. Of these, thrombocytopenia is a known complication which has been associated with a variety of bleeding disorders. However, its magnitude and related factors have not been well-characterized in the era of highly active antiretroviral therapy (HAART) in Ethiopia. Therefore, this study aimed to assess the prevalence of thrombocytopenia before and after initiation of HAART among HIV positive patients attending Black Lion Specialized Hospital, Addis Ababa, Ethiopia...
2018: BMC Hematology
https://www.readbyqxmd.com/read/29760702/emerging-concepts-in-immune-thrombocytopenia
#10
REVIEW
Maurice Swinkels, Maaike Rijkers, Jan Voorberg, Gestur Vidarsson, Frank W G Leebeek, A J Gerard Jansen
Immune thrombocytopenia (ITP) is an autoimmune disease defined by low platelet counts which presents with an increased bleeding risk. Several genetic risk factors (e.g., polymorphisms in immunity-related genes) predispose to ITP. Autoantibodies and cytotoxic CD8+ T cells (Tc) mediate the anti-platelet response leading to thrombocytopenia. Both effector arms enhance platelet clearance through phagocytosis by splenic macrophages or dendritic cells and by induction of apoptosis. Meanwhile, platelet production is inhibited by CD8+ Tc targeting megakaryocytes in the bone marrow...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29758989/the-double-hazard-of-bleeding-and-thrombosis-in-hemostasis-from-a-clinical-point-of-view-a-global-assessment-by-rotational-thromboelastometry-rotem
#11
Olga Meltem Akay
Hemostasis is a complex dynamic process involving bleeding and thrombosis as two end-points. Conventional coagulation tests which are measured in plasma examine only isolated portions of the coagulation cascade, thereby giving no information on important interactions essential to the clinical evaluation of hemostatic function. Thromboelastography (TEG), originally described in 1948 has improved over the decades and become a valuable tool of coagulation testing because of the limitations of standard coagulation tests...
January 1, 2018: Clinical and Applied Thrombosis/hemostasis
https://www.readbyqxmd.com/read/29758592/comparison-of-a-therapeutic-only-versus-prophylactic-platelet-transfusion-policy-for-people-with-congenital-or-acquired-bone-marrow-failure-disorders
#12
REVIEW
Reem Malouf, Asma Ashraf, Andreas V Hadjinicolaou, Carolyn Doree, Sally Hopewell, Lise J Estcourt
BACKGROUND: Bone marrow disorders encompass a group of diseases characterised by reduced production of red cells, white cells, and platelets, or defects in their function, or both. The most common bone marrow disorder is myelodysplastic syndrome. Thrombocytopenia, a low platelet count, commonly occurs in people with bone marrow failure. Platetet transfusions are routinely used in people with thrombocytopenia secondary to bone marrow failure disorders to treat or prevent bleeding. Myelodysplastic syndrome is currently the most common reason for receiving a platelet transfusion in some Western countries...
May 14, 2018: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/29758336/grand-rounds-hepatic-manifestations-of-telomere-biology-disorders
#13
REVIEW
Mrinal M Patnaik, Patrick S Kamath, Douglas A Simonetto
Clinical Case A 51 year old Caucasian male was referred for evaluation of variceal bleeding. Laboratory tests were remarkable for mild thrombocytopenia and moderate alkaline phosphatase elevation. Synthetic liver function was well preserved. Abdominal computed tomography scan revealed moderate splenomegaly, gastric varices, and normal hepatic contour. A transjugular liver biopsy was performed revealing findings of nodular regenerative hyperplasia (NRH) with no significant fibrosis or necroinflammatory activity...
May 11, 2018: Journal of Hepatology
https://www.readbyqxmd.com/read/29751951/biochemical-characterization-and-immunogenicity-of-neureight-a-recombinant-full-length-factor-viii-produced-by-fed-batch-process-in-disposable-bioreactors
#14
Sandrine Delignat, Ivan Peyron, Maria El Ghazaly, Srinivas V Kaveri, Jan Rohde, Frank Mueller, Sebastien Lacroix-Desmazes
Hemophilia A is a X-linked recessive bleeding disorder consecutive to the lack of circulating pro-coagulant factor VIII (FVIII). The most efficient strategy to treat or prevent bleeding in patients with hemophilia A relies on replacement therapy using exogenous FVIII. Commercially available recombinant FVIII are produced using an expensive perfusion technology in stainless steel fermenters. A fed-batch fermentation technology was recently developed to produce 'Neureight', a full-length recombinant human FVIII, in Chinese hamster ovary (CHO) cells...
May 5, 2018: Cellular Immunology
https://www.readbyqxmd.com/read/29750671/successful-treatment-of-severe-menorrhagia-at-menarche-with-recombinant-factor-viia-in-an-adolescent-girl-with-type-iii-von-willebrand-s-disease
#15
Dilek Gurlek Gokcebay, Vildan Culha, Nese Yarali, Namik Yasar Ozbek
: Type III von Willebrand's disease (vWD) is an inherited bleeding disorder, which is frequently associated with menorrhagia in women. Treatment options include antifibrinolytics, desmopressin, von Willebrand factor/factor VIII concentrates and in intractable bleeding circumstances recombinant factor VIIa (rFVIIa). We present an adolescent case with type III vWD who had a menorrhagia at menarche that was refractory to the standard treatment and ultimately was treated with rFVIIa successfully.
May 9, 2018: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/29745623/the-role-of-intraoperative-stroke-volume-variation-on-bleeding-during-functional-endoscopic-sinus-surgery
#16
Roberta Di Mauro, Fabiana Lucci, Federica Martino, Maria B Silvi, Eleonora Gidaro, Santo Di Lorenzo, Nicola Toschi, Stefano Di Girolamo, Mario Dauri
BACKGROUNDː Functional Endoscopic Sinus Surgery (FESS) is a minimally-invasive surgical technique for patients with paranasal sinus pathology. Surgical bleeding reduces operative field visibility and increases the incidence of serious complications. Epinephrine injection into the nasal mucosa and controlled hypotension are used to minimise bleeding. Hypotension carries risks and sometimes does not reduce surgical bleeding. The goal of this study is to discover which haemodynamic parameter better correlates with surgical bleeding...
May 9, 2018: Minerva Anestesiologica
https://www.readbyqxmd.com/read/29744355/herbal-medicine-for-oligomenorrhea-and-amenorrhea-a-systematic-review-of-ancient-and-conventional-medicine
#17
REVIEW
Arezoo Moini Jazani, Kobra Hamdi, Mojgan Tansaz, Hossein Nazemiyeh, Homayoun Sadeghi Bazargani, Seyed Mohammad Bagher Fazljou, Ramin Nasimi Doost Azgomi
Introduction: Menstrual bleeding cessation is one of the most frequent gynecologic disorders among women in reproductive age. The treatment is based on hormone therapy. Due to the increasing request for alternative medicine remedies in the field of women's diseases, in present study, it was tried to overview medicinal plants used to treat oligomenorrhea and amenorrhea according to the pharmaceutical textbooks of traditional Persian medicine (TPM) and review the evidence in the conventional medicine...
2018: BioMed Research International
https://www.readbyqxmd.com/read/29743402/-durable-remission-attained-with-rituximab-therapy-in-a-patient-with-acquired-von-willebrand-syndrome-associated-with-cd20-positive-lymphoproliferative-disorder
#18
Honami Kurahashi, Yoshinari Kawabata, Yoshihiro Michishita, Atsushi Kitabayashi, Takahiro Kobayashi, Akihiro Kitadate, Naoto Takahashi
A 61-year-old female with no history of bleeding was admitted to our hospital owing to persistent bleeding after the left knee joint injection and activated partial thromboplastin time prolongation. Subsequent coagulation tests revealed a critically declined level of the von Willebrand factor (VWF) antigen (<10%) and activity (<10%) measurement besides a significantly declined factor VIII activity (4%). Despite diagnosing her with acquired von Willebrand syndrome (AvWS) and managing her bleeding with desmopressin acetate hydrate (DDAVP), we could not precisely make a definitive diagnosis the underlying disorder...
2018: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/29743396/-congenital-factor-v-and-factor-viii-deficiency-discovered-in-an-elderly-patient-with-abnormal-bleeding-after-trauma
#19
Yoshiyuki Ogawa, Kunio Yanagisawa, Yuri Uchiyama, Akira Matsumoto, Madoka Inoue, Kohtaro Toyama, Yuri Miyazawa, Naomichi Matsumoto, Hiroshi Handa
Congenital combined deficiency of coagulation factor V (FV) and factor VIII (FVIII) (F5F8D) is a rare autosomal recessive bleeding disorder caused by mutations in lectin mannose-binding type 1 (LMAN1) or multiple coagulation factor deficiency 2 (MCFD2) encoding chaperone molecules involved in the intracellular transport of FV and FVIII. Here, we report a case of F5F8D in an elderly patient diagnosed with hematoma after a right thigh injury. A 71-year-old male had a history of abnormal bleeding after tooth extraction and cholecystectomy...
2018: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/29742318/a-comparative-analysis-of-different-automated-von-willebrand-factor-glycoprotein-ib-binding-activity-assays-in-well-typed-von-willebrand-disease-patients
#20
I Vangenechten, K Mayger, P Smejkal, O Zapletal, J J Michiels, G W Moore, A Gadisseur
BACKGROUND: von Willebrand Disease (VWD) is an inherited bleeding disorder caused by quantitative (type 1, 3) or qualitative (type 2) von Willebrand factor (VWF) defect. VWD diagnosis and classification require numerous laboratory tests. VWF: Glycoprotein Ib (GPIb)-binding activity assays are used to distinguish type 1 from type 2 VWD. OBJECTIVES: Three different automated VWF:GPIb-binding activity assays were compared. PATIENTS AND METHODS: BC-VWF:RCo (Siemens Healthcare Diagnostics), HemosIL® VWF:RCo (Instrumentation Laboratory) and INNOVANCE® VWF:Ac (Siemens Healthcare Diagnostics) were performed in a well typed VWD cohort (n=142)...
May 9, 2018: Journal of Thrombosis and Haemostasis: JTH
keyword
keyword
16635
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"