Abstract
The tumor lysis syndrome (TLS) is a life-threatening complication caused by the massive release of nucleic acids, potassium and phosphate into the blood. This complication is the result of tumor cell lysis, which may occur due to treatment of drug sensitive and is characterized by rapid capacity of p...
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PMID: 21112673
PDF is available here.
Abstract
An investigation was conducted to evaluate the ability of Deferasirox (ICL670 or Exjade) following the distribution of cadmium salt in male Wistar rats. Cadmium was introduced to several groups of weanling male Wistar rats through different means, by act of drinking, feeding. A control group was fed...
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PMID: 20401682
PDF is available here.
Abstract
This study showed that the administration of DFO for 5 d a wk in combination with daily administration of DFP at 75 mg/Kg seemed to be the most efficacy and rapid method for reducing iron overload at liver and heart level. Furthermore, the use of different schedules of combined DFO and DFP administr...
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PMID: 20331740
PDF is available here.
Abstract
Bone disease in beta-thalassemia major (betaTM) remains poorly understood. Receptor activator of nuclear factor-kappabeta ligand (RANKL) regulates osteoclast formation and function. RANKL activity is balanced by interaction with its receptor (RANK) and binding to osteoprotegerin (OPG). L-Carnitine (...
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PMID: 20445416
PDF is available here.
Abstract
Use of complementary and alternative medicine (CAM) treatments have been increasing for children with autistic spectrum disorder (ASD). In this study, 38 Turkish parents of children with ASD were surveyed related with their use of CAM treatments, experiences, and views for each treatment. They menti...
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PMID: 19904598
PDF is available here.
Abstract
We assessed the feasibility and efficacy of chronic transfusion with an HbS target of 30% in children with SCD living in the Paris area.
We retrospectively studied 29 children aged 6.8 +/- 3.0 yr (3-15 yr) at inclusion who received chronic transfusion either because of abnormal TCD f...
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PMID: 19912310
PDF is available here.
Abstract
Provided that a multidisciplinary team is available, pregnancy is possible, safe and usually has a favorable outcome in patients with thalassemia. In women with hypogonadotropic hypogonadism, gonadal function is usually intact and fertility is usually retrievable....
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PMID: 19903676
PDF is available here.
Abstract
There are limited studies on renal involvement in beta-thalassemia, mainly involving patients on deferoxamine, reporting both glomerular and tubular dysfunction. The aim of the present study was to investigate renal involvement in young thalassemia patients, using both conventional and early markers...
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PMID: 20185899
PDF is available here.
Abstract
We propose a novel system of chelation therapy through the use of nanoparticles. Nanoparticles conjugated to chelators show unique ability to cross the blood-brain barrier (BBB), chelate metals, and exit through the BBB with their corresponding complexed metal ions. This method may provide a safer a...
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PMID: 20013176
PDF is available here.
Abstract
This investigation was aimed to study the effect of 2,3-dimercapto-1-propanesulfonic acid (DMPS) on mercuric chloride (HgCl(2))-induced alterations in urinary excretion of various carnitine fractions including free carnitine (FC), acylcarnitine (AC), and total carnitine (TC). Different groups of Wis...
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PMID: 20448264
PDF is available here.
Abstract
We have a wide armamentarium of PB but preferences of patients are poorly understood.
To study the patients' preferences and beliefs regarding PB and their influence on adherence and serum phosphate.
A cross-sectional cohort study was performed. A total of 121 hemodialysis patients answered a specif...
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PMID: 20613851
PDF is available here.
Abstract
We used magnetic resonance imaging (MRI) to compare the effect of iron chelation on liver, spleen and bone marrow. We examined 21 beta-thalassemic patients undergoing deferoxamine (DFO) (9/21) or combined therapy [DFO and deferiprone (L1), 12/21] with two abdominal MRI studies using T1-w/Pd-w/T2*-wG...
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PMID: 20113293
PDF is available here.
Abstract
Many patients who undergo hematopoietic cell transplantation (HCT) present with anemia and have received red blood cell transfusions before HCT. As a result, iron overload is frequent and appears to be particularly prominent in patients with myelodysplastic syndromes. There is evidence that peritran...
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PMID: 19863335
PDF is available here.
Abstract
The distribution of follow-up time was similar in the lanthanum carbonate and standard therapy groups (mean 23.7 versus 23.9 months [median 27.0 versus 26.0 months], respectively). Serum phosphorus levels were similar across treatment groups, as patients were treated to target. At follow-up, 19.9% (...
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PMID: 19845495
PDF is available here.
Abstract
The thalassemias are a group of inherited hematologic disorders caused by defects in the synthesis of one or more of the hemoglobin chains. Alpha thalassemia is caused by reduced or absent synthesis of alpha globin chains, and beta thalassemia is caused by reduced or absent synthesis of beta globin...
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PMID: 19678601
PDF is available here.
Abstract
Iron chelation is a treatment option not only for thalassemia patients, but also for those with lower-risk MDS who can be expected to need several years of transfusion therapy....
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PMID: 19727383
PDF is available here.
Abstract
Eighty seven patients with mean age of 10.0 +/- 4.33 years (range 4-27 years) were included in the study. Mean follow up was 8 +/- 3.94 months (range 2-12 months). The mean Ferritin at the start of study was 4656 +/- 2052.5 ug/L (range 1200-14630 ug/L) and at the end of study period was 4139 +/- 171...
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PMID: 19534375
PDF is available here.
Abstract
Preindex (baseline) characteristics were similar between the cohorts. Postindex numbers of prescription utilization, outpatient service utilization and hospitalizations were all higher (p < 0.0001) in diabetic CKD patients with SHPT compared to those without SPHT in both unadjusted and adjusted anal...
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PMID: 19590235
PDF is available here.
Abstract
CONTEXT: Lead poisoning due to retained gunshot bullets is a well-known clinical problem that is fairly frequently described in the literature. The risk factors for this occurrence relate mainly to whether the lead bullet is in contact with the joint fluid or cerebrospinal fluid (CSF). The treatment...
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PMID: 19466296
PDF is available here.
Abstract
Compared with PG, the lead level in the morning urine of TG with DMSA chelating was increased significantly. The level was peaked at (1246.96 +/- 157.91) microg/L on the first day after chelating. While the base line was (40.97 +/- 1.77) microg/L before chelating. Meanwhile, the BLLs were sharply de...
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PMID: 19534872
PDF is available here.
Abstract
We commence with a discussion on occupational manganism and clinical aspects of the disorder. This is followed by novel considerations on Mn transport (see also chapter by Yokel, this volume), advancing new hypotheses on the involvement of several transporters in Mn entry into the brain. This is fol...
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PMID: 19657747
PDF is available here.
Abstract
Thalassemia is endemic in Cyprus with a frequency of 1 in 6 persons being a heterozygote and about 1 in 1,000 a homozygous thalassemia major patient. Cyprus has been a pioneer nation in reducing and almost eliminating the number of births of thalassemia major patients by introducing prenatal and ant...
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PMID: 19814674
PDF is available here.
Hemoglobin (33 Suppl 1) 2009
Abstract
Chronic iron overload from frequent blood transfusions to treat patients with severe anemias leads to significant morbidity and mortality. Although desferrioxamine, the current standard of care, is an effective iron chelator with long-term evidence, it requires tedious subcutaneous infusion that ref...
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PMID: 20001633
PDF is available here.
Abstract
We report advances in thalassemia treatment in Taiwan and suggest treatment guidelines....
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PMID: 19814676
PDF is available here.
Abstract
We applied a standardized chelation protocol by stratifying transfusion-dependent thalassemic patients into three groups, namely well-chelated group (A), poorly-chelated group without (B) or with (C) risk of cardiac complications, based on their serum ferritin (SF) levels and magnetic resonance imag...
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PMID: 19814678
PDF is available here.
Abstract
Chronic blood transfusions are necessary for patients with hereditary anemia such as thalassemia, and for patients with myelodysplastic syndrome (MDS) who become anemic and transfusion-dependent. A common consequence of chronic transfusion is iron accumulation that can lead to organ damage. While th...
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PMID: 19814680
PDF is available here.
Abstract
Substantial progress in the use of chelating drugs for the treatment of iron overload and of non iron loading conditions has been presented during the 17th International Conference on Chelation (ICOC) held in November 2007 at Shenzhen, China. Major challenges lie ahead for the prevention and treatme...
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PMID: 19814673
PDF is available here.
Hemoglobin (33 Suppl 1) 2009
Abstract
In patients suffering from transfusion-dependent anemia, excess iron secondary to regular transfusions cannot be physiologically excreted. This leads to a state of chronic iron overload with iron accumulating in the liver, heart, and endocrine organs, and ultimately results in significant morbidity...
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PMID: 20001632
PDF is available here.
Abstract
Tissue damage caused by oxidative stress is a common characteristic of many conditions involving different major organs such as the brain, heart, liver and kidneys. The treatment of such conditions using classical antioxidants is not in most cases sufficient or effective because it lacks specificity...
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PMID: 19814684
PDF is available here.
Abstract
A new era in iron chelation therapy began with the successful removal of excess iron load and the maintenance of normal iron stores in thalassemia patients using the International Committee on Chelation (ICOC) protocols. This achievement was based on two phases, firstly the introduction of deferipro...
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PMID: 19814679
PDF is available here.
Abstract
Excess cardiac iron deposition leads to congestive cardiac failure and accounts for more than 70% of deaths in thalassemia major patients. In three separate studies involving 145 thalassemia patients, serum ferritin and magnetic resonance imaging (MRI) relaxation times T2 and T2* have been compared...
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PMID: 19814677
PDF is available here.
Sarah A SA Lowndes,
Avril A Adams,
Anthony A Timms,
Nita N Fisher,
Jon J Smythe,
Suzanne M SM Watt,
Simon S Joel,
Fernando F Donate,
Carolyn C Hayward,
Steven S Reich,
Mark M Middleton,
Andrew A Mazar and
Adrian L AL Harris
Abstract
Oral ATN-224 is a well-tolerated therapy and at a loading dose of 300 mg/d leads to a reduction of serum ceruloplasmin levels in 80% patients within 21 days. A loading dose of 300 mg/d for 2 weeks followed by a titrated maintenance dose will be the recommended starting dose for phase II study....
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PMID: 19010871
PDF is available here.
Abstract
In the United States the risk of lead exposure is far higher among poor, urban, and immigrant populations than among other groups. And even slightly elevated blood lead levels increase children's risk of significant neurobehavioral problems extending through adolescence. Research has shown that bloo...
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PMID: 18946264
PDF is available here.
Abstract
Predicting transfusion requirements relies both on epidemiology and therapeutic changes in hematology. The incidence rate of B-cell neoplasias especially non-Hodgkin lymphoma and myelodysplasia is increasing. Chemotherapy related myelodysplasia will reflect the improvement of solid tumor prognostic...
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PMID: 18951820
PDF is available here.
Abstract
We report a splenectomized patient, who was an excellent complier with chelation therapy, who before embarking on a pregnancy showed no evidence of iron overload, with normal cardiac, thyroid function and glucose metabolism. Laboratory findings showed ferritin 67 microg/L, myocardial T(2)* of 34 ms...
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PMID: 18462251
PDF is available here.
Abstract
We show that treatment of TBI rats with the zinc chelator calcium EDTA reduces the numbers of injured, Fluoro-Jade-positive neurons in the rat hippocampus 24 h after injury but does not improve neurobehavioral outcome (spatial memory deficits) 2 weeks post-injury. Our data suggest that zinc chelatio...
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PMID: 18556117
PDF is available here.
Abstract
A cross-sectional study of impaired glucose metabolism was carried out in 48 beta-thalassemic patients receiving hypertransfusions. An oral glucose tolerance test (OGTT) was performed using the method and criteria of the American Diabetes Association (ADA). Diabetes mellitus was diagnosed in two pat...
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PMID: 17899188
PDF is available here.
Abstract
The management of Beta Thalassemia, the commonest form of hemolytic anemia in children, has changed significantly in the last few years. With the availability of better transfusion regimen, iron chelation therapy, proper management of complications and good supportive care, it is now possible for a...
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PMID: 18716746
PDF is available here.
Amal El-Beshlawy,
Chantal Manz,
Mohammed Naja,
Mona Eltagui,
Claudia Tarabishi,
Ilham Youssry,
Hewida Sobh,
Mona Hamdy,
Iman Sharaf,
Azza Mostafa,
Olfat Shaker,
A V Hoffbrand and
Ali Taher
Abstract
Patients with thalassemia major requiring regular blood transfusions accumulate iron that is toxic to the heart, liver, and endocrine systems. The following prospective, randomized trial was carried out to determine the effectiveness, in children and young adults, of combined deferiprone (DFP) and d...
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PMID: 18351337
PDF is available here.
Luciana Scalone,
Lorenzo G Mantovani,
Marieke Krol,
Diana Rofail,
Simona Ravera,
Maria Grazia Bisconte,
Caterina Borgna-Pignatti,
Zelia Borsellino,
Paolo Cianciulli,
Domenico Gallisai,
Luciano Prossomariti,
Ippazio Stefà no and
Maria D Cappellini
Abstract
The management of beta-TM patients undergoing transfusions and ICT is efficacious, although costly, but overall benefits were not always perceived as optimal by patients. Efforts must be focused to improve patients' acceptance and satisfaction with their therapy; this would contribute to a better co...
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PMID: 18507891
PDF is available here.
Abstract
On average, it required slightly more than 1 year (382 days) for a child's BLL to decline to < 10 microg/dL, with the highest BLLs taking even longer. The BLLs of black children [hazard ratio (HR) = 0.84; 95% confidence interval (CI), 0.71-0.99], males (HR(male) = 0.83; 95% CI, 0.71-0.98), and child...
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PMID: 18629325
PDF is available here.
Abstract
Our results indicate that iron chelators significantly reduce myocardial iron content. Further, the choice of deferoxamine versus deferiprone may rest on factors other than their efficacy to reduce cardiac iron load....
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PMID: 18355381
PDF is available here.
Abstract
We report on a 12-year-old boy with Wilson's disease and extensive sub-cortical white matter involvement. After five years of copper chelating therapy, an appreciable improvement of these lesions was obtained. The physiopathology of these unusual cerebral white matter abnormalities is discussed....
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PMID: 18991198
PDF is available here.
Abstract
Whereas iron chelators have been proposed as therapeutic agents in stroke, changes in free iron levels have never been explored after focal brain ischemia. Therefore, free and total iron levels in cortical tissue and free iron levels in plasma were measured before and after (1, 4 and 24h) photothrom...
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PMID: 18485533
PDF is available here.
Abstract
We studied the efficacy of quercetin and a thiol chelating agent, monoisoamyl 2, 3-dimercaptosuccinic acid (MiADMSA) either individually or in combination against arsenic-induced oxidative stress and mobilization of metal in mouse. Animals were chronically exposed to 25 ppm arsenite as sodium arseni...
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PMID: 18183357
PDF is available here.