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Hydroxyurea

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https://www.readbyqxmd.com/read/28328595/hemorrhage-and-infarction-of-the-conjunctiva-and-orbit-in-essential-thrombocythemia
#1
Jaehoon Na, Soo Youn Choi, Sehyun Baek, Hwa Lee
An 82-year-old woman who had a history of essential thrombocythemia presented with ocular pain, bleeding, and decreased visual acuity of the left eye. Orbital computed tomography revealed a relatively well-defined homogenous mass-like lesion in the left subconjunctival and intraconal space. Conjunctival biopsy showed acute inflammation with necrosis, vascular ectasia with thrombosis and hemorrhage. After the treatment with hydroxyurea and anagrelide, her symptom and lesion were markedly improved. Hematologic disorders like essential thrombocythemia should be considered in patients with severe spontaneous bleeding around the eye...
March 21, 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28321531/bone-quality-in-beta-thalassemia-intermedia-relationships-with-bone-quantity-and-endocrine-and-hematologic-variables
#2
Marina Baldini, A Marcon, F M Ulivieri, S Seghezzi, R Cassin, C Messina, M D Cappellini, G Graziadei
We report the first evaluation of bone quality in 70 thalassemia intermedia (TI) patients (37 males, 33 females, age 41 ± 12 years). Thirty-three patients (47%) had been transfused, 34 (49%) had been splenectomized, 39 (56%) were on iron chelation therapy, and 11 (16%) were on hydroxyurea. Mean hemoglobin was 9.2 ± 1.5 g/dl, median ferritin 537 ng/dl (range 14-4893), and mean liver iron concentration 7.6 ± 6.4 mg Fe/g dw. Fifteen patients (21%) had endocrinopathies, and 29 (41%) had vitamin D deficiency...
March 20, 2017: Annals of Hematology
https://www.readbyqxmd.com/read/28306171/barriers-to-hydroxyurea-adherence-and-health-related-quality-of-life-in-adolescents-and-young-adults-with-sickle-cell-disease
#3
Sherif M Badawy, Alexis A Thompson, Frank J Penedo, Jin-Shei Lai, Karen Rychlik, Robert I Liem
OBJECTIVES: To identify barriers to hydroxyurea adherence (negative beliefs, access, and/or recall barriers), and their relationship to adherence rates and health-related quality of life (HRQOL) amongst adolescents and young adults (AYA) with sickle cell disease (SCD). METHODS: A cross-sectional survey was administered to 34 AYAs (12-22 years old) in SCD clinics from January-December 2015. Study measures included Brief Medication Questionnaire, Modified Morisky Adherence Scale 8-items, Visual Analogue scale and Patient Reported Outcomes Measurement Information System...
March 17, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28304249/diagnosis-and-management-of-polycythemia-vera-in-a-ferret-mustela-putorius-furo
#4
Kim Le, Hugues Beaufrère, Laura L Bassel, Sarah Wills, Delphine Laniesse, Shauna L Blois, Dale A Smith
A 5-y-old female ferret (Mustela putorius furo) was evaluated for diarrhea, anorexia, and lethargy for 1 wk. Only mild dehydration was detected on physical examination. CBC analysis revealed marked erythrocytosis with an unremarkable plasma biochemistry panel; follow-up CBC analyses revealed a consistent primary erythrocytosis. Whole-body radiographs and abdominal ultrasonography were unremarkable except for a small nephrolith in the right kidney and a small cyst in the left kidney. The plasma erythropoietin level was 17...
December 1, 2016: Comparative Medicine
https://www.readbyqxmd.com/read/28301910/soluble-fms-like-tyrosine-kinase-1-as-a-link-between-angiogenesis-and-endothelial-dysfunction-in-pediatric-patients-with-%C3%AE-thalassemia-intermedia
#5
Azza Abdel Gawad Tantawy, Amira Abdel Moneam Adly, Eman Abdel Rahman Ismail, Omneya Ibrahim Youssef, Mohamed ElSayed Ali
Endothelial damage has been implicated in the pathogenesis of vascular complications in β-thalassemia intermedia (β-TI). Soluble fms-like tyrosine kinase 1 (sFLT-1) is a member of the vascular endothelial growth factor receptor (VEGFR) family. Soluble fms-like tyrosine kinase 1 is an antiangiogenic protein that induces endothelial dysfunction by adhering to and inhibiting VEGF and placenta growth factor. The aim of this study was to assess the level of sFLT-1 in 35 children and adolescents with β-TI, correlating it with markers of hemolysis and iron overload as well as cardiopulmonary complications...
January 1, 2017: Clinical and Applied Thrombosis/hemostasis
https://www.readbyqxmd.com/read/28298076/chronic-kidney-disease-in-the-bcr-abl1-negative-myeloproliferative-neoplasm-a-single-center-retrospective-study
#6
Seung-Woo Baek, Ji Young Moon, Hyewon Ryu, Yoon-Seok Choi, Ik-Chan Song, Hyo-Jin Lee, Hwan-Jung Yun, Samyoung Kim, Deog-Yeon Jo
Background/Aims: Renal complications related to BCR-ABL1-negative myeloproliferative neoplasms (MPNs) have not been examined fully in Asian populations. Methods: We analyzed estimated glomerular filtration rate (eGFR) and its changes with time retrospectively in patients with BCR-ABL1-negative MPN from 2005 to 2015. Results: The prevalence of chronic kidney disease (CKD) was 11% (6.6% having stage 3 and 4.4% having stage 4). In a linear regression analysis of eGFR versus time (years), overall, patients showed increased eGFR (mL/min/1...
March 17, 2017: Korean Journal of Internal Medicine
https://www.readbyqxmd.com/read/28293403/hydroxycarbamine-from-an-old-drug-used-in-malignant-hemopathies-to-a-current-standard-in-sickle-cell-disease
#7
REVIEW
Giovanna Cannas, Solène Poutrel, Xavier Thomas
While hydroxycarbamide (hydroxyurea, HU) has less and fewer indications in malignant hemopathies, it represents the only widely used drug which modifies sickle cell disease pathogenesis. Clinical experience with HU for patients with sickle cell disease has been accumulated over the past 25 years in Western countries. The review of the literature provides increasing support for safety and efficacy in both children and adults for reducing acute vaso-occlusive events including pain episodes and acute chest syndrome...
2017: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/28286320/earwax-a-neglected-body-secretion-or-a-step-ahead-in-clinical-diagnosis-a-pilot-study
#8
Engy Shokry, Anselmo Elcana de Oliveira, Melissa Ameloti Gomes Avelino, Mariana Moreira de Deus, Nelson Roberto Antoniosi Filho
This work combines the advantages of volatile metabolites profiling as a young growing research field with a non-invasive sampling technique using earwax "a neglected body secretion" for detection and monitoring of biomarkers for diabetes mellitus (types 1 and 2). Earwax samples were collected from 26 diabetic patients of both types, analyzed by headspace gas chromatography mass spectrometry and confronted to the volatile earwax composition of 33 healthy individuals. Data mining analysis was conducted using different models to discriminate the healthy individuals from the diabetic patients and to discriminate between both types of diabetes as well...
March 9, 2017: Journal of Proteomics
https://www.readbyqxmd.com/read/28273188/-treatment-options-and-limitations-in-the-management-of-myelofibrosis
#9
Gábor Körösmezey, Gabriella Gyõri, Gábor Rudas, Hanna Eid, Zsolt Nagy, Judit Demeter
Primary myelofibrosis has the worst outcome among classical chronic myeloproliferative neoplasms. The past decade has brought numerous discoveries elucidating the role of proliferative mutations in disease pathogenesis. Mutations of the genes JAK2, MPL and CALR are present in about 90 percent of all primary myelofibrosis cases. The prognosis of myelofibrosis is considered heterogeneous, the expected survival of patients may range from one year to more than a decade based on several prognostic factors. Estimated survival can be assessed based on clinical prognostic scores...
March 8, 2017: Magyar Onkologia
https://www.readbyqxmd.com/read/28270347/mortality-by-sickle-cell-disease-in-brazil
#10
Giovanna Abadia Oliveira Arduini, Letícia Pinto Rodrigues, Alessandra Bernadete Trovó de Marqui
This work aimed to characterize mortality by sickle cell disease in Brazil. The MEDLINE electronic database was searched using the terms 'mortality' and 'sickle cell disease' and 'Brazil' for articles published in the last five years aiming to provide a current analysis of the subject in question. Eight studies on mortality by sickle cell disease were carried out in the Brazilian states of Maranhão, Bahia, Minas Gerais, Rio de Janeiro and Mato Grosso do Sul. The majority of the deaths occurred in patients with sickle cell anemia, which is the most common genotype and causes the most severe clinical manifestation of the disease...
January 2017: Revista Brasileira de Hematologia e Hemoterapia
https://www.readbyqxmd.com/read/28270345/clinical-and-laboratory-profile-of-patients-with-sickle-cell-anemia
#11
Phelipe Gabriel Dos Santos Sant'Ana, Ariane Moreira Araujo, Cynthia Teixeira Pimenta, Mário Lúcio Pacheco Ker Bezerra, Sílvio Pereira Borges Junior, Viviana Martins Neto, Janaina Sousa Dias, Aline de Freitas Lopes, Danyelle Romana Alves Rios, Melina de Barros Pinheiro
OBJECTIVE: This study aimed to describe and analyze clinical and laboratory characteristics of patients with sickle cell anemia treated at the Hemominas Foundation, in Divinópolis, Brazil. Furthermore, this study aimed to compare the clinical and laboratory outcomes of the group of patients treated with hydroxyurea with those patients that were not treated with hydroxyurea. METHODS: Clinical and laboratorial data were obtained by analyzing medical records of patients with sickle cell anemia...
January 2017: Revista Brasileira de Hematologia e Hemoterapia
https://www.readbyqxmd.com/read/28270342/cholelithiasis-and-its-complications-in-sickle-cell-disease-in-a-university-hospital
#12
Raquel Alves Martins, Renato Santos Soares, Fernanda Bernadelli De Vito, Valdirene de Fátima Barbosa, Sheila Soares Silva, Helio Moraes-Souza, Paulo Roberto Juliano Martins
INTRODUCTION: The clinical manifestations of sickle cell disease are related to the polymerization of hemoglobin S. The chronic hemolysis caused by this condition often causes the formation of gallstones that can migrate and block the common bile duct leading to acute abdomen. OBJECTIVE: This study aimed to evaluate the profile of patients with sickle cell disease and cholelithiasis. METHODS: Patients with sickle cell disease were separated into groups according to the presence or absence of cholelithiasis...
January 2017: Revista Brasileira de Hematologia e Hemoterapia
https://www.readbyqxmd.com/read/28270341/a-phase-ib-open-label-randomized-safety-study-of-sanguinate%C3%A2-in-patients-with-sickle-cell-anemia
#13
Hemant Misra, James Bainbridge, John Berryman, Abraham Abuchowski, Kenneth Mauricio Galvez, Luis Fernando Uribe, Angel Luis Hernandez, Nestor Rodolfo Sosa
BACKGROUND: Treatment of sickle cell anemia is a challenging task and despite the well understood genetic and biochemical pathway of sickle hemoglobin, current therapy continues to be limited to the symptomatic treatment of pain, supplemental oxygen, antibiotics, red blood cell transfusions and hydroxyurea. SANGUINATE is a carbon monoxide releasing molecule and oxygen transfer agent under clinical development for the treatment of sickle cell anemia and comorbidities. METHODS: An open-label randomized Phase Ib study was performed in adult sickle cell anemia patients...
January 2017: Revista Brasileira de Hematologia e Hemoterapia
https://www.readbyqxmd.com/read/28259616/observational-retrospective-study-of-vascular-modulator-changes-during-treatment-in-essential-thrombocythemia
#14
Andrea Piccin, Michael Steurer, Clemens Feistritzer, Ciaran Murphy, Elva Eakins, Muriel Van Schilfgaarde, Daisy Corvetta, Angela Maria Di Pierro, Irene Pusceddu, Luigi Marcheselli, Roberto Gambato, Martin Langes, Dino Veneri, Omar Perbellini, Enrica Pacquola, Michele Gottardi, Filippo Gherlinzoni, Andrea Mega, Martina Tauber, Guido Mazzoleni, Elisa Piva, Mario Plebani, Mauro Krampera, Günther Gastl
Essential thrombocythemia (ET) patients are at risk of developing thrombotic events. Qualitative platelet (PLT) abnormalities and activation of endothelial cells (ECs) and PLTs are thought to be involved. Microparticles (MPs) can originate from PLTs (PMPs), ECs (EMPs), or red cells (RMPs). Previous studies have indicated that MPs contribute to ET pathophysiology. Endothelial modulators (eg, nitric oxide [NO], adrenomedullin [ADM], and endothelin-1 [ET-1]) are also involved in the pathophysiology of this condition...
February 14, 2017: Translational Research: the Journal of Laboratory and Clinical Medicine
https://www.readbyqxmd.com/read/28248149/effect-of-hydroxyurea-therapy-on-pulmonary-function-in-children-with-sickle-cell-anemia
#15
Anya McLaren, Michelle Klingel, Suman Behera, Isaac Odame, Melanie Kirby-Allen, Hartmut Grasemann
No abstract text is available yet for this article.
March 1, 2017: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/28245383/-effect-of-ifn-%C3%AE-on-cytokines-in-serum-of-patients-with-chronic-myeloid-leukemia
#16
Yi Wu, Zhi Cheng, Lin Shi, Lei Feng, Ying-Me Li, Dian-Liang Lyu
OBJECTIVE: To investigate the effect of IFN-α on cytokines in serum of patients with chronic myeloid leukemia(CML). METHODS: Fifty patients with CML from March 2012 to December 2015 in our hospital were randomly divided into routine treatment group (n=25) and combined treatment group (n=25), 30 healthy persons were selected as control (control group). The CML patients in routine treatment group were given orally hydroxyurea, the CML patients in combined treatment group were treated with recombinant human interferon α2b injection based on routine treatment (hydroxyurea plus IFN-α group)...
February 2017: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/28237610/visceral-and-bone-metastases-of-a-who-grade-2-meningioma-a-case-report-and-review-of-the-literature
#17
A Paix, W Waissi, D Antoni, R Adeduntan, G Noël
Meningiomas represent the most common tumours of the central nervous system in adults. Risk factors include ionizing radiation, female hormones exposure, head trauma, cell phone use, breast cancer and family history of meningioma. Despite complete surgical resection, natural history of meningiomas often includes local recurrence but very few metastatic meningiomas have been reported. Here, we report the case of a metastatic meningioma. A 43-year-old woman was firstly treated for a symptomatic parietal meningioma WHO grade II by surgical resection followed by an irradiation of the surgical bed...
February 22, 2017: Cancer Radiothérapie: Journal de la Société Française de Radiothérapie Oncologique
https://www.readbyqxmd.com/read/28237127/-prevalence-and-risk-factors-for-sickle-retinopathy-in-a-sub-saharan-comprehensive-sickle-cell-center
#18
A K Dembélé, B A Toure, Y S Sarro, A Guindo, B Fané, L Offredo, S Kené, I Conaré, O Tessougué, Y Traoré, Y Badiaga, M B Sidibé, D Diabaté, M Coulibaly, M Kanta, B Ranque, D A Diallo
INTRODUCTION: Retinopathy is a chronic complication with severe functional consequences in patients with sickle cell disease. Its prevalence is not well known in sub-Saharan Africa because of the absence of screening. We report here the results of a routine screening for sickle retinopathy in a Comprehensive Sickle Cell Center in Sub-Saharan Africa. METHODS: Screening of sickle retinopathy was carried out in all sickle cell patients aged 10 and over, followed between 2010 and 2012...
February 22, 2017: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/28230817/common%C3%A2-chemical%C3%A2-inductors%C3%A2-of%C3%A2-replication%C3%A2-stress-%C3%A2-focus%C3%A2-on%C3%A2-cell-based%C3%A2-studies
#19
REVIEW
Eva Vesela, Katarina Chroma, Zsofia Turi, Martin Mistrik
DNA replication is a highly demanding process regarding the energy and material supply and must be precisely regulated, involving multiple cellular feedbacks. The slowing down or stalling of DNA synthesis and/or replication forks is referred to as replication stress (RS). Owing to the complexity and requirements of replication, a plethora of factors may interfere and challenge the genome stability, cell survival or affect the whole organism. This review outlines chemical compounds that are known inducers of RS and commonly used in laboratory research...
February 21, 2017: Biomolecules
https://www.readbyqxmd.com/read/28221266/body-mass-index-and-the-association-with-vaso-occlusive-crises-in-pediatric-sickle-cell-disease
#20
Andrea Zivot, Nataly Apollonsky, Edward Gracely, Deepti Raybagkar
BACKGROUND: Children with sickle cell disease (SCD) historically have been underweight and have poor overall growth. Recent studies have demonstrated a trend toward obesity in pediatric SCD populations. MATERIALS AND METHODS: Through retrospective chart review of patients with SCD followed at our center, we collected patient's data, including body mass index (BMI), weight percentiles, sickle cell genotype, baseline hemoglobin, medical and psychiatric comorbidities, 25-hydroxy vitamin D level, treatment with hydroxyurea, and chronic transfusions...
February 17, 2017: Journal of Pediatric Hematology/oncology
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