世界規模でのグリーンパパイヤの研究、土壌細菌叢の研究

THE EFFECTS OF NATURALLY OCURRING ANTIOXIDANTS AND IRON CHELATORS ON THE OXIDANT / ANTIOXIDANT BALANCE IN THE ERYTHROCYTES AND LEUKOCYTES OF THALASSEMIA PATIENTS

Korkina Ludmila¹, Deeva Irina¹,

Afanas’ev Ilia², Ostrachovitch Elena², and Afans’ev Igor³

¹Russian State Medical University, Moscow, Russia, ²Institute of Pharmacology,

Moscow, Russia; ³Vitamin Research Institute, Moscow, Russia

It is reported in a number of works that patients suffering from  β -thalassemia are subjected to in vivo oxidative stress due to the excessive production of reactive oxygen species (ROS) by the reaction of abnormal hemoglobin with molecular oxygen. The crucial role of “free” iron ions bound to the membrane of β-thalassemia erythrocytes in the generation of hydroxyl radicals has also been shown. On these grounds, the application of iron chelators as essential part of the conventional therapy of iron-overload patients is thought to be relevant both in terms of iron removal from the body and accelerated discharge of ROS from these erythrocytes. To our knowledge, little is known about the capacity of circulating white blood cells (WBC) of patients with p-thalassemia to produce ROS or the effects of iron ions on WBC ROS-production and on its antioxidant enzymes. In this study, the erythrocytes of patients with β -thalassemia (major and intermedia) and of β -thalassemia gene carriers were found to produce more superoxide ions than the normal ones, either spontaneously or through induction by different quinones. This enhanced production of superoxide ion was stoichimetrically connected with methemoglobin formation and GSH oxidation. These three reactions were inhibited by iron chelators (Desferal and Deferipron) and by natural non-toxic antioxidants with chelating properties such as bioflavanoid rutin and Bio-Normalizer^®, a functional food produced by the fermentation of papaya fruits. The ROS production by the WBC of β -thalassemic homo­ and heterozygotes was markedly decreased in comparison with donor WBC. However, the sharp increase in the intracellular generation of ROS may have resulted from either an in vitro WBC incubation or the treatment of patients with iron chelators. Elevation in the level of intracellular ROS may be due to the activation of WBC NADPH-oxidase and a decrease of SOD and catalase activities in WBC acted upon by iron chelators. On the other hand, both the antioxidants rutin and Bio-Normalizer suppressed the excessive production of ROS in the WBC of thalassemia patients in a concentration- dependent manner.           Both the iron chelators and the natural antioxidants studied substantially enhanced the total antioxidant capacity of the plasma of thalassemic patients. These findings led us to suggest that a combination of iron chelators and antioxidants might be useful in protecting the erythrocytes and WBC of β -thalassemic patients against oxidative stress associated with genetic defect and iron chelation

REALIZATION OF TREATMENT WITH

DEFEOXAM IN TALASSEMIA MAJOR

Chang Lichun, Fan Yuhuan,

Department of Padiatrics, Redcross Hospital,

To No.1 Zhenhua Road, Shenzhen, Ghuandong, 518031 P.R. China

Object Deferoxam was used to treat thalassemia major with transfusion dependent iron-load, in order to avoid hematochromalosis. Method Deferoxam (Desferal, DFO) manufactured by Swiss was administered by intravenous injection, 30 – 50 mg/kg/day, 1 to 3 days continuously, to control serum ferroprotein under 1000g/L. Result Six β-thalassemia majors with long-term blood transfusion (a half years to three years) were treated with DFO, all of them kept normal growth and development without abnormal function of liver and renal. Conclusion Iron is overload in the body of thalassemia major, who required regular blood transfusion, hematochromalosis can be caused in such patients without administering DFO or trial incorrectly, which effected the growth and development seriously. It is still considered that, what is best dose in primary patients and if it is suitable for the patients with hematochromalosis or splenomegaly, or other methods can be used, and some other index need to be monitored.

                       A SURVEY OF IRON STATUS IN THALASSEMIA   

Youngrong Lai, Yu ying Lu, Guifang, et al

The First Affiliated Hospital of Guangxi Medical University,

Nanning, Guangxi, P.R. China 530021

Object To investigate the iron status in thalassemia patients in Guangxi. Methods            We studied 77 patients with β-thalassemia, 34 patients with hemoglobin H disease and 45 normal subjects. Serum ferritin (SF), free erythrocyte protoprophyrin (FEP), serum iron (SI), total iron-binding capacity (TIBC) and hemoglobin (HB) were determined. Result The levels of SF in 65 patients with β -thalassemia heterozygous were not different with those in the normal subjects (p>0.05).  Iron deficiency was

.