Evaluation of iron deposition and oxidative stress on renal function in children with beta-thalassemia major

Beta-Thalassemia is the commonest form of hemoglobinopathy in Egypt. Shortened red cell life span, rapid iron turnover and tissue deposition of excess iron are major factors responsible for functional and physiological abnormalities found in various forms of thalassemia. Increased deposition of iron had been found in renal parenchyma of thalassemic patients, but no systematic study of the effect of the deposits on renal functions has been available. The purpose of .this study is to describe the functional abnormalities of the kidney in patients with Beta- thalassemia and provide evidence that increased oxidative stress might be one of the factors responsible for the damage. Urine and serum samples from 40 patients with Beta-thalassemia were studied comparing with 10 age matched healthy controls. The patients were dividied into two groups: 22 with severe anaemia [hematocrite [Hct] < 25%] and 18 with moderate anaemia [Hct > 25%]. The result of this study showed that there was significant increase in serum iron, ferritin, urinary malondialedehyde [MDA] an indicator of lipid perioxidiation] and urinary B2 microglobulin [B[2]M]-level in all patients compared with normal children. Decrease in the ability to concentrate urine was detected by reduction in specific gravity in all thalassmic patients. Patients with severe anaemia had significantly higher serum iron, ferritin, urinary MDA and urinary B[2]M than those with moderate anaemia. There was positive correlation between MDA with level of serum ferritin and B[2]M .In conclusion the data indicated that patients have high prevelance of proximal tubular and medullary defects, we also demonstrate that the defects corrleate with the disease severity suggesting that oxidative stress might play a major role in the pathogenesis. Frequent urinary B[2]M estimation is recommended for early detection of tubular abnormalities

Evaluation of the role of plasma levels of thrombomodulin and endothelin-1 in hypertensive pregnant women

The objective of this study was to determine the maternal serum levels of thrombomodulin and endothelin-1 [Et-1] in pregnancy complicated by preeclampsia or gestational hypertension and in normal pregnancy. This clinical study included 40 pregnant women, 20 preeclamptic patients, 10 with gestational hypertension and 10 healthy pregnant women [the control group]. Plasma thrombomodulin and endothelin-1 [Et-1] levels were measure using an enzyme linked immunosorbent assay [ELISA] technique. There was a highly significant elevation of both thrombomodulin and endothelin-1 levels in preeclamptic group than in either gestational hypertensive and control groups [P <0.001]. There was a significant increase in the level of endothelin-1 and no change in thrombomodulin level in gestational hypertensive group when compared to the control group [P < 0.001 and P > 0.05 respectively]. There was a significant difference in endothelin-1 between cases with mild and severe gestational hypertension and also those with mild and severe preeclampsia, [P<0.001] such findings were not true for thrombomodulin. The study demonstrated that the plasma endothelin -1 level could be used as a molecular marker that reflects the vascular endothelial injury in cases of preeclampsia and gestational hypertension particularly those with severe cases. So, it is a good indicator of progression of the disease. On the other hand plasma thrombomodulin elevation comes late in the disease process. The increased level of plasma thrombomodulin in preeclampsia but not in gestational hypertension may suggest different underlying changes or disease enitities among those two pregnancy related hypertensive disorders