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hemophilia children

Patrícia Ribeiro Pinto, Ana Cristina Paredes, Armando Almeida
BACKGROUND: Hemophilia is a rare disorder characterized by spontaneous bleeding, with pain being a critical aspect. However, a systematic assessment of hemophilia-related pain in Portugal has never been conducted. OBJECTIVE: To understand the pain experience among Portuguese people with hemophilia (PWH) by describing its prevalence, characteristics, and impact and uncovering intervention needs in the realm of hemophilia-related pain care. METHODS: A cross-sectional observational survey, with age-adapted versions of questions concerning pain, emotional distress, and quality of life, was answered by 104 adults, 21 children/teenagers (10-17 years), and 19 children (1-9 years)...
February 27, 2019: Pain Medicine: the Official Journal of the American Academy of Pain Medicine
Nadine G Andersson, Elizabeth A Chalmers, Gili Kenet, Rolf Ljung, Anne Mäkipernaa, Hervé Chambost
The optimal mode of delivery for a pregnant hemophilia carrier is still a matter of debate. The aim of the study was to determine the incidence of intracranial hemorrhage and other major bleeds in neonates with moderate and severe hemophilia in relationship to mode of delivery and known family history. A total of 926 neonates, 786 with severe and 140 with moderate hemophilia were included in this PedNet multicentre study. Vaginal delivery was performed in 68.3% (n=633) and Cesarean section in 31.6% (n=293)...
February 21, 2019: Haematologica
Vildan Culha, Sibel Akpinar Tekgündüz, Hüsniye N Yarali, Bahattin Tunç, Namik Y Özbek
In this study, we aimed to investigate changes in calcium (Ca) metabolism in hemophilia patients (PWH). We also aimed to investigate the importance of diagnosis and treatment of factors impairing calcium metabolism and the significance of early diagnosis and prophylaxis with respect to these subjects. For all patients, serum calcium, phosphorus, alkaline phosphatase, 25 hydroxy vitamin D (25-OHD), parathormone (PTH), and calcitonin levels were evaluated. Bone mineral density (BMD) was assessed by dual-energy x-ray absorptiometry...
March 2019: Journal of Pediatric Hematology/oncology
Catherine Lambert, N' Dogomo Meité, Ibrahima Sanogo, Sébastien Lobet, Eusèbe Adjambri, Stéphane Eeckhoudt, Cedric Hermans
BACKGROUND: Little data is available on awareness of hemophilia carrier condition or associated bleeding risk and management in Sub-Saharan African countries. This study sought to identify hemophilia carriers in Côte d'Ivoire in order to collect data on demographics, bleeding phenotype, and laboratory results. Another purpose was to provide Ivorian hemophilia carriers with counseling on their risk of bleeding and of having children with hemophilia. A 12-month prospective study was conducted involving Ivorian hemophilia carriers recruited trough pedigree analysis pertaining to 81 hemophilia patients followed-up at the Yopougon Hemophilia Treatment Center in Abidjan...
February 1, 2019: Orphanet Journal of Rare Diseases
M Neale Weitzmann, Susanne Roser-Page, Tatyana Vikulina, Daiana Weiss, Li Hao, W Hunter Baldwin, Kanglun Yu, Natalia Del Mazo Arbona, Meghan E McGee-Lawrence, Shannon L Meeks, Christine L Kempton
Hemophilia A (HA), a rare X-linked recessive genetic disorder caused by insufficient blood clotting factor VIII, leaves affected individuals susceptible to spontaneous and traumatic hemorrhage. Although males generally exhibit severe symptoms, due to variable X inactivation, females can also be severely impacted. Osteoporosis is a disease of the skeleton predisposing patients to fragility fracture, a cause of significant morbidity and mortality and a common comorbidity in HA. Because the causes of osteoporosis in HA are unclear and in humans confounded by other traditional risk factors for bone loss, in this study, we phenotyped the skeletons of F8 total knockout ( F8 TKO ) mice, an animal model of severe HA...
February 12, 2019: Blood Advances
Claire L McKinley Yoder, Mary Ann Cantrell
PROBLEM: Graduation from high school is an important milestone for all adolescents and affects future health in adulthood. Children with chronic illnesses have additional challenges that affect school attendance, grade retention and graduation. If children with chronic conditions are not able to participate fully in education, this may limit their opportunities for future health. The aim of this study was to integrate the evidence in the past 28 years about educational outcomes of children and adolescents with chronic conditions causing disability...
January 22, 2019: Journal of Pediatric Nursing
Dalia M E El-Mikkawy, Mohja A Elbadawy, Shereen M Abd El-Ghany, Dalia Samaha
OBJECTIVE: To assess serum sclerostin levels in relation to severity of arthropathy and bone mineral density (BMD) in children with hemophilic arthropathy. METHODS: This cross-sectional study included 40 male children suffering from Hemophilia A, and 10 matched healthy controls. Assessment of factor VIII deficiency degree, frequency of bleeding, type of treatment, body mass index (BMI), disease severity using the Hemophilia Joint Health Score (HJHS) and lumbar spine (LS) Z score for bone mineral density (BMD) using dual-energy X-ray absorbiometry was done...
January 22, 2019: Indian Journal of Pediatrics
Azzah Alzahrani, Nada Othman, Tahani Bin-Ali, Huda Elfaraidi, Eman Al Mussaed, Fahad Alabbas, Qanita Sedick, Fatma Albatniji, Ziyad Alshahrani, Mohammed Asiri, Omar Alsuhaibani, Ghaleb Elyamany
Introduction: Preoperative coagulation screening tests in pediatric patients was once routine clinical practice globally and still used as standard practice in some countries before surgical procedures to assess of perioperative bleeding risk. Objective: The study aimed to evaluate unselected routine preoperative coagulation testing in children undergoing elective or invasive surgery to predict abnormal perioperative bleeding. The study also aimed to provide a rational approach of determining bleeding and family history of coagulation disorders as a predictive risk for bleeding...
2019: Clinical Medicine Insights. Blood Disorders
Amy Dunn, Jeremy Patterson, Charmaine F Biega, Alice Grishchenko, John Luna, Joseph R Stanek, Robert Strouse
BACKGROUND: Needles are frequently required for routine medical procedures. Children with severe hemophilia require intensive intravenous (IV) therapy to treat and prevent life-threatening bleeding and undergo hundreds of IV procedures. Fear of needle-related procedures may lead to avoidance of future health care and poor clinical outcomes. Virtual reality (VR) is a promising distraction technique during procedures, but barriers to commercially available VR platforms for pediatric health care purposes have prevented widespread use...
January 9, 2019: JMIR Serious Games
Hiroshi Asai, Rie Shirayama, Koichi Oshida, Yuko Honda, Tetsuji Sato, Michio Sakai, Koichi Kusuhara
Acquired hemophilia A (AHA), a bleeding disorder caused by autoantibodies against FVIII, has the potential for life-threatening bleeding. The annual onset rate is said to be one in 4 million people, but diagnosis examples increase in adults because a disorder concept penetrated. AHA is quite rare in children, with an incidence rate of 0.045 per 1 million, but early detection is crucial because serious bleeding can happen, as in adults. We report a pediatric case who received an early diagnosis of AHA by an activated partial thromboplastin time (APTT) cross-mixing test...
2018: Journal of UOEH
M Gavrel, A Rafowicz, R d'Oiron, S Franchi-Abella, T Lambert, C Adamsbaum
Hemarthroses and muscle bleeds are well-known and well-documented complications in pediatric and young adult hemophilia patients. In contrast, deep bleeds in atypical locations can be a diagnostic challenge, since clinicians and radiologists are often unfamiliar with their clinical and radiological features. Some atypical bleeds, however, can be life-threatening or severely disabling, highlighting the need for prompt, accurate diagnosis. Rare bleeds include central nervous system bleeds (including intracranial and spinal hematomas), urogenital bleeds, intra-abdominal bleeds (mesenteric and gastrointestinal wall hematomas) and pseudo tumors in unusual locations like the sinonasal cavities...
December 14, 2018: Diagnostic and Interventional Imaging
Qareen Haque, Yilifan Abuduaini, Huiping Li, Jianyun Wen, Xuedong Wu, Xiaqin Feng
BACKGROUND: Intracranial hemorrhage (ICH) is a life-threatening condition in children. Inherited bleeding disorders (IBD) have high risk of ICH. AIM: This single center study aims to identify the incidence, risk factors, and neurological outcome of ICH in children who suffer from IBD. METHODS: From 2005 to 2017, 241 children with IBDs from Nanfang hospital, Department of Pediatrics, were evaluated. The ICH episodes were identified by medical history, general physical examination, detailed neurological examination, and computed tomographic or magnetic resonance imaging examination...
December 14, 2018: Journal of Pediatric Hematology/oncology
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
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
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
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)
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
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...
December 2018: Transfusion and Apheresis Science
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
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
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