A Arcasoy’s research while affiliated with Ankara Children's Hematology Oncology Training and Research Hospital and other places

This study has been undertaken to find out whether urinary zinc excretion, which is already increased in patients with thalassemia, is further increased by usual and high doses of desferrioxamine (DF). A total of 11 beta-thalassemia major patients were included. DF infusions have been performed with doses, either 50 mg/kg or 150 mg/kg. Nine age and sex matched normal children were taken as the control group. The mean basal-Zn excretion of the patients was significantly higher than the mean Zn excretion observed in controls. No significant difference is observed between the mean Zn excretion obtained on different doses of DF. However, they are both significantly higher than the mean basal-Zn levels of the controls.

Hemoglobin Beograd (B121 Glu-Val) is a rarely reported hemoglobin variant. It was first reported in Turkey in 1984. This report is a further observation of this variant in a 22-years old Turkish man.

Hemoglobin Pyrgos [β83(EF7) Gly→ASP] is a rare hemoglobin variant. This report describes the first observation of this variant in an 18-year-old Turkish girl living in Isparta.

Background: To evaluate the growth hormone reserve and the growth hormone response to recombinant human growth hormone (GH) in prepubertal thalassemic children with growth retardation. Methods: Twenty thalassemic patients with short stature and delayed bone age were studied. Patients were randomized into GH-treated (n=10) and non-GH treated (control; n=10) groups. The GH-treated group received recombinant human (rh)-GH (Genotropin) at the dose of 0.7 IU/kg per week for 12 months. Results: There was a significant discordance between GH response to pharmacologic stimuli and physiological secretion of GH/GHRH testing. Following the administration of rhGH, growth velocity increased from 2.47~0.48 cm/year to 6.27~0.76 cm/year (P=0.005), whereas there was not a similar change in the non-GH-treated group. The height velocities of the two groups during the 1 year follow-up period were significantly different (6.27~0.76 vs 3.99~0.34 cm/year; P=0.025). There were significant differences between the height velocity improvements and height velocity standard deviation scores of the two groups as well. Conclusion: The present study has demonstrated that rhGH is a safe and efficacious mode of treatment in thalassemic children.

Recently, it has been reported that serum zinc binding capacity (ZnBC) is a very important criterion to evaluate body zinc (Zn) status. It has been shown that chronic Zn deficiency occur in the patients with thalassemia major (TM). Zn deficiency in TM may cause hyperzincuria, high ferritin levels, hepatic iron load, hepatic dysfunction. This study was undertaken to determine serum Zn levels and ZnBC in different thalassemia forms and sickle cell disease (SCD). The study has been carried out on 30 Thalassemia Major (TM), 34 Thalassemia Intermedia (TI), 31 Thalassemia Trait (TT) and 10 SCD. As control group,13 healthy children and 20 adults were included. Serum Zn and ZnBC were determined by atomic absorption, then saturation index (SI%: serum Zn/ZnBC x 100) was calculated. Serum Zn levels in all patients were lower than control (p < 0.01). Serum ZnBC was at a normal level in patients with TT and TI but it was found to be lower in TM and SCD than control (p < 0.01). While serum Zn levels decrease and ZnBC increase in nutritionaL Zn deficiency, serum Zn levels decrease but ZnBC doesn't increase in patients with thalassemia.

Background: Marginal zinc deficiency has been the matter of interest in recent years and investigations are concentrated on it. Subjects with insufficient zinc consumption diet demonstrate systemic symptoms as reduced appetite, growth retardation in preschool children; immune deficiency, dermatological problems, delayed wound healing in geriatric population. In marginal zinc deficiency, diagnostic laboratory criteria are in normal range. Objective: For this reason, it is difficult to diagnose marginal zinc deficiency. Argemi et al. [1988] proposed to measure serum zinc binding capacity in the determination of zinc status of pregnant women. It is possible to establish the quantity of zinc binding to serum protein as serum iron capacity. We used the same method to diagnose marginal zinc deficiency of risky geriatric people. Methods: In 43 subjects, 25 female and 18 male above 65 years of age, living in "Seyran Baglari" Nursing Home, we established serum zinc, serum zinc per gram of serum protein, serum zinc binding capacity, serum zinc binding capacity per gram of serum protein. We included 20 healthy adult subjects as control group. Among the 23 subjects with zinc deficiency, 15 subjects agreed to zinc supplementation. Elementary Zn (ZnSO4) 30 mg/day was given for 90 days as zinc supplementation. All the parameters were repeated at the end of the second and the third months and 15 days after the medication was ceased. Results: In geriatric subjects, serum zinc and zinc per gram of serum protein were reduced; serum zinc binding capacity and zinc binding capacity per gram of serum protein were increased according to the control group. In 15 geriatric subjects supplemented with zinc preperation, serum zinc quantity demonstrated insignificant variation but serum zinc binding capacity was reduced significantly. Conclusion: In conclusion, serum zinc binding capacity seems to be an appropriate method for the diagnosis of marginal zinc deficiency.

In thalassemia, the reasons of growth retardation are tissue hypoxia due to insufficient blood transfusions, iron accumulation, somatomedin C deficiency, and chronic zinc deficiency. In our previous studies on thalassemia, we documented zinc deficiency in the presence of hyperzincuria. The cause of increased urinary zinc excretion in thalassemia is not clear. Zinc is known to bind avidly to certain amino acids, especially cystine and histidine. We investigated the urinary excretion of 13 amino acids and their possible role in hyperzincuria in thalassemic patients with serum amino acid assays. Mean urinary zinc excretion was found to be 1,263 ± 20.72 μg/24 hours, a value significantly higher than the normal urinary zinc excretion. In 24-hour urine samples, the levels of amino acids that have high affinity to zinc, as cystine, histidine, tyrosine, and lysine, were found to be higher than controls, and the increase in levels of serine and valine amino acids was significant when compared with controls (P < 0.01). In addition, serum levels of histidine, lysine, tyrosine, serine, valine, isoleucine tryptophane, and ornitine were increased (P < 0.01). It is concluded that selective amino aciduria may be one of the factors causing hyperzincuria in thalassemia and the mechanisms may be clarified by further studies in future. J. Trace Elem. Exp. Med. 13:199–204, 2000. © 2000 Wiley-Liss, Inc.

To evaluate the growth hormone reserve and the growth hormone response to recombinant human growth hormone (GH) in prepubertal thalassemic children with growth retardation. Twenty thalassemic patients with short stature and delayed bone age were studied. Patients were randomized into GH-treated (n = 10) and non-GH treated (control; n = 10) groups. The GH-treated group received recombinant human (rh)-GH (Genotropin) at the dose of 0.7 IU/kg per week for 12 months. There was a significant discordance between GH response to pharmacologic stimuli and physiological secretion of GH/GHRH testing. Following the administration of rhGH, growth velocity increased from 2.47 +/- 0.48 cm/year to 6.27 +/- 0.76 cm/year (P = 0.005), whereas there was not a similar change in the non-GH-treated group. The height velocities of the two groups during the 1 year follow-up period were significantly different (6.27 +/- 0.76 vs 3.99 +/- 0.34 cm/year; P = 0.025). There were significant differences between the height velocity improvements and height velocity standard deviation scores of the two groups as well. The present study has demonstrated that rhGH is a safe and efficacious mode of treatment in thalassemic children.