Chelation therapy in β-thalassemia major. I. Intravenous and subcutaneous deferoxamine

Graziano, Joseph H.; Markenson, Alicejane L.; Miller, Denis R.; Chang, Hung‐Hao; Bestak, Marc; Myers, Paul R.; Pisciotto, Patricia; Rifkind, Arleen B.

doi:10.1016/s0022-3476(78)80315-1

Cited by 61 publications

(29 citation statements)

References 10 publications

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“…As a result, we are intensifying our chelation efforts in patients with abnormal methoxamine challenge tests in an attempt to improve their clinical well-being. [35][36][37] We hope that the PES-DEs relation determined serially in patients with thalassemia major will prove predictive, and thus aid in the development of improved therapeutic alternatives for this disease, chronic LV volume overload from valvular regurgitation, and others in which progressive cardiomyopathy is a hallmark. Our preliminary results in patients receiving adriamycin indicate that this might be the case.38 39 …”

Section: End-systolic Pressure-dimension Relationmentioning

confidence: 99%

The left ventricular end-systolic pressure-dimension relation in patients with thalassemia major. A new noninvasive method for assessing contractile state.

Borow¹,

Propper²,

Bierman³

et al. 1982

Circulation

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SUMMARY Transfusion-dependent patients with thalassemia major (TM) are at an extremely high risk for cardiomyopathy. Traditional tests of left ventricular (LV) systolic function performed in these patients do not distinguish derangements in loading conditions from abnormalities in contractile state. In this study, we used the LV end-systolic pressure dimension (PES-DEs) relation, which is independent of preload, incorporates afterload and is highly sensitive to contractile state, to assess LV performance in 20 asymptomatic, chronically transfused patients, ages 7-25 years, with TM. All patients had normal resting systolic time intervals and exercise duration on treadmill. Baseline resting percent fractional shortening (% AD) on M-mode echocardiography (echo) was normal in 14 patients (group 1) and abnormal in six patients (group 2). Echo and carotid pulse recordings were made at rest and during i.v. infusion of methoxamine to alter LV afterload. DE was measured directly from echo; PES was estimated from a calibrated carotid pulse tracing. The study population consisted of 20 patients with thalassemia major followed at the Children's Hospital Medical Center. These patients, 12 males and eight females, were 7-25 years old and were treated by standard techniques until 3.0 ± 0.8 years (range 2-5 years) before this study, when they were begun on continuous deferoxamine therapy and a transfusion regimen that continually maintained their hemoglobin at levels greater than 12 g%. The transfusion load before the initiation of deferoxamine and hypertransfu-

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Section: End-systolic Pressure-dimension Relationmentioning

confidence: 99%

The left ventricular end-systolic pressure-dimension relation in patients with thalassemia major. A new noninvasive method for assessing contractile state.

Borow¹,

Propper²,

Bierman³

et al. 1982

Circulation

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“…Traditional therapy for this complication is iron chelating-agents that enhance the urinary excretion of irons. [14,15] Later these irons may be deposited in different tissues/organs of the patients getting red blood cells transfusions, usually more than 100 units of conventional packed red blood cells transfusions and produce siderosis and its sequel: growth failure, hypogonadism, diabetes mellitus, hepatic disease, cardiac failure and death.…”

Section: Introductionmentioning

confidence: 99%

Transfusion of Neocytes Concentrate/Pooled Neocytes and Conventional Packed Red Blood Cells in Beta-Thalassemia Major Patients: A Comparative Study

Kumbhakar¹

2016

jemds

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BACKGROUNDBeta-thalassemia major patients require continuous red blood cells transfusion therapy for maintaining their haemoglobin level between 10-12 gm/dL and survive. Each unit of red blood cells (About 180 ml) transfusion adds chances of transfusion related hazards and adds about 200-250 mg of extra irons to the recipients. Previous studies of neocytes concentrate/pooled neocytes transfusion demonstrated decreased numbers of required red blood cells transfusion by extending transfusion intervals, decreased incidences of transfusion related hazards and reduction of iron overload in these patients.

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“…β-thalassemia major is an autosomal recessive genetic disorder, which is clinically; the most severe form and the affected child is dependent on regular blood transfusion for survival [2]. The current management of β-thalassemia major patient is based on regular transfusion of packed red cells and effective chelating therapy [3][4][5][6].…”

Section: Introductionmentioning

confidence: 99%

Transfusion of neocytes concentrate/pooled neocytes in β-thalassemic patients

Sharma¹,

Rai²,

Agarwal³

et al. 2008

Indian J Hematol Blood Transfus

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Purpose of study Study was aimed to observe benefi ts of transfusion of neocytes/pooled neocytes over packed RBCs by super transfusion regimen in β-thalassemic patients and its comparison with related studies. Materials and methodsTwelve cases were selected and were equally divided into control and study group in which packed RBCs and neocytes were transfused respectively for one year. In study group, average 8 transfusions were required over 14 in control group.Results A marked extension of transfusion interval was observed in study group patients receiving neocytes transfusion (avg. 45.8 days; range, 32-54) over control group patients i.e. those receiving conventional packed RBCs (avg. 26.1; range 22-30).There is an average 40.8% reduction in the volume of RBCs required in the study group compared to control group and this difference is statistically significant (p = 0.00075). In study group, 20.1% reduction in iron overload and increase of iron by 9.6% in control group was reported. ConclusionWe observed that neocytes transfusion is effective in extending the transfusion interval as well as in reducing the blood requirement and iron over load.

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Chelation therapy in β-thalassemia major. I. Intravenous and subcutaneous deferoxamine

Cited by 61 publications

References 10 publications

The left ventricular end-systolic pressure-dimension relation in patients with thalassemia major. A new noninvasive method for assessing contractile state.

The left ventricular end-systolic pressure-dimension relation in patients with thalassemia major. A new noninvasive method for assessing contractile state.

Transfusion of Neocytes Concentrate/Pooled Neocytes and Conventional Packed Red Blood Cells in Beta-Thalassemia Major Patients: A Comparative Study

Transfusion of neocytes concentrate/pooled neocytes in β-thalassemic patients

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