Zinc and Copper Status in Hemoglobin H Disease and β-Thalassemia/Hemoglobin E Disease

S, Vatanavicharn; Pringsulka, P.; Kritalugsana, S; Phuapairoj, P.; Wasi, P

doi:10.1159/000207000

Cited by 9 publications

(8 citation statements)

References 10 publications

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“…In another study, Kwan shows that while serum zinc level decreases and serum zinc binding capacity (ZnBC) increases in nutritional zinc deficiency, in thalassemic patients serum zinc level decreases but ZnBC does not increase simultaneously [22]. Though there are wide variations in different reports, these discrepancies can be attributed to differences in patients' ages, rather than to difference in treatment protocols, including differing transfusion rates or chelation therapies [22][23][24][25][26][27]. Some reports indicate that desferrioxamine increases urinary zinc excretion and may decrease zinc body content [8,9,28].…”

Section: Discussionmentioning

confidence: 99%

ZINC STATUS IN PATIENTS WITH MAJOR Β-Thalassemia

Mehdizadeh

Zamani

Tabatabaee

2008

Pediatric Hematology and Oncology

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To determine the zinc status in patients with major beta-thalassemia and its effect on their growth, the authors studied 64 thalassemic patients in comparison with 64 healthy matched individuals. Demographic and anthropometric data and history of the therapies were collected. Serum zinc level in both groups and ferritin in the thalassemic group were assigned. Interestingly, mean serum zinc level was significantly higher in the thalassemic group. No significant correlation between serum zinc level and short stature, serum ferritin level, desferrioxamine dose, initiating time of blood transfusion, and chelation therapy was found. The study indicates zinc deficiency in thalassemic patients who are on regular blood transfusion is rare and it seems that routine zinc supplementation is not necessary.

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Section: Discussionmentioning

confidence: 99%

ZINC STATUS IN PATIENTS WITH MAJOR Β-Thalassemia

Mehdizadeh

Zamani

Tabatabaee

2008

Pediatric Hematology and Oncology

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“…Alterations of zinc homeostasis in thalassemia are supported by a large number of clinical reports [29, 30, 31, 32, 33, 34]. Since an interaction between the intestinal absorption of iron and zinc has been described in animals and humans [47, 48], the possibility of an increased accumulation of zinc as well as iron in the tissues, as shown in the liver of patients with hereditary hemochromatosis [47], can be considered for TM.…”

Section: Discussionmentioning

confidence: 99%

“…It is known that a reduced plasma level of thymulin may reflect not only a defective production by the thymus but also a reduced activation of the hormone molecules by zinc ions, as it occurs in marginal or profound zinc deficiencies [27, 28]. Alterations of zinc homeostasis have been reported in TM conditions, although the results are somewhat contrasting between zinc plasma and cellular levels [29, 30, 31, 32, 33, 34]. As zinc is relevant to the efficiency of thymus and thymus-dependent functions [35, 36, 37], we measured the plasma level of both active and inactive thymic hormones in young patients affected with TM.…”

Section: Introductionmentioning

confidence: 99%

Immunological Evaluation of Patients with Beta-Thalassemia major

Consolini

Calleri²,

Legitimo³

et al. 2001

Acta Haematol

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Abnormalities in the immune system and zinc homeostasis in patients with β-thalassemia major (TM) have been reported. Since zinc ion is essential for the efficiency of the immune system and is required to induce biological activity to thymulin (Zn-FTS), a biochemically defined thymic hormone, we investigated the plasma levels of zinc and both active thymulin (Zn-FTS) and total zinc saturable thymulin (Zn-FTS+FTS) in 18 patients with TM aged between 2 and 31 years and 22 normal controls of the same age. Inhibitory molecules anti-thymulin and the distribution of lymphocyte subsets were also analyzed. Patients with TM presented significantly lowered plasma zinc and thymulin levels when compared to normal subjects. The significant enhancement of the active form of the hormone after zinc addition in vitro suggests that low thymulin values found in TM are due not to a thymic failure in synthesizing and secreting the thymic hormone, but a defect in zinc saturation of the hormone. An impairment of cell subset distribution was also demonstrated. This study shows that zinc and thymulin deficiency contribute to the complex mechanisms underlying immune dysfunction in TM.

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“…Zinc deficiency has been con firmed to occur in sickle cell disease and it was also found that a further decrease occurs in the zinc level during sickle cell crises [3]. Zinc deficiency was also reported in other related hemoglobin disorders such as /i-thalassemia [11] hemoglobin-H disease and in /Fthalassemia hemoglobin E disease [12]. Zinc defi ciency in sickle cell disease has been attributed to the hemolytic process associated with this disease and the release of RBC zinc into the plasma and consequently into the urine [2].…”

Section: Discussionmentioning

confidence: 99%

Zinc and Copper Status in Patients with Sickle Cell Anemia

et al. 1987

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Plasma zinc and copper concentrations were determined by atomic absorption spectroscopy in 57 patients with sickle cell anemia and in 45 control subjects from the Eastern Province of Saudi Arabia. Plasma zinc and copper levels in patients were found to be close to those of the control subjects. Similarly, there was a difference neither in urinary zinc level nor in the ratio Cu:Zn in patients and control subjects. This is in contrast to the situation which exists in North American Black subjects with sickle cell anemia, who are known to have zinc deficiency as well as a further decrease in zinc level during sickle cell crises. The near-normaLlevels of zinc and copper found in Saudi sickle cell patients therefore exclude zinc deficiency and confirm that this population exhibits a milder form of sickle cell anemia.

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Zinc and Copper Status in Hemoglobin H Disease and β-Thalassemia/Hemoglobin E Disease

Cited by 9 publications

References 10 publications

ZINC STATUS IN PATIENTS WITH MAJOR Β-Thalassemia

ZINC STATUS IN PATIENTS WITH MAJOR Β-Thalassemia

Immunological Evaluation of Patients with Beta-Thalassemia major

Zinc and Copper Status in Patients with Sickle Cell Anemia

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