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Hemophilia A

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https://www.readbyqxmd.com/read/28641667/-research-progress-on-genetic-factors-and-hemophilia-a-clotting-factor-inhibitor-review
#1
Zheng-Bin Hu, Xin Sun, Li-Ya He
Hemophilia A (Hemophilia A, HA) is an X-linked recessive hereditary coagulation function disorder, the deficiency and dysfunction of blood coagulation were caused by the mutations of gene encoding clotting factor VIII. The treatment of hemophilia A still depends on the replacement therapy with blood coagulation factor. However, the repeated infusion of clotting factor will produce the neutralizing antibody against FVIII, then resulting in one of the serious complications. The reports on the incidence of inhibitor are different at home and abroad...
June 2017: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/28638302/extended-stability-and-sterility-of-antihemophilic-factor-human
#2
Sarah Shields, Abby Kim, Joshua Elder
OBJECTIVES: Antihemophilic factor human is a factor VIII product used to supplement those with hemophilia. Recent data show treatment benefit and cost saving opportunities if factor products are administered as a continuous infusion rather than conventional bolus dose. This method has not been widely used given the lack of evidence for safe and effective use beyond 3 hours from preparation. The objectives of this study were to determine the physical and chemical stability and sterility of antihemophilic factor human over a 7-day period...
May 2017: Journal of Pediatric Pharmacology and Therapeutics: JPPT: the Official Journal of PPAG
https://www.readbyqxmd.com/read/28634557/dental-management-of-a-child-with-incidentally-detected-hemophilia-report-of-a-clinical-case
#3
Ricardo Martínez-Rider, Arturo Garrocho-Rangel, Raúl Márquez-Preciado, María Victoria Bolaños-Carmona, Socorro Islas-Ruiz, Amaury Pozos-Guillén
Children with hemophilia (A or B) are at risk for bleeding episodes, which rank from mild mucosal/soft tissues bleeding to life-threatening hemorrhages. This report describes the dental/medical management provided to an 8.10-year-old patient suffering from uncontrolled bleeding after a surgical procedure to expose both permanent upper central incisors, in which hemophilia was a pure incidental finding. Additionally, diverse precautions to be considered during the dental clinical treatment of hemophilic children are discussed...
2017: Case Reports in Dentistry
https://www.readbyqxmd.com/read/28632502/targeting-activated-protein-c-to-treat-hemophilia
#4
Stéphanie G I Polderdijk, Trevor P Baglin, James A Huntington
PURPOSE OF REVIEW: Hemophilia is a debilitating disease, marked by frequent, painful bleeding events, joint deterioration and early death. All current treatments consist of i.v. infusions of replacement factor or other procoagulant factors, and are incompletely effective, due in part to the short half-lives of the proteins. An alternative approach is to rebalance hemostasis by inhibiting natural anticoagulant mechanisms. In this article, we explain why activated protein C (APC) is an appropriate and safe target for the treatment of hemophilia...
June 16, 2017: Current Opinion in Hematology
https://www.readbyqxmd.com/read/28628105/identification-of-liver-specific-enhancer-promoter-activity-in-the-3-untranslated-region-of-the-wild-type-aav2-genome
#5
Grant J Logan, Allison P Dane, Claus V Hallwirth, Christine M Smyth, Emilie E Wilkie, Anais K Amaya, Erhua Zhu, Neeta Khandekar, Samantha L Ginn, Sophia H Y Liao, Sharon C Cunningham, Natsuki Sasaki, Martí Cabanes-Creus, Patrick P L Tam, David W Russell, Leszek Lisowski, Ian E Alexander
Vectors based on adeno-associated virus type 2 (AAV2) are powerful tools for gene transfer and genome editing applications. The level of interest in this system has recently surged in response to reports of therapeutic efficacy in human clinical trials, most notably for those in patients with hemophilia B (ref. 3). Understandably, a recent report drawing an association between AAV2 integration events and human hepatocellular carcinoma (HCC) has generated controversy about the causal or incidental nature of this association and the implications for AAV vector safety...
June 19, 2017: Nature Genetics
https://www.readbyqxmd.com/read/28627086/the-hypertension-of-hemophilia-is-associated-with-vascular-remodeling-in-the-joint
#6
Richard Fw Barnes, Thomas J Cramer, Tudor H Hughes, Annette von Drygalski
OBJECTIVE: Hemophilic arthropathy is associated with pronounced vascular joint remodeling. Also, compared to the general population, PWH have a higher prevalence of hypertension not explained by usual risk factors. Since vascular remodeling in various vascular beds is a hallmark of hypertension, we hypothesized that vascular joint remodeling is associated with elevated blood pressures and hypertension. METHODS: Elbows, knees and ankles of 28 adult PWH were evaluated for vascular abnormalities with MSKUS/PD, as well as for radiographic and clinical status and pain...
June 19, 2017: Microcirculation: the Official Journal of the Microcirculatory Society, Inc
https://www.readbyqxmd.com/read/28625571/micromanaging-tolerance-in-hemophilia-a-gene-therapy
#7
Jennielle Jobson, Brian D Brown
No abstract text is available yet for this article.
June 15, 2017: Molecular Therapy: the Journal of the American Society of Gene Therapy
https://www.readbyqxmd.com/read/28618141/mortality-caused-by-intracranial-bleeding-in-non-severe-hemophilia-a-patients-reply
#8
J I Loomans, K Fijnvandraat
With great interest we read the letter of Dr. Patil et al. (1) in which they present the number and percentage of hemophilia patients registered in the Mumbai hemophilia center who died from (intracranial) bleeding between 2002 and 2015. We thank Dr. Patil et al. for sharing their data and for providing essential insight into fatal intracranial bleeding in nonsevere hemophilia A patients from a large hemophilia center in India. This article is protected by copyright. All rights reserved.
June 15, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28612430/igg-subclasses-of-fviii-inhibitors-in-an-argentine-cohort-of-severe-hemophilia-a-patients-analysis-by-flow-cytometry
#9
M B Irigoyen, L Primiani, M Felippo, M Candela, M M E de Bracco, N V Galassi
INTRODUCTION: FVIII inhibitors consist of a polyclonal population of antibodies. Previous studies have demonstrated different distribution of IgG subclasses. IgG4 was associated to high level of FVIII inhibitors and failure of immune tolerance induction (ITI) treatment. This study monitored the relative distribution of IgG subclasses of anti-FVIII in patients with severe hemophilia A (SHA). METHODS: Anti-FVIII antibodies were measured employing an immunomethod, developed in our laboratory, that combines flow cytometry (FC) with microspheres coupled (FVIII-m) or not (Control-m) to FVIII...
June 14, 2017: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/28607874/robotic-assisted-abdomino-perineal-vesicourethral-anastomotic-reconstruction-for-4-5-centimeter-post-prostatectomy-stricture
#10
Brian F Dinerman, Nicholas J Hauser, Jim C Hu, Rajveer S Purohit
We report surgical management of a disrupted radical prostatectomy vesicourethral anastomosis after bleeding from undiagnosed hemophilia that required re-exploration, pudendal artery embolization, and urinary diversion with nephrostomy and surgical drains. After referral, the 4.5 cm vesicourethral anastomotic defect was reconstructed with a robotic-assisted abdomino-perineal approach. Intra-abdominal robotic-assisted mobilization of the bladder and perineal mobilization of the urethra permitted a tension-free vesicourethral anastomosis while avoiding a pubectomy...
September 2017: Urology Case Reports
https://www.readbyqxmd.com/read/28601434/an-open-label-single-dose-pharmacokinetic-study-of-factor-viii-activity-after-administration-of-moroctocog-alfa-af-cc-in-male-chinese-patients-with-hemophilia-a
#11
Hongzhong Liu, Runhui Wu, Pei Hu, Feifei Sun, Lihong Xu, Yali Liang, Sunil Nepal, Peng Roger Qu, Francois Huard, Joan M Korth-Bradley
PURPOSE: Hemophilia A represents up to 80% of all hemophilia cases in China. In patients with this condition, bleeding can be prevented and controlled by administering clotting factor VIII (FVIII). Since their initial availability, recombinant FVIII products have undergone several iterations to enhance their safety. Moroctocog alfa albumin-free cell culture (AF-CC) is among the third generation of recombinant FVIII products and received regulatory approval in China in August 2012. The present study characterizes the single-dose pharmacokinetic parameters of FVIII activity (FVIII:C) after administration of moroctocog alfa (AF-CC) in male Chinese patients with hemophilia A...
June 7, 2017: Clinical Therapeutics
https://www.readbyqxmd.com/read/28600843/mortality-caused-by-intracranial-bleeding-in-non-severe-hemophilia-a-patients-comment
#12
Rucha Patil, Kanjaksha Ghosh, Shrimati Shetty
We read with interest the study done by Loomans et al (1); wherein the authors have found that out of 148 deceased mild and moderate hemophilia patients, 62 (42%) expired due to excessive bleeding i.e. due to the disorder itself and out of these 12% died of intracranial (IC) bleed. Thus, the authors rightly conclude that non-severe hemophilia A patients also have an increased risk of death due to IC bleed and thus demonstrating the need for specialized care for these patients. India harbors the second highest number of patients with hemophilia globally, majority belonging to the poverty-stricken section (2)...
June 10, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28599169/modifiers-of-clinical-phenotype-in-severe-congenital-hemophilia
#13
Massimo Franchini, Pier Mannuccio Mannucci
Patients with inherited hemophilia A and B usually exhibit a bleeding tendency of a severity proportional to the degree of plasmatic deficiency of the coagulant activity of factor VIII (FVIII:C) and factor IX (FIX:C). Although patients with severe hemophilia (i.e., with FVIII:C and FIX:C levels <1IU/dL) are generally those with the most severe bleeding phenotype, it is common experience that a variable proportion of them experiences a milder bleeding tendency. In this review, we summarize the current knowledge on the possible mechanisms at the basis of the phenotypic heterogeneity of severe hemophilia, focusing in particular on the role of FVIII/FIX gene mutations and thrombophilic polymorphisms...
June 1, 2017: Thrombosis Research
https://www.readbyqxmd.com/read/28598970/seroprevalence-and-clinical-characteristics-of-viral-hepatitis-in-transfusion-dependent-thalassemia-and-hemophilia-patients
#14
Tyng-Yuan Jang, Pei-Chin Lin, Ching-I Huang, Yu-Mei Liao, Ming-Lun Yeh, Yu-Sheng Zeng, Po-Cheng Liang, Wan-Yi Hsu, Shih-Pien Tsai, Zu-Yau Lin, Shinn-Cherng Chen, Jee-Fu Huang, Chia-Yen Dai, Chung-Feng Huang, Shyh-Shin Chiou, Wan-Long Chuang, Ming-Lung Yu
BACKGROUND/AIMS: Transfusion dependent subjects are at a great risk of viral hepatitis infection. We aimed to evaluate the prevalence and factors associated with hepatitis B virus (HBV) and hepatitis C virus (HCV) infection among transfusion-dependent patients in Taiwan. METHODS: A total of 140 patients (67 thalassemic patients, 70 hemophilic patients, two patients with hereditary spherocytosis and one patient with von Willebrand disease) were prospectively enrolled to evaluate the prevalence and factors associated with viral hepatitis and spontaneous HCV clearance...
2017: PloS One
https://www.readbyqxmd.com/read/28597369/first-report-of-real-time-monitoring-of-coagulation-function-potential-and-igg-subtype-of-anti-fviii-autoantibodies-in-a-child-with-acquired-hemophilia-a-associated-with-streptococcal-infection-and-amoxicillin
#15
Masahiro Takeyama, Keiji Nogami, Takahiro Kajimoto, Kenichi Ogiwara, Tomoko Matsumoto, Midori Shima
We describe an 8-year-old boy with acquired hemophilia A (AHA) associated with streptococcal infection and amoxicillin. Laboratory data revealed low factor VIII activity (FVIII:C, 1.5 IU/dl), and FVIII inhibitor (15.9 BU/ml). Comprehensive coagulation function assays, including rotation thromboelastometry (ROTEM(®)), revealed a markedly prolonged clotting time. Thrombin and plasmin generation (TG/PG) appeared to be moderately impaired. The inhibitor epitope of his anti-FVIII autoantibody recognized light and heavy chains...
June 8, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28596656/assessment-of-heart-and-liver-iron-overload-in-thalassemia-major-patients-using-t2-magnetic-resonance-imaging
#16
Hamid Farhangi, Zahra Badiei, Hasan Mottaghi Moghaddam, Mohammad Reza Keramati
Accumulation of excess iron in heart can lead to cardiac dysfunction, which is the most common cause of death in thalassemia major patients. Biopsy is an invasive procedure and therefore not an ideal option to assess iron load. However, standard/usual non-invasive methods, such as ferritin measurement, have some limitations and the results show poor correlations with iron load. Magnetic Response Imaging (MRI-T2*), as a non-invasive and reliable method for iron load assessment in organs such as liver and heart, can be suggested as a favorable alternative...
June 2017: Indian Journal of Hematology & Blood Transfusion
https://www.readbyqxmd.com/read/28591493/-perception-of-quality-of-life-related-with-health-and-depression-in-patients-with-hemophilia
#17
Maricela Osorio-Guzmán, Graciela Gutiérrez-González, Georgina Eugenia Bazán-Riverón, Nora Nancy Núñez-Villegas, Gabriela Jazmín Fernández-Castillo
BACKGROUND: Hemophilia is a hemorrhagic disease characterized by the deficiency of either coagulation factor VIII or IX. It impacts negatively in people's quality of life and it causes side effects, such as depression. The objective was to describe and analyze the health-related quality of life (HRQoL) and depression levels in a group of 50 pediatric patients with hemophilia (PPwH) and their parents. The specific objectives were: to analyze the correlation between HRQoL levels reported by patients and their parents, and to analyze the correlation between HRQoL levels and depression in PPwH...
July 2017: Revista Médica del Instituto Mexicano del Seguro Social
https://www.readbyqxmd.com/read/28589615/safety-and-efficacy-of-recombinant-activated-coagulation-factor-vii-in-congenital-hemophilia-with-inhibitors-in-the-home-treatment-setting-a-review-of-clinical-studies-and-registries
#18
REVIEW
Guy Young, Miguel A Escobar, Steven W Pipe, David L Cooper
Self-administration of factor and bypassing agents by persons with hemophilia in the home setting is recommended to facilitate earlier intervention after bleeding episodes. The objective of this review was to summarize recombinant activated coagulation factor VII (rFVIIa) safety and efficacy data from clinical trials and patient registries documenting use in the home treatment setting in people with congenital hemophilia with inhibitors (CHwI). A total of 16 studies and registries were identified for inclusion; 14 evaluated on-demand treatment of acute bleeding episodes (865 patients, 9024 bleeding episodes) and 2 evaluated use for secondary prophylaxis (108 patients, 42,861 prophylaxis days)...
June 7, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28586248/side-effects-and-potential-risk-factors-of-botulinum-toxin-type-a-intramuscular-injections-in-knee-flexion-contractures-of-hemophiliacs
#19
E Carlos Rodriguez-Merchan, Hortensia De la Corte-Rodriguez
Knee flexion contracture (KFC) is a common complication of recurrent hemarthrosis in children and young adults with hemophilia. If the KFC is not prevented (by means of primary prophylaxis) and treated properly and early (be means of physical medicine and rehabilitation), it will become fixed. Areas covered: The aim of this article is to review the potential role of botulinum toxin type A (BTX-A) intramuscular injections for the treatment of KFC in people with hemophilia (PWH). Expert commentary: Although two recent reports have mentioned the benefits of intramuscular injections of BTX-A in PWH with KFC, the data are still scant and too preliminary...
June 19, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28584905/clinical-characteristics-of-intussusception-secondary-to-pathologic-lead-points-in-children-a-single-center-experience-with-65-cases
#20
Xiao-Kun Lin, Qiong-Zhang Xia, Xiao-Zhong Huang, Yi-Jiang Han, Guo-Rong He, Na Zheng
OBJECTIVE: Intussusception secondary to pathologic lead points (PLPs) is a challenging condition for pediatric surgeons, and few studies have been published on this subject. The aim of this study was to review and analyze clinical data on the diagnosis and management of intussusception secondary to PLPs in children. METHODS: Between 2002 and 2016, a total of 65 pediatric patients with a diagnosis of intussusception secondary to PLPs were retrospectively reviewed...
June 5, 2017: Pediatric Surgery International
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