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https://www.readbyqxmd.com/read/28732370/silibinin-an-old-drug-for-hematological-disorders
#1
REVIEW
Hai Zou, Xing-Xing Zhu, Guo-Bing Zhang, Yuan Ma, Yi Wu, Dong-Sheng Huang
Silibinin (silybin), a non-toxic natural polyphenolic flavonoid, is the principal and the most biologically active component of silymarin. It is efficient in the treatment of acute and chronic liver disorders caused by toxins, drug, alcohol, hepatitis, and gall bladder disorders. Further, in our previous studies, we explored the anti-cancer efficacy in common cancers, such as lung, prostatic, colon, breast, bladder, as well as, hepatocellular carcinoma. Interestingly, silibinin is still not solely limited to the treatment of these diseases...
July 11, 2017: Oncotarget
https://www.readbyqxmd.com/read/28732253/diminished-ovarian-reserve-in-women-with-transfusion-dependent-beta-thalassemia-major-is-iron-gonadotoxic
#2
Aysel Uysal, Gül Alkan, Ayşegül Kurtoğlu, Onur Erol, Erdal Kurtoğlu
OBJECTIVE: Iron accumulation in the endocrine glands has been implicated in the aetiopathogenesis of decreased reproductive capacity in patients with beta-thalassemia major (β-TM). The aim of the current study was to investigate the serum concentration of anti-Müllerian hormone (AMH), a marker of ovarian reserve, in women with transfusion-dependent β-TM. STUDY DESIGN: In this case-control study, we recruited 43 women with transfusion-dependent TM and 44 age-matched healthy controls...
July 5, 2017: European Journal of Obstetrics, Gynecology, and Reproductive Biology
https://www.readbyqxmd.com/read/28730452/transcranial-color-doppler-in-stroke-free-adult-patients-with-sickle-cell-disease
#3
G Graziadei, F M Casoni, F Annoni, I Cortinovis, P Ridolfi, I Gandolfi, A Marcon, E Di Pierro, M D Cappellini
The threshold velocity ≥200 cm/s at transcranial Doppler (TCD) evaluation is a useful cut-off for preventing the stroke (STOP trial) in pediatric patients with sickle cell disease (SCD), term including different types of sickle genotypes. Scanty data are available for adult SCD patients. We compared intracranial blood flow velocities between adult SCD patients and controls using transcranial color Doppler (TCCD), measuring the peak of systolic velocity (PSV) with the insonation angle correction and the pulsatility index (PI), an indicator of endothelial elasticity...
July 20, 2017: Annals of Hematology
https://www.readbyqxmd.com/read/28727978/pulmonary-neuroendocrine-cell-hyperplasia-in-hemoglobin-bart-induced-hydrops-fetalis-a-model-for-chronic-intrauterine-hypoxia
#4
Mana Taweevisit, Boochit Theerasantipong, Kanlaya Taothong, Paul Scott Thorner
The pulmonary neuroendocrine system includes pulmonary neuroendocrine cells (PNECs) and neuroepithelial bodies (NEBs) that are distributed throughout respiratory epithelium and regulate lung growth and maturation antenatally. Abnormalities in this system have been linked to many hypoxia-associated pediatric pulmonary disorders. Hemoglobin (Hb) Bart disease is a severe form of α-thalassemia resulting in marked intrauterine hypoxia with hydrops fetalis (HF) and usually death in utero. Affected fetuses can serve as a naturally occurring human model for the effects of intrauterine hypoxia, and we postulated that these effects should include changes in the pulmonary neuroendocrine system...
July 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28721064/lived-experiences-of-iranian-parents-of-beta-thalassemia-children
#5
Aziz Shahraki-Vahed, Mohammadreza Firouzkouhi, Abdolghani Abdollahimohammad, Jamile Ghalgaie
INTRODUCTION: Thalassemia is a chronic blood disease, which imposes adverse effects on patients and their families. Parents of such patients, given that they had the thalassemia trait, hold themselves responsible for their children's disease in addition to other difficulties, bear the burden of guilt and hopelessness and worry about the health and future of their children. This study aimed to explore the lived experiences of parents of children with thalassemia. METHODS: The present research was conducted using a descriptive phenomenological approach...
2017: Journal of Multidisciplinary Healthcare
https://www.readbyqxmd.com/read/28719510/evaluation-of-the-clinical-and-laboratory-characteristics-of-previously-followed-up-thalassemia-intermedia-patients-to-provide-them-better-care-in-the-future
#6
Özlem Tüfekçi, Berna Atabay, Meral Türker, Şebnem Yilmaz Bengoa, Salih Gözmen, Tuba Karapinar, Hale Ören, Gülersu İrken
The increased awareness about the severity of complications in thalassemia intermedia patients led authorities to develop strategies for better management and follow-up of these patients. In this study, we aimed to define the clinical and laboratory characteristics in previously followed-up β-thalassemia intermedia patients and wanted to gain an insight about the follow-up of this patient population in a developing country to provide them better care in the future. The mean age at diagnosis was 4 years, and the mean hemoglobin was 7...
July 17, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28719386/is-there-a-standard-of-care-for-transfusion-therapy-in-thalassemia
#7
Massimo Franchini, Gian L Forni, Giancarlo M Liumbruno
PURPOSE OF REVIEW: Thalassemia is the most common form of inherited anemia, characterized by variable clinical phenotypes. The purpose of this review is to summarize the transfusion support in thalassemia patients and the management of transfusion-related iron overload. RECENT FINDINGS: The most recent evidence on transfusion strategy and iron chelation therapy in thalassemia arising from clinical trials as well as from recommendation guidelines are critically discussed...
July 14, 2017: Current Opinion in Hematology
https://www.readbyqxmd.com/read/28718348/thalidomide-has-a-significant-effect-in-patients-with-thalassemia-intermedia
#8
YunShuan Li, Quan Ren, Yali Zhou, Pingping Li, Wanhua Lin, Xiaolin Yin
OBJECTIVE: To investigate the effect of thalidomide in patients with thalassemia intermedia. METHODS: We observed the effect of thalidomide in seven patients with thalassemia intermedia requiring blood transfusion. Four of the patients were transfusion-independent, and three patients were transfusion-dependent. RESULTS: For the four transfusion-independent patients, hemoglobin concentration increased significantly (≥2 g/dl) in three and moderately (1-2 g/dl) in one...
July 18, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28713591/the-effect-of-partnership-care-model-on-mental-health-of-patients-with-thalassemia-major
#9
Afzal Shamsi, Fardin Amiri, Abbas Ebadi, Musab Ghaderi
BACKGROUND: Thalassemia major has become a public health problem worldwide, particularly in developing and poor countries, while the role of educating the family and community has not been considered enough in patients' care. OBJECTIVES: This study examines the impact of partnership care model on mental health of patients with beta-thalassemia major. MATERIALS AND METHODS: This experimental study, with pretest and posttest design, was performed on patients with beta-thalassemia major in Jiroft city...
2017: Depression Research and Treatment
https://www.readbyqxmd.com/read/28712490/pneumococcal-vaccination-for-splenectomized-patients-with-thalassemia-major-in-indonesia
#10
Teny Tjitra Sari, Arwin Ali P Akib, Djajadiman Gatot, Alida Roswita Harahap, Saptawati Bardosono, Sri Rezeki S Hadinegoro
INTRODUCTION: Streptococcus pneumoniae is a capsulated bacterium that can cause severe infection in patients with thalassemia major, particularly those who have undergone splenectomy. The absence of the spleen as well as zinc deficiency in splenectomized patients with thalassemia major increases the possibility of developing invasive pneumococcal infection. The aims of this study are to evaluate pneumococcal IgG levels following PCV and PPV immunizations and the effect of zinc supplementation on qualitative specific immune responses in splenectomized patients with thalassemia...
July 13, 2017: Vaccine
https://www.readbyqxmd.com/read/28707012/soluble-form-of-transferrin-receptor-1-level-is-associated-with-the-age-at-first-diagnosis-and-the-risk-of-therapeutic-intervention-and-iron-overloading-in-patients-with-non-transfusion-dependent-thalassemia
#11
Paolo Ricchi, Antonella Meloni, Silvia Costantini, Anna Spasiano, Tiziana Di Matola, Alessia Pepe, Patrizia Cinque, Aldo Filosa
We retrospectively evaluated the relationship between serum transferrin receptor-1 (sTfR1) and some fundamental events in the life and the management (the age at diagnosis, the age at the first red blood cells transfusion, the age at splenectomy, and the overall need of chelation therapy) of 111 patients with non-transfusion-dependent thalassemia (NTDT) subdivided in four genetic entities: patients with homozygous or compound heterozygous state for β-thalassemia, patients with triplicated α genotype associated with β heterozygosity, patients with deletional HbH, and patients with the combination of a β defect plus a β chain variant...
July 14, 2017: Annals of Hematology
https://www.readbyqxmd.com/read/28706555/advances-in-iron-chelation-therapy-transitioning-to-a-new-oral-formulation
#12
Nirmish R Shah
Iron overload is a concern for patients who require repeated red-blood-cell transfusions due to conditions such as sickle cell disease, thalassemia, or myelodysplastic syndromes. The recommended treatment for removing excess iron in these patients is iron chelation therapy. Currently available iron chelators include deferoxamine, which is administered by injection, and deferasirox and deferiprone, both of which are administered orally. Adherence to iron chelator therapy is an important consideration and may be affected by side effects...
2017: Drugs in Context
https://www.readbyqxmd.com/read/28706390/the-role-of-discriminant-functions-in-screening-beta-thalassemia-trait-and-iron-deficiency-anemia-among-laboratory-samples
#13
Ashwani Kumar, Debarshi Saha, Jyoti Kini, Nirupama Murali, Shrijeet Chakraborti, Deepa Adiga
INTRODUCTION: Most important differential diagnosis for microcytosis and hypochromia is beta thalassemia trait (BTT) and iron deficiency anemia. AIM: To study the utility of discriminant functions (DFs) and red cell indices in distinguishing BTT and iron deficiency anemia. METHODS: The study is observational (cross sectional). A total of 350 patients, 43 BTT, and 307 iron-deficiency anemia reflecting actual disease prevalence were included...
July 2017: Journal of Laboratory Physicians
https://www.readbyqxmd.com/read/28702825/olfactory-dysfunction-and-quality-of-life-in-patients-with-transfusion-dependent-thalassemia
#14
Yasin Yilmaz, Zeynep Karakas, Busra Uzun, Comert Sen, Senol Comoglu, Kadir Serkan Orhan, Selime Aydogdu, Ayse Ozkan Karagenc, Deniz Tugcu, Serap Karaman, Crystal Wylie, Richard L Doty
Transfusion-dependent thalassemia (TDT) is a group of thalassemia syndromes that require regular blood transfusions for survival. It is unknown whether the sense of smell of patients with TDT is affected, and if so, whether smell loss has an adverse effect on quality of life (QOL). Olfactory thresholds were measured using Snap & Sniff(®) wands. QOL was assessed via the Short Form-36 (SF-36) questionnaire. Forty-two TDT patients from the Thalassemia Center in Istanbul Medical Faculty were tested (mean age 26...
July 12, 2017: European Archives of Oto-rhino-laryngology
https://www.readbyqxmd.com/read/28698850/comparison-of-the-characteristics-of-two-hemoglobin-variants-hb-d-iran-and-hb-e-eluting-in-the-hb-a2-window
#15
Jasmita Dass, Aastha Gupta, Suchi Mittal, Amrita Saraf, Sabina Langer, Manorama Bhargava
BACKGROUND: Cation exchange-high performance liquid chromatography (CE-HPLC) is most commonly used to evaluate hemoglobin (Hb) variants, which elute in the Hb A2 window. This study aimed to assess prevalence of an uncommon Hb variant, Hb D-Iran, and compare its red cell parameters and peak characteristics with those of Hb E that commonly elutes in the Hb A2 window. METHODS: Generally, we assess abnormal Hb using CE-HPLC as the primary technique along with alkaline and acid electrophoresis...
June 2017: Blood Research
https://www.readbyqxmd.com/read/28696844/comparison-of-micrornas-mediated-in-reactivation-of-the-%C3%AE-globin-in-%C3%AE-thalassemia-patients-responders-and-non-responders-to-hydroxyurea
#16
Mohammad T Hojjati, Azita Azarkeivan, Ali A Pourfathollah, Naser Amirizadeh
Drug induction of Hb F seems to be an ideal therapy for patients with hemoglobin (Hb) disorders, and many efforts have been made to reveal the mechanism behind it. Thus, we examined in vivo expression of some microRNAs (miRNAs) that are thought to be involved in this process. Among β-thalassemia (β-thal) patients who were undergoing hydroxyurea (HU) therapy in the past 3 months and five healthy individuals, five responders and five non-responders, were also included in the study. Erythroid progenitors were isolated by magnetic activated cell sorting (MACS) and miRNA expression analyzed using reverse transcription-polymerase chain reaction (RT-PCR)...
March 2017: Hemoglobin
https://www.readbyqxmd.com/read/28694728/evaluation-of-staff-performance-and-interpretation-of-the-screening-program-for-prevention-of-thalassemia
#17
Simaporn Prommetta, Kanokwan Sanchaisuriya, Goonnapa Fucharoen, Supawadee Yamsri, Attawut Chaiboonroeng, Supan Fucharoen
INTRODUCTION: Thalassemia screening program has been implemented for years in Southeast Asia, but no external quality assessment program has been established. We have developed and initiated the proficiency testing (PT) program for the first time in Thailand with the aim to assess the screening performance of laboratory staff and their competency in interpretation of the screening results. MATERIALS AND METHODS: Three PT cycles per year were organized. From the first to the third cycle of the PT scheme, a total number of participant laboratories increased from 59 to 67...
June 15, 2017: Biochemia Medica: časopis Hrvatskoga Društva Medicinskih Biokemičara
https://www.readbyqxmd.com/read/28694195/hemoglobins-emerging-roles-in-mental-disorders-metabolical-genetical-and-immunological-aspects
#18
REVIEW
Meric A Altinoz, Bahri Ince
Hemoglobin (Hb) expression in the central nervous system is recently shown. Cooccurences of mental disorders (mainly bipolar disorder (BD) and tic disorders) with β- or α-thalassemia trait or erythrocytosis were witnessed, which may be due to peripheral or central hypoxia/hyperoxia or haplotypal gene interactions. β-Globin genes reside at 11p15.5 close to tyrosine hydroxylase, dopamine receptor DRD4 and Brain Derived Neurotrophic Factor, which involve in psychiatric diseases. α-Globin genes reside at 16p13...
July 8, 2017: International Journal of Developmental Neuroscience
https://www.readbyqxmd.com/read/28688717/detection-of-cardiac-iron-overload-with-native-magnetic-resonance-t1-and-t2-mapping-in-patients-with-thalassemia
#19
Rungroj Krittayaphong, Shuo Zhang, Pairash Saiviroonporn, Vip Viprakasit, Prajak Tanapibunpon, Chulaluk Komoltri, Wipaporn Wangworatrakul
BACKGROUND: To investigate the diagnostic performance of native cardiac magnetic resonance (CMR) T1 and T2 mapping for cardiac iron overload (CIO) in thalassemia patients. METHODS: All thalassemia patients who underwent CMR were enrolled on a clinical 1.5T scanner. Native T1 mapping with the Modified Look-Locker Inversion recovery (MOLLI) technique, T2 mapping using a black-blood multi-echo spin-echo technique, and conventional T2* mapping using multi-echo gradient-echo techniques were performed...
June 29, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28687959/stem-cell-therapy-for-fanconi-anemia
#20
Qing-Shuo Zhang
Stem cell therapy is the administration of stem cells to a patient to treat or prevent a disease. Since stem cells possess the long-term self-renewal capacity and provide daughter cells that differentiate into the specialized cells of each tissue, stem cell therapy will theoretically improve the disease condition for the lifetime of the patient. As the most widely used stem cell therapy, bone marrow transplantation is the treatment of choice for many kinds of blood disorders, including anemias, leukemias, lymphomas, and rare immunodeficiency diseases...
July 8, 2017: Advances in Experimental Medicine and Biology
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