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https://www.readbyqxmd.com/read/29453624/hydroxyurea-responses-in-clinically-varied-beta-hbe-beta-thalassaemia-and-sickle-cell-anaemia-patients-of-eastern-india
#1
Tridip Chatterjee, Amit Chakravarty, Sudipa Chakravarty
The haematological and clinical response to hydroxyurea was estimated in HbE-beta, beta thalassaemia and sickle cell anaemia patients of Eastern India, with variable clinical severity and transfusion requirement to determine whether hydroxyurea can help these patients to maintain their steady haemoglobin level without blood transfusions. Three hundred patients (189 HbE-beta thalassaemia, 95 beta thalassaemia and 16 other haemoglobinopathies including sickle cell anaemia) were selected for hydroxyurea therapy and were followed up for 48-60 months...
February 17, 2018: Annals of Hematology
https://www.readbyqxmd.com/read/29451224/hypogonadism-in-male-thalassemia-major-patients-pathophysiology-diagnosis-and-treatment
#2
Vincenzo De Sanctis, Ashraf T Soliman, Mohamed A Yassin, Salvatore Di Maio, Shahina Daar, Heba Elsedfy, Nada Soliman, Christos Kattamis
Failure of pubertal growth, delay or absence of sexual development, infertility and sexual dysfunction due to hypogonadism and defective spermatogenesis are frequent and well recognized disturbances among male patients with transfusion dependent (TD) thalassaemia major (β-thal). These problems are attributed mainly to the damage caused by chronic anaemia and the deposition of excess iron in the pituitary gland and testicles. This is a short review of male pubertal disorders in patients with β-thal written by pediatric endocrinologists and haematologists with an interest and active involvement, in the diagnosis and management of these complications in this group of patients...
February 16, 2018: Acta Bio-medica: Atenei Parmensis
https://www.readbyqxmd.com/read/29450569/pharmacogenomics-in-diabetes-outcomes-of-thiamine-therapy-in-trma-syndrome
#3
Abdelhadi M Habeb, Sarah E Flanagan, Mohamed A Zulali, Mohamed A Abdullah, Renata Pomahačová, Veselin Boyadzhiev, Lesby E Colindres, Guillermo V Godoy, Thiruvengadam Vasanthi, Ramlah Al Saif, Aria Setoodeh, Amirreza Haghighi, Alireza Haghighi, Yomna Shaalan, Andrew T Hattersley, Sian Ellard, Elisa De Franco
AIMS/HYPOTHESIS: Diabetes is one of the cardinal features of thiamine-responsive megaloblastic anaemia (TRMA) syndrome. Current knowledge of this rare monogenic diabetes subtype is limited. We investigated the genotype, phenotype and response to thiamine (vitamin B 1 ) in a cohort of individuals with TRMA-related diabetes. METHODS: We studied 32 individuals with biallelic SLC19A2 mutations identified by Sanger or next generation sequencing. Clinical details were collected through a follow-up questionnaire...
February 15, 2018: Diabetologia
https://www.readbyqxmd.com/read/29449170/a-case-of-severe-foetal-anaemia-due-to-anti-kell-that-could-not-be-detected-by-the-weekly-assessment-of-middle-cerebral-artery-peak-systolic-velocity
#4
Marie Deleers, Meriem Guizani, Jacques Jani, Martine Hulot, Hanane El Kenz
No abstract text is available yet for this article.
January 31, 2018: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/29449166/clinical-characteristics-and-treatment-outcomes-of-pulmonary-invasive-fungal-infection-among-adult-patients-with-hematological-malignancy-in-a-medical-centre-in-taiwan-2008-2013
#5
Chien-Yuan Chen, Wang-Huei Sheng, Feng-Ming Tien, Po-Chu Lee, Shang-Yi Huang, Jih-Luh Tang, Woei Tsay, Hwei-Fang Tien, Po-Ren Hsueh
BACKGROUND/PURPOSE: This study was aimed to investigate clinical characteristics and treatment outcomes of pulmonary invasive fungal infection (IFI) among patients with hematological malignancy. METHODS: All patients with hematological malignancy who were treated at a medical centre from 2008 to 2013 were evaluated. Pulmonary IFI was classified according to the European Organization for Research and Treatment of Cancer 2008 consensus. RESULTS: During the study period, 236 (11...
January 31, 2018: Journal of Microbiology, Immunology, and Infection, Wei Mian Yu Gan Ran za Zhi
https://www.readbyqxmd.com/read/29447888/pathogenesis-of-chronic-rhinosinusitis-in-patients-affected-by-%C3%AE-thalassemia-major-and-sickle-cell-anaemia-post-allogenic-bone-marrow-transplant
#6
F Martino, R Di Mauro, K Paciaroni, J Gaziev, C Alfieri, L Greco, R Floris, S Di Girolamo, M Di Girolamo
OBJECTIVES: Sickle cell anemia (SCA) and β -thalassemia major are well-recognized beta-globin gene disorders of red blood cells associated to mortality and morbidity included bone morbidities due to ineffective erythropoiesis and bone marrow expansion, which affect every part of the skeleton. While there are an abundance of described disease manifestations of the head and neck, the manner of paranasal sinuses involvement and its relations to β-thalassemia and SCA process was not studied yet...
March 2018: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/29446825/phytomedicines-medicines-derived-from-plants-for-sickle-cell-disease
#7
REVIEW
Oluseyi Oniyangi, Damian H Cohall
BACKGROUND: Sickle cell disease, a common recessively inherited haemoglobin disorder, affects people from sub-Saharan Africa, the Middle East, Mediterranean basin, Indian subcontinent, Caribbean and South America. It is associated with complications and a reduced life expectancy. Phytomedicines (medicine derived from plants in their original state) encompass many of the plant remedies from traditional healers which the populations most affected would encounter. Laboratory research and limited clinical trials have suggested positive effects of phytomedicines both in vivo and in vitro...
February 15, 2018: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/29444561/committed-to-be-fit-the-value-of-preoperative-care-in-the-perioperative-medicine-era
#8
Javier Ripollés-Melchor, Francesco Carli, Miquel Coca-Martínez, Macarena Barbero-Mielgo, José M Ramírez-Rodríguez, José A García-Erce
In the era of perioperative medicine, important advances have been made in the perioperative care of patients, usually within those known as Enhanced Recovery After Surgery (ERAS) protocols. These have led to a decrease in postoperative complications and the duration of hospital stays; however, there is still a lack of preoperative care, which could make perioperative medicine more prominent. Elderly patients, malnourished, anaemic and ones with a low physical function before surgery are likely to have sub-optimal recovery from surgery...
February 14, 2018: Minerva Anestesiologica
https://www.readbyqxmd.com/read/29441576/barleria-cristata-perspective-towards-phytopharmacological-aspects
#9
REVIEW
Harish Kumar, Rohini Agrawal, Vipin Kumar
OBJECTIVES: Barleria cristata (Family: Acanthaceae), commonly known as Philippine violet, is used in different ethnomedical systems for the treatment of a wide range of ailments. This review aimed to provide a scientific overview of B. cristata with reference to its ethnobotanical aspects, geographical distribution, medicinal uses, phytochemistry and pharmacological activity, and critical analyses research gaps and future research opportunities for investigations on this plant. KEY FINDINGS: Ethnomedical uses of the plant have been observed in lungs disorders, inflammatory conditions, toothache, anaemia, snake bite, diabetes and tuberculosis...
February 13, 2018: Journal of Pharmacy and Pharmacology
https://www.readbyqxmd.com/read/29441494/cost-minimisation-analysis-of-erythropoiesis-stimulating-agents-in-the-treatment-of-anaemia-in-dialysed-patients-a-pilot-study
#10
Astrid Darsonval, Virginie Besson, Claire Cavalin
OBJECTIVE: This study aimed to assess the cost impact of administering erythropoiesis-stimulating agents once every 4 weeks instead of one to three times a week to treat anaemia in patients undergoing dialysis. METHODS: This was a monocentric retrospective study involving 27 patients who underwent haemodialysis between 2009 and 2013 in a university hospital in Angers, France. The study was a cost-minimisation analysis from the hospital perspective. Only direct medical costs were considered...
September 2017: PharmacoEconomics Open
https://www.readbyqxmd.com/read/29440240/atypical-presentation-of-atypical-haemolytic-uraemic-syndrome
#11
Ratna Basak, Xiaotong Wang, Caitlin Keane, Robert Woroniecki
A 17-year-old girl presented with fever, myalgia, vomiting for 1 month and oliguria and dyspnoea for 4 days. She was tachycardic,hypertensive, with pedal oedema and decreased breath sounds. She had high serum creatinine (3 mg/dL), anaemia, thrombocytopenia, leucocytosis and eosinophilia with schistocytes. Lactate dehydrogenase, transaminases were high , with low haptoglobin and high ferritin (5269 ng/mL). Complement C3/C4 and fibrinogen were normal. Urinalysis showed large blood and protein and stool studies were negative...
February 11, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29438485/lower-than-expected-elevated-tricuspid-regurgitant-jet-velocity-in-adults-with-sickle-cell-disease-in-nigeria
#12
Baba Maiyaki Musa, Chisom N Odoh, Najibah A Galadanci, Hadiza Saidu, Muktar H Aliyu
Background: Cardiopulmonary disease is a major cause of morbidity and mortality in persons with sickle cell disease (SCD). Tricuspid regurgitant jet velocity (TRJV) and predicted forced expiratory volume in 1 s (FEV1%) predicted are independently associated with death in SCD. The goal of this study was to determine the prevalence of elevated TRJV and the association, if any, between TRJV and FEV1% predicted among persons with sickle cell anaemia (SCA) in Nigeria. Methods: Using a cross-sectional design, we enrolled 100 adult Nigerians (≥15 y) with SCA...
February 9, 2018: International Health
https://www.readbyqxmd.com/read/29438118/nsaids-and-the-small-bowel
#13
Foong Way David Tai, Mark E McAlindon
PURPOSE OF REVIEW: The review describes the effects of NSAID gastrointestinal toxicity on the small bowel, wherein injury is as prevalent as in the gastroduodenum. This is well documented by capsule endoscopy, which also provides an endoscopic endpoint for novel treatment strategies. RECENT FINDINGS: Appreciation of the prevalence of NSAID enteropathy has grown with capsule endoscopy and the use of composite end points that include obscure haemoglobin decreases in clinical studies...
February 12, 2018: Current Opinion in Gastroenterology
https://www.readbyqxmd.com/read/29437814/isolated-splenic-mucormycosis-in-a-case-of-aplastic-anaemia
#14
Shefali K Sharma, Prasanth Balasubramanian, Bishan Radotra, Manphool Singhal
Mucormycosis, a rare opportunistic infection seen in immunocompromised hosts, is caused by fungi of Mucorales family. It may be confined to the organs, such as rhinocerebral and pulmonary mucormycosis, or may cause disseminated infection. A 14-year-old boy presented to our clinic with fever and left upper quadrant abdominal pain, and on evaluation was found to have pancytopaenia, and imaging revealed ill-defined splenic collection with thrombus in the splenic vein. He was started on empirical intravenous antibiotics, followed by antifungals empirically as he did not show any improvement clinically...
February 8, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29435979/ibrutinib-alone-or-with-dexamethasone-for-relapsed-or-relapsed-and-refractory-multiple-myeloma-phase-2-trial-results
#15
Paul G Richardson, William I Bensinger, Carol Ann Huff, Caitlin L Costello, Nikoletta Lendvai, Jesus G Berdeja, Larry D Anderson, David S Siegel, Daniel Lebovic, Sundar Jagannath, Jacob P Laubach, Keith E Stockerl-Goldstein, Long Kwei, Fong Clow, Laurence Elias, Zeena Salman, Thorsten Graef, Elizabeth Bilotti, Ravi Vij
Novel therapies with unique new targets are needed for patients who are relapsed/refractory to current treatments for multiple myeloma. Ibrutinib is a first-in-class, once-daily, oral covalent inhibitor of Bruton tyrosine kinase, which is overexpressed in the myeloma stem cell population. This study examined various doses of ibrutinib ± low-dose dexamethasone in patients who received ≥2 prior lines of therapy, including an immunomodulatory agent. Daily ibrutinib ± weekly dexamethasone 40 mg was assessed in 4 cohorts using a Simon 2-stage design...
February 13, 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/29435872/a-phase-ii-trial-of-concurrent-chemoradiotherapy-with-weekly-paclitaxel-and-carboplatin-in-advanced-oesophageal-carcinoma
#16
Kuai-Le Zhao, Yi Xia, Yun-Hai Li, Yun Chen, Qi Liu, Jun-Hua Zhang, Jia-Ying Deng, Ta-Shan Ai, Han-Ting Zhu, Harun Badakhshi
BACKGROUND: This study was performed to assess the efficacy and feasibility of definitive chemoradiotherapy consisting of weekly doses of combined paclitaxel and carboplatin concurrent with radiation therapy, followed by 2 cycles of consolidation chemotherapy for advanced esophageal carcinoma. METHODS: Eligibility criteria included local, advanced, newly diagnosed and postoperative local regional lymph node metastasis; Eastern Cooperative Oncology Group (ECOG) score ≤ 2; and adequate organ function...
February 12, 2018: International Journal of Clinical Oncology
https://www.readbyqxmd.com/read/29435325/psychological-distress-iron-deficiency-active-disease-and-female-gender-are-independent-risk-factors-for-fatigue-in-patients-with-ulcerative-colitis
#17
Börje Jonefjäll, Magnus Simrén, Anders Lasson, Lena Öhman, Hans Strid
Background: Patients with ulcerative colitis often report fatigue. Objectives: To investigate prevalence of and risk factors for fatigue in patients with ulcerative colitis with active disease and during deep remission. Methods: In this cross-sectional study, disease activity was evaluated with endoscopy and calprotectin, and patients were classified as having active disease (n = 133) or being in deep remission (n = 155). Blood samples were analysed to assess anaemia, iron deficiency and systemic immune activity...
February 2018: United European Gastroenterology Journal
https://www.readbyqxmd.com/read/29435217/molecular-and-cellular-mechanisms-of-hif-prolyl-hydroxylase-inhibitors-in-clinical-trials
#18
Tzu-Lan Yeh, Thomas M Leissing, Martine I Abboud, Cyrille C Thinnes, Onur Atasoylu, James P Holt-Martyn, Dong Zhang, Anthony Tumber, Kerstin Lippl, Christopher T Lohans, Ivanhoe K H Leung, Helen Morcrette, Ian J Clifton, Timothy D W Claridge, Akane Kawamura, Emily Flashman, Xin Lu, Peter J Ratcliffe, Rasheduzzaman Chowdhury, Christopher W Pugh, Christopher J Schofield
Inhibition of the human 2-oxoglutarate (2OG) dependent hypoxia inducible factor (HIF) prolyl hydroxylases (human PHD1-3) causes upregulation of HIF, thus promoting erythropoiesis and is therefore of therapeutic interest. We describe cellular, biophysical, and biochemical studies comparing four PHD inhibitors currently in clinical trials for anaemia treatment, that describe their mechanisms of action, potency against isolated enzymes and in cells, and selectivities versus representatives of other human 2OG oxygenase subfamilies...
November 1, 2017: Chemical Science
https://www.readbyqxmd.com/read/29434716/%C3%AE-thalassaemia-combined-with-hereditary-spherocytosis-in-the-same-patient
#19
Xiaohong Li, Lin Liao, Xuelian Deng, Jian Huang, Zengfu Deng, Hongying Wei, Wuning Mo, Faquan Lin
A family of four from the Guangxi Zhuang Autonomous Region of China, including a child with α-thalassaemia and hereditary spherocytosis (HS), underwent laboratory identification, and genetic analysis. After harvesting peripheral blood samples from the child patient and his family members, GAP-polymerase chain reaction (PCR) and reverse dot-blot tests were used to identify thalassaemia genotypes. After amplifying exons and the adjacent introns of solute carrier family 4 member 1 (Diego blood group) (SLC4A1), ankyrin 1, spectrin α erythrocytic 1, spectrin β erythrocytic and erythrocyte membrane protein band 4...
February 2018: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/29434062/glucose-tolerance-insulin-secretion-sensitivity-characteristics-in-indian-children-with-cystic-fibrosis-a-pilot-study
#20
Vandana Jain, Santosh Kumar, Naval K Vikram, Mani Kalaivani, Surya Prakash Bhatt, Rajni Sharma, Kumar Kabra Sushil
Background & objectives: Cystic fibrosis (CF) is a life-limiting genetic condition resulting in chronic respiratory infections, pancreatic enzyme insufficiency and associated complications. This pilot study was undertaken to assess the glucose tolerance and insulin secretion and sensitivity among Indian children with CF. Methods: Children with CF under regular follow up at the Paediatric Pulmonology Clinic of a tertiary care hospital in New Delhi, India, were enrolled...
October 2017: Indian Journal of Medical Research
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