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https://www.readbyqxmd.com/read/28904675/von-willebrand-s-disease-case-report-and-review-of-literature
#1
Hanae Echahdi, Brahim El Hasbaoui, Mohamed El Khorassani, Aomar Agadr, Mohamed Khattab
Von Willebrand Disease (VWD) is the most common human inherited bleeding disorder due to a defect of Von Willebrand Factor (VWF), which a glycoprotein crucial for platelet adhesion to the subendothelium after vascular injury. VWD include quantitative defects of VWF, either partial (type 1 with VWF levels < 50 IU/dL) or virtually total (type 3 with undetectable VWF levels) and also qualitative defects of VWF (type 2 variants with discrepant antigenic and functional VWF levels). The most bleeding forms of VWD usually do not concern type 1 patients with the mildest VWF defects (VWF levels between 30 and 50IU/dL)...
2017: Pan African Medical Journal
https://www.readbyqxmd.com/read/28901996/intracranial-hemorrhage-in-congenital-bleeding-disorders
#2
Shadi Tabibian, Hoda Motlagh, Majid Naderi, Akbar Dorgalaleh
: Intracranial hemorrhage (ICH), as a life-threatening bleeding among all kinds of congenital bleeding disorders (CBDs), is a rare manifestation except in factor XIII (FXIII) deficiency, which is accompanied by ICH, early in life, in about one-third of patients. Most inherited platelet function disorders (IPFDs) are mild to moderate bleeding disorders that can never experience a severe bleeding as in ICH; however, Glanzmann's thrombasthenia, a common and severe inherited platelet function disorder, can lead to ICH and occasional death...
September 9, 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/28895772/flow-cytometry-based-platelet-function-testing-is-predictive-of-symptom-burden-in-a-cohort-of-bleeders
#3
Niklas Boknäs, Sofia Ramström, Lars Faxälv, Tomas L Lindahl
Platelet function disorders (PFDs) are common in patients with mild bleeding disorders (MBDs), yet the significance of laboratory findings suggestive of a PFD remain unclear due to the lack of evidence for a clinical correlation between the test results and the patient phenotype. Herein, we present the results from a study evaluating the potential utility of platelet function testing using whole-blood flow cytometry in a cohort of 105 patients undergoing investigation for MBD. Subjects were evaluated with a test panel comprising two different activation markers (fibrinogen binding and P-selectin exposure) and four physiologically relevant platelet agonists (ADP, PAR1-AP, PAR4-AP, and CRP-XL)...
September 12, 2017: Platelets
https://www.readbyqxmd.com/read/28838029/radionuclide-synovectomy-synoviorthesis-rs-in-patients-with-bleeding-disorders-a-review-of-patient-and-procedure-demographics-and-functional-outcomes-in-the-athndataset
#4
C McGuinn, D Cheng, D Aschman, S L Carpenter, R Sidonio, A Soni, M D Tarantino, A P Wheeler, A L Dunn
INTRODUCTION: Radionuclide synovectomy/synoviorthesis (RS) to manage proliferative synovitis in persons with bleeding disorders has been utilized for decades; however, aggregate US results are limited. AIM: To determine the prevalence of RS utilization, patient and procedure related demographics and functional outcomes in United States haemophilia treatment centres (HTCs). The ATHNdataset includes US patients with bleeding disorders who have authorized the sharing of their demographic and clinical information for research...
August 24, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28830606/thrombin-generation-assays-for-global-evaluation-of-the-hemostatic-system-perspectives-and-limitations
#5
Rita Carolina Figueiredo Duarte, Cláudia Natália Ferreira, Danyelle Romana Alves Rios, Helton José Dos Reis, Maria das Graças Carvalho
The existing techniques to evaluate hemostasis in clinical laboratories are not sensitive enough to detect hypercoagulable and mild hypocoagulable states. Under different experimental conditions, the thrombin generation test may meet these requirements. This technique evaluates the overall balance between procoagulant and anticoagulant forces and has provided new insights in our understanding of the coagulation cascade, as well as of the diagnosis of hypocoagulability and hypercoagulability conditions. Thrombin generated in the thrombin generation test can be quantified as platelet-rich or platelet-poor plasma using the calibrated automated thrombogram method, which monitors the cleavage of a fluorogenic substrate that is simultaneously compared to the known thrombin activity in a non-clotting plasma sample...
July 2017: Revista Brasileira de Hematologia e Hemoterapia
https://www.readbyqxmd.com/read/28819614/acute-compartment-syndrome-after-an-olecranon-fracture-in-a-patient-with-mild-hemophilia-b
#6
John M Reynolds, Christy Christophersen, Mary K Mulcahey
INTRODUCTION: Compartment syndrome is a serious condition characterized by compartmental pressures within 20 mmHg of diastolic blood pressure, or clinical signs of pain, paresthesia, pallor, and lack of pulses. Often a surgical intervention is necessary. Increased surveillance for compartment syndrome is important when a patient with a bleeding disorder sustains a traumatic injury. CASE REPORT: We present a case of forearm compartment syndrome in a patient with mild hemophilia B who sustained an olecranon fracture...
March 2017: Journal of Orthopaedic Case Reports
https://www.readbyqxmd.com/read/28804835/chromogenic-factor-viii-assays-for-improved-diagnosis-of-hemophilia-a
#7
Susan Rodgers, Elizabeth Duncan
Hemophilia A is an inherited bleeding disorder caused by a reduced level of factor VIII coagulant activity (FVIII:C) in blood. Bleeding episodes may occur spontaneously in the severe form of hemophilia or after trauma in the milder forms. It is important that patients are diagnosed correctly, which includes placing them into the correct severity category of the disorder so that appropriate treatment can be given. Diagnosis is made by determination of the amount of FVIII:C in the blood, usually using a one-stage factor VIII:C assay...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28797911/a-prospective-evaluation-of-radiation-free-direct-solitary-cholangioscopy-for-the-management-of-choledocholithiasis
#8
Monique T Barakat, Mohit Girotra, Abhishek Choudhary, Robert J Huang, Saurabh Sethi, Subhas Banerjee
BACKGROUND AND AIMS: Endoscopy has replaced many radiological studies for the GI tract. However, ERCP remains a hybrid endoscopic-fluoroscopic procedure, which limits its portable delivery, creates delays due to fluoroscopy room unavailability and exposes patients/providers to radiation. We evaluated fluoroscopy/radiation-free management of patients with non-complex choledocholithiasis using direct solitary cholangioscopy (DSC). METHODS: Patients underwent fluoroscopy-free biliary cannulation, sphincterotomy then cholangioscopy to establish location, number/size of stones and document distance from ampulla to bifurcation to guide balloon advancement...
August 7, 2017: Gastrointestinal Endoscopy
https://www.readbyqxmd.com/read/28762630/comparison-of-multiple-electrode-aggregometry-with-lumi-aggregometry-for-the-diagnosis-of-patients-with-mild-bleeding-disorders
#9
R Al Ghaithi, S Drake, S P Watson, N V Morgan, P Harrison
BACKGROUND: Multiple electrode aggregometry (MEA) measures changes in electrical impedance caused by platelet aggregation in whole blood. This approach is faster, more convenient and offers the advantage over light transmission aggregometry (LTA) of assessing platelet function in whole blood and reducing pre-analytical errors associated with preparation of platelet rich plasma (PRP). Several studies indicate the utility of this method in assessing platelet inhibition in individuals taking antiplatelet agents e...
August 1, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28743718/twinfilin-2a-is-a-regulator-of-platelet-reactivity-and-turnover-in-mice
#10
Simon Stritt, Sarah Beck, Isabelle C Becker, Timo Vögtle, Markku Hakala, Katrin G Heinze, Xiaoping Du, Markus Bender, Attila Braun, Pekka Lappalainen, Bernhard Nieswandt
Regulated reorganization of the actin cytoskeleton is a prerequisite for proper platelet production and function. Consequently, defects in proteins controlling actin dynamics have been associated with platelet disorders in humans and mice. Twinfilin 2a (Twf2a) is a small actin-binding protein, which inhibits actin filament assembly by sequestering actin monomers and by capping filament barbed ends. Moreover, Twf2a binds heterodimeric capping proteins, but the role of this interaction in cytoskeletal dynamics has remained elusive...
July 25, 2017: Blood
https://www.readbyqxmd.com/read/28720136/bilateral-multiple-pulmonary-artery-aneurysms-associated-with-cavitary-pulmonary-tuberculosis-a-case-report
#11
Pedro Pallangyo, Frederick Lyimo, Smita Bhalia, Hilda Makungu, Bashir Nyangasa, Flora Lwakatare, Pal Suranyi, Mohamed Janabi
BACKGROUND: Pulmonary artery aneurysms constitute <1% of aneurysms occurring in the thoracic cavity. Congenital cardiac defects are responsible for the majority (>50%) of cases, however, pulmonary artery aneurysm is a rare sequelae of pulmonary tuberculosis reported in about 5% of patients with chronic cavitary tuberculosis on autopsy. The natural history of this potentially fatal condition remains poorly understood and guidelines for optimal management are controversial. CASE PRESENTATION: A 24-year-old man, a nursing student of African descent, was referred to us from an up-country regional hospital with a 4-week history of recurrent episodes of breathlessness, awareness of heartbeats and coughing blood 3 weeks after completing a 6-month course of anti-tuberculosis drugs...
July 19, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28707410/treatment-of-an-acquired-factor-xiii-inhibitor-in-an-adolescent-with-systemic-lupus-erythematosus-and-renal-failure
#12
Cara A Rabik, Meredith A Atkinson, Sangeeta Sule, John J Strouse
BACKGROUND: Factor (F)XIII deficiency is a rare inherited bleeding disorder, but can also be acquired due to the development of inhibitors. CASE REPORT: A 17-year-old female with systemic lupus erythematosus and end-stage kidney disease secondary to Class IV lupus nephritis developed spontaneous subcutaneous and muscular hematomas and delayed major bleeding after invasive procedures. She had abnormal kaolin thromboelastography (kTEG; decreased maximal amplitude, representative of clot strength) initially attributed to thrombocytopenia and uremic platelet dysfunction, but her FXIII activity was undetectable, and a high-titer antibody against FXIII was identified...
July 13, 2017: Transfusion
https://www.readbyqxmd.com/read/28668647/toxicological-evaluation-of-the-aqueous-whole-plant-extract-of-aerva-lanata-l-juss-ex-schult-amaranthaceae
#13
Kayode S Omotoso, Flora R Aigbe, Olanrewaju A Salako, Micah C Chijioke, Oluwafunmilayo O Adeyemi
ETHNOPHARMACOLOGICAL RELEVANCE: Aerva lanata (L.) of the family Amaranthaceae is a Nigerian medicinal plant used traditionally for the management of lithiasis, headache, renal disorder, haematemesis, bronchitis, nasal bleeding, cough, scorpion stings, fractures and spermatorrhoea. Studies that show the pharmacological basis for some of such uses have been reported. There is, however, no scientific report on its toxicity profile to the best of our knowledge. AIM OF THE STUDY: This study was therefore aimed at investigating the toxicity profile of the aqueous extract of Aerva lanata...
June 28, 2017: Journal of Ethnopharmacology
https://www.readbyqxmd.com/read/28637620/runx1-deficiency-familial-platelet-disorder-with-predisposition-to-myeloid-leukemia-fpdmm
#14
REVIEW
Brigitte Schlegelberger, Paula G Heller
In this review, we discuss disease-causing alterations of RUNT-related transcription factor 1 (RUNX1), a master regulator of hematopoietic differentiation. Familial platelet disorder with predisposition to myeloid leukemia (FPDMM) typically presents with (1) mild to moderate thrombocytopenia with normal-sized platelets; (2) functional platelets defects leading to prolonged bleeding; and (3) an increased risk to develop myelodysplastic syndromes (MDS), acute myeloid leukemia (AML), or T-cell acute lymphoblastic leukemia (T-ALL)...
April 2017: Seminars in Hematology
https://www.readbyqxmd.com/read/28634189/reduced-platelet-count-but-no-major-platelet-function-abnormalities-are-associated-with-loss-of-function-atp-binding-cassette-1-gene-mutations
#15
REVIEW
Pietro Minuz, Alessandra Meneguzzi, Eti Alessandra Femia, Cristiano Fava, Stefano Calabria, Mariangela Scavone, Donatella Benati, Giovanni Poli, Carlo Zancanaro, Sebastiano Calandra, Tiziano Lucchi, Marco Cattaneo
Loss-of-function mutations of the the ATP-binding cassette-1 (ABCA1) gene are the cause of Tangier disease (TD) in homozygous subjects and familial HDL deficiency (FHD) in heterozygous subjects. These disorders are characterized by reduced plasma HDL-cholesterol (HDL-C) and altered efflux of cholesterol from cells. Previous studies in TD patients and ABCA1(-/-) murine models reported defects in platelet count, morphology, and function, but the issue is still controversial. We analyzed three subjects with low to very low HDL-C levels due to the loss-of-function mutations of the ABCA1 gene...
August 15, 2017: Clinical Science (1979-)
https://www.readbyqxmd.com/read/28600843/mortality-caused-by-intracranial-bleeding-in-non-severe-hemophilia-a-patients-comment
#16
LETTER
R Patil, K Ghosh, S Shetty
No abstract text is available yet for this article.
June 10, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28600043/placenta-associated-pregnancy-complications-in-pregnancies-complicated-with-placenta-previa
#17
Yael Baumfeld, Reli Herskovitz, Zehavi Bar Niv, Salvatore Andrea Mastrolia, Adi Y Weintraub
OBJECTIVES: The purpose of our study was to examine the hypothesis that pregnancies complicated with placenta previa have an increased risk of placental insufficiency associated pregnancy complications (IUGR, preeclampsia, placental abruption and perinatal mortality). MATERIALS AND METHODS: Our study included all deliveries that occurred at Soroka University Medical Center (Beer Sheva, Israel) between January 1998 and December 2013. Of them 1,249 were complicated by placenta previa and represented our study group...
June 2017: Taiwanese Journal of Obstetrics & Gynecology
https://www.readbyqxmd.com/read/28583924/congenital-hypofibrinogenaemia-a-presymptomatic-detection-of-an-extremely-rare-bleeding-disorder-in-preterm-twins
#18
Catherine Mary Breen, Muhammad I Riazat, Naomi McCallion, Michael A Boyle
Twenty-eight-week-old preterm monochorionic-diamniotic twins were admitted to the neonatal intensive care unit secondary to low birth weight and mild respiratory distress syndrome. A low fibrinogen level of less than 0.5 g/L was detected following an abnormal full blood count. They required fibrinogen transfusions until 32 weeks corrected gestation to maintain adequate fibrinogen levels. Parental screening revealed that their mother had a previously undiagnosed hypofibrinogenaemia. Of note, her only symptom was menorrhagia...
June 5, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28581691/recombinant-factor%C3%A2-xiii-prophylaxis-is-safe-and-effective-in-young-children-with-congenital-factor-xiii-a-deficiency-international-phase%C3%A2-3b-trial-results
#19
B A Kerlin, A Inbal, A Will, M Williams, M-L Garly, L Jacobsen, S L Kearney
Essentials Prophylaxis is the standard of care for congenital factor XIII-A (FXIII-A) deficiency. Six children with FXIII-A deficiency received once-monthly prophylaxis with recombinant FXIII-A. Prophylaxis was well tolerated and no anti-FXIII antibodies were detected. Prophylaxis was effective with an annualized bleeding rate of zero. SUMMARY: Background Factor XIII deficiency is a rare, severe congenital bleeding disorder. Monthly prophylaxis with recombinant FXIII A-Subunit (rFXIII) has demonstrated favorable safety and efficacy in patients aged ≥ 6 years, and may similarly benefit younger children...
June 5, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28555414/germline-etv6-mutations-and-predisposition-to-hematological-malignancies
#20
REVIEW
Simone Feurstein, Lucy A Godley
Patients with thrombocytopenia 5 have an autosomal dominant disorder of decreased platelet number with tendency to bleed, usually presenting in childhood, and have been found to have germline mutations in ETV6, which encodes a master hematopoietic transcription factor. Some patients who present similarly have inherited mutations in RUNX1 or ANKRD26. All three germline syndromes are also associated with a predisposition to myelodysplastic syndrome (MDS) and acute leukemia (AL). Since the first description of germline ETV6 mutations, 18 families have been reported...
August 2017: International Journal of Hematology
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