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https://www.readbyqxmd.com/read/30246038/acute-life-threatening-hemorrhage-in-neonates-with-severe-hemophilia-a-a-report-of-3-cases
#1
Alvaro Moreira, Hrishikesh Das
Hemorrhagic shock is a rare, emergent condition that is often fatal in newborns. In this article, we report cases of 3 neonates presenting with acute, life-threatening hemorrhage who were subsequently diagnosed with severe hemophilia (<1% factor VIII). The first infant was tachycardic, pale, and had a precipitous drop in his hemoglobin secondary to a subgaleal hemorrhage. The second patient sustained a splenic rupture, a sequela that has been reported in only 4 other neonatal cases. The last infant presented with tonic-clonic seizures and respiratory distress...
January 2018: Journal of Investigative Medicine High Impact Case Reports
https://www.readbyqxmd.com/read/30230231/alternative-treatment-options-for-pediatric-hemophilia-b-patients-with-high-responding-inhibitors-a-thrombin-generation-guided-study
#2
Assaf Arie Barg, Sarina Levy-Mendelovich, Einat Avishai, Rima Dardik, Mudi Misgav, Gili Kenet, Tami Livnat
Little is known about the challenging treatment of pediatric patients with hemophilia B and inhibitors due to disease rarity. We describe three patients diagnosed in childhood and followed up to 9 years. All three had allergic reactions to Factor IX, but two were later safely treated for bleeding episodes with activated prothrombin complex concentrates (APCC = FEIBA). The third was given only recombinant activated Factor VIIa. Based on ex vivo thrombin generation analysis, a new alternative treatment of combined bypassing agents was administered for bleeding episodes and several minor surgical procedures with no treatment-associated adverse events or thrombosis...
September 19, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/30129541/consensus-statement-of-the-indian-academy-of-pediatrics-in-diagnosis-and-management-of-hemophilia
#3
Anupam Sachdeva, Vinod Gunasekaran, H N Ramya, Jasmita Dass, Jyoti Kotwal, Tulika Seth, Satyaranjan Das, Kapil Garg, Manas Kalra, Rani S Sirisha, Anand Prakash
JUSTIFICATION: Despite having standard principles of management of hemophilia, treatment differs in various countries depending on available resources. Guideline for management of hemophilia in Indian setting is essential. PROCESS: Indian Academy of Pediatrics conducted a consultative meeting on Hemophilia on 18th September, 2016 in New Delhi, which was attended by experts in the field working across India. Scientific literature was reviewed, and guidelines were drafted...
July 15, 2018: Indian Pediatrics
https://www.readbyqxmd.com/read/30122028/-advance-research-of-immune-tolerance-induction-for-pediatric-hemophilia-patients-with-inhibitors
#4
Z K Li, R H Wu
No abstract text is available yet for this article.
July 14, 2018: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
https://www.readbyqxmd.com/read/30049994/polyethylene-glycol-exposure-with-antihemophilic-factor-recombinant-pegylated-rurioctocog-alfa-pegol-and-other-therapies-indicated-for-the-pediatric-population-history-and-safety
#5
Reinhard Stidl, Michael Denne, Jimena Goldstine, Bill Kadish, Katherine I Korakas, Peter L Turecek
Polyethylene glycol (PEG) is an inert, water soluble polymer, used for decades in pharmaceuticals. Although PEG is considered safe, concerns persist about the potential adverse effects of long-term exposure to PEG-containing therapies, specifically in children, following the introduction of PEGylated recombinant factor products used for the treatment of hemophilia. Given the absence of long-term surveillance data, and to evaluate the potential risk, we estimated PEG exposure in the pediatric population receiving PEGylated therapies with pediatric indications administered intravenously or intramuscularly...
July 26, 2018: Pharmaceuticals
https://www.readbyqxmd.com/read/29921547/application-of-the-isth-bleeding-score-in-hemophilia
#6
Munira Borhany, Naveena Fatima, Madiha Abid, Tahir Shamsi, Maha Othman
BACKGROUND: Hemophilia is an inherited bleeding disorder. With proper treatment and self-care, persons with hemophilia can maintain an active, productive lifestyle. Hemophilia can be mild, moderate, or severe, depending on the degree of plasma clotting factor deficiency. The aim of the study was to assess the utility of ISTH-BAT in diagnosis, determining severity of the bleeding condition in newly diagnosed and known hemophilia patients, compare the bleeding score (BS) in adult and pediatric groups and investigate its association with plasma factor levels...
June 15, 2018: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/29895509/usefulness-of-chromogenic-assays-for-potency-assignment-and-recovery-of-plasma-derived-fviii-and-fix-concentrates-or-their-recombinant-long-acting-therapeutic-equivalents-with-potential-application-in-treated-pediatric-hemophiliac-patients
#7
REVIEW
Jean Amiral, Jerard Seghatchian
On demand and prophylaxis usage of FVIII/ FIX concentrates for the therapeutic management of hemophilia has greatly changed quality of life, and healthy life span of affected patients. Availability of recombinant therapeutic FVIII and FIX products, and of their long-acting variants, further improves the treatment constraints, and progressively permits to hemophiliacs to have an almost normal way of life. Unlimited amounts of recombinant or engineered substitutive products become available, and open new avenues for extending the benefits of prophylaxis to all hemophiliac patients, not only in economically advanced territories, but also in emerging and developing countries, worldwide...
June 2018: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/29537665/pulpotomy-management-using-laser-diode-in-pediatric-patient-with-severe-hemophilia-a-under-general-anesthesia-a-case-report
#8
Vo Truong Nhu Ngoc, Trinh Do Van Nga, Dinh-Toi Chu, Le Quynh Anh
Hemophilia-a bleeding disorder due to the lack of clotting factors-is mostly induced by genetic factors. Its most common type is hemophilia A. Hemorrhage in hemophilia A may occur in several different sites of the body, including those inside the oral cavity, such as mucous membrane and gum. However, only a few studies and case reports on dental issues of hemophilia A patients have been conducted. In clinical dentistry, treatment procedures are invasive, possibly leading to more severe bleeding, especially in hemophilia A cases...
May 2018: Special Care in Dentistry
https://www.readbyqxmd.com/read/29520794/critical-developments-of-2017-a-review-of-the-literature-from-selected-topics-in-transfusion-a-committee-report-from-the-aabb-clinical-transfusion-medicine-committee
#9
Melissa M Cushing, James Kelley, Ellen Klapper, David F Friedman, Ruchika Goel, Nancy M Heddle, Courtney K Hopkins, Julie Katz Karp, Monica B Pagano, Ajay Perumbeti, Glenn Ramsey, John D Roback, Joseph Schwartz, Beth H Shaz, Philip C Spinella, Claudia S Cohn, Claudia S Cohn, Melissa M Cushing, James Kelley, Ellen Klapper
BACKGROUND: The AABB compiles an annual synopsis of the published literature covering important developments in the field of Transfusion Medicine. For the first time, an abridged version of this work is being made available in TRANSFUSION, with the full-length report available as an Appendix S1 (available as supporting information in the online version of this paper). STUDY DESIGN AND METHODS: Papers published in 2016 and early 2017 are included, as well as earlier papers cited for background...
April 2018: Transfusion
https://www.readbyqxmd.com/read/29485331/using-the-hemophilia-joint-health-score-for-assessment-of-children-reliability-of-the-spanish-version
#10
Cuesta-Barriuso R, Torres-Ortuño A, Pérez-Alenda S, Carrasco Juan J, Querol F, Nieto-Munuera J, López-Pina Ja
INTRODUCTION: Numerous measuring instruments for the evaluation of hemophilic arthropathy have been developed. One of the most used systems is the Hemophilia Joint Health Score (HJHS) given its sensitivity to clinical changes appearing in the joints because of recurrent hemarthrosis. OBJECTIVE: Assessing the interrater reliability, using the Spanish version of the HJHS (version 2.1) in children with hemophilia. DESIGN: Reliability study to assess the interrater reliability of the Spanish version of HJHS...
February 27, 2018: Physiotherapy Theory and Practice
https://www.readbyqxmd.com/read/29240038/prevalence-of-hypertension-htn-and-cardiovascular-risk-factors-in-a-hospitalized-pediatric-hemophilia-population
#11
Warren Alperstein, Fernando F Corrales-Medina, Leonardo Tamariz, Ana M Palacio, Joanna A Davis
Improved life expectancy in hemophilia has led to a greater interest in age-related disorders. Hypertension (HTN) as well as cardiovascular disease have been increasingly reported in hemophilic adults but there is currently very limited data in the pediatric population. We conducted a cross-sectional study using data from the 2012 National Health Cost and Utilization Project database to determine the prevalence of HTN and associated cardiovascular risk factors in a hospitalized pediatric hemophilia population, between the ages of 0 to 21 years, in comparison with the general pediatric population...
April 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29141317/-study-of-gene-mutation-in-62-hemophilia-a-children
#12
Q Hu, A G Liu, L Q Zhang, A Zhang, Y Q Wang, S M Wang, Y J Lu, X Wang
Objective: To analyze the mutation type of FⅧ gene in children with hemophilia A and to explore the relationship among hemophilia gene mutation spectrum, gene mutation and clinical phenotype. Method: Sixty-two children with hemophilia A from Department of Pediatric Hematology, Tongji Hospital of Tongji Medical College, Huazhong University of Science and Technology between January 2015 and March 2017 were enrolled. All patients were male, aged from 4 months to 7 years and F Ⅷ activity ranged 0.2%-11.0%. Fifty cases had severe, 10 cases had moderate and 2 cases had mild hemophilia A...
November 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/29125443/neurosurgical-management-in-children-with-bleeding-diathesis-auditing-neurological-outcome
#13
Zaitun Zakaria, Chandrasekaran Kaliaperumal, Darach Crimmins, John Caird
OBJECTIVE The aim of this study was to assess the outcome of neurosurgical treatment in children with bleeding diathesis and also to evaluate the current management plan applied in the authors' service. METHODS The authors retrospectively analyzed all cases in which neurosurgical procedures were performed in pediatric patients presenting with intracranial hematoma due to an underlying bleeding tendency over a 5-year period at their institution. They evaluated the patients' neurological symptoms from the initial referral, hematological abnormalities, surgical treatment, neurological outcome, and scores on the Pediatric Glasgow Outcome Scale-Extended (GOS-E Peds) obtained 1 year after the last operation...
January 2018: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/29050499/congenital-factor-deficiencies-in-children-a-report-of-a-single-center-experience
#14
Zafer Şalcıoğlu, Cengiz Bayram, Hülya Şen, Gizem Ersoy, Gönül Aydoğan, Arzu Akçay, Deniz Tuğcu, Ferhan Akıcı, Müge Gökçe, Metin Demirkaya, Ali Ayçiçek, Zafer Başlar
Congenital factor deficiencies (CFDs) refer to inherited deficiency of coagulation factors in the blood. A total of 481 patients with CFDs, who were diagnosed and followed at our Pediatric Hematology and Oncology Clinic between 1990 and 2015, were retrospectively evaluated. Of the 481 cases, 134 (27.8%) were hemophilia A, 38 (7.9%) were hemophilia B, 57 (11.8%) were von Willebrand disease (vWD), and 252 (52.3%) were rare bleeding disorders (RBDs). The median age of the patients at the time of diagnosis and at the time of the study was 4...
September 2018: Clinical and Applied Thrombosis/hemostasis
https://www.readbyqxmd.com/read/28900823/health-related-quality-of-life-developmental-milestones-and-self-esteem-in-young-adults-with-bleeding-disorders
#15
P F Limperg, L Haverman, H Maurice-Stam, M Coppens, C Valk, M J H A Kruip, J Eikenboom, M Peters, M A Grootenhuis
BACKGROUND: The treatment of bleeding disorders improved in the last decades. However, the effect of growing up with bleeding disorders on developmental, emotional, and social aspects is understudied. Therefore, this study assesses HRQOL, developmental milestones, and self-esteem in Dutch young adults (YA) with bleeding disorders compared to peers. METHODS: Ninety-five YA (18-30 years) with bleeding disorders (78 men; mean 24.7 years, SD 3.5) and 17 women (mean 25...
January 2018: Quality of Life Research
https://www.readbyqxmd.com/read/28648539/prothrombin-complex-concentrates-in-pediatric-cardiac-surgery-the-current-state-and-the-future
#16
REVIEW
Elena Ashikhmina, Sameh Said, Mark M Smith, Vilmarie Rodriguez, William C Oliver, Gregory A Nuttall, Joseph A Dearani, Hartzell V Schaff
BACKGROUND: After decades of practice of pediatric cardiac surgery, postoperative bleeding due to the immaturity of hemostasis, hemodilution, and hypothermia remains a concern. Recently, a new approach for adult coagulopathy after bypass has emerged. Prothrombin complex concentrates (PCCs), designed to treat bleeding in hemophilia patients, are safely and efficiently used off label for hemorrhage after bypass. However, optimal dosing, indications and contraindications, and laboratory tests to assess the efficacy of PCC use in children have not yet been established...
October 2017: Annals of Thoracic Surgery
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/28574805/the-development-of-fviii-inhibitors-in-relation-to-il10-gene-polymorphism-in-hemophilia-a-egyptian-pediatric-patients
#18
Hoda Sadek, Ilham Youssry, Nihal Salah Eldeen Ibrahim, Amany Ahmed Abou-Elalla, Gehad Atef, Somaia Mohammed Mousa
BACKGROUND: Development of inhibitors against Factor VIII (FVIII) in hemophilia A patients is a serious complication of therapy. Many cytokines, including interleukin-10 (IL10), may affect inhibitor development; however, literature data are not sufficient to prove this association. The aim of this study was to investigate the relation between FVIII inhibitor formation and IL10-1082A/G polymorphism among Egyptian hemophiliacs. METHODS: Patients were screened for FVIII inhibitors using the Bethesda method...
June 2017: Fetal and Pediatric Pathology
https://www.readbyqxmd.com/read/28534860/hemophilia-care-in-the-pediatric-age
#19
REVIEW
Marta Bertamino, Francesca Riccardi, Laura Banov, Johanna Svahn, Angelo Claudio Molinari
Hemophilia is the most common of the severe bleeding disorders and if not properly managed since early infancy can lead to chronic disease and lifelong disabilities. However, it enjoys the most efficacious and safe treatment among the most prevalent monogenic disorders. Hemophilia should be considered in the neonatal period in the case of unusual bleeding or in the case of positive family history. Later, hemophilia should be suspected mainly in males because of abnormal bruising/bleeding or unusual bleeding following invasive procedures-for example, tonsillectomy or circumcision...
May 19, 2017: Journal of Clinical Medicine
https://www.readbyqxmd.com/read/28532239/status-and-trend-analysis-of-prophylactic-usage-of-recombinant-factor-viii-in-chinese-pediatric-patients-with-hemophilia-a-recare-a-retrospective-phase-iv-non-interventional-study
#20
MULTICENTER STUDY
Changgang Li, Xinsheng Zhang, Yongqiang Zhao, Runhui Wu, Qun Hu, Vicky Xu, Jing Sun, Renchi Yang, Xiaojing Li, Rongfu Zhou, Shinmei Lian, Jian Gu, Junde Wu, Qingsong Hou
BACKGROUND: No study has reported the status and chronological trend of prophylactic recombinant factor VIII (rFVIII) use in Chinese pediatric patients with hemophilia A (HA). OBJECTIVE: We aimed to analyze the status and trend of rFVIII-containing prophylaxis in Chinese pediatric patients with HA. METHODS: ReCARE (Retrospective study in Chinese pediatric hemophilia A patients with rFVIII contained REgular prophylaxis) was a retrospective study conducted in 12 hemophilia treatment centers across China...
September 2017: Current Medical Research and Opinion
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