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Francesca Guidotti, Gioia Piatti, Alessia Marcon, Elena Cassinerio, Marianna Giuditta, Alberto Roghi, Valter Fasano, Dario Consonni, Maria Domenica Cappellini
Although pulmonary function abnormalities in thalassaemia major (TM) were described in 1980, the pathogenetic mechanism is not clear and data are contradictory, probably because of study heterogeneity and the multifactorial nature of the pathogenesis. We retrospectively analysed 73 adult TM patients to evaluate the prevalence of pulmonary dysfunction in adult TM and investigate relationships with iron load. All patients underwent body plethysmography and carbon monoxide diffusion (DLCO) was assessed in 63, in addition to blood tests, echocardiogram and T2* myocardial and liver magnetic resonance imaging...
October 21, 2016: British Journal of Haematology
Wai Cheng Foong, Jacqueline J Ho, C Khai Loh, Vip Viprakasit
BACKGROUND: Non-transfusion dependent beta thalassaemia is a subset of inherited haemoglobin disorders characterised by reduced production of the beta globin chain of the haemoglobin molecule leading to anaemia of varying severity. Although blood transfusion is not a necessity for survival, it is required when episodes of chronic anaemia occur. This chronic anaemia can impair growth and affect quality of life. People with non-transfusion dependent beta thalassaemia suffer from iron overload due to their body's increased capability of absorbing iron from food sources...
October 18, 2016: Cochrane Database of Systematic Reviews
Tsz Kin Lo
No abstract text is available yet for this article.
October 17, 2016: Journal of Obstetrics and Gynaecology: the Journal of the Institute of Obstetrics and Gynaecology
Mark W Lowdell, Amy Thomas
Advanced therapy medicinal products (ATMPs) represent the current pinnacle of 'patient-specific medicines' and will change the nature of medicine in the near future. They fall into three categories; somatic cell-therapy products, gene therapy products and cells or tissues for regenerative medicine, which are termed 'tissue engineered' products. The term also incorporates 'combination products' where a human cell or tissue is combined with a medical device. Plainly, many of these new medicines share similarities with conventional haematological stem cell transplant products and donor lymphocyte infusions as well as solid organ grafts and yet ATMPs are regulated as medicines and their development has remained predominantly in academic settings and within specialist centres...
October 17, 2016: British Journal of Haematology
Angela Vitrano, Giuseppina Calvaruso, Eliana Lai, Grazia Colletta, Alessandra Quota, Calogera Gerardi, Luciana Concetta Rigoli, Lorella Pitrolo, Liana Cuccia, Francesco Gagliardotto, Aldo Filosa, Vincenzo Caruso, Crocetta Argento, Saveria Campisi, Michele Rizzo, Luciano Prossomariti, Carmelo Fidone, Maria Fustaneo, Rosario Di Maggio, Aurelio Maggio
In the last few decades, the life expectancy of regularly transfused β-thalassaemia major (TM) patients has dramatically improved following the introduction of safe transfusion practices, iron chelation therapy, aggressive treatment of infections and improved management of cardiac complications. How such changes, especially those attributed to the introduction of iron chelation therapy, improved the survival of TM patients to approach those with β-thalassaemia intermedia (TI) remains unknown. Three hundred and seventy-nine patients with TM (n = 284, dead 40) and TI (n = 95, dead 13) were followed retrospectively since birth until 30 June 2015 or death...
October 17, 2016: British Journal of Haematology
Alice Trinchero, Marina Marchetti, Cinzia Giaccherini, Carmen J Tartari, Laura Russo, Anna Falanga
BACKGROUND: Patients with thalassaemia may have thromboembolic events and, even without thrombosis, they have a subclinical hypercoagulable state. In this setting, several coagulation laboratory abnormalities have been described, but thus far no studies have explored the contribution of platelet adhesive and procoagulant properties to blood clotting activation. In this study, we dissected the platelet procoagulant effect and influence of blood transfusions on haemostasis and platelet function in thalassaemic patients...
October 4, 2016: Blood Transfusion, Trasfusione del Sangue
Gabriella E Martyn, Kate G R Quinlan, Merlin Crossley
CCAAT boxes are motifs found within the proximal promoter of many genes, including the human globin genes. The highly conserved nature of CCAAT box motifs within the promoter region of both α-like and β-like globin genes emphasises the functional importance of the CCAAT sequence in globin gene regulation. Mutations within the β-globin CCAAT box result in β-thalassaemia, while mutations within the distal γ-globin CCAAT box cause the Hereditary Persistence of Foetal Haemoglobin, a benign condition which results in continued γ-globin expression during adult life...
October 5, 2016: Biochimica et Biophysica Acta
Patricia M Fortin, Karen V Madgwick, Marialena Trivella, Sally Hopewell, Carolyn Doree, Lise J Estcourt
This is the protocol for a review and there is no abstract. The objectives are as follows: To identify and assess the effectiveness of interventions to improve adherence to iron chelation therapy compared to standard care in people with SCD or thalassaemia including: identifying and assessing the effectiveness of different types of interventions (psychological and psychosocial, educational, medication interventions, or multi-component interventions);identifying and assessing the effectiveness of interventions specific to different age groups (children, adolescents, adults)...
September 2016: Cochrane Database of Systematic Reviews
Sarah Allegra, Silvia De Francia, Jessica Cusato, Elisa Pirro, Davide Massano, Antonio Piga, Antonio D'Avolio
OBJECTIVES: Deferasirox adverse effects include the following: gastrointestinal disturbance, mild elevations in serum creatinine levels and intermittent proteinuria; these events are dose-dependent and reversible with drug discontinuation, but this solution can lead to an inadequate iron chelation. For these reasons, interindividual variability of drug plasma concentration could help the clinical management of deferasirox dosage. We sought to describe deferasirox plasma exposure in a cohort of 60 adult patients...
September 27, 2016: Journal of Pharmacy and Pharmacology
Beuy Joob, Viroj Wiwanitkit
No abstract text is available yet for this article.
July 2016: Journal of Clinical and Diagnostic Research: JCDR
Ampaiwan Chuansumrit, Jiraporn Laothamathat, Nongnuch Sirachainan, Witaya Sungkarat, Pakawan Wongwerawattanakoon, Patrapop Kumkrua
BACKGROUND: MRI imaging is an alternative to serum ferritin for assessing iron overload in patients with thalassaemia disease. AIMS: To correlate liver iron concentration (LIC) determined by MRI and clinical and biochemical parameters. METHODS: An MRI study using cardiovascular magnetic resonance (CMR) tools to determine cardiac and liver iron was undertaken in adolescents with thalassaemia disease. RESULTS: Eighty-nine patients (48 males) with thalassaemia disease were enrolled...
August 2016: Paediatrics and International Child Health
Rizwan Ahmed Kiani, Muhammad Anwar, Usman Waheed, Muhammad Javaid Asad, Saleem Abbasi, Hasan Abbas Zaheer
Introduction. Transfusion Transmitted Infections (TTIs) continue to be a major risk in transfusions in many parts of the world. The transfusion-dependent β-thalassaemia patients are particularly at risk of acquiring TTIs. The current study was undertaken to estimate the prevalence of TTIs in transfusion-dependent β-thalassaemia patients. Material and Methods. A cross-sectional study of 1253 multitransfused thalassaemia major patients was conducted in five different centres of Islamabad, Rawalpindi, and Karachi...
2016: Journal of Blood Transfusion
Yukiyo Shimada, Nobuhiro Hidaka, Kana Maehara, Masaharu Murata, Yasuyuki Fujita, Kiyoko Kato
No abstract text is available yet for this article.
August 24, 2016: Journal of Obstetrics and Gynaecology: the Journal of the Institute of Obstetrics and Gynaecology
Sean Wl Ho, Ernest Bk Kwek
No abstract text is available yet for this article.
July 2016: Annals of the Academy of Medicine, Singapore
Bimal Kumar Agrawal, Saurabh Marwaha, Mini Bhatnagar, Shabir A Parry, Usha Agrawal
Systemic Lupus Erythematosus (SLE) is a multisystem chronic inflammatory disease of autoimmune aetiology. It has a predilection for female gender and presence of photosensitive rash over the sun exposed area gives a clue to the diagnosis. Diagnosis in a male patient with atypical manifestations is unusual and difficult. A 25-year-old male presented with fever, fatigue, vomiting, abdominal pain and loss of weight. He had sustained injury on his right arm following which he developed abscess at the trauma site and severe anaemia...
June 2016: Journal of Clinical and Diagnostic Research: JCDR
Stephanie J Snow
This article maps the history of health organisation across Greater Manchester (GM), primarily since the Second World War, to show how against a continuing backdrop of health inequalities, services have been driven (and constrained) by the needs and the politics of each period. Defining 'success' as benefits for patients the article identifies examples such as Salford's mental health services (1950s and 1960s), public health in North Manchester (1970s and 1980s), the creation of centres for diabetes, sickle-cell and thalassaemia (1980s) and the formation of the Joint Health Unit in 2002...
October 2, 2015: Representation (McDougall Trust)
Simon M Dyson, Waqar Iu Ahmad, Karl Atkin
The moral turn within sociology suggests that we need to be attentive to values and have a rapprochement with philosophy. The study of illness narratives is one area of sociology that has consistently addressed itself to moral domains but has tended to focus on stories of living with genetic or chronic illness per se rather than liminal states such as genetic traits. This article takes the case of genetic carriers within racialized minority groups, namely, those with sickle cell or thalassaemia trait, and takes seriously the notion that their narratives are ethical practices In line with the work of Paul Ricoeur, such storied practices are found to link embodiment, social relationships with significant others and wider socio-cultural and socio-political relations...
August 3, 2016: Health (London)
P Hariharan, S Colaco, R Colah, K Ghosh, A Nadkarni
INTRODUCTION: Mutations in the δ-globin gene are not pathogenically relevant, but co-inheritance of δ-globin variants along with β-globin gene defects can mask the diagnosis of β-thalassaemia trait. METHODS: Routine haematological parameters were carried out. Molecular analysis of β-globin gene mutations was carried out by CRDB, ARMS and DNA sequencing. δ- globin gene analysis was carried out by DNA sequencing. RESULTS: In this case study, we report a β-thalassaemia trait (IVS 1-5G→C) (HBB:c...
July 27, 2016: International Journal of Laboratory Hematology
Ch Asadov, Z Alimirzoyeva, M Hasanova, T Mammadova, A Shirinova
Research objective is to study the efficacy of recombinant erythropoietin (epoetin alfa) as alternative method of treatment beta-thalassemia intermedia. Study involved 58 patients with beta-thalassemia intermedia (23 women and 35 men). In all observed patients was defined levels of hemoglobin (Hb), red blood cells (RBC), erythrocyte indexes (MCV, MCH, MCHC), hemoglobin fractions (HbA, HbA2, HbF), serum ferritin, serum erythropoietin before and after administrated rEPO. All patients received rEPO during 6 month at the dose - 10000 IU subcutaneously...
June 2016: Georgian Medical News
Elisa Ferro, Angela Di Pietro, Giuseppa Visalli, Basilia Piraino, Carmelo Salpietro, Maria Angela La Rosa
OBJECTIVE: The hemojuvelin-bone morphogenetic protein axis is the principal iron-dependent mechanism of hepcidin regulation. The determination of soluble hemojuvelin (sHJV) levels could allow for a better understanding of the pathophysiological mechanisms of hepcidin regulation in thalassaemia. METHOD: We have assessed sHJV in 45 transfused and 15 untransfused thalassaemic patients in comparison with 15 healthy subjects, evaluating its relationships with some parameters of iron overload, anaemia and erythropoiesis...
July 21, 2016: European Journal of Haematology
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