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https://www.readbyqxmd.com/read/30317158/cost-effectiveness-of-rfviia-versus-pd-apcc-in-the-management-of-mild-to-moderate-bleeds-in-pediatric-patients-with-hemophilia-a-with-inhibitors-in-mexico
#1
Maria Del Carmen Rodríguez-Zepeda, Lourdes González, Amalia Bravo, Teresa Pompa, Salvador Silva, Rogelio Paredes, Jaime García, Mafalda Ramos, Lars Wilkinson, Mark Lamotte
OBJECTIVE: To compare the costs and clinical consequences of treating mild-to-moderate joint bleeds with recombinant activated factor VII (rFVIIa) versus plasma-derived activated prothrombin complex concentrate (pd-aPCC) in pediatric patients with hemophilia A with inhibitors in Mexico. METHODS: A cost-effectiveness model was developed using TreeAge Pro v14.2.2 software (licensed in the USA) and adapted from a previously published model, with adjustments to reflect local clinical practice...
October 11, 2018: Value in Health Regional Issues
https://www.readbyqxmd.com/read/30293277/-analysis-of-the-arthropathies-on-no-bleeding-history-joints-in-pre-school-age-severe-hemophilia-a-children
#2
D Ai, X J Li, W R Yao, Q Zhang, M Zhou, L Tang, X B Luo, N N Zhang, S Yang, Y Wang, P Ding, S Y Cai, Z P Chen, R H Wu
Objective: To detect the arthropathies on no bleeding history joints in pre-school hemophilia A children in order to provide evidence for further prevention and control of joint disease in children with hemophilia A. Methods: This study was a cross-sectional study based on China Hemophilia Individualized prophylaxis study (CHIPS). The basic data of outpatients with hemophilia in Beijing Children's Hospital and Chengdu Women's and Children's Central Hospital between August 2016 and June 2017 were collected and a three-month follow-up was conducted...
October 2, 2018: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/30277727/cerumen-impaction-diagnosis-and-management
#3
Charlie Michaudet, John Malaty
Cerumen production is a normal and protective process for the ear canal. However, cerumen should be removed when it causes symptoms (e.g., hearing loss, itching, pain, tinnitus) or prevents assessment of the external auditory canal, the tympanic membrane, or audiovestibular system. Cerumen should also be removed when it limits examination in patients who cannot communicate their symptoms, such as those with dementia or developmental delay, nonverbal patients with behavioral changes, and young children with fever, speech delay, or parental concerns...
October 15, 2018: American Family Physician
https://www.readbyqxmd.com/read/30256429/oral-hygiene-and-dentition-status-in-children-and-adults-with-hemophilia-a-case-control-study
#4
Mathangi Kumar, Keerthilatha M Pai, Annamma Kurien, Ravindranath Vineetha
BACKGROUND & OBJECTIVES: People with hemophilia constitute a significant proportion of the population and an oral health care professional faces a considerable challenge while treating them. This study aimed to assess the oral health and dentition status as well as fear of dental treatment in patients with hemophilia and compare it with age-matched healthy subjects. PATIENTS & METHODS: This single-center, case-control cross-sectional study was performed on 100 subjects with hemophilia and 100 age-matched healthy controls...
September 26, 2018: Special Care in Dentistry
https://www.readbyqxmd.com/read/30254423/glycopegylated-recombinant-factor-ix-for-hemophilia-b-in-context
#5
REVIEW
Elena Santagostino, Maria Elisa Mancuso
Decisions over hemophilia treatment selection and switching involve balancing many clinical and patient-related factors. The current standard of care for patients with hemophilia B is prophylaxis with plasma-derived or recombinant factor IX (rFIX) concentrates. However, several extended half-life (EHL) rFIX products have recently been developed to improve treatment convenience and clinical outcomes for these patients. Nonacog beta pegol, an rFIX product that combines the FIX protein with a 40 kDa polyethylene glycol moiety, has been evaluated in 115 previously treated patients with hemophilia B (including 25 children) in the paradigm clinical trial program...
2018: Drug Design, Development and Therapy
https://www.readbyqxmd.com/read/30191308/-pain-therapy-for-children-and-adolescents-with-hemophilia-recommendations-by-an-expert-panel
#6
REVIEW
W Stromer, B Messerer, R Crevenna, S H Hemberger, B Jauk, R Schwarz, W Streif, K Thom, B Wagner, K Zwiauer, R Likar
BACKGROUND: Children and adolescents with severe hemophilia commonly suffer from acute and chronic pain as a consequence of hemophilia-related bleeding. Intervention-related pain also plays a major role. Despite its high prevalence in this patient group, hemophilia-related pain is not always adequately addressed and sufficiently treated. OBJECTIVES: This paper discusses how to improve pain management for children and adolescents (0-18 years) with hemophilia and which specific features in this population should influence decisions in pain management...
September 6, 2018: Der Schmerz
https://www.readbyqxmd.com/read/30179272/reliability-and-validity-of-patient-reported-outcome-instruments-in-us-adults-with-hemophilia-b-and-caregivers-in-the-b-hero-s-study
#7
Tyler W Buckner, Robert Sidonio, Christine Guelcher, Craig M Kessler, Michelle Witkop, David Clark, Wendy Owens, Moshe Fridman, Neeraj N Iyer, David L Cooper
OBJECTIVE: To assess the reliability and validity of 6 patient-reported outcomes (PRO) instruments for evaluating health-related quality of life in adults with mild-severe hemophilia B and caregivers of children with hemophilia B, including affected women/girls. METHODS: Adults with hemophilia B and caregivers completed separate online surveys containing several PRO instruments, which were administered to adult participants only (EQ-5D-5L, Brief Pain Inventory v2 Short Form, Hemophilia Activities List, and International Physical Activities Questionnaire), both adults and caregivers (Patient Health Questionnaire [PHQ-9]), or caregivers only (Generalized Anxiety Disorder 7-Item [GAD-7] scale)...
September 4, 2018: European Journal of Haematology
https://www.readbyqxmd.com/read/30129541/consensus-statement-of-the-indian-academy-of-pediatrics-in-diagnosis-and-management-of-hemophilia
#8
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/30128815/prevention-and-management-of-bleeding-episodes-in-children-with-hemophilia
#9
REVIEW
Rolf C R Ljung
Regular prophylactic treatment with factor VIII (FVIII) and factor IX (FIX) concentrates in hemophilia A and B, respectively, is introduced in early infancy and has resulted in dramatic improvement of the conditions. Recombinant FVIII and FIX concentrates have been available for > 25 years and have been modified and refined through the years; however, unfortunately frequent intravenous administrations are still necessary. The half-lives of these products have now been extended (EHL) by fusion with albumin, the Fc-portion of IgG, or by being PEGylated...
October 2018: Paediatric Drugs
https://www.readbyqxmd.com/read/30066606/reaching-independence-through-forced-learning-learning-processes-and-illness-management-in-parents-of-children-affected-by-hemophilia
#10
Linda Myrin Westesson, Catarina Wallengren, Fariba Baghaei, Carina Sparud-Lundin
Hemophilia is a complex condition to manage, especially for parents to newly diagnosed children. This grounded theory study explores parents' learning processes and illness management in daily life during the first year after the start of their child's treatment. Using a longitudinal qualitative design, eight parents of four children were interviewed repeatedly during 12 to 14 months. The core category, reaching independence through forced learning, reflected the parents' learning process and their experiences of the challenges during the first year after start of treatment...
August 1, 2018: Qualitative Health Research
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
#11
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/30013766/bay-81-8973-a-full-length-recombinant-factor-viii-for-the-treatment-of-hemophilia-a-product-review
#12
REVIEW
Johnny N Mahlangu, Sanjay P Ahuja, Jerzy Windyga, Nikki Church, Anita Shah, Lawrence Schwartz
BAY 81-8973 (Kovaltry® ) is an unmodified, full-length recombinant factor VIII (rFVIII) approved for the prevention and treatment of bleeding episodes in patients with hemophilia A. The amino acid sequence for BAY 81-8973 is identical to that of sucrose-formulated rFVIII (rFVIII-FS; Kogenate® FS/KOGENATE® , Bayer), but the two products differ in their manufacturing approaches. The manufacture of BAY 81-8973 includes several modifications and enhancements, such as the introduction of the gene for human heat shock protein 70, a molecular chaperone protein that facilitates folding of proteins; no addition of human- or animal-derived proteins in the cell culture, purification process, or final formulation; and use of a 20-nm filter to remove any potential aggregates and pathogens...
July 2018: Therapeutic Advances in Hematology
https://www.readbyqxmd.com/read/30004151/risk-factors-for-cardiovascular-disease-in-children-and-young-adults-with-haemophilia
#13
Jacqueline Limjoco, Courtney D Thornburg
INTRODUCTION: The origins of cardiovascular disease (CVD) begin in childhood. The primary objective of this cross-sectional cohort study was to determine the prevalence of cardiovascular risk factors in patients with congenital haemophilia A or B followed at Rady Children's Hospital San Diego Hemophilia and Thrombosis Treatment Center (HTC). We hypothesized that cardiovascular risk factors could be identified as part of a comprehensive clinic visit. MATERIALS AND METHODS: Standardized measurement of weight, height, waist circumference and blood pressure plus non-fasting glucose and lipid panel were performed...
September 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29848837/pharmacokinetic-studies-of-factor-viii-in-chinese-boys-with-severe-hemophilia-a-a-single-center-study
#14
Zhen-Ping Chen, Pei-Jing Li, Gang Li, Ling Tang, Ying-Zi Zhen, Xin-Yi Wu, Xiao-Ling Cheng, Koon Hung Luke, Victor S Blanchette, Man-Chiu Poon, Qiu-Lan Ding, Run-Hui Wu
Background: Although much attention has been paid to the pharmacokinetics (PKs) of different factor VIII (FVIII) concentrates in persons with hemophilia A (HA), limited information is available in young boys with severe HA. In this study, we aimed to assess the PK parameters of FVIII products in boys with severe HA in China. Methods: A total of 36 boys (plasma-derived [pd]-FVIII, n = 15; recombinant [r] FVIII, n = 21) were enrolled between January 2015 and May 2016 in Beijing Children's Hospital...
August 5, 2018: Chinese Medical Journal
https://www.readbyqxmd.com/read/29803274/evolving-complexity-in-hemophilia-management
#15
REVIEW
Stacy E Croteau
Rapid expansion of therapeutic options have increased the complexity of hemophilia care. Previously, on-demand therapy aimed to reduce morbidity and early mortality; however, now aggressive prophylaxis, particularly in children, encourages an active lifestyle. Accurate diagnosis, recognition of early threats to musculoskeletal health, and optimization of therapy are critical for both males and females affected by hemophilia. The diversity of emerging hemophilia therapies, from modified factor protein concentrates, to gene therapy, to nonfactor hemostatic strategies, provide an exciting opportunity to target unmet needs in the bleeding disorder community...
June 2018: Pediatric Clinics of North America
https://www.readbyqxmd.com/read/29735454/-prophylactic-treatment-with-low-and-intermediate-dose-factor-viii-in-children-with-severe-hemophilia-a-comprehensive-evaluation-of-joint-outcomes-and-correlation-analysis
#16
Jin-Mu Zhuang, Xue-Yan Sun, Xuan Zhou, Zhu-Qin Liu, Jing Sun
OBJECTIVE: To study the effect of low- and intermediate-dose factor VIII (FVIII) for prophylactic treatment of severe hemophilia A in children by comprehensively evaluating the outcomes of the joints. METHODS: Forty-seven children with severe hemophilia A (FVIII activity ≤2%) were enrolled in this study. Eighteen of the children received prophylactic treatment with low-dose FVIII (10 U/kg, 2-3 times a week), 20 received prophylactic treatment with intermediate-dose FVIII (15-30 U/kg, 3 times a week), and 9 received on-demand treatment with FVIII infusion when bleeding occurred according to the Chinese Expert Consensus on the Diagnosis and Treatment of Hemophilia...
April 20, 2018: Nan Fang Yi Ke da Xue Xue Bao, Journal of Southern Medical University
https://www.readbyqxmd.com/read/29723892/treatment-regimens-with-bypassing-agents-in-patients-with-hemophilia-a-and-inhibitors-a-survey-from-the-italian-association-of-hemophilia-centers-aice
#17
Antonio Coppola, Massimo Franchini, Giancarlo Castaman, Elena Santagostino, Cristina Santoro, Rita Carlotta Santoro, Massimo Morfini, Giovanni Di Minno, Angiola Rocino
The development of neutralizing antibodies (inhibitors) against infused factor VIII currently represents the main complication of replacement therapy in patients with severe hemophilia A. Inhibitors, indeed, particularly high-titer inhibitors (>5 BU/mL), greatly complicate the management of bleeding, exposing patients to an increased morbidity and mortality risk, thus representing a significant burden for physicians of Hemophilia Treatment Centers (HTCs). Although bypassing agents (i.e., activated prothrombin complex concentrate [APCC] and recombinant activated factor VII [rFVIIa]) are available for the treatment and prevention of bleeding in inhibitor patients, their efficacy, safety, and cost-benefit outcomes are poorly known in the long term and should be further improved...
September 2018: Seminars in Thrombosis and Hemostasis
https://www.readbyqxmd.com/read/29695583/relevance-of-abusive-head-trauma-to-intracranial-hemorrhages-and-bleeding-disorders
#18
James D Anderst, Shannon L Carpenter, Rodney Presley, Molly Curtin Berkoff, Allison P Wheeler, Robert F Sidonio, J Michael Soucie
BACKGROUND: Bleeding disorders and abusive head trauma (AHT) are associated with intracranial hemorrhage (ICH), including subdural hemorrhage (SDH). Because both conditions often present in young children, the need to screen for bleeding disorders would be better informed by data that include trauma history and are specific to young children. The Universal Data Collection database contains information on ICH in subjects with bleeding disorders, including age and trauma history. Study objectives were to (1) characterize the prevalence and calculate the probabilities of any ICH, traumatic ICH, and nontraumatic ICH in children with congenital bleeding disorders; (2) characterize the prevalence of spontaneous SDH on the basis of bleeding disorder; and (3) identify cases of von Willebrand disease (vWD) that mimic AHT...
May 2018: Pediatrics
https://www.readbyqxmd.com/read/29537123/first-year-results-of-an-expanded-humanitarian-aid-programme-for-haemophilia-in-resource-constrained-countries
#19
G F Pierce, A Haffar, G Ampartzidis, F Peyvandi, S Diop, M El-Ekiaby, H M van den Berg
INTRODUCTION: The gaps in haemophilia treatment around the world are enormous; approximately 60% of an estimated 475 000 individuals are not identified. Of the 187 000 diagnosed, 30% (57 000) access clotting factor replacement therapy. Since 1996, humanitarian aid distributed by the World Federation of Hemophilia (WFH) has played a minor, yet vital role providing life-saving clotting factor to countries in emergency situations. Donated amounts have been small and sporadic, often salvaging short-dated products, providing little opportunity to leverage donations with governments...
March 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/29505680/impact-of-hemophilia-b-on-quality-of-life-in-affected-men-women-and-caregivers-assessment-of-patient-reported-outcomes-in-the-b-hero-s-study
#20
Tyler W Buckner, Michelle Witkop, Christine Guelcher, Robert Sidonio, Craig M Kessler, David B Clark, Wendy Owens, Neil Frick, Neeraj N Iyer, David L Cooper
INTRODUCTION: Health-related quality of life (HRQoL) is impaired in patients with hemophilia; however, the impact in mild/moderate hemophilia B and affected women is not well characterized. OBJECTIVE: To evaluate factors that affect HRQoL in adults with hemophilia B and caregivers of affected children. METHODS: US adult patients and caregivers of affected children completed distinct ~1-hour online surveys including patient-reported outcome instruments...
June 2018: European Journal of Haematology
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