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https://www.readbyqxmd.com/read/30510462/phenotypical-variability-in-congenital-fvii-deficiency-follows-the-isth-ssc-severity-classification-guidelines-a-review-with-illustrative-examples-from-the-clinic
#1
REVIEW
Shilpa Jain, Jennifer Donkin, Mary-Jane Frey, Skye Peltier, Sriya Gunawardena, David L Cooper
Background: One of the most common rare inherited bleeding disorders, congenital factor VII (FVII) deficiency typically has a milder bleeding phenotype than other rare bleeding disorders. Categorizing severity in terms of factor activity associated with hemophilia (severe <1%, moderate 1%-5%, mild 6%-40%) has led to the observation that bleeding phenotype does not follow closely with FVII activity. Over the past decade, large-scale global registries have investigated bleeding phenotype more thoroughly...
2018: Journal of Blood Medicine
https://www.readbyqxmd.com/read/30507712/a-history-of-prophylaxis-in-haemophilia
#2
Manuel Moreno Moreno, Rubén Cuesta-Barriuso
Prophylaxis entails long-term continuous intravenous administration of concentrates of the deficient factor with a view to preventing spontaneous bleeds and the development of hemophilic arthropathy. Initiation of prophylaxis at an early age and continuous uninterrupted factor administration in patients with hemophilia have been hailed as essential by such organizations.The most widely used prophylaxis regimens include the Swedish (Malmö), the Dutch and the Canadian protocols. Different international groups have hailed prophylaxis as the most effective treatment in patients with hemophilia...
November 29, 2018: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/30473893/progressive-intramuscular-haematoma-in-a-12-year-old-boy-a-case-of-acquired-haemophilia-a
#3
Manori Gamage, Sadeepa Weerasinghe, Mohamed Nasoor, A M P W Karunarathne, Sashi Praba Abeyrathne
Acquired hemophilia A (AHA) is a rare bleeding disorder due to acquired antibodies against coagulation factor VIII (FVIII). It is rare in children less than 16 years old, and the incidence is 0.45/million/year. An otherwise healthy, 12-year-old boy was admitted to the ward with a history of swelling of the right and left forearms, for 1 day duration. He did not have any history of trauma or bleeding disorder. He had prolonged APPTT level with very high antibody titer against factor VIII. His gene expression for factor VIII was found to be normal...
2018: Case Reports in Hematology
https://www.readbyqxmd.com/read/30431602/musculoskeletal-ultrasonography-for-arthropathy-assessment-in-patients-with-hemophilia-a-single-center-cross-sectional-study-from-shanxi-province-china
#4
Cui-Ming Zhang, Jun-Feng Zhang, Jing Xu, Yu-Lin Guo, Gang Wang, Lin-Hua Yang
Magnetic resonance imaging (MRI) is currently considered the gold standard for assessing hemophilic arthropathy (HA) severity; however, MRI is often costly, time-consuming, and difficult to perform in children. In the present study, we evaluated the joint status of hemophilic patients from Shanxi Province, China, using musculoskeletal ultrasonography (MSKUS) and identified the factors that most strongly correlated with disease severity.The study included 104 patients with hemophilia, who underwent MSKUS examination...
November 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/30427100/measurement-of-joint-health-in-persons-with-haemophilia-a-systematic-review-of-the-measurement-properties-of-haemophilia-specific-instruments
#5
Samantha C Gouw, Merel A Timmer, Alok Srivastava, Piet de Kleijn, Pamela Hilliard, Marjolein Peters, Victor Blanchette, Kathelijn Fischer
INTRODUCTION: Accurate assessment of joint health in persons with haemophilia is crucial. Several haemophilia-specific measurement tools are available, but an overview of the measurement properties is lacking. AIM: To provide an overview of the measurement properties of haemophilia-specific measurement tools to assess clinical joint health. METHODS: MEDLINE and EMBASE were searched for reports on reliability, validity or responsiveness of the World Federation of Haemophilia Orthopedic Joint Score (WFH), Colorado Physical Examination Score (CPE), joint examination score by Petrini (PJS) and Hemophilia Joint Health Score (HJHS)...
November 14, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/30396835/management-of-acquired-hemophilia-a-review-of-current-evidence
#6
Janie Charlebois, Georges-Étienne Rivard, Jean St-Louis
Acquired hemophilia A (AHA) is a rare acquired bleeding disorder caused by autoantibodies against autologous factor VIII (FVIII). It is a disease that most commonly affects the elderly, but it has been described in children and during the post-partum period. It is idiopathic in 50% of cases and is associated with autoimmune disease, malignancy, pregnancy, infection or certain medications in the other 50%. The diagnosis should be suspected in patients with an isolated prolonged aPPT without previous personal or familial bleeding history...
October 30, 2018: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/30343446/an-update-on-the-role-of-rankl-rank-osteoprotegerin-and-wnt-%C3%A3-catenin-signaling-pathways-in-pediatric-diseases
#7
Giacomina Brunetti, Gabriele D'Amato, Mariangela Chiarito, Apollonia Tullo, Graziana Colaianni, Silvia Colucci, Maria Grano, Maria Felicia Faienza
BACKGROUND: Bone remodeling is a lifelong process due to the balanced activity of osteoclasts (OCs), the bone-reabsorbing cells, and osteoblasts (OBs), and the bone-forming cells. This equilibrium is regulated by numerous cytokines, but it has been largely demonstrated that the RANK/RANKL/osteoprotegerin and Wnt/β-catenin pathways play a key role in the control of osteoclastogenesis and osteoblastogenesis, respectively. The pro-osteoblastogenic activity of the Wnt/β-catenin can be inhibited by sclerostin and Dickkopf-1 (DKK-1)...
October 20, 2018: World Journal of Pediatrics: WJP
https://www.readbyqxmd.com/read/30341523/a-retrospective-analysis-of-intracranial-hemorrhage-in-children-with-hemophilia-a
#8
Qun Hu, Ai Zhang, Ai-Guo Liu, Song-Mi Wang, Ya-Qin Wang, Liu-Qing Zhang
To investigate the incidence, risk factors, clinical manifestations and prognosis of intracranial hemorrhage (ICH) in children with hemophilia A in a center of China, we conducted a retrospective analysis of 126 children with hemophilia A at our hospital in recent 4 years. Thirty-six children with hemophilia A (including 19 severe cases, and 17 moderate cases complicated with joint diseases) received low dose factor VIII (FVIII) prophylaxis, and none of them had ICH. However, 13 cases of hemophilia A not given prophylaxis were complicated with ICH (12 severe cases, and 1 moderate case) and demonstrated an incidence of 10...
October 2018: Current medical science
https://www.readbyqxmd.com/read/30339654/inpatient-health-care-utilization-in-children-with-hemophilia-before-and-after-the-joint-outcome-study-publication
#9
Meghan Drayton Jackson, Sarah H O'Brien, Joseph Stanek, Amy L Dunn, Bryce A Kerlin
The "Joint Outcomes Study" (JOS) demonstrated improved joint outcomes for patients receiving primary prophylaxis versus on-demand therapy. The impact of primary prophylaxis on inpatient health care utilization is not well-defined. To evaluate changes in hospitalization care of children with hemophilia before and after the 2007 JOS publication, this study utilized the Pediatric Health Information System (PHIS) to evaluate admissions for patients with hemophilia A or B (age, 2 to 7) admitted between January 2002 and 2006 (pre-JOS) and January 2010 and 2014 (post-JOS)...
October 18, 2018: Journal of Pediatric Hematology/oncology
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
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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...
December 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
#16
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 six 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)...
December 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
#17
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
#18
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
#19
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...
December 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
#20
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
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