Total antioxidant status in patients with major beta-thalassemia

Beta-thalassemia major is an autosomal recessive disease causing severe and hemolytic anemia, which begins about 2-6 months after birth. Iron overload, which arises from recurrent transfusion and ineffective erythropoiesis, can enhance oxidative stress in thalassemic patients. The aim of this study was to evaluate the serum total antioxidant capacity of patients with beta-Thalassemia major. Sixty six Iranian patients with beta-thalassemia major and 66 age-gender matched controls were evaluated for serum total antioxidant status [TAS], uric acid [UA], bilirubin and albumin. In addition, serum ferritin and transaminases were recorded in these subjects. Significant increases of TAS, UA, and bilirubin were observed in the patient group, compared with the control group [P<0.01]. Mean TAS and bilirubin in male patients was higher than in females [P=0.005 and P=0.008, respectively]. There was also direct correlation between TAS and albumin [P<0.001], bilirubin [P<0.001] and UA [P=0.002]. Endogenous antioxidants such as ferritin, UA and bilirubin can result in increased level of TAS in the patients with Beta-thalassemia major. Compensatory excess of TAS to oxidative stress could also be the reason for difference between our findings and previous studies

Neonatal giant cell hepatitis in an infant with cystic fibrosis

Cystic fibrosis is a hereditary disease of mucus and sweat glands characterized by respiratory infections and pancreatic insufficiency. We describe a girl infant with cholestasis as of the first clinical presentation at the age of 1 month. She developed severe anemia which required transfusion for several times. High level of direct bilirubin, low level of albumin and positive sweat tests were detected in laboratory tests. Histopathology report of liver biopsy indicated giant cell neonatal hepatitis. Although neonatal hepatitis is common cause of neonatal cholestasis, such condition is quite rare in cystic fibrosis, which easily could be misdiagnosed. Early diagnosis and appropriate treatment could prevent further complications of the disease