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hemophilia review

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https://www.readbyqxmd.com/read/29147591/upper-limb-deep-vein-thrombosis-in-patient-with-hemophilia-a-and-heterozygosity-for-prothrombin-g20210a-a-case-report-and-review-of-the-literature
#1
Fares Darawshy, Yosef Kalish, Issam Hendi, Ayman Abu Rmelieh, Tawfik Khoury
Deep vein thrombosis (DVT) is a rare disease in patients with hemophilia A. We report a case of 22-year-old male with severe hemophilia A who presented to the emergency room with 5-day history of right arm pain that was attributed initially to bleeding event. In the absence of external signs of bleeding or hematoma and normal hemoglobin level, we suspected an underlying DVT. Doppler ultrasonography of the right upper limb revealed thrombosis of the subclavian vein and this was confirmed by CT venography. The d-dimer level was normal and investigations for prothrombotic state revealed heterozygosity for prothrombin G20210A mutation...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/29145340/open-repair-of-intraoperative-popliteal-artery-injury-during-total-knee-arthroplasty-in-a-patient-with-severe-hemophilia-a-a-case-report-and-literature-review
#2
Bin Feng, Ke Xiao, Jiang Shao, Yu Fan, Xisheng Weng
RATIONALE: Arterial injuries during total knee arthroplasty (TKA) though rare may occur. Hemophilia may further challenge the management of such injury. PATIENT CONCERNS: A 48-year-old male patient with severe hemophilia type A and stiff knee arthropathy underwent bilateral TKAs. Left popliteal artery injury was detected at the end of the left TKA. DIAGNOSES: Urgent angiography confirmed the diagnosis of the left popliteal artery transection...
November 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29144625/acquired-hemophilia-presenting-as-gross-hematuria-following-kidney-stone-a-case-report-and-review-of-the-literature
#3
Max Schmidt-Bowman, Lael Reinstatler, Eric P Raffin, Joseph E Yared, John D Seigne, Einar F Sverrisson
A rare condition in itself, acquired hemophilia A, seldom presents as isolated gross hematuria. It is a serious condition with a high mortality rate and thus clinical suspicion followed by prompt diagnosis is imperative (1). In fact, only 8 cases of such presentation of this condition have been reported thus far in the literature. Of these, none describe the initial presentation of hematuria with the inciting event of a kidney stone. We present a case of a 67-year-old man with signs and symptoms of nephrolithiasis accompanied by profuse hematuria, who was subsequently found to have developed expression of factor VIII inhibitor leading to acquired hemophilia A...
November 19, 2017: International Braz J Urol: Official Journal of the Brazilian Society of Urology
https://www.readbyqxmd.com/read/29095073/surgical-management-of-haemophilic-pseudotumors-experience-in-a-developing-country
#4
Yu He, Xi Zhou, Haomin Cui, Guixing Qiu, Xisheng Weng, Baozhong Zhang, Yong Liu
AIM: Hemophilic pseudotumors result from repeated episodes of bleeding into bone, subperiosteum, and soft tissue. Since clotting factors became available, uncontrolled perioperative bleeding is a less significant problem for surgeons in developed countries. However, they are more difficult to come by in China. Additionally, patients often have to undergo surgery for giant masses and suffer complications. We wanted to present our experience in the surgical management of hemophilic pseudotumors over a 40-year period...
November 2, 2017: Journal of Investigative Surgery: the Official Journal of the Academy of Surgical Research
https://www.readbyqxmd.com/read/29051801/current-and-emerging-factor-viii-replacement-products-for-hemophilia-a
#5
REVIEW
Lorraine A Cafuir, Christine L Kempton
Hemophilia A is a congenital X-linked bleeding disorder caused by coagulation factor VIII (FVIII) deficiency. Routine infusion of factor replacement products is the current standard of care; however, the development of alloantibodies against FVIII remains a challenge. The treatment of hemophilia has undergone major advances over the past century to improve safety, effectiveness, manufacturing, and convenience of factor products. Major recent advances in the treatment of hemophilia A include the emergence of extended half-life products, factor VIII orthologs, and gene therapy products...
October 2017: Therapeutic Advances in Hematology
https://www.readbyqxmd.com/read/29050497/simoctocog-alfa-for-the-treatment-of-hemophilia-a
#6
Massimo Morfini
Hemophilia A is the most frequent inherited bleeding disorder and most challenging coagulation disorder. To combat this, a number of new improved rFVIII/IX concentrates have recently been approved. Some of them are derived from protein fusion biotechnology or pegylation to extend their half-life (HL). However, prophylaxis has become a standard of care to prevent arthropathy in hemophiliacs though the need of frequent venipunctures is a major obstacle to primary prophylaxis. The new Extended Half-Life (EHL) rFIX concentrates allow increased intervals, while the improved HL of new rFVIII was moderate...
October 20, 2017: Expert Opinion on Biological Therapy
https://www.readbyqxmd.com/read/29019885/two-incision-laparoscopic-appendectomy-for-a-severe-hemophilia-a-child-patient-with-coagulation-factor-vii-deficiency-case-report-and-review-of-literature
#7
Jin Peng He, Jie Xiong Feng
RATIONALE: The main complication of patients with severe hemophilia is recurrent bleeding events that usually affected musculoskeletal contractures. And replacement therapy methods were continuously improved to minimize adverse impacts brought by those complications. However, only several cases reported about the appendectomy for hemophilia A. We report a case of acute appendicitis treated by two-incision laparoscopy in a boy with hemophilia A and coagulation factor VII deficiency for the first time...
October 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28969551/aptamer-oligonucleotides-as-potential-therapeutics-in-hematologic-diseases
#8
Weibin Li, Meng Zhao, Kaiyu Wang, Huihui Yan, XIaopeng Lan
Aptamers are single-stranded DNA or RNA oligonucleotides generated by a novel in vitro selection technique termed Systematic Evolution of Ligands by Exponential Enrichment (SELEX). During the past two decades, various aptamer drugs have been developed and many of them have entered into clinical trials. In the present review, we focus on aptamers as potential therapeutics for hematological diseases, including anemia of chronic inflammation (ACI) and anemia of chronic disease (ACD), hemophilia, thrombotic thrombocytopenic purpura (TTP) or VWD type-2B, and sickle cell disease (SCD), in particular, those that have entered into clinical trials...
October 2, 2017: Mini Reviews in Medicinal Chemistry
https://www.readbyqxmd.com/read/28959338/implementation-of-plasma-fractionation-in-biological-medicines-production
#9
REVIEW
Kamran Mousavi Hosseini, Mehran Ghasemzadeh
CONTEXT: The major motivation for the preparation of the plasma derived biological medicine was the treatment of casualties from the Second World War. Due to the high expenses for preparation of plasma derived products, achievement of self-sufficiency in human plasma biotechnological industry is an important goal for developing countries. EVIDENCE ACQUISITION: The complexity of the blood plasma was first revealed by the Nobel Prize laureate, Arne Tiselius and Theodor Svedberg, which resulted in the identification of thousands of plasma proteins...
December 2016: Iranian journal of biotechnology
https://www.readbyqxmd.com/read/28944952/bypassing-agent-prophylaxis-in-people-with-hemophilia-a-or-b-with-inhibitors
#10
REVIEW
Chatree Chai-Adisaksopha, Sarah J Nevitt, Mindy L Simpson, Maissaa Janbain, Barbara A Konkle
BACKGROUND: People with hemophilia A or B with inhibitors are at high risk of bleeding complications. Infusion of bypassing agents, such as recombinant activated FVII (rFVIIa) and plasma-derived activated prothrombin complex concentrate, are suggested as alternative therapies to factor VIII (haemophilia A) or IX (haemophilia B) for individuals who no longer respond to these treatments because they develop inhibitory antibodies. The ultimate goal of treatment is to preserve the individual's joints, otherwise destroyed by recurrent bleeds...
September 25, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28932649/hepatocyte-transplantation-cell-sheet-technology-for-liver-cell-transplantation
#11
REVIEW
Kohei Tatsumi, Teruo Okano
PURPOSE OF REVIEW: We will review the recent developments of cell sheet technology as a feasible tissue engineering approach. Specifically, we will focus on the technological advancement for engineering functional liver tissue using cell sheet technology, and the associated therapeutic effect of cell sheets for liver diseases, highlighting hemophilia. RECENT FINDINGS: Cell-based therapies using hepatocytes have recently been explored as a new therapeutic modality for patients with many forms of liver disease...
2017: Current Transplantation Reports
https://www.readbyqxmd.com/read/28919830/identification-of-patients-with-congenital-hemophilia-in-a-large-electronic-health-record-database
#12
Michael Wang, Anissa Cyhaniuk, David L Cooper, Neeraj N Iyer
BACKGROUND: Electronic health records (EHRs) are an important source of information with regard to diagnosis and treatment of rare health conditions, such as congenital hemophilia, a bleeding disorder characterized by deficiency of factor VIII (FVIII) or factor IX (FIX). OBJECTIVE: To identify patients with congenital hemophilia using EHRs. DESIGN: An EHR database study. SETTING: EHRs were accessed from Humedica between January 1, 2007, and July 31, 2013...
2017: Journal of Blood Medicine
https://www.readbyqxmd.com/read/28895303/coronary-angiography-with-or-without-percutaneous-coronary-intervention-in-patients-with-hemophilia-systematic-review
#13
Christian Boehnel, Hans Rickli, Lukas Graf, Micha T Maeder
OBJECTIVES: We aimed to summarize the evidence for periprocedural and long-term strategies to both minimize the bleeding risk and ensure sufficient anticoagulation and antiaggregation in hemophilia patients undergoing coronary angiography with or without percutaneous coronary interventions (PCI). BACKGROUND: Hemophilia patients undergoing coronary angiography and PCI are at risk of bleeding due to deficiency of the essential clotting factors VIII or IX combined with the need of peri-interventional anticoagulation and antiaggregation and dual antiplatelet therapy (DAPT) after PCI...
September 12, 2017: Catheterization and Cardiovascular Interventions
https://www.readbyqxmd.com/read/28891453/management-of-hemophilic-arthropathy-of-the-ankle
#14
E Carlos Rodriguez-Merchan
BACKGROUND: Hemophilic arthropathy (HA) of the ankle is prevalent in people with hemophilia (PWH). It is frequently severe and incapacitating, due to recurrent bleeding into the ankle articulation during infancy. Around 50% of hemophilic patients suffer from ankle pain and radiological signs of HA. OBJECTIVE: To review current treatment of HA of the ankle in PWH. METHOD: A literature review of hemophilic ankle arthropathy in PWH was performed utilizing MEDLINE (PubMed) and the Cochrane Library...
September 7, 2017: Cardiovascular & Hematological Disorders Drug Targets
https://www.readbyqxmd.com/read/28886353/initial-manifestation-of-acquired-hemophilia-a-after-a-routine-tooth-extraction-a-case-report-and-literature-review
#15
Nicholas A Bennetts, James E Mergelmeyer, Eric J Reimer, James C Melville
Although surgical treatment of patients on anticoagulation regimens is common practice among oral and maxillofacial surgeons, unexpected and unknown coagulopathies can have devastating and catastrophic consequences for the most routine of procedures. Acquired hemophilia A (AHA) is an extremely rare life-threatening bleeding disorder characterized by autoantibodies directed against circulating coagulation factor VIII. The effects of AHA can produce catastrophic bleeding and hematomas. The effect of this uncontrolled hemorrhage after dentoalveolar surgery can mimic severe head and neck infection by causing dysphagia, odynophagia, and acute airway complications...
August 12, 2017: Journal of Oral and Maxillofacial Surgery
https://www.readbyqxmd.com/read/28874064/the-association-of-aging-with-von-willebrand-factor-levels-and-bleeding-risk-in-type-1-von-willebrand-disease
#16
Craig D Seaman, Margaret V Ragni
Little is known about aging in von Willebrand disease (VWD). It is uncertain whether VWD patients experience an age-related increase in von Willebrand factor (VWF) levels, and if so, it is unknown whether normalization of VWF levels with aging ameliorates bleeding risk. We aimed to determine the association of age with VWF levels and bleeding risk in patients with type 1 VWD. This is a retrospective chart review of patients with type 1 VWD presenting to the Hemophilia Clinic of Western Pennsylvania for regularly scheduled clinic visits...
January 1, 2017: Clinical and Applied Thrombosis/hemostasis
https://www.readbyqxmd.com/read/28838728/perioperative-use-of-coagulation-factor-concentrates-in-patients-undergoing-cardiac-surgery
#17
REVIEW
Mark M Smith, Elena Ashikhmina, Nathan J Brinkman, David W Barbara
Coagulopathy and bleeding are common in patients undergoing cardiac surgery, with a perioperative transfusion rate in excess of 50%. The mechanism of coagulopathy associated with cardiac surgery using cardiopulmonary bypass is multifactorial. Historically, coagulation factor-mediated bleeding in such instances has been treated with allogeneic plasma transfusion. Coagulation factor concentrate use for treatment of hemophilia, congenital factor deficiencies and, more recently, emergency warfarin reversal is common...
October 2017: Journal of Cardiothoracic and Vascular Anesthesia
https://www.readbyqxmd.com/read/28795008/sports-participation-recommendations-for-patients-with-bleeding-disorders
#18
REVIEW
Cailly Howell, Katherine Scott, Dilip R Patel
There are several sets of guidelines recommending sports participation for patients with bleeding disorders. Though there is a perceived risk of injury and the associated bleeding complications, several studies have been done in this patient population and have found no increased risk. In addition, there are many other benefits to participation including improvement in physical fitness status, better health outcomes, improvement in quality of life, and the development of increased flexibility, gait coordination, and muscular strength, which may actually reduce the risk of subsequent injury...
July 2017: Translational pediatrics
https://www.readbyqxmd.com/read/28769599/identification-of-people-with-acquired-hemophilia-in-a-large-electronic-health-record-database
#19
Michael Wang, Anissa Cyhaniuk, David L Cooper, Neeraj N Iyer
BACKGROUND: Electronic health records (EHRs) can provide insights into diagnoses, treatment patterns, and clinical outcomes. Acquired hemophilia (AH) is an ultrarare bleeding disorder characterized by factor VIII inhibiting autoantibodies. AIM: To identify patients with AH using an EHR database. METHODS: Records were accessed from a large EHR database (Humedica) between January 1, 2007 and July 31, 2013. Broad selection criteria were applied using the International Classification of Diseases, Ninth Revision, clinical modification (ICD-9-CM) code for intrinsic circulating anticoagulants (286...
2017: Journal of Blood Medicine
https://www.readbyqxmd.com/read/28758960/ultrasound-for-early-detection-of-joint-disease-in-patients-with-hemophilic-arthropathy
#20
REVIEW
Matteo Nicola Dario Di Minno, Gianluigi Pasta, Sonia Airaldi, Federico Zaottini, Antonio Storino, Ernesto Cimino, Carlo Martinoli
Joint bleeding represents the most commonly reported type of hemorrhage in patients affected by hemophilia. Although the widespread use of prophylaxis has been able to significantly reduce the onset of arthropathy, it has been shown that a non-negligible percentage of patients develop degenerative changes in their joints despite this type of treatment. Thus, periodic monitoring of the joint status in hemophilia patients has been recommended to identify early arthropathic changes and prevent the development or progression of hemophilic arthropathy...
July 31, 2017: Journal of Clinical Medicine
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